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Home , Cardiopulmonary bypass

Marla Kiess, MD, FRCPC

History and evolution of the treatment of adult congenital disease

Surgical developments and other advances mean that more congenital heart disease patients are reaching adulthood and requiring the support of a team that includes cardiologists, nurses, psychologists, and social workers with knowledge of adult CHD.

ABSTRACT: experts tostomy was developed in 1966 to of the many advances made since around the world, including many promote mixing at the atrial level and the 1930s, children born with CHD Canadians, have contributed to dramatically improved the outcome today are much more likely to grow dramatic surgical, interventional, for newborns with complete trans- to adulthood, but they are also like- and diagnostic advances since the position of the great . Be- ly to require multiple operations for 1930s. These developments began ginning with innovative use of X-ray scarring and narrowing of arteries or when Dr Helen Taussig established imaging, diagnostic techniques sup- and insertion or replacement the pediatric cardiology clinic at ported both surgical and nonsurgical of conduits and valves. Patients Johns Hopkins Hospital in Balti- interventions. Right heart catheter- with moderate to severe disease are more in 1930 and Dr Maude Abbott ization became available in the late rarely cured and face a lifetime of re- of Montreal published the Atlas of 1940s and left heart catheterization peat surgical and interventional pro- Congenital Heart Disease in 1936. was developed in the 1950s. The cedures. Each year, BC Children’s The first surgical procedure was advent of two-dimensional echocar- Hospital registers approximately ligation of a patent ductus arterio- diography in the 1970s permitted a 500 newly diagnosed CHD patients sus performed by Dr Robert Gross major step forward in the treatment and moves 300 previously diagnosed at the Children’s Hospital in Boston of congenital heart disease (CHD), as patients from pediatric to adult care. in 1938. Intracardiac repair first be- did the establishment of standard- Approximately 150 patients per year came possible with the development ized nomenclature. Canadian doctor will require follow-up in an adult CHD of technol- Wilfred Bigelow determined how to clinic. A review of advances in the ogy in the 1950s, followed in the use total body for open treatment of CHD reveals dramatic 1970s by the development of deep heart in 1953, and the first progress beginning in the 1930s and hypothermia with circulatory arrest, open heart procedure in Canada was continuing to the present. Cardiology which made lengthier performed by Dr John Callaghan in experts around the world, including possible. Interventional techniques Edmonton in 1954. In British Colum- many Canadians, have contributed went hand in hand with surgical ad- bia, Dr Ross Robertson performed a to a variety of surgical, intervention- vances. Balloon dilatation of the pul- Blalock-Taussig shunt, closed a pat- al, and diagnostic developments. monary valve was first described in ent ductus arteriosus, and repaired a the 1950s and became widely used coarctation of the at Vancouver Dr Kiess is director of the Pacific Adult after static balloon dilatation was in- General Hospital in 1947. In the late Congenital Heart (PACH) clinic, Division of troduced in 1982. Balloon atrial sep- 1950s Dr Harold Rice built the first Cardiology, St. Paul’s Hospital. She is also cardiopulmonary bypass machine a clinical professor in the Division of Cardi- This article has been peer reviewed. used at St. Paul’s Hospital. Because ology at the University of British Columbia.

368 bc medical journal vol. 58 no. 7, september 2016 bcmj.org History and evolution of the treatment of adult congenital heart disease

