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Cardiovascular Surgery at The Children’s Hospital of Philadelphia
Author’s Accepted Manuscript Cardiovascular Surgery at The Children′s Hospital of Philadelphia Thomas L. Spray, Stephanie Fuller, Christopher E. Mascio, J. William Gaynor www.elsevier.com/locate/buildenv
PII: DOI: Reference:
S1043-0679(16)30160-5 http://dx.doi.org/10.1053/j.semtcvs.2016.11.003 YSTCS924
To appear in: Seminars in Thoracic and Cardiovascular Surgery Cite this article as: Thomas L. Spray, Stephanie Fuller, Christopher E. Mascio and J. William Gaynor, Cardiovascular Surgery at The Children′s Hospital of Philadelphia, Seminars in Thoracic and Cardiovascular Surgery, http://dx.doi.org/10.1053/j.semtcvs.2016.11.003 This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting galley proof before it is published in its final citable form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.
Cardiovascular Surgery at The Children’s Hospital of Philadelphia Thomas L. Spray, M.D., Stephanie Fuller, M.D., Christopher E. Mascio, M.D, and J. William Gaynor, M.D.
Division of Cardiothoracic Surgery, The Children’s Hospital of Philadelphia, Philadelphia, Pennsylvania
Corresponding Author: Thomas L. Spray, M.D. Division of Cardiothoracic Surgery The Children’s Hospital of Philadelphia 34th and Civic Center Boulevard Philadelphia, PA 19104, USA Phone: 215-590-2708 / Fax: 215-590-2715 E-mail: [email protected]
Abstract Surgery for congenital heart disease at the Children’s Hospital of Philadelphia (CHOP) began in the earliest days of cardiac surgery. In a rapidly advancing field, surgeons at CHOP are recognized as innovators in the field. The Division of Cardiac Surgery is dedicated to providing outstanding clinical care, educating the next generation of congenital cardiac surgeons, and advancing the specialty through clinical and translational research.
The Children’s Hospital of Philadelphia (CHOP) was founded in 1855 as the nation’s first hospital dedicated exclusively to the care of children. Drs. Francis West Lewis, Thomas Hewson Bache and R.A.F. Penrose were Philadelphia physicians who had traveled to London and visited the newly established Great Ormond Street Hospital for Sick Children. The physicians returned to Philadelphia to establish the nation’s first pediatric hospital which was located on Blight St. in downtown Philadelphia with 12 beds. The hospital treated sixty-three patients in its first year. In 1866, CHOP moved to its second location at 22nd Street between Walnut St. and Locust St. and beds increased to thirty-five. In 1873, the first resident physician was appointed to CHOP. His duties included compounding of all prescriptions. And in 1895, the Ingersoll Training School for Nurses, the birthplace of pediatric nursing in America, was established and operated until 1945. The Department for the Prevention of Disease was established in 1914, and was the first such program in the nation. The second major relocation of the hospital to 18th St. and Bainbridge St. occurred in 1916 and expanded the bed capacity significantly. (Figure 1) Joseph Stokes Jr. was appointed as Physician-In-Chief at CHOP in 1929. Dr. Stokes is credited with transforming CHOP into a world leader in teaching and research. The Joseph Stokes Research Institute was founded to honor his memory. Amongst early interventions, the original whooping cough vaccine was developed at CHOP in 1936. This was the first in a series of childhood vaccines, including those for influenza and mumps, pioneered at CHOP by Drs. Werner and Gertrude Henle, which have had worldwide impact on prevention of childhood diseases. In 1938, the first closed incubator was developed at CHOP for the use in newborns and in 1940 the first professional play program for patients, which developed into Child Life Services was initiated at The Children’s Hospital. The institution gained national prominence when Dr. C. Everett Koop joined the medical staff in 1946 and by 1948 was appointed as the Surgeon-InChief of The Children’s Hospital. He was internationally recognized for his expertise in separating conjoined twins. Dr. Koop went on to become the nation’s Surgeon General under President Ronald Reagan. In 1962, the first Neonatal Intensive Care Unit in the nation was established at CHOP and the first Pediatric Intensive Care Unit followed in 1967. In 1963, Stanley Plotkin developed the Rubella vaccine and the initial clinical trials were conducted at CHOP. A major advancement in the treatment of congenital heart disease occurred in 1965 when Dr. William Rashkind, Chief of Pediatric Cardiology at CHOP, developed the balloon atrial septostomy thus permitting improvement in cyanosis and stabilization in patients with transposition of the great arteries with intact ventricular septum.1 (Figure 2) In 1974, the hospital made its third move to its current location at 34th Street and Civic Center Boulevard, which adjacent to the old Philadelphia General Hospital, an institution that was recognized for the pioneering procedures for closed mitral valvuloplasty by Dr. Charles Bailey. (Figure 3) Since the move to West Philadelphia, The Children’s Hospital of Philadelphia has expanded significantly with the addition of several research buildings, a new seven hundred thousand square foot ambulatory care facility, multiple additions to the main hospital, new specialty care ambulatory care facilities and many pediatric practices. (Figure 4) The Children’s Hospital of Philadelphia is now a health system providing care to thousands of patients annually from the Delaware Valley, the United States and internationally. The CHOP Care Network consists of 16 Specialty Care Centers, 4 Ambulatory Surgery Centers, 31 Primary Care practices, 15 community hospital affiliations, and 3 after-hours urgent care facilities. The Children’s Hospital of Philadelphia has been recognized by the US News and World Report as one of the top two pediatric institutions in the United States and is in the top four of nine of the ten specialty rankings. The continued emphasis on research led to CHOP being designated as a Human
