Persistent truncus arteriosus

Abstract: Based upon a series of 57 necropsy cases and upon the literature, it was found that there are two basic types of common aorticopulmonary (A-P) trunk: those with a ventricular septal defect, and those without. The latter is exceedingly rare and did not occur in this series. Truncus with a ventricular septal defect was found to consist of the following: (1) absence of the distal portion of the pulmonary infundibulum (very similar to tetralogy of Fallot with pulmonary atresia); (2) partial or complete absence of the pulmonary valve; (3) partial or complete absence of the aorticopulmonary septum; and (4) arterial arches 4 and 6 varied inversely in their development (e.g., well developed aortic arch with absent ductus arteriosus, or interrupted aortic arch with a large patent ductus arteriosus). Thus, there appeared to be no such thing as “true” persistent truncus arteriosus in the timehonored sense of persistence of an undivided conotruncal channel. While it is correct that the truncus is undivided (the aorticopulmonary septum being partially or totally absent), at the level of the conus the anomaly is not a failure of septation but absence of the entire distal portion of the pulmonary infundibulum (septum and free wall). Hence, at the level of the conus, common trunk is not a large infundibular septal defect but an extreme tetralogy of Fallot. At the distal conus, nothing has persisted. When the aortic arch was present, there was a high incidence of right aortic arch (27%) similar to tetralogy. The truncal valve basically was the aortic valve, explaining why it usually was tricuspid (67%). When some pulmonary leaflet tissue persisted, made possible by absence of the aorticopulmonary septum at the semilunar valve level, the truncal valve then was quadricuspid (24%). Failure of leaflet separation either in a basically tricuspid or quadricuspid valve occasionally resulted in a bicuspid truncal valve (7%). Truncus is not a form of transposition of the great arteries. Mitral-aortic fibrous continuity was present in all cases. The ventricular septal defect is not primarily “membranous.” It is due to absence of the distal pulmonary infundibulum. The coexistence of single ventricle and common aorticopulmonary trunk has been considerably overestimated. This combination did not occur in this series and is extremely rare (?non-existent). A revised and simplified classification of truncus is proposed.

Abstract: General Considerations. Developmental Biology. Cardiopulmonary Bypass, Hypothermia, And Circulatory Arrest. Myocardial Preservation in the Immature Heart. Anesthesia for Cardiovascular Surgery . Perioperative Care. Conduits: Clinical and Experimental Aspects. Interventional Cardiology. Congenital Heart Defects and Procedures. Artial Septal Defect. Anomalies of the Pulmonary Veins. Atrioventricular Canal Defects. Ventricular Septal Defect. Patent Ducus Arteriosus. Tetralogy of Fallot. Pulmonary Atresia with Intact Ventricular Septum. Single Ventricle/Tricuspid Atresia. Ebstein's Anomaly. Truncus Arteriosus. Aortopulmonary Window. Coronary Artery Anomalies. Obstruction of the Left Ventricular Outflow Tract. Aortic Coarctation. Interrupted Aortic Arch. Hypoplastic Left Heart Syndrome. Pediatric Valve Replacement. Vascular Rings, Slings, And Tracheal Anomalies. D-Transposition of the Great Arteries. Corrected Transposition of the Great Arteries. Double-Outlet Right Ventricle. Cardiac Masses. Future Developments. Cardiac Transplantation. Myocardial Preservation. Cerebral Protection. Fetal Intervention and Congenital Heart Disease. Index.

Abstract: The objective of this study was to ascertain the prevalence and survival rate of children born with a heart defect. A total of 816,569 children live-born between 1980 and 1990 in Bohemia (52,478 km2, population 6.314 million, western Czech Republic) were followed up and those with suspected heart disease referred to a center. Echocardiography was done in all of them. All dead children were autopsied. Congenital heart disease was found in 5030 of 816,569 children (6.16 per 1000 live births). The most frequent conditions were ventricular septal defect (41.59%), atrial septal defect (8.67%), aortic (7.77%) and pulmonary (5.81%) stenoses, transposition of the great arteries (5.39%), coarctation of the aorta (5.29%) and persistent ductus arteriosus (5.07%). The first week was survived by 92.46%, the first month by 89.14%, 6 months by 82.42%, and the first year of life by 80.02%, and 77.11% (95% CI 75.91–78.31%) survived to age 15 years. The best prognosis was found in pulmonary stenosis (15-year survival 95.55%), atrial septal defect (92.04%), persistent ductus arteriosus (90.59%), ventricular septal defect (89.37%) and aortic stenosis (88.39%). The worst results were attained in hypoplastic left heart, truncus arteriosus and pulmonary atresia with intact ventricular septum. In conclusion, the prevalence of congenital heart disease was 6.16 per 1000 live births; 77.11% of patients survived to age 15 years.

Abstract: One hundred sixty-one cases of DiGeorge syndrome (111 previously reported in which details concerning individual patients were given and 50 observed) were analyzed for occurrence and type of cardiovascular anomalies. Only 5 patients had a normal heart. Interrupted aortic arch type B was the major anomaly in 48 patients and persistent truncus arteriosus in 37. Therefore, in about half of the patients with DiGeorge syndrome the major anomaly was one that is rare. Conversely, of those patients with interrupted aortic arch, 68% had DiGeorge syndrome, as did 33% of all patients with truncus arteriosus. Although tetralogy of Fallot was also seen often in DiGeorge syndrome (10 patients), these cases represented less than 2% of the total number of cases of tetralogy of Fallot. Similarly, less than 1% of children with isolated ventricular septal defect or transposition of the great arteries had DiGeorge syndrome. The primary cardiovascular anomaly always involved the aortic arch system or the arterial pole of the heart. Recent studies show that neural crest cells play a crucial role in development of pharyngeal (bronchial) pouch derivatives, e.g., thymus and parathyroid glands, as well as the aortic arches and the truncoconal part of the heart. These studies and present observations support the view that DiGeorge syndrome and the associated cardiovascular anomalies are due to an abnormal developmental process involving the neural crest. Curiously, no instances of aortopulmonary septal defect or anomalous origin of a pulmonary artery from the ascending aorta (hemitruncus) have been associated with DiGeorge syndrome.(ABSTRACT TRUNCATED AT 250 WORDS)