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Bokhari SFH, Faizan Sattar SM, Mehboob U, Umais M, Ahmad M, Malik A, Bakht D, Iqbal A, Dost W. Advancements in prenatal diagnosis and management of hypoplastic left heart syndrome: A multidisciplinary approach and future directions. World J Cardiol 2025; 17:103668. [DOI: 10.4330/wjc.v17.i3.103668] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/26/2024] [Revised: 02/12/2025] [Accepted: 02/27/2025] [Indexed: 03/21/2025] Open
Abstract
Hypoplastic left heart syndrome is a severe congenital defect involving underdeveloped left-sided cardiac structures, leading to significant mortality and morbidity. Prenatal diagnosis using fetal ultrasound and echocardiography enables early detection, family counseling, and improved clinical decision-making. Advanced prenatal interventions, such as fetal aortic valvuloplasty and atrial septostomy, show promise but require careful patient selection. A multidisciplinary approach involving obstetricians, neonatologists, and pediatric cardiologists is vital for effective management. Future directions include refining imaging techniques, such as three-dimensional ultrasound, cardiovascular magnetic resonance imaging, and exploring bioengineering solutions, stem cell therapies, and genetic research. These advancements aim to improve therapeutic options and address current limitations, including transplant scarcity and postoperative complications. Although surgical innovations have improved survival rates, challenges remain, including neurological risks and long-term hemodynamic issues. Ongoing research and technological advancements are essential to enhance outcomes and quality of life for hypoplastic left heart syndrome patients.
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Affiliation(s)
| | - Syed M Faizan Sattar
- Department of Medicine and Surgery, King Edward Medical University, Lahore 54000, Punjab, Pakistan
| | - Umair Mehboob
- Department of Medicine and Surgery, King Edward Medical University, Lahore 54000, Punjab, Pakistan
| | - Muhammad Umais
- Department of Medicine and Surgery, King Edward Medical University, Lahore 54000, Punjab, Pakistan
| | - Muhammad Ahmad
- Department of Medicine and Surgery, King Edward Medical University, Lahore 54000, Punjab, Pakistan
| | - Ahmad Malik
- Department of Medicine and Surgery, King Edward Medical University, Lahore 54000, Punjab, Pakistan
| | - Danyal Bakht
- Department of Medicine and Surgery, King Edward Medical University, Lahore 54000, Punjab, Pakistan
| | - Asma Iqbal
- Department of Medicine and Surgery, King Edward Medical University, Lahore 54000, Punjab, Pakistan
| | - Wahidullah Dost
- Curative Medicine, Kabul University of Medical Sciences, Kabul 10001, Kābul, Afghanistan
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Tulzer A, Hochpoechler J, Scharnreitner I, Tomek V, Weber R, Sames‐Dolzer E, Kreuzer M, Mair R, Mair R, Tulzer G. Postinterventional fetal aortic regurgitation: prevalence, outcome and effects on fetal circulation in large single-center cohort. ULTRASOUND IN OBSTETRICS & GYNECOLOGY : THE OFFICIAL JOURNAL OF THE INTERNATIONAL SOCIETY OF ULTRASOUND IN OBSTETRICS AND GYNECOLOGY 2025; 65:325-333. [PMID: 39954230 PMCID: PMC11872343 DOI: 10.1002/uog.29182] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Received: 07/06/2024] [Revised: 12/26/2024] [Accepted: 01/03/2025] [Indexed: 02/17/2025]
Abstract
OBJECTIVES To analyze the prevalence and severity of fetal aortic regurgitation (AR) after undergoing successful fetal aortic valvuloplasty (FAV) and to evaluate its effects on fetal circulation and left ventricular (LV) growth. METHODS This was a retrospective review of all fetuses with critical aortic stenosis who underwent successful FAV at our center between 2010 and 2024 for whom postnatal echocardiograms were available in digital format. Fetal and postnatal echocardiographic examinations were analyzed for ventricular and valvular dimensions and characteristics, and Z-scores were calculated for middle cerebral artery (MCA) pulsatility index (PI), umbilical artery (UA) PI and cerebroplacental ratio. AR severity was classified into no/mild AR or significant (moderate/severe) AR. The balloon-to-aortic valve ratio (BVR) was calculated as the ratio between the maximum actual balloon diameter and the aortic valve (AV) annulus diameter. The primary endpoints of this study were the prevalence, severity and risk factors for fetal AR following successful FAV. RESULTS Ninety-nine fetuses who underwent successful FAV were included. Immediate post-FAV echocardiograms showed that 87% of fetuses developed some degree of AR, including 45% of all fetuses with significant AR. BVR was significantly higher in fetuses with significant AR compared to those with no/mild AR (mean, 1.09 (95% CI, 1.06-1.12) vs 1.02 (95% CI, 0.99-1.04); P < 0.001). In a subgroup of 66/99 fetuses with available postnatal echocardiograms, the prevalence of AR decreased significantly from 86% before birth to 58% after birth (P < 0.001), with the proportion of fetuses with significant AR reducing from 47% before birth to 17% after birth (P < 0.001). In the overall cohort of fetuses, AV maximum velocity (Vmax) increased significantly from post-FAV to after birth (mean, 1.93 (95% CI, 1.75-2.11) m/s vs 3.21 (95% CI, 2.89-3.55) m/s; P < 0.001), regardless of AR severity, but Vmax after birth was lower in the significant-AR group compared with the no/mild-AR group (mean, 2.85 m/s vs 3.55 m/s; P = 0.020). Fetuses with significant AR exhibited higher relative LV length increases from immediately post-FAV to after birth than did those with no/mild AR (25% (95% CI, 16-33%) vs 14% (95% CI, 6-21%); P = 0.044), although there was no significant difference in mean LV length Z-score after birth between the two groups. FAV led to significant short-term increases in MCA-PI and UA-PI Z-scores, with greater increases observed in fetuses with significant AR. CONCLUSIONS FAV is associated with a high prevalence of fetal AR, which lessens in severity over the course of gestation. Significant fetal AR had the largest association with greater BVR and had significant impact on fetal hemodynamics. © 2025 The Author(s). Ultrasound in Obstetrics & Gynecology published by John Wiley & Sons Ltd on behalf of International Society of Ultrasound in Obstetrics and Gynecology.
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Affiliation(s)
- A. Tulzer
- Children's Heart Center Linz, Department of Pediatric CardiologyKepler University HospitalLinzAustria
- Medical FacultyJohannes Kepler University LinzLinzAustria
| | - J. Hochpoechler
- Children's Heart Center Linz, Department of Pediatric CardiologyKepler University HospitalLinzAustria
- Medical FacultyJohannes Kepler University LinzLinzAustria
| | - I. Scharnreitner
- Medical FacultyJohannes Kepler University LinzLinzAustria
- Department of Gynecology, Obstetrics and Gynecological EndocrinologyKepler University HospitalLinzAustria
| | - V. Tomek
- Children's Heart Center, Second Faculty of MedicineCharles University in Prague and Motol University HospitalPragueCzech Republic
| | - R. Weber
- Pediatric Heart Center ZurichUniversity Children's HospitalZurichSwitzerland
| | - E. Sames‐Dolzer
- Medical FacultyJohannes Kepler University LinzLinzAustria
- Children's Heart Center Linz, Division of Pediatric and Congenital Cardiac SurgeryKepler University HospitalLinzAustria
| | - M. Kreuzer
- Medical FacultyJohannes Kepler University LinzLinzAustria
- Children's Heart Center Linz, Division of Pediatric and Congenital Cardiac SurgeryKepler University HospitalLinzAustria
| | - R. Mair
- Medical FacultyJohannes Kepler University LinzLinzAustria
- Children's Heart Center Linz, Division of Pediatric and Congenital Cardiac SurgeryKepler University HospitalLinzAustria
| | - R. Mair
- Medical FacultyJohannes Kepler University LinzLinzAustria
- Children's Heart Center Linz, Division of Pediatric and Congenital Cardiac SurgeryKepler University HospitalLinzAustria
| | - G. Tulzer
- Children's Heart Center Linz, Department of Pediatric CardiologyKepler University HospitalLinzAustria
- Medical FacultyJohannes Kepler University LinzLinzAustria
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Dangel JH, Clur SA, Sharland G, Herberg U. Recommendations for the training and practice of fetal cardiology from the Association of European Paediatric Cardiology. Cardiol Young 2024:1-10. [PMID: 39526698 DOI: 10.1017/s1047951124025885] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/16/2024]
Abstract
Prenatal detection of cardiac abnormalities has increased significantly over the past few decades, such that fetal cardiology has developed into a sub-specialty of paediatric and congenital cardiology. As this speciality develops further and extends across Europe and more globally, it is important to standardize the requirements for training and subsequent practice, to optimize prenatal diagnosis and perinatal care. In addition to the knowledge and technical skills required to make a correct diagnosis, the counseling of families after diagnosis and the planning of appropriate perinatal management is equally important. The aim of these recommendations is to provide a framework for both basic and advanced training for paediatric cardiologists wishing to practice as fetal cardiologists, as well as highlighting requisites for a fetal cardiology service. All aspects regarding training in fetal cardiology and service provision are addressed including diagnosis, counseling and management.
