Unmasking of severe mitral regurgitation after pericardiectomy.

Mitral valve prolapse in the young is the most common cause of mitral regurgitation (MR). Constrictive pericarditis (CP) is a chronic and end-stage manifestation of the inflammatory process of the pericardium. Apart from equalization of diastolic pressure of the chambers as well as increased ventricular interdependence, the thickened pericardial sheath in end-stage pericarditis fails to accommodate the intrathoracic venous return due to restricted cardiac chamber filling. Intracardiac valvular pathologies might co-exist in CP. This report presents a case in which effusive CP was associated with MR and highlights the pivotal role of intraoperative transesophageal echocardiography (TEE) in the decision-making of such co-existent lesions.

Holt-oram syndrome in adult presenting with heart failure: a rare presentation.

Holt-Oram syndrome is a rare inherited disorder involving the hands, arms, and the heart. The defects involve carpal bones of the wrist and the thumb and the associated cardiac anomalies like atrial or ventricular septal defects. Congenital cardiac and upper-limb malformations frequently occur together and are classified as heart-hand syndromes. The most common amongst the heart-hand disorders is the Holt-Oram syndrome, which is characterized by septal defects of the heart and preaxial radial ray abnormalities. Its incidence is one in 100,000 live births. Approximately three out of four patients have some cardiac abnormality with common associations being either an atrial septal defect or ventricular septal defect. Herein, we report a rare sporadic case of Holt-Oram syndrome with atrial septal defect with symptoms of heart failure in a forty-five-year-old lady who underwent emergency cardiac surgery for the symptoms.