double outlet right ventricle

Summary

Summary: Incomplete transposition of the great vessels in which both the aorta and the pulmonary artery arise from the right ventricle, often associated with a subaortic ventricular septal defect.

Top Publications

  1. ncbi Surgery for right ventricle to pulmonary artery conduit obstruction: risk factors for further reoperation
    Siamak Mohammadi
    Department of Pediatric Cardiac Surgery, Marie Lannelongue Hospital, 133 ave de la Résistance, 92350 Le Plessis Robinson, France
    Eur J Cardiothorac Surg 28:217-22. 2005
  2. ncbi Establishing right ventricle-pulmonary artery continuity by autologous tissue: an alternative approach for prosthetic conduit repair
    Yukihisa Isomatsu
    Department of Cardiovascular Surgery, The Heart Institute of Japan, Tokyo Women s Medical University, Japan
    Ann Thorac Surg 78:173-80. 2004
  3. ncbi Surgical repair for double outlet right ventricle and intact ventricular septum
    D E Troise
    Department of Pediatric Cardiac Surgery, Giovanni XXIII Hospital, Bari, Italy
    Ann Thorac Surg 71:1018-9. 2001
  4. ncbi Surgical management of double outlet right ventricle with intact ventricular septum
    Carlo Pace Napoleone
    Department of Pediatric Cardiac Surgery, S Orsola Malpighi Hospital, Bologna, Italy
    Ann Thorac Surg 75:586-7. 2003
  5. ncbi Double outlet right ventricle: aetiologies and associations
    D Obler
    Department of Cardiology, Children s Hospital, Boston, MA 02115, USA
    J Med Genet 45:481-97. 2008
  6. doi New mutations in ZFPM2/FOG2 gene in tetralogy of Fallot and double outlet right ventricle
    Alessandro De Luca
    CSS Mendel Institute, Casa Sollievo della Sofferenza Hospital, IRCCS, San Giovanni Rotondo, Rome, Italy
    Clin Genet 80:184-90. 2011
  7. ncbi Transcatheter creation and enlargement of ventricular septal defects for relief of ventricular hypertension
    Jeffery Meadows
    Department of Cardiology, Children s Hospital Boston, Boston, Mass 02115, USA
    J Thorac Cardiovasc Surg 133:912-8. 2007
  8. ncbi Biventricular repair in double outlet right ventricle: surgical results based on the STS-EACTS International Nomenclature classification
    John H Artrip
    Denver Children s Hospital, Denver, CO, USA
    Eur J Cardiothorac Surg 29:545-50. 2006
  9. doi Impact of prenatal diagnosis and anatomical subtype on outcome in double outlet right ventricle
    Maria E Lagopoulos
    Hospital for Sick Children, Labatt Family Heart Centre, University of Toronto, Toronto, Ontario, Canada
    Am Heart J 160:692-700. 2010
  10. ncbi Congenital Heart Surgery Nomenclature and Database Project: double outlet right ventricle
    H L Walters
    Department of Surgery, Wayne State University School of Medicine, Children s Hospital of Michigan, Detroit 48201, USA
    Ann Thorac Surg 69:S249-63. 2000

Detail Information

Publications196 found, 100 shown here

  1. ncbi Surgery for right ventricle to pulmonary artery conduit obstruction: risk factors for further reoperation
    Siamak Mohammadi
    Department of Pediatric Cardiac Surgery, Marie Lannelongue Hospital, 133 ave de la Résistance, 92350 Le Plessis Robinson, France
    Eur J Cardiothorac Surg 28:217-22. 2005
    ..To identify the surgical approaches and risk factors which influence longevity of right ventricle to pulmonary artery (RV-PA) conduits following first reoperation for obstruction...
  2. ncbi Establishing right ventricle-pulmonary artery continuity by autologous tissue: an alternative approach for prosthetic conduit repair
    Yukihisa Isomatsu
    Department of Cardiovascular Surgery, The Heart Institute of Japan, Tokyo Women s Medical University, Japan
    Ann Thorac Surg 78:173-80. 2004
    ..This study was undertaken to compare long-term results of the use of equine pericardial conduits, autologous pericardial conduits, and direct anastomosis repair...
  3. ncbi Surgical repair for double outlet right ventricle and intact ventricular septum
    D E Troise
    Department of Pediatric Cardiac Surgery, Giovanni XXIII Hospital, Bari, Italy
    Ann Thorac Surg 71:1018-9. 2001
    We report the case of a neonate with a complex cardiac anomaly that consisted of a double outlet right ventricle, intact ventricular septum, small left ventricle, and pulmonary stenosis who underwent surgical repair with a successful ..
  4. ncbi Surgical management of double outlet right ventricle with intact ventricular septum
    Carlo Pace Napoleone
    Department of Pediatric Cardiac Surgery, S Orsola Malpighi Hospital, Bologna, Italy
    Ann Thorac Surg 75:586-7. 2003
    A very rare case of double outlet right ventricle with intact ventricular septum and unrestricted pulmonary flow was successfully palliated with pulmonary banding and delayed bidirectional cavopulmonary anastomosis and mitral avulsion...
  5. ncbi Double outlet right ventricle: aetiologies and associations
    D Obler
    Department of Cardiology, Children s Hospital, Boston, MA 02115, USA
    J Med Genet 45:481-97. 2008
    b>Double outlet right ventricle (DORV), a clinically significant congenital heart defect, occurs in 1-3% of individuals with congenital heart defects...
  6. doi New mutations in ZFPM2/FOG2 gene in tetralogy of Fallot and double outlet right ventricle
    Alessandro De Luca
    CSS Mendel Institute, Casa Sollievo della Sofferenza Hospital, IRCCS, San Giovanni Rotondo, Rome, Italy
    Clin Genet 80:184-90. 2011
    ..with CTD, including tetralogy of Fallot with or without pulmonary atresia (TOF, 178 patients), double outlet right ventricle (DORV, 13 patients), and truncus arteriosus (11 patients)...
  7. ncbi Transcatheter creation and enlargement of ventricular septal defects for relief of ventricular hypertension
    Jeffery Meadows
    Department of Cardiology, Children s Hospital Boston, Boston, Mass 02115, USA
    J Thorac Cardiovasc Surg 133:912-8. 2007
    ..We report the results of a novel technique for transcatheter creation or enlargement of ventricular septal defects as an alternative to reoperation when decompression of an isolated ventricle is indicated...
  8. ncbi Biventricular repair in double outlet right ventricle: surgical results based on the STS-EACTS International Nomenclature classification
    John H Artrip
    Denver Children s Hospital, Denver, CO, USA
    Eur J Cardiothorac Surg 29:545-50. 2006
    ..International Nomenclature for Congenital Heart Surgery (CHS) defines four anatomic subtypes of double outlet right ventricle (DORV) based on the relationship of the ventricular septal defect (VSD) with the great vessels and the ..
  9. doi Impact of prenatal diagnosis and anatomical subtype on outcome in double outlet right ventricle
    Maria E Lagopoulos
    Hospital for Sick Children, Labatt Family Heart Centre, University of Toronto, Toronto, Ontario, Canada
    Am Heart J 160:692-700. 2010
    We sought to investigate the influence of prenatal diagnosis and risk factors for adverse outcomes in double outlet right ventricle (DORV) not associated with heterotaxy.
  10. ncbi Congenital Heart Surgery Nomenclature and Database Project: double outlet right ventricle
    H L Walters
    Department of Surgery, Wayne State University School of Medicine, Children s Hospital of Michigan, Detroit 48201, USA
    Ann Thorac Surg 69:S249-63. 2000
    b>Double outlet right ventricle (DORV) is a type of ventriculoarterial connection in which both great vessels arise entirely or predominantly from the right ventricle...
  11. ncbi Argatroban anticoagulation for cardiac surgery with cardiopulmonary bypass in an infant with double outlet right ventricle and a history of heparin-induced thrombocytopenia
    Michael L Ciccolo
    Cardiothoracic Surgery, Cardiovascular Surgical Associates, Las Vegas, Nevada, USA
    Congenit Heart Dis 3:299-302. 2008
    ..We report the use of the anticoagulant argatroban for cardiac surgery with cardiopulmonary bypass in an infant with double outlet right ventricle and a history of HIT.
