Richard Van Praagh

Harvard University, Cambridge, Massachusetts, United States

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Publications (155)712.7 Total impact

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    ABSTRACT: A persistent anastomosis between the pulmonary veins that connect with the left atrium and the systemic vein that drains into the right atrium has occasionally been reported. We report characteristics and transcatheter therapy in partially abnormal pulmonary venous return with additional drainage to the left atrium. We retrospectively studied such patients in 5 institutions. Ten patients (6 girls) presented at a median age of 8 (0.1 to 54) years with 2 anatomic types: 8 vertical vein types with drainage of the left upper lobe to the innominate vein via a large vertical vein (left superior cardinal vein) and to the left atrium via the left upper pulmonary vein; and 2 scimitar vein (SV) types with drainage of the right middle and lower pulmonary veins into the inferior vena cava and to the left atrium via an anomalous connecting vein. Associated malformations were aortic coarctation (n=2) and secundum atrial septal defects (n=3). Two patients of the vertical vein type were operated. Transcatheter occlusion of the abnormal pulmonary venous return was performed in 7 cases, associated with occlusion of systemic arterial supply (n=2), secundum atrial septal closure (n=2), left upper pulmonary vein stenosis stenting (n=1), and coarctation stenting (n=1). Including previously published cases, 18 patients (13 vertical veins and 5 scimitar veins) underwent transcatheter repair. Patients over 40years of age tend to be symptomatic at presentation (p=0.056). In partially abnormal pulmonary venous return with dual drainage, transcatheter therapy can be offered in the majority of patients.
    No preview · Article · Oct 2013 · International journal of cardiology
  • Richard Van Praagh

    No preview · Article · Jul 2013 · Annals of Pediatric Cardiology
  • E Delmo Walter · T Komoda · R Van Praagh · R Hetzer

