- Email: [email protected]
A Tribute to Giancarlo Rastelli
OUR SURGICAL HERITAGE
A Tribute to Giancarlo Rastelli Igor E. Konstantinov, MD, PhD, Felice Rosapepe, MD, PhD, Joseph A. Dearani, MD, Vladimir V. Alexi-Meskishvili, MD, PhD, and Jia Li, MD, PhD
Giancarlo Rastelli (1933–1970) was a pioneer cardiac surgeon who developed a classification of atrioventricular canal and a novel surgical procedure that revolutionized the management of children with congenital heart disease. Rastelli lived a short, yet fascinating life. His work was ahead of its time and laid the foundation for the treatment of complex congenital cardiac anomalies. (Ann Thorac Surg 2005;79:1819 –23) © 2005 by The Society of Thoracic Surgeons
Biographical Notes
G
iancarlo Rastelli (1933–1970) was born on June 25, 1933 in Pescara, Italy. His father, Vito Rastelli, was a journalist for a local newspaper. His mother, Luisa Bianchi, was an elementary school teacher. Rastelli had a younger sister Rosangela. She now lives in Milan, and some biographical information presented herein is based on her personal account. In 1945, Rastelli’s family moved to Parma, the native town of both his parents. Here Rastelli attended the Classic Lyceum of Romagna and then studied medicine at the University of Parma. Rastelli was a hard-working student during his medical school years, but he had also developed a passion for both classical music and mountain trekking (Fig 1A). He spent much of his spare time either listening to his collection of records or exploring mountain paths. As a medical student Rastelli completed his research projects first at the department of anatomy directed by Professor Gaetano Ottaviani from 1951 to 1953, and then at the department of general pathology directed by Professor Domenico Rezzi from 1953 to 1955. During those years Rastelli developed a strong interest in the anatomy and pathology of the heart. Rastelli graduated cum laude in 1957 and won “Le Petit” prize for the best graduation thesis entitled “Changes in the ATPase activity of the myocardium during deep hypothermia.” In 1957, Rastelli began his training in surgery at the department of surgery headed by Professor Antonio Bobbio at the University of Parma Hospital. At that time, Rastelli’s particular interest was in diagnostic techniques (Fig 1B). Being a compassionate person, Rastelli always tried to explain to his patient the exact underlying cause of the illness, as well as the anatomy, pathology, and treatment options. Yet Rastelli often did not have an answer for his patients, nor did he have a surgical option to offer to his Address reprint requests to Dr Konstantinov, Division of Cardiovascular Surgery, Hospital for Sick Children, 555 University Ave, Toronto M5G 1X8, Ontario, Canada; e-mail: [email protected].
© 2005 by The Society of Thoracic Surgeons Published by Elsevier Inc
patients with congenital cardiac malformations. This quest for answers stimulated Rastelli, together with another surgical trainee, Dr Carlo Battistini, to undertake intense surgical research. Drs Battistini, Bobbio, and Rastelli also shared a passion for classical music. They spent hours together listening to classical music records and discussing a variety of subjects. This friendship also facilitated successful research because they worked very effectively as a team. This productive teamwork helped Rastelli win a North Atlantic Treaty Organization (NATO) scholarship in 1960 that allowed him to continue his studies at the Mayo Clinic. Rastelli’s choice of the Mayo Clinic was inspired by the pioneering work of John W. Kirklin (1917–2004), who truly revolutionized heart surgery in the 1950s using the Mayo-Gibbon heart-lung machine. Doctor Kirklin became professor and chairman of the department of surgery at the Mayo Clinic in 1960, and he held the latter position until 1966 when he became chairman of the department of surgery at the University of Alabama. Inspired by the work of Kirklin, Rastelli (Fig 2A) became a member of the Mayo research team that was performing truly groundbreaking cardiovascular research. After completing his scholarship, Rastelli remained at the Mayo Clinic as a research assistant (1962–1964) and research associate (1964 –1968). During those years, Rastelli worked tirelessly spending most of his time in the clinic, conducting research, and writing scientific papers. Rastelli met his wife to be, Anna Anghileri, in 1959 in the ski camp in Borneo, Italy when she was only 19 years old. While in America, he continued to correspond with Anna almost daily. On August 11, 1964, Rastelli returned to Italy and 1 day later married Anna Anghileri. Shortly thereafter, they traveled to the United States. Rastelli had a happy, loving family, an interesting and productive profession, and the future looked extremely promising. However, a few days after his honeymoon, Rastelli was diagnosed with Hodgkin’s disease. Even today, with modern chemotherapy, the overall 5-year survival rate with all cases of Hodgkin’s disease is only 55% to 60%, although patients with stage I or stage II disease have a 5-year survival of about 80%. Rastelli was certainly aware of his poor prognosis, yet he chose to get the most from the years left to him. In 1965, Rastelli received the Allen Welkind Award for outstanding research in cardiovascular surgery. In 1966, Rastelli’s daughter Antonella Luisa was born. In late 1966, Rastelli became increasingly symptomatic. Despite being fully aware of his bleak future, Rastelli continued 0003-4975/05/$30.00 doi:10.1016/j.athoracsur.2004.11.037
