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Zhang WD, Ma B, Qi PA, Wang G, Su XR, Ma XY, Li TG. Anomalous drainage of the ductus venosus into the coronary sinus: prenatal ultrasound diagnosis utilizing two-dimensional and three-dimensional imaging techniques and differential diagnosis. BMC Pregnancy Childbirth 2025; 25:437. [PMID: 40221658 PMCID: PMC11992718 DOI: 10.1186/s12884-025-07560-w] [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: 01/28/2025] [Accepted: 04/02/2025] [Indexed: 04/14/2025] Open
Abstract
BACKGROUND Anomalous drainage of the ductus venosus (DV) into the coronary sinus (CS) is a rare fetal vascular anomaly that poses challenges for prenatal diagnosis. This study aimed to investigate the role of two-dimensional (2D) and three-dimensional (3D) ultrasound imaging, specifically spatiotemporal image correlation (STIC) technology, in improving the prenatal diagnosis of this anomaly. METHODS We retrospectively reviewed eight cases of fetuses diagnosed with anomalous DV drainage into the CS at Gansu Provincial Maternal and Child Health Hospital between September 2019 and September 2024. The gestational age of the fetuses ranged from 24 to 30 weeks. Ultrasound examinations, including 2D and Doppler imaging, along with HDlive Flow combined with STIC technology, were used for diagnosis. Differential diagnoses were made based on imaging findings. Descriptive statistics were employed to summarize the results. RESULTS Eight fetuses with anomalous DV drainage into the CS were identified. Of these, five cases were isolated anomalies, while three had associated malformations, such as aberrant right subclavian artery and right aortic arch. Dilated CS was observed in all cases, with an average inner diameter of 5.7 mm. STIC imaging successfully visualized the abnormal course of the DV, enhancing diagnostic confidence. Postnatal follow-up indicated favorable outcomes for most neonates (except for Case NO.3 and Case NO.7), although persistent CS dilation was observed in these cases, without significant hemodynamic compromise or clinical symptoms. CONCLUSIONS Prenatal diagnosis of anomalous DV drainage into the CS can be effectively achieved using 2D and 3D ultrasound, with STIC technology providing added diagnostic clarity. Early and accurate detection is crucial for ensuring appropriate clinical management and favorable outcomes. Ongoing surveillance of CS dilation in the postnatal period is recommended.
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Affiliation(s)
- Weng-Dong Zhang
- Ultrasound Medicine Center, Gansu Provincial Maternity and Child-Care Hospital, Lanzhou, People's Republic of China
| | - Bin Ma
- Ultrasound Medicine Center, Gansu Provincial Maternity and Child-Care Hospital, Lanzhou, People's Republic of China
| | - Ping-An Qi
- Ultrasound Medicine Center, Gansu Provincial Maternity and Child-Care Hospital, Lanzhou, People's Republic of China
| | - Gang Wang
- Ultrasound Medicine Center, Gansu Provincial Maternity and Child-Care Hospital, Lanzhou, People's Republic of China
| | - Xiao Rong Su
- Ultrasound Medicine Center, Gansu Provincial Maternity and Child-Care Hospital, Lanzhou, People's Republic of China
| | - Xiao Yun Ma
- Ultrasound Medicine Center, Gansu Provincial Maternity and Child-Care Hospital, Lanzhou, People's Republic of China
| | - Tian-Gang Li
- Ultrasound Medicine Center, Gansu Provincial Maternity and Child-Care Hospital, Lanzhou, People's Republic of China.
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Ren JY, Chen CA, Zhu M, Liu K, Chen LJ, Dong SZ. Prenatal MR Diagnosis of Total Anomalous Pulmonary Venous Connection and Related Brain Growth Changes. J Magn Reson Imaging 2024. [PMID: 39630603 DOI: 10.1002/jmri.29671] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/05/2024] [Revised: 11/19/2024] [Accepted: 11/19/2024] [Indexed: 12/07/2024] Open
Abstract
BACKGROUND Prenatal diagnosis of total anomalous pulmonary venous connection (TAPVC) is challenging, and little is known about how it affects brain development. PURPOSE To evaluate the utility of fetal MRI to diagnose TAPVC and related brain growth changes. STUDY TYPE Retrospective case-control study. POPULATION Twenty-one fetuses (23.0 to 30.8 weeks, mean 26.4 weeks) with pre-natal MRI diagnosis of TAPVC. Post-natal images and surgery were available in 18 fetuses. Brain volumes in TAPVC fetuses were compared with age and sex matched 100 cases of normal controls and 38 fetuses with tetralogy of Fallot (TOF). SEQUENCE Single shot turbo spin echo sequence for evaluating fetal brain, and steady-state free precession (SSFP) sequence for evaluating fetal cardiovascular structures at 1.5 T. ASSESSMENT TAPVC type was determined by visualizing the drainage of the common pulmonary vein and dilated coronary sinus: supracardiac, intracardiac and infracardiac. The fetal pulmonary edema was evaluated, and fetal brain volumes were measured using automatic segmentation. STATISTICAL TESTS One-way analysis of variance and post hoc least square difference tests to evaluate differences in variables between TAPVC, TOF and control groups. A P value <0.05 was considered significant. RESULTS Of the 21 cases of TAPVC, 10 (47.6%) were identified as supracardiac, 8 (38.1%) as intracardiac, and 3 (14.3%) as infracardiac. Eighteen cases were confirmed by postnatal imaging and surgery; the remaining three cases had no confirmation. Six cases were associated with other cardiovascular abnormalities. Key MRI features of fetal TAPVC included a dilated coronary sinus and vertical vein. Fetal pulmonary edema was seen in six cases. Compared to controls, TAPVC fetuses had lower cerebellum and brainstem volumes and higher e-CSF, while had larger subcortical brain tissue, cerebellum, brainstem, e-CSF, and intracranial cavity volumes than those of TOF cases. DATA CONCLUSION Fetal MRI may be a useful modality for evaluating fetal TAPVC and altered brain development. EVIDENCE LEVEL 3 TECHNICAL EFFICACY: Stage 3.
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Affiliation(s)
- Jing-Ya Ren
- Department of Radiology, Shanghai Children's Medical Center, Shanghai Jiao Tong University School of Medicine, Shanghai, China
| | - Chang-An Chen
- Department of Radiology, Shanghai Children's Medical Center, Shanghai Jiao Tong University School of Medicine, Shanghai, China
| | - Ming Zhu
- Department of Radiology, Shanghai Children's Medical Center, Shanghai Jiao Tong University School of Medicine, Shanghai, China
| | - Ke Liu
- Department of Radiology, Shanghai Children's Medical Center, Shanghai Jiao Tong University School of Medicine, Shanghai, China
| | - Li-Jun Chen
- Department of Cardiology, Shanghai Children's Medical Center, Shanghai Jiao Tong University School of Medicine, Shanghai, China
| | - Su-Zhen Dong
- Department of Radiology, Shanghai Children's Medical Center, Shanghai Jiao Tong University School of Medicine, Shanghai, China
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Yang X, Su XH, Zeng Z, Fan Y, Wu Y, Guo LL, Xu XY. Integrated analysis of comorbidity, pregnant outcomes, and amniotic fluid cytogenetics of fetuses with persistent left superior vena cava. World J Cardiol 2023; 15:500-507. [PMID: 37900905 PMCID: PMC10600788 DOI: 10.4330/wjc.v15.i10.500] [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: 07/09/2023] [Revised: 08/29/2023] [Accepted: 09/28/2023] [Indexed: 10/24/2023] Open
Abstract
BACKGROUND Persistent left superior vena cava (PLSVC) is the most common venous system variant. The clinical characteristics and amniotic fluid cytogenetics of fetuses with PLSVC remain to be further explored. AIM To develop reliable prenatal diagnostic recommendations through integrated analysis of the clinical characteristics of fetuses with PLSVC. METHODS Cases of PLSVC diagnosed using prenatal ultrasonography between September 2019 and November 2022 were retrospectively studied. The clinical characteristics of the pregnant women, ultrasonic imaging information, gestational age at diagnosis, pregnancy outcomes, and amniocentesis results were summarized and analyzed using categorical statistics and the chi-square test or Fisher's exact test. RESULTS Of the 97 cases diagnosed by prenatal ultrasound, 49 (50.5%) had isolated PLSVC and 48 (49.5%) had other structural abnormalities. The differences in pregnancy outcomes and amniocentesis conditions between the two groups were statistically significant (P < 0.05). No significant differences were identified between the two groups in terms of advanced maternal age and gestational age (P > 0.05). According to the results of the classification statistics, the most common intracardiac abnormality was a ventricular septal defect and the most common extracardiac abnormality was a single umbilical artery. In the subgroup analysis, the concurrent combination of intra- and extracardiac structural abnormalities was a risk factor for adverse pregnancy outcomes (odds ratio > 1, P < 0.05). Additionally, all abnormal cytogenetic findings on amniocentesis were observed in the comorbidity group. One case was diagnosed with 21-trisomy and six cases was diagnosed with chromosome segment duplication. CONCLUSION Examination for other structural abnormalities is strongly recommended when PLSVC is diagnosed. Poorer pregnancy outcomes and increased amniocentesis were observed in PLSVC cases with other structural abnormalities. Amniotic fluid cytogenetics of fetuses is recommended for PLSVC with other structural abnormalities.