  12. ncbi The impact of the length between the top of the interventricular septum and the aortic valve on the indications for a biventricular repair in patients with a transposition of the great arteries or a double outlet right ventricle
    Yasuhiro Fujii
    Department of Cardiovascular Surgery, Okayama University Hospital, 2 5 1 Shikata cho, Kita ku, Okayama City, Okayama, 700 8558, Japan
    Interact Cardiovasc Thorac Surg 10:900-5; discussion 905. 2010
    ..in patients with transposition of the great arteries (TGA)/ventricular septal defect (VSD) or double outlet right ventricle (DORV)...
  13. ncbi Outcomes of definitive surgical repair for congenitally corrected transposition of the great arteries or double outlet right ventricle with discordant atrioventricular connections: risk analyses in 189 patients
    Toshiharu Shin'oka
    Department of Cardiovascular Surgery, The Heart Institute of Japan, Tokyo Women s Medical University, Tokyo, Japan
    J Thorac Cardiovasc Surg 133:1318-28, 1328.e1-4. 2007
    ..types of surgical repairs for either congenitally corrected transposition of the great arteries or double outlet right ventricle with discordant atrioventricular connections, and to analyze the risk factors that affect early and ..
  14. ncbi Biventricular repair of double outlet right ventricle with noncommitted ventricular septal defect
    Francois Lacour-Gayet
    Department of Cardiac Surgery, Eppendorf University Hospital, Hamburg, Germany
    Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 5:163-72. 2002
    b>Double outlet right ventricle (DORV) with noncommitted ventricular septal defect (VSD) (DORVncVSD) represents the most extreme form of DORV, raising challenging surgical difficulties for biventricular repair...
  15. ncbi Biventricular repair of double outlet right ventricle with non-committed ventricular septal defect (VSD) by VSD rerouting to the pulmonary artery and arterial switch
    F Lacour-Gayet
    Department of Pediatric Cardiac Surgery, Eppendorf University Hospital UKE, Martinistrasse 52, D 20246 Hamburg, Germany
    Eur J Cardiothorac Surg 21:1042-8. 2002
    Biventricular repair of double outlet right ventricle non-committed ventricular septal defect (DORVncVSD) is usually achieved by a VSD rerouting to the aorta...
  16. ncbi [Indications of arterial switch operation for complex congenital heart defect with severe pulmonary hypertension and ventriculoarterial discordant connection]
    Ying Long Liu
    Pediatric Center of Cardiac Surgery, Cardiovascular Institute and Fuwai Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100037, China
    Zhonghua Yi Xue Za Zhi 86:23-5. 2006
    ..To summarize the experience in arterial switch operation (ASO) for complex congenital heart defect (CHD) with severe pulmonary hypertension (PH) and ventriculoarterial discordant connection in children older than 6 months...
  17. ncbi Laryngotracheoesophageal cleft type 3 and double outlet right ventricle: unique combination
    Abdullah Alabdulgader
    King Fahad Hospital, P O Box 9596, Prince Sultan Cardiac Centre, Hofuf 31982, Eastern Province, Saudi Arabia
    Ann Diagn Pathol 9:323-6. 2005
    ..Endoscopic evaluation revealed type 3 laryngotracheoesophageal cleft. Echocardiogram revealed double outlet right ventricle with subpulmonic ventricular septal defect...
  18. ncbi Surgical repair of incomplete cleft sternum and cardiac anomalies in early infancy
    Massimo Antonio Padalino
    Division of Pediatric and Congenital Cardiac Surgery, Department of Pediatrics, University of Padova, Medical School, Padova, Italy
    Ann Thorac Surg 81:2291-4. 2006
    ..We report two cases of incomplete superior cleft sternum associated with cardiac anomalies that underwent successful complete surgical correction...
  19. ncbi Ten-year experience with the arterial switch operation
    R Sharma
    Department of Cardiothoracic and Vascular Surgery, Cardiothoracic Centre, All India Institute of Medical Sciences, New Delhi
    Indian Heart J 54:681-6. 2002
    ..Arterial level repair is considered the most appropriate procedure for transposition of the great arteries. This report describes our experience with the arterial switch operation over the past decade...
  20. ncbi Tricuspid valve detachment for transatrial closure of ventricular septal defects
    Sajan Koshy
    Division of Pediatric Cardiac Surgery, Department of Cardiovascular and Thoracic Surgery, Amrita Institute of Medical Sciences and Research Center, Kochi, Cochin, Kerala 682026, India
    Asian Cardiovasc Thorac Ann 10:314-7. 2002
    ..The benefits of temporary leaflet detachment for transatrial repair of various difficult defects far outweigh the risk of postoperative tricuspid regurgitation...
  21. ncbi The fenestrated Kawashima operation for single ventricle with interrupted inferior vena cava
    Robert L Hannan
    Division of Cardiovascular Surgery, Miami Children s Hospital, Miami, Florida 33155, USA
    Ann Thorac Surg 75:271-3. 2003
    An 8-month-old boy with double outlet right ventricle with hypoplastic left ventricle, heterotaxy, left atrial isomerism, bilateral superior vena cavae without bridging vein, and interruption of the inferior vena cava with azygous ..
  22. ncbi Normally connected anomalously draining obstructed pulmonary veins in an infant with mitral atresia: clinical presentation and catheter management
    M R Ebeid
    Division of Cardiology, Department of Pediatrics, Children s Hospital, University of Mississippi Medical Center, 2500 North State Street, Jackson, MS 39216, USA
    Pediatr Cardiol 24:403-5. 2003
    A patient with hypoplastic left ventricle and double outlet right ventricle underwent pulmonary artery band as a newborn...
  23. ncbi [Single-stage arterial switch operation for transposition of the great arteries and Taussig-Bing with aortic arch obstruction]
    Zhi Wei Xu
    Department of Thoracic and Cardiovascular Surgery, Xinhua Hospital, Shanghai Second Medical University, Shanghai 200127, China
    Zhonghua Wai Ke Za Zhi 43:1441-3. 2005
    ..To evaluate one-stage arterial Switch operation for transposition of the great arteries (TGA) and Taussig-Bing with aortic arch obstruction...
  24. ncbi DORV with non-committed VSD and Taussig-Bing hearts. Controversial anatomic entities
    Jose M Caffarena
    Eur J Cardiothorac Surg 23:136; author reply 136-7. 2003
  25. ncbi Primary repair of pulmonary artery sling with double outlet right ventricle and distal tracheal stenosis
    Y Oshima
    Department of Cardio thoracic Surgery and Pediatric Surgery, Kobe Children s Hospital, Kobe, Japan
    J Cardiovasc Surg (Torino) 43:849-51. 2002
    ..We present a case of successful simultaneous one-stage repair of pulmonary artery sling, double outlet right ventricle and distal tracheal obstruction...
  26. ncbi Resternotomy for a retrosternal cardiac pseudoaneurysm in a 1.5-year- old child: a case report
    Ghassan M Baslaim
    Department of Cardiovascular Diseases and Radiology, King Faisal Specialist Hospital and Research Center, Jeddah, Saudi Arabia
    J Card Surg 20:497-500. 2005
    ..with the surgical approach of a child with a cardiac pseudoaneurysm who had undergone a biventricular repair of a double outlet right ventricle with non-committed ventricular septal defect in the form of intraventricular tunneling.
  27. ncbi Improvement of protein-losing enteropathy by coil embolization of the left azygos vein
    Akira Ishii
    Department of Pediatrics, Sapporo Medical University School of Medicine, Sapporo, Japan
    Catheter Cardiovasc Interv 59:399-401. 2003
    ....
  28. ncbi [Two-stage repair of the transposition of great arteries with interruption/coarctation of the aorta]
    Fumio Fukumura
    Department of Cardiovascular Surgery, Kyushu Koseinenkin Hospital, Kitakyushu, Japan
    Kyobu Geka 58:66-70. 2005
    ..Two-stage repair for TGA with IAA/CoA is still a useful method with a good operative result. However, strict follow-up is necessary because of the high frequency of late morbidity...
  29. ncbi Posterior descending coronary artery flow reserve assessment by Doppler echocardiography in children with and without congenital heart defect: comparison with invasive technique
    M Aoki
    Department of Pediatrics, Akita University School of Medicine, I 1 1 Hondo, 010 8543, Akita, Japan
    Pediatr Cardiol 25:647-53. 2004
    ....
