Congenital heart surgery at Children's Hospital of Wisconsin - A journey
S. Bert Litwin, MD, pediatric cardiothoracic surgeon and medical director emeritus, Cardiothoracic Surgery, Children's Hospital of Wisconsin.
Origins of cardiac care
The records of Children's Hospital of Wisconsin (called Milwaukee Children's Hospital until 1985) show that as early as the 1920s patients with cardiac disease were seen in the clinics with the diagnoses of rheumatic fever and infectious diseases and an occasional patient with congenital heart disease. In the 1940s, a formal Cardiology Clinic was established and was staffed by pediatricians, internists and adult cardiologists including Drs. Feldt, Rosenbaum, Lustok and others. Malcolm Rogers, MD, was the head of this new Cardiac Department. Parallel activities in other parts of the world included the first successful closure of a patent ductus arteriosus by Gross in Boston (1938), first successful Blalock-Taussig shunt in Baltimore (1944), first successful coarctation repair by Crafoord in Stockholm (1944) and by Gross in Boston (1945), and the first successful descending aorta to pulmonary artery shunt by Potts in Chicago (1946).
In 1946, Lustok felt there were patients with surgically correctable heart disease in the Milwaukee area. The cardiac group became a more formal organization and established lectures on pediatric heart disease. The following year this group approached Children's Hospital administration with the idea of developing surgery for these anomalies at our institution. In late 1947, John Steele, MD, was appointed director of this new program and monthly discussion conferences were established. In 1948 Steele and Paul Hausman, MD, were asked to manage a patient with pulmonary atresia. During the next two years they carried out ductal ligations on eight additional patients.
The first cardiac catheterization laboratory in Southeastern Wisconsin was built in 1952 at the Emergency Hospital on Wisconsin Avenue at 20th Street. Meanwhile closed heart surgery programs were being developed in adult hospitals in the city. Around this time Gibbon in Philadelphia (1953) performed the first successful open heart operation using cardiopulmonary bypass and Lillehei at the University of Minnesota (1954) performed the first repair of tetralogy of Fallot using cross circulation without a heart lung machine. Children from southeastern Wisconsin were being sent to one of 10 designated regional Public Health Service Hospitals (Chicago, Mayo Clinic and University of Minnesota were three) for surgery so Children's Hospital physicians felt we should proceed with plans for a cardiac unit at our institution. In 1956 J.C. Peterson, MD, the director of Pediatrics at Children's Hospital, and Howard Correll, MD, the director of Research at Marquette University Medical School, obtained funds through a U.S. Public Health Service grant for construction of a cardiac catheterization laboratory at Children's Hospital. William Gallen, MD, had completed pediatrics training in 1953-55 followed by pediatric cardiology training at John Hopkins Hospital with the renowned Helen Taussig, MD, and further training at the Karolinska Institute in Stockholm. After that he returned to Milwaukee and was named director of the new Catheterization Laboratory. Simultaneously a surgical program was taking root. Derward Lepley, MD, completed surgical training at the Milwaukee County Hospital in 1953 where he developed an interest in heart surgery. He spent the next year in Minnesota with Lillehei for further training after which he returned to Milwaukee. In 1955 he and Wilson Weisel, MD, began animal experiments at County Hospital to develop open heart surgery.
The first cardiac catheterization procedure was carried out by Gallen at Children's Hospital in 1958 while Lepley and Hausman were nearing completion of their experiments to develop open heart techniques. On March 10, 1959 the first open heart operation in southeastern Wisconsin was performed at Children's Hospital by Lepley, Hausman, Weisel, and General Pediatric Surgeon Shimpei Sakaguchi. George Krueter, MD, delivered anesthesia and Ace Adams ran the heart lung machine. The surgery was repair of an atrial septal defect, and it was successful. During the next three months 10 additional patients underwent open heart repair of anomalies.
