Biomedical subjects

R Van Praagh

Publications and source records attributed to R Van Praagh.

At least 19 recordsLinked to original sources

PubMed2003

Superoinferior ventricles with superior left ventricle and inferior right ventricle: a newly recognized form of congenital heart disease.

We report the first known case of supero-inferior ventricles with a superior morphologically left ventricle and an inferior morphologically right ventricle. This 2 1/2-year-old boy also had dextrocardia, double-outlet right ventricle [S,L,L], right-sided mitral atresia, left-sided tricuspid regurgitation, a large conoventricular type of ventricular septal defect, and pulmonary outflow tract stenosis. This very rare form of superoinferior ventricles appears to be due to excessive levorotation (approximately equal to 170 degrees) of discordant L-loop ventricles.

Child, Preschool↗

PubMed2002

Biatrial or left atrial drainage of the right superior vena cava: anatomic, morphogenetic, and surgical considerations--report of three new cases and literature review.

Since the posterior wall of the right superior vena cava (RSVC) is contiguous with the anterior wall of the right upper pulmonary veins, a localized defect in this common wall may create a cavopulmonary venous confluence without eliminating the normal connection of the same right pulmonary veins with the left atrium (LA). Through this defect, blood of the unroofed right pulmonary veins will drain into the RSVC and right atrium (RA), and blood from the RSVC may shunt into the right pulmonary veins and LA. Hemodynamically, the RSVC will become biatrial. If the RSVC blood flows preferentially into the LA, its right atrial orifice will become stenotic or even atretic. If atretic, the normally positioned RSVC will drain entirely into the LA. In this report, we present the clinical and anatomical findings of two postmortem cases with biatrial drainage of the RSVC. We also document the clinical, echocardiographic, angiocardiographic, and surgical data of a living patient with left atrial drainage of the RSVC and tetralogy of Fallot with pulmonary atresia. The relevant literature and surgical treatment are reviewed, and the morphogenesis of the biatrial and left atrial RSVC is considered.

Abnormalities, Multiple↗

PubMed2002

Single origin of right and left pulmonary artery branches from ascending aorta with nonbranching main pulmonary artery: relevance to a new understanding of truncus arteriosus.

We report the third known case of origin of the right and left pulmonary artery branches from the ascending aorta via a short common pulmonary artery. A large unbranching main pulmonary artery opened through a patent ductus arteriosus into the descending thoracic aorta. Preductal coarctation of the aorta and multiple congenital anomalies were also present. This rare cardiovascular malformation facilitates a new anatomic and developmental understanding of truncus arteriosus.

Aorta↗

PubMed2001

Healing response to the Clamshell device for closure of intracardiac defects in humans.

The healing response to intracardiac devices in humans is largely unknown. During regulatory trials using the Clamshell device in over 800 patients, attempts were made to perform histopathological evaluation of all explanted devices. We reviewed all those with complete histopathological examination (n = 12) from Fontan baffles (n = 4), ventricular septal defects (n = 2), and atrial septal defects (ASD; n = 6), explanted at 2.7 months to 3.6 years (median, 1.6 years), at autopsy (n = 1) or surgery (n = 11), performed for residual defects (n = 5), atrial masses (n = 3), or Fontan revision (n = 3). All but one were nearly (n = 3) or completely (n = 8) covered by pseudointima, composed of fibroelastic tissue, predominantly collagen, with focal foreign body reaction in contact with fabric, without acute inflammation or infection. Atrial masses of granulation tissue were present in three cases (ASD), opposite to protruding fractured arms. No associations were identified between coverage and closure status, position, arm fractures, or implant period. In conclusion, the healing response to transcatheter Clamshell implantation in humans is characterized by a relatively rapid development of a nonthrombotic pseudointima composed of fibroelastic tissue with minimal foreign body reaction. Cathet Cardiovasc Intervent 2001;54:101-111.

Adolescent↗

PubMed2001

Three-dimensional echocardiography of the atrial septum.

This article details the important contribution of three-dimensional echocardiography for catheterization device closure of secundum atrial septal defects. Aspects presented include three-dimensional echocardiographic application in preselection of patients and in selection of the type and size of the atrial septal occluder devices. Unique three-dimensional echocardiographic imaging planes are shown that depict the size and shape of the defect, the important rim tissue surrounding the defect, and the images that demonstrate successful device placement. Details of the acquisition phase, digital reformatting, and the eventual rendering of standard three-dimensional echocardiographic imaging planes of the atrial septum are shown. Three-dimensional echocardiography not only provides important additional information, but also enhances understanding of standard two-dimensional studies.