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Affiliation(s)
- Joanna H Dangel
- Department of Perinatal Cardiology and Congenital Defects, Centre of Postgraduate Medical Education, Warsaw, Poland
| | - Sally-Ann Clur
- Department of Paediatric Cardiology, Emma Children's Hospital, Academic Medical Centre, Amsterdam University Medical Centres, Amsterdam, The Netherlands
| | - Gurleen Sharland
- Fetal Cardiology Unit, Department of Congenital Heart Disease, Evelina London Children's Hospital, London, UK
| | - Ulrike Herberg
- Department of Paediatric Cardiology, University Hospital Aachen, University of Aachen, Aachen, Germany
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Green L, Chan WX, Tulzer A, Tulzer G, Yap CH. Myocardial biomechanical effects of fetal aortic valvuloplasty. Biomech Model Mechanobiol 2024; 23:1433-1448. [PMID: 38683446 PMCID: PMC11436463 DOI: 10.1007/s10237-024-01848-0] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/23/2023] [Accepted: 04/07/2024] [Indexed: 05/01/2024]
Abstract
Fetal critical aortic stenosis with evolving hypoplastic left heart syndrome (CAS-eHLHS) can progress to a univentricular (UV) birth malformation. Catheter-based fetal aortic valvuloplasty (FAV) can resolve stenosis and reduce the likelihood of malformation progression. However, we have limited understanding of the biomechanical impact of FAV and subsequent LV responses. Therefore, we performed image-based finite element (FE) modeling of 4 CAS-eHLHS fetal hearts, by performing iterative simulations to match image-based characteristics and then back-computing physiological parameters. We used pre-FAV simulations to conduct virtual FAV (vFAV) and compared pre-FAV and post-FAV simulations. vFAV simulations generally enabled partial restoration of several physiological features toward healthy levels, including increased stroke volume and myocardial strains, reduced aortic valve (AV) and mitral valve regurgitation (MVr) velocities, reduced LV and LA pressures, and reduced peak myofiber stress. FAV often leads to aortic valve regurgitation (AVr). Our simulations showed that AVr could compromise LV and LA depressurization but it could also significantly increase stroke volume and myocardial deformational stimuli. Post-FAV scans and simulations showed FAV enabled only partial reduction of the AV dissipative coefficient. Furthermore, LV contractility and peripheral vascular resistance could change in response to FAV, preventing decreases in AV velocity and LV pressure, compared with what would be anticipated from stenosis relief. This suggested that case-specific post-FAV modeling is required to fully capture cardiac functionality. Overall, image-based FE modeling could provide mechanistic details of the effects of FAV, but computational prediction of acute outcomes was difficult due to a patient-dependent physiological response to FAV.
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Affiliation(s)
- Laura Green
- Department of Biomedical Engineering, Imperial College London, L2 Bessemer Building, South Kensington Campus, London, SW7 2AZ, UK
| | - Wei Xuan Chan
- Department of Biomedical Engineering, Imperial College London, L2 Bessemer Building, South Kensington Campus, London, SW7 2AZ, UK
- BHF Centre of Research Excellence, Imperial College London, London, UK
| | - Andreas Tulzer
- Department of Pediatric Cardiology, Children's Heart Center Linz, Kepler University Hospital, Linz, Austria
- Medical Faculty, Johannes Kepler University Linz, Altenberger Strasse 69, 4040, Linz, Austria
| | - Gerald Tulzer
- Department of Pediatric Cardiology, Children's Heart Center Linz, Kepler University Hospital, Linz, Austria
- Medical Faculty, Johannes Kepler University Linz, Altenberger Strasse 69, 4040, Linz, Austria
| | - Choon Hwai Yap
- Department of Biomedical Engineering, Imperial College London, L2 Bessemer Building, South Kensington Campus, London, SW7 2AZ, UK.
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Corroenne R, Meot M, Salomon LJ, Szezepanski I, Baghdadi H, Stos B, Levy M, Le Bidois J, Laux D, Gaudin R, Raisky O, Ville Y, Bonnet D, Stirnemann J, Malekzadeh-Milani S. Postnatal outcome following fetal aortic valvuloplasty for critical aortic stenosis. ULTRASOUND IN OBSTETRICS & GYNECOLOGY : THE OFFICIAL JOURNAL OF THE INTERNATIONAL SOCIETY OF ULTRASOUND IN OBSTETRICS AND GYNECOLOGY 2024; 64:339-347. [PMID: 38629579 DOI: 10.1002/uog.27658] [Citation(s) in RCA: 1] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Received: 10/19/2023] [Revised: 03/07/2024] [Accepted: 03/27/2024] [Indexed: 08/16/2024]
Abstract
OBJECTIVE To report our experience of fetal aortic valvuloplasty (FAV) for critical aortic stenosis (AS), with a focus on the postnatal evolution of the patients. METHODS This was a retrospective study including all fetuses with critical AS which underwent FAV in a single center between January 2011 and June 2022. FAV was performed under ultrasound guidance. Technical success was based upon balloon inflation across the aortic valve and improvement of the antegrade aortic flow across the aortic valve. At birth, a biventricular circulation (BVC) strategy was decided assuming the left ventricular (LV) systolic and diastolic function would ensure the systemic circulation. RESULTS Sixty-three FAV procedures were performed in 58 fetuses, at a median (range) gestational age of 26.2 (20.3-32.2) weeks. The procedure was technically successful in 50/58 (86.2%) fetuses. There were 11/58 (19.0%) cases of in-utero demise and 9/58 (15.5%) terminations of pregnancy. No patient was liveborn after an unsuccessful procedure. Thirty-eight (65.5%) infants were liveborn, at a median (range) gestational age of 38.1 (29.0-40.6) weeks, of whom 21 (55.3%) required prostaglandin treatment. Twenty-eight of the 38 (73.7%) liveborn children (48.3% of the study population) entered the BVC pathway at birth. Among them, 20 (71.4%) required an aortic valvuloplasty procedure at birth (11 (55.0%) percutaneous balloon, nine (45.0%) surgical) and eight (28.6%) did not require any treatment at birth, but, of these, five (62.5%) underwent surgical valvuloplasty between day 26 and day 1200 of age. Eleven (39.3%) of the infants with BVC at birth required a second intervention and four (14.3%) of them required a third intervention. Two (7.1%) infants who entered the BVC pathway at birth underwent conversion to univentricular circulation (UVC). None of the surviving children with BVC developed pulmonary hypertension. The overall survival rate in those with BVC at birth was 22/28 (78.6%) at a median (range) follow-up of 23.3 (2.0-112.6) months. Ten of the 58 (17.2%) patients had UVC at birth. Among these, six (60.0%) received compassionate care from birth and four (40.0%) underwent surgery. Three of the 10 patients who had UVC at birth were still alive at the latest follow-up assessment, at a median (range) gestational age of 24.3 (8.3-48.7) months. CONCLUSIONS FAV for critical AS led to increase of antegrade aortic flow in 86.2% of fetuses, with BVC being achieved in 48.3% (73.7% of the liveborn cases). Among patients with BVC at birth, the rate of reintervention was high, but 78.6% of these children were alive at the latest evaluation. © 2024 The Authors. Ultrasound in Obstetrics & Gynecology published by John Wiley & Sons Ltd on behalf of International Society of Ultrasound in Obstetrics and Gynecology.
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Affiliation(s)
- R Corroenne
- Department of Obstetrics, Necker-Enfants Malades Hospital, AP-HP, Paris, France
| | - M Meot
- M3C Pediatric Cardiology, Necker-Enfants Malades Hospital, AP-HP, Paris, France
| | - L J Salomon
- Department of Obstetrics, Necker-Enfants Malades Hospital, AP-HP, Paris, France
| | - I Szezepanski
- Department of Obstetrics, Necker-Enfants Malades Hospital, AP-HP, Paris, France
| | - H Baghdadi
- M3C Pediatric Cardiology, Necker-Enfants Malades Hospital, AP-HP, Paris, France
| | - B Stos
- M3C Pediatric Cardiology, Necker-Enfants Malades Hospital, AP-HP, Paris, France
- UE3C Pediatric Cardiology, Lowendal, Paris, France
| | - M Levy
- M3C Pediatric Cardiology, Necker-Enfants Malades Hospital, AP-HP, Paris, France
- UE3C Pediatric Cardiology, Lowendal, Paris, France
| | - J Le Bidois
- M3C Pediatric Cardiology, Necker-Enfants Malades Hospital, AP-HP, Paris, France
- UE3C Pediatric Cardiology, Lowendal, Paris, France
| | - D Laux
- M3C Pediatric Cardiology, Necker-Enfants Malades Hospital, AP-HP, Paris, France
- UE3C Pediatric Cardiology, Lowendal, Paris, France
| | - R Gaudin
- Department of Paediatric Cardiac Surgery, Necker-Enfants Malades Hospital, AP-HP, Paris, France
| | - O Raisky
- Department of Paediatric Cardiac Surgery, Necker-Enfants Malades Hospital, AP-HP, Paris, France
| | - Y Ville
- Department of Obstetrics, Necker-Enfants Malades Hospital, AP-HP, Paris, France
- Université de Paris Cité, Paris, France
| | - D Bonnet
- M3C Pediatric Cardiology, Necker-Enfants Malades Hospital, AP-HP, Paris, France
- Department of Paediatric Cardiac Surgery, Necker-Enfants Malades Hospital, AP-HP, Paris, France
- Université de Paris Cité, Paris, France
| | - J Stirnemann
- Department of Obstetrics, Necker-Enfants Malades Hospital, AP-HP, Paris, France
- Université de Paris Cité, Paris, France
| | - S Malekzadeh-Milani
- M3C Pediatric Cardiology, Necker-Enfants Malades Hospital, AP-HP, Paris, France
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Ren M, Chan WX, Green L, Buist ML, Yap CH. Biventricular finite element modeling of the fetal heart in health and during critical aortic stenosis. Biomech Model Mechanobiol 2024; 23:1331-1345. [PMID: 38589684 DOI: 10.1007/s10237-024-01842-6] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/12/2023] [Accepted: 03/15/2024] [Indexed: 04/10/2024]
Abstract
Finite Element simulations are a robust way of investigating cardiac biomechanics. To date, it has only been performed with the left ventricle (LV) alone for fetal hearts, even though results are likely different with biventricular (BiV) simulations. In this research, we conduct BiV simulations of the fetal heart based on 4D echocardiography images to show that it can capture the biomechanics of the normal healthy fetal heart, as well as those of fetal aortic stenosis better than the LV alone simulations. We found that performing LV alone simulations resulted in overestimation of LV stresses and pressures, compared to BiV simulations. Interestingly, inserting a compliance between the LV and right ventricle (RV) in the lumped parameter model of the LV only simulation effectively resolved these overestimations, demonstrating that the septum could be considered to play a LV-RV pressure communication role. However, stresses and strains spatial patterns remained altered from BiV simulations after the addition of the compliance. The BiV simulations corroborated previous studies in showing disease effects on the LV, where fetal aortic stenosis (AS) drastically elevated LV pressures and reduced strains and stroke volumes, which were moderated down with the addition of mitral regurgitation (MR). However, BiV simulations enabled an evaluation of the RV as well, where we observed that effects of the AS and MR on pressures and stroke volumes were generally much smaller and less consistent. The BiV simulations also enabled investigations of septal dynamics, which showed a rightward shift with AS, and partial restoration with MR. Interestingly, AS tended to enhance RV stroke volume, but MR moderated that down.
