Case presentation
A 30-month-old female weighing 10800 gms, was brought to our hospital with complaints of dyspnea and recurrent respiratory infections, and was diagnosed to have visceral situs inversus. She was the 3rd of five siblings, born to non-consanguineous marriage couple, and she was delivered with no relevant birth details. Her siblings were normal. Clinical examination revealed systolic murmur in the base of the heart, the liver was clear on the left side. Chest x-ray (CXR) showed dextrocardia with important cardiomegaly along with increased pulmonary vascular markings (Figure 1). Transthoracic echocardiography (TTE) confirmed the diagnosis of SIT along with large ASD measuring 8*12 mm with bidirectional flow, and a non-significant patent ductus arteriosus (PDA). There was severe right ventricular enlargement with mild to moderate tricuspid valve regurgitation. The left atrium cavity was small. Estimated systolic pressure in the right ventricle was 75 mmHg (calculated from the flow velocity of the tricuspid regurgitation jet and the assumed central venous pressure), and the mean pressure in the pulmonary artery was estimated to be 55 mmHg. The right pulmonary veins were seen draining to the morphologically left atrium, but the left ones were not visible, therefore computed tomography angiography (CTA) was requested. CTA revealed that the left pulmonary veins were draining anomalously to the left-sided right atrium with normal drainage of the right pulmonary veins to the left atrium (Figure 2). The patient was operated on through median sternotomy approach. The pericardium was opened. The morphological right atrium was to the left and the apex of the heart was right-sided (Figure 3-A). Large PDA was seen to the right between the right-sided pulmonary artery and the aorta. It was dissected and closed appropriately. A total cardiopulmonary bypass (CPB) was prepared. The aorta was clamped and the heart was arrested by antegrade del Nido cardioplegic solution. The right atrium was opened with an incision parallel to the atrioventricular groove. The left pulmonary veins were seen draining directly to the right atrium just above the large ASD, whereas the right ones were draining to the left atrium. A large fresh autologous pericardial patch was fashioned to close the ASD and create a baffle draining the anomalous right pulmonary veins to the left atrium beneath the patch (Figure 3-B). The right atriotomy was closed, and the aortic cross clamp was released. The patient was weaned off the CPB uneventfully. Chest was closed in standard fashion. Mechanical ventilation was required for 8 hours. The patient’s convalescence in the intensive care unit was uneventful. Postoperative TTE showed an excellent result of the operation with estimated systolic pressure in the right ventricle of about 35 mmHg, along with mild tricuspid regurgitation. The patient was discharged on the fifth day without any complications. The patient was followed-up for 12 months, and she was without any complaints along with important clinical and radiological improvement.