    No preview · Article · Jan 2013 · The Thoracic and Cardiovascular Surgeon
  • Eva Maria B. Delmo Walter · Richard Van Praagh · Roland Hetzer
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    ABSTRACT: Background: The degree of involvement of left ventricular inflow tract obstruction is the predominant factor determining outcome in Shone's anomaly. In this series of patients with Shone's anomaly, we evaluated the impact of mitral valve (MV) repair strategies performed to correct the components of this anomaly on growth of the valve and long-term functional outcome in children. Methods: In the last 25 years, 45 children, mean age 5.16 ± 5.0 years (median, 3.9; range, 2 months-16.8 years), underwent surgical correction of Shone's anomaly. Coarctation of the aorta was found in 40%, subaortic stenosis due to fibromuscular hypertrophy was found in 55%, and subvalvar membrane was found in 66% of these patients. Left ventricular inflow tract obstruction was brought about by fused commissures with dysplastic and shortened chordae in 53.3%, valve hypoplasia in 11.1%, supravalvar mitral ring in 100%, and parachute valve in 17.8 of patients%. Results: Various repair strategies were performed according to the presenting morphologic characteristics in patients with either previously corrected or concomitant correction of the left-sided obstructive lesions. Mean duration of follow-up was 17.5 ± 1.5 years. Freedom from reoperation was 52.8% ± 11.8%, wherein 23 patients underwent repeated MV repair and 1 patient underwent MV replacement after failed attempts at repair. The cumulative survival rate was 70.3% ± 8.9% at 15 years. Severity and type of mitral abnormalities, left ventricular outflow tract lesions, and pulmonary hypertension are risk factors for reoperation and mortality (p < 0.05). Conclusions: Repair allowed growth of the MV. Long-term outcome of MV repair in Shone's anomaly is related to the degree that the obstructive lesions can be relieved.
    No preview · Article · Nov 2012 · The Annals of thoracic surgery
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    ABSTRACT: Objectives: Having assembled information on a large series of patients with Shone's anomaly with the longest follow-up, we studied the operative results and long-term outcome of mitral valve (MV) repair techniques performed to correct the left ventricular inflow tract lesions of this congenital anomaly. Methods: Between 1986 and 2011, 45 infants and children [mean age 5.16 ± 5.0 (median 3.9; range 2 months to 16.8 years] underwent surgical correction of Shone's anomaly. Left ventricular outflow tract obstructive lesions consisted of coarctation of the aorta, found in 89%, subaortic stenosis due to fibromuscular hypertrophy in 71% and subvalvular membrane in 51%. Left ventricular inflow tract obstructions concentrated on the mitral valve included mitral ring in all cases and the gamut of small and narrowed mitral orifice, commissural fusion, dysplastic or thickened leaflets, shortened and fused chordae, fibrous obliteration of interchordal spaces, underdeveloped papillary muscles, parachute valve, and supravalvular mitral ring-all contributing to mitral stenosis. Results: MV repair was performed using commissurotomy, division of chordae tendinae, papillary muscle splitting and fenestration, and resection of mitral ring, applied according to the presenting morphology in patients with either previously-corrected or concomitant correction of the left-sided obstructive lesions. Postoperative echocardiography showed absence of MV stenosis and immediate improvement of symptoms, except in a 3-month-old infant who died 18 days postoperatively due to myocardial failure. During the 23-year follow-up, 23 patients underwent repeat MV repair and one underwent MV replacement after failed attempts at repair. Mean duration of follow-up was 17.5 ± 1.5 years (range 6.4-22.7 years). Freedom from reoperation was 97.6 ± 2.4%, 89.3 ± 5.1%, 77.1 ± 7.2%, 72.0 ± 8.3% and 52.8 ± 11.8%, at 30 days, 1, 5, 10 and 15 years postoperatively, respectively Cumulative survival rate was 97.6 ± 2.4%, 92.3 ± 4.3%, 83.8 ± 6.1%, 75.7 ± 7.8% and 70.3 ± 8.9%, at 30 days, 1, 5, 10 and 15 years postoperatively, respectively. Mortality unrelated to valve repair accounted for nine deaths (20%). Age at operation, severity of mitral abnormalities and concomitant left ventricular outflow tract lesions proved to be independent risk factors for reoperation and mortality (P < 0.05). Conclusions: Long-term functional outcome of mitral valve repair in children with Shone's anomaly is satisfactory. Repeat MV repair and/or replacement may be deemed necessary during the course of follow-up.
    Preview · Article · Aug 2012 · European journal of cardio-thoracic surgery: official journal of the European Association for Cardio-thoracic Surgery
  • Richard Van Praagh

