Congenital pulmonary vein stenosis: structural changes in a patient with normal pulmonary artery wedge pressure.

A male infant is described who died at 13 months of age with stenosis of all extrapulmonary veins except the left upper vein. The pulmonary artery wedge pressure was normal, the first time this is reported in this condition. At autopsy, there were structural changes of the pulmonary arteries and veins in all lobes with or without pulmonary vein stenosis. Arterial changes-muscle extension, medial hypertrophy and decreased arterial size--analyzed quantitatively were found to be similar in all lobes. Venous medial hypertrophy was more marked in obstructed lobes. These anatomic changes are presumably due to fixed venous obstruction in the pulmonary lobes drained by stenotic veins and to high flow in the left upper lobe.

Marital stability and congenital heart disease.

The incidence of divorce or legal separation was studied in 438 families of children born with heart disease who entered the New England Regional Infant Cardiac Program between 1968 and 1973. The parents were interviewed when the children were 5 1/2 years old. The rate of divorce in 438 families of children with critical congenital heart disease was not significantly different from the rate in two comparison groups: (1) 25 families of children whose cardiac defect was spontaneously cured, and (2) 26 families of children catheterized in infancy for suspected cardiac defect but who were found to be free of heart disease. Rates of divorce or legal separation for the three groups were: critical congenital heart disease, 12.1%, spontaneously cured, 4.2% free of heart disease, 11.5% these rates were not significantly different. The average national divorce rate was 20.3% for the same period.

Extracardiac abnormalities in infants with congenital heart disease.

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.

Tricuspid atresia: clinical course in 101 patients.

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.

Radionuclide angiocardiographic assessment of pulmonary vascular reactivity in patients with left to right shunt and pulmonary hypertension.

Radionuclide angiocardiography was used to assess pulmonary vascular reactivity in eight patients (nine studies) with a large, relatively unrestrictive intracardiac defect and pulmonary arterial hypertension. Radionuclide angiocardiograms, using technetium-99m pertechnetate, were performed first with the patient breathing room air and then after 10 minutes of breathing a mixture containing 90 percent or more of oxygen. The pulmonary to systemic flow ratios obtained by gamma variate analysis of the radionuclide time-activity curves were compared with those calculated with the Fick principle at the time of cardiac catheterization. There was a good correlation between the two methods both in room air studies (r = 0.88) and in those obtained with 90 percent or more of oxygen (r = 0.94). All six studies (in five patients) with a reactive pulmonary vasculature (judged by a pulmonary vascular resistance at cardiac catheterization of less than 6 units/m2 with oxygen or after tolazoline) had a radionuclide pulmonary to systemic flow ratio of 3.0 or greater with oxygen. The three patients with a nonreactive pulmonary vasculature had a radionuclide pulmonary to systemic flow ratio of 2.3 or less with oxygen, a value that was unchanged from the room air value. These data suggest that radionuclide angiocardiography may be a useful, relatively noninvasive method of assessing pulmonary vascular reactivity in patients with a large, relatively unrestrictive intracardiac defect.

Results of pulmonary arterial banding in infancy. Survey of 5 years' experience in the New England Regional Infant Cardiac Program.

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.

Anterior chest wall deformities and congenital heart disease.

Pectus excavatum and pectus carinatum usually exist as isolated abnormalities. Only 19 cases of associated congenital heart defects have been reported. Significant complications related to uncorrected pectus excavatum have been described either during or after cardiac operations. Therefore we reviewed our experience with these coexisting lesions to assess the risk of surgical repair of chest wall deformities before and after correction of congenital cardiac anomalies. Among 20,860 infants and children with congenital heart disease seen at our institution, 36 (0.17%) had associated anterior thoracic deformities, 22 of whom underwent surgical correction of pectus excavatum or pectus carinatum. Ten of these 22 patients had pectus repair after a cardiac operation. Pleural or pericardial entry was avoided in all and none required a blood transfusion. Ten other patients had pectus repair either before cardiac repair (five patients) or without a subsequent cardiac operation. Another patient had a cardiac operation performed through a median sternotomy both before and after pectus repair, and the remaining patient, early in the series, had simultaneous banding of the main pulmonary artery and repair of pectus excavatum complicated by chest wall instability and a lethal intrathoracic hemorrhage. The experience indicates that congenital chest wall deformities can be safely and effectively repaired after early correction of congenital heart defects through a median sternotomy, although repair of the chest wall deformity after cardiac surgery also gives good results. However, in children who require an extracardiac conduit for repair of their congenital heart defect, we recommend initial repair of the pectus excavatum followed at 6 weeks or later by repair of the cardiac lesion to eliminate possible extrinsic compression of the conduit by the depressed sternum. We avoid simultaneous cardiac and pectus excavatum repair because of potential associated major complications.

Origin of pulmonary artery branch from ascending aorta. Primary surgical repair in infancy.

1. Surgical repair of origin of a pulmonary artery branch from the ascending aorta should be performed as early as possible in order to prevent death from congestive heart failure or the development of irreversible pulmonary vascular obstructive disease. 2. Deep hypothermic circulatory arrest greatly facilitates surgical repair of this lesion in infancy, as is illustrated by the three cases reported herein. 3. Direct anastomosis of the ectopic pulmonary artery branch to the main pulmonary artery is the surgical technique of choice. 4. Origin of the RPA or the LPA from the ascending aorta results from origin of the RPA or LPA from the aortic sac, instead of from the confluent sixth arches. Typically, the RPA has failed to migrate leftward because of abnormal development of the wall of the aortic sac, abnormal development of the sixth arches, or both. 5. Origin of the RPA or the LPA from the ascending aorta should be distinguished from origin of the "RPA" or of the "LPA" from the innominate artery or from the aortic arch via a PDA or a collateral artery.

Psychological functioning in children with cyanotic heart defects.

The relationship between congenital heart disease (CHD) and psychological functioning was studied in 63 children with transposition of the great arteries (TGA) and 77 children with tetralogy of Fallot (TF). These youngsters were compared with a group of 36 children originally diagnosed with CHD, but who spontaneously recovered (SR) without medical intervention. All children were under one year of age when diagnosed and were given psychological testing between 5.5 and 6.3 years. Children with TGA and TF had poorer overall psychological functioning and significantly greater central nervous system (CNS) impairment than the SR children. However when the effects of IQ and CNS impairment were controlled, there were no differences between groups. The diagnosis of a severe cyanotic heart defect does not appear to make a child more likely to have emotional disorder in the absence of other factors.