Genome Center by the National Institutes of Health. CHOP researchers were instrumental in the mapping of chromosome 22, which was completed in 1999. In 1995, the Center for Fetal Diagnosis and Treatment (CFDT) was established which provides fetal surgery and intervention for complex congenital malformations and cardiac diseases. A multi-billion dollar expansion of the facilities was undertaken in 2001, and has led to a large medical campus in West Philadelphia encompassing the main hospital and ambulatory care building, several research buildings for the Stokes Institute of Research and the Children’s Seashore House, a rehabilitation facility which was relocated from the Atlantic Shore to the main campus. Surgery for congenital heart disease at CHOP began with Dr. Julian Johnson who was the 43 President of the American Association for Thoracic Surgery.2, 3 (Figure 5) Dr. Johnson completed his surgical training in Philadelphia. In the Pacific Theater, Johnson served in the 20th General Hospital which had been organized from the Hospital of the University of Pennsylvania by Drs. I.S. Ravdin and Thomas Fitzhugh. He returned to Philadelphia after the war and eventually served as Chief of Cardiothoracic Surgery at The University of Pennsylvania until retirement in 1973. Early in his career, Dr. Johnson developed an interest in the emerging field of congenital heart surgery.4, 5 He was an early adopter of the innovative techniques being introduced to treat the problem of congenital heart disease; including ligation of patent ductus arteriosus, repair of coarctation of the aorta, and the Blalock-Taussig shunt for palliation of tetralogy of Fallot. He quickly mastered the procedure for Blalock-Taussig shunt and was referred many patients by Dr. Blalock.6 After the first successful use of cardiopulmonary bypass by John Gibbon in 1953, Johnson initiated the open heart surgery programs at both the University of Pennsylvania and CHOP. By 1964, he with his associates, Charles Kirby and Gordon Danielson, reported a series from both institutions of 100 consecutive open heart surgeries with a mortality of ten percent using the newly developed Mayo-Gibbon pump.7 rd
In 1966 John Waldhausen joined the faculty at The University of Pennsylvania under Dr. Johnson’s leadership. (Figure 6) Dr. Koop, the founding Chief of Surgery at CHOP, recruited Dr. Waldhausen to develop an independent dedicated pediatric cardiac surgery service at The Children’s Hospital of Philadelphia. Dr. Waldhausen developed open cardiac repairs in children and then extended these repairs into infancy. By 1971, he had reported use of a disc oxygenator for open cardiac repair in congenital heart disease with a survival rate of 78 percent. With Dr. William Rashkind, Dr. Waldhausen reported an operative survival rate of 81 percent patients for transposition of the great arteries in 1971, with a 100 percent survival rate for 18 patients with simple transposition.8, 9 Dr. Waldhausen was known for technical expertise and his excellent results in infant pediatric cardiac surgery. He developed an international reputation as one of the leaders in congenital heart surgery. Dr. Waldhausen is perhaps best remembered by congenital heart surgeons for the introduction of the subclavian flap aortoplasty for coarctation of the aorta.10-12 In 1969, Dr. Waldhausen was offered the founding Chairman of Surgery at the newly formed Milton Hershey Medical Center at the Pennsylvania State University College of Medicine and left Philadelphia for Hershey, Pennsylvania. He served as the 72nd President of the American Association for Thoracic Surgery.13, 14 After Dr. Waldhausen’s departure, the pediatric cardiac surgical program at The Children’s Hospital of Philadelphia was led by Dr. Eoin Aberdeen. (Figure 7) Dr. Aberdeen had been a consultant cardiothoracic surgeon at the Hospital for Sick Children at Great Ormond Street in London since 1963. His work focused on open heart surgery in infants and small children and he attained international fame for his work on transposition of the great arteries.15-18 In 1971, he
moved to Philadelphia to assume the Chief of Cardiac Surgery position at CHOP where he remained chief until 1974. During his time at CHOP, Dr. Aberdeen was instrumental in founding The Congenital Heart Surgeons Society, a prestigious group of North American heart surgeons which was based on the European Pediatric Cardiac Surgery Club which Aberdeen was an active member. With the exit of Eoin Aberdeen, cardiac surgery at CHOP was performed by adult cardiac surgeons with an interest in congenital heart disease from The University of Pennsylvania including L. Henry Edmunds (past Editor of The Annals of Thoracic Surgery) , Richard Edie, and Lawrence Stephenson. (Figures 8 and 9) James Alexander was a congenital heart surgeon at CHOP for a brief period of time prior to moving to the University of Florida in Gainesville. A major advance in the cardiac surgical program at The Children’s Hospital of Philadelphia occurred in 1984 when William I. Norwood was recruited from Boston Children’s Hospital to become the Chief of Cardiac Surgery. (Figure 10). Dr. Norwood brought with him an extensive knowledge of complex congenital surgery in newborns and specific expertise in the treatment of hypoplastic left heart syndrome (HLHS) for which the first stage operation is now known as the Norwood Operation.19 The cardiac surgical program expanded under Dr. Norwood with dedicated operating rooms and an intensive care unit specifically for the care of patients with congenital heart disease. A dedicated Division of Cardiac Anesthesia was established in 1984 under the leadership of Dr. Susan C. Nicolson. Dr. Norwood was joined by Dr. John Pigott in 1985 and by Dr. Marshall L. Jacobs in 1990. During this time, CHOP became a major center for the treatment of HLHS, performing more surgical procedures for this condition than any other institution in the world. Many of the techniques now considered standard for management of infants with complex single ventricle heart defects were introduced and refined during this time. 20-22
In 1994, Dr. Norwood left CHOP to begin an international practice in cardiac surgery in Genolier, Switzerland and Dr. Thomas L. Spray was recruited from Washington University in St. Louis and the St. Louis Children’s Hospital to become the Chief of Cardiac Surgery. (Figure 11) Prior to moving to CHOP, Dr. Spray started the first dedicated pediatric lung transplant program at Washington University in 1990.23, 24 Members of the Division of Cardiac Surgery since 1995 have included Tom R. Karl, M.D., William M. DeCampli, M.D., and Peter J. Gruber, M.D, Ph.D. Current members of the Division are J. William Gaynor, M.D., Stephanie Fuller, M.D. and Christopher E. Mascio, M.D. (Figure 12) Dr. Spray is the holder of the Alice Langdon Warner Endowed Chair in Pediatric Cardiac Surgery. Dr. Gaynor is the holder of the Daniel M. Tabas Endowed Chair in Pediatric Cardiac Surgery. Dr. Spray was the 89th President of the American Association for Thoracic Surgery. He is the third Chief of Cardiac Surgery at CHOP to hold this prestigious position. Since Dr. Spray’s appointment, the cardiac surgery program has continued to expand and evolve. In 1994, a new Cardiac Intensive Care Unit (CICU) was opened as a dedicated unit for the care of cardiac patients. This was one of the few such dedicated units in the nation at that time. After his appointment, the cardiac transplantation program was expanded and a lung and heart-lung transplantation program was started. In addition, an active mechanical circulatory support program was begun. In 1998, The Cardiac Center was established integrating cardiology, cardiothoracic surgery, cardiac anesthesia and cardiac nursing to optimize the care of patients with congenital heart disease. A combined Adult Congenital Heart Disease Program with the Hospital of the University of Philadelphia was begun in 2004. Drs. Fuller and Mascio