  30. ncbi Long-term predictors of aortic root dilation and aortic regurgitation after arterial switch operation
    Marcy L Schwartz
    Department of Cardiology, Children s Hospital, 300 Longwood Avenue, Boston, Mass 02115, USA
    Circulation 110:II128-32. 2004
    ..Neo-aortic root dilation (ARD) and neo-aortic regurgitation (AR) may be progressive after arterial switch operation (ASO) for d-loop transposition of the great arteries (dTGA). We sought to identify predictors of ARD and AR after ASO...
  31. ncbi [Risk factors associated with arterial switch operation for transposition of the great arteries]
    Juan A García Hernández
    Unidad de Cuidados Intensivos Pediatricos, Hospital Universitario Virgen del Rocio, Hospital Infantil, Sevilla, Spain
    Rev Esp Cardiol 58:815-21. 2005
    ..The present study was undertaken to determine the risk factors for early mortality following an arterial switch operation...
  32. ncbi [Arterial switch operation for transposition of the great arteries and double outlet of right ventricle with subpulmonary ventricular septum defect]
    Zhi Wei Xu
    Department of Thoracic and Cardiovascular Surgery, Xinhua Hospital, Shanghai Second Medical University, Shanghai 200127, China
    Zhonghua Wai Ke Za Zhi 42:451-4. 2004
    ..To summarize the clinical experience on the arterial switch operation for complex congenital heart disease in recent 3 years in our hospital...
  33. ncbi Selective timing for the arterial switch operation
    Brian W Duncan
    Department of Pediatric and Congenital Heart Surgery, The Children s Hospital at the Cleveland Clinic, Cleveland, Ohio 44195, USA
    Ann Thorac Surg 77:1691-6; discussion 1697. 2004
    ..To determine outcomes for the arterial switch operation individualized according to the underlying anatomy and clinical status...
  34. ncbi Combined aortic arch repair and arterial switch without coronary reimplantation
    Kozo Ishino
    Department of Cardiovascular Surgery, Okayama University Graduate School of Medicine, Japan
    J Thorac Cardiovasc Surg 127:577-8. 2004
  35. ncbi Redefining the impact of oxygen and hyperventilation after the Norwood procedure
    Scott M Bradley
    Division of Cardiothoracic Surgery, Medical University of South Carolina, Charleston 29425, USA
    J Thorac Cardiovasc Surg 127:473-80. 2004
    ..This study determines the effects of these interventions on mixed venous saturation and systemic oxygen delivery in postoperative neonates...
  36. ncbi Complex double-outlet right ventricle repair in a neonate with complete tracheal agenesis
    Guido Oppido
    Department of Pediatric Cardiac Surgery, S Orsola Malpighi Hospital, University of Bologna Medical School, Italy
    J Thorac Cardiovasc Surg 127:283-5. 2004
  37. ncbi Risk factors for neo-aortic root enlargement and aortic regurgitation following arterial switch operation
    C J McMahon
    Lillie Frank Abercrombie Division of Pediatric Cardiology, Texas Children s Hospital and Baylor College of Medicine, Houston, TX 77030, USA
    Pediatr Cardiol 25:329-35. 2004
    ..Surgical intervention for AR was rare (2%), however, serial surveillance of such patients is vital to monitor for neo-aortic root enlargement and potential aortic valve dysfunction...
  38. ncbi Acquired coronary artery fistula after open heart surgery for congenital heart disease
    Shuenn Nan Chiu
    Department of Pediatrics, National Taiwan University Hospital, School of Medicine, National Taiwan University, 7 Chung Shan S Rd, Taipei 100, Taiwan
    Int J Cardiol 103:187-92. 2005
    ..Most coronary artery fistulas were reported as congenital. Acquired coronary artery fistula occurring after cardiac surgery has rarely been reported...
  39. ncbi Double-outlet technique for tetralogy of Fallot-type disease with an anomalous coronary artery
    M Asano
    Department of Cardiovascular Surgery, Nagoya City University Graduate School of Medical Sciences, Kawasumi 1, Mizuho ku, Nagoya 467 8601, Japan
    Pediatr Cardiol 26:710-2. 2005
    ..If the anomalous coronary artery exists in the RV outflow tract, the procedure can not be done...
  40. ncbi [Arterial switch operation in older infants with severe pulmonary hypertension]
    Qing Yu Wu
    Department of Cardiovascular Surgery, Cardiovascular Institute and Fuwai Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100037, China
    Zhonghua Yi Xue Za Zhi 83:478-81. 2003
    ..To investigate the clinical efficacy of arterial swith operation on transposition of great artery (TGA) and Tausing-Bing anomaly...
  41. ncbi Chromosome 22q11 deletions in patients with conotruncal heart defects
    A Khositseth
    Department of Pediatrics, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, Bangkok 10400, Thailand
    Pediatr Cardiol 26:570-3. 2005
    ..A subgroup of patients with IAA, TA, SPVSD, and PAVSD associated with phenotypic features or abnormalities warrants evaluation for the presence of 22q11 deletions...
  42. ncbi Midterm results after restoration of the morphologically left ventricle to the systemic circulation in patients with congenitally corrected transposition of the great arteries
    Stephen M Langley
    Department of Cardiac Surgery, Diana Princess of Wales Children s Hospital, Birmingham, United Kingdom
    J Thorac Cardiovasc Surg 125:1229-41. 2003
    ..This study was undertaken to determine the outcomes of patients with congenitally corrected transposition of the great arteries after restoration of the morphologically left ventricle to the systemic circulation...
  43. ncbi Modified fenestration technique for the Kawashima operation in a young infant
    Dante Picarelli
    Division of Cardiac Surgery, American Cardiac Center, American Hospital, Montevideo, Uruguay
    J Thorac Cardiovasc Surg 129:451-2. 2005
  44. ncbi Potential goals for the dimensions of the pulmonary arteries and aorta with stenting after the Fontan operation
    Hideshi Tomita
    Department of Pediatrics, National Cardiovascular Center, Osaka, Japan
    Catheter Cardiovasc Interv 56:246-53. 2002
    ..In conclusion, smaller-sized stents should be acceptable for both the pulmonary artery and descending aorta in the Fontan circulation...
  45. ncbi Is preoperative delineation of coronary artery pattern a prerequisite for arterial switch operation?
    Ghassan M Baslaim
    Division of Cardiothoracic Surgery, Department of Cardiovascular Diseases, King Faisal Specialist Hospital and Research Center, Jeddah, Saudi Arabia
    J Card Surg 21:465-70. 2006
    ..To determine the effect of preoperative echocardiographic delineation of coronary artery pattern on the immediate postoperative result of arterial switch procedures...
  46. ncbi Cardiac outcome up to 15 years after the arterial switch operation
    Sharon H Wong
    Green Lane Paediatric and Congenital Cardiac Service, Starship Children s Hospital, Auckland, New Zealand
    Heart Lung Circ 17:48-53. 2008
    ..To assess the cardiac outcome and risk factors for mortality of infants following the arterial switch operation (ASO)...
  47. ncbi Surgical repair of coronary sinus orifice atresia with persistent left superior vena cava in heterotaxia
    Shin Takabayashi
    Department of Thoracic and Cardiovascular Surgery, Mie University Graduate School of Medicine, 2 174 Edobashi, Tsu, Mie 514 8507, Japan
    Gen Thorac Cardiovasc Surg 55:197-9. 2007
    ..Bidirectional Glenn anastomosis and edge-to-edge common atrioventricular valve repair were concomitantly performed. After a 1-year follow-up period, the patient is alive and well without any ischemic event...
  48. ncbi Repair of double outlet right ventricle with doubly-committed ventricular septal defect
    H Uemura
    Department of Cardiovascular Surgery, National Cardiovascular Center, Osaka, Japan
    Cardiol Young 11:415-9. 2001
    OBJECTIVE: To investigate our surgical results of intraventricular rerouting in patients having double outlet right ventricle with doubly-committed ventricular septal defect...
  49. ncbi Echocardiographic characteristics and outcome of straddling mitral valve
    A Fraisse
    Department of Cardiology, Children's Hospital, Boston, Massachusetts 02115, USA
    J Am Coll Cardiol 38:819-26. 2001
    ..Although a biventricular approach is feasible in selected patients, a functional univentricular palliation is indicated in those with major straddling and markedly hypoplastic ventricles...