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Division of Cardiovascular Surgery, Hospital for Sick Children, Toronto, Ontario, Canada; Department of Pediatric Cardiac Surgery, Azienda Ospedaliera Monaldi, Napoli, Italy; Division of Cardiovascular Surgery, Mayo Clinic, Rochester, Minnesota; Deutsches Herzzentrum Berlin, Berlin, Germany
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of which Rastelli was a coauthor (Fig 2B). The first was the Billings Gold Medal in 1968 for anatomical classification of the atrioventricular canal that facilitated the development of a new surgical technique [1]. The technique was quickly adopted by his colleagues at the Mayo Clinic (Drs John W. Kirklin and Dwight C. McGoon [1925–1999]). The second medal was the Hektoen Gold Medal for developing an operation using an extracardiac conduit for surgical correction of truncus arteriosus [4]. In 1968, Rastelli was appointed head of cardiovascular research at the Mayo Clinic, but his tenure was to be short-lived. Doctor Rastelli died at the Methodist Hospital in Rochester on February 2, 1970 at the age of 36 years.
The Rastelli Classification
Fig 1. (A) Giancarlo Rastelli during mountain trekking, ca 1955. (B) Dr. Rastelli during his residency in Parma, ca 1958. (Courtesy of Rosangela Rastelli Zavattaro.)
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to work fervently. It is during this period that he developed the classification of atrioventricular (AV) canal [1] and the surgical procedure [2, 3] that bears his name, (ie, “the Rastelli classification” and “the Rastelli operation”). During this time, Rastelli received the Mayo Clinic Staff Memorial Award in 1968 for his outstanding research work, and the American Medical Association awarded the Gold Medal to Rastelli for 2 consecutive years [4]. More precisely, the medals were awarded to two exhibits, Fig 2. (A) Dr Rastelli upon arrival to Mayo Clinic in 1961. (B) Dr Giancarlo Rastelli, Dwight C. McGoon, and Jack L. Titus discussing Rastelli’s operation in front of their exhibit at the American Medical Association meeting in 1969. (Courtesy of Mayo Clinic Foundation.)
In the early 1960s, Rastelli devoted much of his research time to the understanding of the morphology of the common AV canal and published several articles on the subject [5–7]. This allowed him to classify all forms of the complete AV canals into 3 major types. In 1967, Rastelli, Kirklin and Kincaid published an article describing classification of the common AV canal [7]. The classification is based on the morphology of the anterior bridging leaflet, and is comprised of types A, B, and C (Fig 3). In the subsequent year, Rastelli and his colleagues published an article describing their experience with the surgical repair of the complete AV canal in 38 patients operated on at the Mayo Clinic between December 1955 and September 1967. The hospital mortality was 60% before 1964 and this decreased to 20% for those operated on between 1964 and 1967 [1]. The authors concluded that “the recognition of three anatomic types of complete atrioventricular canal has given a better understanding of the problems involved in surgical repair” [1]. Rastelli was very eager to publish a monograph on AV canal defects. Among the several projects that entirely engrossed Rastelli during his last years, this monograph was one of the most important. Acutely aware of the rapid progression of his disease, Rastelli was in a hurry to finish the book, but it was left for the others to finish and publish it [8]. Dwight C. McGoon wrote in the introduction to this monograph: “Prerequisite to the accurate and
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therefore the successful repair of any cardiac deformity is a precise understanding of its anatomic characteristics. Such was not available when the first surgical efforts were made to correct complete atrioventricular canal. Simultaneously with the urgent need to develop a more successful technique was the investigation by Gian Rastelli, under the tutelage of Drs Jack Titus and John Kirklin, of the characteristics of this anomaly and ultimately of its anatomic classification. The result was a strikingly improved outlook for the child born with this affliction, and thus also represents one of Gian’s more significant contributions” [8]. Much progress has been made since the first successful repairs of AV canal reported by Rastelli and colleagues. In the most recent study of 209 children operated on for complete AV canal at the Hospital for Sick Children in Toronto between 1995 and 2002, operative mortality was only 2.9% [9]. However, it should be remembered that the modern advances in AV canal repair would not be possible without the early work of Rastelli and his accurate classification.