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Affiliation(s)
- Meifeng Ren
- Department of Biomedical Engineering, National University of Singapore, 4, Engineering Drive 3, E4-04-08, Singapore, 117583, Singapore
| | - Wei Xuan Chan
- Department of Bioengineering, Imperial College London, L2 Bessemer Building, South Kensington Campus, London, SW7 2AZ, UK
| | - Laura Green
- Department of Bioengineering, Imperial College London, L2 Bessemer Building, South Kensington Campus, London, SW7 2AZ, UK
| | - Martin L Buist
- Department of Biomedical Engineering, National University of Singapore, 4, Engineering Drive 3, E4-04-08, Singapore, 117583, Singapore.
| | - Choon Hwai Yap
- Department of Bioengineering, Imperial College London, L2 Bessemer Building, South Kensington Campus, London, SW7 2AZ, UK.
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Voges I, Hansen JH. Critical aortic stenosis - The long-term burden. Int J Cardiol 2024; 408:132134. [PMID: 38705208 DOI: 10.1016/j.ijcard.2024.132134] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/25/2024] [Accepted: 05/02/2024] [Indexed: 05/07/2024]
Affiliation(s)
- Inga Voges
- Department of Congenital Heart Disease and Pediatric Cardiology, University Hospital Schleswig-Holstein, Kiel, Germany; DZHK (German Centre for Cardiovascular Research), partner site Hamburg/Kiel/Lübeck, Kiel, Germany.
| | - Jan Hinnerk Hansen
- Department of Congenital Heart Disease and Pediatric Cardiology, University Hospital Schleswig-Holstein, Kiel, Germany; DZHK (German Centre for Cardiovascular Research), partner site Hamburg/Kiel/Lübeck, Kiel, Germany
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8
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Reitz JG, Meier JM, Berg C, Weber EC, Gembruch U, Wolter A, Sterzbecher V, Bedei I, Axt-Fliedner R. Two-dimensional speckle tracking echocardiography in fetuses with critical aortic stenosis before and after fetal aortic valvuloplasty. Arch Gynecol Obstet 2024; 310:817-824. [PMID: 38363396 DOI: 10.1007/s00404-024-07376-7] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/05/2023] [Accepted: 01/02/2024] [Indexed: 02/17/2024]
Abstract
BACKGROUND Critical aortic stenosis (AS) in fetuses may progress to hypoplastic left heart syndrome (HLHS) with need for postnatal single ventricular (SV) palliation. Fetal aortic valvuloplasty (FAV) is performed to achieve postnatal biventricular (BV) circulation. However, the impact of FAV on fetal myocardial function is difficult to measure. Prediction of postnatal circulatory status and, therefore, counseling is challenging. METHODS Retrospective study of fetuses with critical AS who underwent FAV. Global Longitudinal Peak Systolic Strain (GLPSS) of the left ventricle (LV) and right ventricle (RV) were retrospectively analyzed before and after intervention. Fisher's Exact Test and Mann-Whitney-U Test were used for univariant statistical analysis. RESULTS 23 fetuses with critical AS were included. After intervention fetuses demonstrated more negative LV-GLPSS mean values post- vs. pre-intervention (- 5.36% vs. - 1.57%; p < 0.05). RV-GLPSS was decreased in all fetuses, there was no peri-interventional change. 20 fetuses were born alive. Postnatally, 10 had BV and 10 SV circulation. Improved post-interventional LV-GLPSS strain values correlated with BV outcome (p < 0.05). Pre-interventional continuous LV-GLPSS values correlated with postnatal SV vs. BV outcome (p < 0.05). CONCLUSION In some fetuses, LV myocardial function assessed by speckle tracking echocardiography (STE) improves after FAV. Improved post-interventional LV-GLPSS correlates with biventricular postnatal outcome. Furthermore, pre-interventional LV- and RV-GLPSS correlate with postnatal outcome. Further studies are needed to asses, if pre-interventional STE parameters might predict which fetuses will benefit from FAV with postnatal BV circulation.
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Affiliation(s)
- Justus G Reitz
- Department of Cardiovascular Surgery, University Hospital Giessen, Justus-Liebig University, Giessen, Germany
| | - Johanna M Meier
- Division of Prenatal Medicine, Department of Obstetrics and Gynecology, University Hospital Giessen, Justus-Liebig University, Giessen, Germany
| | - Christoph Berg
- Department of Obstetrics and Prenatal Medicine, University Hospital Bonn, Bonn, Germany
| | - Eva C Weber
- Department of Obstetrics and Gynecology, University Hospital Cologne, Cologne, Germany
| | - Ulrich Gembruch
- Department of Obstetrics and Prenatal Medicine, University Hospital Bonn, Bonn, Germany
| | - Aline Wolter
- Division of Prenatal Medicine, Department of Obstetrics and Gynecology, University Hospital Giessen, Justus-Liebig University, Giessen, Germany
| | - Vanessa Sterzbecher
- Division of Prenatal Medicine, Department of Obstetrics and Gynecology, University Hospital Giessen, Justus-Liebig University, Giessen, Germany
| | - Ivonne Bedei
- Division of Prenatal Medicine, Department of Obstetrics and Gynecology, University Hospital Giessen, Justus-Liebig University, Giessen, Germany
| | - Roland Axt-Fliedner
- Division of Prenatal Medicine, Department of Obstetrics and Gynecology, University Hospital Giessen, Justus-Liebig University, Giessen, Germany.
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Qasim A, Morris SA, Belfort MA, Qureshi AM. Current Understanding of Indications, Technical Aspects and Outcomes of Fetal Cardiac Interventions. Interv Cardiol Clin 2024; 13:319-331. [PMID: 38839166 DOI: 10.1016/j.iccl.2024.03.005] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 06/07/2024]
Abstract
With the improvement in the detection of congenital heart disease in fetal life, fetal cardiac interventions are pushing the envelope in hopes of either altering the natural history of disease or improving survival in certain high-risk lesions. These interventions include fetal aortic valvuloplasty for evolving hypoplastic left heart syndrome, fetal atrial septoplasty with or without atrial septal stenting for hypoplastic left heart syndrome and variants with intact or severely restrictive atrial septum, and fetal pulmonary valvuloplasty for severe pulmonary stenosis or pulmonary atresia with intact ventricular septum. This review discusses their indications, technical aspects, and outcomes based on available literature.
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Affiliation(s)
- Amna Qasim
- Department of Cardiology, Arkansas Children's Hospital, University of Arkansas for Medical Sciences, Little Rock, AR, USA
| | - Shaine A Morris
- The Lillie Frank Abercrombie Division of Cardiology, Texas Children's Hospital, Baylor College of Medicine, Houston, TX, USA
| | - Michael A Belfort
- Department of Obstetrics and Gynecology, Texas Children's Hospital, Baylor College of Medicine
| | - Athar M Qureshi
- CE Mullins Cardiac Catheterization Laboratories, The Lillie Frank Abercrombie Division of Cardiology, Texas Children's Hospital, Baylor College of Medicine, Internal Medicine/Cardiology, Baylor St. Luke's Medical Center, 6651 Main Street, E 1920, Houston, TX 77030, USA.
