Ann Thorac Surg 1997;64:1533-1539
© 1997 The Society of Thoracic Surgeons
Supplement: Cardiovascular Surgery: Then and Now
The Early History of Congenital Heart Surgery: Closed Heart Operations
John A. Waldhausen, MD
Division of Cardiothoracic Surgery, Department of Surgery, The Pennsylvania State University College of Medicine, The Milton S. Hershey Medical Center, Hershey, Pennsylvania
Abstract
The history of cardiovascular surgery before the development of open heart techniques is presented. Emphasis is placed on the creativeness and boldness of the visionary pioneers whose skills and determination led to the modern era of the routine surgical treatment of heart disease.
Cardiac surgery is an accomplishment of the twentieth century. A comprehensive review of the development of cardiac surgery has been written by Harris B. Schumacker, Jr, and is an in-depth analysis of the contributions of many surgeons to the development of this new field [1]. The present report will cover only some of the highlights focusing on surgery for congenital cardiac defects and take the story up to the development of open heart surgery.
Initial attempts at operating on the heart were exclusively confined to the repair of cardiac wounds. Several attempts had been made and documented in the literature, but it was Ludwig Rehn who in 1896 was the first to successfully close a stab wound of the right ventricle [2]. Thereafter, little occurred in clinical cardiac surgery. However, of greatest significance to the future development was the work of Alexis Carrel (Fig 1A
), a French experimental surgeon working at the Rockefeller Institute. Among his many contributions to cardiovascular surgery and transplantation, the development of the principles of vascular anastomosis were the most important, initially laying down the foundation of the Blalock-Taussig shunt, coarctation repair, and virtually all of modern cardiac and vascular procedures [3]. For his work he was awarded the Nobel Prize in Medicine in 1912.
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Fig 1. . (A) Alexis Carrel (1872–1944), Associate, Rockefeller Institute, and Nobel Laureate. (Courtesy National Library of Medicine, Bethesda, Maryland.) (B) Robert E. Gross (1905–1988), Professor of Surgery, Harvard University and Boston Children's Hospital. (Courtesy National Library of Medicine, Bethesda, Maryland.)
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Elliott Cutler at Harvard Medical School and the Peter Bent Brigham Hospital tried to open a stenotic mitral valve in 1923 using a tenotome knife. Later he developed a "cardiovalvulotome" of his own design. Altogether he operated on 7 patients, with poor results and an 86% mortality [4].
No further advances in cardiac surgery were made until the late 1930s. Why was there this long hiatus? In part, it was due to a lack of refinement in anesthesia, problems related to ventilation of the lung, and, of course, many of the other now routine perioperative support techniques, such as blood transfusions. However, the next advances, primarily related to congenital heart disease, would come in rapid progression.
In 1937, John Streider of Boston tried unsuccessfully to ligate a patent ductus arteriosus [5]. However, in 1938 Robert Gross (Fig 1B
), also of Boston, was successful in ligating, for the first time, a patent ductus arteriosus [6]. Legend has it that he had been told by his chief, Dr Ladd, not to do the operation. However, when the latter was out of town, Gross did the operation and many have chosen this date as the start of modern heart surgery. There is no question that shortly thereafter a host of new operations came into being, most notably the Blalock-Taussig shunt for cyanotic congenital heart lesions. Helen Taussig (Fig 2A) of Baltimore had devoted her career to the diagnosis of congenital heart disease in infants and children. Before her time, congenital heart lesions were more a curiosity in the pathology laboratory and few physicians, if any, had been able to make a clinical diagnosis in living patients. Using simple methods such as physical examination, a chest roentgenogram, and an electrocardiogram, Dr Taussig was able to identify specific cardiac lesions. Thus, a blue baby with a small heart, anemic lung fields, and right-axis deviation on the electrocardiogram was most likely in the tetralogy group. On the other hand, a cyanotic baby with an enlarged heart, plethoric lung fields, and biventricular hypertrophy on the electrocardiogram was more likely to have transposition of the great arteries. Doctor Taussig recognized that patients with tetralogy had inadequate pulmonary blood flow and suggested to Dr Blalock (Fig 2B) that perhaps these babies could be helped by creating an artificial ductus. For some time, Dr Blalock had been working on creating an artificial ductus in his physiologic experiments to produce pulmonary hypertension. In 1944, he performed the first subclavian-to-pulmonary artery shunt operation in a baby. Shortly thereafter Drs Blalock and Taussig reported their results in the first 3 patients in the Journal of the American Medical Association (Fig 3) [7]. It was an electrifying report and was perhaps the greatest stimulus thus far to the development of cardiac surgery. It came at a time when the Second World War was coming to an end and many bright young men and women were being discharged from the Armed Forces who could devote their time to the research laboratory as well as to the development of this new field.
