Left hemitruncus with normal right-sided pressures in an adult

Abstract

Hemitruncus is a rare congenital heart disease. Anomalous origin of the left pulmonary artery is not only rare but also pathogenetically different from anomalous origin of the right pulmonary artery from the ascending aorta. In most cases in isolated hemitrucus pressures in the right ventricle and the normally originating pulmonary artery are systemic or suprasystemic. We present a rare case of anomalous origin of the left pulmonary artery from the ascending aorta diagnosed in an adult with normal pressures in the right ventricle and normally originating pulmonary artery. To the best of our knowledge, this unique haemodynamics has never been reported in the literature.

Multimodality hemodynamic evaluation for optimizing management of anomalous origin of the right pulmonary artery from the aorta in an adolescent

“…[ 1 , 2 ] In a few, however, it exists as an isolated anomaly. [ 1 , 2 , 5 - 8 ] Irrespective of associated lesions, anomalous origin of the PA is a serious disease, with most patients presenting with heart failure during infancy and up to 70% dying within the 1 st year if left unoperated. [ 1 , 3 , 4 ] Presentation beyond the first decade is limited to case reports.…”

“…[ 1 , 3 , 4 ] Presentation beyond the first decade is limited to case reports. [ 3 , 5 - 8 , 11 - 13 ]…”

“…AAo: Ascending aorta, Inn vein: Innominate vein, LA: Left atrium, LPA: Left pulmonary artery, MPA: Main pulmonary artery, RPA: Right pulmonary artery, SCV: Superior caval vein. Modified with permission from Gupta and Gupta[ 8 ]…”

“…[ 3 , 4 ] In contrast, operability assessment in an older child, even in the absence of coexisting heart disease, may be challenging. [ 5 - 8 ] Differential blood flow and vascular resistance in the left and the right lung makes the hemodynamic assessment difficult. [ 6 , 7 ] We demonstrate stepwise multimodality hemodynamic evaluation that helped in successful surgical repair in an adolescent boy.…”

Left Pulmonary Artery from the Ascending Aorta: A Case Report and Review of Published Cases

“…In Table 2, we list the described associated defects or malformations in these cases, having excluded 12 cases because of insufficient details [4][5][6][7][8][9]. Of the remaining 113 cases, 15 (13.3%) were not associated with intracardiac or extracardiac malformations [10][11][12][13][14][15][16][17][18][19][20][21][22][23]. Tetralogy of Fallot was the most frequently associated cardiac malformation, reported in 59 (52.2%) cases [2,3,.…”