Pediatric Annals

EDITORIAL 

Further Knowledge in Pediatric Cardiology

Milton I Levine, MD

Abstract

This is the second issue of Pediatric Annals presented as a tribute to the late Dr. Helen Brooke Taussig, recognized as the founder of pediatrie cardiology. It was in 1930 that Dr. Edward A. Park, Professor of Pediatrics at Johns Hopkins Medical College, organized special clinics in his department and appointed Dr. Taussig as director of the cardiac clinic.

I came from what may be termed the pre-Taussig days when our primary cardiac concerns, as practicing pediatricians, were the consequences of rheumatic fever. We were trained to be good physical diagnosticians; we had roentgenograms, fluoroscopes, and electrocardiograms, but no cardiac surgery.

Dr. Taussig soon became an important figure in pediatric cardiology. It is interesting to note that, in the leading pediatrie textbook, the 1940 edition of Holt's Diseases of Infancy and Childhood, the section on the heart was written by Dr. Taussig. I have just reread this whole chapter and noted with interest her suggestion that the cardiac defects found in children with "mongolian idiocy" might indicate an arrest in fetal development at a specific time. This was, of course, not correct. We had no knowledge of the chromosomal abnormality then. But it did show her intense effort, which continued throughout her life, to find some basic cause for congenital cardiac defects. She didn't say so, but I wonder if when she went to Europe to study the tragic thalidomide babies she wasn't seeking a possibility of cardiac defects in such children.

If I am not mistaken, corrective surgery on the heart was first instituted by Gross and Hubbard who, in 1939, described the ligation of the patent ductus. Then in 1944, Dr. Taussig discussed her operative proposal with Dr. Blalock, resulting in the development of the Blalock-Taussig operation which immediately "turned blue babies pink." At the time of this history-making operation, Dr. Mary Alien Engle, the Guest Editor of this symposium, was a substitute intern on the staff of Dr. Blalock. The years that followed produced tremendous advances in knowledge, diagnosis, therapy, and surgery in pediatric cardiology.

Six years ago (1981) we last reviewed in Pediatrie Armais the subject of pediatrie cardiology. This review was also under the Guest Editorship of Dr. Engle. The newer approaches discussed at that time included echocardiography, exercise tolerance testing, cardiac catheter ization, the use of prostaglandin in the therapy of ductal-dependent congenital heart disease, the use of indomethacin, and certain amazing advances in infantile cardiac surgery. Of the surgical successes, the d-transposition of the great arteries was especially noted. Whereas previously 90% of infants with this abnormality died within six months, modern surgical repair gives these infants a long-term survival rate of almost 95%.

As in almost all branches of medicine the field of pediatrie cardiology has continued to advance rapidly. The most important recent achievements are presented in this symposium from The New York Hospital-Cornell Medical Center.

The first contribution to this second portion of the present symposium discusses "Pulmonary Function Testing in Children with Cardiac Disease" and has been written by Dr. Ingrid K. Rosner, CoDirector, Division of Pediatrie Allergy, Immunology and Pulmonary Diseases, and Assistant Professor of Pediatrics.

Dr. Rosner describes clearly the necessity of knowing, prior to operation, the child's pulmonary function, for in thoracic surgery the pain may reduce the inspiratory capacity by 50%. For complete respiratory safety one should be able to predict the child's ability to maintain adequate ventilation postoperatively. Three case histories are presented.

The next article, "Cardiac Surgery Without Preoperative Cardiac Catheterization" is authored by Dr. Mary Alien Engle, the Guest Editor for this symposium. Dr. Engle is Stavros S. Niarchos Professor of Pediatrie…

This is the second issue of Pediatric Annals presented as a tribute to the late Dr. Helen Brooke Taussig, recognized as the founder of pediatrie cardiology. It was in 1930 that Dr. Edward A. Park, Professor of Pediatrics at Johns Hopkins Medical College, organized special clinics in his department and appointed Dr. Taussig as director of the cardiac clinic.

I came from what may be termed the pre-Taussig days when our primary cardiac concerns, as practicing pediatricians, were the consequences of rheumatic fever. We were trained to be good physical diagnosticians; we had roentgenograms, fluoroscopes, and electrocardiograms, but no cardiac surgery.

Dr. Taussig soon became an important figure in pediatric cardiology. It is interesting to note that, in the leading pediatrie textbook, the 1940 edition of Holt's Diseases of Infancy and Childhood, the section on the heart was written by Dr. Taussig. I have just reread this whole chapter and noted with interest her suggestion that the cardiac defects found in children with "mongolian idiocy" might indicate an arrest in fetal development at a specific time. This was, of course, not correct. We had no knowledge of the chromosomal abnormality then. But it did show her intense effort, which continued throughout her life, to find some basic cause for congenital cardiac defects. She didn't say so, but I wonder if when she went to Europe to study the tragic thalidomide babies she wasn't seeking a possibility of cardiac defects in such children.

If I am not mistaken, corrective surgery on the heart was first instituted by Gross and Hubbard who, in 1939, described the ligation of the patent ductus. Then in 1944, Dr. Taussig discussed her operative proposal with Dr. Blalock, resulting in the development of the Blalock-Taussig operation which immediately "turned blue babies pink." At the time of this history-making operation, Dr. Mary Alien Engle, the Guest Editor of this symposium, was a substitute intern on the staff of Dr. Blalock. The years that followed produced tremendous advances in knowledge, diagnosis, therapy, and surgery in pediatric cardiology.