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Affiliation(s)
- Xin Yang
- Department of Obstetrics and Gynecology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan 430030, Hubei Province, China
| | - Xin-Hui Su
- Department of Obstetrics and Gynecology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan 430030, Hubei Province, China
| | - Zhen Zeng
- Department of Obstetrics and Gynecology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan 430030, Hubei Province, China
| | - Yao Fan
- Department of Obstetrics and Gynecology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan 430030, Hubei Province, China
| | - Yuan Wu
- Department of Obstetrics and Gynecology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan 430030, Hubei Province, China
| | - Li-Li Guo
- Department of Obstetrics and Gynecology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan 430030, Hubei Province, China
| | - Xiao-Yan Xu
- Department of Obstetrics and Gynecology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan 430030, Hubei Province, China.
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Lopes KRM, Bartsota M, Doughty V, Carvalho JS. Single left superior vena cava: antenatal diagnosis, associated anomalies and outcomes. ULTRASOUND IN OBSTETRICS & GYNECOLOGY : THE OFFICIAL JOURNAL OF THE INTERNATIONAL SOCIETY OF ULTRASOUND IN OBSTETRICS AND GYNECOLOGY 2022; 60:640-645. [PMID: 35656845 PMCID: PMC9828089 DOI: 10.1002/uog.24966] [Citation(s) in RCA: 7] [Impact Index Per Article: 2.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Received: 02/04/2022] [Revised: 05/17/2022] [Accepted: 05/25/2022] [Indexed: 06/04/2023]
Abstract
OBJECTIVES To describe the associated cardiac and extracardiac findings and estimate the prevalence of single left superior vena cava (LSVC) among fetuses referred for fetal echocardiography. METHODS This was a retrospective case series of fetuses diagnosed with situs solitus and single LSVC at the Brompton Centre for Fetal Cardiology, London, UK, from October 2006 to December 2020. Prenatal and postnatal outcome data were collected. Prenatal diagnosis was based on abnormal vessel alignment at the three-vessel view and/or three-vessel-and-trachea view, showing a vessel to the left of the pulmonary artery (i.e. the LSVC) and absence of the usual vessel to the right of the ascending aorta (i.e. the right superior vena cava), and further visualization of the LSVC draining into the coronary sinus. RESULTS Of 19 968 fetal echocardiograms performed during the study period, 34 cases of single LSVC were identified (a prevalence of 0.17%). Of these, 32 pregnancies had a live birth, one was lost to follow-up and one resulted in intrauterine demise. Single LSVC was isolated in 79.4% of cases. No major congenital heart disease was identified. One fetus showed mild isthmus hypoplasia, with no aortic coarctation postnatally. Two fetuses had umbilical vessel abnormalities. A genetic abnormality was found in one case (15q24.1-q24.2 deletion). CONCLUSIONS Antenatal diagnosis of single LSVC in the setting of situs solitus is usually a benign isolated finding. Nevertheless, investigation of other cardiac, extracardiac and genetic disorders should be considered. © 2022 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)
- K. R. M. Lopes
- Brompton Centre for Fetal CardiologyRoyal Brompton and Harefield HospitalsLondonUK
- Fetal Medicine UnitSt George's University Hospitals NHS Foundation TrustLondonUK
| | - M. Bartsota
- Brompton Centre for Fetal CardiologyRoyal Brompton and Harefield HospitalsLondonUK
| | - V. Doughty
- Brompton Centre for Fetal CardiologyRoyal Brompton and Harefield HospitalsLondonUK
| | - J. S. Carvalho
- Brompton Centre for Fetal CardiologyRoyal Brompton and Harefield HospitalsLondonUK
- Fetal Medicine UnitSt George's University Hospitals NHS Foundation TrustLondonUK
- Cardiovascular Clinical Academic GroupSt George's University of London Molecular and Clinical Sciences Research InstituteLondonUK
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Tonni G, Bonasoni MP, Grisolia G, Bellotti M, Araujo Júnior E. Heterotaxy Syndrome with Increased Nuchal Translucency and Normal Karyotype Associated with Complex Systemic Venous Return. Ultrasound Diagnosis with Autopsy Correlation. Fetal Pediatr Pathol 2022; 41:852-860. [PMID: 34629036 DOI: 10.1080/15513815.2021.1988011] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 10/20/2022]
Abstract
Background: Prenatal ultrasound (US) detection of heterotaxy syndrome can be challenging, especially in identifying cardiovascular and associated anomalies. We present a new case of heterotaxy syndrome with anomalous systemic venous return (ASVR) fully displayed at autopsy. Case report: Left heterotaxy syndrome was diagnosed in a 19 weeks' of gestation fetus with right-sided stomach. The heart showed both ventricles with left morphology, a large ventricular septal defect, persistent left superior vena cava draining into the coronary sinus, ASVR with interrupted inferior vena cava (IVC) and azygous continuation. Autopsy dissection further identified the azygous draining into the left lower pulmonary vein (LLPV). Prenatal a-CGH on villous sampling showed 22q13.1 microduplication inherited from the father, not contributory to the phenotype. Conclusion/discussion: Heterotaxy syndrome requires US accuracy for anomaly identification, as they allow legal termination of pregnancy. Our case is unusual as IVC drained into the azygous vein and then into the LLPV.
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Affiliation(s)
- Gabriele Tonni
- Department of Obstetrics and Neonatology, Istituto di Ricovero e Cura a Carattere Scientifico (IRCCS), AUSL di Reggio Emilia, Reggio Emilia, Italy
| | - Maria Paola Bonasoni
- Department of Pathology Unit, Istituto di Ricovero e Cura a Carattere Scientifico (IRCCS), AUSL di Reggio Emilia, Reggio Emilia, Italy
| | - Gianpaolo Grisolia
- Department of Obstetrics and Gynecology, Carlo Poma Hospital, Mantua, Italy
| | | | - Edward Araujo Júnior
- Department of Obstetrics, Paulista School of Medicine - Federal University of São Paulo (EPM-UNIFESP), Sao Paulo, Brazil
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Keleş A, Yılmaz O, Dağdeviren G, Çelik ÖY, Yücel A, Şahin D. Persistent Left Superior Vena Cava: Why is Prenatal Diagnosis Important? Fetal Pediatr Pathol 2022; 41:592-602. [PMID: 34106033 DOI: 10.1080/15513815.2021.1933662] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/12/2023]
Abstract
ObjectiveTo investigate fetal anomalies and pregnancy outcomes in pregnancies with persistent left superior vena cava (PLSVC) to provide assistance in prenatal counseling.MethodsCases diagnosed with PLSVC between January 2015 and January 2020 were obtained from the hospital's electronic system and were analyzed retrospectively.ResultsTwenty-seven cases were analyzed. The prevalence of PLSVC among congenital heart diseases (CHD) was 6.9%. Conotruncal anomalies and renal anomalies were the most common accompanying cardiac and extracardiac anomalies, respectively. Chromosomal abnormality was detected in one fetus.In the postpartum period coarctation of aorta (CoA) was found in one fetus.ConclusionsWhen PLSVC is detected during prenatal ultrasonography, fetal anatomy should be carefully examined because of the anomalies that may accompany it. Prenatal genetic counseling should be given especially to cases with additional anomalies. In isolated cases, cardiac anatomy should be evaluated with repeated echocardiography because of the risk of CoA.