Surgical developments Lillehei and his colleagues to develop riosus, and atrial septal defects. A The organized study of congenital a pump .9 However, even major advance was the development heart disease (CDH) began with the with this innovation the preservation of prosthetic pulmonary valves by establishment of Dr Helen Taussig’s of flow to the brain was not Dr Bonhoeffer13 and prosthetic aortic pediatric cardiology clinic at Johns always optimal and had to valves by Drs Cribier14 and Webb15 in Hopkins Hospital in Baltimore in work quickly until the development the 2000s. 19301 and the publication of Dr of deep hypothermia with circulatory Diagnostic techniques, begin- Maude Abbott’s incredible atlas de- arrest in the early 1970s made length- ning with Dr Taussig’s innovative scribing 1000 CHD cases in 1936.2 ier surgeries possible. use of X-ray imaging, supported both The first surgical procedure was li- gation of a patent ductus arteriosus (PDA) performed by Dr Robert Gross at the Children’s Hospital in Boston in 1938.3 Dr Taussig had observed that some children became progressively more cyanotic with spontaneous clos- Canadians have been at the ure of the ductus arteriosus and pro- posed using an arterial to pulmonary forefront of improvements for patients shunt. She convinced Dr Alfred with congenital heart disease. Blalock of the merit of this idea and eventually Blalock collaborated with his technician, Vivien Thomas, to construct a shunt from the right sub- clavian artery to the right in a cyanotic child. A report on the procedure was published in 1945.4 Interventional and surgical and nonsurgical interven- Also in 1945, Drs Crafoord and Nylin diagnostic techniques tions. Right heart catheterization of Stockholm performed surgery on a Interventional techniques went hand became available in the late 1940s and patient with coarctation of the aorta.5 in hand with surgical advances. left heart catheterization was devel- In 1948, Sir Russell Brock, working in Although balloon dilatation of the oped in the 1950s. M-mode echocar- Guy’s Hospital in London, England, was described in diograms, first available in the 1960s, published a report describing three 1953 by Rubio-Alvarez and col- were helpful, but it was the advent of cases of pulmonary stenosis that were leagues,10 the procedure did not two-dimensional repaired with pulmonary valvotomy.6 become widely used until Kan and in the 1970s that permitted a major In 1950, Drs Blalock and Hanlon per- colleagues11 introduced static bal- step forward. Important advances in formed atrial septectomy using a sur- loon dilatation in 1982. Balloon atrial pathology included the establishment gical clamp devised by Vivien Thom- septostomy, developed in 1966 by of standardized nomenclature by as.7 With the development of cardio- Drs Rashkind and Miller,12 promoted Richard and Stella Van Praagh work- pulmonary bypass technology, intra- mixing at the atrial level and dramati- ing in Toronto, Chicago, and then cardiac repair became possible. The cally improved the outcome for new- Boston, and by Robert Anderson, first procedure done with the use of borns with complete transposition working in London, England. a heart- machine was for closure of the great arteries. There was an of an and was per- explosion of catheter-based therapies Canadian contributions formed by Dr Gibbon in Philadelphia in the 1980s, including balloon dila- Canadians have been at the forefront in 1953.8 Later that year, Dr Lillehei, tation for repair of coarctation of the of improvements for patients with working in Minneapolis, performed aorta and stenotic valves, shunts, and congenital heart disease, beginning open heart surgery using cross- conduits. The development of with Dr Maude Abbott of Montreal, circulation between the child and a vastly improved long-term results. who wrote the Atlas of Congenital parent. This procedure was found to Various devices became available to Heart Disease already mentioned. have a high mortality rate, which led address fistulae, patent ductus arte- Dr Wilfred Bigelow16 of the Toronto

bc medical journal vol. 58 no. 7, september 2016 bcmj.org 369 History and evolution of the treatment of adult congenital heart disease

