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History and Evolution of the Treatment of Adult Congenital Heart Disease

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History and Evolution of the Treatment of Adult Congenital Heart Disease Marla Kiess, MD, FRCPC History and evolution of the treatment of adult congenital heart 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: Cardiology 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 arteries. 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- veins 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 cardiopulmonary bypass technol- Wilfred Bigelow determined how to clinic. A review of advances in the ogy in the 1950s, followed in the use total body hypothermia for open treatment of CHD reveals dramatic 1970s by the development of deep heart surgery 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 surgeries 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 aorta 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 oxygenator.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 blood flow to the brain was not Dr Bonhoeffer13 and prosthetic aortic pediatric cardiology clinic at Johns always optimal and surgeons 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 artery 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 pulmonary artery 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, pulmonary valve 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 echocardiography 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-lung machine was for closure of the great arteries. There was an of an atrial septal defect 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 stents 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 Mayo Clinic to lungs. 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 vein 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 superior vena cava 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.
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