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Affiliation(s)
- Ayşe Keleş
- Perinatology Department, University of Health Sciences Etlik Zübeyde Hanım Women's Health Care, Training and Research Hospital, Ankara, Turkey
| | - Osman Yılmaz
- Pediatric Cardiology Department, University of Health Sciences Etlik Zübeyde Hanım Women's Health Care, Training and Research Hospital, Ankara, Turkey
| | - Gülşah Dağdeviren
- Perinatology Department, University of Health Sciences Etlik Zübeyde Hanım Women's Health Care, Training and Research Hospital, Ankara, Turkey
| | - Özge Yücel Çelik
- Perinatology Department, University of Health Sciences Etlik Zübeyde Hanım Women's Health Care, Training and Research Hospital, Ankara, Turkey
| | - Aykan Yücel
- Perinatology Department, Ministry of Health Ankara City Hospital, Ankara, Turkey
| | - Dilek Şahin
- Perinatology Department, Ministry of Health Ankara City Hospital, Ankara, Turkey
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7
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Poenaru MO, Hamoud BH, Sima RM, Valcea ID, Chicea R, Ples L. Persistent Left Superior Vena Cava Significance in Prenatal Diagnosis—Case Series. J Clin Med 2022; 11:jcm11144020. [PMID: 35887792 PMCID: PMC9316240 DOI: 10.3390/jcm11144020] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/20/2022] [Revised: 06/24/2022] [Accepted: 06/30/2022] [Indexed: 11/16/2022] Open
Abstract
The persistent left superior vena cava (PLSVC) is a congenital heart anomaly reported in 0.3–0.5% of the general population and can be associated with congenital heart diseases in up to 8% of cases. Prenatal identification of PLSVC is important to prompt an extended cardiac and extracardiac fetal examination. We retrospectively reevaluated anomaly scans performed in our unit in a 2-year interval according to the national guidelines to evaluate the incidence of PLSVC and its association with prenatal morbidity. In our population, the incidence of PLSVC was 0.31%, and we found a low association with cardiac and extracardiac anomalies. The standard sections (three-vessel and trachea view, four-chamber view and outflow tract’s view) are insufficient to exclude cardiac anomalies whenever PLSVC is found. In our case series, only one newborn required postnatal surgery for total pulmonary vein anomaly, and at 2 years of life all babies had a normal evolution. Prenatal diagnosis of PLSVC can raise counseling issues; therefore, awareness of its good outcome when isolated and need for an extended examination to rule out other anomalies is very important.
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Affiliation(s)
- Mircea-Octavian Poenaru
- Department of Obstetrics and Gynecology, The ‘Carol Davila’ University of Medicine and Pharmacy, 020021 Bucharest, Romania; (M.-O.P.); (L.P.)
- The “Bucur” Maternity—‘Saint John’ Hospital, 040294 Bucharest, Romania;
| | - Bashar Haj Hamoud
- Department for Gynecology, Obstetrics and Reproductive Medicine, Saarland University Hospital, 66421 Homburg, Germany;
| | - Romina-Marina Sima
- Department of Obstetrics and Gynecology, The ‘Carol Davila’ University of Medicine and Pharmacy, 020021 Bucharest, Romania; (M.-O.P.); (L.P.)
- The “Bucur” Maternity—‘Saint John’ Hospital, 040294 Bucharest, Romania;
- Correspondence:
| | | | - Radu Chicea
- Faculty of Medicine, ‘Lucian Blaga’ University of Sibiu, 550024 Sibiu, Romania;
| | - Liana Ples
- Department of Obstetrics and Gynecology, The ‘Carol Davila’ University of Medicine and Pharmacy, 020021 Bucharest, Romania; (M.-O.P.); (L.P.)
- The “Bucur” Maternity—‘Saint John’ Hospital, 040294 Bucharest, Romania;
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8
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Esin D, Aslan Çetin B, Şenol G, Selçuki NFT, Gedik Özköse Z, Acar Z, Yüksel MA. Clinical significance of prenatally diagnosed persistent left superior vena cava. J Gynecol Obstet Hum Reprod 2022; 51:102332. [PMID: 35123124 DOI: 10.1016/j.jogoh.2022.102332] [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: 05/14/2020] [Revised: 02/22/2021] [Accepted: 02/01/2022] [Indexed: 11/28/2022]
Abstract
AIMS The aim of our study was to investigate the persistent left superior vena cava (PLSVC) cases diagnosed in the prenatal period in our clinic and to compare the obstetric and genetic outcomes of isolated PLSVC cases with cases accompanied by other cardiac or extracardiac anomalies. METHODS The cases diagnosed as PLSVC between January 2015 and January 2019 in our perinatology clinic were evaluated retrospectively. Patients were divided into two subgroups as isolated PLSVC and PLSVC accompanied by another anomaly. Furthermore, patients with extra anomalies were divided into three groups which are cardiac anomaly, extracardiac anomaly and those with both. The groups were compared in terms of genetic results and obstetric outcomes. RESULTS 89 patients were included in our study. Cases with positive pregnancy outcomes were significantly higher in the isolated PLSVC group than with extra anomaly group (p<0.001). No karyotype anomaly was observed in the isolated group. Pregnancy results were significantly worse (postpartum demise, termination of pregnancy, in utero demise) in with both cardiac and extracardiac anomalies group (p<0.001). There was no significant difference between the groups in terms of karyotype results (p=0.535). CONCLUSION The diagnosis of PLSVC has gained importance and it can be made easier due to the fact that anatomic imaging can be performed in more detail. The isolated PLSVC cases have a very good prognosis. Obstetric outcomes vary according to the accompanying anomaly.
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Affiliation(s)
- Didem Esin
- Kanuni Sultan Suleyman Research and Training Hospital, Department of Obstetrics and Gynecology, Istanbul, Turkey
| | - Berna Aslan Çetin
- Kanuni Sultan Suleyman Research and Training Hospital, Department of Obstetrics and Gynecology, Istanbul, Turkey.
| | - Gökalp Şenol
- Osmangazi University, Department of Perinatology, Eskişehir, Turkey
| | - Nura Fitnat Tobaş Selçuki
- Şişli Hamidiye Etfal Research and Training Hospital, Department of Obstetrics and Gynecology, Istanbul, Turkey
| | - Zeynep Gedik Özköse
- Kanuni Sultan Suleyman Research and Training Hospital, Department of Obstetrics and Gynecology, Istanbul, Turkey
| | - Zuat Acar
- Şişli Hamidiye Etfal Research and Training Hospital, Department of Obstetrics and Gynecology, Istanbul, Turkey
| | - Mehmet Aytaç Yüksel
- Beykent University Medical Faculty, Department of Obstetrics and Gynecology, Istanbul, Turkey
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Galal M, Ghoneem A, Hafeez M, El Bolkini M. Case 2: An Uncommon Peripherally Inserted Central Catheter Position with an Uncommon Diagnosis. Neoreviews 2020; 21:e765-e767. [PMID: 33139514 DOI: 10.1542/neo.21-11-e765] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/24/2022]
Affiliation(s)
- Mahmoud Galal
- Department of Pediatrics, Neonatal Intensive Care Unit, Dubai Hospital, Dubai.,Department of Pediatrics, Dubai Medical College, Dubai
| | - Alaa Ghoneem
- Department of Pediatrics, Neonatal Intensive Care Unit, Dubai Hospital, Dubai
| | - Muzammil Hafeez
- Department of Pediatrics, Neonatal Intensive Care Unit, Dubai Hospital, Dubai
| | - Mostafa El Bolkini
- Department of Pediatrics, Neonatal Intensive Care Unit, Dubai Hospital, Dubai
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10
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Batouty NM, Sobh DM, Gadelhak B, Sobh HM, Mahmoud W, Tawfik AM. Left superior vena cava: cross-sectional imaging overview. Radiol Med 2019; 125:237-246. [PMID: 31823296 DOI: 10.1007/s11547-019-01114-9] [Citation(s) in RCA: 22] [Impact Index Per Article: 3.7] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/31/2019] [Accepted: 11/13/2019] [Indexed: 02/07/2023]
Abstract
Persistent left-sided superior vena cava (PLSVC) is the commonest systemic venous anomaly in the thorax with a reported prevalence of up to 0.5% in otherwise normal population and up to 10% in patients with congenital heart disease (CHD). In the absence of associated CHD, it is usually asymptomatic, discovered incidentally. It may complicate catheter or pacemaker lead placement. PLSVC typically drains into the right atrium through the coronary sinus. In children with CHD, the presence of a PLSVC may affect the choice of certain surgical procedures. PLSVC is significantly more common in association with situs ambiguous than with situs solitus or inversus, up to 60-70%. In patients with situs ambiguous, the drainage of LSVC is variable, more commonly directly into the atria rather than through the coronary sinus (CS). Rarely, there is a PLSVC draining into the CS with absent right SVC. PLSVC draining into the right atrium via the CS will not usually cause blood shunting between the right and the left sides. However, shunting occurs when PLSVC is associated with unroofed CS, or when it directly drains into the left atrium. With an increased use of CT and MRI for chest and cardiac imaging, PLSVC is being more encountered by radiologists than before. In this article, we will discuss the embryology of PLSVC, its anatomic course and drainage pathways, as well as its clinical relevance and relation to congenital heart disease and viscero-atrial situs.