The Rastelli Operation In 1967, Rastelli and colleagues described an experimental approach to the repair of truncus arteriosus by means of aortic homograft used as a right ventricular outflow [3]. The technique evolved into an operation applicable to a great variety of cardiac anomalies, including those children born with transposition of the great arteries (TGA), ventricular septal defect (VSD), and pulmonary stenosis
[2]. The Rastelli operation was the first procedure described for TGA that incorporated the left ventricle into the systemic circulation (Fig 4). This procedure was first performed by Dr Robert B. Wallace (1931–) at the Mayo Clinic on July 26, 1968 [10]. The operation opened possibilities for total correction of many congenital anomalies, including persistent truncus arteriosus and pulmonary atresia with VSD. In 1969, the world’s first successful repair of truncus arteriosus was performed at the Mayo Clinic using the technique described by Rastelli [11]. Today the term “Rastelli operation” is often applied loosely to describe any operation in which the VSD is closed in a way that leaves the aorta coming from the morphologic LV, whereas the morphologic right ventricle is connected to the pulmonary artery by means of extracardiac conduit. The Rastelli operation is indicated in patients with truncus arteriosus, VSD with pulmonary atresia, double outlet right ventricle, TGA with VSD and subpulmonary obstruction, and in congenitally corrected TGA with pulmonary stenosis [12, 13]. In a study of 160 patients with TGA who underwent the Rastelli operation between 1968 and 1990 at the Mayo Clinic, and who were followed for at least 10 years, there was an actuarial survival of 74% at 10 years and 59% at 20 years [14]. This was despite an early mortality of 24% for those operated on between 1968 and 1977, which decreased to 4% for the patients operated on from 1988 to 1997. The outcome in the early patients was complicated by pulmonary hypertension. A 10-year survival of 101 patients with TGA who underwent the Rastelli operation at Boston Children’s Hospital from 1973 to 1998 was 70%
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Fig 3. Rastelli classification of complete common atrioventricular canal. (A) Type A: Anterior (A) common atrioventricular leaflet is divided into two portions, one mitral valve (MV) and one tricuspid valve (TV), attached medially to interventricular septum with long, nonfused chordae tendineae. In posterior common AV leaflet, MV and TV portions are not separated. (L ⫽ lateral; P ⫽ posterior; RA ⫽ right atrium.) (B) Type B: Anterior common AV leaflet is divided but not attached to the septum. Mitral and tricuspid components are both attached medially to abnormal papillary muscle arising in right ventricle near septum. Free interventricular communication occurs under anterior common leaflet. (RV ⫽ right ventricle.) (C) Type C: Anterior common AV leaflet is not divided and is not attached to the septum so that free interventricular communication, extending to vicinity of aortic cusps, occurs underneath this leaflet. (Reprinted from Rastelli GC, et al. Mayo Clin Proc 1967;42:200 –9 [7], with permission.)
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Fig 4. (A–E) Rastelli operation. (A ⫽ aorta; Ant. ⫽ anterior; IVC ⫽ inferior vena cava; PA ⫽ pulmonary artery; RA ⫽ right atrium; RV ⫽ right ventricle; SVC ⫽ superior vena cava; TV ⫽ tricuspid valve; VSD ⫽ ventricular septal defect.) (Reprinted from Rastelli GC, et al. Circulation 1969;39:83–95 [10], with permission.)
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[15]. Although the Rastelli operation has made an enormous impact, it is not without its flaws. Most of the children who underwent the Rastelli operation will still require a conduit replacement at least once in their lives [14 –16]. A similar evolution of improvement has occurred for this surgery. In a review of 1,095 patients who underwent a ventricle to pulmonary artery conduit at the Mayo Clinic, an early mortality for conduit replacement in 306 patients operated between 1964 and 2001 was 4.9%, which has decreased to 1.7% since 1989 [16].
Epilogue During his last years, Rastelli worked long hours despite the severity of his illness. In response to his sister’s pledge to slow down and take it easy for a while, Rastelli once said: “To stop the research is to cease to live.” He also continued to support the patients who came to the Mayo Clinic from Italy for surgical repair. During his last years, Rastelli always saw Italian patients before and after their surgery to
comfort them in their native language. He also kept a big poster in his office that said, “Love always wins” (Fig 5A) signed by all his Italian patients. One of Rastelli’s Italian patients was Vincenzo Ferrante. Ferrante was a 12-year old boy with a diagnosis of TGA, VSD, and pulmonary stenosis who was first operated on in Houston in 1966, when he underwent a Blalock-Hanlon operation. Ferrante went to Houston in 1969 for a follow-up in hopes of getting a complete repair, yet he was considered to be inoperable. In despair, Ferrante’s parents contacted Rastelli. Ferrante was brought to the Mayo Clinic and underwent the Rastelli operation on November 11, 1969. The postoperative course was complicated and Ferrante was reoperated on in a week for recurrent VSD. Ferrante had to spend the Christmas season at the hospital. Although at the time Dr Rastelli felt very sick, he visited Ferrante daily to keep his spirits high (Fig 5B), and once he spent the whole night at Ferrante’s bedside. Rastelli died less than 1 month after Ferrante’s discharge. Now Ferrante is a university grad-
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uate who is married and currently works as a civil engineer in Napoli, Italy. For him, Rastelli’s research made the difference. It also made the difference for many other Italian children who signed the “Love always wins” poster in Dr. Rastelli’s office. Doctor Rastelli’s daughter Antonella Luisa was only 4 when her father died. She followed her father footsteps, graduated from medical school in Verona, Italy, completed her residency in internal medicine and became a staff physician at Washington University in St. Louis, Missouri. Doctor Rastelli’s life was short, yet he accomplished the work of many life times in only one. What he had planned for the future, we can only imagine. Disease was not able to break his spirit but only fueled his creativity instead. His legacy lives on in the children helped by his innovations and in the continued application of the operation that carries his name.