Cardiac Catheterization↗

PubMed2001

Congenital left ventricular aneurysm: clinical, imaging, pathologic, and surgical findings in seven new cases.

BACKGROUND: Congenital left ventricular aneurysm is a poorly understood and potentially lethal entity. Methods and Results In a clinicopathologic study of 7 new cases, the major presenting features in 6 patients were congestive heart failure in 4, ventricular arrhythmias in a 32-week fetus, and multiple congenital anomalies in a fetus with trisomy 13. Accurate diagnosis was achieved in all 3 living patients by echocardiography, angiocardiography, and magnetic resonance imaging. The aneurysm was predominantly apical in 3 and involved most of the left ventricular free wall in 4. Of the 3 living patients, medical management alone sufficed in 2. The third, a newborn boy, underwent a new and successful aneurysm-exclusion left ventriculoplasty. The mitral valve was abnormal in all 4 autopsied cases, the papillary muscles being short, thin, or absent. The aneurysm was thinner and its area was larger than that of the nonaneurysmal left ventricle in all necropsied patients. CONCLUSIONS: Congenital left ventricular aneurysm appears to be a developmental anomaly, an idiopathic dysplasia of left ventricular endocardium and myocardium. No evidence of a viral etiology was found. Some neonates can be managed medically, but others require urgent surgical intervention. A new surgical operation is presented, a functional left ventricular aneurysmectomy that minimizes intraoperative and postoperative blood loss and that preserves the coronary arteries.

Female↗

PubMed2000

Extracardiac anomalies in the heterotaxy syndromes with focus on anomalies of midline-associated structures.

The extracardiac defects in patients with heterotaxy have not been examined as extensively as cardiac defects. We found a high incidence of midline-associated defects in 160 autopsied cases of heterotaxy (asplenia, polysplenia, or single right-sided spleen). Fifty-two percent of patients with left-sided polysplenia had a midline-associated defect, as did 45% of those with asplenia. Most common were musculoskeletal or genitourinary anomalies, as well as cleft palate. Fused adrenal glands and anal stenosis or atresia occurred exclusively among patients with asplenia. A midline anomaly was twice as likely to be detected on complete autopsy than from clinical findings alone. Linkage studies should take into account that affected subjects may have isolated subclinical midline defects. The high incidence of midline-associated defects supports the theory that the midline plays a critical role in establishing left-right asymmetry in the body. Comparison of these defects with mouse models of laterality defects suggests that mutations that disrupt the transforming growth factor beta pathway may result in heterotaxy.

Abnormalities, Multiple↗

PubMed2000

Acute animal studies of the STARFlex system: a new self-centering cardioSEAL septal occluder.

The STARFlex system is a modified CardioSEAL device with a flexible self-centering mechanism comprised of nitinol springs strung between opposing arms, a connecting ball (sleeve joint that allows the device to pivot prerelease), and a front-loading delivery system. It was designed to allow a smaller device/defect sizing ratio and delivery profile, provide centering capability, and improve closure rates. To test this system, 13 devices (23, 28, and 33 mm) were deployed in six sheep within created atrial septal defects (12- to 22-mm diameter; n = 10), in the left atrium (n = 2), and in inferior vena cava (n = 1). All implantations in atrial septal defects were successful, with device/defect ratio ranging from 1.3 to 1.9 (median, 1.3), with no residual leak by angiography or echocardiography in seven (3/10 had </= small immediate leaks). The STARFlex system was effective in closing created atrial septal defects using a 10 Fr delivery sheath and low device/defect sizing ratios, comparing favorably with the standard CardioSEAL. Cathet. Cardiovasc. Intervent. 49:225-233, 2000.

Animals↗

PubMed2000

Surgical closure of apical ventricular septal defects through a right ventricular apical infundibulotomy.

BACKGROUND: We present a new understanding of the anatomic position of apical ventricular septal defects and its surgical relevance. These defects occur between the left ventricular apex and the infundibular apex, rather than between the left and right ventricular apices. Often a sizable apical recess, the infundibular apex lies anteriorly and inferiorly to the moderator band and is the most leftward part of the right ventricle. METHODS: Four patients (2 boys and 2 girls) with a mean age of 109 days (range, 48 to 217 days) underwent patch closure through an apical infundibulotomy, which allowed complete visualization of the muscular apical ventricular septal defect. RESULTS: There were no early or late deaths at operation. No significant residual shunt at ventricular level was detected by postoperative two-dimensional and Doppler echocardiography. Intraoperative comparison of right atrial and pulmonary arterial blood samples showed a difference of less than 5%. At a mean follow-up of 18 months, all the patients are asymptomatic and growing well. CONCLUSIONS: The successful outcome of these 4 patients indicates that surgical closure of apical ventricular septal defects can be achieved safely and completely in early infancy through a limited right ventricular apical infundibulotomy. Long-term follow-up of these and similar patients is needed to provide further evaluation of this approach.

