Congenitally unguarded tricuspid valve (TV) orifice, a variant of TV dysplasia, is a rare malformation with protean manifestations. This report describes a symptomatic adult male with gross right heart failure and atrial fibrillation, who was found to have an unguarded TV orifice with isolation of the trabecular apical cavity of the right ventricle (RV) and muscular ridges separating outflow tract (forme-fruste of the double-chambered RV). The right ventricular outflow tract remained patent.
Although acquired left ventricular outflow obstruction has been reported in a variety of conditions, there are scant reports of its occurrence following mitral valve replacement (MVR). This study describes two female patients, who developed severe discrete subaortic stenosis, five years following MVR. In both cases, the mitral valve was replaced by a porcine Carpentier-Edwards 27-mm bioprosthesis with preservation of mitral valve leaflets. The risk of very late left ventricular outflow tract obstruction after bio-prosthetic MVR with preservation of subvalvular apparatus needs to be kept in mind in symptomatic patients.
A 23-year-young female presented with mild exertional dyspnoea and palpitation since early childhood. By deploying 2D- and 3D echocardiography, she was detected to have situs solitus, atrioventricular and ventriculoarterial discordance with L-malposition of great vessels, valvular pulmonary stenosis, large secundum atrial septal defect, bicuspid aortic valve, right-sided aortic arch, and moderately severe mitral and tricuspid valve regurgitation. Typical parachute deformities of the morphologically mitral and tricuspid valves were observed. 3D echocardiography revealed a single papillary muscle in the morphologically left ventricle placed anteriorly and providing insertion to tendinous cords and only a moderator band with no other muscle bundles in the morphologically right ventricle placed posteriorly and providing attachment to two strings of cords. Considering the minimal symptoms, conservative treatment was pursued.
Left atrial dissection is a very uncommon complication of cardiac surgery and usually causes significant hemodynamic compromise. Little is known about spontaneous dissection of the left atrium. Two patients, one middle-aged man and another elderly woman were evaluated following stroke. Routine trans-thoracic echocardiogram showed vertical division of the left atrium with both chambers communicating with each other through an orifice. Detailed trans-oesophageal echocardiographic study revealed dissection of the left atrium producing an additional false chamber (pseudo-aneurysm) placed posterior to the left atrial appendage and above the postero-lateral aspect of mitral annulus. Spontaneous dissection of the left atrium is extremely rare, and there is no report of cerebral embolism associated with it. Review of literature reveals interesting facets of this rare entity.
In adult patients, a high index of suspicion is required to detect congenital mitral stenosis/regurgitation with large solitary PM (resembling a parachute mitral valve) which may masquerade as hypertrophic cardiomyopathy or rheumatic mitral valve disease.
Systolic AR is a unique hemodynamic phenomenon in patients with acute rheumatic carditis involving both mitral and aortic valves and occurs in presence of severe MR.
Hypoplastic right heart syndrome is a rare cyanotic congenital heart disease with under-development of the right ventricle, tricuspid, and pulmonary valves leading to right-to-left shunting of the blood through inter-atrial septal defect. Perinatal mortality is high with very few patients surviving to adulthood without corrective surgery. This report describes a 26-year-old young woman, who had recurrent abortions and stillbirths and detected to have marked cyanosis with hypoplastic right heart, sub-arterial ventricular septal defect, absent pulmonary valve, non-compaction of the left ventricle, and bicuspid aortic valve with aortic regurgitation. The patient died owing to progressive heart failure 4 years after the diagnosis was made.
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