are the primary surgeons for this rapidly growing program. The Cardiac Operative and Imaging Complex (COIC) opened in 2006. This facility incorporates 2 dedicated cardiac operating rooms, 2 catheterization rooms, a hybrid suite and a MRI unit. It is located on the same floor as the CICU, facilitating patient transfer. The Garbose Family Special Delivery Unit (SDU) was established as part of the CFDT in 2008 as the world’s first birthing unit within a pediatric hospital dedicated to healthy mothers carrying babies with serious and life-threatening birth defects. It is located immediately adjacent to the COIC and CICU. For newborns with complex heart defects, this allows immediate access to care and the mothers are able to remain close to their child. The cardiac surgery program at CHOP remains one of the highest volume centers in North America performing over 550 open cardiac procedures and over 800 total cases each year. These include the full spectrum of cardiac surgery including fetal cardiac interventions, neonatal reconstruction, thoracic organ transplantation, mechanical circulatory support and management of the adult with congenital heart disease. Recently, surgeons at CHOP performed the first successful in utero surgical resection of an intra-pericardial teratoma in a 24 week gestational age fetus with survival to term.25 (Figure 13) Since Dr. Norwood’s arrival, there has been a focus on management of hypoplastic left heart syndrome and other forms of complex single ventricle heart defects. Under Dr. Spray’s leadership, CHOP remains a referral center for these complex infants and has the world’s largest experience with HLHS.26-34 With improved survival for these vulnerable children has come an increased focus on long term outcomes. The Single Ventricle Survivorship Program was established in 2011 and currently follows one of the largest cohorts of children, adolescents and young adults with single ventricle congenital heart disease in the world.35 Many of these children will ultimately require heart transplantation. Surgeons at CHOP have continued to expand the use of mechanical circulatory support in these patients; including the first successful use of the total artificial heart after the Fontan procedure and expanded utilization of ventricular assist devices in toddlers.36 (Figure 14) Education and research are priorities at CHOP. Training the next generation of congenital heart surgeons is of utmost importance. Since 1995, 43 surgical trainees (both from North America and overseas) have undertaken a fellowship at CHOP. The Congenital Heart Surgery Fellowship was approved by the Accreditation Council for Graduate Medical Education (ACGME) in 2008 and there have been 7 accredited fellows. There is an active clinical and translational research program in the Division of Cardiac Surgery. Research interests include genetic modifiers of outcome and neurodevelopmental outcomes after surgery for congenital heart defects. These efforts have provided strong evidence that patient factors, especially genetic variants, are important determinants of the risk of death and developmental disability after surgery.37-40 We have identified and validated an association of apo-lipoprotein E (APOE) with neurodevelopmental disability in this population.41, 42 In 2014, the Center for Fetal Neuroprotection and Neuroplasticity was established at CHOP and we initiated the world’s first randomized clinical trial of fetal neuroprotection to reduce brain injury and improve neurodevelopmental outcomes for the fetus with congenital heart disease. The Division of Cardiac Surgery at CHOP has a proud tradition of innovation and outstanding clinical care. Surgery for congenital heart disease at the Children’s Hospital of Philadelphia (CHOP) began in the earliest days of cardiac surgery. The Division of Cardiac Surgery is dedicated to providing outstanding clinical care, educating the next generation of
congenital cardiac surgeons, and advancing the specialty through clinical and translational research. References 1. Rashkind WJ and Miller WW. Creation of an atrial septal defect without thoracotomy. A palliative approach to complete transposition of the great arteries. JAMA. 1966;196:991-2. 2. Johnson J. A surgeon and something more. Journal of Thoracic & Cardiovascular Surgery. 1963;46:141-149. 3. Gaynor JW. Historical perspectives of The American Association for Thoracic Surgery: Julian Johnson (1906-1987). J Thorac Cardiovasc Surg. 2011;142:493-5. 4. Ash R, Johnson J, Koop CE, Friedman S and Rashkind W. Cardiovascular surgery in a children's hospital. II. Cyanotic lesions. A review of 193 operations. J Pediatr. 1959;54:348-62. 5. Ash R, Johnson J, Koop CE, Friedman S and Rashkind W. Cardiovascular surgery in a children's hospital. I. Acyanotic lesions; a review of 241 operations. J Pediatr. 1959;54:133-42. 6. Rhoads JE. Julian Johnson 1906-1987. Transactions and Studies of the College of Physicians of Philadelphia. 1987;9:300-303. 7. Johnson J, Kirby CK, Danielson GK and Verhagen AD. An Analysis of 100 Consecutive Operations Utilizing the Mayo-Gibbon Heart-Lung Machine. Surgery. 1964;56:711-8. 8. Waldhausen JA, Pierce WS, Park CD, Rashkind WJ and Friedman S. Physiologic correction of transposition of the great arteries. Indications for and results of operation in 32 patients. Circulation. 1971;43:738-47. 9. Waldhausen JA, Pierce WS, Rashkind WJ, Miller WW and Friedman S. Total correction of transposition of the great arteries following balloon atrioseptostomy. Circulation. 1970;41:II123-9. 10. Myers JL, McConnell BA and Waldhausen JA. Coarctation of the aorta in infants: does the aortic arch grow after repair? Ann Thorac Surg. 1992;54:869-74; discussion 874-5. 11. Shumacker HB, Jr., Waldhausen J and King H. The Surgical Treatment of Coarctation of the Aorta. Bull Soc Int Chir. 1964;23:289-98. 12. Waldhausen JA and Nahrwold DL. Repair of coarctation of the aorta with a subclavian flap. J Thorac Cardiovasc Surg. 1966;51:532-3. 13. Heinle JS and Gaynor JW. Historical perspectives of The American Association for Thoracic Surgery: John Anton Waldhausen (1929-2012). J Thorac Cardiovasc Surg. 2014;148:381-6. 14. Waldhausen JA. The Association at seventy-five: the challenge of the future. J Thorac Cardiovasc Surg. 1992;104:1183-94. 15. Aberdeen E. Correction of uncomplicated cases of transposition of the great arteries. Br Heart J. 1971;33:Suppl:66-8. 16. Deverall P, Lincoln N, Aberdeen E and al e. Aortopulmonary window. J Thorac Cardiovasc Surg. 1969;57:479. 17. Stark J, Tynan M, Tatooles CJ, Aberdeen E and Waterston DJ. Banding of the pulmonary artery for transposition of the great arteries and ventricular septal defect. Circulation. 1970;41:II116-22. 18. Stark J, Hucin B, Aberdeen E and Waterston DJ. Cardiac surgery in the first year of life: experience with 1,049 operations. Surgery. 1971;69:483-97.