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Fig 2. . (A) Helen B. Taussig (1898–1986), Professor of Pediatrics, The Johns Hopkins University. (Courtesy National Library of Medicine. Bethesda, Maryland.) (B) Alfred Blalock (1899–1964), Professor of Surgery, The Johns Hopkins University. (Personal photograph owned by Dr John A. Waldhausen.)
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Fig 3. . Blalock-Taussig shunt. (Reprinted with permission from Blalock A. Taussig HB. The surgical treatment of malformations of the heart in which there is pulmonary stenosis or pulmonary atresia. JAMA 1945;128:189–202. Copyright 1945, American Medical Association.)
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Shortly thereafter, Willis Potts of Chicago performed a shunt operation anastomosing the descending thoracic aorta, just distal to the left subclavian artery, to the left pulmonary artery (Fig 4) [8]. The obvious advantage of this operation was that the subclavian artery was not sacrificed. Ultimately, however, this operation proved to be unsatisfactory because it was difficult to make the size of the anastomosis precisely large enough without producing excessive pulmonary flow. One of the unique features of the Blalock-Taussig operation was the fact that the subclavian artery was almost always of sufficient size to provide adequate pulmonary blood flow but not in an excessive amount. Ultimately, the problem of having to divide the subclavian artery in the Blalock-Taussig shunt was overcome by the insertion of a polytetrafluoroethylene shunt between the subclavian artery and the pulmonary artery, thereby achieving appropriate flow without sacrificing the subclavian artery [9].
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Fig 4. . Potts anastomosis. (Reprinted with permission from Potts WJ, Smith S, Gibson S. Anastomosis of the aorta to a pulmonary artery. JAMA 1946;132:627–31. Copyright 1946, American Medical Association.)
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Another type of systemic–pulmonary shunt was developed independently by David Waterston [10] of London and by Denton Cooley [11] of Houston. A posterior aortic-to-right pulmonary artery anastomosis was made. Its advantages were thought to be bidirectional flow into the pulmonary arteries and leaving the subclavian artery intact. Its major disadvantage was frequent distortion of the right pulmonary artery, a major risk factor in subsequent total correction of the underlying lesion.
It was in 1958 that William W. L. Glenn of Yale University performed a superior vena cava-to-right pulmonary artery anastomosis, thus in part bypassing the right heart (Fig 5) [12]. Initially, this operation was done on patients with a variety of congenital cardiac lesions with inadequate pulmonary blood flow. As time went on, however, it became most widely used in the management of infants requiring total right heart bypass: the Fontan operation. Today it is used in a modified form, a so-called bidirectional Glenn, as the first stage of a modified Fontan repair. In the bidirectional Glenn procedure, the superior vena cava is anastomosed to the right pulmonary artery, which remains in confluence with the left pulmonary artery.
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Fig 5. . Glenn anastomosis. (R.A. = radial artery; R.P.A. = right pulmonary artery; S.V.C. = superior vena cava.) (Reprinted with permission from Glenn WWL. Circulatory bypass of the right side of the heart. IV. Shunt between superior vena cava and distal right pulmonary artery—report of the clinical application. N Engl J Med 1958;259:117–20. Copyright 1958, Massachusetts Medical Society. All rights reserved.)
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Coarctation of the aorta was recognized as a serious malformation that was possibly amenable to surgical correction. Of great concern to most surgeons interested in the problem was the fear that clamping the aorta, necessary during the anastomosis, would result in paraplegia. Doctor Blalock, at the suggestion of Edwards A. Park (the chairman of Pediatrics at Johns Hopkins), tried to resolve this problem experimentally in the dog by performing a subclavian turndown operation, anastomosing the distal end of the transected subclavian artery to the descending aorta [13]. Unfortunately, paraplegia developed in many of the dogs after clamping of the aorta, but the subclavian artery did carry sufficient blood to the descending aorta. Clarence Crafoord (Fig 6A) of Stockholm, Sweden, had less concern about the possible paraplegia because he had briefly clamped the aorta in his operations to close the patent ductus. In 1944 he carried out the first successful operation on a patient with coarctation, and 12 days later he did the second [14]. Both had a good outcome. Robert Gross also had been interested in the problem but unfortunately his first patient died. His second patient survived [15]. Coarctation of the aorta was thought by many to have been resolved once excision of the coarctation had been accomplished and an anastomosis had been performed. Some patients, however, needed a graft to bridge the long defect, and it was again Robert Gross and his associates who first successfully repaired this defect with the insertion of a homograft aorta [16]. Ultimately, this had enormous implications for the development of aortic surgery.
