Hazards of cardiac catheterization in children with primary pulmonary vascular obstruction.
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Biomedical subjects
Publications and source records attributed to D C Fyler.
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Thirty specially designed 4 French pigtail white Teflon catheters 50 cm in length were utilized for angiography using the retrograde arterial approach in 26 infants and children. Angiographic opacification was considered very satisfactory. Apart from one episode of temporary occlusion of the right coronary artery ostium, there were no other significant complications. The catheters were introduced percutaneously in 21 instances and distal pulses were palpable in 18 patients by the second day after catheterization. At postcatheterization testing with increasing volumes and flow rates, the first rupture occurred, delivering 13 ml at 13 ml/second. On testing an additional 15 catheters using fixed volumes of 5, 10, and 15 ml at increasing flow rates, rupture first occurred at 34, 22, and 18 ml/second, respectively. We conclude that this small catheter is relatively safe and satisfactory for the retrograde arterial approach in the study of infants and children.
Infants born in New England with congenital heart defects were examined for association with time of year or population density. Moderate seasonal peaks in births were noted for complex ventricular septal defect, malposition defects, and transposition of the great arteries. Positive associations with population density were found for pulmonary atresia with ventricular septal defect, ventricular septal defect with secondary anomalies, and tricuspid atresia.
A total of 161 infants and children, ranging in age from 1 day to 17 years at initial encounter (mean, 3.7 years), was seen over a 30 year period with primary myocardial disease (idiopathic myocarditis, nonobstructive cardiomyopathy, endocardial fibroelastosis, and an anatomically unknown category). These patients were observed from 1 hour to 23 years after initial encounter and cardiac disease has resolved in 27 per cent, resulted in death in 35 per cent, and continues in 38 per cent. The majority were first referred to us with congestive heart failure; all exhibited ST-T changes and cardiomegaly, 67 of 150 had left ventricular hypertrophy, 23 of 151 arrhythmias, and 55 of 153 pulmonary vascular congestion. Initial ventricular depolarization abnormalities were very frequent. Significant clinical predictors of fatal outcome included pulmonary vascular congestion, "northwest" axis deviation, and a cardiac index less than three L./min./M.2. Death occurred during the first year after initial encounter in 44 of 57 who died, and in all 13 with proved myocarditis. Primary myocardial disease is a serious disease of infancy and childhood, resulting in death or residual cardiac disease in three fourths of those affected.
Data from 30 infants with interrupted aortic arch in the New England Regional Infant Cardiac Program, 1968 to 1974, were reviewed. All patients had major associated cardiac abnormalities: approximately one third had ventricular septal defect with patent ductus arteriosus, one third had complex ventricular septal defect with left ventricular outflow obstruction, and one third had complex intracardiac lesions incompatible with survival. Twenty-one of the patients had either palliative or reconstructive surgery, with a hospital mortality rate of 76%. Nine patients did not have surgery; eight of them died at a median age of four days. One-stage primary repair of interrupted aortic arch including the associated cardiac defects, using deep hypothermia and circulatory arrest, is proposed as the current method of treatment in such infants.
The records of the New England Regional Infant Cardiac Program, a service program covering all of New England, provide a useful source of information about the characteristics of children born with congenital heart defects. Data were analyzed on more than 2000 children born in New England who were diagnosed with a congenital heart defect before the first birthday. Children with arterio-venous fistula, aortic stenosis, transposition of the great arteries or hypoplastic left ventricle were predominantly male; children with persistent ductus arteriosus and endocardial cushion defect were predominantly female. Positive trends in risk with increasing birth order were present for pulmonic stenosis and transposition of the great arteries, and a negative trend was seen for persistent ductus arteriosus. What evidence there was for associations with maternal age was greatly reduced after controlling for confounding by birth order.
The results of pulmonary arterial banding in 238 infants, 12 percent of the infants admitted to the New England Regional Infant Cardiac Program, is reviewed. Overall survival to age 1 year was 63 percent. Survival was least likely (37 percent) in those who required banding within the 1st month of life. Additional surgery decreased the survival rate in those operated on after 1 month of age. Infants with anomalies for which no corrective surgical procedure is available (23 of 238) have only a 30 percent chance of survival. Those with lesions correctable within the 1st year (133 of 238) have a 74 percent survival rate; 52 percent (82 of 238) of those for whom a curative operation is available after the 1st year survive. These pulmonary arterial banding data coupled with results of primary correction should provide the data base required for an intelligent decision in respect to appropriate surgical treatment of infants with critical heart disease.
The clinical profile and course of 101 patients with tricuspid atresia seen at the Children's Hospital Medical Center, Boston, were reviewed; the electrocardiograms, hemodynamic data, associated anomalies, complications of the malformation and surgical results were outlined and a definition of anatomic types and radiologic groups formed in order to facilitate therapeutic decisions. Overall survival to 15 years of age was approximately 50 percent. Tricuspid atresia type 1 (normally related great arteries), group A (decreased pulmonary vascularity) was the most frequent category (54 percent); without surgery, these patients had only a 10 percent chance of survival beyond the 1st year of life. Surgical intervention improved chance of survival to 15 years of age to 50 percent. Overall surgical mortality was 23 percent. The Waterston shunt is the procedure of choice in symptomatic small infants with diminished pulmonary flow (group A). Type II patients with increased pulmonary blood flow (group B) would benefit from a pulmonary arterial band. In contrast, patients with group B, type I atresia would rarely need a pulmonary arterial band. Patients with spontaneously changing hemodynamics (group C) usually require a shunt later in life than those in group A, with more favorable operative results. Our experience indicates the continued need of early surgical palliation. New surgical approaches, such as the right atrial-pulmonary arterial anastomosis, may result in a more prolonged survival.
Thirty-one left ventricular (LV) biplane angiograms were performed in 28 patients with tricuspid atresia. Measurements of left ventricular end-diastolic volume (LVEDV) and left ventricular end-systolic volume were obtained by the modified Simpson's rule and systolic ejection fraction (EF) calculated. Left ventricular volumes and ejection fractions were also obtained in 19 control patients with no significant heart disease. The patients with tricuspid atresia were classified according to the appearance of the pulmonary vascularity on initial radiologic examination: Group A, decreased pulmonary vascularity; Group B, increased pulmonary vascularity. In the 13 group A infants who were unoperated, LVEDV was increased and EF mildly diminished. In the group B patients LVEDV was increased and EF normal. In the 12 group A patients with surgical shunts LVEDV was elevated. The five group A patients with long-standing systemic artery to pulmonary artery anastomoses (greater than 10 years) showed the largest LVEDV and the poorest EF. The angiographic data indicate that patients with tricuspid atresia experience significant LV dysfunction as a consequence of longstanding LV volume overload. The early detection of LV dysfunction may be an indication for a right ventricular bypass procedure in these patients.
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Extracardiac anomalies (ECA) occur in 25% of infants seen during the first year of life for significant cardiac disease. Often the ECA are multiple and one third of the affected infants have some established syndrome. In infants with endocardial cushion defect, patent ductus arteriosus, atrial septal defect, ventricular septal defect, malpositions, tetralogy of Fallot, and complex coarctation ECA occur in 25% or more of the cases. The most frequent ECA are in the musculoskeletal system or associated with a specific syndrome. The presence of an ECA significantly increases the mortality in infants with CHD.
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