  50. ncbi Right ventricular outflow tract obstruction after arterial switch operation for the Taussig-Bing heart
    Nicodeme Sinzobahamvya
    Department of Pediatric Thoracic and Cardiovascular Surgery, Congenital Cardiac Center Deutsches Kinderherzzentrum, Sankt Augustin, Germany
    Eur J Cardiothorac Surg 31:873-8. 2007
    ..We evaluated the risk to develop RVOTO after ASO for Taussig-Bing heart...
  51. ncbi Arterial switch operation in patients with Taussig-Bing anomaly--influence of staged repair and coronary anatomy on outcome
    Massimo Griselli
    Department of Paediatric Cardiac Surgery, Diana, Princess of Wales Children s Hospital, Steelhouse Lane, Birmingham B4 6NH, United Kingdom
    Eur J Cardiothorac Surg 31:229-35. 2007
    This study evaluated the results of arterial switch operation and closure of ventricular defects (ASO+VSDc) for double outlet right ventricle with sub-pulmonary ventricular septal defect (Taussig-Bing anomaly).
  52. ncbi Intermediate-term results of the Senning or Mustard procedures combined with the Rastelli operation for patients with discordant atrioventricular connections associated with discordant ventriculoarterial connections or double outlet right ventricle
    Jürgen Hörer
    Department of Cardiovascular Surgery, German Heart Centre Munich at the Technical University, Munich, Germany
    Cardiol Young 17:158-65. 2007
    ..In this study, we have evaluated the outcomes in the intermediate term for this complex surgical procedure...
  53. ncbi The optimal procedure for the great arteries and left ventricular outflow tract obstruction. An anatomical study
    Mark Hazekamp
    Department of Cardiothoracic Surgery, Leiden University Medical Center, Leiden, The Netherlands
    Eur J Cardiothorac Surg 31:879-87. 2007
    ..To describe the optimal surgical strategy in heart specimens with transposition of the great arteries (TGA) and left ventricular outflow tract obstruction (LVOTO)...
  54. ncbi [Surgical treatment on residual shunt after repair of ventricular septal defect]
    Wen Gen Gao
    Cardiovascular Department of General Hospital of Shenyang Military District, Shenyang 110016, China
    Zhonghua Wai Ke Za Zhi 42:462-4. 2004
    ..To summarize the experience in surgical treatment of residual shunt after repair of ventricular septal defect and investigate the position of the residual shunts...
  55. ncbi Current risk factors and outcomes for the arterial switch operation
    Zuhab A Qamar
    Division of Pediatric Cardiovascular Surgery, C S Mott Children s Hospital, Ann Arbor, Michigan, USA
    Ann Thorac Surg 84:871-8; discussion 878-9. 2007
    ..The arterial switch operation is the preferred treatment for dextrotransposition of the great arteries and some forms of double-outlet right ventricle...
  56. ncbi [Double switch operation (Senning and Rastelli operation) with left atrial augumentation by in situ autologous pericardium: a case report]
    M Ogawa
    Department of Pediatric Cardiovascular Surgery, Kyoto Prefectural University of Medicine, Kyoto, Japan
    Kyobu Geka 54:1099-102. 2001
    ..For the purpose of atrial augumentation without artificial materials, our modified Senning procedure is useful. But longer follow-up is necessary to determine whether this technique is indeed beneficial...
  57. ncbi Arterial switch operation with a single coronary artery
    Albertus M Scheule
    Department of Cardiac Surgery, Children s Hospital, Harvard Medical School, Boston, MA 02115, USA
    J Thorac Cardiovasc Surg 123:1164-72. 2002
    ..Our purpose was to evaluate the impact of coronary pattern on survival and reintervention in patients who underwent the arterial switch operation with a single coronary artery...
  58. ncbi [The effects of coronary abnormalities on reconstruction of right ventricular outflow tract in patients with complex congenital heart disease]
    Fan dong Li
    Department of Cardiovascular Surgery, Shandong Provencial Hospital, Shandong University, Jinan 250012, China
    Zhonghua Wai Ke Za Zhi 44:655-7. 2006
    ..To explore the effects of coronary abnormalities on reconstruction of right ventricular outflow tract in patients with complex congenital heart disease...
  59. ncbi [Technical modification and late results of arterial switch operation]
    Masaaki Yamagishi
    Department of Pediatric Cardiovascular Surgery, Children s Research Hospital, Kyoto Prefectural University of Medicine, Kyoto, Japan
    Kyobu Geka 61:297-302. 2008
    ..No coronary events occurred (i.e. inclusive of coronary stenosis, myocardial infarction, and coronary death). The bay window technique is an innovative and simple surgical adjunct for translocating complex coronary arteries...
  60. ncbi Analysis of surgical outcome in complex double-outlet right ventricle with heterotaxy syndrome or complete atrioventricular canal defect
    Koh Takeuchi
    Department of Cardiac Surgery, Children s Hospital, Harvard Medical School, Boston, Massachusetts, USA
    Ann Thorac Surg 82:146-52. 2006
    ..Heterotaxy syndrome, which is often associated with total anomalous pulmonary venous connection and complete atrioventricular canal defect, has been considered a risk factor for surgical repair of double-outlet right ventricle...
  61. doi Isolated atrioventricular discordance: surgical experience
    Rajesh Sharma
    Departments of Pediatric Cardiology and Cardiovascular Surgery, Narayana Hrudayalaya Institute of Medical Sciences, Bangalore, India
    Ann Thorac Surg 85:1403-6. 2008
    ..Published literature is scant, mainly because of the rarity of this cardiac anomaly. We review our surgical experience with this lesion...
  62. ncbi Total cavo-pulmonary connection without foreign material for asplenic heart associated with partial anomalous pulmonary venous connection
    Kouta Agematsu
    Department of Cardiovascular Surgery, Chiba Children s Hospital, 579 1, Heta Cho, Midori ku, Chiba City, Chiba, 266 0007, Japan
    Interact Cardiovasc Thorac Surg 7:344-6. 2008
    The presented case was a 3-year-old boy diagnosed with asplenia (SLL), double outlet right ventricle, pulmonary stenosis, atrioventricular septal defect, hypoplastic left ventricle and partial anomalous pulmonary venous connection to the ..
  63. ncbi The effect of p.Arg25Cys alteration in NKX2-5 on conotruncal heart anomalies: mutation or polymorphism?
    M I Akçaboy
    Division of Pediatric Genetics, Ankara University School of Medicine, Birlik Mah 65 Sok No 20 7, Cankaya, Ankara 06610, Turkey
    Pediatr Cardiol 29:126-9. 2008
    ..These results show that no genetic support exists for the pathogenecity of this alteration, although a previous in vitro study and theoretical predictions suggest a structural/functional difference in the altered protein region...
  64. ncbi Hybrid approach as a bridging procedure to biventricular repair for aortic hypoplasia with ventricular septal defect in a 1720-g premature infant
    Pedro Betrián Blasco
    Pediatric Cardiology and Hemodynamics Department, Valle de Hebron Hospital, Barcelona, Spain
    J Thorac Cardiovasc Surg 134:516-8. 2007
  65. ncbi Double switch operation for superior-inferior ventricles
    N Hibino
    Department of Pediatric Cardiovascular Surgery, The Heart Institute of Japan, Tokyo Women's Medical College
    Ann Thorac Surg 72:2119-21. 2001
    ..This is the first report we have been able to locate of a double switch operation for superior-inferior ventricles...
  66. ncbi Images in cardiovascular medicine. Virtual cardiotomy for preoperative planning
    Thomas Sangild Sørensen
    CAVI University of Aarhus, Aabogade 34, 8200 Aarhus N, Denmark
    Circulation 115:e312. 2007
  67. ncbi [Lung pathology of complex congenital heart defect with diminished pulmonary blood flow but without aortopulmonary collateral artery and patent ductus arteriosus in infants and young children]
    Xiao Tong Zhang
    Center of Pediatric Cardiac Surgery, Cardiovascular Institute and Fuwai Hospital, Chinese Academy of Medical Sciences Peking Union Medical College, Beijing 100037, China
    Zhongguo Dang Dai Er Ke Za Zhi 10:311-4. 2008
    ....
  68. ncbi Right atrial isomerism: preponderance in Asian fetuses. Using the stomach-distance ratio as a possible diagnostic tool for prediction of right atrial isomerism
    Ying Liu Yan
    Antenatal Diagnostic Centre, KK Women s and Children s Hospital, Singapore
    Ann Acad Med Singapore 37:906-12. 2008
    ..34 as a diagnostic tool to predict right atrial isomerism...