    No preview · Article · Aug 2012 · Pediatric and Developmental Pathology
  • Richard Van Praagh
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    ABSTRACT: The subarterial conal free walls in Homo sapiens normally perform an embryonic aortic switch procedure by 38 to 45 days of age in utero. The right-sided subaortic conal free wall normally undergoes complete resorption. Simultaneously, the left-sided subpulmonary conal free wall grows and expands, elevating the pulmonary valve superiorly and anteriorly-above the developing right ventricle, away from the interventricular foramen. Resorption of the right-sided subaortic conal free wall permits the aortic valve to move posteriorly, inferiorly, and leftward - through the interventricular foramen to above the developing left ventricle. This asymmetric or opposite rightleft development of the subarterial conal free walls results in normally related great arteries (solitus and inversus). There is only one way of doing the embryonic aortic switch right (with solitus and inversus isomers), and many ways of doing it wrong. All of the conotruncal malformations have anomalies of rightleft conal free wall asymmetry. Molecular genetic evidence suggests that malformations of right-left development may be caused by one or more mutations in the Nodal cascade. The heterotaxy syndromes, often misunderstood as malformations of bilateral symmetry (bilateral right-sidedness or bilateral left-sidedness), are also anomalies of right-left asymmetry. The other important evolutionary development was the appearance of the right ventricular sinus (inflow tract) beneath the proximal or apical part of the conus. The right ventricular sinus is the lung pump. Hence, a double circulation - systemic and pulmonary - evolved in fully terrestrial vertebrates. The cardiovascular system is the first system that must become functional in the human embryo to permit the rapid development of multicelled animals such as ourselves. Congenital heart disease is the commonest congenital malformation in live-born humans - almost 1% (0.8%). Most human congenital heart disease consists of anomalies of one or more of the four components that make up the right ventricle. Anomalies of the left ventricle are comparatively infrequent.our phylum Chordata, the morphologically left ventricle is at least 500 million years old. By contrast, the morphologically right ventricle is only about 180 million years old, i.e., only about 36% as old as the left ventricle. We are still having trouble with our comparatively recent cardiovascular evolutionary adaptations to air-breathing and permanent land-living: the development of the right ventricular sinus and the embryonic aortic switch. These two cardiovascular evolutionary developments helped to make possible our long terrestrial prehistory, our history, our cultures, our science, our "everything".
    No preview · Article · Mar 2011 · Polish journal of cardio-thoracic surgery
  • Richard Van Praagh
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    ABSTRACT: The conus arteriosus or infundibulum was the site of the major cardiovascular evolutionary and developmental adaptation that made possible air-breathing and permanent land-living for vertebrates, including mammals such as ourselves. The subarterial conal free walls perform an embryonic aortic switch procedure by 35 to 44 days of age in utero, based on growth of the left-sided subpulmonary conal free wall and resorption of the right-sided subaortic conal free wall, i.e., complete right-left asymmetry in the development of the subarterial conal free walls. There is only one way of doing the developmental aortic aortic switch procedure right (one way in situs solitus, and its mirror-image in situs inversus), and there are many ways of doing it wrong, resulting in the conotruncal anomalies. The proximal or apical part of the conus arteriosus, the septal band, was the mother of the right ventricular sinus (the lung pump). The conus transformed the single (systemic) circulation of fish into our double (systemic and pulmonary) circulations. The right ventricle (RV) is only about 36% as old as the left ventricle (LV). Most congenital heart disease involves anomalies of the more recently developed RV, congenital heart disease being the most frequent anomaly in liveborn children - almost 1 percent (0.8%).
    No preview · Article · Oct 2010 · World Journal for Pediatric and Congenital Hearth Surgery
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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.
    Preview · Article · Feb 2009 · Pediatric Cardiac Surgery Annual of the Seminars in Thoracic and Cardiovascular Surgery
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    G M Aru · A Juraszek · I Moskowitz · R Van Praagh
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    ABSTRACT: Two rare patients are reported with tetralogy of Fallot and congenital aortic valvar stenosis. The anatomic and developmental interrelationship between tetralogy of Fallot and truncus arteriosus is summarized. A study of 100 randomly selected postmortem cases of tetralogy revealed aortic valve pathology in 8%, myxomatous aortic valve leaflets without stenosis in 4%, bicuspid aortic valves without stenosis in 3%, and congenital aortic valvar stenosis in 1%. The frequency of systemic semilunar valve pathology in truncus was much higher (66%): moderate to marked myxomatous change in 44%, mild myxomatous change in 22%, truncal valvar stenosis in 11%, and truncal valvar regurgitation in 15%. Being aware of the tetralogy-truncus interrelationship and knowing that myxomatous aortic valves are prone to premature calcific aortic stenosis and/or regurgitation, physicians should follow the aortic valves of surgically repaired patients with tetralogy of Fallot and truncus arteriosus long term with great care. Timely aortic valvuloplasty or replacement may well prove life-saving in such patients.
    Preview · Article · Jan 2006 · Pediatric Cardiology
  • A.L. Juraszek · H Cohn · R Van Praagh · S Van Praagh
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    ABSTRACT: When the common pulmonary vein fails to develop, the embryonic connections of the pulmonary veins to one or more of the systemic veins almost always persist. Anomalous pulmonary venous connections to the inferior vena cava (IVC) are typically characterized by hypoplasia of the involved pulmonary veins and pulmonary artery, as well as abnormal parenchyma of the involved lung. Such cases have been described as "scimitar syndrome." We report the case of a young female patient in whom all the left pulmonary veins converged into a common vessel that drained into the IVC but who had a normal left pulmonary artery and left lung. Surgical intervention was successful, and our patient is still alive.
    No preview · Article · Nov 2005 · Pediatric Cardiology

