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Richard Van Praagh
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ABSTRACT: Stella Van Praagh, MD (1927-2006) of Children's Hospital Boston was one of the greatest pediatric cardiologists and pediatric cardiac pathologists of the 20th and early 21st centuries. Née Stella Zacharioudaki from Crete, Greece, in addition to her stellar professional attainments, she was also an outstanding cuisinière, hostess, linguist, philosopher, and philanthropist. In 1962, she married Richard Van Praagh, MD, beginning a life-long collaboration that was in every sense an affaire de coeur. They had three children and seven grandchildren. Dr Stella was the author of more than 110 scientific publications which helped to clarify the pathologic anatomy, the clinical and laboratory diagnosis, and often the surgical management of many different forms of congenital heart disease, including dextrocardia, single ventricle, truncus arteriosus, tetralogy of Fallot (TOF), transposition of the great arteries, double-outlet right ventricle, sinus venosus defect, anomalous pulmonary venous drainage, the heterotaxy syndromes with asplenia or polysplenia, juxtaposition of the atrial appendages, and apical muscular ventricular septal defect. In 1999, Dr Stella Van Praagh received the Distinguished Achievement Award of the Society for Cardiovascular Pathology, and in 2004, she was honored with the Paul Dudley White Award of the American Heart Association. Dr. Stella Van Praagh was that vanishingly rare combination of brilliant clinician, internationally renowned medical scientist, and deeply cultivated humanist. The anomaly now known as the TOF was first described by Niels Stensen in 1671, with other early reports by Edouard Sandifort (1777), William Hunter (1784), and many others. In 1888, Etienne-Louis Arthur Fallot published five serialized contributions in Marseille Médical concerning what he called the "blue malady," in which he described the now classical tetralogy of pulmonary outflow tract obstruction, ventricular septal defect, aortic overriding, and right ventricular hypertrophy. The other outstanding feature of Fallot's report was its emphasis on clinicopathologic correlation. In 1924, Maude Abbott coined the term "tetralogy of Fallot." In 1970, Van Praagh and colleagues presented the concept that the TOF is basically just one anomaly, a failure of normal expansile growth of the subpulmonary infundibulum and its sequelae. The anatomy of TOF is presented angiocardiographically, diagrammatically, and anatomically. A morphometric study of typical neonatal TOF is presented, based on 16 autopsied heart specimens with age-matched normal controls. The morphometric study documents that TOF is characterized by a low-volume subpulmonary infundibulum. The diagnostic and surgical significance of these findings is highlighted. Two rare and recently discovered forms of TOF are presented: tetralogy {S,D,I}, and tetralogy {I,D,S}. Because tetralogy {I,D,S} has atrioventricular discordance, in addition to a standard TOF repair, such patients also need an inverted (mirror-image) atrial switch operation (inverted Senning or inverted Mustard procedure). Because associated malformations can be very important to the surgical outcome of patients with tetralogy, the associated anomalies found in 100 randomly selected autopsied cases are presented.
Pediatric Cardiac Surgery Annual of the Seminars in Thoracic and Cardiovascular Surgery 02/2009;
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Journal of Thoracic and Cardiovascular Surgery 01/2005; 128(6):944-5. · 3.41 Impact Factor
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ABSTRACT: The atrial switch operation was developed by the efforts of many surgeons, with the most notable contributions made by Blalock, Hanlon, Albert, Baffes, Senning, and Mustard. The atrial switch operation was the first definitive repair for patients with transposition of great arteries and produced good results. Although it is rarely performed today, the atrial switch is not merely of historical interest as there remain a few important indications for this operation. A thorough understanding of the atrial switch is still required for surgeons dealing with complex congenital cardiac malformations. Herein we summarize the history, review long-term results, and discuss the future of the atrial switch operation.
The Annals of Thoracic Surgery 07/2004; 77(6):2250-8. · 3.74 Impact Factor
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ABSTRACT: Cleft mitral valve without an ostium primum defect, referred to as isolated cleft mitral valve, has been the subject of many reports; yet its morphology and operability remain incompletely understood.
The anatomic findings in 36 postmortem cases, five explanted hearts, and relevant clinical data constitute the material basis of this report. Cardiac catheterization data were available in 29 cases and two-dimensional echocardiograms in 13 cases.
Twenty cases had normally related great arteries with subpulmonary conus. Of these cases 4 (20%) had tetralogy of Fallot and 1 had tricuspid atresia. Twenty-one cases had abnormal ventriculoarterial relationships with subaortic or bilateral conus resulting in transposition in 16 (76%) and double-outlet right ventricle in 5 (24%). In the cases with normally related great arteries, the morphology of the ventricular septal defect and the mitral cleft were similar to those of the more complete forms of atrioventricular canal defects. The mitral cleft usually resulted in progressive mitral regurgitation, which can be treated by surgical closure of the cleft. In the cases with abnormal conus, the morphology of the ventricular septal defect and the mitral cleft did not resemble atrioventricular canal defects. The attachment of the cleft usually produced obstruction of the left ventricular pulmonary outflow tract. Surgical repair of the cleft cannot eliminate this obstruction.
There are two different anatomic types of isolated cleft mitral valve: the canal type, and abnormal conus type. Diagnosis of the associated ventriculoarterial relationships helps to guide their surgical treatment.
The Annals of Thoracic Surgery 07/2003; 75(6):1752-62. · 3.74 Impact Factor
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ABSTRACT: We report a case of an infant presenting with the rare association of tetralogy of Fallot, hypoplasia of the pulmonary arteries, and stenotic bicuspid aortic valve. Surgical correction, performed at 16 months of age, included aortic valvular commissurotomy, opening the right ventricular outflow tract (transannular patch), and ventricular septal defect closure. The postoperative course was favorable, and the child was discharged from the hospital. Three months after the procedure, the patient is in excellent condition, without cardiac medication.
The Annals of Thoracic Surgery 04/2002; 73(3):967-9. · 3.74 Impact Factor
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ABSTRACT: Apical ventricular septal defects (VSDs) are difficult to visualize and close transatrially. We described their distinctive anatomic features, which have seldom been documented angiocardiographically and pathologically, in order to develop an effective approach for their surgical management.
Fourteen postmortem cases, two explanted hearts, 9 successfully operated patients, and 1 unoperated living patient were included in this report. Angiocardiographic documentation of the apical VSD was available in 14 of 16 (87.5%) of the postmortem and transplanted cases, and in 6 of 10 (60%) of the living patients. Echocardiograms were available in 23 of all 26 cases (88%).
Severe associated malformations were present in 14 of 16 (87%) of the pathologically documented cases. Large VSDs allowed extensive communication between the left ventricular and the right ventricular sinuses in 4 patients. In 12 of the pathologically documented cases and in the 10 living patients, the left ventricular apex communicated with the right ventricular apical infundibular recess.
Extremely large apical VSDs with severe biventricular dysplasia and dysfunction may require cardiac transplantation. Large apical VSDs can be successfully closed through a small apical infundibulotomy. This approach, applicable even in small infants, can avoid pulmonary artery banding or left ventriculotomy.
The Annals of Thoracic Surgery 02/2002; 73(1):48-56; discussion 56-7. · 3.74 Impact Factor
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01/2002