  69. ncbi Initial experience with a bicuspid polytetrafluoroethylene pulmonary valve in 41 children and adults: a new option for right ventricular outflow tract reconstruction
    James A Quintessenza
    The Congenital Heart Institute of Florida CHIF, All Children s Hospital, University of South Florida, St Petersburg 33701, United States
    Ann Thorac Surg 79:924-31. 2005
    ..We report our initial experience in 41 consecutive patients treated with placement of a surgically created polytetrafluoroethylene bicuspid pulmonary valve...
  70. ncbi Early and long-term prognostic value of Troponin-I after cardiac surgery in newborns and children
    Tomaso Bottio
    Cardiovascular Institute, School of Medicine of Brescia, Brescia, Italy
    Eur J Cardiothorac Surg 30:250-5. 2006
    ....
  71. ncbi Transcatheter pulmonary valve implantation using the Edwards SAPIEN transcatheter heart valve
    Robert H Boone
    St Paul s Hospital, University of British Columbia, Vancouver, Canada
    Catheter Cardiovasc Interv 75:286-94. 2010
    ..Transcatheter pulmonary valve implantation improves conduit hemodynamics through a minimally invasive approach. We present data for 7 patients treated with the Edwards SAPIEN transcatheter heart valve (THV)...
  72. ncbi Conotruncal anomalies in the trisomy 16 mouse: an immunohistochemical analysis with emphasis on the involvement of the neural crest
    B R Waller
    Department of Cell Biology and Anatomy, Cardiovascular Developmental Biology Center, Medical University of South Carolina, Charleston, South Carolina 29425, USA
    Anat Rec 260:279-93. 2000
    ..5 to 14.5 embryonic days. Conotruncal malformations observed in the Ts 16 embryos included double outlet right ventricle, persistent truncus arteriosus, Tetralogy of Fallot, and right-sided aortic arch...
  73. ncbi New indicator for the Fontan operation: diameters of the pulmonary veins in patients with univentricular heart
    Y Kawahira
    Department of Cardiovascular Surgery, Osaka Medical Center, Japan
    J Card Surg 14:259-65. 1999
    ..Operative survival after the Fontan procedure is good; however, there are some patients with disappointing results, especially those with atrial isomerism...
  74. ncbi The role of the three vessels and trachea view (3VT) in the diagnosis of congenital heart defects
    F Vinals
    Centro AGB Ultrasonografía, Ultrasound Department, Clínica Sanatorio Alemán, Concepcion, Chile
    Ultrasound Obstet Gynecol 22:358-67. 2003
    ....
  75. ncbi Operative strategies for pulmonary artery occlusion secondary to mediastinal fibrosis
    Morgan L Brown
    Division of Cardiovascular Surgery, Mayo Clinic, Rochester, Minnesota 55905, USA
    Ann Thorac Surg 88:233-7. 2009
    ..Our objectives were to assess the surgical strategies and outcomes of repair of pulmonary artery occlusion attributable to mediastinal fibrosis...
  76. ncbi Reoperations and survival after primary repair of congenital heart defects in children
    James L Monro
    Department of Cardiac Surgery, The General Hospital, Tremona Road, Southampton SO16 6YD, UK
    J Thorac Cardiovasc Surg 126:511-20. 2003
    ..The objective of this article is to evaluate the incidence of reoperation and the associated risk and survival after primary repair of congenital heart defects using cardiopulmonary bypass in children...
  77. doi The current strategy of repair of tetralogy of Fallot in children and adults
    Guo Wei He
    TEDA International Cardiovascular Hospital, Nankai University, Tianjin, China
    Cardiol Young 18:608-14. 2008
    ....
  78. ncbi Bovine valved xenograft in pulmonary position: medium-term follow-up with excellent hemodynamics and freedom from calcification
    Antonio F Corno
    Department of Cardiovascular Surgery, Centre Hospitalier Universitaire Vaudois, Lausanne, Switzerland
    Ann Thorac Surg 78:1382-8; discussion 1382-8. 2004
    ..This study was designed to evaluate the outcome of Contegra xenograft valved conduit (Contegra, Medtronic Inc, Minneapolis, MN)...
  79. ncbi De Vega tricuspid annuloplasty for tricuspid regurgitation in children
    K R Kanter
    Department of Surgery, Emory University School of Medicine, Atlanta, Georgia 30322, USA
    Ann Thorac Surg 72:1344-8. 2001
    ..Significant tricuspid valve regurgitation (TR) occurs with other congenital heart defects, typically after repair of right-sided obstructive lesions. Since 1991, we applied the De Vega tricuspid annuloplasty technique for TR in children...
  80. ncbi Midterm results of surgical treatment of systemic ventricular outflow obstruction in Fontan patients
    Takeshi Hiramatsu
    Department of Cardiovascular Surgery, Tokyo Women s Medical University, Heart Institute of Japan
    Ann Thorac Surg 73:855-60; discussion 860-1. 2002
    ..Because SVOO can progress after the Fontan procedure if there is morphologic obstruction, we have adopted a policy of relieving obstructions to systemic blood flow...
  81. ncbi Demonstration of coronary arteries and major cardiac vascular structures in congenital heart disease by cardiac multidetector computed tomography angiography
    Omer Faruk Dogan
    Department of Cardiovascular Surgery, Hacettepe University Medical Faculty, Ankara, Turkey
    Heart Surg Forum 10:E90-4. 2007
    ..We sought to demonstrate the feasibility and usefulness of MDCT angiography for anatomical evaluation of coronary arteries in CHD patients with suspected coronary artery anomalies...
  82. ncbi Cri du chat syndrome and congenital heart disease: a review of previously reported cases and presentation of an additional 21 cases from the Pediatric Cardiac Care Consortium
    Christine Hills
    Department of Pediatrics, University of Minnesota, Minneapolis, Minnesota, USA
    Pediatrics 117:e924-7. 2006
    ....
  83. ncbi Endovascular stent implantation in the pulmonary arteries of infants and children without the use of a long vascular sheath
    Robert H Pass
    Division of Pediatric Cardiology, Department of Pediatrics, Children s Hospital of New York New York Presbyterian Hospital, Columbia University College of Physicians and Surgeons, New York, New York 10032, USA
    Catheter Cardiovasc Interv 55:505-9. 2002
    ..5) and median weight was 11.8 kg (range, 4.6-65). Patient diagnoses were tetralogy of Fallot (five), double outlet right ventricle (three), branch peripheral pulmonary artery stenosis (two), single ventricle s/p cavopulmonary shunt (..
  84. ncbi One-stage total repair of aortic arch anomaly using regional perfusion
    Hong Gook Lim
    Department of Thoracic and Cardiovascular Surgery, Sejong General Hospital, Sejong Heart Institute, Bucheon, Republic of Korea
    Eur J Cardiothorac Surg 31:242-8. 2007
    ..Deep hypothermic circulatory arrest prolongs myocardial ischemia and might induce cerebral and myocardial dysfunction...
  85. ncbi [Follow-up of patients treated by conduits between the right ventricle and the pulmonary artery]
    S Chraibi
    , , , Suisse
    Arch Mal Coeur Vaiss 93:527-32. 2000
    ..of the pulmonary outflow tract (N = 22), tetralogy of Fallot (N = 16), truncus arteriosus (N = 9), double outlet right ventricle with pulmonary stenosis or atresia (N = 8) and agenesis of the pulmonary valve with pulmonary stenosis ..
  86. ncbi Repair of conotruncal abnormalities with the use of the valved conduit: improved early and midterm results with the cryopreserved homograft
    J M Pearl
    Department of Surgery, University of California, Los Angeles Medical Center 90024
    J Am Coll Cardiol 20:191-6. 1992
    ..1%) after a mean follow-up interval of 93 months, and 8 patients required reoperation for conduit obstruction. Among 102 homograft recipients there were two late deaths (1.9%).(ABSTRACT TRUNCATED AT 250 WORDS)..
  87. ncbi Coronary artery embryogenesis in cardiac defects induced by retinoic acid in mice
    Anna Ratajska
    Department of Pathological Anatomy, Medical University of Warsaw, Warsaw, Poland
    Birth Defects Res A Clin Mol Teratol 73:966-79. 2005
    ..Although normal coronary artery embryogenesis is well described in the literature, little is known about the development of coronary vessels in abnormal hearts...