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Fig 6. . (A) Clarence Crafoord (1899–1984), Professor of Thoracic Surgery, Karolinska Institute. (Personal photograph owned by Dr John A. Waldhausen.) (B) Lord Brock (Russell Claude Brock) (1903–1981), Surgeon, Guy's Hospital. (Reprinted with permission from Schumacker HB Jr. The evolution of cardiac surgery. Bloomington, IN: Indiana University Press, 1992.)
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The problems of coarctation, however, were not over. There was a group of patients, primarily in early infancy, who generally succumbed to the disease whether treated medically or surgically, although the surgical results were somewhat better. This high operative mortality in infants with coarctation was not resolved until the 1970s and 1980s when the role of the ductus arteriosis was recognized. If the ductus was kept patent using prostaglandin E1, the circulation through the mouth of the ductus to the descending aorta could be maintained [17]. This allowed adequate circulation to the lower half of the body and allowed the patient to stabilize. If, on the other hand, the ductus had closed before the development of adequate collateral arteries around the coarctation, there was ischemia of the lower part of the body, particularly of the kidneys, with inadequate urine output, acidosis, and exacerbation of congestive heart failure. With the ability to stabilize infants using prostaglandin E1, operative intervention became far safer. However, new problems became evident; coarctation in many of these infants recurred. The cause of this was not initially evident but was ultimately attributable to the presence of ductal tissue in the aorta itself as well as to the lack of growth of the aortic suture line. This problem was resolved at first by using a subclavian flap operation that enlarged the aortic isthmus with normal tissue (Fig 7) [18]. The recurrence rate dramatically dropped, but the problem was even further resolved by widely excising the isthmus tissue and then anastamosing the pliable descending aorta to the aortic arch. The use of absorbable sutures was of significant help in preventing an anastomotic stricture [19].
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Fig 7. . Subclavian flap operation for coarctation of the aorta. (Reprinted with permission from Waldhausen JA, Nahrwold DA. Repair of coarctation of the aorta with a subclavian flap. J Thorac Cardiovasc Surg 1966;51:532–3.)
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Long-term follow-up of patients with coarctation of the aorta showed an alarming incidence of hypertension, even in the presence of a patent anastomosis [20]. It was difficult to ascertain what the exact causes of this late hypertension were. Although a number of different theories were proposed, it appears that the operation is best performed early in life, and there is good evidence to show that late hypertension is less likely to develop in those patients operated on between the first and second year of life.
Shortly after Blalock's and Taussig's monumental contribution, it was recognized that there were patients with pulmonic stenosis and subpulmonic stenosis who might benefit by a direct approach to the pulmonary valve. Although the problem had been discussed by a number of surgeons, the credit belongs to T. Holmes Sellors in London, who in December of 1946 performed the first successful valvotomy [21]. Originally, Sellors had planned to do a systemic-to-pulmonary shunt on a patient who was diagnosed as having severe tetralogy of Fallot. But because of bilateral pulmonary tuberculosis, this approach was abandoned. Using a long tenotomy knife that was passed through the right ventricle into the pulmonary artery, he incised the stenotic valve. Unaware of this event, Russell Brock (Fig 6B), also in London, was working on the problem of pulmonary stenosis. Originally he tried to approach the valve from above but abandoned that in favor of a transventricular approach using a valvulotome (Fig 8) [22]. This soon became the standard approach for pulmonic stenosis until the development of open heart surgery, when the valve was approached directly through the pulmonary artery. Today the operation is almost exclusively performed using balloon catheter dilation.
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Fig 8. . Transventricular pulmonary valvotomy. (Reprinted with permission from Waldhausen JA, Pierce WS. Johnson's surgery of the chest, fifth ed. Chicago: Yearbook Medical Publishers.)