We are grateful to Rosangela Rastelli Zavattaro, a sister of Dr Giancarlo Rastelli, for providing us with unique photographs and detailed biographical information and to Drs Andrew N. Redington, Richard Van Praagh, and Jack L. Titus for their most valuable suggestions.
References 1. Rastelli GC, Ongley PA, Kirklin JW, McGoon DC. Surgical repair of the complete form of persistent common atrioventricular canal. J Thorac Cardiovasc Surg 1968;55:299 –308. 2. Rastelli GC. A new approach to “anatomic” repair of transposition of the great arteries. Mayo Clinic Proc 1969;44:1–12. 3. Rastelli GC, Titus JL, McGoon DC. Homograft of ascending aorta and aortic valve as a right ventricular outflow. An experimental approach to the repair of truncus arteriosus. Arch Surg 1967;95:698 –708. 4. Squarcia U, Squarcia A. Giancarlo Rastelli, MD. Mayo Clin Proc 2001;76:874.
5. Rastelli GC, Kirklin JW, Titus JL. Anatomic observations on complete form of persistent common atrioventricular canal with special reference to atrioventricular valves. Mayo Clin Proc 1966;41:296 –308. 6. Rastelli GC, Weidman WH, Kirklin JW. Surgical repair of the partial form of persistent common atrioventricular canal with special reference to the problem of mitral valve incompetence. Circulation 1965;31(Suppl 1):31–5. 7. Rastelli GC, Kirklin JW, Kincaid OW. Angiocardiography in persistent common atrioventricular canal. Mayo Clin Proc 1967;42:200 –9. 8. Feldt RH, ed. Atrioventricular canal defects. Philadelphia: Saunders, 1976. 9. Fortuna RS, Ashburn DA, Carias De Oliveira N, et al. Atrioventricular septal defects: effect of bridging leaflet division on early valve function. Ann Thorac Surg 2004;77:895–902. 10. Rastelli GC, Wallace RB, Ongley PA. Complete repair of transposition of the great arteries with pulmonary stenosis: a review and report of a case corrected by using a new surgical technique. Circulation 1969;39:83–95. 11. Wallace RB, Rastelli GC, Ongley PA, Titus JL, McGoon DC. Complete repair of truncus arteriosus defects. J Thorac Cardiovasc Surg 1969;57:95–107. 12. Konstantinov IE, Williams WG. Atrial switch and Rastelli operation for congenitally corrected transposition with ventricular septal defect and pulmonary stenosis. Thorac Cardiovasc Surg 2003;8:160 – 6. 13. Ilbawi MN, DeLeon SY, Backer CL, et al. An alternative approach to the surgical management of physiologically corrected transposition with pulmonary stenosis or atresia. J Thorac Cardiovasc Surg 1990;100:410 –5. 14. Dearani JA, Danielson GK, Puga FJ, et al. Late results of the Rastelli operation for transposition of the great arteries. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2001;4:3–15. 15. Kreutzer C, DeVive J, Oppido G, et al. Twenty-five year experience with Rastelli repair for transposition of the great arteries. J Thorac Cardiovasc Surg 2000;120:211–23. 16. Dearani JA, Danielson GK, Puga FJ, et al. Late follow-up of 1,095 patients undergoing operation for complex congenital heart disease utilizing pulmonary ventricle to pulmonary artery conduits. Ann Thorac Surg 2003;75:399 – 411.
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Fig 5. (A) A poster signed by all Italian children operated on at the Mayo Clinic in the late 1960s that Dr Rastelli kept in his office. The Italian inscription on the poster says, “Love always wins.” (B) Vincenzo Ferrante a few days after the Rastelli operation with Dr Rastelli at the Mayo Clinic in 1969. (Courtesy of Rosangela Rastelli Zavattaro with permission of Vincenzo Ferrante.)
