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A 7-year-old boy with a history of frequent respiratory tract infections in infancy, presented with easy fatigability and exertional dyspnoea. On examination, he had mild cyanosis, clubbing, left parasternal heave and a loud pulmonary component of the second heart sound with no murmurs. An electrocardiogram showed right ventricular hypertrophy with right axis deviation. An echocardiogram revealed right ventricular hypertrophy with dilated main pulmonary artery and left pulmonary artery. Right pulmonary artery was not visualised. A diagnostic cardiac catheterisation was done. A right ventricular angiogram showed the main pulmonary artery and left pulmonary artery with absence of the right pulmonary artery and a moderate sized patent ductus arterious (panel A). An aortic root angiogram showed the right pulmonary artery arising from the posterior part of the ascending aorta (panel B). The left pulmonary artery pressure was 130/76 mm Hg and right pulmonary artery pressure was 125/78 mm Hg with no fall after 100% oxygen. Patient was put on medical management.
Hemitruncus is best defined as a condition in which one branch of the pulmonary artery originates from the ascending aorta and the other branch of the pulmonary artery courses normally from the main pulmonary artery which arises from the right ventricle.1 2 The anomalous origin of one pulmonary artery from the ascending aorta should be suspected when unexplained pulmonary hypertension is diagnosed and no structural intracardiac defects are observed. This illustrates the classic angiographic images of a rare anomaly.
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