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Grinenco S, Aiello HA, Meller CH, Lucini V, Nápoli N, Trentacoste L, Córdoba A, Saenz Tejeira M, Osuna JM, Barretta J, Villa AB, Marantz P, Otaño L. Fetal aortic valvuloplasty as the first step in a complex therapeutic strategy. Prenat Diagn 2024; 44:739-746. [PMID: 38666896 DOI: 10.1002/pd.6579] [Citation(s) in RCA: 1] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/30/2024] [Revised: 04/02/2024] [Accepted: 04/11/2024] [Indexed: 06/17/2024]
Abstract
BACKGROUND Fetal aortic valvuloplasty (FAV) is proposed to prevent hypoplastic left heart syndrome due to fetal critical aortic stenosis. OBJECTIVE to report our experience on FAV as the first step in a complex therapeutic strategy. METHOD Series of patients with FAV over an 18-year period. RESULTS 27 FAVs were performed in 26 fetuses, with technical success in 82% (22/27) and periprocedural fetal demise in 22% (6/27), decreasing to 15% in the second half-cohort. Loss to follow-up was due to birth or postnatal therapy in other centers (5) and termination of pregnancy (1), A normal-sized LV at birth was observed in 46% (6/13), 4 neonates underwent aortic valvuloplasty and 2 cardiac surgeries, with 5/6 achieving biventricular circulation at 28 days, and 3 transplant-free survival at mid-term follow-up. The 7/13 born with a borderline LV underwent LV rehabilitation strategy, with survival at 28 days in 4/7 and at mid-term in 3: one with biventricular circulation, one with a ventricle-and-a-half repair, and one lost to follow-up. CONCLUSION FAV was feasible in most cases, with no maternal complications, and biventricular circulation at 28 days in ∼40% of survivors. After FAV, a diverse range of postnatal cardiac interventions are performed, reflecting the challenging innovation in current cardiovascular therapy.
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Affiliation(s)
- Sofía Grinenco
- Pediatric Cardiology Service, Hospital Italiano de Buenos Aires, Instituto Universitario Hospital Italiano, Buenos Aires, Argentina
| | - Horacio Antonio Aiello
- Maternal-Fetal Medicine Unit, Obstetrics Service, Hospital Italiano de Buenos Aires, Instituto Universitario Hospital Italiano, Buenos Aires, Argentina
| | - César Hernán Meller
- Maternal-Fetal Medicine Unit, Obstetrics Service, Hospital Italiano de Buenos Aires, Instituto Universitario Hospital Italiano, Buenos Aires, Argentina
| | - Victorio Lucini
- Hemodinamics Service, Hospital Italiano de Buenos Aires, Instituto Universitario Hospital Italiano, Buenos Aires, Argentina
| | - Natalia Nápoli
- Pediatric Cardiology Service, Hospital Italiano de Buenos Aires, Instituto Universitario Hospital Italiano, Buenos Aires, Argentina
| | - Luis Trentacoste
- Hemodinamics Service, Hospital Italiano de Buenos Aires, Instituto Universitario Hospital Italiano, Buenos Aires, Argentina
| | - Antonela Córdoba
- Pediatric Cardiology Service, Hospital Italiano de Buenos Aires, Instituto Universitario Hospital Italiano, Buenos Aires, Argentina
| | - Mercedes Saenz Tejeira
- Pediatric Cardiology Service, Hospital Italiano de Buenos Aires, Instituto Universitario Hospital Italiano, Buenos Aires, Argentina
| | - Juan Manuel Osuna
- Pediatric Cardiovascular Surgery Service, Hospital Italiano de Buenos Aires, Instituto Universitario Hospital Italiano, Buenos Aires, Argentina
| | - Jorge Barretta
- Pediatric Cardiovascular Surgery Service, Hospital Italiano de Buenos Aires, Instituto Universitario Hospital Italiano, Buenos Aires, Argentina
| | - Alejandra Beatriz Villa
- Pediatric Cardiology Service, Hospital Italiano de Buenos Aires, Instituto Universitario Hospital Italiano, Buenos Aires, Argentina
| | - Pablo Marantz
- Pediatric Cardiology Service, Hospital Italiano de Buenos Aires, Instituto Universitario Hospital Italiano, Buenos Aires, Argentina
| | - Lucas Otaño
- Maternal-Fetal Medicine Unit, Obstetrics Service, Hospital Italiano de Buenos Aires, Instituto Universitario Hospital Italiano, Buenos Aires, Argentina
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11
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Yilmaz Furtun B, Morris SA. Catheter-Based Fetal Cardiac Interventions. J Cardiovasc Dev Dis 2024; 11:167. [PMID: 38921667 PMCID: PMC11204342 DOI: 10.3390/jcdd11060167] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/16/2024] [Revised: 05/20/2024] [Accepted: 05/23/2024] [Indexed: 06/27/2024] Open
Abstract
Fetal cardiac intervention (FCI) is an emerging and rapidly advancing group of interventions designed to improve outcomes for fetuses with cardiovascular disease. Currently, FCI is comprised of pharmacologic therapies (e.g., trans-placental antiarrhythmics for fetal arrhythmia), open surgical procedures (e.g., surgical resection of pericardial teratoma), and catheter-based procedures (e.g., fetal aortic valvuloplasty for aortic stenosis). This review focuses on the rationale, criteria for inclusion, technical details, and current outcomes of the three most frequently performed catheter-based FCI procedures: (1) aortic valvuloplasty for critical aortic stenosis (AS) associated with evolving hypoplastic left heart syndrome (HLHS), (2) atrial septal intervention for HLHS with severely restrictive or intact atrial septum (R/IAS), and (3) pulmonary valvuloplasty for pulmonary atresia with intact ventricular septum (PA/IVS).
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Affiliation(s)
| | - Shaine Alaine Morris
- Texas Children’s Hospital, Baylor College of Medicine, 6651 Main Street, Suite E1920, Houston, TX 77030, USA
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12
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Nield LE, Dahan M, Guerra V, Mustafa S, Okun N, Freud L, Han RK, Kirsch R. Fetal Cardiology Bioethics: An Innovative New Curriculum for Cardiology Trainees. Pediatr Cardiol 2024; 45:703-709. [PMID: 38386036 DOI: 10.1007/s00246-024-03431-x] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/24/2023] [Accepted: 01/25/2024] [Indexed: 02/23/2024]
Abstract
Decision-making in fetal cardiology is fraught with ethical issues yet education in bioethics for trainees is limited or nonexistent. In this innovation report, we describe the development of a fetal cardiology bioethics curriculum designed to address this gap. The curriculum was developed to supplement the core curriculum for cardiology fellows and fetal cardiology subspecialty trainees. The series combines didactic and interactive teaching modalities and contains 5 key components: (1) introduction to bioethics and its role in fetal cardiology, (2) counseling and pathways for compassionate terminal care, (3) case vignette-based ethical analysis and discussion cases, (4) fetal counseling considerations for shared decision-making and recommendations, (5) facilitated communications role play. The curriculum was refined using session evaluations from end users. This report describes the innovative curriculum as a starting point for further incorporation and study of bioethical education in pediatric cardiology and fetal training programs.
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Affiliation(s)
- Lynne E Nield
- Division of Cardiology, Department of Pediatrics, The Hospital for Sick Children, Toronto, Canada
- Department of Pediatrics, University of Toronto, Toronto, Canada
- Sunnybrook Health Sciences Centre, Toronto, Canada
| | - Maya Dahan
- Sunnybrook Health Sciences Centre, Toronto, Canada
| | - Vitor Guerra
- Division of Cardiology, Department of Pediatrics, The Hospital for Sick Children, Toronto, Canada
- Department of Pediatrics, University of Toronto, Toronto, Canada
| | - Sonila Mustafa
- Division of Cardiology, Department of Pediatrics, The Hospital for Sick Children, Toronto, Canada
| | - Nanette Okun
- Sunnybrook Health Sciences Centre, Toronto, Canada
| | - Lindsay Freud
- Division of Cardiology, Department of Pediatrics, The Hospital for Sick Children, Toronto, Canada
- Department of Pediatrics, University of Toronto, Toronto, Canada
| | - Ra K Han
- St. Michael's Hospital, Toronto, Canada
| | - Roxanne Kirsch
- Department of Pediatrics, University of Toronto, Toronto, Canada.
- Division Cardiac Critical Care, Department of Critical Care, The Hospital for Sick Children, University of Toronto, 555 University Avenue, Dept Critical Care, Toronto, ON, M5G 1X8, Canada.
- Department of Bioethics, The Hospital for Sick Children, Toronto, Canada.
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13
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Freud LR. Beyond Technical Success of Fetal Aortic Valvuloplasty. JACC. ADVANCES 2024; 3:100836. [PMID: 38938831 PMCID: PMC11198671 DOI: 10.1016/j.jacadv.2024.100836] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Key Words] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Indexed: 06/29/2024]
Affiliation(s)
- Lindsay R. Freud
- Division of Cardiology, Department of Paediatrics, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada
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14
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Callahan R, Friedman KG, Tworetzky W, Esch JJ, Sleeper LA, Lu M, Mizrahi-Arnaud A, Brusseau R, Lafranchi T, Wilkins-Haug LE, Guseh SH, Benson CB, Frates MC, Keochakian M, Porras D. Technical Success and Serious Adverse Events for Fetal Aortic Valvuloplasty in a Large 20-Year Cohort. JACC. ADVANCES 2024; 3:100835. [PMID: 38938833 PMCID: PMC11198497 DOI: 10.1016/j.jacadv.2024.100835] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 12/08/2022] [Revised: 10/02/2023] [Accepted: 10/27/2023] [Indexed: 06/29/2024]
Abstract
Background FAV is offered to fetuses with severe aortic valve stenosis and evolving hypoplastic left heart syndrome. An inferential analysis of TS and SAE in a large series has not been reported. Objectives The purpose of this study was to determine factors associated with fetal aortic valvuloplasty (FAV) technical success (TS) and serious adverse events (SAEs). Methods Retrospective, single-center, cohort analysis of attempted FAV from March 1, 2000, to December 31, 2020. The primary outcome was the TS of FAV, and the secondary outcome was the presence of an SAE. Results A total of 165 FAVs were attempted in 163 patients with a median gestational age of 24.6 weeks (IQR: 22.9-27.1 weeks). FAV TS was 85% (141/165) and was higher in the 2010 to 2020 era (94% [85/90] vs 75% [56/75]; P < 0.001). Pre-FAV echocardiographic left ventricle (LV) long axis dimension z-score >-0.10 (P < 0.001) and higher LV ejection fraction (P = 0.037) were independently associated with a higher odds of TS. There were 117 SAEs in 67 attempted FAVs (41%), 13 of which were fetal deaths (7.9%). By classification and regression tree analysis, gestational age <21 weeks or in older fetuses, a procedure time of ≥39.6 minutes was associated with higher SAE rate. In the multivariable logistic regression model correcting for gestational age, fetuses with an LV end-diastolic volume <4.09 mL had an age-adjusted OR of 4.71 (95% CI: 1.67-13.29; P = 0.004) for experiencing an SAE. Conclusions TS of FAV has improved over time, and failure is associated with smaller fetal left heart sizes. SAEs are common and are associated with smaller left hearts and longer procedure times.
