Incomplete atrioventricular septal defect with hypoplastic left ventricle and left atrioventricular valve stenosis. 2001

M Nishimura, and M Yamagishi, and K Fujiwara, and M Yoshida, and N Kitamura
Department of Pediatric Cardiovascular Surgery, Children's Research Hospital, Kyoto Prefectural University of Medicine, Kawaramachi, Hirokoji, Kamigyo-ku, Kyoto 602-8566, Japan.

A 2-month-old male infant with incomplete atrioventricular septal defect associated with a hypoplastic left ventricle and left atrioventricular valve stenosis successfully underwent biventricular repair. Echocardiography showed marked dilatation in the right ventricle and a diminutive left ventricle. However, the left ventricular chamber occupied the apex. Left atrioventricular valve stenosis was due to a solitary papillary muscle. Cardiac catheterization showed pulmonary/systemic flow ratio of 3.61, left ventricular end-diastolic volume of 63% of normal, and right ventricular end-diastolic volume of 324% of normal. During surgical repair, the solitary papillary muscle was divided longitudinally and the ostium primum was closed with a bovine pericardium. Postoperative left ventricular function was appropriate. Even when the preoperative end-diastolic left ventricular volume is small, if the left ventricle chamber is at the apex, then the left ventricular performance can be expected to be appropriate to tolerate the volume load after ostium primum closure.

UI MeSH Term Description Entries
D007223 Infant A child between 1 and 23 months of age. Infants
D008297 Male Males
D006344 Heart Septal Defects, Atrial Developmental abnormalities in any portion of the ATRIAL SEPTUM resulting in abnormal communications between the two upper chambers of the heart. Classification of atrial septal defects is based on location of the communication and types of incomplete fusion of atrial septa with the ENDOCARDIAL CUSHIONS in the fetal heart. They include ostium primum, ostium secundum, sinus venosus, and coronary sinus defects. Atrial Septal Defects,Ostium Primum Atrial Septal Defect,Persistent Ostium Primum,Atrial Septal Defect,Atrial Septal Defect Ostium Primum,Ostium Secundum Atrial Septal Defect,Defect, Atrial Septal,Ostium Primum, Persistent,Primum, Persistent Ostium,Septal Defect, Atrial
D006345 Heart Septal Defects, Ventricular Developmental abnormalities in any portion of the VENTRICULAR SEPTUM resulting in abnormal communications between the two lower chambers of the heart. Classification of ventricular septal defects is based on location of the communication, such as perimembranous, inlet, outlet (infundibular), central muscular, marginal muscular, or apical muscular defect. Ventricular Septal Defects,Intraventricular Septal Defects,Ventricular Septal Defect,Defect, Intraventricular Septal,Defect, Ventricular Septal,Defects, Intraventricular Septal,Intraventricular Septal Defect,Septal Defect, Intraventricular,Septal Defect, Ventricular,Septal Defects, Intraventricular,Septal Defects, Ventricular
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
D014264 Tricuspid Valve Stenosis The pathologic narrowing of the orifice of the TRICUSPID VALVE. This hinders the emptying of RIGHT ATRIUM leading to elevated right atrial pressure and systemic venous congestion. Tricuspid valve stenosis is almost always due to RHEUMATIC FEVER. Stenoses, Tricuspid Valve,Stenosis, Tricuspid Valve,Tricuspid Valve Stenoses,Valve Stenoses, Tricuspid,Valve Stenosis, Tricuspid
D016277 Ventricular Function, Left The hemodynamic and electrophysiological action of the left HEART VENTRICLE. Its measurement is an important aspect of the clinical evaluation of patients with heart disease to determine the effects of the disease on cardiac performance. Left Ventricular Function,Function, Left Ventricular,Functions, Left Ventricular,Left Ventricular Functions,Ventricular Functions, Left

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