19. Norwood WI, Lang P and Hansen DD. Physiologic repair of aortic atresia-hypoplastic left heart syndrome. N Engl J Med. 1983;308:23-6. 20. Jacobs ML, Rychik J, Rome JJ, Apostolopoulou S, Pizarro C, Murphy JD and Norwood WI, Jr. Early reduction of the volume work of the single ventricle: the hemi-Fontan operation. Ann Thorac Surg. 1996;62:456-61; discussion 461-2. 21. Norwood WI and Jacobs ML. Fontan's procedure in two stages. Am J Surg. 1993;166:548-51. 22. Norwood WI and Stellin GJ. Aortic atresia with interrupted aortic arch: reparative operation. J Thorac Cardiovasc Surg. 1981;81:239-44. 23. Spray TL, Mallory GB, Canter CE, Huddleston CB and Kaiser LR. Pediatric lung transplantation for pulmonary hypertension and congenital heart disease. Ann Thorac Surg. 1992;54:216-23; discussion 224-5. 24. Sweet SC, Spray TL, Huddleston CB, Mendeloff E, Canter CE, Balzer DT, Bridges ND, Cohen AH and Mallory GB, Jr. Pediatric lung transplantation at St. Louis Children's Hospital, 1990-1995. Am J Respir Crit Care Med. 1997;155:1027-35. 25. Rychik J, Khalek N, Gaynor JW, Johnson MP, Adzick NS, Flake AW and Hedrick HL. Fetal intrapericardial teratoma: natural history and management including successful in utero surgery. Am J Obstet Gynecol. 2016. 26. Gaynor JW, Bridges ND, Cohen MI, Mahle WT, Decampli WM, Steven JM, Nicolson SC and Spray TL. Predictors of outcome after the Fontan operation: is hypoplastic left heart syndrome still a risk factor? Journal of Thoracic & Cardiovascular Surgery. 2002;123:237-45. 27. Gaynor JW, Collins MH, Rychik J, Gaughan JP and Spray TL. Long-term outcome of infants with single ventricle and total anomalous pulmonary venous connection. J Thorac Cardiovasc Surg. 1999;117:506-13; discussion 513-4. 28. Gaynor JW, Ittenbach RF, Gerdes M, Bernbaum J, Clancy RR, McDonald-McGinn DM, Zackai EH, Wernovsky G, Nicolson SC and Spray TL. Neurodevelopmental outcomes in preschool survivors of the Fontan procedure. J Thorac Cardiovasc Surg. 2014;147:1276-82; discussion 1282-1283 e5. 29. Gaynor JW, Mahle WT, Cohen MI, Ittenbach RF, DeCampli WM, Steven JM, Nicolson SC and Spray TL. Risk factors for mortality after the Norwood procedure. European Journal of Cardio-Thoracic Surgery. 2002;22:82-9. 30. Mahle WT, Spray TL, Wernovsky G, Gaynor JW and Clark BJ, 3rd. Survival after reconstructive surgery for hypoplastic left heart syndrome: A 15-year experience from a single institution. Circulation. 2000;102:III136-41. 31. Mitchell ME, Ittenbach RF, Gaynor JW, Wernovsky G, Nicolson S and Spray TL. Intermediate outcomes after the Fontan procedure in the current era. Journal of Thoracic & Cardiovascular Surgery. 2006;131:172-80. 32. Petko M, Myung RJ, Wernovsky G, Cohen MI, Rychik J, Nicolson SC, Gaynor JW and Spray TL. Surgical reinterventions following the Fontan procedure. European Journal of Cardio-Thoracic Surgery. 2003;24:255-9. 33. Rychik J, Szwast A, Natarajan S, Quartermain M, Donaghue DD, Combs J, Gaynor JW, Gruber PJ, Spray TL, Bebbington M and Johnson MP. Perinatal and early surgical outcome for the fetus with hypoplastic left heart syndrome: a 5-year single institutional experience. Ultrasound in Obstetrics & Gynecology. 2010;36:465-70.
34. Weinstein S, Gaynor JW, Bridges ND, Wernovsky G, Montenegro LM, Godinez RI and Spray TL. Early survival of infants weighing 2.5 kilograms or less undergoing first-stage reconstruction for hypoplastic left heart syndrome. Circulation. 1999;100:II167-70. 35. Rogers LS, Glatz AC, Ravishankar C, Spray TL, Nicolson SC, Rychik J, Rush CH, Gaynor JW and Goldberg DJ. 18 years of the Fontan operation at a single institution: results from 771 consecutive patients. J Am Coll Cardiol. 2012;60:1018-25. 36. Rossano JW, Goldberg DJ, Fuller S, Ravishankar C, Montenegro LM and Gaynor JW. Successful use of the total artificial heart in the failing Fontan circulation. Ann Thorac Surg. 2014;97:1438-40. 37. Gaynor JW, Stopp, C., Wypij, D., Andropoulos, D.B., Atallah, J., Beca, J., Duncan, K., Ghanayem, N.S., Goldberg, C.S., Hovels-Guurich, H., Ichida, F., Justo, R., Latal, B., Mahle, W.T., McQuillen, P., Pizarro, C., Shekerdemian, L.S., Synnes, A., Bellinger, D.C., Newburger, J.W., International Cardiac Collaborative on Neurodevelopment (ICCON) Investigators, Pediatric Heart Network (PHN) Investigators Early Neurodevelopmental Outcomes after Cardiac Surgery in Infancy Have Not Improved: A Multi-center Retrospective Analysis of 1,718 Patients Circulation. 2012;126:A12437. 38. Gaynor JW, Wernovsky G, Jarvik GP, Bernbaum J, Gerdes M, Zackai E, Nord AS, Clancy RR, Nicolson SC and Spray TL. Patient characteristics are important determinants of neurodevelopmental outcome at one year of age after neonatal and infant cardiac surgery. J Thorac Cardiovasc Surg. 2007;133:1344-53. 39. Kim DS, Kim JH, Burt AA, Crosslin DR, Burnham N, Kim CE, McDonald-McGinn DM, Zackai EH, Nicolson SC, Spray TL, Stanaway IB, Nickerson DA, Heagerty PJ, Hakonarson H, Gaynor JW and Jarvik GP. Burden of potentially pathologic copy number variants is higher in children with isolated congenital heart disease and significantly impairs covariate-adjusted transplant-free survival. J Thorac Cardiovasc Surg. 2016;151:1147-1151 e4. 40. Kim DS, Stanaway IB, Rajagopalan R, Bernbaum JC, Solot CB, Burnham N, Zackai EH, Clancy RR, Nicolson SC, Gerdes M, Nickerson DA, Hakonarson H, Gaynor JW and Jarvik GP. Results of genome-wide analyses on neurodevelopmental phenotypes at four-year follow-up following cardiac surgery in infancy. PLoS One. 2012;7:e45936. 41. Gaynor JW, Gerdes M, Zackai EH, Bernbaum J, Wernovsky G, Clancy RR, Newman MF, Saunders AM, Heagerty PJ, D'Agostino JA, McDonald-McGinn D, Nicolson SC, Spray TL and Jarvik GP. Apolipoprotein E genotype and neurodevelopmental sequelae of infant cardiac surgery. J Thorac Cardiovasc Surg. 2003;126:1736-45. 42. Gaynor JW, Kim DS, Arrington CB, Atz AM, Bellinger DC, Burt AA, Ghanayem NS, Jacobs JP, Lee TM, Lewis AB, Mahle WT, Marino BS, Miller SG, Newburger JW, Pizarro C, Ravishankar C, Santani AB, Wilder NS, Jarvik GP, Mital S and Russell MW. Validation of association of the apolipoprotein E epsilon2 allele with neurodevelopmental dysfunction after cardiac surgery in neonates and infants. J Thorac Cardiovasc Surg. 2014.