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Affiliation(s)
- Ryan Callahan
- Department of Pediatrics, Children’s Hospital of Philadelphia, and University of Pennsylvania Perelman School of Medicine, Philadelphia, Pennsylvania, USA
| | - Kevin G. Friedman
- Department of Cardiology, Boston Children’s Hospital and Harvard Medical School, Boston, Massachusetts, USA
| | - Wayne Tworetzky
- Department of Cardiology, Boston Children’s Hospital and Harvard Medical School, Boston, Massachusetts, USA
| | - Jesse J. Esch
- Department of Cardiology, Boston Children’s Hospital and Harvard Medical School, Boston, Massachusetts, USA
| | - Lynn A. Sleeper
- Department of Cardiology, Boston Children’s Hospital and Harvard Medical School, Boston, Massachusetts, USA
| | - Minmin Lu
- Department of Cardiology, Boston Children’s Hospital and Harvard Medical School, Boston, Massachusetts, USA
| | - Arielle Mizrahi-Arnaud
- Department of Anesthesia, Boston Children’s Hospital and Harvard Medical School, Boston, Massachusetts, USA
| | - Roland Brusseau
- Department of Anesthesia, Boston Children’s Hospital and Harvard Medical School, Boston, Massachusetts, USA
| | - Terra Lafranchi
- Department of Cardiology, Boston Children’s Hospital and Harvard Medical School, Boston, Massachusetts, USA
| | - Louise E. Wilkins-Haug
- Department of Obstetrics and Gynecology, Brigham and Women’s Hospital and Harvard Medical School, Boston, Massachusetts, USA
| | - Stephanie H. Guseh
- Department of Obstetrics and Gynecology, Brigham and Women’s Hospital and Harvard Medical School, Boston, Massachusetts, USA
| | - Carol B. Benson
- Department of Radiology, Brigham and Women’s Hospital and Harvard Medical School, Boston, Massachusetts, USA
| | - Mary C. Frates
- Department of Radiology, Brigham and Women’s Hospital and Harvard Medical School, Boston, Massachusetts, USA
| | - Mirjam Keochakian
- Department of Cardiology, Boston Children’s Hospital and Harvard Medical School, Boston, Massachusetts, USA
| | - Diego Porras
- Department of Cardiology, Boston Children’s Hospital and Harvard Medical School, Boston, Massachusetts, USA
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15
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Green L, Chan WX, Prakash I, Tulzer A, Tulzer G, Yap CH. Pre-intervention myocardial stress is a good predictor of aortic valvoluplasty outcome for fetal critical aortic stenosis and evolving HLHS. J Physiol 2024; 602:663-681. [PMID: 38324229 DOI: 10.1113/jp285475] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/14/2023] [Accepted: 01/17/2024] [Indexed: 02/08/2024] Open
Abstract
Fetal critical aortic stenosis with evolving hypoplastic left heart syndrome (CAS-eHLHS) causes biomechanical and functional aberrations, leading to a high risk of progression to hypoplastic left heart syndrome (HLHS) at birth. Fetal aortic valvuloplasty (FAV) can resolve outflow obstruction and may reduce progression risk. However, it is currently difficult to accurately predict which patients will respond to the intervention and become functionally biventricular (BV) at birth, as opposed to becoming functionally univentricular (UV). This prediction is important for patient selection, parental counselling, and surgical planning. Therefore, we investigated whether biomechanics parameters from pre-FAV image-based computations could robustly distinguish between CAS-eHLHS cases with BV or UV outcomes in a retrospective cohort. To do so we performed image-based finite element biomechanics modelling of nine CAS-eHLHS cases undergoing intervention and six healthy fetal control hearts, and found that a biomechanical parameter, peak systolic myofibre stress, showed a uniquely large difference between BV and UV cases, which had a larger magnitude effect than echocardiography parameters. A simplified equation was derived for quick and easy estimation of myofibre stress from echo measurements via principal component analysis. When tested on a retrospective cohort of 37 CAS-eHLHS cases, the parameter outperformed other parameters in predicting UV versus BV outcomes, and thus has a high potential of improving outcome predictions, if incorporated into patient selection procedures. Physiologically, high myocardial stresses likely indicate a healthier myocardium that can withstand high stresses and resist pathological remodelling, which can explain why it is a good predictor of BV outcomes. KEY POINTS: Predicting the morphological birth outcomes (univentricular versus biventricular) of fetal aortic valvuloplasty for fetal aortic stenosis with evolving HLHS is important for accurate patient selection, parental counselling and management decisions. Computational simulations show that a biomechanics parameter, pre-intervention peak systolic myofibre stress, is uniquely robust in distinguishing between such outcomes, outperforming all echo parameters. An empirical equation was developed to quickly compute peak systolic myofibre stress from routine echo measurements and was the best predictor of outcomes among a wide range of parameters tested.
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Affiliation(s)
- Laura Green
- Department of Bioengineering, Imperial College London, London, UK
| | - Wei Xuan Chan
- Department of Bioengineering, Imperial College London, London, UK
- BHF Centre of Research Excellence, Imperial College London, London, UK
| | - Indumita Prakash
- Department of Bioengineering, Imperial College London, London, UK
| | - Andreas Tulzer
- Department of Pediatric Cardiology, Children's Heat Center Linz, Kepler University Hospital, Linz, Austria
- Medical Faculty, Johannes Kepler University Linz, Linz, Austria
| | - Gerald Tulzer
- Department of Pediatric Cardiology, Children's Heat Center Linz, Kepler University Hospital, Linz, Austria
- Medical Faculty, Johannes Kepler University Linz, Linz, Austria
| | - Choon Hwai Yap
- Department of Bioengineering, Imperial College London, London, UK
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16
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Gierlinger G, Emani SM. Endocardial Fibroelastosis Resection: When it Works and When it Does Not. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2023; 27:19-24. [PMID: 38522867 DOI: 10.1053/j.pcsu.2023.12.006] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/30/2023] [Revised: 12/14/2023] [Accepted: 12/18/2023] [Indexed: 03/26/2024]
Abstract
Endocardial fibroelastosis (EFE) is a thickening of the endocardial layer by accumulation of collagen and elastic fibers. Endothelial to mesenchymal transformation is proposed to be the underlying mechanism of formation. Although EFE can occur in both right and left ventricles, this article will focus on management of left ventricular EFE. Through its fibrous, nonelastic manifestation EFE restricts the myocardium leading to diastolic and systolic ventricular dysfunction and prevents ventricular growth in neonates and infants. The presence of EFE may be a marker for underlying myocardial fibrosis as well. The extent of EFE within the left ventricular cavity can be variable ranging from patchy to confluent distribution. Similarly the depth of penetration and degree of infiltration into myocardium can be variable. The management of EFE is controversial, although resection of EFE has been reported as part of the staged ventricular recruitment therapy. Following resection, EFE recurs and infiltrates the myocardium after primary resection. Herein we review the current experience with EFE resection.
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Affiliation(s)
- Gregor Gierlinger
- Department of Cardiac Surgery, Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts
| | - Sitaram M Emani
- Department of Cardiac Surgery, Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts..
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17
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Deren O, Aykan HH, Ozyuncu O, Aypar E, Cagan M, Ozen O, Karagoz T. Fetal cardiac interventions: First-year experience of a tertiary referral center in Turkey. J Obstet Gynaecol Res 2023. [PMID: 37004995 DOI: 10.1111/jog.15649] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/23/2022] [Accepted: 03/22/2023] [Indexed: 04/04/2023]
Abstract
AIM To present the first-year experience of fetal cardiac interventions (FCIs) in a tertiary referral hospital and to evaluate the outcomes. METHODS This retrospective study consisted of four pregnant women who underwent fetal pulmonary or aortic balloon valvuloplasty between November 2020 and June 2021. The procedures were performed with a percutaneous cardiac puncture under the ultrasonography guidance. Gestational age at intervention, procedural success, complications, and perinatal outcomes were evaluated. Procedural complications defined as fetal bradyarrhythmia requiring treatment, pericardial effusion requiring drainage, balloon rupture, and fetal death. The procedure was considered technically successful if the valve was dilated with a balloon catheter. Ultimately successful procedure was defined as the discharge of infants alive with biventricular circulation. RESULTS A total of 5 FCIs attempted between 26 + 3 and 28 + 2 gestational weeks. While the procedure was technically successful in 2 cases with pulmonary stenosis, both attempts were unsuccessful in the fetus with pulmonary atresia. Although the procedure was technically successful in the patient with critical aortic stenosis, it ultimately failed. No fetal death occurred in our series and there were no procedure-related significant maternal complications. However, three interventions were complicated by fetal bradycardia and pericardial effusion necessitating treatment, and balloon rupture cropped up in one case. CONCLUSION FCIs may lead to improving the likelihood of a biventricular outcome for selected fetuses. Careful selection of patients and centralization of experience are essential for obtaining favorable outcomes. Operators should be aware of procedural complications. Improved procedural techniques with a lower complication rate will be achieved through advanced medical technology and special balloon catheters.