General Hospital determined how to wife, Dorothy, was born with an atri- formed on older children. Congenital use total body hypothermia for open al septal defect, and in 1953 she was heart lesions frequently resulted in heart surgery in 1953. The first open the fourth patient in the world and too much or too little blood flow to the heart procedures in Canada were for the first patient at the to . Infants and young children with closure of an atrial septal defect and have open heart surgery under hypo- reduced blood flow to the lungs were a ventricular septal defect and were thermic cardiac arrest. An anomalous palliated with arterial shunts, either performed by Dr John Callaghan in pulmonary discovered at surgery Blalock-Taussig (subclavian artery Edmonton in 1954. Dr William Mus- could not be repaired until the advent to pulmonary artery), Potts (descend- tard at the Hospital for Sick Children of cardiopulmonary bypass, and she ing aorta to pulmonary artery), or Waterston (ascending aorta to pulmo- nary artery), and those with excess blood flow to the lungs were palli- ated with pulmonary artery banding. The flow through these arterial shunts was difficult to control and pulmo- In the early days of cardiac nary hypertension was a significant surgery, intracardiac repairs risk. Dr Glenn felt that venous shunts would be superior, anastomosing the could only be performed to the pulmonary on older children. artery in 1959.20 Many patients had repeat operations with ligation of arterial shunts and replacement with right and/or left Glenn shunts. When the child patient reached an adequate size, usually around age 4, intracar- diac repair was performed, the shunts in Toronto significantly advanced the had a second procedure in 1958 at the were ligated, or the pulmonary band care of patients with complete trans- Mayo Clinic when she was in her late was removed. Dr Fontan developed position of the great arteries with his forties. Drs Bob Gourlay, Ted Mus- total right heart bypass for patients atrial switch operation (Mustard pro- grove, and Gerry Coursley closed with single- physiology in cedure) in 1963.17 an atrial septal defect in a 12-year- 197121 and subsequent modifica- In British Columbia, Dr Ross old girl using Dr Rice’s machine at tions to improve hemodynamics were Robertson performed a Blalock- St. Paul’s Hospital in 1960. Cardiac developed by him and Dr de Leval.22 Taussig shunt, closed a patent ductus catheterization was first performed In the early 1980s, Dr Aldo Castenada arteriosus, and repaired a coarctation at Vancouver General Hospital by perfected neonatal repairs at the Bos- of the aorta at Vancouver General Drs Morris Young and Dennis Vince, ton Children’s Hospital.23 Hospital in 1947. Dr Jack Stenstrom starting in the mid-1950s. Dr Doris started performing PDA ligations and Kavanagh performed the first cardiac Adult congenital heart Blalock-Taussig shunts in Victoria in catheterization at St. Paul’s Hospital disease care in BC 1949. In 1957, Dr Peter Allen, with in 1959. The need for this procedure As in the past, many children born the assistance of Drs Phil Ashmore, was great. After her first successful with congenital heart disease today Bill Trapp, and Ross Robertson, per- study, Dr Kavanagh was asked by Dr will require multiple operations as formed the first open heart procedure Young if she could catheterize some they grow to adulthood for various at Vancouver General Hospital, clos- of his patients and he sent her a list of reasons, including scarring and nar- ing an atrial septal defect in a 9-year- 400 patients who had been waiting for rowing of arteries or veins and inser- old boy.18 In the late 1950s, Dr Harold as long as 4 years. tion or replacement of conduits and Rice built the first cardiopulmonary valves. Patients with moderate to bypass machine used at St. Paul’s Further developments severe disease are rarely cured and Hospital.19 He had a very person- In the early days of , face a lifetime of repeat surgical and al reason for wanting to do this: his intracardiac repairs could only be per- interventional procedures.

370 bc medical journal vol. 58 no. 7, september 2016 bcmj.org History and evolution of the treatment of adult congenital heart disease