A Tribute to Giancarlo Rastelli Igor E. Konstantinov, MD, PhD, Felice Rosapepe, MD, PhD, Joseph A. Dearani, MD, Vladimir V. Alexi-Meskishvili, MD, PhD, and Jia Li, MD, PhD
Giancarlo Rastelli (1933–1970) was a pioneer cardiac surgeon who developed a classification of atrioventricular canal and a novel surgical procedure that revolutionized the management of children with congenital heart disease. Rastelli lived a short, yet fascinating life. His work was ahead of its time and laid the foundation for the treatment of complex congenital cardiac anomalies. (Ann Thorac Surg 2005;79:1819 –23) © 2005 by The Society of Thoracic Surgeons
Biographical Notes
G
iancarlo Rastelli (1933–1970) was born on June 25, 1933 in Pescara, Italy. His father, Vito Rastelli, was a journalist for a local newspaper. His mother, Luisa Bianchi, was an elementary school teacher. Rastelli had a younger sister Rosangela. She now lives in Milan, and some biographical information presented herein is based on her personal account. In 1945, Rastelli’s family moved to Parma, the native town of both his parents. Here Rastelli attended the Classic Lyceum of Romagna and then studied medicine at the University of Parma. Rastelli was a hard-working student during his medical school years, but he had also developed a passion for both classical music and mountain trekking (Fig 1A). He spent much of his spare time either listening to his collection of records or exploring mountain paths. As a medical student Rastelli completed his research projects first at the department of anatomy directed by Professor Gaetano Ottaviani from 1951 to 1953, and then at the department of general pathology directed by Professor Domenico Rezzi from 1953 to 1955. During those years Rastelli developed a strong interest in the anatomy and pathology of the heart. Rastelli graduated cum laude in 1957 and won “Le Petit” prize for the best graduation thesis entitled “Changes in the ATPase activity of the myocardium during deep hypothermia.” In 1957, Rastelli began his training in surgery at the department of surgery headed by Professor Antonio Bobbio at the University of Parma Hospital. At that time, Rastelli’s particular interest was in diagnostic techniques (Fig 1B). Being a compassionate person, Rastelli always tried to explain to his patient the exact underlying cause of the illness, as well as the anatomy, pathology, and treatment options. Yet Rastelli often did not have an answer for his patients, nor did he have a surgical option to offer to his Address reprint requests to Dr Konstantinov, Division of Cardiovascular Surgery, Hospital for Sick Children, 555 University Ave, Toronto M5G 1X8, Ontario, Canada; e-mail: [email protected].
© 2005 by The Society of Thoracic Surgeons Published by Elsevier Inc
patients with congenital cardiac malformations. This quest for answers stimulated Rastelli, together with another surgical trainee, Dr Carlo Battistini, to undertake intense surgical research. Drs Battistini, Bobbio, and Rastelli also shared a passion for classical music. They spent hours together listening to classical music records and discussing a variety of subjects. This friendship also facilitated successful research because they worked very effectively as a team. This productive teamwork helped Rastelli win a North Atlantic Treaty Organization (NATO) scholarship in 1960 that allowed him to continue his studies at the Mayo Clinic. Rastelli’s choice of the Mayo Clinic was inspired by the pioneering work of John W. Kirklin (1917–2004), who truly revolutionized heart surgery in the 1950s using the Mayo-Gibbon heart-lung machine. Doctor Kirklin became professor and chairman of the department of surgery at the Mayo Clinic in 1960, and he held the latter position until 1966 when he became chairman of the department of surgery at the University of Alabama. Inspired by the work of Kirklin, Rastelli (Fig 2A) became a member of the Mayo research team that was performing truly groundbreaking cardiovascular research. After completing his scholarship, Rastelli remained at the Mayo Clinic as a research assistant (1962–1964) and research associate (1964 –1968). During those years, Rastelli worked tirelessly spending most of his time in the clinic, conducting research, and writing scientific papers. Rastelli met his wife to be, Anna Anghileri, in 1959 in the ski camp in Borneo, Italy when she was only 19 years old. While in America, he continued to correspond with Anna almost daily. On August 11, 1964, Rastelli returned to Italy and 1 day later married Anna Anghileri. Shortly thereafter, they traveled to the United States. Rastelli had a happy, loving family, an interesting and productive profession, and the future looked extremely promising. However, a few days after his honeymoon, Rastelli was diagnosed with Hodgkin’s disease. Even today, with modern chemotherapy, the overall 5-year survival rate with all cases of Hodgkin’s disease is only 55% to 60%, although patients with stage I or stage II disease have a 5-year survival of about 80%. Rastelli was certainly aware of his poor prognosis, yet he chose to get the most from the years left to him. In 1965, Rastelli received the Allen Welkind Award for outstanding research in cardiovascular surgery. In 1966, Rastelli’s daughter Antonella Luisa was born. In late 1966, Rastelli became increasingly symptomatic. Despite being fully aware of his bleak future, Rastelli continued 0003-4975/05/$30.00 doi:10.1016/j.athoracsur.2004.11.037