  88. ncbi The Contegra conduit in the right ventricular outflow tract induces supravalvular stenosis
    Bart Meyns
    Department of Cardiac Surgery, University Hospital Gasthuisberg, Herestraat 49, 3000 Leuven, Belgium
    J Thorac Cardiovasc Surg 128:834-40. 2004
    ..We sought to evaluate the incidence and nature of pulmonary stenosis after implantation of the bovine jugular vein graft (Contegra; Medtronic, Inc, Minneapolis, Minn) in the right ventricular outflow tract...
  89. ncbi Determinants of right ventricular outflow tract conduit longevity: a multinational analysis
    Marieke B A Niemantsverdriet
    Pediatric Cardiology, Center for Congenital Abnormalities of Heart, Amsterdam Leiden, The Netherlands
    Congenit Heart Dis 3:176-84. 2008
    ..We compared two diverse surgical centers with considerable practice variation in Europe and the United States to identify modifiable risk factors that can increase conduit longevity...
  90. ncbi Primary repair for aortic arch obstruction associated with ventricular septal defect
    Martin Kostelka
    Klinik fur Herzchirurgie, Universitat Leipzig, Herzzentrum, Leipzig, Germany
    Ann Thorac Surg 78:1989-93; discussion 1993. 2004
    ....
  91. ncbi Management and outcome of patients with abnormal ventriculo-arterial connections and mitral valve cleft
    Alain Fraisse
    Service de Cardiologie Pediatrique, Hopital Necker Enfants Malades, Paris, France
    Ann Thorac Surg 74:786-91. 2002
    ..It may cause outflow obstruction, mitral regurgitation, and complicate biventricular repair...
  92. ncbi [Results of surgical treatment of 1,387 infants under 6 months of age with congenital heart disease]
    Hui Li Zhang
    Department of Cardiac Surgery, Cardiovascular Institute and Fuwai Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100037, China
    Zhonghua Er Ke Za Zhi 47:250-4. 2009
    ..To summarize and review the result of surgical repair of congenital heart disease in infants under 6 months of age...
  93. ncbi Liver and cardiac function in the long term after Fontan operation
    Sonia Camposilvan
    Department of Pediatric Hepatology, University of Padova, Padova, Italy
    Ann Thorac Surg 86:177-82. 2008
    ..Patients who underwent Fontan operation have some degree of liver disease. We aimed to assess the long-term liver and cardiac function after Fontan operation...
  94. ncbi [Cumulative incidence and distribution of congenital heart diseases in newborns in Beirut and its southern suburb (1999-2002)]
    Ghassan Chehab
    Départements de Pédiatrie, Université Libanaise, Faculté des Sciences médicales, Beyrouth, Liban
    J Med Liban 52:121-5. 2004
    ..To analyze the incidence and distribution of congenital heart diseases (CHD) in Beirut and its southern suburb...
  95. ncbi The role of Pitx2 during cardiac development. Linking left-right signaling and congenital heart diseases
    Diego Franco
    Department of Experimental Biology, University of Jaen, Jaen, Spain
    Trends Cardiovasc Med 13:157-63. 2003
    ..Ectopic Pitx2c expression in the developing myocardium correlates with double outlet right ventricle (DORV) in laterality mutants...
  96. doi The complete three-vessel view in prenatal detection of congenital heart defects
    Theera Tongsong
    Department of Obstetrics and Gynecology, Faculty of Medicine, Chiang Mai University, Chiang Mai, Thailand
    Prenat Diagn 30:23-9. 2010
    ..To evaluate the effectiveness of the complete three-vessel view (c3VV) in assessment of fetuses with prenatally diagnosed congenital heart defects (CHD)...
  97. ncbi Preoperative identification of coronary arterial anatomy in complete transposition, and outcome after the arterial switch operation
    Colin J McMahon
    Section of Pediatric Cardiology, Division of Congenital Heart Surgery, LillieFrank Abercrombie Division of Pediatric Cardiology, Texas Children s Hospital, 6621 Fannin, Houston, Texas 77030, USA
    Cardiol Young 12:240-7. 2002
    ..Perceived correlation between the coronary arterial anatomy in patients with complete transposition, and the outcome of the arterial switch procedure, has made preoperative identification of their patterns standard practice...
  98. ncbi Biventricular repair of conotruncal anomalies associated with aortic arch obstruction: 103 patients
    F Lacour-Gayet
    Pediatric Cardiac Surgery Department of Pr Cl Plancé, Marie Lannelongue Hospital, Paris Sud University, France
    Circulation 96:II-328-34. 1997
    ..Biventricular repair of conotruncal anomalies associated with aortic arch obstruction is a complex surgical procedure that combines a cardiac repair and a aortic arch reconstruction...
  99. doi Double-root translocation for double-outlet right ventricle with noncommitted ventricular septal defect or double-outlet right ventricle with subpulmonary ventricular septal defect associated with pulmonary stenosis: an optimized solution
    Shengshou Hu
    Cardiovascular Surgery Department, Fuwai Hospital, Peking Union Medical College, Chinese Academy of Medical Science, Beijing, China
    Ann Thorac Surg 89:1360-5. 2010
    ..Here we present an early result of an alternative solution for these anomalies by double-root translocation technique...
  100. ncbi Transthoracic three-dimensional echocardiography for the assessment of straddling tricuspid or mitral valves
    M Vogel
    GUCH Department, Middlesex Hospital, London, UK
    Cardiol Young 10:603-9. 2000
    ....
  101. ncbi Cardiac malformations, adrenal agenesis, neural crest defects and exencephaly in mice lacking Cited2, a new Tfap2 co-activator
    S D Bamforth
    Department of Cardiovascular Medicine, University of Oxford, Wellcome Trust Center for Human Genetics, Roosevelt Drive, Oxford, OX3 7BN UK
    Nat Genet 29:469-74. 2001
    ..As certain Tfap2 isoforms are essential in neural crest, neural tube and cardiac development, we propose that abnormal embryogenesis in mice lacking Cited2 results, at least in part, from its role as a Tfap2 co-activator...

Research Grants70

  1. Function of jumonji in cardiovascular development
    Youngsook Lee; Fiscal Year: 2002
    ..revealed that jmj mutant embryos showed cardiac malformations including ventricular septal defect, double outlet right ventricle and non- compaction of the ventricular wall (thin ventricular wall), recapitulating human congenital ..
  2. Function of jumonji in cardiovascular development
    Youngsook Lee; Fiscal Year: 2003
    ..revealed that jmj mutant embryos showed cardiac malformations including ventricular septal defect, double outlet right ventricle and non- compaction of the ventricular wall (thin ventricular wall), recapitulating human congenital ..
  3. Function of jumonji in cardiovascular development
    Youngsook Lee; Fiscal Year: 2004
    ..revealed that jmj mutant embryos showed cardiac malformations including ventricular septal defect, double outlet right ventricle and non- compaction of the ventricular wall (thin ventricular wall), recapitulating human congenital ..
  4. Function of jumonji in cardiovascular development
    Youngsook Lee; Fiscal Year: 2002
    ..revealed that jmj mutant embryos showed cardiac malformations including ventricular septal defect, double outlet right ventricle and non- compaction of the ventricular wall (thin ventricular wall), recapitulating human congenital ..
  5. Function of jumonji in cardiovascular development
    Youngsook Lee; Fiscal Year: 2005
    ..revealed that jmj mutant embryos showed cardiac malformations including ventricular septal defect, double outlet right ventricle and non- compaction of the ventricular wall (thin ventricular wall), recapitulating human congenital ..
  6. Hedgehog signaling during cardiovascular patterning in the mouse
    John Klingensmith; Fiscal Year: 2007
    ..tract development result in severe congenital heart defects such as persistent truncus arteriosus and double outlet right ventricle. Similarly, atrio-ventricular (A-V) cushions contribute to the formation of the mitral and tricuspid ..
  7. Hedgehog signaling during cardiovascular patterning in the mouse
    John Klingensmith; Fiscal Year: 2009
    ..tract development result in severe congenital heart defects such as persistent truncus arteriosus and double outlet right ventricle. Similarly, atrio-ventricular (A-V) cushions contribute to the formation of the mitral and tricuspid ..
  8. Hedgehog signaling during cardiovascular patterning in the mouse
    JOHN A KLINGENSMITH; Fiscal Year: 2010
    ..tract development result in severe congenital heart defects such as persistent truncus arteriosus and double outlet right ventricle. Similarly, atrio-ventricular (A-V) cushions contribute to the formation of the mitral and tricuspid ..