Figure Headings Figure 1. The Children’s Hospital of Philadelphia prior to moving to current location Figure 2. The Children’s Hospital of Philadelphia Figure 3.Dr. William Rashkind and balloon septostomy catheter Figure 4. Aerial View of CHOP main Campus Figure 5. Julian Johnson, M.D., 43rd President of the American Association for Thoracic Surgery Figure 6. John Waldhausen M.D., 72nd President of the American Association for Thoracic Surgery (right) with Dr. Jonathon Rhoads Figure 7. Dr.Eoin Aberdeen Figure 8. L. Henry Edmunds, M.D. Figure 9. Larry Stephenson, M.D. Figure 10. William I. Norwood, M.D. Figure 11. Thomas L. Spray, M.D., 89th President of the American Association for Thoracic Surgery Figure 12. Division of Cardiac Surgery at CHOP. Drs. Mascio, Fuller, Spray and Gaynor Figure 13A. Hysterotomy and exposure of fetal chest Figure 13B. Resection of intra-pericardial teratoma Figure 14. Heartware ventricular assist device in a 3 year old with a failing Fontan
PII: DOI: Reference:
S1043-0679(16)30160-5 http://dx.doi.org/10.1053/j.semtcvs.2016.11.003 YSTCS924
To appear in: Seminars in Thoracic and Cardiovascular Surgery Cite this article as: Thomas L. Spray, Stephanie Fuller, Christopher E. Mascio and J. William Gaynor, Cardiovascular Surgery at The Children′s Hospital of Philadelphia, Seminars in Thoracic and Cardiovascular Surgery, http://dx.doi.org/10.1053/j.semtcvs.2016.11.003 This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting galley proof before it is published in its final citable form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.
Cardiovascular Surgery at The Children’s Hospital of Philadelphia Thomas L. Spray, M.D., Stephanie Fuller, M.D., Christopher E. Mascio, M.D, and J. William Gaynor, M.D.
Division of Cardiothoracic Surgery, The Children’s Hospital of Philadelphia, Philadelphia, Pennsylvania
Corresponding Author: Thomas L. Spray, M.D. Division of Cardiothoracic Surgery The Children’s Hospital of Philadelphia 34th and Civic Center Boulevard Philadelphia, PA 19104, USA Phone: 215-590-2708 / Fax: 215-590-2715 E-mail: [email protected]
Abstract Surgery for congenital heart disease at the Children’s Hospital of Philadelphia (CHOP) began in the earliest days of cardiac surgery. In a rapidly advancing field, surgeons at CHOP are recognized as innovators in the field. The Division of Cardiac Surgery is dedicated to providing outstanding clinical care, educating the next generation of congenital cardiac surgeons, and advancing the specialty through clinical and translational research.
The Children’s Hospital of Philadelphia (CHOP) was founded in 1855 as the nation’s first hospital dedicated exclusively to the care of children. Drs. Francis West Lewis, Thomas Hewson Bache and R.A.F. Penrose were Philadelphia physicians who had traveled to London and visited the newly established Great Ormond Street Hospital for Sick Children. The physicians returned to Philadelphia to establish the nation’s first pediatric hospital which was located on Blight St. in downtown Philadelphia with 12 beds. The hospital treated sixty-three patients in its first year. In 1866, CHOP moved to its second location at 22nd Street between Walnut St. and Locust St. and beds increased to thirty-five. In 1873, the first resident physician was appointed to CHOP. His duties included compounding of all prescriptions. And in 1895, the Ingersoll Training School for Nurses, the birthplace of pediatric nursing in America, was established and operated until 1945. The Department for the Prevention of Disease was established in 1914, and was the first such program in the nation. The second major relocation of the hospital to 18th St. and Bainbridge St. occurred in 1916 and expanded the bed capacity significantly. (Figure 1) Joseph Stokes Jr. was appointed as Physician-In-Chief at CHOP in 1929. Dr. Stokes is credited with transforming CHOP into a world leader in teaching and research. The Joseph Stokes Research Institute was founded to honor his memory. Amongst early interventions, the original whooping cough vaccine was developed at CHOP in 1936. This was the first in a series of childhood vaccines, including those for influenza and mumps, pioneered at CHOP by Drs. Werner and Gertrude Henle, which have had worldwide impact on prevention of childhood diseases. In 1938, the first closed incubator was developed at CHOP for the use in newborns and in 1940 the first professional play program for patients, which developed into Child Life Services was initiated at The Children’s Hospital. The institution gained national prominence when Dr. C. Everett Koop joined the medical staff in 1946 and by 1948 was appointed as the Surgeon-InChief of The Children’s Hospital. He was internationally recognized for his expertise in separating conjoined twins. Dr. Koop went on to become the nation’s Surgeon General under President Ronald Reagan. In 1962, the first Neonatal Intensive Care Unit in the nation was established at CHOP and the first Pediatric Intensive Care Unit followed in 1967. In 1963, Stanley Plotkin developed the Rubella vaccine and the initial clinical trials were conducted at CHOP. A major advancement in the treatment of congenital heart disease occurred in 1965 when Dr. William Rashkind, Chief of Pediatric Cardiology at CHOP, developed the balloon atrial septostomy thus permitting improvement in cyanosis and stabilization in patients with transposition of the great arteries with intact ventricular septum.1 (Figure 2) In 1974, the hospital made its third move to its current location at 34th Street and Civic Center Boulevard, which adjacent to the old Philadelphia General Hospital, an institution that was recognized for the pioneering procedures for closed mitral valvuloplasty by Dr. Charles Bailey. (Figure 3) Since the move to West Philadelphia, The Children’s Hospital of Philadelphia has expanded significantly with the addition of several research buildings, a new seven hundred thousand square foot ambulatory care facility, multiple additions to the main hospital, new specialty care ambulatory care facilities and many pediatric practices. (Figure 4) The Children’s Hospital of Philadelphia is now a health system providing care to thousands of patients annually from the Delaware Valley, the United States and internationally. The CHOP Care Network consists of 16 Specialty Care Centers, 4 Ambulatory Surgery Centers, 31 Primary Care practices, 15 community hospital affiliations, and 3 after-hours urgent care facilities. The Children’s Hospital of Philadelphia has been recognized by the US News and World Report as one of the top two pediatric institutions in the United States and is in the top four of nine of the ten specialty rankings. The continued emphasis on research led to CHOP being designated as a Human