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Affiliation(s)
- Ozgur Deren
- Division of Perinatology, Department of Obstetrics and Gynecology, Faculty of Medicine, Hacettepe University, Ankara, Turkey
| | - Hayrettin Hakan Aykan
- Division of Pediatric Cardiology, Department of Pediatrics, Faculty of Medicine, Hacettepe University, Ankara, Turkey
- Life Support Center, Hacettepe University, Ankara, Turkey
| | - Ozgur Ozyuncu
- Division of Perinatology, Department of Obstetrics and Gynecology, Faculty of Medicine, Hacettepe University, Ankara, Turkey
| | - Ebru Aypar
- Division of Pediatric Cardiology, Department of Pediatrics, Faculty of Medicine, Hacettepe University, Ankara, Turkey
| | - Murat Cagan
- Division of Perinatology, Department of Obstetrics and Gynecology, Faculty of Medicine, Hacettepe University, Ankara, Turkey
| | - Ozge Ozen
- Department of Anesthesiology and Reanimation, Faculty of Medicine, Hacettepe University, Ankara, Turkey
| | - Tevfik Karagoz
- Division of Pediatric Cardiology, Department of Pediatrics, Faculty of Medicine, Hacettepe University, Ankara, Turkey
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18
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Mendel B, Kohar K, Amirah S, Vidya AP, Utama KE, Prakoso R, Siagian SN. The outcomes of fetal aortic valvuloplasty in critical aortic stenosis: A systematic review and meta-analysis. Int J Cardiol 2023; 382:106-111. [PMID: 36996909 DOI: 10.1016/j.ijcard.2023.03.050] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/06/2022] [Revised: 03/08/2023] [Accepted: 03/21/2023] [Indexed: 04/01/2023]
Abstract
BACKGROUND Critical aortic stenosis that appears in mid-gestation tends to develop to growth retardation of left ventricle, known as hypoplastic left heart syndrome (HLHS). Despite better clinical management of HLHS, the morbidity and mortality rates of univentricular circulation patients remain high. In this paper, we sought to perform a systematic review and meta-analysis to know the outcomes of fetal aortic valvuloplasty in critical aortic stenosis patients. METHODS This systematic review and meta-analysis was conducted according to the Preferred Reporting Items for Systematic Review and Meta-Analyses (PRISMA) statement. A systematic search on fetal aortic valvuloplasty procedure for critical aortic stenosis was performed through PubMed, Scopus, EBSCOhost, ProQuest, and Google Scholar. The primary endpoint of each group was overall mortality. We used R software (version 4.1.3) to estimate the overall proportion of each outcome using random-effects model of proportional meta-analysis. RESULTS A total of 389 fetal subjects from 10 cohort studies were included in this systematic review and meta-analysis. Fetal aortic valvuloplasty (FAV) was successfully performed in 84% of patients. It revealed a successful conversion to biventricular circulation rate of 33% with a mortality rate of 20%. Bradycardia and pleural effusion requiring treatment were two most common fetal complications, whereas maternal complication reported was only placental abruption in one patient. CONCLUSIONS FAV has a high technical success rate with the ability to achieve biventricular circulation and a low rate of procedure-related mortality if carried out by experienced operators.
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Affiliation(s)
- Brian Mendel
- Division of Pediatric Cardiology and Congenital Heart Disease, Department of Cardiology and Vascular Medicine, National Cardiovascular Center Harapan Kita, Universitas Indonesia, Jakarta, Indonesia; Department of Cardiology and Vascular Medicine, Sultan Sulaiman Government Hospital, Serdang Bedagai, North Sumatera, Indonesia.
| | - Kelvin Kohar
- Faculty of Medicine, Universitas Indonesia, Jakarta, Indonesia
| | - Shakira Amirah
- Faculty of Medicine, Universitas Indonesia, Jakarta, Indonesia
| | | | | | - Radityo Prakoso
- Division of Pediatric Cardiology and Congenital Heart Disease, Department of Cardiology and Vascular Medicine, National Cardiovascular Center Harapan Kita, Universitas Indonesia, Jakarta, Indonesia
| | - Sisca Natalia Siagian
- Division of Pediatric Cardiology and Congenital Heart Disease, Department of Cardiology and Vascular Medicine, National Cardiovascular Center Harapan Kita, Universitas Indonesia, Jakarta, Indonesia
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19
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Srisupundit K, Luewan S, Tongsong T. Prenatal Diagnosis of Fetal Heart Failure. Diagnostics (Basel) 2023; 13:diagnostics13040779. [PMID: 36832267 PMCID: PMC9955344 DOI: 10.3390/diagnostics13040779] [Citation(s) in RCA: 4] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/24/2022] [Revised: 02/03/2023] [Accepted: 02/17/2023] [Indexed: 02/22/2023] Open
Abstract
Fetal heart failure (FHF) is a condition of inability of the fetal heart to deliver adequate blood flow for tissue perfusion in various organs, especially the brain, heart, liver and kidneys. FHF is associated with inadequate cardiac output, which is commonly encountered as the final outcome of several disorders and may lead to intrauterine fetal death or severe morbidity. Fetal echocardiography plays an important role in diagnosis of FHF as well as of the underlying causes. The main findings supporting the diagnosis of FHF include various signs of cardiac dysfunction, such as cardiomegaly, poor contractility, low cardiac output, increased central venous pressures, hydropic signs, and the findings of specific underlying disorders. This review will present a summary of the pathophysiology of fetal cardiac failure and practical points in fetal echocardiography for diagnosis of FHF, focusing on essential diagnostic techniques used in daily practice for evaluation of fetal cardiac function, such as myocardial performance index, arterial and systemic venous Doppler waveforms, shortening fraction, and cardiovascular profile score (CVPs), a combination of five echocardiographic markers indicative of fetal cardiovascular health. The common causes of FHF are reviewed and updated in detail, including fetal dysrhythmia, fetal anemia (e.g., alpha-thalassemia, parvovirus B19 infection, and twin anemia-polycythemia sequence), non-anemic volume load (e.g., twin-to-twin transfusion, arteriovenous malformations, and sacrococcygeal teratoma, etc.), increased afterload (intrauterine growth restriction and outflow tract obstruction, such as critical aortic stenosis), intrinsic myocardial disease (cardiomyopathies), congenital heart defects (Ebstein anomaly, hypoplastic heart, pulmonary stenosis with intact interventricular septum, etc.) and external cardiac compression. Understanding the pathophysiology and clinical courses of various etiologies of FHF can help physicians make prenatal diagnoses and serve as a guide for counseling, surveillance and management.
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20
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Fluid Mechanical Effects of Fetal Aortic Valvuloplasty for Cases of Critical Aortic Stenosis with Evolving Hypoplastic Left Heart Syndrome. Ann Biomed Eng 2023:10.1007/s10439-023-03152-x. [PMID: 36780051 DOI: 10.1007/s10439-023-03152-x] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/28/2022] [Accepted: 01/16/2023] [Indexed: 02/14/2023]
Abstract
Fetuses with critical aortic stenosis (FAS) are at high risk of progression to HLHS by the time of birth (and are thus termed "evolving HLHS"). An in-utero catheter-based intervention, fetal aortic valvuloplasty (FAV), has shown promise as an intervention strategy to circumvent the progression, but its impact on the heart's biomechanics is not well understood. We performed patient-specific computational fluid dynamic (CFD) simulations based on 4D fetal echocardiography to assess the changes in the fluid mechanical environment in the FAS left ventricle (LV) directly before and 2 days after FAV. Echocardiograms of five FAS cases with technically successful FAV were retrospectively analysed. FAS compromised LV stroke volume and ejection fraction, but FAV rescued it significantly. Calculations to match simulations to clinical measurements showed that FAV approximately doubled aortic valve orifice area, but it remained much smaller than in healthy hearts. Diseased LVs had mildly stenotic mitral valves, which generated fast and narrow diastolic mitral inflow jet and vortex rings that remained unresolved directly after FAV. FAV further caused aortic valve damage and high-velocity regurgitation. The high-velocity aortic regurgitation jet and vortex ring caused a chaotic flow field upon impinging the apex, which drastically exacerbated the already high energy losses and poor flow energy efficiency of FAS LVs. Two days after the procedure, FAV did not alter wall shear stress (WSS) spatial patterns of diseased LV but elevated WSS magnitudes, and the poor blood turnover in pre-FAV LVs did not significantly improve directly after FAV. FAV improved FAS LV's flow function, but it also led to highly chaotic flow patterns and excessively high energy losses due to the introduction of aortic regurgitation directly after the intervention. Further studies analysing the effects several weeks after FAV are needed to understand the effects of such biomechanics on morphological development.
