A 45-year-old woman presents with discomfort in her left leg with walking, dizziness, headaches, and a cold right hand. She has no chest pain or shortness of breath. There is no significant past medical history and she does not smoke.
Physical Examination:
What is the most likely diagnosis?
Takayasu arteritis. Takayasu arteritis (“pulseless disease”) is an inflammatory disease of large sized arteries. Both stenotic lesions and aneurysms may be present with involvement of proximal segments of the aortic arch vessels. Aortic regurgitation is not uncommon due to involvement of the aortic root and aortic valve with inflammatory disease. Although most commonly described in Japanese patients, it has been described throughout the world.
A diagnostic classification proposed by the American College of Rheumatology requires three of six criteria:
A 21-year-old woman with Turner syndrome has a history of surgical repair for periductal coarctation of the aorta at age 4 years. Her presentation was for HTN and heart failure, both of which resolved after the procedure. She was lost to follow-up after childhood and recently reestablished with a cardiologist. She has been experiencing dyspnea and claudication in the past year.
Which of the following statements is correct regarding the echocardiographic image from this patient obtained from the suprasternal notch (figure below).
Presence of systolic and diastolic flow is consistent with severe coarctation. The peak Doppler pressure gradient as obtained from the simplified Bernoulli often overestimates the gradient obtained across the coarctation as measured by invasive hemodynamics. Since the precoarctation velocity is often increased due to abnormality of aortic valve, it must be accounted for in the Bernoulli equation. This requires using the modified Bernoulli equation, where pressure = 4 (V2 2 – V1 2 ), in which V2 is the peak Doppler velocity across the coarctation and V1 is the precoarctation velocity. The typical sawtooth pattern of aortic coarctation is shown in this example with an elevated systolic velocity and a lower velocity forward flow in diastole. The presence of a continuous flow across the coarctation is consistent with severe obstruction regardless of the peak pressure gradient. Transthoracic suprasternal notch (SSN) view with continuous Doppler in the upper descending aorta. The Doppler profile shows a high-velocity antegrade velocity of 316 cm/s consistent with a 40- mmHg systolic gradient. There is a lower velocity antegrade flow during diastole. These findings are consistent with coarctation of the aorta (see figure in question).
Which of the following statements is correct regarding the echocardiographic image from this patient obtained from the abdominal aorta (figure below).
The hallmark of coarctation is the presence of persistent antegrade flow in diastole. The image is a pulsed Doppler spectral recording from the abdominal aorta consistent with coarctation of the aorta. The findings consistent with coarctation are a blunted or low-velocity systolic flow and persistent antegrade flow in diastole (absent retrograde flow). The presence of early diastolic reversal excludes significant coarctation. Other findings consistent with coarctation include a low systolic-to-diastolic velocity ratio, decreased pulsatility, and a prolonged pulse delay. Transthoracic subcostal view with pulsed Doppler in the abdominal aorta. A blunted or low-velocity systolic flow and persistent antegrade flow in diastole (absent retrograde flow) are specific features consistent with coarctation of the aorta (see figure in question).
Which of the following recommendations is most appropriate regarding reintervention in this patient (figure below).
Balloon angioplasty with or without stents is generally recommended. The patient has severe recoarctation of the aorta after surgical repair. The choice of intervention for coarctation remains controversial and should be made in a center experienced with congenital heart disease among a team of surgical and interventional specialists. However, for recurrent coarctation after surgical repair, catheter intervention with or without stents is generally preferred if the anatomy is suitable. Favorable anatomy is a discrete, nontortuous short segment of narrowing without aneurysms, pseudoaneurysm, or hypoplasia of the aorta. Additional consideration should be given to women considering pregnancy where there is concern regarding the tissue fragility and risk of aortic complications with residual coarctation tissue. In that setting, surgery may be considered. Cardiac MR angiography. Sagittal oblique maximum intensity projection showing a discrete, short region of coarctation in the upper descending aorta without associated dilatation of the adjacent segments (see figure in question).
Which of the following is an indication for aortic coarctation intervention?
Asymptomatic patient with a peak-to-peak gradient across the coarctation site of 15 mmHg with extensive collaterals. According to the American College of Cardiology (ACC)/American Heart Association (AHA) 2008 guidelines for adults with congenital heart disease intervention for coarctation of the aorta is recommended if (a) peak-to-peak gradient across the coarctation is ≤20 mmHg or (b) peak-to-peak gradient across the coarctation is <20 mmHg but there is anatomic evidence of severe obstruction and radiologic evidence of collateral flow. For the patient undergoing aortic valve replacement for bicuspid severe aortic regurgitation, the coarctation of the aorta should be reassessed after surgery to determine whether it is clinically significant.
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