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Biomedical subjects

S Van Praagh

Publications and source records attributed to S Van Praagh.

At least 19 recordsLinked to original sources
PubMed1992

Mitral atresia with a large left ventricle and an underdeveloped or absent right ventricular sinus: clinical profile, anatomic data and surgical considerations.

In mitral atresia with a large left ventricle, the tricuspid valve is either straddling and biventricular or entirely left ventricular. To learn how to assess the identity of the tricuspid valve in such cases 15 heart specimens were examined as well as the echocardiograms of 10 living patients. When the right ventricular sinus was underdeveloped (11 cases), a straddling tricuspid valve was present; when it was absent (14 cases), the tricuspid valve was entirely left ventricular. Regardless of biventricular or exclusively left ventricular attachments, the tricuspid valve was tricommissural (at postmortem examination or on echocardiography) in 22 cases (88%). Its chordal attachments showed considerable variations but were usually paraseptal or on the ventricular septal crest or conal septum. When biventricular, the tricuspid valve straddled through an inlet ventricular septal defect. Clinical or anatomic evidence, or both, of tricuspid regurgitation was present in 14 cases (56%). It is concluded that 1) the identity of the atrioventricular valves is reflected in their chordal attachments more accurately than in their leaflet morphology and depends primarily on the type of ventricular loop present; 2) as a rule, the tricuspid valve is right-sided in D-looped and left-sided in L-looped ventricles; 3) valve identity expressed as the number and position of the papillary muscle attachments is generally recognizable echocardiographically and can be used to diagnose the type of ventricular loop that is present; and 4) the presence and degree of tricuspid regurgitation deserve attention when choosing optimal palliative surgery.

Adult↗
PubMed1991

Tricuspid atresia or severe stenosis with partial common atrioventricular canal: anatomic data, clinical profile and surgical considerations.

The anatomic findings in 11 cases of tricuspid atresia and in two cases of severe tricuspid stenosis, both combined with partial common atrioventricular (AV) canal, are presented in detail. Twelve cases were documented by postmortem examination and the diagnosis was confirmed by echocardiography and surgical observation in the one living patient. Clinical data available in nine cases and cardiac catheterization data obtained in eight are included in this report. In three cases (23%)--two with tricuspid atresia and one with extreme tricuspid stenosis--the tricuspid valve and right ventricle exhibited characteristics seen in Ebstein's anomaly. In all 13 cases, the great arteries were normally related. The ventricular septal defect(s) in 10 (83%) of the 12 postmortem cases rapidly became smaller and this resulted in marked diminution of the pulmonary blood flow and severe hypoxia. Only three of the eight patients with available cardiac catheterization and angiocardiographic data showed the scooped-out appearance of the left ventricular septal surface characteristic of AV canal defects. By contrast, two-dimensional echocardiography, available in the three most recent cases, accurately demonstrated all the defects present and represents the diagnostic method of choice. Early surgical intervention to establish a systemic to pulmonary artery anastomosis is essential for survival. More definitive surgical treatment can be achieved later by an atriopulmonary or cavopulmonary anastomosis with or without replacement of the cleft and often regurgitant mitral valve. The one living patient exemplifies this approach. This is the largest series of this unusual type of tricuspid atresia reported to date.

Cardiac Catheterization↗
PubMed1991

Double-inlet single left ventricle: echocardiographic anatomy with emphasis on the morphology of the atrioventricular valves and ventricular septal defect.

The echocardiographic anatomy of double-inlet single left ventricle was studied in 57 patients, aged 1 day to 27 years (mean 6 years); the variables examined included morphology, size and function of the atrioventricular (AV) valves and ventricular septal defect and their relation to pulmonary stenosis, aortic stenosis and aortic arch obstruction. The visceroatrial situs was solitus and the heart was in the left side of the chest in all 57 patients. A d-loop ventricle was present in 21 patients and an l-loop ventricle in 36. The great arteries were normally related (Holmes heart) in 8 patients and transposed in 49. In all hearts, the right AV valve was anterior to the left AV valve. In 53 patients, the tricuspid valve (right valve in d-loop and left valve in l-loop) was closer to and had attachments on the septum. The tricuspid valve straddled the outflow chamber in eight patients. No significant difference was noted in the mean AV valve diameter when comparing mitral and tricuspid valves within the same group or between the groups with a d- or l-loop ventricle. The right AV valve diameter had a significant direct correlation with the aortic valve diameter and the size of the ventricular septal defect regardless of ventricular loop. Both AV valves were functionally normal in 34 patients. Among patients with AV valve dysfunction, the tricuspid valve tended to be stenotic in patients with an l-loop ventricle and regurgitant in patients with a d-loop ventricle. Mitral valve dysfunction was uncommon. The ventricular septal defect (46 patients) was separated from the semilunar valves in 24 patients (muscular defect) and adjacent to the anterior semilunar valve as a result of hypoplasia or malalignment, or both, of the infundibular septum (subaortic defect) in 19 patients. Multiple defects were present in three patients. The defect was unrestrictive in 26 patients, restrictive in 23 and could not be evaluated in 8. Pulmonary artery banding had been performed in 8 of the 26 patients with an unrestrictive defect and in 10 of the 23 patients with a restrictive defect. Only 4 of 19 subaortic defects compared with 16 of 24 muscular defects were restrictive. The size of the defect was significantly correlated with the measured pressure gradient. Among patients with transposition, only 2 of 13 with pulmonary stenosis had a restrictive ventricular septal defect compared with 15 of 30 without pulmonary stenosis. In patients with transposition, the defect size was significantly smaller when coarctation was present.

Child↗
PubMed1991

Straddling mitral valve with hypoplastic right ventricle, crisscross atrioventricular relations, double outlet right ventricle and dextrocardia: morphologic, diagnostic and surgical considerations.

The clinical, surgical and morphologic findings in five cases of a rare form of straddling mitral valve are presented. Three patients were diagnosed by two-dimensional echocardiography, cardiac catheterization and angiocardiography and two had diagnostic confirmation at autopsy. All five cases shared a distinctive and consistent combination of anomalies: 1) dextrocardia; 2) visceroatrial situs solitus, concordant ventricular D-loop and double outlet right ventricle with the aorta positioned to the left of and anterior to the pulmonary artery; 3) hypoplasia of right ventricular inflow (sinus) with tricuspid valve stenosis or hypoplasia; 4) large right ventricular infundibulum (outflow); 5) malalignment conoventricular septal defect; 6) straddling mitral valve with chordal attachments to the left ventricle and right ventricular infundibulum; 7) severe subpulmonary stenosis with well developed pulmonary arteries; and 8) superoinferior ventricles with crisscross atrioventricular (AV) relations. The degree of malalignment between the atrial and ventricular septa was studied quantitatively by measuring the AV septal angle projected on the frontal plane. The AV septal angle in the two postmortem cases was 150 degrees, reflecting marked malalignment of the ventricles relative to the atria. This AV malalignment appears to play an important role in the morphogenesis of straddling mitral valve. As judged by a companion study of seven postmortem cases, the more common form of straddling mitral valve with a hypertrophied and enlarged right ventricular sinus had less severe ventricular malposition than did the five rare study cases with hypoplastic right ventricular sinus. A competent mitral valve, low pulmonary vascular resistance and low left ventricular end-diastolic pressure were found at cardiac catheterization in the three living patients who underwent a modified Fontan procedure and are doing well 2.2 to 5.8 years postoperatively.

Adult↗
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↗