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Affiliation(s)
- Nihal M Batouty
- Department of Diagnostic and Interventional Radiology, Faculty of Medicine, Mansoura University Hospital, 12 El-Gomhoreya Street, Mansoura, 35112, Egypt
| | - Donia M Sobh
- Department of Diagnostic and Interventional Radiology, Faculty of Medicine, Mansoura University Hospital, 12 El-Gomhoreya Street, Mansoura, 35112, Egypt
| | - Basma Gadelhak
- Department of Diagnostic and Interventional Radiology, Faculty of Medicine, Mansoura University Hospital, 12 El-Gomhoreya Street, Mansoura, 35112, Egypt
| | - Hoda M Sobh
- Department of Cardiology, Faculty of Medicine, Mansoura University, Mansoura, Egypt
| | - Walaa Mahmoud
- Department of Diagnostic and Interventional Radiology, Faculty of Medicine, Mansoura University Hospital, 12 El-Gomhoreya Street, Mansoura, 35112, Egypt
| | - Ahmed M Tawfik
- Department of Diagnostic and Interventional Radiology, Faculty of Medicine, Mansoura University Hospital, 12 El-Gomhoreya Street, Mansoura, 35112, Egypt.
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11
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Wertaschnigg D, Rolnik DL, Ramkrishna J, da Silva Costa F, Meagher S. The gap between the aorta and the superior vena cava: A sonographic sign of persistent left superior vena cava and associated abnormalities. Prenat Diagn 2019; 39:1213-1219. [PMID: 31600828 DOI: 10.1002/pd.5569] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/28/2019] [Revised: 09/01/2019] [Accepted: 09/09/2019] [Indexed: 11/05/2022]
Abstract
OBJECTIVES To assess the distance between the right superior vena cava (SVC) and the aorta in fetuses with bilateral superior vena cava as a possible sonographic marker for this. METHODS This was a nested case-control study including 20 cases of bilateral SVC and 40 gestational age-matched controls. The distance between the right SVC and the aorta was measured at the level of the three-vessel trachea view in stored images, as well as the diameters of the aorta and the right SVC. RESULTS The distance between the aorta and the right SVC was significantly larger in the cases of a left SVC compared with controls, P < .001. A distance of 2.0 mm or more was found in 70% of the cases and 5% of the controls, with a gestational-age adjusted area under the receiver-operating characteristics (ROC) curve for the diagnosis of left SVC of 0.93 (95% CI 0.87-0.99). The aorta and the right SVC were significantly smaller in cases compared with controls, and there was a significant association with other cardiac and extracardiac abnormalities amongst cases of persistent left SVC. CONCLUSION An increased distance between the aorta and the right SVC is associated with the diagnosis of bilateral SVC.
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Affiliation(s)
- Dagmar Wertaschnigg
- Department of Obstetrics and Gynaecology, Monash University, Melbourne, Victoria, Australia.,Department of Obstetrics and Gynecology, Paracelsus Medical University, Salzburg, Austria
| | - Daniel L Rolnik
- Department of Obstetrics and Gynaecology, Monash University, Melbourne, Victoria, Australia.,Monash Ultrasound for Women, Melbourne, Victoria, Australia.,School of Clinical Sciences, Monash University, Melbourne, Victoria, Australia
| | | | - Fabricio da Silva Costa
- Department of Obstetrics and Gynaecology, Monash University, Melbourne, Victoria, Australia.,Department of Gynecology and Obstetrics, Ribeirão Preto Medical School, University of São Paulo, Ribeirão Preto, São Paulo, Brazil
| | - Simon Meagher
- Monash Ultrasound for Women, Melbourne, Victoria, Australia
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Özsürmeli M, Büyükkurt S, Sucu M, Arslan E, Akçabay Ç, Mısırlıoğlu S, Kayapınar M, Özbarlas N, Demir SC, Evrüke C. Prenatal diagnosis of persistent left superior vena cava: a retrospective study of associated congenital anomalies. Turk J Obstet Gynecol 2019; 16:23-28. [PMID: 31019836 PMCID: PMC6463432 DOI: 10.4274/tjod.galenos.2019.02679] [Citation(s) in RCA: 7] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/05/2018] [Accepted: 01/06/2019] [Indexed: 12/02/2022] Open
Abstract
Objective: To evaluate persistent left superior vena cava (PLSVC) cases according to associated cardiac, extracardiac, and chromosomal anomalies in the prenatal period and to review their outcomes. Materials and Methods: The data of patients with a prenatal diagnosis of PLSVC between January 2013 and December 2017 were reviewed retrospectively. Results: Data of 32 cases were reviewed. Nineteen (60%) cases were associated with cardiac defects, 5 (15%) were associated with both cardiac and extracardiac defects, and 8 (25%) had no associated anomalies. Two fetuses had karyotype anomalies. All patients with isolated PLSVC survived. Among the cases associated with extracardiac anomalies, cardiac anomalies, and with both extracardiac and cardiac anomalies, the survival rate was 40%, 40%, and 25%, respectively. Outcome was more favorable in cases with isolated PLSVC (100% vs. 40%). Conclusion: Prenatally diagnosed PLSVC is associated with cardiac and extracardiac anomalies in the majority of cases. The prognosis is good in isolated cases, but worsens when accompanied by cardiac or extracardiac anomalies.
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Affiliation(s)
- Mehmet Özsürmeli
- University of Health Sciences, Derince Training and Research Hospital, Clinic of Obstetrics and Gynecology, Kocaeli, Turkey
| | - Selim Büyükkurt
- Çukurova University Faculty of Medicine, Department of Obstetrics and Gynecology, Perinatology Unit, Adana, Turkey
| | - Mete Sucu
- Çukurova University Faculty of Medicine, Department of Obstetrics and Gynecology, Perinatology Unit, Adana, Turkey
| | - Erol Arslan
- Çukurova University Faculty of Medicine, Department of Obstetrics and Gynecology, Perinatology Unit, Adana, Turkey
| | - Çiğdem Akçabay
- Çukurova University Faculty of Medicine, Department of Obstetrics and Gynecology, Perinatology Unit, Adana, Turkey
| | - Selahattin Mısırlıoğlu
- Çukurova University Faculty of Medicine, Department of Obstetrics and Gynecology, Perinatology Unit, Adana, Turkey
| | - Masum Kayapınar
- Çukurova University Faculty of Medicine, Department of Obstetrics and Gynecology, Perinatology Unit, Adana, Turkey
| | - Nazan Özbarlas
- Çukurova University Faculty of Medicine, Department of Pediatrics, Pediatric Cardiology Unit, Adana, Turkey
| | - Süleyman Cansun Demir
- Çukurova University Faculty of Medicine, Department of Obstetrics and Gynecology, Perinatology Unit, Adana, Turkey
| | - Cüneyt Evrüke
- Çukurova University Faculty of Medicine, Department of Obstetrics and Gynecology, Perinatology Unit, Adana, Turkey
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Kao CC, Hsieh CC, Cheng PJ, Chiang CH, Huang SY. Total Anomalous Pulmonary Venous Connection: From Embryology to a Prenatal Ultrasound Diagnostic Update. J Med Ultrasound 2017; 25:130-137. [PMID: 30065477 PMCID: PMC6029298 DOI: 10.1016/j.jmu.2017.08.002] [Citation(s) in RCA: 13] [Impact Index Per Article: 1.6] [Reference Citation Analysis] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/16/2017] [Accepted: 08/02/2017] [Indexed: 01/03/2023] Open
Affiliation(s)
- Chuan-Chi Kao
- Department of Obstetrics and Gynecology, Keelung Chang Gung Memorial Hospital, Keelung, Taiwan
| | - Ching-Chang Hsieh
- Department of Obstetrics and Gynecology, Taipei Chang Gung Memorial Hospital, Taipei, Taiwan
| | - Po-Jen Cheng
- Department of Obstetrics and Gynecology, Chang Gung Memorial Hospital, Chang Gung University College of Medicine, Tao-Yuan, Taiwan
| | - Chi-Hsin Chiang
- Department of Obstetrics and Gynecology, Taipei Chang Gung Memorial Hospital, Taipei, Taiwan
| | - Shih-Yin Huang
- Department of Obstetrics and Gynecology, Keelung Chang Gung Memorial Hospital, Keelung, Taiwan
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14
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Gustapane S, Leombroni M, Khalil A, Giacci F, Marrone L, Bascietto F, Rizzo G, Acharya G, Liberati M, D'Antonio F. Systematic review and meta-analysis of persistent left superior vena cava on prenatal ultrasound: associated anomalies, diagnostic accuracy and postnatal outcome. ULTRASOUND IN OBSTETRICS & GYNECOLOGY : THE OFFICIAL JOURNAL OF THE INTERNATIONAL SOCIETY OF ULTRASOUND IN OBSTETRICS AND GYNECOLOGY 2016; 48:701-708. [PMID: 26970258 DOI: 10.1002/uog.15914] [Citation(s) in RCA: 47] [Impact Index Per Article: 5.2] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Received: 01/18/2016] [Revised: 02/29/2016] [Accepted: 03/05/2016] [Indexed: 02/05/2023]