Seeds of a full-time cardiac surgical program
In the early 1960s multiple surgeons continued to be interested in congenital heart surgery and performed open heart repairs at our hospital. In 1965 Marvin Glicklich, MD, a general pediatric surgeon who trained with Potts in Chicago, came to Milwaukee and began performing closed heart procedures. Results of open heart surgery were less than ideal since no single surgeon gained the needed experience. For two years beginning in 1968 Lepley decided the open cases should be performed at his base hospital, St. Luke's Medical Center. It later was realized that this limited the experience at Children's Hospital, so in 1970 Lepley and his new associate, Robert Flemma, MD, returned to Children's Hospital to carry out children's open heart surgery. That same year David Friedberg, MD, joined Gallen in Cardiology bringing with him extensive experience in the care of infants and the regionalization of pediatric heart care. Friedberg came from Boston where he was instrumental in developing the New England Regional Cardiac Program. During the next few years it was becoming apparent that a full time cardiac surgeon was needed at Children's Hospital if the open and closed heart program was to develop appropriately. I had completed training under Gross in Boston and was working on staff at Boston Children's Hospital, which was a world leader in infant heart surgery. I was recruited to take this new position and arrived in December 1972 with the support of all involved surgeons and cardiologists and the mandate to develop our pediatric cardiac surgery.
Children's Hospital, located on Wisconsin Avenue at 17th Street, had just completed construction of a new intensive care unit. This was ideal for the development of the new service. At the time there was no widely accepted pediatric respirator. Children's Hospital neonatologists Grauss and Raab had developed a device suitable for infants, and it was used effectively for many years. The anesthesia department with Kreuter was helpful from the beginning and he, in 1973, recruited Samir Mitra, MD, to perform cardiac anesthesia. In 1975 John Thomas, MD, finished his training and was a very productive addition to the cardiology group. For one year in 1977 Dale Geiss, MD, was an associate surgeon; however he left to become director of Cardiac Surgery at St. Francis Hospital in Peoria. Leonard Kleinman, MD, completed training in 1978 and joined the staff of St. Luke's as well as Children's Hospital. He was very supportive on a part time basis for many years and remains on the staff today. Between 1978 and 1982 Jessica Lewis, MD, became the first full-time intensivist in the ICU. She left to take a position in Houston and in 1983 Drs. Tom Rice and Kevin Kelly came to continue ICU development.
In 1985 Nancy France, MD, was hired as the new full time director of the Anesthesiology Department. She recruited many outstanding physicians some of whom became interested in cardiac work. George Hoffman, MD, was one of these, and he became director of that department in 1987. After Gallen's retirement in 1989, Friedberg was appointed chief of Cardiology. He began a major recruitment effort which included the hiring of Drs. Peter and Michele Frommelt who developed the institution based field of echocardiography. One significant development was the introduction of intraoperative transesophageal ECHO, which revolutionized evaluation of intra cardiac repairs. He also hired Stuart Berger, MD, who was working at the University of Chicago in pediatric cardiology and intensive care. Friedberg retired from the chief position in 1991, and Berger was appointed to that post. With the growth of the surgical program in 1993 to more than 420 cases per year, a second full time surgeon was needed. James Tweddell, MD, had completed training at the Children's Hospital of St. Louis and joined our staff. He was recruited in part because of his expertise in the field of transplantation. The volume of work increased further and Robert Jaquiss, MD, was added to the surgical staff in 2000. In 2001 I stepped down as director of Cardiothoracic Surgery, and Tweddell was appointed to that position.
Many services have played an outstanding role in the development of cardiac surgery. Not the least of these is the Department of Radiology, initially under the direction of Donald Babbitt, MD. Jack Sty, MD, came to our hospital in 1976 and assumed that directorship in 1981. He brought to this institution many techniques which are applicable to cardiac patients, including nuclear cardiology and CT and MRI scanning. Physicians in Nephrology, Pulmonary Physiology, Infectious Diseases, Laboratory Medicine, Neurology and many others have played a vital role in the care of cardiac patients. In 1979 Jon Vice came to Children's Hospital as executive vice president and in 1981 was named president of the Board of Directors. From that time administrative support for the rapidly growing cardiac program was a vital ingredient in the program's success.