  9. GENE THAT CONTROLS LATERALITY IN EARLY CARDIOGENESIS
    FRANCIS MANASEK; Fiscal Year: 1990
    ..frequency of cardiac malformations such as complete AV canal, transposition of the great arteries, and double outlet right ventricle. We will also utilize an experimental avian model...
  10. Neural Crest Modulates FGF Signaling in the Pharynx
    MARGARET KIRBY; Fiscal Year: 2006
    ..The malalignment defects are a component of two conotruncal malformations called double outlet right ventricle and tetralogy of Fallot...
  11. CONGENITAL HEART MALFORMATIONS AND SITUS INVERSUS
    WILLIAM LAYTON; Fiscal Year: 1980
    ..including: common persistant atrio-ventricular canal, transposition of the great arteries, double outlet right ventricle, anomalous pulmonic and systemic venous return and atrial isomerism...
  12. SYMBOLIC LOGIC ANALYSIS OF CONGENITAL HEART DISEASE
    GROVER HUTCHINS; Fiscal Year: 1980
    ..Similar invistigations are currently in progress for ventricular septal defect, double outlet right ventricle, and persistent left superior vena cava...
  13. NEUROFIBROMIN, NEURAL CREST AND CARDIAC DEVELOPMENT
    Jonathan Epstein; Fiscal Year: 2004
    ..The cardiac defects include pulmonic stenosis, double outlet right ventricle and thinned myocardium, defects that are seen in other mouse and chick models of cardiac neural crest-..
  14. NEUROFIBROMIN, NEURAL CREST AND CARDIAC DEVELOPMENT
    Jonathan Epstein; Fiscal Year: 2006
    ..The cardiac defects include pulmonic stenosis, double outlet right ventricle and thinned myocardium, defects that are seen in other mouse and chick models of cardiac neural crest-..
  15. NEUROFIBROMIN, NEURAL CREST AND CARDIAC DEVELOPMENT
    Jonathan Epstein; Fiscal Year: 2007
    ..The cardiac defects include pulmonic stenosis, double outlet right ventricle and thinned myocardium, defects that are seen in other mouse and chick models of cardiac neural crest-..
  16. NEUROFIBROMIN, NEURAL CREST AND CARDIAC DEVELOPMENT
    Jonathan Epstein; Fiscal Year: 2005
    ..The cardiac defects include pulmonic stenosis, double outlet right ventricle and thinned myocardium, defects that are seen in other mouse and chick models of cardiac neural crest-..
  17. Mutational Analysis of TGFbeta Function
    Thomas Doetschman; Fiscal Year: 2004
    ..beta 2 (TGFbeta2) knockout mice have congenital heart abnormalities that are commonly found in humans: double outlet right ventricle, double inlet left ventricle, ventral and atrial septal defects, and valve defects...
  18. Mutational Analysis of TGFbeta Function
    Thomas Doetschman; Fiscal Year: 2003
    ..beta 2 (TGFbeta2) knockout mice have congenital heart abnormalities that are commonly found in humans: double outlet right ventricle, double inlet left ventricle, ventral and atrial septal defects, and valve defects...
  19. Mutational Analysis of TGFbeta Function
    Thomas Doetschman; Fiscal Year: 2002
    ..beta 2 (TGFbeta2) knockout mice have congenital heart abnormalities that are commonly found in humans: double outlet right ventricle, double inlet left ventricle, ventral and atrial septal defects, and valve defects...
  20. Mutational Analysis of TGFbeta Function
    Thomas Doetschman; Fiscal Year: 2005
    ..beta 2 (TGFbeta2) knockout mice have congenital heart abnormalities that are commonly found in humans: double outlet right ventricle, double inlet left ventricle, ventral and atrial septal defects, and valve defects...
  21. Mutational Analysis of TGFbeta Function
    Thomas Doetschman; Fiscal Year: 2001
    ..beta 2 (TGFbeta2) knockout mice have congenital heart abnormalities that are commonly found in humans: double outlet right ventricle, double inlet left ventricle, ventral and atrial septal defects, and valve defects...
  22. Novel regulator of Notch signaling in determination of left-right asymmetry durin
    Yoichi Kato; Fiscal Year: 2010
    ..present with complications of congenital heart disease including transposition of the great arteries, double outlet right ventricle, double inlet left ventricle, atrioventricular septal defects and total anomalous pulmonary venous ..
  23. Neural Crest Modulates FGF Signaling in the Pharynx
    MARGARET KIRBY; Fiscal Year: 2006
    ..arterial pole and this results in conotruncal malalignment defects such as overriding aorta and double outlet right ventricle. Our preliminary results indicate that the failure of myocardial addition to the outflow tract and ..
  24. Modeling conotruncal malformations in zebrafish embryos
    MARGARET KIRBY; Fiscal Year: 2007
    ..arterial pole of the heart leads to congenital defects classified as conotruncal malformations such as double outlet right ventricle and tetralogy of Fallot...
  25. Neural Crest Modulates FGF Signaling in the Pharynx
    MARGARET KIRBY; Fiscal Year: 2007
    ..arterial pole and this results in conotruncal malalignment defects such as overriding aorta and double outlet right ventricle. Our preliminary results indicate that the failure of myocardial addition to the outflow tract and ..
  26. Modeling conotruncal malformations in zebrafish embryos
    MARGARET KIRBY; Fiscal Year: 2009
    ..arterial pole of the heart leads to congenital defects classified as conotruncal malformations such as double outlet right ventricle and tetralogy of Fallot...
  27. Neural Crest Modulates FGF Signaling in the Pharynx
    MARGARET KIRBY; Fiscal Year: 2009
    ..arterial pole and this results in conotruncal malalignment defects such as overriding aorta and double outlet right ventricle. Our preliminary results indicate that the failure of myocardial addition to the outflow tract and ..
  28. Modeling conotruncal malformations in zebrafish embryos
    Margaret Loewy Kirby; Fiscal Year: 2010
    ..arterial pole of the heart leads to congenital defects classified as conotruncal malformations such as double outlet right ventricle and tetralogy of Fallot...
  29. Neural Crest Modulates FGF Signaling in the Pharynx
    Margaret Loewy Kirby; Fiscal Year: 2010
    ..arterial pole and this results in conotruncal malalignment defects such as overriding aorta and double outlet right ventricle. Our preliminary results indicate that the failure of myocardial addition to the outflow tract and ..
  30. Neural Crest Modulates FGF Signaling in the Pharynx
    MARGARET KIRBY; Fiscal Year: 2009
    ..arterial pole and this results in conotruncal malalignment defects such as overriding aorta and double outlet right ventricle. Our preliminary results indicate that the failure of myocardial addition to the outflow tract and ..
  31. REGULATION OF CARDIAC MORPHOGENESIS
    ANTHONY FIRULLI; Fiscal Year: 2007
    ..Specific Aim 2 will determine what motifs distinguish Handl and Hand2 function and how Handl and 2 functionally interact with Hey2. ..
  32. REGULATION OF CARDIAC MORPHOGENESIS
    ANTHONY FIRULLI; Fiscal Year: 2009
    ..Specific Aim 2 will determine what motifs distinguish Handl and Hand2 function and how Handl and 2 functionally interact with Hey2. ..
  33. REGULATION OF CARDIAC MORPHOGENESIS
    Anthony B Firulli; Fiscal Year: 2011
    ..Specific Aim 2 will determine what motifs distinguish Handl and Hand2 function and how Handl and 2 functionally interact with Hey2. ..
  34. MYOCARDIALIZATION AND EPICARDIALIZATION OF CUSHION TISSUE
    A Wessels; Fiscal Year: 2000
    ..to perturbation of myocardialization resulting in a specific set of congenital malformations including double outlet right ventricle (DORV) and double inlet left ventricle (DILV), and (2) to describe the myocardialization-induced ..
  35. THE GENETIC ARCHITECTURE OF CONGENITAL HEART DISEASE
    Patrick Y Jay; Fiscal Year: 2011
    ..normal hearts, simple atrial and ventricular septal defects (ASD, VSD), and complex malformations like double outlet right ventricle. To characterize the sources of phenotypic variability, we crossed inbred mouse strains to show how ..
  36. Neurofibromin, Ras & NFAT in Cardiovascular Development
    FRAZ ISMAT; Fiscal Year: 2008
    ..Together this training program will prepare me for independent investigation, lead to a better understanding of cardiovascular abnormalities in neurofibromatosis, and address fundamental issues of cardiovascular development. ..
