Sections View Full Chapter Figures Tables Videos Annotate Full Chapter Figures Tables Videos Supplementary Content + Download Section PDF Listen ++ For further information, see CMDT Part 10-05: Coarctation of the Aorta + Key Features Download Section PDF Listen +++ +++ Essentials of Diagnosis ++ Usual presentation is systemic hypertension Echocardiography/Doppler is diagnostic; a peak gradient of > 20 mm Hg may be significant due to collaterals around the coarctation reducing gradient despite severe obstruction Associated bicuspid aortic valve in 50–80% of patients Delayed pulse in femoral artery compared to brachial artery Systolic pressure is higher in upper extremities than in lower extremities; diastolic pressures are similar +++ General Considerations ++ Coarctation of the aorta consists of localized narrowing of the aortic arch just distal to the origin of the left subclavian artery If the stenosis is severe, collateral circulation develops around the coarctation site through the intercostal arteries and the branches of the subclavian arteries; a lower transcoarctation gradient can result by enabling blood flow to bypass the obstruction Coarctation is a cause of secondary hypertension and should be considered in young patients with elevated blood pressure The renin-angiotensin system is often abnormal and contributes to the residual hypertension occasionally seen even after coarctation repair A bicuspid valve is seen in approximately 50–80% of the cases, and there is an increased incidence of cerebral berry aneurysms Significant native or recurrent aortic coarctation has been defined as follows Upper extremity/lower extremity resting peak-to-peak gradient > 20 mm Hg or mean Doppler systolic gradient > 20 mm Hg Upper extremity/lower extremity gradient > 10 mm Hg or mean Doppler gradient > 10 mm Hg when there is either decreased LV systolic function or aortic regurgitation Upper extremity/lower extremity gradient > 10 mm Hg or mean Doppler gradient > 10 mm Hg when there is evidence for collateral flow around the coarctation + Clinical Findings Download Section PDF Listen +++ ++ Usually no symptoms until hypertension produces left ventricular (LV) failure Cerebral hemorrhage occurs rarely Strong arterial pulsations in the neck and suprasternal notch Hypertension in the arms, but blood pressure is normal or low in the legs Delayed or weak femoral pulsations A continuous murmur heard superiorly and midline in the back or over the left anterior chest may be present when large collaterals occur and is a clue that the coarctation is severe Aortic regurgitation or stenosis murmur due to an associated bicuspid aortic valve may be present Coarctation is associated with Turner syndrome (a sex chromosomal abnormality [XO]); a webbed neck may be present in these patients + Diagnosis Download Section PDF Listen +++ +++ Imaging ++ Radiography May show scalloping of the inferior portion of the ribs (rib notching) due to enlarged collateral intercostal arteries Dilation of the left subclavian artery and poststenotic aortic dilation along with LV enlargement may be present The coarctation region and the poststenotic dilation of the descending aorta may result in a "3" sign along the aortic shadow on the PA chest radiograph (the notch in the "3" representing the area of coarctation) Echocardiography/Doppler Usually diagnostic May provide additional evidence for a bicuspid aortic valve. Both MRI and CT can provide excellent images of the coarctation anatomy, and one or the other should always be done to define the coarctation anatomic structure MRI and echocardiography/Doppler can also provide estimates of the gradient across the lesion +++ Diagnostic Procedures ++ The ECG usually shows LV hypertrophy (LVH) Cardiac catheterization provides definitive gradient information and is necessary if percutaneous stenting is to be considered + Treatment Download Section PDF Listen +++ ++ Patients with a demonstrated peak gradient of > 20 mm Hg should be considered for intervention, especially if there is evidence of collateral blood vessels The percutaneous interventional procedure of choice is endovascular stenting; when anatomically feasible, self-expanding and balloon-expandable covered stents have been shown to be advantageous over bare metal stents Most coarctation repair in adults is percutaneous Otherwise, surgical resection (usually with end-to-end anastomosis) should be performed + Outcome Download Section PDF Listen +++ +++ Follow-Up ++ Recurrence of the coarctation stenosis following intervention requires long-term follow-up +++ Prognosis ++ Many untreated patients with severe coarctation die of hypertension, rupture of the aorta, infective endarteritis, or cerebral hemorrhage before the age of 50 Aortic dissection also occurs with increased frequency Resection of the coarctation site has a surgical mortality rate of 1–4% and includes risk of spinal cord injury Coarctation of any significance may be poorly tolerated in pregnancy because of the inability to support the placental flow About 25–50% of surgically corrected patients continue to be hypertensive years after surgery because of permanent changes in the renin-angiotensin system, endothelial dysfunction, aortic stiffness, altered arch morphology, and increased ventricular stiffness +++ When to Refer ++ All patients with aortic coarctation and any detectable gradient should be referred to a cardiologist with expertise in adult congenital heart disease + References Download Section PDF Listen +++ + +Brown ML et al. Coarctation of the aorta: lifelong surveillance is mandatory following surgical repair. J Am Coll Cardiol. 2013 Sep 10;62(11):1020–5. [PubMed: 23850909] + +Fedchenko M et al. Cardiovascular risk factors in adults with coarctation of the aorta. Congenit Heart Dis. 2019 Jul;14(4):549–58. [PubMed: 31099471] + +Haji Zeinali AM et al. Midterm to long-term safety and efficacy of self-expandable nitinol stent implantation for coarctation of aorta in adults. Catheter Cardiovasc Interv. 2017 Sep 1;90(3):425–31. [PubMed: 28707350] + +Lee MGY et al. Long-term mortality and cardiovascular burden for adult survivors of coarctation of the aorta. Heart. 2019 Aug;105(15):1190–6. [PubMed: 30923175] + +Morgan GJ et al. Optimus covered stent: advanced covered stent technology for complex congenital heart disease. Congenit Heart Dis. 2018 May;13(3):458–62. [PubMed: 29468813] + +Schneider H. Modern management of coarctation of the aorta: transcatheter and surgical options. J Cardiovasc Surg (Torino). 2016 Aug;57(4):557–68. [PubMed: 27243624]