Cardiac Surgical Procedures↗

PubMed1999

Truncus arteriosus with anomalous pulmonary venous connection.

The anatomic, diagnostic, and management findings of 6 patients with truncus arteriosus and anomalous pulmonary venous connections are described. Additional risk factors indicative of poor prognosis were found in 3 of 4 patients with truncus arteriosus and totally anomalous pulmonary venous connection and in 1 patient with partially anomalous pulmonary venous connection.

Cause of Death↗

PubMed1999

Straddling tricuspid valve as a sign of ventriculoatrial malalignment: A morphometric study of 19 postmortem cases.

BACKGROUND: Straddling tricuspid valve, despite extensive investigation, remains an incompletely understood form of complex congenital heart disease. METHODS: A morphometric study of 19 postmortem cases of straddling tricuspid valve was performed, and the results were compared with 32 normal control heart specimens. RESULTS: In straddling tricuspid valve, marked malalignment of the ventricles was always found relative to the atria. The angle between the ventricular septum and the atrial septum in the short-axis projection averaged 61 degrees +/- 24 degrees, the normal ventriculoatrial septal angle averaging 5 degrees +/- 2 degrees (P <. 001). The right ventricular sinus (inflow tract) was significantly smaller than the left (P <.01). A ventricular septal defect was present in 79%: atrioventricular canal type in 42%, atrioventricular canal type confluent with a conoventricular defect in 26%, and a conoventricular defect in 11%. When the straddling tricuspid valve adhered to the crest of the muscular ventricular septum (n = 4 cases, 21%), the 2 salient findings were (1) an intact ventricular septum and (2) double-outlet right atrium. The nonstraddling part of the tricuspid valve opened into the small right ventricle. The straddling part of the tricuspid valve opened into the larger left ventricle. The mitral valve also opened into the left ventricle. Hence hearts with double-outlet right atrium had 3 atrioventricular valves. Congenital mitral stenosis was present in 26% of this series. CONCLUSION: Straddling tricuspid valve was always characterized by marked ventriculoatrial malalignment, indicated by an abnormally large ventriculoatrial septal angle, best seen in the short-axis projection.

Cadaver↗

PubMed1999

The modified Fontan procedure: morphometry and surgical implications.

BACKGROUND: The modified Fontan procedure for patients with only one well-formed ventricle is now widely regarded as palliative, not curative. METHODS: To improve the surgical management and postoperative follow-up of such patients, a morphometric study of 33 postmortem cases was done. RESULTS: The three main causes of death were congestive heart failure (82%), arrhythmias (12%), and central nervous system dysfunction (6%). The cross-sectional area of the Fontan anastomosis (FA) relative to the systemic venous area (SVA) and relative to the body surface area (BSA) revealed that the Fontan pathway was often obstructive. The mean FA/SVA index was 73% less than normal: 0.54 +/- 0.22, range 0.13 to 0.98. The mean FA/BSA index was 70% less than normal: 143.52 +/- 50.01 mm2/M2, range 55.09 to 261.67 mm2/M2. CONCLUSIONS: The main surgical challenge is to minimize or eliminate prepulmonary stenosis. Although significant postoperative obstruction was often not evident hemodynamically because of small or absent gradients, the presence of important obstruction of the Fontan pathway was clearly revealed by morphometry.

Adolescent↗

PubMed1998

Interrupted inferior vena cava in asplenia syndrome and a review of the hereditary patterns of visceral situs abnormalities.

We present the clinical and postmortem findings of the first photographically documented case of asplenia and interrupted inferior vena cava and the anatomic findings of 5 previously reported cases. A brief review of the various hereditary patterns of visceral situs abnormalities suggests that, at least in some cases, the asplenia and polysplenia syndromes are etiologically and pathogenetically interrelated.

Abnormalities, Multiple↗

PubMed1998

Pathologic anatomy of corrected transposition of the great arteries: medical and surgical implications.