Genome Center by the National Institutes of Health. CHOP researchers were instrumental in the mapping of chromosome 22, which was completed in 1999. In 1995, the Center for Fetal Diagnosis and Treatment (CFDT) was established which provides fetal surgery and intervention for complex congenital malformations and cardiac diseases. A multi-billion dollar expansion of the facilities was undertaken in 2001, and has led to a large medical campus in West Philadelphia encompassing the main hospital and ambulatory care building, several research buildings for the Stokes Institute of Research and the Children’s Seashore House, a rehabilitation facility which was relocated from the Atlantic Shore to the main campus. Surgery for congenital heart disease at CHOP began with Dr. Julian Johnson who was the 43 President of the American Association for Thoracic Surgery.2, 3 (Figure 5) Dr. Johnson completed his surgical training in Philadelphia. In the Pacific Theater, Johnson served in the 20th General Hospital which had been organized from the Hospital of the University of Pennsylvania by Drs. I.S. Ravdin and Thomas Fitzhugh. He returned to Philadelphia after the war and eventually served as Chief of Cardiothoracic Surgery at The University of Pennsylvania until retirement in 1973. Early in his career, Dr. Johnson developed an interest in the emerging field of congenital heart surgery.4, 5 He was an early adopter of the innovative techniques being introduced to treat the problem of congenital heart disease; including ligation of patent ductus arteriosus, repair of coarctation of the aorta, and the Blalock-Taussig shunt for palliation of tetralogy of Fallot. He quickly mastered the procedure for Blalock-Taussig shunt and was referred many patients by Dr. Blalock.6 After the first successful use of cardiopulmonary bypass by John Gibbon in 1953, Johnson initiated the open heart surgery programs at both the University of Pennsylvania and CHOP. By 1964, he with his associates, Charles Kirby and Gordon Danielson, reported a series from both institutions of 100 consecutive open heart surgeries with a mortality of ten percent using the newly developed Mayo-Gibbon pump.7 rd
In 1966 John Waldhausen joined the faculty at The University of Pennsylvania under Dr. Johnson’s leadership. (Figure 6) Dr. Koop, the founding Chief of Surgery at CHOP, recruited Dr. Waldhausen to develop an independent dedicated pediatric cardiac surgery service at The Children’s Hospital of Philadelphia. Dr. Waldhausen developed open cardiac repairs in children and then extended these repairs into infancy. By 1971, he had reported use of a disc oxygenator for open cardiac repair in congenital heart disease with a survival rate of 78 percent. With Dr. William Rashkind, Dr. Waldhausen reported an operative survival rate of 81 percent patients for transposition of the great arteries in 1971, with a 100 percent survival rate for 18 patients with simple transposition.8, 9 Dr. Waldhausen was known for technical expertise and his excellent results in infant pediatric cardiac surgery. He developed an international reputation as one of the leaders in congenital heart surgery. Dr. Waldhausen is perhaps best remembered by congenital heart surgeons for the introduction of the subclavian flap aortoplasty for coarctation of the aorta.10-12 In 1969, Dr. Waldhausen was offered the founding Chairman of Surgery at the newly formed Milton Hershey Medical Center at the Pennsylvania State University College of Medicine and left Philadelphia for Hershey, Pennsylvania. He served as the 72nd President of the American Association for Thoracic Surgery.13, 14 After Dr. Waldhausen’s departure, the pediatric cardiac surgical program at The Children’s Hospital of Philadelphia was led by Dr. Eoin Aberdeen. (Figure 7) Dr. Aberdeen had been a consultant cardiothoracic surgeon at the Hospital for Sick Children at Great Ormond Street in London since 1963. His work focused on open heart surgery in infants and small children and he attained international fame for his work on transposition of the great arteries.15-18 In 1971, he
moved to Philadelphia to assume the Chief of Cardiac Surgery position at CHOP where he remained chief until 1974. During his time at CHOP, Dr. Aberdeen was instrumental in founding The Congenital Heart Surgeons Society, a prestigious group of North American heart surgeons which was based on the European Pediatric Cardiac Surgery Club which Aberdeen was an active member. With the exit of Eoin Aberdeen, cardiac surgery at CHOP was performed by adult cardiac surgeons with an interest in congenital heart disease from The University of Pennsylvania including L. Henry Edmunds (past Editor of The Annals of Thoracic Surgery) , Richard Edie, and Lawrence Stephenson. (Figures 8 and 9) James Alexander was a congenital heart surgeon at CHOP for a brief period of time prior to moving to the University of Florida in Gainesville. A major advance in the cardiac surgical program at The Children’s Hospital of Philadelphia occurred in 1984 when William I. Norwood was recruited from Boston Children’s Hospital to become the Chief of Cardiac Surgery. (Figure 10). Dr. Norwood brought with him an extensive knowledge of complex congenital surgery in newborns and specific expertise in the treatment of hypoplastic left heart syndrome (HLHS) for which the first stage operation is now known as the Norwood Operation.19 The cardiac surgical program expanded under Dr. Norwood with dedicated operating rooms and an intensive care unit specifically for the care of patients with congenital heart disease. A dedicated Division of Cardiac Anesthesia was established in 1984 under the leadership of Dr. Susan C. Nicolson. Dr. Norwood was joined by Dr. John Pigott in 1985 and by Dr. Marshall L. Jacobs in 1990. During this time, CHOP became a major center for the treatment of HLHS, performing more surgical procedures for this condition than any other institution in the world. Many of the techniques now considered standard for management of infants with complex single ventricle heart defects were introduced and refined during this time. 20-22
In 1994, Dr. Norwood left CHOP to begin an international practice in cardiac surgery in Genolier, Switzerland and Dr. Thomas L. Spray was recruited from Washington University in St. Louis and the St. Louis Children’s Hospital to become the Chief of Cardiac Surgery. (Figure 11) Prior to moving to CHOP, Dr. Spray started the first dedicated pediatric lung transplant program at Washington University in 1990.23, 24 Members of the Division of Cardiac Surgery since 1995 have included Tom R. Karl, M.D., William M. DeCampli, M.D., and Peter J. Gruber, M.D, Ph.D. Current members of the Division are J. William Gaynor, M.D., Stephanie Fuller, M.D. and Christopher E. Mascio, M.D. (Figure 12) Dr. Spray is the holder of the Alice Langdon Warner Endowed Chair in Pediatric Cardiac Surgery. Dr. Gaynor is the holder of the Daniel M. Tabas Endowed Chair in Pediatric Cardiac Surgery. Dr. Spray was the 89th President of the American Association for Thoracic Surgery. He is the third Chief of Cardiac Surgery at CHOP to hold this prestigious position. Since Dr. Spray’s appointment, the cardiac surgery program has continued to expand and evolve. In 1994, a new Cardiac Intensive Care Unit (CICU) was opened as a dedicated unit for the care of cardiac patients. This was one of the few such dedicated units in the nation at that time. After his appointment, the cardiac transplantation program was expanded and a lung and heart-lung transplantation program was started. In addition, an active mechanical circulatory support program was begun. In 1998, The Cardiac Center was established integrating cardiology, cardiothoracic surgery, cardiac anesthesia and cardiac nursing to optimize the care of patients with congenital heart disease. A combined Adult Congenital Heart Disease Program with the Hospital of the University of Philadelphia was begun in 2004. Drs. Fuller and Mascio
