A 30-year-old man with a history of congenital heart disease is referred to you because of symptoms of dysphagia and an abnormal CXR. As a child he was told that his CXR was abnormal because of an “aortic anomaly” and that it was benign.
Physical Examination:
The patient’s dysphagia is likely caused by what abnormality associated with his aortic anomaly (Right-sided aortic arch)?
Aberrant left subclavian artery and diverticulum of Kommerell. Aortic arch anomalies may result in dysphagia, stridor, or wheezing associated with vascular rings that compress the esophagus or trachea. With the non-mirror image right aortic arch, the left subclavian artery arises from the right side and crosses posterior to the esophagus and trachea. This causes a vascular ring that may cause compressive symptoms. Aberrant vessels such as the left subclavian artery may arise from an aneurysmal segment called a diverticulum of Kommerell. An aberrant right subclavian artery may be an isolated anomaly in a normal left-sided aortic arch and is the most common aortic arch abnormality. Although generally asymptomatic, it may also be associated with a vascular ring.
A 30-year-old man presents for follow-up after undergoing a surgical coarctation repair at age 9. He has no records and is uncertain of the type of repair. He has not had any testing done in the past 10 years. He has used antibiotic prophylaxis for dental procedures. He takes no other medications. He feels well, is active, and has no complaints.
Which test would be least appropriate for this patient?
24-Hour Holter monitoring. CTA or MRA of the aorta is required to assess postoperative complications of prior coarctation repair. Complications include recoarctation and aneurysms in the ascending aorta, aortic arch, or descending aorta in the region of the repair. In adults, a TTE does not adequately visualize the descending thoracic aorta, although gradients can be obtained to assess hemodynamic significance. Exercise-induced HTN may occur late after coarctation repair even among patients who are normotensive at rest and should be assessed for by exercise stress testing. TTE is important to assess the aortic valve and the possibility of a bicuspid valve, as well as associated structural abnormalities. Arrhythmias are an infrequent consequence of a corrected coarctation in an asymptomatic patient and therefore Holter monitoring is not necessary.
How often should the coarctation repair site be evaluated by chest imaging in this patient (CTA or MRA)?
Generally every 5 years. The ACC/AHA 2008 guidelines for adults with congenital heart disease recommend at least a one-time CT or MR angiogram for repaired or unrepaired coarctation for complete evaluation of the thoracic aorta. In addition, follow-up should be performed at intervals of 5 years or less depending on the prior findings.
A 38-year-old man is referred for a cardiac surgical evaluation for bicuspid aortic regurgitation. He has a long-standing aortic valve disorder for 10 years. He is fully active with no limitations. He plays tennis on a regular basis.
What is the most appropriate surgical recommendation?
Observation only. Based on examination and echocardiography, the patient has mixed but predominantly severe congenital bicuspid aortic regurgitation. Holodiastolic flow reversal in the descending aorta is the key confirmatory finding supporting severe aortic regurgitation. The three murmurs described include the systolic murmur of bicuspid aortic stenosis and the diastolic murmurs of aortic regurgitation with the associated Austin Flint murmur. There are no clinical or echocardiographic indications for surgery based on aortic valve or aortic disease. β-blockers for aortic root dimensions >4.0 cm should generally be used only if severe aortic regurgitation is not present because of concern of increasing regurgitant volume and fraction with slower heart rates.
Six months later the patient has developed dyspnea with moderate exertion and a significant reduction in exercise tolerance. Repeat echocardiogram shows similar LV cavity dimensions and LVEF calculated at 57%. Aortic root dimension is 4.0 cm and the mid-ascending aorta is 4.6 cm.
What is the appropriate recommendation at this time?
Aortic valve replacement and aortic graft. Surgery is indicated in this patient both for symptomatic aortic regurgitation and because of significant progression in aortic size. ACC/AHA valvular heart disease guidelines for bicuspid aortic valves recommend replacement of the aortic root or ascending aorta if the diameter is greater than 4.5 cm at the time of aortic valve replacement. Aortic valve repair can be performed successfully in select patients with bicuspid aortic regurgitation but is usually not performed when stenosis is present or if there are unfavorable anatomic findings such as calcification. Therefore, aortic valve replacement with either a mechanical or bioprosthetic valve is indicated along with an aortic graft. The aortic graft may incorporate the aortic root or may be in the supracoronary position.
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