Abstract
OBJECTIVES To quantify the prevalence of chromosomal anomalies in fetuses with persistent left superior vena cava (PLSVC), assess the strength of the association between PLSVC and coarctation of the aorta and ascertain the diagnostic accuracy of antenatal ultrasound in correctly identifying isolated cases of PLSVC. METHODS MEDLINE, EMBASE, CINHAL and the Cochrane databases were searched from the year 2000 onwards using combinations of keywords 'left superior vena cava' and 'outcome'. Two authors reviewed all abstracts independently. Quality assessment of the included studies was performed using the Newcastle-Ottawa Scale for cohort studies. The rates of the following outcomes were analyzed: chromosomal abnormalities; associated intracardiac anomalies (ICAs) and extracardiac anomalies (ECAs) diagnosed prenatally; additional ICAs and ECAs detected only at postnatal imaging or clinical evaluation but missed at prenatal imaging; and association of PLSVC and coarctation of the aorta. Meta-analyses of proportions were used to combine data. RESULTS In total, 2708 articles were identified and 13 (n = 501) were included in the systematic review. Associated ICAs and ECAs were detected at the prenatal ultrasound examination or at a follow-up assessment in 60.7% (95% CI, 44.2-75.9%) and 37.8% (95% CI, 31.0-44.8%) of cases, respectively. Chromosomal anomalies occurred in 12.5% (95% CI, 9.0-16.4%) of cases in the overall population of fetuses with PLSVC and in 7.0% (95% CI, 2.7-13.0%) of isolated cases. Additional ICAs and ECAs were detected only after birth and missed at ultrasound in 2.4% (95% CI, 0.5-5.8%) and 6.7% (95% CI, 2.2-13.2%) of cases, respectively. Coarctation of the aorta was associated with isolated PLSVC in 21.3% (95% CI, 13.6-30.3%) of cases. CONCLUSIONS PLSVC is commonly associated with ICAs, ECAs and chromosomal anomalies. Fetuses with isolated PLSVC should be followed up throughout pregnancy in order to rule out coarctation of the aorta. As most of the data in this review were derived from high-risk pregnancies, the rate of associated abnormalities is likely to be higher than that in the general population of fetuses with PLSVC, for which more data are needed. Revisión sistemática y metaanálisis de la persistencia de la vena cava superior izquierda en la ecografía prenatal: anomalías asociadas, precisión del diagnóstico y resultado postnatal RESUMEN OBJETIVOS: Cuantificar la prevalencia de anomalías cromosómicas en fetos con vena cava superior izquierda persistente (VCSIP), evaluar la solidez de la asociación entre la VCSIP y la coartación aórtica, y determinar la precisión del diagnóstico de la ecografía prenatal como método para identificar correctamente casos aislados de VCSIP. MÉTODOS: Se buscó en las bases de datos de MEDLINE, EMBASE, CINHAL y Cochrane artículos publicados desde el año 2000 en adelante, usando combinaciones de las palabras clave "vena cava superior izquierda" y "resultado". Dos de los autores revisaron de forma independiente todos los resúmenes encontrados. La evaluación de calidad de los estudios incluidos se realizó mediante la escala Newcastle-Ottawa para estudios de cohortes. Se analizaron las tasas de los siguientes resultados: anomalías cromosómicas; anomalías intracardíacas (AIC) y anomalías extracardíacas (AEC) asociadas diagnosticadas prenatalmente; AIC y AEC adicionales detectadas sólo en ecografías postnatales o mediante evaluación clínica, pero no observadas en ecografías prenatales; y la asociación entre la VCSIP y la coartación aórtica. Se utilizó un meta-análisis de proporciones para combinar los datos. RESULTADOS En total, se identificaron 2708 artículos y se incluyeron 13 (n = 501) en la revisión sistemática. En la ecografía prenatal o en una revisión de seguimiento se detectaron AIC y AEC asociadas en el 60,7% (IC 95%, 44,2-75,9%) y el 37,8% (IC 95%, 31,0-44,8%) de los casos, respectivamente. Se produjeron anomalías cromosómicas en el 12,5% (IC 95%, 9,0-16,4%) de los casos en la población general de fetos con VCSIP y en el 7,0% (IC 95%, 2,7-13,0%) de casos aislados. Las AIC y AEC adicionales sólo se detectaron después del nacimiento y en el 6,7% (IC 95%, 2,2-13,2%) de los casos, respectivamente. La coartación aórtica se encontró asociada con la VCSIP aislada en un 21,3% (IC 95%, 13,6-30,3%) de los casos. CONCLUSIONES La VCSIP está comúnmente asociada a AIC, AEC y anomalías cromosómicas. Los fetos con VCSIP aislada deben ser objeto de seguimiento durante todo el embarazo, con el fin de descartar la coartación aórtica. Como la mayoría de los datos de esta revisión proceden de embarazos de alto riesgo, es probable que la tasa de anomalías asociadas sea más alta que la de la población general de fetos con VCSIP, por lo que se necesitan más datos.
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Affiliation(s)
- S Gustapane
- Department of Obstetrics and Gynaecology, SS. Annunziata Hospital, G. D'Annunzio University of Chieti-Pescara, Chieti, Italy
| | - M Leombroni
- Department of Obstetrics and Gynaecology, SS. Annunziata Hospital, G. D'Annunzio University of Chieti-Pescara, Chieti, Italy
| | - A Khalil
- Fetal Medicine Unit, Division of Developmental Sciences, St George's University of London, London, UK
| | - F Giacci
- Department of Obstetrics and Gynaecology, SS. Annunziata Hospital, G. D'Annunzio University of Chieti-Pescara, Chieti, Italy
| | - L Marrone
- Department of Obstetrics and Gynaecology, SS. Annunziata Hospital, G. D'Annunzio University of Chieti-Pescara, Chieti, Italy
| | - F Bascietto
- Department of Obstetrics and Gynaecology, SS. Annunziata Hospital, G. D'Annunzio University of Chieti-Pescara, Chieti, Italy
| | - G Rizzo
- Department of Obstetrics and Gynecology, Università di Roma, Rome, Italy
| | - G Acharya
- Women's Health and Perinatology Research Group, Department of Clinical Medicine, Faculty of Health Sciences, UiT - The Arctic University of Norway, Tromsø, Norway
- Department of Obstetrics and Gynaecology, University Hospital of Northern Norway, Tromsø, Norway
| | - M Liberati
- Department of Obstetrics and Gynaecology, SS. Annunziata Hospital, G. D'Annunzio University of Chieti-Pescara, Chieti, Italy
| | - F D'Antonio
- Women's Health and Perinatology Research Group, Department of Clinical Medicine, Faculty of Health Sciences, UiT - The Arctic University of Norway, Tromsø, Norway
- Department of Obstetrics and Gynaecology, University Hospital of Northern Norway, Tromsø, Norway
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15
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Du L, Xie HN, Zhu YX, Li LJ, Peng R, Zheng J. Fetal persistent left superior vena cava in cases with and without chromosomal anomalies. Prenat Diagn 2014; 34:797-802. [PMID: 24711103 DOI: 10.1002/pd.4380] [Citation(s) in RCA: 23] [Impact Index Per Article: 2.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/20/2013] [Revised: 03/18/2014] [Accepted: 04/03/2014] [Indexed: 11/11/2022]
Abstract
OBJECTIVES The objectives of this study are to determine and compare the prevalence of persistent left superior vena cava (PLSVC) in chromosomally normal and abnormal fetuses and to evaluate the potential of PLSVC as a screening marker for chromosomal abnormalities. METHODS Women undergoing routine fetal sonographic examinations were evaluated once for the presence of PLSVC. PLSVC was diagnosed on the basis of the identification of an additional vessel in the left of the pulmonary artery in a three-vessel trachea view. Associated abnormalities, karyotypes, and outcomes were analyzed. RESULTS A total of 164 (0.7%, 164/25 171) cases of PLSVC were detected and successfully followed-up. The detection rates were 0.5% (81/17 535) and 1.1% (83/7636) in the low-risk and high-risk cases, respectively. The incidence of PLSVC was lower among the chromosomally or clinically normal (0.4%, 110/24 914) compared with chromosomally abnormal fetuses (7.8%, 20/257, p < 0.001). Additional defects were identified in 90% (18/20) of the PLSVC fetuses with chromosomal anomalies, a rate that was higher than those fetuses with chromosomal normal (61.8%, 68/110). CONCLUSIONS Persistent left superior vena cava is more common among chromosomally abnormal than normal fetuses, and PLSVC fetuses with other defects are more highly associated with chromosomal disorders than isolated PLSVC fetuses. Isolated PLSVC is a benign vascular anomaly and may not affect outcomes.