Based on a Canada-wide incidence cine, and the support and expertise of have supported the development of rate of 12 to 14 cases per 1000 live a multidisciplinary team (nurses, psy- both surgical and nonsurgical inter- births,24 500 to 600 infants with CHD chologists, social workers) who have ventions. The many advances made are born per year in British Colum- knowledge of CHD. since the 1930s mean that children bia. Data suggest that as of 2010 over A recent study from Quebec has born with CHD today are much more 24 000 individuals with CHD born in shown that these complex patients likely to grow to adulthood. However, BC had survived to adulthood. Some have higher rates of hospitalization, they are also likely to require multiple of these adults have simple defects more visits to emergency rooms, operations for scarring and narrowing and have little need for medical care. greater use of outpatient cardiolo- of arteries or veins and insertion or However, over 12 000 adults have moderate to severe defects and will require lifelong care by an array of health professionals with expertise in the field of CHD. BC Children’s Hospital currently BC Children’s Hospital currently registers approximately 500 newly diagnosed patients with CHD every registers approximately 500 newly year and moves 300 patients from diagnosed patients with CHD every pediatric to adult care each year. Ap- proximately 50% of these patients, or year and moves 300 patients from 150 per year, will have moderate to pediatric to adult care each year. severe CHD and require follow-up in an adult CHD clinic. These patients need ongoing evaluation to determine whether they require further interven- tion or medical management. About 110 of these patients (60%) can be gist care, and more days in critical replacement of conduits and valves, expected to require specialized con- care.25 The Canadian Cardiovascular and to require the support and exper- tinuing care for optimal quality of Society,26 American College of Car- tise of a multidisciplinary team with life. A smaller but significant number diology,27 and European Society of knowledge of CHD. of individuals present later in child- Cardiology28 have all recognized the hood or early adulthood with congen- urgent need for trained medical staff, Competing interests ital defects that have gone undetected allied health personnel, and special- None declared. due to the sometimes insidious nature ized clinics to deliver appropriate care of CHD progression, and like patients to this rapidly growing population of References with known CHD, these newly diag- adults with CHD. 1. Taussig HB. Congenital malformations of nosed patients may need advice re- the heart. Vol 1 and 2. Cambridge, MA: garding pregnancy risks and cardiac Summary Harvard University Press; 1960. surgery options. Many advances have followed the 2. Abbott ME. Atlas of congenital cardiac dis- The range of abnormalities, the first successful ligation of a patent ease. New York, NY: American Heart As- complexities of postoperative anato- ductus arteriosus in 1938. Intracar- sociation; 1939:62. my, and the challenges of multisys- diac repair became possible with the 3. Gross RE, Hubbard JP. Surgical ligation of tem involvement mean a full under- development of cardiopulmonary a patent ductus arteriosus: Report of first standing of CHD is now well beyond bypass technology in the 1950s, while successful case. Am Med Assoc J 1939; the education and experience of the lengthier surgeries became possible 112:729-731. typical cardiologist caring for adult after the development of deep hypo- 4. Blalock A, Taussig HB. The surgical treat- patients. To care for these patients, thermia with circulatory arrest in ment of malformations of the heart in practitioners require knowledge and the 1970s. Interventional techniques which there is pulmonary stenosis or pul- training in congenital heart disease, have accompanied surgical advances, monary atresia. J Am Med Assoc 1945; adult cardiology, and general medi- and a variety of imaging innovations 128:189-192.

