Coarctation of the aorta: Management from infancy to adulthood

Table 1 Notable studies and guideline statements in the treatment and outcome of coarctation in adults and children

Ref.	n	Follow-up	Outcome
Cowley et al[60]	36	Mean 14 yr	Randomized trial comparing BA and surgery for native coarctation in children. Aortic aneurysm developed in 35% of BA patients and none of the surgical patients
Carr[81]	846	Mean 36 mo for catheter-based group and 7.8 yr for surgical group	Meta-analysis comparing catheter vs surgical intervention for adults with coarctation. Higher risk of restenosis and need for reintervention found in catheter-based group
Forbes et al[68]	578	Median 12 mo	Retrospective multicenter analysis at intermediate follow-up after stent placement for coarctation. Exceeding a balloon:coarct ratio of 3.5 and prestent BA increased risk of aortic wall injury
Warnes et al[24]	-	-	ACC/AHA guidelines for management of coarctation in adults
Holzer et al[67]	302	3-60 mo	Prospective analysis of acute, intermediate, and long-term follow-up after stent placement for coarctation using CCISC registry. At long-term follow-up, recoarctation in 20% of patients, 4% required unplanned reintervention, and 1% had aortic wall injury
Feltes et al[27]	-	-	AHA guidelines for transcatheter intervention in children with coarctation
Forbes et al[69]	350	Mean 1.7 yr	Multicenter observational study comparing surgery, BA, and stent placement for native coarctation in children using CCISC registry. Significantly lower acute complication rates in stent group but higher planned reintervention rates. Hemodynamic and arch imaging outcomes superior in stent and surgical patients compared to BA group
Harris et al[55]	130	3-60 mo	Prospective, multicenter analysis of short and intermediate outcomes for BA in native and recurrent coarctation in children. Trend toward increased acute aortic wall injury and restenosis in native coarctation patients
Sohrabi et al[75]	120	Mean 31.1 mo	Randomized clinical trial comparing covered and bare CP stents for native coarctation in adolescents and adults. Trend of increased rates of restenosis and lower rates of pseudoaneurysm in bare stent group
Meadows et al[70]	105	2 yr	Prospective, multicenter, single-arm study assessing safety and efficacy of CP stent in children and adults with coarctation. Two year follow-up of 86% showed 23 fractured stents with no significant clinical effects, 6 aortic aneurysms, 19 repeat catheter interventions, and no surgical interventions

BA: Balloon angioplasty; ACC: American College of Cardiology; AHA: American Heart Association; CCISC: Congenital Cardiovascular Interventional Study Consortium; CP: Cheatham platinum.

Table 2 Executive summary on the diagnosis and treatment of coarctation in children and adults

Diagnosis

Accounts for 5%-7% of congenital heart disease diagnoses

Neonates often present with heart failure, acidosis, and shock with critical coarctation

Less severe coarctation often detected during evaluation for hypertension or murmur in the older child or adult

Diminished or delayed lower extremity pulses and a systolic pressure gradient between the upper and lower extremities are the most useful exam findings

Transthoracic echocardiogram is initial test of choice; CT and MRI useful if echocardiogram inconclusive and for surgical planning

Treatment

Surgical repair

Extended end-to-end anastomosis typically preferred surgical method, as it avoids prosthetic material, allows resection of the coarctation, and has a wider incision that is less prone to restenosis

Surgical repair typically preferred over transcatheter approaches in the infant and young child with native coarctation, patients requiring repair of associated cardiac defects, or in those with complex coarctation anatomy

Balloon angioplasty

Often the preferred intervention for recurrent coarctation

Concern for recoarctation and aneurysm formation in native coarctation

Endovascular stent

Provides structural support and decreased rates of aortic wall injury and aneurysm compared to balloon angioplasty

Covered stents may protect against shear stress and subsequent restenosis, though care must be taken to avoid overlying vital branch vessels

Use of stents in small children controversial due to need for large sheath size and limitations in accommodating for somatic growth

Patient follow-up

Lifelong follow-up with at least annual cardiology visits and repeat imaging every 5 yr to assess coarctation site

High suspicion and aggressive treatment of baseline and exercise- induced hypertension

Future perspectives

Further long-term data analysis needed to determine optimal intervention based on patient anatomy, size, and age

CT: Computed tomography; MRI: Magnetic resonance imaging.