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Abstract

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  2. Abstract
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Pediatric Cardiology as a discipline has been proposed to have been born on August 26, 1938, when Robert Gross at the age of 33 years, successfully ligated a patent ductus arteriosus of a 7 years girl at the Children's Hospital in Boston. In November 1944, Helen Taussig convinced Alfred Blalock to anastomose the left subclavian artery to the left pulmonary artery after Robert Gross had declined to cooperate with her. About the 1950s, at the University of Minneapolis, Clarence Walton Lillehei worked on a controlled “crossed circulation” in which the cardiopulmonary bypass machine was another human, generally one of the patient's parents. In 1966 Williams Rashkind introduced ballon septostomy as a palliative approach to complete transposition of the Great Arteries, followed later by Jean Kan's balloon valvuloplasty to open the pulmonary valve. During the 1960s Giancarlo Rastelli developed a new classification of the Atrio Ventricular Canal defect which allowed to have a strikingly better surgical results. Today, even the hypoplastic left heart syndrome (HLHS), at one time a fatal condition, is operable. The completion of the Human Genome Project has been an enormous help in the understanding the genetic causes of cardiac anomalies. However, there are very few approved application for stem cells, and stem cells will not likely replace organ transplantation any time soon. Recently, the protein survivin has been described as a novel player in cardioprotection against myocardial ischemia/reperfusion injury. The science needs to be made with love to warrant the humanity of Research.

The authors have no funding, financial relationships, or conflicts of interest to disclose.

Pediatric cardiology as a discipline was born on August 26, 1938, when at the age of 33 years, Robert Gross successfully ligated a patent ductus arteriosus of a 7-year-old female at the Children's Hospital in Boston, Massachusetts.1,2 Few years later, in November 1944, Helen Taussig convinced Alfred Blalock to anastomose the left subclavian artery to the left pulmonary artery after Robert Gross had declined to collaborate with her.3 The first Blalock and Taussig shunt was put in place to save the life of a severely cyanotic child affected by tetralogy of Fallot (TOF), the first of thousands children with TOF and/or other cyanotic cardiac defects saved by this operation.3 In 1945, Clarence Crayfoord and Gustave Nylin in Stockolm, Sweden, successfully performed a correction of a coarctation of the aorta, with the goal of ending anastomosis.4 In 1948, Sir Russell Brock achieved relief of pulmonary stenosis by the transventricular approach.5 All of these surgical procedures were performed on a beating heart while cardiopulmonary bypass was needed to repair intracardiac lesions.1

In the early 1950s, the surgical efforts culminated with “get inside the heart.”1,6 At the University of Minneapolis, Clarence Walton Lillehei worked on controlled “crossed circulation,” in which the cardiopulmonary bypass machine was another human, usually 1 of the patient's parents.7 The parent's femoral vessels were cannulated, and his or her oxygenated blood was pumped into the patient undergoing cardiac repair.7 Beginning in March 1954, a total of 45 such operations were done over some few months; most of these patients were <2 years old.7,8 Although cross-circulation was not adopted for widespread use because it posed a serious risk to the donor, this procedure paved the way for the open heart surgery era.8

At the same time, a number of teams were working on developing a machine for cardiopulmonary bypass. In Philadelphia, Pennsylvania, John Gibbon had been working on such a device since 1937, in cooperation with Thomas John Watson, chairman of International Business Machines.1,9 On May 6, 1953, Gibbon successfully closed an atrial septal defect in an 18-year-old female, the first successful procedure before John Webster Kirklin's work.9 Kirklin and his team at the Mayo Clinic, starting from the Gibbon–IBM pump-oxygenator, made refinements and modifications to obtain a screen-type machine for supporting circulation.10 The first procedure was performed on March 22, 1955, and in May 1955 the Mayo Clinic Proceedings reported a series of 8 cases operated on with cardiopulmonary bypass using a mechanical pump oxygenator.10

