Partial anomalous left pulmonary artery in heterotaxy syndrome. A case report and review of literature

Utility of Three-Dimensional Printed Model in Biventricular Repair of Complex Congenital Cardiac Defects: Case Report and Review of Literature

“…The patient is a 5-month-old male who was diagnosed with multiple congenital anomalies including heterotaxy syndrome, polysplenia, intestinal malrotation, absent septum pellucidum, cleft lip and palate, small left kidney with hydronephrosis and right inguinal hernia at birth. His post-natal echocardiogram revealed mesocardia with apex to the left, midline liver, atrial situs ambiguous with a common atrium and ipsilateral pulmonary veins (left pulmonary veins draining into the left-sided atrium and right pulmonary veins draining into the right-sided atrium), D-looped ventricles, normally related great arteries (A,D,S), interrupted inferior vena cava (IVC) with hemiazygos continuation to the left superior vena cava (SVC) draining into the left and anterior aspect of the common atrium, hepatic veins draining to the common atrium, a partial AV canal, small anterior muscular ventricular septal defect (VSD), and a moderate sized patent ductus arteriosus (PDA) [ 6 ]. Genetic testing detected an interstitial deletion on chromosome 20.…”

“…Cardiac computerized tomography angiography (CTA) confirmed the cardiac anatomy previously seen on post-natal echocardiogram ( Figure 1 and Figure 2 ). It also revealed an anomalous origin of an accessory left pulmonary artery (LPA) arising from the proximal right pulmonary artery (RPA), which resulted in severe compression of the left mainstem bronchus against the descending aorta, forming a partial left pulmonary artery sling [ 6 ]. Given his complex medical history and cardiac anatomy, a three-dimensional (3D) cardiac printed model was used to further delineate his cardiac anatomy and determine the safest plan for surgical intervention.…”

“…Normal biventricular size and systolic function with no evidence of significant intracardiac shunt was obtained on cardiac MRI performed 5 weeks postoperatively. His postoperative course from the cardiac perspective remained uneventful until discharge [ 6 ]. Figure 5 and Figure 6 show the post-operative CT imaging of the patient.…”