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Coarctation of Aorta in a 52-Year-Old Female

Significant ring-like stenosis in the thoracic descending aorta.Significant ring-like stenosis in the thoracic descending aorta.Significant gradient across the stenosis in the thoracic descending aorta.Moderately sclerotic bicuspid aortic valve.Moderately sclerotic bicuspid aortic valve.Coarctation with significant color turbulance.Coarctation with significant color turbulance.Significant gradient by pulse Doppler.
VOLUME: 20 PUBLICATION DATE: Jan 24 2008
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Mouhammed Joumaa, MD, David Graham, MD, Howard Rosman, MD

Coarctation of the aorta accounts for 5–10% of all congenital cardiovascular malformations and carries a high morbidity and mortality risk. Symptoms depend on the severity of the disease and other coexisting anomalies.

Case Presentation. A 52-year-old female presented with vague chest discomfort, increasing fatigue and exertional dyspnea. Her past medical history was significant for tobacco abuse and mild hypertension. Her physical examination was normal except for an apical gallop sound (S4). Her echocardiogram showed segmental wall motion abnormalities.
Due to the significance of the cardiac dysfunction and her clinical presentation, the patient underwent a cardiac catheterization to evaluate her coronary artery disease. The left ventricular ejection fraction was significantly reduced. There was no evidence of mitral valve prolapse or mitral insufficiency. Aortography showed a mildly dilated aortic root, no aortic valve insufficiency and a significant ring-like stenosis in the thoracic descending aorta (Figures 1 and 2). The gradient through this stenosis measured 54 mmHg (Figure 3). The coronary angiography was negative for significant focal coronary artery obstruction.

The patient then underwent a transesophageal echocardiogram (TEE) to evaluate the aortic valve and the aortic root. TEE showed a normal-sized aortic root and a bicuspid aortic valve with no significant aortic regurgitation (Figure 4). The coarctation was visualized in the thoracic aortic views (Figure 5) and the gradient measured 64 mmHg (Figure 6).
The patient was then referred to cardiothoracic surgery to evaluate her candidacy for surgical or percutaneous therapy.
Discussion. Aortic coarctation is a congenital malformation that usually presents early in life. It manifests as childhood hypertension, lower extremity fatigue or weakness, diminished lower extremity pulses and/or congestive heart failure.

Diagnosis is usually based on clinical suspicion and physical findings. The latter include blood pressure difference between the upper and lower extremities, pulse delay and systolic murmur over the thoracic spine. Other manifestations can include bicuspid aortic valve systolic ejection sound and/or murmur and neurological complaints. Prognosis and survival depend on the disease severity and patient’s age at the time of correction.
Treatment consists of aggressive hypertension therapy, endocarditis prophylaxis and corrective treatment for coarctation lesions with a high gradient. In this case report, we present aortic coarctation with bicuspid aortic valve in a 52-year-old female. Our patient was relatively asymptomatic until she presented with chest discomfort, fatigue and dyspnea in her fifth decade of life.

References: 

References
1. Bauer M, Alexi-Meskishvili V, Bauer U, et al. Benefits of surgical repair of coarctation of the aorta in patients older than 50 years. Ann Thorac Surg 2001; 72: 2060– 2064.
2. Cevik S, Izgi C, Cevik C. Asymptomatic severe aortic coarctation in an 80-year-old man. Tex Heart Inst J 2004;31:429–431.

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