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Division of Cardiovascular Surgery, Hospital for Sick Children, Toronto, Ontario, Canada; Department of Pediatric Cardiac Surgery, Azienda Ospedaliera Monaldi, Napoli, Italy; Division of Cardiovascular Surgery, Mayo Clinic, Rochester, Minnesota; Deutsches Herzzentrum Berlin, Berlin, Germany
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of which Rastelli was a coauthor (Fig 2B). The first was the Billings Gold Medal in 1968 for anatomical classification of the atrioventricular canal that facilitated the development of a new surgical technique [1]. The technique was quickly adopted by his colleagues at the Mayo Clinic (Drs John W. Kirklin and Dwight C. McGoon [1925–1999]). The second medal was the Hektoen Gold Medal for developing an operation using an extracardiac conduit for surgical correction of truncus arteriosus [4]. In 1968, Rastelli was appointed head of cardiovascular research at the Mayo Clinic, but his tenure was to be short-lived. Doctor Rastelli died at the Methodist Hospital in Rochester on February 2, 1970 at the age of 36 years.
The Rastelli Classification
Fig 1. (A) Giancarlo Rastelli during mountain trekking, ca 1955. (B) Dr. Rastelli during his residency in Parma, ca 1958. (Courtesy of Rosangela Rastelli Zavattaro.)
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to work fervently. It is during this period that he developed the classification of atrioventricular (AV) canal [1] and the surgical procedure [2, 3] that bears his name, (ie, “the Rastelli classification” and “the Rastelli operation”). During this time, Rastelli received the Mayo Clinic Staff Memorial Award in 1968 for his outstanding research work, and the American Medical Association awarded the Gold Medal to Rastelli for 2 consecutive years [4]. More precisely, the medals were awarded to two exhibits, Fig 2. (A) Dr Rastelli upon arrival to Mayo Clinic in 1961. (B) Dr Giancarlo Rastelli, Dwight C. McGoon, and Jack L. Titus discussing Rastelli’s operation in front of their exhibit at the American Medical Association meeting in 1969. (Courtesy of Mayo Clinic Foundation.)
In the early 1960s, Rastelli devoted much of his research time to the understanding of the morphology of the common AV canal and published several articles on the subject [5–7]. This allowed him to classify all forms of the complete AV canals into 3 major types. In 1967, Rastelli, Kirklin and Kincaid published an article describing classification of the common AV canal [7]. The classification is based on the morphology of the anterior bridging leaflet, and is comprised of types A, B, and C (Fig 3). In the subsequent year, Rastelli and his colleagues published an article describing their experience with the surgical repair of the complete AV canal in 38 patients operated on at the Mayo Clinic between December 1955 and September 1967. The hospital mortality was 60% before 1964 and this decreased to 20% for those operated on between 1964 and 1967 [1]. The authors concluded that “the recognition of three anatomic types of complete atrioventricular canal has given a better understanding of the problems involved in surgical repair” [1]. Rastelli was very eager to publish a monograph on AV canal defects. Among the several projects that entirely engrossed Rastelli during his last years, this monograph was one of the most important. Acutely aware of the rapid progression of his disease, Rastelli was in a hurry to finish the book, but it was left for the others to finish and publish it [8]. Dwight C. McGoon wrote in the introduction to this monograph: “Prerequisite to the accurate and
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therefore the successful repair of any cardiac deformity is a precise understanding of its anatomic characteristics. Such was not available when the first surgical efforts were made to correct complete atrioventricular canal. Simultaneously with the urgent need to develop a more successful technique was the investigation by Gian Rastelli, under the tutelage of Drs Jack Titus and John Kirklin, of the characteristics of this anomaly and ultimately of its anatomic classification. The result was a strikingly improved outlook for the child born with this affliction, and thus also represents one of Gian’s more significant contributions” [8]. Much progress has been made since the first successful repairs of AV canal reported by Rastelli and colleagues. In the most recent study of 209 children operated on for complete AV canal at the Hospital for Sick Children in Toronto between 1995 and 2002, operative mortality was only 2.9% [9]. However, it should be remembered that the modern advances in AV canal repair would not be possible without the early work of Rastelli and his accurate classification.