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Surgery to correct septal defects, in general, had to await the development of open heart surgery. However, three operations that were performed in the closed heart era deserve mention. In 1952, Dr Gross and his associates first described the use of the atrial well technique to close atrial septal defects (Fig 9). A rubber funnel was sutured to an incision in the atrial wall and the defect was closed through the blood that filled the funnel [23]. Thus, the operation was not done under direct vision but by palpation. The technique was also applicable to the creation of atrial septal defects, which had previously been performed in 1948 by Blalock and Hanlon at Hopkins using a complex technique in which a portion of the left and right atria were occluded with a partial exclusion clamp while the pulmonary artery and veins to that lung were also occluded [24]. By incision of both the right and left atria, the septum between the two was freed and then could be excised. The right atrial wall was then sutured to the left atrial wall (Fig 10). These operations were performed primarily for transposition of the great arteries to allow mixing of systemic and pulmonary venous blood at the atrial level and were the only palliative operations for that condition until they were superseded by balloon atrial septostomy in 1966 [25]. Closure of ventricular septal defects had to await the development of open heart surgery. But of great importance was the development of pulmonary artery banding in 1952 by Muller and Dammann, which created stenosis of the pulmonary artery and thus reduced the excessive pulmonary blood flow associated with ventricular septal defects (Fig 11) [26]. It became an important palliative operation for this lesion, even after direct closure was possible, and was done particularly in infants, among whom operative mortality from direct closure initially remained high. With refined techniques in cardiopulmonary bypass, the operation was superseded by direct closure, but it is still used today in specific instances such as multiple ventricular septal defects.
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Fig 9. . Application of a rubber well to the right atrium. (Reprinted with permission from Gross RE, Pomeranz AA, Watkins E Jr, Goldsmith EI. Surgical closure of defects of the interauricular septum by use of an atrial well. N Engl J Med 1952;247;455–60. Copyright 1952, Massachusetts Medical Society. All rights reserved.)
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Fig 10. . Blalock-Hanlon atrial septectomy. (Reprinted with permission from Waldhausen JA, Pierce WS. Johnson's surgery of the chest, fifth ed. Chicago: Yearbook Medical Publishers.)
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Fig 11. . Pulmonary artery banding. (Reprinted with permission from the American College of Surgeons [Surg Gynecol Obstet 1952;95:213–9].)
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Although anomalous origin of the left coronary artery was known for some time, its physiopathology was not understood. It was not until 1958 that Case and Morrow (Fig 12) of the National Institutes of Health confirmed that the flow was in the direction of the pulmonary artery rather than the reverse [27]. Aware of David Sabiston's perfusion studies at Hopkins demonstrating this flow from the coronary artery into the pulmonary artery, they suggested ligation of the left coronary artery at its origin from the pulmonary artery [28]. Unfortunately, their patient died. The operation based on these principles was successfully carried out in 1957 by Edgar Davis at the Washington Children's Hospital [29]. Today, this operation has been abandoned in favor of direct reimplantation of the coronary artery into the aorta.
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Fig 12. . Andrew G. Morrow (1922–1982). Chief, Clinic of Surgery, National Heart Institute, National Institutes of Health. (Personal photograph owned by Dr John A. Waldhausen.)
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Although these attempts by surgeons to correct or palliate cardiac lesions had enormous importance, it was quite evident that only an intracardiac approach would ultimately resolve these problems. Today, however, extracardiac operations such as the modified Blalock-Taussig shunt, the bidirectional Glenn shunt, repair of coarctation of the aorta, and even pulmonary artery banding are important parts of the armamentarium of the cardiac surgeon in managing congenital heart disease. On a busy surgical service for congenital heart disease, approximately one third of the operations are done without the use of the heart-lung machine. Some of the lesions that originally were corrected by a direct approach, such as patent ductus arteriosis and pulmonic stenosis, are now often being managed by using transcatheter techniques. Even atrial septal defects as well as ventricular septal defects are being successfully closed in that manner.
This relatively brief chapter in cardiac surgery before the development of the heart-lung machine was a glorious one, demanding the skills of the best surgeons in the world. Their inventiveness and their boldness represent some of the finest accomplishments in the history of surgery.
Footnotes
Presented at Cardiovascular Surgery—Then and Now, University of Virginia Medical Center, Charlottesville, VA, April 26, 1997.
Address reprint requests to Dr Waldhausen, Division of Cardiothoracic Surgery, Department of Surgery, The Pennsylvania State University College of Medicine, The Milton S. Hershey Medical Center, PO Box 850, Hershey, PA 17033.
References
- Shumacker HB Jr. The evolution of cardiac surgery. Bloomington, IN: Indiana University Press, 1992.
- Rehn L. Ueber penetrirende Herzwunden and Herznaht. Arch Klin Chir 1897;55:315–27.
- Carrel A. On the experimental surgery of the thoracic aorta and the heart. Ann Surg 1910;52:83–95.[Medline]
- Cutler EC, Beck CS. The present status of the surgical procedures in chronic valvular disease of the heart. Final report of all surgical cases. Arch Surg 1929;18:403–16.[Abstract/Free Full Text]
- Streider J. Discussion of Blalock A, Levy SE. Tuberculosis pericarditis. J Thorac Surg 1937;7:132–52.
