Jane Somerville

I have witnessed this history and want to share it…

Cardiology in the 1950s was just emerging as a specialty, strongly resisted by the professors of medicine in the United Kingdom and in Europe, where threats of resignation occurred should this separate from the overlords of general medicine. Such were the powers of the professors of medicine in the university cities. This delayed the formation of specialty cardiology, but did not stop it. The possibility and intentions of cardiac surgeons hastened and made it mandatory. The seeds of specialty cardiology were planted in the 1920s and beginning to show above ground, stimulated by the invention of roentgenology (x-rays) screening, Eindhoven’s electrocardiogram, and later in the 1930s such towers of medical strength, D. Evan Bedford and John Parkinson in the United Kingdom, Gallavardin in France and Paul Dudley White in the United States. World War II interrupted the development despite Cournand’s 1941 invention of cardiac catheterization in 1941, for which he received the Nobel Prize in 1956. War also interrupted the flourishing of cardiac surgery for congenital heart defects initiated by Gross ligating an infected duct (1938) as suggested to him by the pediatrician. Cardiac catheterization with heart chamber pressures and saturations proved to be mandatory for accurate diagnosis required by the cardiac surgeons emerging as specialists from established thoracic surgery. It was accompanied by angiocardiography, initiated by A. Castellanos in Mexico, to show anatomy illustrating the valve by injecting mercury into the veins of a Cuban boy, with disastrous result. The interest in congenital heart disease flourished because of the challenge to cardiac surgeons and the new cardiology, which could make accurate diagnosis. The efforts of physicians to keep up with the needs of the innovative surgeons in the 1950s and 1960s brought correct preoperative diagnosis.

In this era, having heard of the new triumphs of cardiac surgeons, many patients with congenital heart problems (children, adolescents, and adults) staggered in wherever there was some expertise and interest—the National Heart Hospital, Middlesex Hospital, Guy’s Hospital, Hammersmith Postgraduate Hospital led by Sir John MacMichael in London, other cities outside the United Kingdom, Paris with Dubost, Munich, Italy, Boston, Mayo, and many more in the United States.

Mortality, morbidity, and inaccurate diagnoses were high. Competition between units was intense, and conferences to share and show off achievements started. Atrial septal defects were most frequent, and coarctation, ventricular septal defects, and lesions requiring surgery with many complications appeared. However, the greatest challenge came from the blue patients with Fallot tetralogy, often having already survived a shunt procedure (of sorts) or just a thoracotomy. The presence of pulmonary hypertension received much effort along with the need to understand the most serious and common patients with Eisenmenger reaction, so clearly elucidated and clarified by the wisdom of Paul Wood in his Croonian lectures (1958). Various forms of aortic and pulmonary stenosis were assessed by invasive tests because surgical treatment was routine, and some frightening complications occurred after “successful” operations for coarctation, until the importance and control of postoperative hypertension was understood. Success was survival and leaving the hospital. No long-term goals were considered. This is surprising, and many clearly visible abnormalities remained in these hearts.

As cardiac surgery and diagnosis improved, surgical ambition and ability expanded. In addition, the need for treatment of congenital heart diseases for infants and newborns was recognized because more than 65% of those born with congenital heart anomalies died in the first year of life. This brought new challenges in anesthesia, nursing, technology, and the obvious need for the new specialty of pediatric cardiology.

Pediatric cardiology suffered many of the same difficulties and fights as cardiology had to separate itself from general pediatric departments and their professors, particularly in Europe. It was less difficult to separate from cardiology because cardiologists did not understand the new language of congenital heart disease, although they accepted their right to continue care of adults with congenital heart diseases because they had always done so, and these patients were “interesting” and still are! In the United States, they were less territorial. North America, with its penchant for orders and numbers, had established boards, examinations, and training standards where the volume of patients attracted by cardiac surgery were adequate. South America muddled on for decades with occasional islands of hope and help, such as San Paolo, Brazil, and Children’s Hospital in Buenos Aires and Santiago, Chile, and the work of an extraordinary and “scary” surgeon, Dr. H. Jaeger who, with German thoroughness, made good services available for children by the late 1960s. By the 1970s, successful pediatric cardiac surgical units were established with centralization of patients in the United Kingdom with the help of designated above region (supraregional) centers launched in a number of areas, although these centers were not always well chosen or audited. This occurred with less restriction on centers in the United States and much of Western Europe.