  • No preview · Article · Jan 2005 · Journal of Thoracic and Cardiovascular Surgery
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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.
    Full-text · Article · Jul 2004 · The Annals of Thoracic Surgery
  • D Porras · C Kratz · M Loukas · N H van Doesburg · A Davignon · R Van Praagh
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    ABSTRACT: 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.
    No preview · Article · Dec 2003 · Pediatric Cardiology
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    Stella Van Praagh · Diego Porras · Guido Oppido · Tal Geva · Richard Van Praagh
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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.
    Full-text · Article · Jul 2003 · The Annals of Thoracic Surgery
  • S Van Praagh · T Geva · J E Lock · P. J. del Nido · M S Vance · R Van Praagh
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    ABSTRACT: 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.
    No preview · Article · Jul 2003 · Pediatric Cardiology

  • No preview · Article · Jun 2003 · The Annals of Thoracic Surgery
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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.
    No preview · Article · Apr 2002 · The Annals of Thoracic Surgery
  • A Vizcaino · J Campbell · S Litovsky · R Van Praagh
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    ABSTRACT: 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.
    No preview · Article · Mar 2002 · Pediatric Cardiology
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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.
    Preview · Article · Feb 2002 · The Annals of Thoracic Surgery

Publication Stats

5k Citations
712.70 Total Impact Points


  • 1995-2013
    • Harvard University
      Cambridge, Massachusetts, United States
  • 1974-2013
    • Boston Children's Hospital
      • • Department of Cardiac Surgery
      • • Department of Pathology
      • • Department of Pediatrics
      Boston, Massachusetts, United States
  • 1968-2006
    • Harvard Medical School
      • • Department of Pathology
      • • Department of Pediatrics
      Boston, Massachusetts, United States
  • 2000
    • University-Hospital of Padova
      Padua, Veneto, Italy
  • 1999
    • Valley Children's Hospital
      Мадера, California, United States
  • 1998
    • Università degli studi di Cagliari
      Cagliari, Sardinia, Italy
    • Cincinnati Children's Hospital Medical Center
      • Division of Pathology
      Cincinnati, Ohio, United States
  • 1996
    • McGill University
      • Division of Cardiothoracic Surgery
      Montréal, Quebec, Canada
  • 1990
    • Tufts University
      Бостон, Georgia, United States
    • Dartmouth–Hitchcock Medical Center
      LEB, New Hampshire, United States
  • 1988-1989
    • University of Massachusetts Boston
      Boston, Massachusetts, United States
  • 1985
    • Beverly Hospital, Boston MA
      BVY, Massachusetts, United States
  • 1982
    • Children's Hospital & Medical Center
      Omaha, Nebraska, United States
  • 1975-1979
    • Boston Medical Center
      Boston, Massachusetts, United States
  • 1978
    • University of Nebraska Medical Center
      • Department of Pediatrics
      Omaha, Nebraska, United States
  • 1969-1976
    • Boston College, USA
      Boston, Massachusetts, United States
  • 1971
    • Hartford Hospital
      • Department of Pediatrics
      Hartford, Connecticut, United States
  • 1970
    • Children's Memorial Hospital
      Chicago, Illinois, United States
  • 1965-1969
    • Hektoen Institute of Medicine
      Chicago, Illinois, United States
  • 1966
    • University of Toronto
      • Department of Paediatrics
      Toronto, Ontario, Canada