are the primary surgeons for this rapidly growing program. The Cardiac Operative and Imaging Complex (COIC) opened in 2006. This facility incorporates 2 dedicated cardiac operating rooms, 2 catheterization rooms, a hybrid suite and a MRI unit. It is located on the same floor as the CICU, facilitating patient transfer. The Garbose Family Special Delivery Unit (SDU) was established as part of the CFDT in 2008 as the world’s first birthing unit within a pediatric hospital dedicated to healthy mothers carrying babies with serious and life-threatening birth defects. It is located immediately adjacent to the COIC and CICU. For newborns with complex heart defects, this allows immediate access to care and the mothers are able to remain close to their child. The cardiac surgery program at CHOP remains one of the highest volume centers in North America performing over 550 open cardiac procedures and over 800 total cases each year. These include the full spectrum of cardiac surgery including fetal cardiac interventions, neonatal reconstruction, thoracic organ transplantation, mechanical circulatory support and management of the adult with congenital heart disease. Recently, surgeons at CHOP performed the first successful in utero surgical resection of an intra-pericardial teratoma in a 24 week gestational age fetus with survival to term.25 (Figure 13) Since Dr. Norwood’s arrival, there has been a focus on management of hypoplastic left heart syndrome and other forms of complex single ventricle heart defects. Under Dr. Spray’s leadership, CHOP remains a referral center for these complex infants and has the world’s largest experience with HLHS.26-34 With improved survival for these vulnerable children has come an increased focus on long term outcomes. The Single Ventricle Survivorship Program was established in 2011 and currently follows one of the largest cohorts of children, adolescents and young adults with single ventricle congenital heart disease in the world.35 Many of these children will ultimately require heart transplantation. Surgeons at CHOP have continued to expand the use of mechanical circulatory support in these patients; including the first successful use of the total artificial heart after the Fontan procedure and expanded utilization of ventricular assist devices in toddlers.36 (Figure 14) Education and research are priorities at CHOP. Training the next generation of congenital heart surgeons is of utmost importance. Since 1995, 43 surgical trainees (both from North America and overseas) have undertaken a fellowship at CHOP. The Congenital Heart Surgery Fellowship was approved by the Accreditation Council for Graduate Medical Education (ACGME) in 2008 and there have been 7 accredited fellows. There is an active clinical and translational research program in the Division of Cardiac Surgery. Research interests include genetic modifiers of outcome and neurodevelopmental outcomes after surgery for congenital heart defects. These efforts have provided strong evidence that patient factors, especially genetic variants, are important determinants of the risk of death and developmental disability after surgery.37-40 We have identified and validated an association of apo-lipoprotein E (APOE) with neurodevelopmental disability in this population.41, 42 In 2014, the Center for Fetal Neuroprotection and Neuroplasticity was established at CHOP and we initiated the world’s first randomized clinical trial of fetal neuroprotection to reduce brain injury and improve neurodevelopmental outcomes for the fetus with congenital heart disease. The Division of Cardiac Surgery at CHOP has a proud tradition of innovation and outstanding clinical care. Surgery for congenital heart disease at the Children’s Hospital of Philadelphia (CHOP) began in the earliest days of cardiac surgery. The Division of Cardiac Surgery is dedicated to providing outstanding clinical care, educating the next generation of
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19. Norwood WI, Lang P and Hansen DD. Physiologic repair of aortic atresia-hypoplastic left heart syndrome. N Engl J Med. 1983;308:23-6. 20. Jacobs ML, Rychik J, Rome JJ, Apostolopoulou S, Pizarro C, Murphy JD and Norwood WI, Jr. Early reduction of the volume work of the single ventricle: the hemi-Fontan operation. Ann Thorac Surg. 1996;62:456-61; discussion 461-2. 21. Norwood WI and Jacobs ML. Fontan's procedure in two stages. Am J Surg. 1993;166:548-51. 22. Norwood WI and Stellin GJ. Aortic atresia with interrupted aortic arch: reparative operation. J Thorac Cardiovasc Surg. 1981;81:239-44. 23. Spray TL, Mallory GB, Canter CE, Huddleston CB and Kaiser LR. Pediatric lung transplantation for pulmonary hypertension and congenital heart disease. Ann Thorac Surg. 1992;54:216-23; discussion 224-5. 24. Sweet SC, Spray TL, Huddleston CB, Mendeloff E, Canter CE, Balzer DT, Bridges ND, Cohen AH and Mallory GB, Jr. Pediatric lung transplantation at St. Louis Children's Hospital, 1990-1995. Am J Respir Crit Care Med. 1997;155:1027-35. 25. Rychik J, Khalek N, Gaynor JW, Johnson MP, Adzick NS, Flake AW and Hedrick HL. Fetal intrapericardial teratoma: natural history and management including successful in utero surgery. Am J Obstet Gynecol. 2016. 26. Gaynor JW, Bridges ND, Cohen MI, Mahle WT, Decampli WM, Steven JM, Nicolson SC and Spray TL. Predictors of outcome after the Fontan operation: is hypoplastic left heart syndrome still a risk factor? Journal of Thoracic & Cardiovascular Surgery. 2002;123:237-45. 27. Gaynor JW, Collins MH, Rychik J, Gaughan JP and Spray TL. Long-term outcome of infants with single ventricle and total anomalous pulmonary venous connection. J Thorac Cardiovasc Surg. 1999;117:506-13; discussion 513-4. 28. Gaynor JW, Ittenbach RF, Gerdes M, Bernbaum J, Clancy RR, McDonald-McGinn DM, Zackai EH, Wernovsky G, Nicolson SC and Spray TL. Neurodevelopmental outcomes in preschool survivors of the Fontan procedure. J Thorac Cardiovasc Surg. 2014;147:1276-82; discussion 1282-1283 e5. 29. Gaynor JW, Mahle WT, Cohen MI, Ittenbach RF, DeCampli WM, Steven JM, Nicolson SC and Spray TL. Risk factors for mortality after the Norwood procedure. European Journal of Cardio-Thoracic Surgery. 2002;22:82-9. 30. Mahle WT, Spray TL, Wernovsky G, Gaynor JW and Clark BJ, 3rd. Survival after reconstructive surgery for hypoplastic left heart syndrome: A 15-year experience from a single institution. Circulation. 2000;102:III136-41. 31. Mitchell ME, Ittenbach RF, Gaynor JW, Wernovsky G, Nicolson S and Spray TL. Intermediate outcomes after the Fontan procedure in the current era. Journal of Thoracic & Cardiovascular Surgery. 2006;131:172-80. 32. Petko M, Myung RJ, Wernovsky G, Cohen MI, Rychik J, Nicolson SC, Gaynor JW and Spray TL. Surgical reinterventions following the Fontan procedure. European Journal of Cardio-Thoracic Surgery. 2003;24:255-9. 33. Rychik J, Szwast A, Natarajan S, Quartermain M, Donaghue DD, Combs J, Gaynor JW, Gruber PJ, Spray TL, Bebbington M and Johnson MP. Perinatal and early surgical outcome for the fetus with hypoplastic left heart syndrome: a 5-year single institutional experience. Ultrasound in Obstetrics & Gynecology. 2010;36:465-70.