Operations first performed at Children's Hospital
After I arrived in December, 1972 heart care and pump teams were assembled. Pumps and other equipment were purchased, cardiac OR nursing was organized, anesthesia coverage arranged, and ICU care established. A new perfusionist was hired and placed in a three month training program. In early December, before our open heart surgery capability was organized, a surgical emergency arose. A young boy with tunnel type of subaortic stenosis was in crisis. With left ventricular pressures over 300, the patient was in serious heart failure with refractory angina pectoris. Something had to be done immediately. An older heart lung machine was brought in and equipment borrowed from other hospitals. Adams, the senior perfusionist from the St. Luke's group, agreed to run the pump. On Dec. 14 the child was taken to surgery and successful repair of severe subaortic stenosis carried out. There were no other open heart emergencies until the first planned open heart surgery was carried out on March 1, 1973. Three months later, the use of deep hypothermic circulatory arrest was started to provide an ideal operating environment for complex intracardiac repairs in small infants. In 1976, the use of activated clotting time to precisely monitor heparin and protamine dosage and usage intraoperatively was started. The following year the first patient underwent surgery using the newly developed technique of aortic clamping and cardioplegia which resulted in improved myocardial preservation and provided a much better operating field as compared to the previously used intermittent coronary perfusion and fibrillation by intermittent aortic cross clamping. In 1978 the first patient had surgery using the cosmetically attractive transverse skin incision for median sternotomy.
Although Lepley and Flemma had performed some complex operations for anomalies such as tetralogy of Fallot and transposition of the great arteries, the era of routine performance of complex infant surgery started in 1973. Primary total repair of tetralogy of Fallot was used for most patients reserving palliative shunts mainly for small infants who would need reconstruction of the right ventricular outflow tract at the time of later repair. As years passed, primary total repair was used for smaller patients so that today initial palliative shunts are rarely performed for this anomaly. Transatrial repair was first carried out in 1986 and advantages of this operation now make it the gold standard for tetralogy repair. From 1973 through 2001, 434 tetralogy repairs were performed at Children's Hospital with survival of 95.9 percent. The early deaths occurred in patients with complex tetralogy with 100 percent survival in patients with simple tetralogy. In the past six years 133 corrective operations have been performed with 100 percent survival.
With a large backlog of older patients with transposition of the great arteries, reparative surgery for this anomaly was badly needed, and this was started in 1973. The Mustard repair for atrial switch or redirection of venous return to the heart was the operation utilized since it was the one with most predictable results. A patch of pericardium was placed in the atrium to divert SVC and IVC blood to the mitral valve and pulmonary venous blood to the tricuspid valve. During the next ten years this operation was performed in 72 patients with 86.1 percent survival.
As with transposition there were many patients seen with various forms of atrio ventricular communis. The crux of the heart was underdeveloped with a high VSD, low primum ASD, and single intracardiac AV valve. Corrective surgery was the best option for these children even though most centers performed pulmonary artery banding as palliation in those younger than two years. Complete repair of AV canal was first carried out here in 1974. There were no survivors across the world following surgery under age 2 but progressively over the coming years here and at other hospitals the age at the time of operation was reduced. With increased experience in complex operations in small infants mortality statistics for repair of AV canal have improved greatly with operations performed at a younger age. Now most corrective procedures are carried out at 3-5 months of age. At Children's Hospital, from 1974 through 2001, 218 patients have undergone total repair of complete AV canal with 91.3 percent survival. In the past five years 75 have had repair with 98.7 percent survival.
For tricuspid atresia and single ventricle anomalies the Fontan procedure (connection of the excluded right atrium to the pulmonary artery) was being performed at experienced institutions. The first Fontan procedure performed at Children's Hospital was in 1977. From that year until 1992, 41 single stage operations were performed with 71 percent survival. In view of this less than ideal survival, a staged repair for these anomalies started in 1989 when the first bi-directional caval pulmonary shunt or first stage Fontan operation was performed. Since then this has become the gold standard for repair of single ventricle. Between 1989 and 2001, 234 infants and young children have undergone first stage repair with 99 percent survival while 148 of these have gone on the second stage or completion Fontan repair (connection of the IVC to pulmonary artery) with 96.6 percent survival.
