Cor triatriatum sinister with situs inversus totalis in an infant
A five month old female child was referred for cardiac murmur evaluation. History of suck rest suck cycle was present. No family history of congenital heart disease was noted. Physical examination revealed a pulse rate of 145 beats/minute, blood pressure of 94/50 mmHg, with baseline oxygen saturation of 99%. On cardiac examination, the second heart sound was normal and there was no evidence of pulmonary hypertension. Auscultation of the right chest revealed a 3/6 systolic murmur at the right parasternal border. Other systems were normal. Laboratory investigation showed a hemoglobin level of 10 g% and a white cell count of 16,000 mm3. The chest X-ray demonstrated dextrocardia, cardiomegaly and normal pulmonary vascular markings. A gastric air bubble was noted on the right side, and a liver shadow on the left side was present. The electrocardiogram(ECG) revealed a sinus rhythm with dextrocardia. Abdominal ultrasound demonstrated the stomach and spleen on the right side and the liver on the left. The aorta and the inferior vena cava were inverted. Transthoracic echocardiography (TTE) demonstrated {I,L,I} (I, situs visceroatrialis inversus; L, L-loop of the ventricles; I, normally related inverted great arteries).\nIt also showed a fibromuscular membrane across the left atrium, dividing it into two compartments suggestive of cor triatriatum sinister (. & ). The two chambers communicated through two restrictive orifices (gradient:16mmHg) (). A small size patent ductus arteriosus (gradient 20/6mmHg) with left to right shunt was present (). Other cardiac anomalies like patent foramen ovale (PFO), atrial septal defect (ASD) and anomalous venous connections were ruled out and the diagnosis of cor triatriatum sinister associated with TSI was reconfirmed.