34. Weinstein S, Gaynor JW, Bridges ND, Wernovsky G, Montenegro LM, Godinez RI and Spray TL. Early survival of infants weighing 2.5 kilograms or less undergoing first-stage reconstruction for hypoplastic left heart syndrome. Circulation. 1999;100:II167-70. 35. Rogers LS, Glatz AC, Ravishankar C, Spray TL, Nicolson SC, Rychik J, Rush CH, Gaynor JW and Goldberg DJ. 18 years of the Fontan operation at a single institution: results from 771 consecutive patients. J Am Coll Cardiol. 2012;60:1018-25. 36. Rossano JW, Goldberg DJ, Fuller S, Ravishankar C, Montenegro LM and Gaynor JW. Successful use of the total artificial heart in the failing Fontan circulation. Ann Thorac Surg. 2014;97:1438-40. 37. Gaynor JW, Stopp, C., Wypij, D., Andropoulos, D.B., Atallah, J., Beca, J., Duncan, K., Ghanayem, N.S., Goldberg, C.S., Hovels-Guurich, H., Ichida, F., Justo, R., Latal, B., Mahle, W.T., McQuillen, P., Pizarro, C., Shekerdemian, L.S., Synnes, A., Bellinger, D.C., Newburger, J.W., International Cardiac Collaborative on Neurodevelopment (ICCON) Investigators, Pediatric Heart Network (PHN) Investigators Early Neurodevelopmental Outcomes after Cardiac Surgery in Infancy Have Not Improved: A Multi-center Retrospective Analysis of 1,718 Patients Circulation. 2012;126:A12437. 38. Gaynor JW, Wernovsky G, Jarvik GP, Bernbaum J, Gerdes M, Zackai E, Nord AS, Clancy RR, Nicolson SC and Spray TL. Patient characteristics are important determinants of neurodevelopmental outcome at one year of age after neonatal and infant cardiac surgery. J Thorac Cardiovasc Surg. 2007;133:1344-53. 39. Kim DS, Kim JH, Burt AA, Crosslin DR, Burnham N, Kim CE, McDonald-McGinn DM, Zackai EH, Nicolson SC, Spray TL, Stanaway IB, Nickerson DA, Heagerty PJ, Hakonarson H, Gaynor JW and Jarvik GP. Burden of potentially pathologic copy number variants is higher in children with isolated congenital heart disease and significantly impairs covariate-adjusted transplant-free survival. J Thorac Cardiovasc Surg. 2016;151:1147-1151 e4. 40. Kim DS, Stanaway IB, Rajagopalan R, Bernbaum JC, Solot CB, Burnham N, Zackai EH, Clancy RR, Nicolson SC, Gerdes M, Nickerson DA, Hakonarson H, Gaynor JW and Jarvik GP. Results of genome-wide analyses on neurodevelopmental phenotypes at four-year follow-up following cardiac surgery in infancy. PLoS One. 2012;7:e45936. 41. Gaynor JW, Gerdes M, Zackai EH, Bernbaum J, Wernovsky G, Clancy RR, Newman MF, Saunders AM, Heagerty PJ, D'Agostino JA, McDonald-McGinn D, Nicolson SC, Spray TL and Jarvik GP. Apolipoprotein E genotype and neurodevelopmental sequelae of infant cardiac surgery. J Thorac Cardiovasc Surg. 2003;126:1736-45. 42. Gaynor JW, Kim DS, Arrington CB, Atz AM, Bellinger DC, Burt AA, Ghanayem NS, Jacobs JP, Lee TM, Lewis AB, Mahle WT, Marino BS, Miller SG, Newburger JW, Pizarro C, Ravishankar C, Santani AB, Wilder NS, Jarvik GP, Mital S and Russell MW. Validation of association of the apolipoprotein E epsilon2 allele with neurodevelopmental dysfunction after cardiac surgery in neonates and infants. J Thorac Cardiovasc Surg. 2014.
Figure Headings Figure 1. The Children’s Hospital of Philadelphia prior to moving to current location Figure 2. The Children’s Hospital of Philadelphia Figure 3.Dr. William Rashkind and balloon septostomy catheter Figure 4. Aerial View of CHOP main Campus Figure 5. Julian Johnson, M.D., 43rd President of the American Association for Thoracic Surgery Figure 6. John Waldhausen M.D., 72nd President of the American Association for Thoracic Surgery (right) with Dr. Jonathon Rhoads Figure 7. Dr.Eoin Aberdeen Figure 8. L. Henry Edmunds, M.D. Figure 9. Larry Stephenson, M.D. Figure 10. William I. Norwood, M.D. Figure 11. Thomas L. Spray, M.D., 89th President of the American Association for Thoracic Surgery Figure 12. Division of Cardiac Surgery at CHOP. Drs. Mascio, Fuller, Spray and Gaynor Figure 13A. Hysterotomy and exposure of fetal chest Figure 13B. Resection of intra-pericardial teratoma Figure 14. Heartware ventricular assist device in a 3 year old with a failing Fontan