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5. Crafoord C, Nylin G. Congenital coarcta- 16. Trusler G, McBirnie J, Pearson F, et al. A disease: Executive summary. Can J Car- tion of the aorta and its surgical treatment. study of hibernation in relation to the tech- diol 2010;26:143-150. J Thorac Surg 1945;14:347-361. nique of hypothermia for intracardiac sur- 27. Warnes CA, Williams RG, Bashore TM, et 6. Brock RC. Pulmonary valvotomy for the gery. Surg Forum 1953;4:72-77. al. ACC/AHA 2008 Guidelines for the man- relief of congenital pulmonary stenosis: 17. Mustard WT. Successful two-stage cor- agement of adults with congenital heart Report of three cases. BMJ 1948;1:1121- rection of transposition of the great ves- disease. A report of the American College 1126. sels. Surgery 1964;55:469-472. of Cardiology/American Heart Association 7. Blalock A, Hanlon CR. The surgical treat- 18. Burr L. Some early history of cardiac sur- Task Force on Practice Guidelines (Writing ment of complete transposition of the gery in British Columbia. The Surgical Committee to Develop Guidelines on the aorta and the pulmonary artery. Surg Gy- Times. Newsletter of the UBC Depart- Management of Adults with Congenital necol Obstet 1950;90:1-15. ment of Surgery, 2007. Heart Disease). Circulation 2008;118:e 8. Gibbon JH Jr. Application of a mechanical 19. Lemon K. Spirit of discovery: The history 714-833. heart and lung apparatus to cardiac sur- of cardiopulmonary pioneers at St. Paul’s 28. Baumgartner H, Bonhoeffer P, De Groot gery. Minn Med 1954;37:171-180. Hospital. Ottawa, ON: Catholic Health Al- NMS, et al. Task Force on the Manage- 9. Lillehei CW, Cohen M, Warden HE, Varco liance of Canada; 2000. Accessed 13 June ment of Grown-up Congenital Heart Dis- RL. The direct-vision intracardiac correc- 2016. www.chac.ca/about/history/books/ ease, European Society of Cardiology tion of congenital anomalies by controlled bc/Vancouver_St.%20Pauls’Hospital_ (ESC). ESC Guidelines for the manage- cross circulation: Results in thirty-two pa- Cardiopulmonary%20Pioneers_2000. ment of grown-up congenital heart dis- tients with ventricular septal defect, tetral- pdf. ease (new version 2010). Eur Heart J ogy of Fallot, and atrioventricularis com- 20. Glenn WWL. Circulatory bypass of the 2010:31;2915-2957. munis defects. Surgery 1955;38:11-29. right side of the heart. IV. Shunt between 10. Rubio-Alvarez V, Limon-Larson R, Soni J. superior vena cava and distal right pulmo- [Intracardiac by means of a nary artery; report of clinical application. N catheter]. Arch Inst Cardiol Mexico 1953; Engl J Med 1958;259:117-120. 23:183-192. 21. Fontan F, Baudet E. Surgical repair of tri- 11. Kan SJ, White RI Jr, Mitchell SE, Gardner cuspid atresia. Thorax 1971;26:240-248. TJ. Percutaneous balloon valvuloplasty: A 22. de Leval MR, Kilner P, Gewillig M, Bull C. new method for treating congenital pul- Total cavopulmonary connection: A logical monary valve stenosis. N Engl J Med alternative to atriopulmonary connection 1982;307:540-542. for complex Fontan operations. Experi- 12. Rashkind WJ, Miller WW. Creation of an mental studies and early clinical experi- atrial septal defect without thoracotomy: ence. J Thorac Cardiovasc Surg 1988;96: A palliative approach to complete transpo- 682-695. sition of the great arteries. JAMA 1966; 23. Castaneda AR, Jonas RA, Mayer JE Jr, 196:991-992. Hanley FL. Cardiac surgery of the neonate 13. Bonhoeffer P, Boudjemline Y, Saliba Z, et and infant. Philadelphia, PA: WB Saun- al. Percutaneous replacement of pulmo- ders; 1994:409-438. nary valve in a right-ventricle to pulmonary- 24. Health Canada. Congenital anomalies in artery prosthetic conduit with valve dys- Canada – a perinatal health report, 2002. function. Lancet 2000;356(9239):1403- Ottawa: Minister of Public Works and 1405. Government Services Canada, 2002. 14. Cribier A, Eltchaninoff H, Bash A, et al. 25. Marelli AJ, Therrien J, Mackie AS, et al. Percutaneous transcatheter implantation Planning the specialized care of adult con- of an prosthesis for calcific genital heart disease patients: From num- aortic stenosis: First human case descrip- bers to guidelines; an epidemiologic ap- tion. Circulation 2002;106:3006-3008. proach. Am Heart J 2009;157:1-8. 15. Chandavimol M, McClure S, Carere R, et 26. Silversides CK, Marelli AJ, Beauchesne L, al. Percutaneous aortic valve implantation: et al. Canadian Cardiovascular Society A case report. Can J Cardiol 2006;22:1159- 2009 Consensus Conference on the man- 1161. agement of adults with congenital heart

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