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21
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Ashkanase J, Wong D. Update in Pediatric Cardiology. UPDATE IN PEDIATRICS 2023:79-108. [DOI: 10.1007/978-3-031-41542-5_3] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/03/2025]
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Yasuhara J, Schultz K, Bigelow AM, Garg V. Congenital aortic valve stenosis: from pathophysiology to molecular genetics and the need for novel therapeutics. Front Cardiovasc Med 2023; 10:1142707. [PMID: 37187784 PMCID: PMC10175644 DOI: 10.3389/fcvm.2023.1142707] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/12/2023] [Accepted: 04/14/2023] [Indexed: 05/17/2023] Open
Abstract
Congenital aortic valve stenosis (AVS) is one of the most common valve anomalies and accounts for 3%-6% of cardiac malformations. As congenital AVS is often progressive, many patients, both children and adults, require transcatheter or surgical intervention throughout their lives. While the mechanisms of degenerative aortic valve disease in the adult population are partially described, the pathophysiology of adult AVS is different from congenital AVS in children as epigenetic and environmental risk factors play a significant role in manifestations of aortic valve disease in adults. Despite increased understanding of genetic basis of congenital aortic valve disease such as bicuspid aortic valve, the etiology and underlying mechanisms of congenital AVS in infants and children remain unknown. Herein, we review the pathophysiology of congenitally stenotic aortic valves and their natural history and disease course along with current management strategies. With the rapid expansion of knowledge of genetic origins of congenital heart defects, we also summarize the literature on the genetic contributors to congenital AVS. Further, this increased molecular understanding has led to the expansion of animal models with congenital aortic valve anomalies. Finally, we discuss the potential to develop novel therapeutics for congenital AVS that expand on integration of these molecular and genetic advances.
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Affiliation(s)
- Jun Yasuhara
- Center for Cardiovascular Research, Abigail Wexner Research Institute, Nationwide Children’s Hospital, Columbus, OH, United States
- Heart Center, Nationwide Children’s Hospital, Columbus, OH, United States
- Correspondence: Jun Yasuhara Vidu Garg
| | - Karlee Schultz
- Medical Student Research Program, The Ohio State University College of Medicine, Columbus, OH, United States
| | - Amee M. Bigelow
- Heart Center, Nationwide Children’s Hospital, Columbus, OH, United States
- Department of Pediatrics, The Ohio State University, Columbus, OH, United States
| | - Vidu Garg
- Center for Cardiovascular Research, Abigail Wexner Research Institute, Nationwide Children’s Hospital, Columbus, OH, United States
- Heart Center, Nationwide Children’s Hospital, Columbus, OH, United States
- Department of Pediatrics, The Ohio State University, Columbus, OH, United States
- Department of Molecular Genetics, The Ohio State University, Columbus, OH, United States
- Correspondence: Jun Yasuhara Vidu Garg
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23
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Kohl T. Lifesaving Treatments for the Tiniest Patients-A Narrative Description of Old and New Minimally Invasive Approaches in the Arena of Fetal Surgery. CHILDREN (BASEL, SWITZERLAND) 2022; 10:67. [PMID: 36670618 PMCID: PMC9856479 DOI: 10.3390/children10010067] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Subscribe] [Scholar Register] [Received: 10/29/2022] [Revised: 12/14/2022] [Accepted: 12/21/2022] [Indexed: 12/31/2022]
Abstract
Fetal surgery has become a lifesaving reality for hundreds of fetuses each year. The development of a formidable spectrum of safe and effective minimally invasive techniques for fetal interventions since the early 1990s until today has led to an increasing acceptance of novel procedures by both patients and health care providers. From his vast personal experience of more than 20 years as one of the pioneers at the forefront of clinical minimally invasive fetal surgery, the author describes and comments on old and new minimally invasive approaches, highlighting their lifesaving or quality-of-life-improving potential. He provides easy-to-use practical information on how to perform partial amniotic carbon dioxide insufflation (PACI), how to assess lung function in fetuses with pulmonary hypoplasia, how to deal with giant CPAMS, how to insert shunts into fetuses with LUTO and hydrothorax when conventional devices are not available, and how to resuscitate a fetus during fetal cardiac intervention. Furthermore, the author proposes a curriculum for future fetal surgeons, solicits for the centralization of patients, for adequate maternal counseling, for adequate pain management and adequate hygienic conditions during interventions, and last but not least for starting the process of academic recognition of the matured field as an independent specialty. These steps will allow more affected expectant women and their unborn children to gain access to modern minimally invasive fetal surgery and therapy. The opportunity to treat more patients at dedicated centers will also result in more opportunities for the research of rare diseases and conditions, promising even better pre- and postnatal care in the future.
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Affiliation(s)
- Thomas Kohl
- German Center for Fetal Surgery & Minimally-Invasive Therapy (DZFT), Mannheim University Hospital (UMM), Theodor-Kutzer-Ufer 1-3, 68167 Mannheim, Germany
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24
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Bradford VR, Tworetzky W, Callahan R, Wilkins-Haug LE, Benson CB, Porras D, Guseh SH, Lu M, Sleeper LA, Gellis L, Friedman KG. Hemodynamic and Anatomic Changes after Fetal Aortic Valvuloplasty are associated with Procedural Success and Postnatal Biventricular Circulation. Prenat Diagn 2022; 42:1312-1322. [PMID: 35924422 DOI: 10.1002/pd.6216] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/18/2022] [Revised: 07/08/2022] [Accepted: 07/13/2022] [Indexed: 11/05/2022]
Abstract
BACKGROUND There are minimal data characterizing the trajectory of left heart growth and hemodynamics following fetal aortic valvuloplasty (FAV). METHODS This retrospective study included patients who underwent FAV between 2000 and 2019 with echocardiograms performed pre-FAV, immediately post-FAV, and in late gestation. RESULTS Of 118 fetuses undergoing FAV, 106 (90%) underwent technically successful FAV, of which 55 (52%) had biventricular circulation. Technically successful FAV was associated with improved aortic valve growth (p<0.001), sustained antegrade aortic arch (AoA) flow (p=0.02), improved mitral valve inflow pattern (p=0.002), and favorable patent foramen ovale (PFO) flow pattern (p=0.004) from pre-FAV to late gestation. Compared to patients with univentricular outcome, patients with biventricular outcome had less decrement in size of the left ventricle (LV) (p<0.001) and aortic valve (p=0.005), as well more physiologic PFO flow (p<0.001) and antegrade AoA flow (p<0.001) from pre-FAV to late gestation. In multivariable analysis, echocardiographic predictors of biventricular outcome were less decline in LV end diastolic dimension (p<0.001), improved PFO flow (p=0.004), and sustained antegrade AoA flow (p=0.002) from pre-FAV to late gestation. CONCLUSION Stabilization of left heart growth and improved hemodynamics following successful FAV through late gestation are associated with postnatal biventricular circulation. This article is protected by copyright. All rights reserved.
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Affiliation(s)
- Victoria R Bradford
- Cardiology, Boston CObstetrics and Gynecology andhildren's Hospital, Departments of, Boston, MA, USA.,Pediatrics, Boston, MA, USA
| | - Wayne Tworetzky
- Cardiology, Boston CObstetrics and Gynecology andhildren's Hospital, Departments of, Boston, MA, USA.,Pediatrics, Boston, MA, USA
| | - Ryan Callahan
- Cardiology, Boston CObstetrics and Gynecology andhildren's Hospital, Departments of, Boston, MA, USA.,Pediatrics, Boston, MA, USA
| | - Louise E Wilkins-Haug
- Obstetrics and Gynecology and, Boston, MA, USA.,Obstetrics and Gynecology and, Boston, MA, USA
| | - Carol B Benson
- Radiology, Brigham and Women's Hospital, Department of, Boston, MA, USA.,Radiology, Harvard Medical School, Boston, MA, USA
| | - Diego Porras
- Cardiology, Boston CObstetrics and Gynecology andhildren's Hospital, Departments of, Boston, MA, USA.,Pediatrics, Boston, MA, USA
| | - Stephanie H Guseh
- Obstetrics and Gynecology and, Boston, MA, USA.,Obstetrics and Gynecology and, Boston, MA, USA
| | - Minmin Lu
- Cardiology, Boston CObstetrics and Gynecology andhildren's Hospital, Departments of, Boston, MA, USA
| | - Lynn A Sleeper
- Cardiology, Boston CObstetrics and Gynecology andhildren's Hospital, Departments of, Boston, MA, USA.,Pediatrics, Boston, MA, USA
| | - Laura Gellis
- Cardiology, Boston CObstetrics and Gynecology andhildren's Hospital, Departments of, Boston, MA, USA.,Pediatrics, Boston, MA, USA
| | - Kevin G Friedman
- Cardiology, Boston CObstetrics and Gynecology andhildren's Hospital, Departments of, Boston, MA, USA.,Pediatrics, Boston, MA, USA
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25
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Wong HS, Wiputra H, Tulzer A, Tulzer G, Yap CH. Fluid Mechanics of Fetal Left Ventricle During Aortic Stenosis with Evolving Hypoplastic Left Heart Syndrome. Ann Biomed Eng 2022; 50:1158-1172. [PMID: 35731342 PMCID: PMC9363377 DOI: 10.1007/s10439-022-02990-5] [Citation(s) in RCA: 4] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/19/2022] [Accepted: 06/05/2022] [Indexed: 11/28/2022]
Abstract
In cases of fetal aortic stenosis and evolving Hypoplastic Left Heart Syndrome (feHLHS), aortic stenosis is associated with specific abnormalities such as retrograde or bidirectional systolic transverse arch flow. Many cases progressed to hypoplastic left heart syndrome (HLHS) malformation at birth, but fetal aortic valvuloplasty can prevent the progression in many cases. Since both disease and intervention involve drastic changes to the biomechanical environment, in-vivo biomechanics likely play a role in inducing and preventing disease progression. However, the fluid mechanics of feHLHS is not well-characterized. Here, we conduct patient-specific echocardiography-based flow simulations of normal and feHLHS left ventricles (LV), to understand the essential fluid dynamics distinction between the two cohorts. We found high variability across feHLHS cases, but also the following unifying features. Firstly, feHLHS diastole mitral inflow was in the form of a narrowed and fast jet that impinged onto the apical region, rather than a wide and gentle inflow in normal LVs. This was likely due to a malformed mitral valve with impaired opening dynamics. This altered inflow caused elevated vorticity dynamics and wall shear stresses (WSS) and reduced oscillatory shear index at the apical zone rather than mid-ventricle. Secondly, feHLHS LV also featured elevated systolic and diastolic energy losses, intraventricular pressure gradients, and vortex formation numbers, suggesting energy inefficiency of flow and additional burden on the LV. Thirdly, feHLHS LV had poor blood turnover, suggesting a hypoxic environment, which could be associated with endocardial fibroelastosis that is often observed in these patients.
