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Biomedical subjects

R Van Praagh

Publications and source records attributed to R Van Praagh.

At least 91 records · Page 5Linked to original sources
PubMed1976

Postoperative pathology of congenital heart disease.

Complete and unselected data concerning the postoperative pathology of congenital heart disease are presented for the first time. This study was based on 2,365 autopsies performed at the Children's Hospital Medical Center, Boston, in the 9 years from 1966 through 1974. Of these, 586 autopsies (25 percent) revealed congenital heart disease--238 performed in medically treated patients (41 percent) and 348 in surgically treated patients (59 percent). Tetralogy of Fallot, including cases with pulmonary outflow tract atresia and other associated malformations, was the congenital heart disease most often encountered in the postoperative autopsy series (88 cases, 25 percent of that series). D-transposition of the great arteries, including cases with other associated anomalies, was second (54 cases, 15.5 percent). Early death (hospital mortality) accounted for 320 (92 percent) of the 348 surgical cases; late death occurred in 28 patients (8 percent). Causes of late postoperative death included arrhythmias, excessively small ventricular septal defect with tricuspid atresia, massive hemoptysis, rupture of the pulmonary artery, cyanotic spell, congestive heart failure and infection. Prophylactic penicillin is recommended for patients with the asplenia syndrome because of their probably enhanced vulnerability to fulmfulminating septicemia by encapsulated bacteria such as the pneumococcus. Completeness and lack of selection in reporting data are essential in the interests of perspective and comparability of findings.

Autopsy↗
PubMed1976

Truncus arteriosus communis. Clinical, angiocardiographic, and pathologic findings in 100 patients.

Salient clinical, hemodynamic, angiocardiographic, and pathologic findings are presented in 100 patients with truncus arteriosus communis, 79 of whom were studied at autopsy. In this study of typical truncus, all had a ventricular septal defect (type A). Truncus with a partially formed aorticopulmonary septum (type A1) was much the commonest form (50%). Cases with no remnant of aorticopulmonary septum (type A2) were second in frequency (21%). The distinction between types A1 and A2 could not be made with certainty in 9%, because these types merge into one another. Cases with absence of either pulmonary artery branch (type A3) were the least frequent form (8%). Truncus with interruption, atresia, preductal coarctation, or severe hypoplasia of the aortic arch (type A4) constituted 12%. The diagnosis of truncus is primarily angiocardiographic. The plane of the truncal valve in the lateral projection is distinctive. It tilts anteriorly, facing the patient's toes, which can be of assistance in differential diagnosis. The angiocardiographic features of type A4, although unfamiliar, are pathognomonic. Since the median age at death was only 5 weeks, and in view of the difficulties associated with pulmonary artery banding, our goal should be the surgical correction of truncus during the first and second months of life.

Adolescent↗
PubMed1976

Total anomalous pulmonary venous connection: Report of 93 autopsied cases with emphasis on diagnostic and surgical considerations.

Total anomalous pulmonary venous connection (TAPVC) is failure of development of the common pulmonary vein, with consequent ersistence and enlargement of embryonic collaterals between the lungs and the systemic veins. In the great majority (91 of 93 autopsied cases, 97 per cent, no remnant of the common pulmonary vein was found, supporting the concept of agenesis or involution of the common pulmonary vein. Occasionally, a cordlike strand was found in the location of the common pulmonary vein (in three of 93 cases, 3 per cent), supporting the concept of atresia of the common pulmonry vein. The order of frequency of the various types of TAPVC was: left innominate vein ("snowman"), 26 perent cent; subdiaphragmatic, 24 per cent; coronary sinus, 18 per cent; right superior vena cava, 15 per cent; right atrium, 8 percent; mixed, 5 per cent; azygos, 2 per cent; and left supeior vena cava, 2 per cent...

Adolescent↗
PubMed1976

Tetralogy of Fallot with discrete fibrous subaortic stenosis.

This is the first reported case of discrete fibrous subaortic stenosis in tetralogy of Fallot. Transaortic excision of the subaortic membrane was readily accomplished. To detect this and other forms of left ventricular outflow-tract obstruction in tetralogy of Fallot before surgery, routine selective left ventriculography is advocated.

Adolescent↗
PubMed1975

Double outlet right ventricle (S,D,L) with subaortic ventricular septal defect and pulmonary stenosis. Report of six cases.

The clinical, hemodynamic, angiocardiographic and pathologic findings are presented in an infrequent but surgically correctable type of double outlet right ventricle. This study is based on six cases, one with autopsy confirmation. In all, the viscera and atria were in situs solitus (S). A ventricular d-loop was present (D). There was I-malposition of the great arteries, the aorta being to the left of, and anterior to, the pulmonary artery (L). Hence, this anomaly may conveniently be represented as double outlet right ventricle (S,D,L,). The ventricular septal defect to the ventricular septum. A bilateral conus was present beneath both the aortic and pulmonary valves, preventing any semilunar-atrioventricular fibrous continuity. The subpulmonary conus was poorly expanded, resulting in pulmonary infundibular and valvular (annular) stenosis. The clinical features were those of cyanosis, clubbing and accentuation of the second heart sound in the pulmonary area (related to aortic valve closure). There was a systolic ejection murmur along the upper left sternal border, related to pulmonary outflow tract stenosis. Selective right and left ventricular angiocardiography was diagnostic. Relatively early surgical correction is suggested to minimize the progression of pulmonary infundibular stenosis and to avoid acquired atresia. In this malformation, pulmonary outflow tract reconstruction is more difficult than in tetralogy of Fallot because of the rather posterior location of the pulmonary outflow tract, and because the right coronary artery crosses the stenotic pulmonary outflow tract in front of the pulmonary valve.

Angiocardiography↗
PubMed1975

Anatomically corrected malposition of the great arteries (S, D, L).

Two autopsy-proved cases are presented of a rare form of congenital heart disease, anatomically corrected malposition (S, D, L). Anatomically corrected malposition means that despite the abnormal relationship between the great arteries, the aorta arises nonetheless above the anatomically left ventricle and the pulmonary artery originates above the anatomically right ventricle. (S, D. L) briefly indicates the segmental set or combination: situs solitus of viscera and atria (S), ventricular D-loop (D), AND L-MALPOSITION OF THE GREAT ARTERIES (L). These are the first cases of anatomically corrected malposition (ACM) in whom the presence of a subaortic muscular conus only has been documented pathologically. This established that ACM can occur with a subaortic conus, as well as with a bilateral conus. From the diagnostic standpoint, the distributions or epicardial courses of the coronary arteries are recommended as a helpful approach to ventricular identification. This diagnostic method has widespread angiocardiographic and surgical applications. Symbolic terminology, exemplified by anatomically corrected malposition (S, D. L) is brief and accurate. This approach may conveniently be applied to many other forms of comples congenital heart disease, and it considerably facilitates computer data processing.

Angiocardiography↗