Management of congenital heart disease was considered to be a pediatric problem requiring refined new skills by the mid-1970s, but with no thought beyond the horizon of adolescence. Patients with so-called “total corrections” performed for Fallot and other lesions, the much promoted dream of cardiac surgeons, were already returning with new medical needs. Now adolescents or adults, but with no warnings given to parents or patients of future problems. Who knew? Who cared? Certainly not the cardiologists who avoided or ignored the complex issues, leaving patients to pediatric cardiologists unprepared and untrained (despite the wishes of many to retain “parental control” of the patient). They understood the language and the defect but did not have the facilities or skills to deal with adult patients.

The need for a new subspecialty was clear and recognized by Joe Perloff, who was profoundly influenced by Paul Wood in his training as a fellow in adult cardiology at the National Heart Hospital, London. He learned from Wood the art of bedside cardiology, so useful when working with older congenital heart patients. In 1975, an adolescent cardiac unit, rightly named for Paul Wood, was opened at the National Heart Hospital to provide for the growing community of survivors from the successful efforts of David Waterston and Dick Bonham-Carter, a harmonious team of an impeccable surgeon and clever pediatrician in Great Ormond Street Hospital for Sick Children, London.

In 1959, the forward-thinking pediatric cardiologist, John Keith, of the Toronto Sick Children’s Hospital and author of the best textbook, saw the obvious need for continued follow-up of his patients, mainly for rheumatic heart disease, through to adulthood to ensure that the vital prophylaxis continued. In Toronto, led by Mustard (1958), an orthopedic surgeon who followed Senning (1954) in doing early atrial switches for transposition of the great arteries, had produced a number of long-term survivors, who had many late cardiac problems. Soon congenital heart patients exceeded the rheumatic hearts, just as the numbers of grown-up congenital heart (GUCH) patients will now exceed their pediatric counterparts. Keith asked John Evans from his own clinic to start an adolescent/adult clinic in the Toronto General Hospital, joined by a bridge to the Children’s Hospital, allowing the necessary independence and autonomy of the new services. Whether he wanted to redirect John Evans, a restless entrepreneur, into a different clinic is unknown. John Evans did not stay long as a cardiologist, and left to become a successful businessman. The clinic was small, but continued by Dr. John Woolfe until the 1980s. Gary Webb, appointed cardiologist at Toronto General Hospital in 1973, took over the adult congenital service in 1980 as his major responsibility as a result of the need created by the successful cardiac surgeons of Toronto Sick Children’s Hospital. Gary, master communicator, set up a good, integrated group of cardiologists, organized care of GUCH disease across Canada, and received a steady flow of patients/graduates of the Toronto Sick Children’s Hospital. Dick Rowe and Bob Freedom supported this need, which was clearly better than “obstructive” developments elsewhere in the world, where many pediatric cardiologists of necessity continued to see these patients in a pediatric clinic. The Toronto group was particularly concerned about the many survivors of the Mustard operation and I attended a celebration party for all of them in the 1980s.

Trained as an adult cardiologist and having spent years at Great Ormond Street Hospital seeing real congenital heart disease patients, not just the tail of surviving adolescents and adults, I was rewarded by Dick Bonham-Carter with two sessions as a Senior Lecturer at Great Ormond Street in 1968, one of which I kept until I retired, 30 years later. Having failed to get on any shortlist for appointment as a consultant in pediatric or adult cardiology (I was not considered by the hierarchy as useful because I was not a pediatrician or a conventional cardiologist), I established a new specialty, treating the older patients with congenital heart disease, because their numbers were bound to increase. I believed that this approach would make me the only applicant for a job if such a need was ever recognized.

In 1972, I was appointed as a consultant cardiologist for congenital heart disease at the National Heart Hospital, London. This was a unique appointment and title with no age barrier. It had access to adult beds but also to four children’s beds, the latter created against the wishes of some of the senior physicians. The professor of cardiology at the time did not particularly like these complex patients in the unit beds, and therefore supported the new appointment in the National Health Service and in the university. The success of this adventure was not only a result of Dick Bonham-Carter’s referrals of his older survivors and tricky new adolescents, which he had always referred to Paul Wood for diagnosis, but mainly to the remarkable surgical innovations and successes of Donald Ross, whose homograft valves repaired pulmonary atresia, Fallots, other cyanotic complexities, and challenging patients with aortic stenosis, and brought nationwide referrals during the golden era of the National Heart Hospital. This created contact opportunities to talk around the world about the emerging needs of adolescents and adults with congenital heart disease and for training enthusiastic young colleagues.

