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The conduction system in hearts with pulmonary atresia and intact ventricular septum. 2003

Aneel Ansari, and Diane Goltz, and Karen Patricia McCarthy, and Andrew Cook, and Siew Yen Ho
Department of Paediatrics, Imperial College, Faculty of Medicine, London, United Kingdom.

BACKGROUND Although a regular course of the conduction system is anticipated in hearts with pulmonary atresia and intact ventricular septum (PAIVS), it has never been demonstrated anatomically. In view of one of the surgical options in repair being debulking of the right ventricular wall, it is important to establish the location of the major conduction pathways. METHODS Four hearts belonging to fetuses aged 18, 20, 22, and 29 weeks were examined. Entire hearts were serially sectioned and step sections were stained. The sinus node and atrioventricular conduction bundles were identified and their locations described. RESULTS The conduction system in hearts with PAIVS is similar to that in normal hearts, although there was a minor variation in one of them. This was a heart affected by Ebstein malformation of the tricuspid valve. In this case, the right bundle branch terminated abruptly soon after its origin. CONCLUSIONS Confirmation of the regular course of the cardiac conduction system is important to the development of strategies for surgical repair of PAIVS.

UI MeSH Term Description Entries
D004437 Ebstein Anomaly A congenital heart defect characterized by downward or apical displacement of the TRICUSPID VALVE, usually with the septal and posterior leaflets being attached to the wall of the RIGHT VENTRICLE. It is characterized by a huge RIGHT ATRIUM and a small and less effective right ventricle. Ebstein's Anomaly,Ebstein's Malformation,Familial Ebstein's Anomaly,Anomaly, Ebstein,Anomaly, Ebstein's,Ebstein Malformation,Ebstein's Anomaly, Familial,Ebsteins Anomaly,Ebsteins Malformation,Familial Ebstein Anomaly,Familial Ebsteins Anomaly,Malformation, Ebstein's
D005333 Fetus The unborn young of a viviparous mammal, in the postembryonic period, after the major structures have been outlined. In humans, the unborn young from the end of the eighth week after CONCEPTION until BIRTH, as distinguished from the earlier EMBRYO, MAMMALIAN. Fetal Structures,Fetal Tissue,Fetuses,Mummified Fetus,Retained Fetus,Fetal Structure,Fetal Tissues,Fetus, Mummified,Fetus, Retained,Structure, Fetal,Structures, Fetal,Tissue, Fetal,Tissues, Fetal
D006329 Heart Conduction System An impulse-conducting system composed of modified cardiac muscle, having the power of spontaneous rhythmicity and conduction more highly developed than the rest of the heart. Conduction System, Heart,Conduction Systems, Heart,Heart Conduction Systems,System, Heart Conduction,Systems, Heart Conduction
D006346 Heart Septum This structure includes the thin muscular atrial septum between the two HEART ATRIA, and the thick muscular ventricular septum between the two HEART VENTRICLES. Cardiac Septum,Heart Septa,Septa, Heart,Septum, Cardiac,Septum, Heart
D006352 Heart Ventricles The lower right and left chambers of the heart. The right ventricle pumps venous BLOOD into the LUNGS and the left ventricle pumps oxygenated blood into the systemic arterial circulation. Cardiac Ventricle,Cardiac Ventricles,Heart Ventricle,Left Ventricle,Right Ventricle,Left Ventricles,Right Ventricles,Ventricle, Cardiac,Ventricle, Heart,Ventricle, Left,Ventricle, Right,Ventricles, Cardiac,Ventricles, Heart,Ventricles, Left,Ventricles, Right
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D018633 Pulmonary Atresia A congenital heart defect characterized by the narrowing or complete absence of the opening between the RIGHT VENTRICLE and the PULMONARY ARTERY. Lacking a normal PULMONARY VALVE, unoxygenated blood in the right ventricle can not be effectively pumped into the lung for oxygenation. Clinical features include rapid breathing, CYANOSIS, right ventricle atrophy, and abnormal heart sounds (HEART MURMURS). Atresia, Pulmonary,Pulmonary Valve Atresia,Atresia, Pulmonary Valve,Atresias, Pulmonary,Atresias, Pulmonary Valve,Pulmonary Atresias,Pulmonary Valve Atresias,Valve Atresia, Pulmonary,Valve Atresias, Pulmonary

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