Double-outlet left atrium: Ventriculo-atrial malalignment defect
A four-month-old, female infant, weighing two kilograms, presented to us with failure to thrive and easy fatigability during feeding, since birth. She had two episodes of lower respiratory tract infection in the past and had a history of mild cyanosis on crying. The physical examination revealed a heart rate of 124 beats/minute, blood pressure of 84/52 mmHg, respiratory rate of 32/minute with oxygen saturation (SaO2) of 92% in room air. A pansystolic murmur at the left lower sternal border and a split second heart sound were heard on auscultation. Mild hepatomegaly was noted. The electrocardiogram (EKG) showed left atrial enlargement, with left axis deviation, without ventricular hypertrophy. The chest radiograph showed cardiomegaly and increased pulmonary vascularity. A transthoracic echocardiography [ and ] revealed situs solitus, levocardia, and d-loop ventricles, with normal pulmonary and systemic venous drainage. Well-defined right and left atria were present. There was right atrial outlet Atresia, with the only outlet for the right atrium being a secundum ASD. The left atrium emptied into the right and left ventricles through two AV valves. The right-sided AV valve had septal attachments and opened into the trabeculated right ventricle, while the left AV valve opened into a smooth-walled left ventricle. Thus, morphologically the right and left AV valves resembled the tricuspid and mitral valves, respectively. There was no significant AV regurgitation. There was a restrictive inlet ventricular septal defect (VSD) shunting left to right with a gradient of 40 mmHg [ and ]. The great vessels were normal in origin and position. There was no right ventricular outflow obstruction. The left ventricular outflow tract (LVOT) was somewhat elongated []. The coronary sinus and aortic arch anatomy were normal.\nShe was planned for an initial pulmonary artery banding followed by total repair at a later date. However, she died at home from a possible chest infection few weeks after presentation to the hospital.