I was doing a stint as a visiting professor in Toronto, invited by a rare, real friend in pediatric cardiology, Bob Freedom, who arranged a lunch with Gary Webb. Realizing there were too few patients coming to Dr. Webb’s service, I persuaded Freedom that all patients needed to transfer, and this led to an increased flow of selected patients across the bridge, from the Hospital for Sick Children to the General Hospital, Toronto. The Toronto General group, led by Gary Webb, made a large contribution to establishing adult congenital heart services with excellent imaging directed by Peter Liu, good surgery by Bill Williams, excellent diagnostic cardiac catheterization, and subsequent interventions by Peter McLoughlin, and a world-class, designated congenital heart database. They had it all with Gary on the Internet, long before anyone else had thought of its use to unite, communicate, and establish patient groups; furthermore, the Toronto group promoted education with their annual adult congenital heart course.

The years after produced clarion calls to create designated cardiac services for these unique groups of GUCH patients. The final plenary session on the Future of Paediatric Cardiology during the first World Congress of Paediatric Cardiology in London in 1980 was on “Adolescent Survivors’ Triumphs and Disasters.” From then on, the battle to establish GUCH services was waged. Lectures and conferences, world congresses, official national reports from Canada, Bethesda reports in the United States, also influenced by pediatricians, European Society of Cardiology, and British Cardiac Society Working group reports, were almost destroyed by pediatric cardiologists, but rescued and launched with influence of the president of the latter, Professor John Camm. Patient associations formed in the United Kingdom and Canada, followed by the United States, Holland, Norway, and Germany working with interested physicians. Joe Perloff from the University of California, Los Angeles (UCLA) devoted time to write his first book in the field, with more to follow.

One wonders why the services for GUCHs have been so difficult to establish with so many patients in need. There still seem to be many obstacles to this effort in many parts of the world. There are many different names for the same patient group; one can use what suits the language, but the need is the same. It is not something to squabble about.

Financing is a problem, and allotting funds, areas, and training are present difficulties. Pediatric cardiology is often a main stumbling block because many do not want to give up patients, particularly where money is concerned (as in United States), parents do not like change after years of the same good care and understanding, and pediatricians register concern about no specialist unit to which their patients can be referred. Perhaps this is a chicken and egg problem? Equally, adult cardiology has not wanted to understand or have any training in the last 30 years, nor do they want to give funds or beds. They want the adult cardiology department filled with money-generating coronary artery disease patients, for interventions. Cardiac surgery for GUCHs (adults!), where it should be done, and by whom, is an ongoing battle. The jointly trained adult/pediatric cardiac surgeon is the answer, but this involves long training and joint appointments, although now obligatory, in specialized linked units, which are few and difficult to staff. One in five admissions to a specialist GUCH unit is for surgery, often combined with catheter intervention; where this is done may be problematic because pediatric ICU nurses and staff may not be familiar with adult care, whereas care in an environment of adult care unfamiliar with congenital heart matters may not be safe. Health care administrators are generally disinterested unless catastrophe reaches the press or someone with political influence has a child with congenital heart disease. There have been a few exceptions to this, as at the Royal Brompton Hospital for example, where the CEO thought GUCH was a unique selling point (USP). Physicians are “selfies” and constantly in rivalry, or are part of hospitals, units, or departments that want to keep their patients irrespective of expertise or needs. Recently in the United Kingdom, pediatricians have managed to obtain a ruling that they can keep patients under their care until the age of 19 years. Not helpful and, when GUCH patients are too mature to be admitted to children’s hospitals, they are rapidly referred when pregnant, when needing contraception, or when gravely ill with arrhythmias, when septic, or when needing surgery.

Pediatric cardiology philosophy had to be modified by the ban on admitting patients older than 16 years to children’s hospitals, particularly in the United States, but also in other countries. Management of GUCHs, or whatever name is chosen, has created a formula for chaos and mistakes. There are training issues only recently addressed by a formal plan and process. It is easier in countries with a funded health service, as in the United Kingdom and other European countries, and best when pediatric cardiologists are willing and share their patients’ care and expertise with physicians treating adults; Malta, a small area that is full of GUCHs, is a good example of this desirable collaboration.

Education of all is vital to establishing and maintaining optimal care. This book is necessary, and it is remarkable and praiseworthy that the authors are prepared to update this work. It is now accepted that only a few congenital hearts can be considered as totally corrected, although a good life, close to normal, is possible for many patients, provided they receive good, lifelong care. Education is clearly paramount to improve further care and patients’ understanding of their condition. This book will serve to achieve this worthwhile goal.