PubMed Health⌕ Search

Biomedical subjects

S Van Praagh

Publications and source records attributed to S Van Praagh.

At least 37 records · Page 2Linked to original sources
PubMed1989

Cardiac malformations in trisomy-18: a study of 41 postmortem cases.

The cardiac malformations in 41 karyotyped and autopsy cases of trisomy-18 are presented in detail. The salient findings were a ventricular septal defect in all cases; tricuspid valve anomalies in 33 cases (80%); pulmonary valve anomalies in 30 (70%); aortic valve malformations in 28 (68%); mitral valve anomalies in 27 (66%); polyvalvular disease (that is, malformations of more than one valve) in 38 (93%); a subpulmonary infundibulum (conus) in 40 (98%); a bilateral conus with a short subaortic infundibulum in 1 case with double outlet right ventricle (this being the only documented case of bilateral infundibulum in trisomy-18); double outlet right ventricle in 4 cases (10%), three having a subpulmonary infundibulum only and all 4 having mitral atresia; tetralogy of Fallot in 6 cases (15%), 2 having pulmonary atresia; and a striking absence of transposition of the great arteries and inversion at any level (visceral or cardiac), findings that appear to be characteristic of all trisomies. These data suggest that excessive chromosomal material (as in trisomies) may result in situs solitus at all levels. The malformations of the atrioventricular and semilunar valves were characterized by redundant or thick myxomatous leaflets, long chordae tendineae and hypoplastic or absent papillary muscles. The ventricular septal defect was associated with anterosuperior conal septal malalignment in 25 cases (61%). On the basis of the characteristic valvular lesions, the type of ventricular septal defect and the absence of transposition or inversions, two-dimensional echocardiographic diagnosis of trisomy-18 in the fetus may become possible.

Chromosomes, Human, Pair 18↗
PubMed1989

Transcatheter closure of atrial septal defects. Experimental studies.

Recent experience with the spring-loaded patent ductus arteriosus (PDA) occluder has demonstrated several advantages of this device in the closure of intracardiac defects compared with previously described techniques. Pathologic and animal model studies were performed to identify which atrial septal defects (ASDs) might be suitable for device closure and to test a new septal closure double umbrella. Fifty specimens from the Cardiac Registry with unrepaired ASD secunda (2 degrees) were analyzed. Mean ASD size was 8 x 10 mm (range, 3 x 4 to 30 x 30 mm); 80% (n = 40) of ASDs were judged closable with the new device. ASD closure was attempted in four lambs with the Rashkind (hooked single umbrella) ASD occluder. One of four umbrellas was correctly positioned; no ASDs were closed. A new double-hinged ("clamshell") umbrella device was designed: eight ASD closures were attempted with this device (defects ranged from 8 to 20 mm in diameter). Six of eight umbrellas were correctly positioned; four of four animals observed more than 1 day appeared to have complete closure on postmortem examination with endothelialization of the device. We conclude that 1) most ASD 2 degrees are far enough from vital structures to permit closure, 2) initial placement of hooked umbrellas is often incorrect and cannot be altered, 3) clamshell double umbrellas were successfully placed in six of eight attempts, and 4) endothelialization of closed ASDs appears complete within weeks of implantation. These preliminary studies appear promising and support testing the clamshell ASD device in clinical trials.

Animals↗
PubMed1989

Transposition of the great arteries with straddling tricuspid valve. Report of two rare cases with acquired subaortic stenosis after main pulmonary artery banding.

The clinical, hemodynamic, angiocardiographic, and postmortem findings of a previously unreported type of subaortic stenosis are presented in two patients who also had straddling tricuspid valve and transposition of the great arteries. The subaortic stenosis became apparent after banding of the main pulmonary artery and was due to hypertrophy of a probably abnormally positioned moderator band resulting in a double-chambered right ventricle. Fibrous tissue accumulation at the stenotic os infundibuli also contributed to the subaortic obstruction. Both patients had situs solitus of the atria. Patient 1 had ventricular inversion (L-loop ventricles) and atresia of the right-sided mitral valve. Patient 2 had normally positioned ventricles (D-loop) and two atrioventricular valves. The presence of a large left ventricle and a small right ventricle in the angiocardiogram led to the erroneous diagnosis of a single left ventricle with an infundibular outlet chamber in both patients. Consequently, the subaortic obstruction was thought preoperatively to be at the site of a restrictive bulboventricular foramen. Patient 1 died 36 hours after placement of a valved conduit from the left ventricle to the descending aorta. Patient 2 was operated on successfully and the surgical procedures performed are described.

Aortic Valve Stenosis↗
PubMed1988

Echocardiographic and anatomic findings in atrioventricular discordance with ventriculoarterial concordance.