BACKGROUND: Because the double-switch operation (atrial switch plus arterial switch) has recently become feasible in selected patients with congenitally physiologically corrected transposition of the great arteries, a detailed understanding of the pathologic anatomy is now mandatory for cardiologists, radiologists, and surgeons. METHODS: A detailed study of the pathologic anatomy, the clinical implications, and the surgical implications was undertaken on 33 postmortem cases with two ventricles. A companion study was also performed of 44 postmortem cases with functionally only one ventricle. Hence this was an investigation of 77 postmortem cases. RESULTS: Three main anatomic types of corrected transposition of the great arteries (TGA) with two ventricles were found: (1) TGA with solitus atria (S), L-loop ventricles (L), and L-TGA (L), that is, TGA [S,L,L] in 31 cases (94%); (2) TGA with solitus atria (S), L-loop ventricles (L), and D-TGA (D), that is, TGA [S,L,D] in 1 case (3%); and (3) TGA with inverted atria (I), D-loop ventricles (D), and D-TGA (D), that is, TGA [I,D,D] in 1 case (3%). Associated malformations resulted in 13 anatomic subtypes. In classical corrected TGA [S,L,L] with two ventricles, anomalies of the left-sided systemic tricuspid valve were present in 97%, with malformations of the left-sided systemic right ventricle in 91%. CONCLUSIONS: The findings in corrected TGA with two ventricles and in cases with single ventricle support the view that anatomic repair such as the double-switch procedure, or left-sided right ventricle bypass such as the modified Norwood procedure followed by the modified Fontan procedure, is indicated in selected patients.

Adolescent↗

PubMed1998

The large window ductus: a surgical trap.

A rare window type of patent ductus arteriosus is reported that was large (15 mm in maximal transverse dimension) but had virtually no length and hence was externally invisible. The smaller aortic isthmus (4 mm in diameter), which was intrapericardial, was mistaken for the ductus and was inadvertently clip-occluded, leading to death. After a specific diagnosis is made, the large window ductus should be patched on cardiopulmonary bypass with a transpulmonary approach.

Aorta, Thoracic↗

PubMed1997

Patch repair of subsemilunar conal septal defect resulting in severe left ventricular outflow tract obstruction.

A subsemilunar conal septal defect was closed at 8 months of age with a redundant Dacron patch that bowed into the left ventricular outflow tract, resulting in severe subaortic stenosis and massive left ventricular hypertrophy. Mistaken for cardiomyopathy or myocarditis, this rare complication of subsemilunar ventricular septal defect patch closure led to orthotopic cardiac transplantation followed by death.

Abnormalities, Multiple↗

PubMed1997

Absent right superior vena cava in visceroatrial situs solitus.

Absence of the right superior vena cava (SVC) in visceroatrial situs solitus is rare (0.07% to 0.13% of congenital cardiovascular malformations), and little is known about the type and frequency of additional heart defects and arrhythmias. We reviewed previous publications and present 9 new cases. Based on 121 known cases, we found that this anomaly is typically characterized by: (1) persistence of the left SVC draining into the right atrium by way of the coronary sinus, and (2) left-sided azygos vein draining into the left SVC. Less constant features were: (3) additional cardiovascular malformations (46%), and (4) rhythm abnormalities (36%) that usually appeared related to the complications of old age. Since absence of the right SVC is clinically silent, its status should be assessed echocardiographically prior to invasive medical or surgical procedures. This is important to avoid various management difficulties during the following procedures: (1) implantation of a transvenous pacemaker, (2) placement of a pulmonary artery catheter for intraoperative or intensive care unit monitoring without fluoroscopy, (3) systemic venous cannulation for extracorporeal membrane oxygenation, (4) systemic venous cannulation for cardiopulmonary bypass, (5) partial or total cavopulmonary anastomoses; and (6) orthotopic heart transplantation and endomyocardial biopsies.

Abnormalities, Multiple↗

PubMed1997

Aortic outflow obstruction in visceral heterotaxy: a study based on twenty postmortem cases.

Aortic outflow tract obstruction can complicate the clinical course and surgical management of patients with heterotaxy syndromes, but its anatomic basis has not been described in detail. In 20 postmortem cases with asplenia (n = 4) or polysplenia (n = 16), the anatomic causes of aortic outflow tract obstruction were absence of the subaortic conus in association with (1) narrowing of the subaortic outflow tract between the conal septum anteriorly and the common atrioventricular (AV) valve posteriorly in six (30%) patients; (2) aortic valvar atresia in four (25%), three with asplenia and one with polysplenia; (3) redundant AV valve leaflets in four (20%); (4) excessive AV valve fibrous tissue in four (20%); (5) marked hypoplasia of the mitral valve and left ventricle in two (10%); and (6) aneurysm of membranous septum in one (5%). One patient belonged to group (1) and (4). Aortic outflow tract obstruction was much more common with polysplenia (28%) than with asplenia (4%) (p < 0.001).

Abnormalities, Multiple↗