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Tetralogy of Fallot with aortic stenosis is an extremely rare entity which bears some morphological similarities with the common arterial trunk. Through two illustrative cases of TOF with aortic stenosis we describe the shared anatomical peculiarities of the two anomalies with a review of the possible genetic and developmental factors responsible for the association. [ABSTRACT FROM AUTHOR]

Background: Pulmonary stenosis in patients with common arterial trunk protects the pulmonary vasculature. In our recently published prospective study of common arterial trunk, some patients with sinusal origin of the pulmonary arterial segment had pulmonary stenosis induced by systolic excursion of a truncal valvar leaflet. We aimed to determine the detailed morphologic characteristics of this unusual finding. Methods and Results: All 70 patients underwent echocardiography and computed tomographic angiography as per predefined study protocol. In selected cases, we also performed cardiac catheterization. Among 27 patients with aortic dominance, we found sinusal origin of the pulmonary arterial segment. In 5 of these patients, pulmonary stenosis was induced by systolic excursion of a truncal valvar leaflet. In all these patients, the truncal valve was trisinusate, albeit with asymmetric sinuses. The pulmonary arterial segment arose from the largest left sinus with its relatively large leaflet obstructing the pulmonary orifice during systole. In the remaining 22 patients, without pulmonary stenosis but with sinusal origin of pulmonary arterial segment, the truncal valve was quadrisinusate in 7, bisinusate in 2, and trisinusate in 13. None of the patients with quadrisinusate and bisinusate truncal valves had pulmonary stenosis. Among the 13 patients with trisinusate valves, the sinuses of the truncal valve were symmetrical in 6, while in 7, the pulmonary orifice originated from a smaller asymmetric sinus. Conclusion: Pulmonary stenosis, produced by a relatively large leaflet of an asymmetric truncal sinus, may prevent early development of pulmonary vascular disease. Timely recognition of this unusual mechanism of pulmonary stenosis is important for optimal management. [ABSTRACT FROM AUTHOR]

The pulmonary dominant variant of the common arterial trunk has always been reported to be associated with aortic coarctation, or interruption of the aortic arch, along with a duct-dependent systemic circulation. This mandates a complex surgical repair with attendant high surgical mortality. We report a 23-day-old baby with a pulmonary dominant trunk with mild aortic hypoplasia but with an arch free of coarctation or interruption, who underwent successful surgical repair. In the preoperative evaluation of a common arterial trunk, pulmonary dominance may not necessarily denote an adverse risk factor when the aorta is only mildly hypoplastic. [ABSTRACT FROM AUTHOR]

Embryologically, the common arterial trunk (CAT) is due to the failure of septation by the major outflow cushions. This invariably leads to the presence of an associated large juxta-arterial ventricular septal defect. Overriding of the ventricular septum leads to its biventricular origin in more than 2/3rd of cases. Uncommonly, CAT may be associated with an intact ventricular septum (IVS) and univentricular origin. We describe an infant with CAT, intact IVS, hypoplastic right ventricle, and Ebstein's anomaly of the tricuspid valve who presented to us and palliated successfully. The embryological mechanisms are discussed in this report. [ABSTRACT FROM AUTHOR]

Background: The true incidence of truncus arteriosus in underdeveloped countries is difficult to determine. This is due largely to underreporting as a result of nonavailability of technologically advanced facilities to make definitive diagnosis prenatally. There is a lack of data on the profile and outcome of patients with persistent truncus arteriosus (PTA) in Nigeria. This study aims to document the demographic characteristics, mode of presentation, indications for echocardiography, associated anomalies, average age at diagnosis, and outcome of patients with truncus arteriosus in our center. Methods: Prospective and cross-sectional involving consecutive patients diagnosed with PTA using echocardiography at the Paediatric Department of Lagos State University Teaching Hospital, Lagos, Nigeria as part of a large study between January 2008 and December 2015. Results: Only 25 patients had PTA during the study period. The prevalence of PTA among children presenting at the study center during the study period was 7.9/100,000. It constituted 2.4% of the cases of congenital heart disease and 7.1% of cases of cyanotic congenital heart disease. The male:female ratio was 1:1.1. The ages of the patients at diagnosis ranged between 0.75 and 153 months with a mean age at diagnosis ± standard deviation of 18.4 months ± 37.7. Only about 40% of patients were diagnosed within the neonatal period. Cyanosis was the most frequent indication for evaluation. Conclusion: PTA is as common in Nigeria as in the other parts of the world but diagnosed late. Cyanosis is the most common presenting feature. [ABSTRACT FROM AUTHOR]

Embryologically, incomplete conotruncal septation with resultant single aortopulmonary trunk and defective ventricular septation defines the congenital cardiac lesion known as persistent truncus arteriosus (PTA). Torrential pulmonary blood flow is inevitable when this rare lesion is further compounded by patency of the arterial ductus. Such was the case of a patient who presented with fast breathing, reduced suck, darkening of the tongue, and extremities. Urgent echocardiographic diagnosis was PTA (Type A1) with patent ductus arteriosus and pulmonary hypertension and left ventricular systolic dysfunction. [ABSTRACT FROM AUTHOR]

We report a newborn with truncus arteriosus and a very unusual variant of "crossed" pulmonary arteries. The left pulmonary artery arose anterior and slightly to the right side of the common trunk and the right pulmonary artery arose from the posterior and left side of the common trunk. Computed tomographic images and a diagram are provided. [ABSTRACT FROM AUTHOR]