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Affiliation(s)
- Liu Du
- Department of Ultrasonic Medicine and Fetal Medical Centre, The First Affiliated Hospital of Sun Yat-sen University, Guangzhou, Guangdong, China
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16
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Esmer AÇ, Yüksel A, Calı H, Ozsürmeli M, Omeroğlu RE, Kalelioğlu I, Has R. Prenatal diagnosis of persistent left superior vena cava and its clinical significance. Balkan Med J 2014; 31:50-4. [PMID: 25207167 DOI: 10.5152/balkanmedj.2014.9426] [Citation(s) in RCA: 14] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/07/2013] [Accepted: 12/26/2013] [Indexed: 11/22/2022] Open
Abstract
BACKGROUND Persistent left superior vena cava (PLSVC) is a variant of systemic venous return which is observed in 0.3% of autopsies in the general population and in 4-8% of patients with congenital heart disease. AIMS To evaluate associated cardiac, extracardiac and chromosomal anomalies in prenatally diagnosed cases of PLSVC and to review their outcome. STUDY DESIGN Retrospective comparative study. METHODS The data of patients with a prenatal diagnosis of PLSVC between May 2008 and January 2013 were reviewed retrospectively. RESULTS Data of 31 cases were reviewed. Fifteen (48.4%) cases were associated with cardiac defects and 17 (54.8%) cases had associated extracardiac sonographic or postpartum findings. Two fetuses had karyotype anomalies. Outcome was significantly more favorable in cases not associated with cardiac defects in comparison to those associated with cardiac anomalies (84.6% vs. 33.3%, p=0.009). All cases with isolated PLSVC survived, while among the cases associated with extracardiac anomalies, with cardiac anomalies and with both extra-cardiac and cardiac anomalies, the survival rate was 75%, 50% and 22.2%, respectively. The most frequent group of cardiac anomalies associated with PLSVC was septal defects and VSD was the most common heart defect individually, being observed in nine fetuses. CONCLUSION Prenatally diagnosed PLSVC is associated with cardiac and extracardiac anomalies in the majority of cases. Outcome is significantly worse if PLSVC is associated with a cardiac defect, and the prognosis is excellent in isolated cases.
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Affiliation(s)
- Aytül Çorbacıoğlu Esmer
- Department of Obstetrics and Gynecology, İstanbul University Faculty of Medicine, İstanbul, Turkey
| | - Atıl Yüksel
- Department of Obstetrics and Gynecology, İstanbul University Faculty of Medicine, İstanbul, Turkey
| | - Halime Calı
- Department of Obstetrics and Gynecology, İstanbul University Faculty of Medicine, İstanbul, Turkey
| | - Mehmet Ozsürmeli
- Department of Obstetrics and Gynecology, İstanbul University Faculty of Medicine, İstanbul, Turkey
| | - Rukiye Eker Omeroğlu
- Department of Pediatric Cardiology, İstanbul University Faculty of Medicine, İstanbul, Turkey
| | - Ibrahim Kalelioğlu
- Department of Obstetrics and Gynecology, İstanbul University Faculty of Medicine, İstanbul, Turkey
| | - Recep Has
- Department of Obstetrics and Gynecology, İstanbul University Faculty of Medicine, İstanbul, Turkey
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Ben Brahim F, Hazelzet T, Cohen L, Durand I, Blanc J, Barre E, Daudruy MB, David N. Aberrant drainage of the umbilical vein into the coronary sinus without ductus venosus agenesis. JOURNAL OF ULTRASOUND IN MEDICINE : OFFICIAL JOURNAL OF THE AMERICAN INSTITUTE OF ULTRASOUND IN MEDICINE 2014; 33:535-542. [PMID: 24567466 DOI: 10.7863/ultra.33.3.535] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 06/03/2023]
Abstract
We describe a case series of 4 fetuses with ectopic connections of the ductus venosus to the coronary sinus detected prospectively between August 2011 and February 2012 in 2 congenital cardiologic centers. An enlarged coronary sinus alerted the sonographer. Fetal echocardiography showed ectopic connection of the ductus venosus in an enlarged coronary sinus in all 4 cases. To our knowledge, this anatomic form of ectopic umbilical vein drainage has not previously been reported. The infants were doing well. This venous variant should be considered in cases of isolated coronary sinus dilatation after elimination of a left superior vena cava and a totally anomalous pulmonary vein connection.
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Affiliation(s)
- Foued Ben Brahim
- Department of Obstetrics and Gynecology, Sud Francilien Hospital, 116 rue Jean Jaures, F-91100 Corbeil-Essonnes, France.
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Saha S, Paoletti D, Robertson M. Persistent left superior vena cava - considerations in fetal, pediatric and adult populations. Australas J Ultrasound Med 2012; 15:61-66. [PMID: 28191144 PMCID: PMC5025113 DOI: 10.1002/j.2205-0140.2012.tb00228.x] [Citation(s) in RCA: 11] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/09/2022] Open
Abstract
Persistent left superior vena cava (LSVC) is the commonest congenital anomaly of the thoracic venous system. It is within the group of anomalous systemic venous return (ASVR) and the group is subdivided in cephalic, involving the superior vena cava (SVC) and caudal, involving the inferior vena cava (IVC) types. It is also important to recognise that there can be a persistent LSVC with or without a normal right superior vena cava (RSVC). In most cases, a persistent LSVC drains into the right atrium via the coronary sinus without any clinical symptoms. In this article we discuss embryology, diagnostic and further management approaches and a review of the literature related to persistent LSVC.
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Affiliation(s)
- Sumi Saha
- Department of Obstetrics and Gynaecology Australian National University Medical School Canberra Australian Capital Territory 0200 Australia
| | - Debra Paoletti
- Fetal Medicine Unit Division of Women Youth and Children Canberra Hospital Garran Australian Capital Territory 2605 Australia
| | - Meiri Robertson
- Department of Obstetrics and GynaecologyAustralian National University Medical SchoolCanberraAustralian Capital Territory0200Australia; Fetal Medicine Unit Division of Women Youth and ChildrenCanberra HospitalGarranAustralian Capital Territory2605Australia
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Development of the cardiac venous system in prenatal human life. Open Med (Wars) 2011. [DOI: 10.2478/s11536-010-0073-y] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/20/2022] Open
Abstract
AbstractThe human coronary sinus is an evolutionary modification of the terminal part of the left sinus horn. Anatomically, the coronary sinus is a short, broad vessel that runs along the coronary groove situated on the diaphragmatic surface of the heart. This structure, which opens into the right atrium, collects blood from the great cardiac vein and from other veins of the heart as well. In this study, we assessed the growth and dimensions of the coronary sinus at the fourth and eighth months of fetal development from whole material received from the Nicolaus Copernicus University, Collegium Medicum, Department of Histology and Embryology in Bydgoszcz. A group of 219 specimens, 105 male and 114 female fetuses, presented no visible malformations or developmental abnormalities. The results of this study determined that the dimension of the coronary sinus during prenatal development is not sexually dimorphic. Furthermore, following a monthly period of rapid growth in length of this structure, there are no further increases in length after the six months gestation. Finally, we concluded that the dimensions of the coronary sinus obtained during autopsy are similar to those determined through intravital ultrasound examination. The diameter of the coronary sinus is the best parameter to monitoring the fetal age and the growing of the fetus. Accordingly, we suggest that the best way of estimate for proper blood drainage from heart veins is study of coronary sinus volume.