Those remarkable advances in the surgical treatment of congenital heart disease (CHD) were accompanied by similar remarkable advances in accurate diagnosis and in understanding the pathophysiology of CHD. In 1929, William Forsmann passed a catheter from his own arm vein into his right atrium.11 In 1966, William Rashkind introduced balloon septostomy as a palliative approach to complete transposition of the great arteries, followed later by Jean Kan's balloon valvuloplasty to open the pulmonary valve.1,12–14

Cardiac catheterization laboratories developed in many centers, including the Mayo Clinic under Earl Howard Wood.15 Among the major achievements obtained in those years at the Mayo Clinic are the results of the anatomic and experimental investigation done by Giancarlo Rastelli.16 He was a young surgeon who graduated from Parma University and landed at the Mayo Clinic in the early 1960s with a passion for research.16,17 One of his sayings before leaving for the United States, which was also found in his letters, was “to stop the research is to stop the life itself.”

Giancarlo Rastelli worked with John Kirklin and Jack Titus.16 He developed a new classification of the atrioventricular canal defect, which contributed to a better understanding of the anatomical characteristic of the anomaly and had strikingly better surgical results.16,17 Working in the experimental surgical laboratories, Giancarlo Rastelli demonstrated the possibility of an anatomically based correction of the transposition of great arteries vessels with a ventricular septal defect and pulmonary stenosis.16,17 His proposed surgical technique became the Rastelli operation, a concept that still holds true after 40 years. Rastelli became ill with a fever from Hodgkin disease and died at the age of only 36 years.16

Successful anatomic repair of the transposition of the great arteries (TGA) was accomplished by Adib Jatene in San Paulo, Brazil, in 1976.1,18 He performed anatomic switching of the great vessels with the associated coronary arteries. TGA, previously a highly lethal condition, thus became treatable in the newborn with a high degree of success.1,18 Coceani and Olley in 1973 reported that prostaglandin E1 in fetal lambs would dilate the ductus.19 This discovery had a significant influence on the treatment of heart disease in the neonate, allowing needed surgery to be carried out on a stable rather than a critical patient.19 Since the 1980s, cardiac ultrasound, color flow Doppler, and magnetic resonance imaging have made diagnostic cardiac catheterizations almost unnecessary, and instead, interventional cardiac catheterizations rapidly developed to the point that many cardiac defects can now be treated by this means.20–22 Today we can say that all CHD, even the most complex, can be treated successfully. Even hypoplastic left heart syndrome, once a fatal condition, became operable.23,24

Today, a team of geneticists, molecular biologists, and other basic scientists are working to solve the real challenging problem of the prevention of CHD. The completion of the Human Genome Project has been an enormous help in understanding the genetic causes of cardiac anomalies, and we now know that genes play a bigger role than previously suspected.25 The use of myocardial stem cells in patients with heart failure or cardiomyopathy will hopefully replace the need for cardiac transplantation.26 However, there are very few approved applications for stem cells, and stem cells will not likely replace organ transplantation any time soon.27 Bioengineered valves will be developed to replace the mechanical and biological valves.28 Pharmacogenomics will consent to tailor drug treatment depending on the patient's genotype.1 Recently, it has been demonstrated that protein survivin represent a novel player in cardio-protection against myocardial ischemia/reperfusion injury.29 Fetal intervention will become more available and more successful.

In front of all those milestones that have been set up during the last decade, the epidemiology of CHD has changed because of the relevant number of termination of pregnancies (ToP) following the fetal diagnosis of CHD.1,30 The ethical positions are well known and have been expressed in detail in many instances. Even those who are counseling for ToP believe that it is an act of compassion toward the family, who can look at future pregnancies for normal, “not perfect” babies. The other side believes that quality of life cannot be measured and weighed.1,30,31 In this respect, much more than the philosophical concepts or the ethical dogma, are the cultural attitude or the fashion of the times that dictate behavior. Another saying of the young surgeon from the University of Parma, Giancarlo Rastelli, was “To know without knowing to love is nothing, is less than nothing.”16 Our duty as scientists and researchers is not to surrender to the already known. We must dare for the future, but even science needs to be made with love because only love assures and warrants the humanity of research.

References

  1. Top of page
  2. Abstract
  3. References