- Gross RE, Hubbard JP. Surgical ligation of a patent ductus arteriosus. Report of first successful case. JAMA 1939;112:729–31.[Abstract/Free Full Text]
- Blalock A, Taussig HB. The surgical treatment of malformations of the heart in which there is pulmonary stenosis or atresia. JAMA 1945;128:189–202.[Abstract/Free Full Text]
- Potts WJ, Smith S, Gibson S. Anastamosis of the aorta to a pulmonary artery. JAMA 1946;132:627–31.[Abstract/Free Full Text]
- De Leval MR, McKay R, Jones M, Stark J, Macartney FJ. Modified Blalock-Taussig shunt. Use of subclavian artery orifice as flow regulator in prosthetic systemic–pulmonary artery shunts. J Thorac Cardiovasc Surg 1981;81:112–9.[Abstract]
- Waterson DJ. Treatment of Fallot's tetralogy in children under one year of age. Rozhl Chir 1962;41:181.[Medline]
- Cooley DA, Hallman GL. Intrapericardial aortic–right pulmonary arterial anastomosis. Surg Gynecol Obstet 1966;122:1084–6.[Medline]
- Glenn WWL. Circulatory bypass of the right side of the heart. IV. Shunt between superior vena cava and distal right pulmonary artery—report of the clinical application. N Engl J Med 1958;259:117–20.[Medline]
- Blalock A, Park EA. The surgical treatment of experimental coarctation (atresia) of the aorta. Ann Surg 1944;119:445–56.[Medline]
- Crafoord C, Nylin G. Congenital coarctation of the aorta and its surgical treatment. J Thorac Surg 1945;14:347–61.
- Gross RE, Hufnagel CA. Coarctation of the aorta. Experimental studies regarding its surgical correction. N Engl J Med 1945;223:287–93.
- Gross RE, Bill AH Jr, Peirce EC II. Methods for preservation and transplantation of arterial grafts. Observations on arterial grafts in dogs. Report of transplantation of preserved arterial grafts in 9 human cases. Surg Gynecol Obstet 1949;88:689–701.[Medline]
- Heymann MA, Berman W Jr, Rudolph AM, Whitman V. Dilation of the ductus arteriosus by prostaglandin E1 in aortic arch abnormalities. Circulation 1979;59:169–73.[Abstract/Free Full Text]
- Waldhausen JA, Nahrwold DL. Repair of coarctation of the aorta with a subclavian flap. J Thorac Cardiovasc Surg 1966;51:532–3.[Medline]
- Myers JL, Campbell DB, Waldhausen JA. The use of absorbable monofilament polydioxane suture in pediatric cardiovascular surgery. J Thorac Cardiovasc Surg 1986;92:771–5.[Abstract]
- Maron BJ, Humphries JO, Rowe RD, et al. Prognosis of surgically corrected coarctation of the aorta: a 20-year postoperative appraisal. Circulation 1973;47:119–26.[Abstract/Free Full Text]
- Sellors TH. Surgery of pulmonic stenosis. A case in which the pulmonary valve was successfully divided. Lancet 1948;1:988–9.[Medline]
- Brock RC. Pulmonary valvulotomy for the relief of congenital pulmonary stenosis. Guy's Hosp Rep 1950;99:236.
- Gross RE, Pomeranz AA, Watkins E Jr, Goldsmith EI. Surgical closure of defects of the interauricular septum by use of an atrial well. N Engl J Med 1952;247:455–60.[Medline]
- Blalock A, Hanlon CR. The surgical treatment of complete transposition of the aorta and the pulmonary artery. Surg Gynecol Obstet 1950;90:1–15.[Medline]
- Rashkind WJ, Miller WW. Creation of an atrial septal defect without thoracotomy—a palliative approach to complete transposition of the great arteries. JAMA 1966;196:991.[Abstract/Free Full Text]
- Muller WH Jr, Dammann JF Jr. The treatment of certain congenital malformations of the heart by the creation of pulmonic stenosis to reduce pulmonary hypertension and excessive pulmonary blood flow. A preliminary report. Surg Gynecol Obstet 1952;95:213–9.[Medline]
- Case RB, Morrow AG, Stainsley W, Nestor JO. Anomalous origin of the left coronary artery. The physiologic defect and suggested surgical treatment. Circulation 1958;17:1062–8.[Medline]
- Sabiston DC Jr, Neill CA, Taussig HB. The direction of blood flow in anomalous left coronary artery arising from the pulmonary artery. Circulation 1960;22:591–7.[Abstract/Free Full Text]
- Davis C Jr, Dillon RF, Fell EH, Gasul BM. Anomalous coronary simulating patent ductus arteriosus. JAMA 1956;160:1047–50.