Atrioventricular (AV) discordance with ventriculoarterial (VA) concordance is a rare form of congenital heart disease that consists of 5 different anatomic types. The salient therapeutic consideration uniting these 5 different anatomic entities is that anatomic correction can be achieved by an atrial switch procedure with closure of any associated septal defect. Three patients who had AV discordance with VA concordance are presented, with emphasis upon 2-dimensional echocardiographic diagnosis and surgical management. One of these patients had ventricular inversion with inverted normally related great arteries in situs solitus (i.e., [S,L,I]), thus representing a form of congenital heart disease that until now has not been documented anatomically. Although these are complex defects, multiple-plane imaging with 2-dimensional echocardiography was found to be extremely reliable in ascertaining the anatomy when a comprehensive, segmental analysis is applied. This approach includes determination of visceroatrial situs, ventricular loop and great arterial position and alignment. A surgical approach to closure of the ventricular septal defect through the left-sided infundibulum in isolated ventricular inversion afforded excellent exposure of the defect and avoided AV conduction block.

Aorta↗
PubMed1988

Double-orifice mitral valve: a study of 27 postmortem cases with developmental, diagnostic and surgical considerations.

What is double-orifice mitral valve anatomically and embryologically? In 27 postmortem cases, an anomaly of the tensor apparatus was always found. These malformations may be summarized as: (1) chordal ring; (2) accessory papillary muscle or muscles; (3) subdividing muscular ridge; (4) fused papillary muscles (parachute mitral valve); (5) crossing chordae tendineae; and (6) central fibrous subdivision. More than 1 of these anomalies often coexisted per case. Double-orifice mitral valve almost always consisted of abnormal holes in essentially normal leaflets, rather than of abnormal fibrous bridges or adhesions between normal leaflets. Since these fibrous "bridges" between the smaller accessory orifice and the larger main orifice are composed of mitral leaflet tissue and chordae, not fibrous adhesions, these bridges should not be transected surgically, to avoid iatrogenic mitral regurgitation. The accessory (smaller) orifice was at the anterolateral commissure in 11 cases (41%), at the posteromedial commissure in 12 (44%) and there was a central fibrous subdivision with approximately equal-sized orifices in 4 (15%). The atrioventricular (AV) canal was normally divided in 12 cases (44%) and a common AV canal was present in 15 (56%). When the accessory orifice was at the anterolateral commissure, the AV canal usually was normally divided (8 of 11, 73%). But when the accessory orifice was at the posteromedial commissure, a common AV canal almost always was present (11 of 12, 92%). Functionally, the mitral valve was normal in 13 (48%), regurgitated in 7 (26%) and stenotic in 7 (26%). The key to the diagnostic and surgical understanding of the double-orifice mitral valve is the underlying tensor apparatus.

Humans↗
PubMed1983

Aristotle's "triventricular" heart and the relevant early history of the cardiovascular system.

Aristotle said that the human heart has three ventricles--right, left, and middle--a concept that has often been viewed as an astonishing error. But was it? Aristotle did not miscount ventricles. In the third century BC, all cardiac chambers were called "ventricles," meaning "cavities." The "ears" (auricles) were distinguished from the "cavities" (ventricles) by Herophilus of Alexandria (c 300 BC) and by Rufus and Ephesus (a contemporary of Jesus Christ). Aristotle regarded the right atrium as a venous dilatation, not as a part of the heart. Aristotle's "right ventricle" was our right ventricle. His "left ventricle" was our left atrium. His "middle ventricle" was our left ventricle. Because he did not count the right atrium, Aristotle considered the human heart to be three-chambered or "triventricular," consisting of the right ventricle, the left atrium, and the left ventricle. This report summarizes the relevant early history of the cardiovascular system.

Anatomy↗
PubMed1982

What is a ventricle? The single-ventricle trap.

Whether a chamber is or is not a ventricle is determined by its myocardial morphological characteristics, not by the entering atrioventricular (AV) valves. The anatomic characteristics of the morphologically right ventricle and the morphologically left ventricle have been well described, are widely understood, and do not need to be changed. What a ventricle is should not be redefined in terms of the AV connections because such redefinition is based on the classic definition of single ventricle, which itself is not satisfactory. This old definition is wrong in principle, violates the morphological method of diagnosis and designation of the cardiac principle, violates the morphological method of diagnosis and designation of the cardiac chambers, and has two kinds of exceptions. Consequently the classic definition of single ventricle must not be used as a paradigm (model) for the redefinition of what a ventricle is or is not. The approach and terminology based on the use of the unsatisfactory single-ventricle is or is not. The approach and terminology based on the use of the unsatisfactory single-ventricle paradigm approach and terminology based on the use of the unsatisfactory single-ventricle paradigm should be discontinued, eg, "primitive ventricle," "main chamber," "accessory chamber," "trabecular pouch." Being unnecessary, these nonmorphological components of the terminology of congenital heart disease may be omitted. Morphological anatomy, unadorned, is the key to clarity, simplicity, and accuracy.

Heart↗
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↗