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Barrea C, Ovaert C, Moniotte S, Biard JM, Steenhaut P, Bernard P. Prenatal diagnosis of abnormal cardinal systemic venous return without other heart defects: a case series. Prenat Diagn 2011; 31:380-8. [PMID: 21254147 DOI: 10.1002/pd.2699] [Citation(s) in RCA: 15] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/07/2010] [Accepted: 12/09/2010] [Indexed: 12/21/2022]
Abstract
OBJECTIVES To describe fetal spectrum and echocardiographic characteristics of anomalous systemic venous return (ASVR, cardinal veins) without other structural heart defects (isolated ASVR), evaluate associated extracardiac and genetic anomalies and review neonatal outcome. METHODS From 2003 to 2009, 369 consecutive fetuses were diagnosed with cardiac malformation, including 27 cases with an isolated ASVR. The following variables were collected: type of ASVR, extracardiac anomalies, karyotype and short-term outcome. RESULTS The most common forms of ASVR were persistent left superior vena cava (SVC) (n = 15) and interrupted inferior vena cava (IVC) (n = 6). The other ASVR types were a persistent left SVC without a right SVC (n = 4), bilateral agenesis of the SVC with cephalic venous drainage toward the IVC through the azygos system (n = 1) and total ASVR to the coronary sinus (n = 1). Genetic disorders were confirmed in five cases, all with a persistent left SVC, with extracardiac anomalies present in four of them. Ten other patients had extracardiac anomalies detected prenatally or postnatally. When available, ASVR diagnosis was confirmed postnatally in all cases. CONCLUSION Isolated ASVRs can be accurately diagnosed prenatally and are frequently associated with extracardiac and genetic anomalies. A detailed morphological ultrasound and fetal karyotype should be suggested. Neonatal outcome was mainly related to the associated anomalies.
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Affiliation(s)
- Catherine Barrea
- Pediatric Cardiology Unit, Department of Pediatrics, Cliniques universitaires Saint Luc, UCL, Brussels, Belgium.
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21
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Jaramillo JF, Estrada Perea MH, Muriel YY. Persistencia de la vena cava superior izquierda. Diagnóstico e importancia prenatal. REVISTA COLOMBIANA DE CARDIOLOGÍA 2010. [DOI: 10.1016/s0120-5633(10)70221-7] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/30/2022] Open
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Freund M, Stoutenbeek P, ter Heide H, Pistorius L. 'Tobacco pipe' sign in the fetus: patent left superior vena cava with absent right superior vena cava. ULTRASOUND IN OBSTETRICS & GYNECOLOGY : THE OFFICIAL JOURNAL OF THE INTERNATIONAL SOCIETY OF ULTRASOUND IN OBSTETRICS AND GYNECOLOGY 2008; 32:593-594. [PMID: 18690613 DOI: 10.1002/uog.5392] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 05/26/2023]
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Jeanty P, Chaoui R, Tihonenko I, Grochal F. A review of findings in fetal cardiac section drawings. Part 3: the 3-vessel-trachea view and variants. JOURNAL OF ULTRASOUND IN MEDICINE : OFFICIAL JOURNAL OF THE AMERICAN INSTITUTE OF ULTRASOUND IN MEDICINE 2008; 27:109-117. [PMID: 18096736 DOI: 10.7863/jum.2008.27.1.109] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 05/25/2023]
Abstract
OBJECTIVE The goal of this presentation is to review some of the common and rare fetal heart abnormalities and to provide an easy approach to these findings with the schematic drawings. METHODS Over the past 10 years, we collected cases in which the common views of the heart were abnormal and the differential diagnoses that existed for each. This presentation shows the normal sonographic sections and then variations of these sections and the associated anomalies. We used illustrative drawings to present these findings, enabling us to point out the main sonographic features of abnormalities of the heart. RESULTS This work reviews 21 fetal heart abnormalities in schematic drawings. CONCLUSIONS This short review highlights several of the anomalies that can be recognized on the common sonographic views. The drawings tend to simplify the findings but should serve as a basis for those doing fetal echocardiography when they encounter an unusual finding.
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Espinoza J, Hassan SS, Gotsch F, Kusanovic JP, Lee W, Erez O, Gonçalves LF, Schoen ML, Romero R. A systematic approach to the use of the multiplanar display in evaluation of abnormal vascular connections to the fetal heart using 4-dimensional ultrasonography. JOURNAL OF ULTRASOUND IN MEDICINE : OFFICIAL JOURNAL OF THE AMERICAN INSTITUTE OF ULTRASOUND IN MEDICINE 2007; 26:1461-7. [PMID: 17957040 PMCID: PMC2678920 DOI: 10.7863/jum.2007.26.11.1461] [Citation(s) in RCA: 10] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Subscribe] [Scholar Register] [Received: 05/01/2007] [Accepted: 06/20/2007] [Indexed: 05/13/2023]
Abstract
OBJECTIVE The multiplanar display is a modality that allows the simultaneous visualization of 3 orthogonal planes from volume data sets obtained with 3- and 4-dimensional ultrasonography. Simultaneous display of standard views used in fetal echocardiography and their orthogonal planes may provide novel ultrasonographic views for examination of the fetal heart and its vascular connections. This study was designed to determine the clinical utility of the multiplanar display in the examination of abnormal vascular connections to the fetal heart. METHODS We reviewed 4-dimensional volume data sets, acquired with the spatiotemporal image correlation technique, from patients with abnormal vascular connections to the fetal heart. Multiplanar views of the fetal heart were used to simultaneously display standard planes used in fetal echocardiography and their corresponding orthogonal planes. RESULTS This study included 4 volume data sets from fetuses with confirmed abnormal vascular connections to the heart, including: (1) an interrupted inferior vena cava with azygos or hemiazygos vein continuation; (2) a persistent left superior vena cava draining into a dilated coronary sinus; and (3) a dilated superior vena cava associated with a thoracic lymphangioma. Simultaneous visualization of orthogonal planes displaying abnormal vascular connections to the fetal heart facilitated identification of the abnormal vessels and their spatial relationships with other vascular structures. CONCLUSIONS Multiplanar imaging can be used to assess abnormal vascular connections to the fetal heart and may provide novel ultrasonographic planes for fetal echocardiography using 3- and 4-dimensional ultrasonography.
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Affiliation(s)
- Jimmy Espinoza
- Perinatology Research Branch, NICHD, NIH, DHHS, Bethesda, Maryland and Detroit, Michigan
- Wayne State University, Department of Obstetrics and Gynecology, Detroit, Michigan
| | - Sonia S Hassan
- Perinatology Research Branch, NICHD, NIH, DHHS, Bethesda, Maryland and Detroit, Michigan
| | - Francesca Gotsch
- Wayne State University, Department of Obstetrics and Gynecology, Detroit, Michigan
| | - Juan Pedro Kusanovic
- Perinatology Research Branch, NICHD, NIH, DHHS, Bethesda, Maryland and Detroit, Michigan
| | - Wesley Lee
- Division of Fetal Imaging, William Beaumont Hospital, Royal Oak, Michigan
| | - Offer Erez
- Perinatology Research Branch, NICHD, NIH, DHHS, Bethesda, Maryland and Detroit, Michigan
| | - Luis F. Gonçalves
- Perinatology Research Branch, NICHD, NIH, DHHS, Bethesda, Maryland and Detroit, Michigan
- Wayne State University, Department of Obstetrics and Gynecology, Detroit, Michigan
| | - Mary Lou Schoen
- Wayne State University, Department of Obstetrics and Gynecology, Detroit, Michigan
| | - Roberto Romero
- Perinatology Research Branch, NICHD, NIH, DHHS, Bethesda, Maryland and Detroit, Michigan
- Wayne State University, Center for Molecular Medicine and Genetics, Detroit, Michigan
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Volpe P, Campobasso G, De Robertis V, Di Paolo S, Caruso G, Stanziano A, Volpe N, Gentile M. Two- and four-dimensional echocardiography with B-flow imaging and spatiotemporal image correlation in prenatal diagnosis of isolated total anomalous pulmonary venous connection. ULTRASOUND IN OBSTETRICS & GYNECOLOGY : THE OFFICIAL JOURNAL OF THE INTERNATIONAL SOCIETY OF ULTRASOUND IN OBSTETRICS AND GYNECOLOGY 2007; 30:830-7. [PMID: 17914751 DOI: 10.1002/uog.5145] [Citation(s) in RCA: 34] [Impact Index Per Article: 1.9] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 05/17/2023]
Abstract
OBJECTIVES To explore whether the use of four dimensional (4D) ultrasound examination with B-flow imaging and spatiotemporal image correlation (STIC) can supply additional information with respect to two-dimensional (2D) gray-scale and color Doppler echocardiography in the prenatal characterization of isolated total anomalous pulmonary venous connection (TAPVC). METHODS The study population comprised a group of three TAPVC fetuses that had been examined exclusively by conventional echocardiography, and a group of four additional cases initially identified by conventional echocardiography and examined further by 4D ultrasonography; a thorough postnatal work-up was available for all fetuses. RESULTS At our center, isolated TAPVC was found in seven of 1040 fetuses with cardiac defects (0.67%). Anomalous drainage was supracardiac to the innominate vein in three cases, cardiac to the coronary sinus in two, and infracardiac to the portal vein in the remaining two cases. An evident asymmetry between left heart and right heart structures was observed in 4/7 cases. The confluence of the anomalous pulmonary veins (PVs) was visualized in 4/7 cases and the connecting vertical vein was identified in 3/5 cases at 2D echocardiography. 4D ultrasound imaging with B-flow imaging and STIC clearly visualized the anomalous PV confluence and the draining vertical vein in all four cases examined. CONCLUSION 2D and color Doppler echocardiography appears to diagnose reliably TAPVC, albeit with some limitations in thorough assessment of the pathology, depending on the anatomy of the defect, on the technical adequacy of the equipment used and on the experience of the operator. 4D ultrasound examination with B-flow imaging and STIC is apparently able to facilitate identification of the anatomical features of TAPVC, thus supplying additional information over that provided by 2D fetal sonography.
