Pacman heart coexisting with coarctation of aorta and bicuspid aortic valve
A 21-year-old male patient presented with headache and hypertension (160/95 mmHg) and his clinical history was unremarkable. On physical examination, diastolic murmur was detected in the parasternal 3rd and 4th intercostal region. In transthoracic echocardiography, left ventricle diameters were within normal range, ejection fraction was preserved and moderate eccentric aortic regurgitation was observed. Modified parasternal short axis view revealed aortic valve with two cusp (figure 1 and video 1). Parasternal long axis view showed an incomplete muscular VSD whose mouth was opened at diastole and closed in systole (figure 2 and video 2). Although high blood pressure is frequently encountered as primary hypertension, it is seen in young patients due to secondary causes such as CoA, pheochromocytoma and renal artery fibromuscular dysplasia. Coexistence of aortic coartation with BAV is a well known congenital anomaly. In this case severe stenosis in the proximal part of the descending aorta was detected in computed tomographic angiography (figure 3). Patient was referred to the cardiovascular surgeon for surgical intervention due to moderate aortic insufficiency and CoA.
Pacman heart (incomplete muscular ventricular septal defect) is extremely rare congenital deformity (1,2), and combination of partial VSD, BAV and CoA occurring in the same patient has not been reported yet.