  37. Neurofibromin, Ras & NFAT in Cardiovascular Development
    FRAZ ISMAT; Fiscal Year: 2007
    ..Together this training program will prepare me for independent investigation, lead to a better understanding of cardiovascular abnormalities in neurofibromatosis, and address fundamental issues of cardiovascular development. ..
  38. Regulation of Heart Function by a Gata4-Fog2 transcriptional complex
    WILLIAM TSWENCHING PU; Fiscal Year: 2010
    ..This knowledge may lead to improved therapies that can reverse gene expression changes and disease course in heart disease. ..
  39. Epicardial Contribution to the Developing and Postnatal Heart
    William Pu; Fiscal Year: 2009
    ..These experiments will prepare the foundation for future studies on potential therapeutic uses of epicardium as a source of cells and signals to treat heart failure. ..
  40. HAND GENES IN CARDIAC DEVELOPMENT
    William Pu; Fiscal Year: 2004
    ..This experience, and the information generated in this plan, will launch the applicant's career as an independent investigator. ..
  41. Analysis of a Novel Homeobox gene in CV Development
    Jonathan Epstein; Fiscal Year: 2009
    ..We hope to determine if HDAC2 and INPP5F represent new targets for the development of specific therapies for heart disease. ..
  42. PAX3 REGULATION OF CARDIAC CONOTRUNCAL DEVELOPMENT
    Jonathan Epstein; Fiscal Year: 1999
    ..Together, these approaches should begin to unravel the molecular cascades involved in the neural crest contribution to cardiac development and will provide candidate genes to examine as causes of human congenital heart disease. ..
  43. LBX2 AND UROGENITAL DEVELOPMENT
    Jonathan Epstein; Fiscal Year: 2002
    ....
  44. NEUROFIBROMIN, NEURAL CREST AND CARDIAC DEVELOPMENT
    Jonathan Epstein; Fiscal Year: 2001
    ..3. To inactivate the Nf1 gene selectively in neural crest cells and separately in myocardial cells using tissue specific promoters and a Cre/lox strategy. ..
  45. PAX3 REGULATION OF CARDIAC CONOTRUNCAL DEVELOPMENT
    Jonathan Epstein; Fiscal Year: 2001
    ..Together, these approaches should begin to unravel the molecular cascades involved in the neural crest contribution to cardiac development and will provide candidate genes to examine as causes of human congenital heart disease. ..
  46. Epicardial Contribution to the Developing and Postnatal Heart
    WILLIAM TSWENCHING PU; Fiscal Year: 2010
    ..These experiments will prepare the foundation for future studies on potential therapeutic uses of epicardium as a source of cells and signals to treat heart failure. ..
  47. LBX2 AND UROGENITAL DEVELOPMENT
    Jonathan Epstein; Fiscal Year: 2001
    ....
  48. The Genetic Etiology of Left-Sided Cardiac Defects
    Elizabeth Goldmuntz; Fiscal Year: 2005
    ..In addition, these investigations will lead to future studies that assess the relationship of genotype to clinical outcome, and allow us to improve upon our clinical management accordingly. ..
  49. NEUROFIBROMIN, NEURAL CREST AND CARDIAC DEVELOPMENT
    Jonathan A Epstein; Fiscal Year: 2010
    ....
  50. LBX2 AND UROGENITAL DEVELOPMENT
    Jonathan Epstein; Fiscal Year: 2000
    ....
  51. The Genetic Etiology of Left-Sided Cardiac Defects
    Elizabeth Goldmuntz; Fiscal Year: 2003
    ..In addition, these investigations will lead to future studies that assess the relationship of genotype to clinical outcome, and allow us to improve upon our clinical management accordingly. ..
  52. Epicardial Contribution to the Developing and Postnatal Heart
    William Pu; Fiscal Year: 2009
    ..These experiments will prepare the foundation for future studies on potential therapeutic uses of epicardium as a source of cells and signals to treat heart failure. ..
  53. Genome-wide discovery of alternative splicing in human heart failure
    William Pu; Fiscal Year: 2008
    ..This understanding will lead to new hypotheses about how heart failure develops and about how we might improve its treatment. [unreadable] [unreadable] [unreadable]..
  54. The Genetic Etiology of Left-Sided Cardiac Defects
    Elizabeth Goldmuntz; Fiscal Year: 2006
    ..In addition, these investigations will lead to future studies that assess the relationship of genotype to clinical outcome, and allow us to improve upon our clinical management accordingly. [unreadable] [unreadable]..
  55. Bridge to neonatal Fontan repair of single ventricle
    Mark Rodefeld; Fiscal Year: 2006
    ..abstract_text> ..
  56. 2005 NNFF Consortium for NF1, NF2 and Schwannomatosis
    Jonathan Epstein; Fiscal Year: 2005
    ....
  57. The Genetic Etiology of Left-Sided Cardiac Defects
    Elizabeth Goldmuntz; Fiscal Year: 2004
    ..In addition, these investigations will lead to future studies that assess the relationship of genotype to clinical outcome, and allow us to improve upon our clinical management accordingly. ..
  58. PAX3 REGULATION OF CARDIAC CONOTRUNCAL DEVELOPMENT
    Jonathan Epstein; Fiscal Year: 2004
    ..Aim 3 examines the role of neural crest during development of the conduction system, and specifically examines Pax3 regulation of DCT. ..
  59. The Genetic Etiology of Conotruncal Cardiac Defects
    Elizabeth Goldmuntz; Fiscal Year: 2006
    ..With this data, the impact of genotype on clinical outcome can be assessed and improved management strategies devised for the future. [unreadable] [unreadable]..
  60. PAX3 REGULATION OF CARDIAC CONOTRUNCAL DEVELOPMENT
    Jonathan Epstein; Fiscal Year: 2007
    ..Aim 3 examines the role of neural crest during development of the conduction system, and specifically examines Pax3 regulation of DCT. [unreadable] [unreadable]..
  61. The Genetic Etiology of Left-Sided Cardiac Defects
    Elizabeth Goldmuntz; Fiscal Year: 2007
    ..In addition, these investigations will lead to future studies that assess the relationship of genotype to clinical outcome, and allow us to improve upon our clinical management accordingly. [unreadable] [unreadable]..
  62. Genome-wide discovery of alternative splicing in human heart failure
    William Pu; Fiscal Year: 2007
    ..This understanding will lead to new hypotheses about how heart failure develops and about how we might improve its treatment. [unreadable] [unreadable] [unreadable]..
  63. NEUROFIBROMIN, NEURAL CREST AND CARDIAC DEVELOPMENT
    Jonathan Epstein; Fiscal Year: 2008
    ..The cardiac defects include pulmonic stenosis, double outlet right ventricle and thinned myocardium, defects that are seen in other mouse and chick models of cardiac neural crest-..
  64. Analysis of a Novel Homeobox gene in CV Development
    Jonathan Epstein; Fiscal Year: 2008
    ..We hope to determine if HDAC2 and INPP5F represent new targets for the development of specific therapies for heart disease. ..
  65. PAX3 REGULATION OF CARDIAC CONOTRUNCAL DEVELOPMENT
    Jonathan Epstein; Fiscal Year: 2008
    ..Aim 3 examines the role of neural crest during development of the conduction system, and specifically examines Pax3 regulation of DCT. [unreadable] [unreadable]..
  66. The Genetic Etiology of Conotruncal Cardiac Defects
    Elizabeth Goldmuntz; Fiscal Year: 2004
    ..With this data, the impact of genotype on clinical outcome can be assessed and improved management strategies devised for the future. ..
  67. The Genetic Etiology of Conotruncal Cardiac Defects
    Elizabeth Goldmuntz; Fiscal Year: 2005
    ..With this data, the impact of genotype on clinical outcome can be assessed and improved management strategies devised for the future. ..
  68. Cavopulmonary Assist: Circulatory Support for the Univentricular Fontan Circulat
    Mark D Rodefeld; Fiscal Year: 2010
    ..This will dramatically improve the healthcare of children and adults born with single ventricle heart disease. ..
  69. The Genetic Etiology of Conotruncal Cardiac Defects
    Elizabeth Goldmuntz; Fiscal Year: 2007
    ..With this data, the impact of genotype on clinical outcome can be assessed and improved management strategies devised for the future. [unreadable] [unreadable]..