The Rastelli Operation In 1967, Rastelli and colleagues described an experimental approach to the repair of truncus arteriosus by means of aortic homograft used as a right ventricular outflow [3]. The technique evolved into an operation applicable to a great variety of cardiac anomalies, including those children born with transposition of the great arteries (TGA), ventricular septal defect (VSD), and pulmonary stenosis
[2]. The Rastelli operation was the first procedure described for TGA that incorporated the left ventricle into the systemic circulation (Fig 4). This procedure was first performed by Dr Robert B. Wallace (1931–) at the Mayo Clinic on July 26, 1968 [10]. The operation opened possibilities for total correction of many congenital anomalies, including persistent truncus arteriosus and pulmonary atresia with VSD. In 1969, the world’s first successful repair of truncus arteriosus was performed at the Mayo Clinic using the technique described by Rastelli [11]. Today the term “Rastelli operation” is often applied loosely to describe any operation in which the VSD is closed in a way that leaves the aorta coming from the morphologic LV, whereas the morphologic right ventricle is connected to the pulmonary artery by means of extracardiac conduit. The Rastelli operation is indicated in patients with truncus arteriosus, VSD with pulmonary atresia, double outlet right ventricle, TGA with VSD and subpulmonary obstruction, and in congenitally corrected TGA with pulmonary stenosis [12, 13]. In a study of 160 patients with TGA who underwent the Rastelli operation between 1968 and 1990 at the Mayo Clinic, and who were followed for at least 10 years, there was an actuarial survival of 74% at 10 years and 59% at 20 years [14]. This was despite an early mortality of 24% for those operated on between 1968 and 1977, which decreased to 4% for the patients operated on from 1988 to 1997. The outcome in the early patients was complicated by pulmonary hypertension. A 10-year survival of 101 patients with TGA who underwent the Rastelli operation at Boston Children’s Hospital from 1973 to 1998 was 70%
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Fig 3. Rastelli classification of complete common atrioventricular canal. (A) Type A: Anterior (A) common atrioventricular leaflet is divided into two portions, one mitral valve (MV) and one tricuspid valve (TV), attached medially to interventricular septum with long, nonfused chordae tendineae. In posterior common AV leaflet, MV and TV portions are not separated. (L ⫽ lateral; P ⫽ posterior; RA ⫽ right atrium.) (B) Type B: Anterior common AV leaflet is divided but not attached to the septum. Mitral and tricuspid components are both attached medially to abnormal papillary muscle arising in right ventricle near septum. Free interventricular communication occurs under anterior common leaflet. (RV ⫽ right ventricle.) (C) Type C: Anterior common AV leaflet is not divided and is not attached to the septum so that free interventricular communication, extending to vicinity of aortic cusps, occurs underneath this leaflet. (Reprinted from Rastelli GC, et al. Mayo Clin Proc 1967;42:200 –9 [7], with permission.)
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Fig 4. (A–E) Rastelli operation. (A ⫽ aorta; Ant. ⫽ anterior; IVC ⫽ inferior vena cava; PA ⫽ pulmonary artery; RA ⫽ right atrium; RV ⫽ right ventricle; SVC ⫽ superior vena cava; TV ⫽ tricuspid valve; VSD ⫽ ventricular septal defect.) (Reprinted from Rastelli GC, et al. Circulation 1969;39:83–95 [10], with permission.)
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[15]. Although the Rastelli operation has made an enormous impact, it is not without its flaws. Most of the children who underwent the Rastelli operation will still require a conduit replacement at least once in their lives [14 –16]. A similar evolution of improvement has occurred for this surgery. In a review of 1,095 patients who underwent a ventricle to pulmonary artery conduit at the Mayo Clinic, an early mortality for conduit replacement in 306 patients operated between 1964 and 2001 was 4.9%, which has decreased to 1.7% since 1989 [16].
Epilogue During his last years, Rastelli worked long hours despite the severity of his illness. In response to his sister’s pledge to slow down and take it easy for a while, Rastelli once said: “To stop the research is to cease to live.” He also continued to support the patients who came to the Mayo Clinic from Italy for surgical repair. During his last years, Rastelli always saw Italian patients before and after their surgery to
comfort them in their native language. He also kept a big poster in his office that said, “Love always wins” (Fig 5A) signed by all his Italian patients. One of Rastelli’s Italian patients was Vincenzo Ferrante. Ferrante was a 12-year old boy with a diagnosis of TGA, VSD, and pulmonary stenosis who was first operated on in Houston in 1966, when he underwent a Blalock-Hanlon operation. Ferrante went to Houston in 1969 for a follow-up in hopes of getting a complete repair, yet he was considered to be inoperable. In despair, Ferrante’s parents contacted Rastelli. Ferrante was brought to the Mayo Clinic and underwent the Rastelli operation on November 11, 1969. The postoperative course was complicated and Ferrante was reoperated on in a week for recurrent VSD. Ferrante had to spend the Christmas season at the hospital. Although at the time Dr Rastelli felt very sick, he visited Ferrante daily to keep his spirits high (Fig 5B), and once he spent the whole night at Ferrante’s bedside. Rastelli died less than 1 month after Ferrante’s discharge. Now Ferrante is a university grad-
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uate who is married and currently works as a civil engineer in Napoli, Italy. For him, Rastelli’s research made the difference. It also made the difference for many other Italian children who signed the “Love always wins” poster in Dr. Rastelli’s office. Doctor Rastelli’s daughter Antonella Luisa was only 4 when her father died. She followed her father footsteps, graduated from medical school in Verona, Italy, completed her residency in internal medicine and became a staff physician at Washington University in St. Louis, Missouri. Doctor Rastelli’s life was short, yet he accomplished the work of many life times in only one. What he had planned for the future, we can only imagine. Disease was not able to break his spirit but only fueled his creativity instead. His legacy lives on in the children helped by his innovations and in the continued application of the operation that carries his name.