Six years ago (1981) we last reviewed in Pediatrie Armais the subject of pediatrie cardiology. This review was also under the Guest Editorship of Dr. Engle. The newer approaches discussed at that time included echocardiography, exercise tolerance testing, cardiac catheter ization, the use of prostaglandin in the therapy of ductal-dependent congenital heart disease, the use of indomethacin, and certain amazing advances in infantile cardiac surgery. Of the surgical successes, the d-transposition of the great arteries was especially noted. Whereas previously 90% of infants with this abnormality died within six months, modern surgical repair gives these infants a long-term survival rate of almost 95%.

As in almost all branches of medicine the field of pediatrie cardiology has continued to advance rapidly. The most important recent achievements are presented in this symposium from The New York Hospital-Cornell Medical Center.

The first contribution to this second portion of the present symposium discusses "Pulmonary Function Testing in Children with Cardiac Disease" and has been written by Dr. Ingrid K. Rosner, CoDirector, Division of Pediatrie Allergy, Immunology and Pulmonary Diseases, and Assistant Professor of Pediatrics.

Dr. Rosner describes clearly the necessity of knowing, prior to operation, the child's pulmonary function, for in thoracic surgery the pain may reduce the inspiratory capacity by 50%. For complete respiratory safety one should be able to predict the child's ability to maintain adequate ventilation postoperatively. Three case histories are presented.

The next article, "Cardiac Surgery Without Preoperative Cardiac Catheterization" is authored by Dr. Mary Alien Engle, the Guest Editor for this symposium. Dr. Engle is Stavros S. Niarchos Professor of Pediatrie Cardiology, and Director of Pediatrie Cardiology.

In this very interesting article Dr. Engle reviews the changes that have occurred in evaluating cardiac lesions prior to operation. In the past, cardiac catheterization and selective angiography were routine procedures prior to all operations except for the simple patent ductus arteriosus. But today, with advances in the use of echocardiography and Doppler echocardiography, the necessity for catheterization is eliminated in many conditions.

Step by step, Dr. Engle reviews the most common cardiac defects, indicating in which cases preoperative diagnosis can be made without the need for catheterization. One cannot help but be impressed by the ever improving safe and noninvasive techniques in our diagnostic armament.

The next article presents a recent and fascinating therapeutic approach, "Percutaneous Balloon Valvuloplasty for the Treatment of Valvular Pulmonic Stenosis." It has been written by Dr. Arthur A. Klein and Dr. Aaron R. Levin, CoDirector and Director respectively of the Pediatric Catheterization Laboratory of the Division of Pediatric Cardiology.

As the authors point out at the beginning of their article, a good many interventional cardiac catheterization techniques have been described for palliation of congenital heart disease. Of these the percutaneous balloon dilatation technique for pulmonary valve stenosis has gained widespread acceptance.

Doctors Klein and Levin carefully describe the technique of this procedure - the early preparation with angiographie studies for location and size of the stenosis and the use of the balloon dilatation catheters. Results show that this approach can be as successful as surgical intervention with low morbidity and mortality. To date, its use in neonates with severe pulmonic stenosis has been unsuccessful. However, the authors suggest that with the development of smaller catheters this difficulty may be resolved.

The fourth article in the symposium discusses the "Detection and Management of Cardiac Involvement in the Kawasaki Syndrome." It is presented by Dr. Nunzia S. Fatica, Dr. Fukiko Ichida, Dr. John E. O'Loughlin, and Dr. Mary Alien Engle, all of the Division of Pediatrie Cardiology.

This syndrome with a most unusual symptom complex was first described in Japan in 1963 where it occurred in epidemic form. Recently it has appeared in the United States. Diagnosis of the condition with its multiple symptoms is important, since coronary aneurysms occur in approximately 20% of the cases. Under proper care these aneurysms subside completely.

The use of acetylsalicylic acid and intravenous gamma globulin are described in this condition for the prevention of aneurysms. However, early recognition of Kawasaki syndrome is important, for if treatment is to be effective it should be given early.

The final article concerns the "Recognition, Management, and Prevention of Respiratory Syncytial Virus Infection in the Child with Cardiopulmonary Disease." It is written by Dr. Deborah A. Laufer and Dr. Paul J. Edelson of the Division of Pediatrie Infectious Diseases and Immunology, Department of Pediatrics.

This virus infects 50% of children under the age of one, and according to the authors, by the time children are three years of age approximately 100% have had at least one infection. Most children handle the infection with little difficulty, but in children with preexisting cardiac or pulmonary disease, the condition may be life-threatening. A study is reported from Rochester, New York where RSV-infected children with congenital heart abnormalities had a 37% fatality rate as compared with 1% of otherwise healthy children.

The respiratory syncytial virus is not spread by aerosol but by handling the respiratory secretions, handling the infant, or through fomites. This emphasizes the important of handwashing. Because of the danger of this infection for children with congenital cardiac conditions, it is advised that they not be hospitalized during the winter months - the period of high incidence of the RSV infection.

The authors present an excellent clinical history of these infected children with a review of their chest x-rays. The rapid and reliable test for the infection is detailed. If a child with a congenital heart condition develops signs of an RSV infection he or she should be hospitalized at once. Since these children are almost always hypoxic, supplemental oxygen is an essential initial therapy. Various approaches to treatment are discussed, with the use of nbavirin, a drug with specific antiviral effectiveness, advised. In several reported studies using ribavirin, the symptoms were more rapidly decreased, and length of hospital ization was definitely decreased. The drug is well tolerated.

10.3928/0090-4481-19870801-05

Sign up to receive

Journal E-contents