Coarctation of the Aorta

In a neonate or young infant, deterioration after ductal closure with heart failure or shock should trigger urgent evaluation for critical, ductal-dependent coarctation. Critical coarctation most often presents when the ductus closes; primary management in infants is surgical repair after stabilization and anatomic definition. ACCACCManagement of Recurrent Coarctation in Children - American College of Cardiology

In adolescents and adults, suspect native coarctation or recoarctation in upper-extremity hypertension, a murmur, diminished or delayed femoral pulses, arm-leg pressure disparity, lower-extremity hypoperfusion symptoms, or unexplained left-ventricular hypertrophy. Untreated disease is associated with systemic hypertension, heart failure, aortic aneurysm or dissection, and premature mortality. ScienceDirect+1ScienceDirectAortic Coarctation - an overview | ScienceDirect TopicsPubMedSurgical repair of aortic coarctation in adults: half a century of a single centre clinical experience - PMC

Do not use a low resting Doppler or cuff gradient to exclude clinically important obstruction when extensive collaterals are present. Collateral pathways can decompress the pressure gradient despite significant anatomic narrowing; define anatomy and collateral burden with cross-sectional imaging and use invasive hemodynamics when intervention is being considered. PubMed+2PubMedCurrent management of coarctation of the aortaPubMedCoarctation of the aorta in adults: what is the best treatment? Case report and literature reviewPubMedCatheter Management of Coarctation - StatPearls - NCBI Bookshelf

At initial assessment, obtain upper- and lower-extremity cuff pressures, complete pulse examination, transthoracic echocardiography, and ECG-based assessment for hypertensive cardiac effects. Echocardiography confirms obstructive physiology and assesses left-ventricular hypertrophy or dysfunction, aortic regurgitation, and associated bicuspid aortic valve disease. ScienceDirect+1ScienceDirectAortic Coarctation - an overview | ScienceDirect TopicsjaccManagement of Women With Congenital or Inherited Cardiovascular Disease From Pre-Conception Through Pregnancy and Postpartum: JACC Focus Seminar 2/5

Obtain cardiovascular MR angiography when feasible, or CT angiography when MR is unsuitable, to map the site and length of narrowing, transverse arch hypoplasia, collateral vessels, repair-site aneurysm or pseudoaneurysm, and thoracic aortic dimensions. MR provides superior visualization of the adult arch and collateral circulation compared with echocardiography and can estimate flow and peak gradient with phase-contrast analysis. ScienceDirectScienceDirectMRI in adult patients with aortic coarctation: diagnosis and follow-up - ScienceDirect

When noninvasive findings suggest clinically significant obstruction or are discordant, catheterization provides the peak-to-peak pressure gradient used for intervention decisions. A catheter peak-to-peak gradient is generally lower than the peak systolic gradient reported by Doppler echocardiography, so do not equate the two measurements. PubMedPubMedCoarctation of the aorta in adults: what is the best treatment? Case report and literature review

For native or recurrent coarctation meeting hemodynamic or anatomic criteria, choose repair after multidisciplinary review of patient size, lesion length, arch hypoplasia, prior repair type, associated cardiac disease, and aneurysm anatomy. ACC/AHA guidance supports either surgical repair or percutaneous catheter intervention for recurrent discrete coarctation. PubMed+1PubMedSurgical repair of aortic coarctation in adults: half a century of a single centre clinical experience - PMCPubMedCatheter Management of Coarctation - StatPearls - NCBI Bookshelf

Surgery remains the primary treatment for infant coarctation and is particularly relevant when arch hypoplasia or complex anatomy requires reconstruction. In children with recurrent discrete coarctation, balloon angioplasty is a reasonable initial option; recurrent obstruction with arch hypoplasia more often requires surgical relief of the hypoplastic segments. ACCACCManagement of Recurrent Coarctation in Children - American College of Cardiology

In older children, adolescents, and adults with anatomy suitable for a transcatheter approach, stent implantation is commonly used because a stent can be dilated toward adult size. In a multicenter cohort of children weighing more than 10 kg, surgery and stenting produced lower short-term upper-to-lower extremity gradients than balloon angioplasty; stenting had fewer reported complications than surgery or balloon angioplasty, whereas balloon angioplasty carried more aortic wall injury. jacc+1jaccComparison of Surgical, Stent, and Balloon Angioplasty Treatment of Native Coarctation of the Aorta: An Observational Study by the CCISC (Congenital Cardiovascular Interventional Study Consortium)ACCManagement of Recurrent Coarctation in Children - American College of Cardiology

Arrange lifelong follow-up with a cardiologist experienced in adult congenital heart disease for all adults with native or repaired coarctation. Surveillance must include recurrent obstruction, systemic hypertension, aortic aneurysm formation, associated valve disease, and cardiovascular risk factors. PubMed+2PubMedCatheter Management of Coarctation - StatPearls - NCBI BookshelfACCClinical Practice Algorithm For the Follow-Up of Repaired Coarctation of the Aorta - American College of CardiologyPubMedCurrent management of coarctation of the aorta - PMC

At follow-up, repeat arm-leg pressure assessment and echocardiography, with cross-sectional imaging used as an adjunct—especially in older children and adults whose arch imaging by echocardiography is limited. Younger patients and those with greater-than-mild residual lesions need closer surveillance; exercise testing can help detect an abnormal blood-pressure response. ACCACCClinical Practice Algorithm For the Follow-Up of Repaired Coarctation of the Aorta - American College of Cardiology

Treat hypertension as an ongoing disease target rather than assuming it will resolve after repair. Blood pressure often falls after successful relief of obstruction, but persistent or recurrent hypertension and disproportionate exercise systolic hypertension remain common and contribute to premature coronary and cerebrovascular disease. PubMed+1PubMedCurrent management of coarctation of the aortaPubMedCurrent management of coarctation of the aorta - PMC

Women with repaired coarctation are classified as modified WHO II–III, while unrepaired severe coarctation is modified WHO IV. Before conception, assess residual obstruction, hypertension, bicuspid aortic valve, thoracic aortic anatomy and dimensions, and intracranial berry aneurysms with baseline MRA; repair residual or recurrent coarctation before pregnancy when feasible. BMJ+1BMJPregnancy outcomes in women with aortic coarctation - HeartjaccManagement of Women With Congenital or Inherited Cardiovascular Disease From Pre-Conception Through Pregnancy and Postpartum: JACC Focus Seminar 2/5

Pregnancy risk rises with residual obstruction defined by a gradient greater than 20 mm Hg or minimal aortic lumen less than 12 mm, hypertension, or aortic aneurysm. If aortic diameter exceeds 4.0 cm, consider serial noncontrast MRA during pregnancy. Individualize blood-pressure goals because excessive lowering may worsen fetal perfusion in the setting of residual coarctation. jaccjaccManagement of Women With Congenital or Inherited Cardiovascular Disease From Pre-Conception Through Pregnancy and Postpartum: JACC Focus Seminar 2/5

Vaginal delivery is generally preferred and epidural analgesia is recommended in the cited congenital heart disease pregnancy framework. Consider cesarean delivery for ascending aortic aneurysm greater than 5.0 cm or acute aortic syndrome. Anticipate postpartum hypertension, with blood pressure expected to peak 3 to 8 days after delivery. jaccjaccManagement of Women With Congenital or Inherited Cardiovascular Disease From Pre-Conception Through Pregnancy and Postpartum: JACC Focus Seminar 2/5