We are grateful to Rosangela Rastelli Zavattaro, a sister of Dr Giancarlo Rastelli, for providing us with unique photographs and detailed biographical information and to Drs Andrew N. Redington, Richard Van Praagh, and Jack L. Titus for their most valuable suggestions.
References 1. Rastelli GC, Ongley PA, Kirklin JW, McGoon DC. Surgical repair of the complete form of persistent common atrioventricular canal. J Thorac Cardiovasc Surg 1968;55:299 –308. 2. Rastelli GC. A new approach to “anatomic” repair of transposition of the great arteries. Mayo Clinic Proc 1969;44:1–12. 3. Rastelli GC, Titus JL, McGoon DC. Homograft of ascending aorta and aortic valve as a right ventricular outflow. An experimental approach to the repair of truncus arteriosus. Arch Surg 1967;95:698 –708. 4. Squarcia U, Squarcia A. Giancarlo Rastelli, MD. Mayo Clin Proc 2001;76:874.
5. Rastelli GC, Kirklin JW, Titus JL. Anatomic observations on complete form of persistent common atrioventricular canal with special reference to atrioventricular valves. Mayo Clin Proc 1966;41:296 –308. 6. Rastelli GC, Weidman WH, Kirklin JW. Surgical repair of the partial form of persistent common atrioventricular canal with special reference to the problem of mitral valve incompetence. Circulation 1965;31(Suppl 1):31–5. 7. Rastelli GC, Kirklin JW, Kincaid OW. Angiocardiography in persistent common atrioventricular canal. Mayo Clin Proc 1967;42:200 –9. 8. Feldt RH, ed. Atrioventricular canal defects. Philadelphia: Saunders, 1976. 9. Fortuna RS, Ashburn DA, Carias De Oliveira N, et al. Atrioventricular septal defects: effect of bridging leaflet division on early valve function. Ann Thorac Surg 2004;77:895–902. 10. Rastelli GC, Wallace RB, Ongley PA. Complete repair of transposition of the great arteries with pulmonary stenosis: a review and report of a case corrected by using a new surgical technique. Circulation 1969;39:83–95. 11. Wallace RB, Rastelli GC, Ongley PA, Titus JL, McGoon DC. Complete repair of truncus arteriosus defects. J Thorac Cardiovasc Surg 1969;57:95–107. 12. Konstantinov IE, Williams WG. Atrial switch and Rastelli operation for congenitally corrected transposition with ventricular septal defect and pulmonary stenosis. Thorac Cardiovasc Surg 2003;8:160 – 6. 13. Ilbawi MN, DeLeon SY, Backer CL, et al. An alternative approach to the surgical management of physiologically corrected transposition with pulmonary stenosis or atresia. J Thorac Cardiovasc Surg 1990;100:410 –5. 14. Dearani JA, Danielson GK, Puga FJ, et al. Late results of the Rastelli operation for transposition of the great arteries. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2001;4:3–15. 15. Kreutzer C, DeVive J, Oppido G, et al. Twenty-five year experience with Rastelli repair for transposition of the great arteries. J Thorac Cardiovasc Surg 2000;120:211–23. 16. Dearani JA, Danielson GK, Puga FJ, et al. Late follow-up of 1,095 patients undergoing operation for complex congenital heart disease utilizing pulmonary ventricle to pulmonary artery conduits. Ann Thorac Surg 2003;75:399 – 411.
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Fig 5. (A) A poster signed by all Italian children operated on at the Mayo Clinic in the late 1960s that Dr Rastelli kept in his office. The Italian inscription on the poster says, “Love always wins.” (B) Vincenzo Ferrante a few days after the Rastelli operation with Dr Rastelli at the Mayo Clinic in 1969. (Courtesy of Rosangela Rastelli Zavattaro with permission of Vincenzo Ferrante.)