Anomalous origin of the left brachiocephalic trunk from the main pulmonary artery with right aortic arch is an extremely rare congenital anomaly. These types of cases are incidentally diagnosed in adults and children with symptoms of vertebral basilar insufficiency. We report the clinical findings, investigation, and management of a case of this rare congenital anomaly.
Anomalous origin of the right coronary artery (RCA) from the pulmonary artery is a rare entity that causes chronic left-to-right shunting of blood from the RCA, through the coronary collaterals into the pulmonary artery. This results in persistent myocardial ischemia and ventricular dysfunction. Association of this anomaly with an aortopulmonary window worsens the condition further due to an additional shunt. We encountered a combination of these two anomalies along with an ostium secundum atrial septal defect in a 10-month-old baby who presented with excessive crying and failure to thrive. Evaluation by transthoracic echocardiography and cardiac computed tomography scan confirmed the diagnosis. Intrapulmonary baffle was done using a pericardial patch to connect the RCA origin to ascending aorta through the aortopulmonary window, similar to Takeuchi procedure. Concomitantly, ostium secundum atrial septal defect was also closed.
A 40-year old male presented with history of dyspnea and recurrent syncope. Preliminary evaluation revealed findings suggestive of severe pulmonary hypertension. His echocardiogram revealed severe stenosis of the right pulmonary artery (RPA) with severe pulmonary hypertension, which was confirmed on 64-slice MSCT. On cardiac catheterization, the stenotic segment could not be crossed. Hence he underwent surgical reconstruction of the RPA. Post-operatively, he developed arterial desaturation and hypotension due to compression of the reconstructed segment by the ascending aorta. Subsequently, intraoperative direct stenting of the RPA was performed on a beating heart. Post-procedure there was a significant drop in right heart pressures which was sustained on 3-month follow up. The patient also had occlusion of the Coeliac and Superior Mesenteric arteries which was demonstrated on MSCT and angiography, suggesting Aortoarteritis as the etiology. This case illustrates an unusual presentation of Aortoarteritis and the role of hybrid procedures in situations where percutaneous intervention may not be technically feasible.
The anomalies of drainage of systemic venous communications to the heart are many. But only few cause significant hemodynamic alteration and thus, complications. When they do, they need to be surgically corrected. We report an unusual case of a patient who was found to have left pulmonary veins draining into the left superior venacava (LSVC), which in turn was opening into the roof of the left atrium that had unroofed coronary sinus morphology. Innominate vein was absent. LSVC was divided cranial to where the pulmonary veins were draining into it. Lower end was suture closed. Proximal part of the LSVC was anastamosed to left pulmonary artery.
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