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Affiliation(s)
- Hong Shen Wong
- Department of Bioengineering, Imperial College London, London, UK
| | - Hadi Wiputra
- Department of Biomedical Engineering, University of Minnesota, Minneapolis, USA
| | - Andreas Tulzer
- Department of Pediatric Cardiology, Children's Heart Center Linz, Kepler University Hospital, Linz, Austria
| | - Gerald Tulzer
- Department of Pediatric Cardiology, Children's Heart Center Linz, Kepler University Hospital, Linz, Austria
| | - Choon Hwai Yap
- Department of Bioengineering, Imperial College London, London, UK.
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26
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Wada S, Ozawa K, Sago H. New challenges of fetal therapy in Japan. J Obstet Gynaecol Res 2022; 48:2100-2111. [PMID: 35676616 PMCID: PMC9544758 DOI: 10.1111/jog.15320] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/03/2022] [Accepted: 05/20/2022] [Indexed: 12/01/2022]
Abstract
Aim To review new challenges of fetal therapy in Japan after the establishment of four existing fetal therapies as standard prenatal care with National Health Insurance coverage over the past 20 years. Methods Reported studies and our current research activities related to four fetal therapies newly performed in Japan were reviewed. Results Fetoscopic endoluminal tracheal occlusion (FETO) for congenital diaphragmatic hernia (CDH) aims to occlude the trachea using a detachable balloon to promote lung growth. Following the recent successful completion of an international randomized controlled trial for CDH, in which we participated, FETO is offered for severe left CDH to perform balloon insertion at 27–29 weeks and removal at 34 weeks of gestation. Fetal cystoscopy (FC) for low urinary tract obstruction was introduced to overcome the demerits of vesicoamniotic shunting. FC may provide a proper diagnosis by visual observation of the urethra and physiological treatment of the posterior urethral valve. The effectiveness of open fetal surgery for myelomeningocele (MMC), direct surgery with laparotomy and hysterotomy, for ameliorating hindbrain herniation and the motor function was demonstrated, but it was also associated with substantial maternal and fetal risks. Fetal aortic valvuloplasty (FAV), ultrasound‐guided fetal aortic balloon dilation for critical aortic stenosis with evolving hypoplastic left heart syndrome may improve left heart development and maintain biventricular circulation. Feasibility and safety studies for FC, MMC open fetal surgery, and FAV are currently ongoing. Conclusions Clinical research on FETO, FC, MMC open fetal surgery, and FAV has proceeded with careful preparations in Japan.
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Affiliation(s)
- Seiji Wada
- Center for Maternal-Fetal, Neonatal and Reproductive Medicine, National Center for Child Health and Development, Tokyo, Japan
| | - Katsusuke Ozawa
- Center for Maternal-Fetal, Neonatal and Reproductive Medicine, National Center for Child Health and Development, Tokyo, Japan
| | - Haruhiko Sago
- Center for Maternal-Fetal, Neonatal and Reproductive Medicine, National Center for Child Health and Development, Tokyo, Japan
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27
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Intrauterine Valvuloplasty in Severe Aortic Stenosis-A Ten Years Single Center Experience. J Clin Med 2022; 11:jcm11113058. [PMID: 35683446 PMCID: PMC9181328 DOI: 10.3390/jcm11113058] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/30/2022] [Revised: 05/13/2022] [Accepted: 05/27/2022] [Indexed: 02/05/2023] Open
Abstract
Objective: To assess the course and outcome of fetal aortic valvuloplasty (FAV) in fetuses with severe aortic stenosis (SAS) in a single center. Methods: All fetuses with a prenatal diagnosis of SAS with subsequent FAV were retrospectively collected in one tertiary center for fetal medicine over a period of 10 years. In the study, period fetuses with SAS were considered suitable for FAV in the presence of markedly elevated left ventricular pressures (maximum velocity of mitral regurgitation (MR Vmax) >250 cm/s and/or maximum velocity of aortic stenosis (AS Vmax) >250 cm/s), retrograde flow in the transverse aortic arch and a left ventricular length Z-score >−1. Results: In the study period 29 fetuses with AS were treated with 38 FAV. If reinterventions are included 82.7% of fetuses received a technically successful FAV. Procedure related death occurred in three (10.3%) cases, spontaneous fetal death in 2 (6.9%), and termination of pregnancy was performed in 3 cases (10.3%). Among the 21 live births (72.4%), four died in infancy. Among the remaining survivors, 8/17 (47.1%) had a biventricular outcome at the age of one year, 8/17 (47.1%) were univentricular and one infant (5.9%) is biventricular at the age of eight months. Fetuses with biventricular outcome had significantly greater left ventricular (LV) length Z-scores (p = 0.031), and lower tricuspid to mitral valve (TV/MV) ratios (p = 0.003). Conclusions: FAV has a high technical success rate and a low rate of procedure related mortality if performed in experienced hands. The success rate of biventricular circulation at the age of one year is moderate and seems to depend rather on the center’s experience and postnatal surgical strategies than solely on prenatal selection criteria. In the absence of randomized controlled trials, FAV remains an experimental intervention.
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28
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Flow-Mediated Factors in the Pathogenesis of Hypoplastic Left Heart Syndrome. J Cardiovasc Dev Dis 2022; 9:jcdd9050154. [PMID: 35621865 PMCID: PMC9144087 DOI: 10.3390/jcdd9050154] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/30/2022] [Revised: 05/01/2022] [Accepted: 05/06/2022] [Indexed: 12/03/2022] Open
Abstract
Hypoplastic left heart syndrome (HLHS) is a life-threatening congenital heart disease that is characterized by severe underdevelopment of left heart structures. Currently, there is no cure, and affected individuals require surgical palliation or cardiac transplantation to survive. Despite these resource-intensive measures, only about half of individuals reach adulthood, often with significant comorbidities such as liver disease and neurodevelopmental disorders. A major barrier in developing effective treatments is that the etiology of HLHS is largely unknown. Here, we discuss how intracardiac blood flow disturbances are an important causal factor in the pathogenesis of impaired left heart growth. Specifically, we highlight results from a recently developed mouse model in which surgically reducing blood flow through the mitral valve after cardiogenesis led to the development of HLHS. In addition, we discuss the role of interventional procedures that are based on improving blood flow through the left heart, such as fetal aortic valvuloplasty. Lastly, using the surgically-induced mouse model, we suggest investigations that can be undertaken to identify the currently unknown biological pathways in left heart growth failure and their associated therapeutic targets.
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29
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Tulzer A, Huhta JC, Hochpoechler J, Holzer K, Karas T, Kielmayer D, Tulzer G. Hypoplastic Left Heart Syndrome: Is There a Role for Fetal Therapy? Front Pediatr 2022; 10:944813. [PMID: 35874565 PMCID: PMC9304816 DOI: 10.3389/fped.2022.944813] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/15/2022] [Accepted: 06/22/2022] [Indexed: 12/03/2022] Open
Abstract
During fetal life some cardiac defects may lead to diminished left heart growth and to the evolution of a form of hypoplastic left heart syndrome (HLHS). In fetuses with an established HLHS, severe restriction or premature closure of the atrial septum leads to left atrial hypertension and remodeling of the pulmonary vasculature, severely worsening an already poor prognosis. Fetal therapy, including invasive fetal cardiac interventions and non-invasive maternal hyperoxygenation, have been introduced to prevent a possible progression of left heart hypoplasia, improve postnatal outcome, or secure fetal survival. The aim of this review is to cover patient selection and possible hemodynamic effects of fetal cardiac procedures and maternal hyperoxygenation in fetuses with an evolving or established hypoplastic left heart syndrome.
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Affiliation(s)
- Andreas Tulzer
- Children's Heart Center Linz, Department of Pediatric Cardiology, Kepler University Hospital, Linz, Austria.,Medical Faculty, Johannes Kepler University Linz, Linz, Austria
| | - James C Huhta
- Perinatal Cardiology, St. Joseph Hospital, Tampa, FL, United States
| | - Julian Hochpoechler
- Children's Heart Center Linz, Department of Pediatric Cardiology, Kepler University Hospital, Linz, Austria
| | - Kathrin Holzer
- Children's Heart Center Linz, Department of Pediatric Cardiology, Kepler University Hospital, Linz, Austria
| | - Thomas Karas
- Children's Heart Center Linz, Department of Pediatric Cardiology, Kepler University Hospital, Linz, Austria
| | - David Kielmayer
- Children's Heart Center Linz, Department of Pediatric Cardiology, Kepler University Hospital, Linz, Austria
| | - Gerald Tulzer
- Children's Heart Center Linz, Department of Pediatric Cardiology, Kepler University Hospital, Linz, Austria
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