In 1982 the first Senning atrial switch for transposition of the great arteries was carried out. This operation was taken up in order to be able to offer atrial switch safely to infants under six months of age. The technique of this operation allows the surgeon to do a more precise repair on the small neonatal or infant heart. From then until 1994, when the procedure was abandoned in favor of the arterial switch repair, 64 infants and children underwent the Senning procedure. Survival was 100 percent until the 59th patient which was the only mortality yielding a total survival of 98.4 percent.
In 1975 Jatene in Brazil reported the first survivor after arterial switch or anatomic repair for transposition. During subsequent years world results were poor with about 25 percent survival. In 1984 it seemed appropriate for us to utilize this procedure in selected cases, Yacoub in London had refined the operation obtaining increased survival of 50-60 percent. At Children's Hospital results for the Senning procedure were excellent and the change to the arterial switch concept was thought provoking. The theoretical advantages of arterial switch including a marked decrease in late arrhythmias and avoidance of late systemic ventricular failure as seen following atrial switch seemed attractive. We performed our first arterial switch in 1984, and there were 16 survivors in the first 17 cases. By 1994 we used it more and more and since then it is the procedure carried out exclusively for repair of transposition. Between 1984 and 2001, 167 patients have undergone arterial switch with 92.8 percent survival. From 1996 through 2001, 67 operations were performed with 98.5 percent survival.
In 1984 Children's Hospital became one of three centers in the country to start using homograft aortic and pulmonary valves to reconstruct the right or left ventricular outflow tract of malformed hearts. Our first homograft was placed that year and since then 245 procedures have been carried out. In 1992 the first Ross operation was done (pulmonary autograph for aortic valve replacement in conjunction with pulmonary homograft for right ventricular outflow tract reconstruction). Finally, in 1996 the first mitral valve homograft replacement was performed.
In 1986, after much thought about the need for a ventricular support program as an adjunct for very sick patients following complex surgery, the ECMO program was started. I, along with Glicklich, trained in Michigan under Robert Bartlett, MD, who developed many of the basic techniques. On returning to Milwaukee, as plans for the program were underway, Rice and Sue Day, MD, from the Pediatric Intensive Care Unit became close collaborators in establishing this program. The first ECMO procedure was performed in 1986 for respiratory support of a very sick infant. Since then the program has included 262 patients. In 1997 the technique first was used for ventricular support and since then 77 cardiac patients have benefited from this technique.
In the 1980s pediatric heart transplantation was a rapidly growing field. It was felt that this technique should be available for infants and children as a last resort when no other surgical or invasive procedure could prevent death. In conjunction with Drs. Ed Dunn and Robert McManus, I established the program with the first transplant procedure in 1991. The same planning was used to establish a lung transplant program. When the first clinical case was near, Tweddell joined our staff and carried out the procedure. He was hired to not only take an active role in the cardiac surgery program but also to foster and bring forward the entire transplant program. He has done well and between 1991 and 2001, 24 heart transplants have been performed with long term survival of 79.2 percent. In addition he also has performed 4 heart/lung transplants (survival 50 percent) as well as 15 lung transplantations (survival 73.3 percent).
For many years beginning in 1969, I was interested in surgery for hypoplastic left heart syndrome establishing the concept of placing the right ventricle in the systemic circulation. William Norwood, MD, working initially in Boston and later in Philadelphia, refined a technique for plastic reconstruction of the aortic root reporting his first successful case in 1984. In 1990 at Children's Hospital the first successful case underwent surgery. Results across the world continued to include a survival of only 40-50 percent. Berger, the new chief of Cardiology, and I traveled to Philadelphia for training with Norwood and continued to make progress in the development of our program. Between 1992 and 2001, 139 Norwood procedures were performed with 77 percent survival. In the past four years 84 operations have been carried out with 93 percent survival; while in 2001, 23 Norwood procedures were performed with 100 percent survival. These superb recent results largely are due to the efforts of Tweddell and Hoffman and involve major advances in postoperative care as well as technological aspects of the surgery.
From humble beginnings we have become one of the leading congenital heart centers in the country if not the world. From 1972 through 2001, 9,373 heart operations have been performed with 95.2 percent total survival. In the past five years we have carried out 3,001 procedures with a 98.4 percent survival, 1,734 of which were in infants with a 98.4 percent survival. And the best is yet to come.
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