Asymptomatic bicuspid aortic valve with dilated ascending aorta in adult patient

Abstract

Introduction and case report description: Bicuspid aortic valve is a congenital malformation which is result of an abnormal cusp formation during early embryogenesis. The most frequent finding in association with this abnormality is dilation of the proximal ascending aorta. We present a case of 53year old patient who was incidentally diagnosed with bicuspid aortic valve and dilated ascending aorta. Our patient had been well until September 2010, when he had a car accident and a fracture of the hip and contusion of the right shoulder. Since then, he had a several episodes of a dull chest pain and because of this symptoms, he visited his physician who directed him to our hospital for further examination, because he incidentally detected a diastolic heart murmur. The patient had no remarkable medical history,his ECG on admission was normal. Because of the presence of early diastolic murmur heard on the third intercostal space, echocardiography was performed. He was diagnosed with bicuspid aortic valve, and dilated ascending aorta (59mm), with moderate aortic regurgitation. CT scan was also performed to evaluate the diameter and the extent of the dilatation, and the intraluminal diameter of the ascending aorta was 57x56mm, with no signs of dissection. He underwent coronary angiography and carotid ultrasound (he had no significant stenosis of the coronary and carotid arteries) and was further directed in Cardiac Surgery Center, for aortic valve and ascending aorta replacement. According to the latest guidelines on BAV, our patient meets the criteria for surgical treatment of the condition(surgery should be considered for any patient with aortic root diameter >/=55mm). BAV malformations are inherited, particularly in males, so imaging of the first degree relatives is appropriate if the patient has an associated aortopathy or a family history of VHD/aortopathy. TTE is usually the primary imaging technique for diagnosing BAV. Nevertheless, CT or MRI scan is recommended for evaluation of the entire ascending aorta because TTE may not visualize the entire ascending aorta and may fail to calculate the largest diameter. Bicuspid aortic valve is the most common congenital cardiac malformation, which is rather recognized as a syndrome which incorporates aortic wall abnormalities, including aortic dilatation. This condition may also be complicated with aortic valve stenosis and/or regurgitation. Individuals may have a normal functioning BAV and may be not aware of the potential complicatios. Delayed identifying of this abnormality or the complications may have a fatal consequences. In our case, the patient was asymptomatic and was diagnosed incidentally. Regular follow-up becomes mandatory after BAV has been diagnosed, in order to closely observe such patients with regard to progression of the disease itself and its complications, and in order to suggest treatments. For patients who have a severely diseased aortic valve and aorta, aortic valve replacement and ascending aorta replacement is the treatment of choice.