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ABSTRACT Introduction: Homografts and bovine jugular vein are the most commonly used conduits for right ventricular outflow tract reconstruction at the time of primary repair of truncus arteriosus. Methods: We reviewed all truncus patients from 1990 to 2020 in two mid-volume centers. Inclusion criteria were primary repair, age under one year, and implantation of either homograft or bovine jugular vein. Kaplan-Meier analysis was used to estimate survival, freedom from reoperation on right ventricular outflow tract, and freedom from right ventricular outflow tract reoperation or catheter intervention. Results: Seventy-three patients met the inclusion criteria, homografts were implanted in 31, and bovine jugular vein in 42. There was no difference in preoperative characteristics between the two groups. There were 25/73 (34%) early postoperative deaths and no late deaths. Follow-up for survivals was 17.5 (interquartile range 13.5) years for homograft group, and 11.5 (interquartile range 8.5) years for bovine jugular vein group (P=0.002). Freedom from reoperation on right ventricular outflow tract at one, five, and 10 years in the homograft group were 100%, 83%, and 53%; and in bovine jugular vein group, it was 100%, 85%, and 50% (P=0.79). There was no difference in freedom from reoperation or catheter intervention (P=0.32). Conclusion: Bovine jugular vein was equivalent to homografts up to 10 years in terms of survival and freedom from right ventricular outflow tract reoperation or catheter intervention. The choice of either valved conduit did not influence the durability of the right ventricle-pulmonary artery conduit in truncus arteriosus.

We describe a neonate with a unique variant of truncus arteriosus with interrupted aortic arch, an absent ascending aorta, persistent right dorsal aorta, and an unusual brachiocephalic artery pattern in which all head and neck vessels were supplied from the ductal arch-descending aorta continuum. (Level of Difficulty: Beginner.)

Abstract Objectives Persistent truncus arteriosus represents less than 3% of all congenital heart defects. We aim to analyze mid-term outcomes after primary Truncus arteriosus repair at different ages and to identify the risk factors contributing to mortality and the need for intervention after surgical repair. Methods This retrospective cohort study included 36 children, underwent repair of Truncus arteriosus in the period from January 2011 to December 2018 in two institutions. We recorded the clinical and echocardiographic data for the patients preoperatively, early postoperative, 6 months postoperative, then every year until their last documented follow-up appointment. Results Thirty-six patients had truncus arteriosus repair during the study period. Thirty-one patients had open sternum post-repair, and two patients required extracorporeal membrane oxygenation. Bleeding occurred in 15 patients (41.67%), and operative mortality occurred in 5 patients (14.7%). Patients with truncus arteriosus type 2 (p = 0.008) and 3 (p = 0.001) and who were ventilated preoperatively (p

Congenital heart defects in adulthood constitute the sufficient proportion of cardiac pathology. The common arterial trunk (CAT) is one of the сongenital cardiac defects that is described in the adult population. Without surgical intervention, patients who survived become inoperable quickly due to the development of early congestive heart insufficiency and pulmonary hypertension. The purpose of the research is to analyze the autopsy case of unoperated congenital cardiac defects in adulthood. Materials and methods. The pathomorphological analysis of the case of death of a 33-year-old male with untreated complex congenital heart defect – CAT, ventricular septal defect, Eisenmenger's syndrome was made using standard and elective histological techniques. Results. In the forensic examination of the body of the deceased 33-year-old male, congenital heart defect was diagnosed as CAT and ventricular septal defect. Coronary, pulmonary and major arteries of a large circle of blood circulate from the CAT. Congenital heart defect has been complicated by the development of cardiomegaly (heart weight 1500 g) with myocardial hypertrophy of both ventricles and led to decompensation of cardiac activity. During the pathohistological examination of the valve of the CAT we determined the foci of fibrinoid necrosis, myxomatosis, single accumulation of lymphocytes, focal fibrosis. In the myocardium of the right and left ventricles we observed hypertrophy, focal contraction and fragmentation of cardiomyocytes, expressed intramuscular edema, sclerosis of the walls of the small intramural arteries with perifocal areas of vacuolar degeneration of cardiomyocytes, stromal cardiosclerosis, isolated interstitial infiltration of lymphocytes, dilatation of lymphatic vessels. In the pulmonary tissue diffuse hemosiderosis, multiple intravalveolar hemorrhages, sclerosis of the small pulmonary arteries with the formation of two- and three-sided lumen, sclerosis of interalveolar membranes were detected. Morphological changes in the myocardium and lungs confirm prolonged congestive heart failure and pulmonary hypertension. Conclusions. We describe a unique case of long-term survival of a patient with CAT without surgical intervention. The complex of pathomorphological changes of the described case testifies to cardiovascular remodeling, which is the base of hemodynamic evolution of complex congenital heart disease and allowed the man to live up to 33 years without surgical intervention.

The embryonic process of forming a complex structure such as the heart remains poorly understood. Here, we show that Six2 marks a dynamic subset of second heart field progenitors. Six2-positive (Six2+) progenitors are rapidly recruited and assigned, and their descendants are allocated successively to regions of the heart from the right ventricle (RV) to the pulmonary trunk. Global ablation of Six2+ progenitors resulted in RV hypoplasia and pulmonary atresia. An early stage-specific ablation of a small subset of Six2+ progenitors did not cause any apparent structural defect at birth but rather resulted in adult-onset cardiac hypertrophy and dysfunction. Furthermore, Six2 expression depends in part on Shh signaling, and Shh deletion resulted in severe deficiency of Six2+ progenitors. Collectively, these findings unveil the chronological features of cardiogenesis, in which the mammalian heart is built sequentially by temporally distinct populations of cardiac progenitors, and provide insights into late-onset congenital heart disease.

Persistent Truncus Arteriosus (PTA) is an extremely rare defect accounting for under 1% of all congenital heart disease, and over 85% of patients succumb to heart failure in infancy. We report a case of van Praagh type A3 PTA, which represents its rarest variant and accounts for only 8–10% of all patients with PTA. The presence of ductal stenosis in such cases is further exceedingly rare.