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Affiliation(s)
- P Volpe
- Department of Obstetrics and Gynecology, Hospital Di Venere, Bari, Italy.
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Galindo A, Gutiérrez-Larraya F, Escribano D, Arbues J, Velasco JM. Clinical significance of persistent left superior vena cava diagnosed in fetal life. ULTRASOUND IN OBSTETRICS & GYNECOLOGY : THE OFFICIAL JOURNAL OF THE INTERNATIONAL SOCIETY OF ULTRASOUND IN OBSTETRICS AND GYNECOLOGY 2007; 30:152-61. [PMID: 17616965 DOI: 10.1002/uog.4045] [Citation(s) in RCA: 52] [Impact Index Per Article: 2.9] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 05/16/2023]
Abstract
OBJECTIVES To determine the prevalence and clinical significance of persistent left superior vena cava (PLSVC) in fetuses with and without cardiac and extracardiac anomalies. METHODS Charts and recorded images were reviewed from high-risk patients who underwent fetal echocardiography between January 2000 and December 2005. This retrospective study included 54 fetuses with confirmed PLSVC who were diagnosed based on the presence of an additional vessel identified to the left of the pulmonary artery in the three-vessel view of the heart. Associated congenital heart defects (CHDs), extracardiac abnormalities, including first trimester nuchal translucency (NT) thickness, and fetal/postnatal outcome were analyzed. RESULTS Of 5,737 referrals, 5,233 had a normal heart, and PLSVC was observed in 10 of these fetuses (0.2%; Group 1). CHDs were present in 504 and PLSVC was observed in 44 of these cases (9%). In the latter group, 18/44 (41%) fetuses had heterotaxy syndrome (Group 2) where the most common structural heart defects were atrioventricular septal defect and double-outlet right ventricle. Fetuses without heterotaxy syndrome (Group 3) accounted for 26/44 (59%) cases of CHDs associated with PLSVC. In this group of fetuses the most common CHDs were left outflow tract obstructive defects and conotruncal anomalies. Increased NT was observed in 29%, without differences among the three groups. The survival rates among fetuses in Groups 1, 2 and 3 were 100%, 44% and 50%, respectively. After excluding patients who underwent pregnancy termination, there were no significant differences in the survival rates among the groups, probably due to the small size of the samples. CONCLUSIONS PLSVC is associated with CHDs. The identification of PLSVC should prompt a thorough examination of the fetus to identify additional cardiac and extracardiac anomalies. The prognosis of affected fetuses largely depends on whether or not the PLSVC is associated with a CHD.
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Affiliation(s)
- A Galindo
- Ultrasound and Fetal Physiopathology Unit, Department of Obstetrics and Gynaecology, Hospital Universitario '12 de Octubre', Madrid, Spain.
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Berg C, Knüppel M, Geipel A, Kohl T, Krapp M, Knöpfle G, Germer U, Hansmann M, Gembruch U. Prenatal diagnosis of persistent left superior vena cava and its associated congenital anomalies. ULTRASOUND IN OBSTETRICS & GYNECOLOGY : THE OFFICIAL JOURNAL OF THE INTERNATIONAL SOCIETY OF ULTRASOUND IN OBSTETRICS AND GYNECOLOGY 2006; 27:274-80. [PMID: 16456841 DOI: 10.1002/uog.2704] [Citation(s) in RCA: 70] [Impact Index Per Article: 3.7] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 05/06/2023]
Abstract
OBJECTIVE To evaluate the associated conditions and the outcome of persistent left superior vena cava (PLSVC) detected in fetal life. METHODS This was a retrospective review of all cases of PLSVC detected prenatally between 1998 and 2004 in two tertiary referral centers in Germany. Patient charts, ultrasound video recordings and still frames of all cases were reviewed for associated conditions and outcome. RESULTS Eighty-two cases of PLSVC were detected in the study period. Thirty-seven cases (45%) were associated with heterotaxy syndromes, 19 (23%) with isolated cardiac malformations, seven (9%) with aneuploidy, six (7%) with complex malformation syndromes and six (7%) with isolated extracardiac malformations. Seven cases (9%) had no associated condition. Eighty-three percent of the fetuses in this series had associated cardiac malformations; the most frequent cardiac malformations in those with heterotaxy syndromes were complete atrioventricular septal defect (75%) and right outflow tract obstruction (58%). After exclusion of cases with heterotaxy, most congenital heart defects were ventricular septal defects (41%) and coarctation (34%). The outcome of PLSVC was determined solely by the associated conditions. After exclusion of terminated cases, heterotaxy syndromes as well as complete atrioventricular septal defects were associated significantly with perinatal and infant death. In contrast, all cases with isolated PLSVC or associated correctable extracardiac malformations survived and were doing well at the time of writing. CONCLUSIONS PLSVC detected in fetal life has to be followed by a meticulous inspection of the fetal anatomy as it is frequently associated with heterotaxy syndromes, other cardiac/non-cardiac malformations and aneuploidy that determine the outcome. Isolated PLSVC is a benign vascular anomaly and may not affect the outcome.
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Affiliation(s)
- C Berg
- Department of Prenatal Medicine and Obstetrics, University of Bonn, Bonn, Germany.
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Abstract
The present knowledge of coronary arteries in prenatal diagnosis is reviewed with a focus on three aspects: the physiology and visualization of coronary flow, malformations involving the coronary arteries, and the "heart-sparing effect". Visualization of coronary arteries in a healthy human fetus is possible in real-time and colour Doppler during the last 10 wk of gestation when ultrasound conditions are excellent. Visualization at an earlier gestational age (up to 13 wk) is feasible mainly in association with malformations and impending hypoxia. The main coronary malformations that can be visualized in utero are the ventriculo-coronary communications in fetuses with pulmonary atresia. In the last few years, interest has been focused on the "heart-sparing effect", defined as the increased perfusion of the coronary arteries in fetuses with severe growth restriction and abnormal Doppler velocimetry in the peripheral vessels. Increased perfusion detectable with colour and pulsed Doppler is a late sign of fetal compromise in hypoxaemia. It confirms animal experiments that have demonstrated dilatory reserves of the fetal coronary arteries under chronic hypoxaemia. The outcome of 21 fetuses showing the "heart-sparing effect" before 32 wk gestation was poor: nine fetuses died in utero and two after birth, the median weight at birth was 630 g. In summary, our knowledge of the coronary arteries in the fetus is based on the diagnostic means used in prenatal diagnosis. New information in this field may also contribute to a better understanding of coronary heart disease later in life.
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Affiliation(s)
- R Chaoui
- Department of Obstetrics and Gynaecology, Charité Medical School, Humboldt University, Berlin, Germany.
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Volpe P, Buonadonna AL, Campobasso G, Di Carlo A, Stanziano A, Gentile M. Cat-eye syndrome in a fetus with increased nuchal translucency: three-dimensional ultrasound and echocardiographic evaluation of the fetal phenotype. ULTRASOUND IN OBSTETRICS & GYNECOLOGY : THE OFFICIAL JOURNAL OF THE INTERNATIONAL SOCIETY OF ULTRASOUND IN OBSTETRICS AND GYNECOLOGY 2004; 24:485-487. [PMID: 15343614 DOI: 10.1002/uog.1723] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 05/24/2023]
Affiliation(s)
- P Volpe
- Department of Obstetrics and Gynecology, Hospital Di Venere, University of Bari, Bari, Italy
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Current awareness in prenatal diagnosis. Prenat Diagn 2003; 23:772-8. [PMID: 14533646 DOI: 10.1002/pd.533] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/08/2022]
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