D G McNamara

Status of the adult and adolescent after repair of tetralogy of Fallot.

Because increasing numbers of patients with surgically repaired tetralogy of Fallot (TOF) are surviving to adulthood, this study was done to provide follow-up data for physicians concerned with their care. We reviewed the current status of 233 patients born before January 1962 who had repair ofTOF at a median age of 9.7 years (range 11 months to 36 years). There were 26 surgical deaths (11.1%), 12 late cardiacrelated deaths (5.9%) and three noncardiac deaths (1.3%). Eight of the 12 late deaths occurred suddenly and unexpectedly. In each of these eight patients we had previously documented elevated right ventricular systolic pressure as well as ventricular premature depolarizations (VPDs). Among the 95 patients who responded to a questionnaire, 84 denied symptoms, 28 were married, 44 had attended college and all were employed. Cardiac catheterization revealed an unsatisfactory hemodynamic result in 39 of 120 patients, 30 of whom were asymptomatic. The poor result was due to a large left-to-right ventricular shunt in eight patients, persistent right ventricular outflow obstruction in 25 patients, pulmonary vascular obstructive disease in five patients, and left ventricular cardiomyopathy in one patient. Among the 21 patients with VPDs, 17 underwent cardiac catheterization: 15 had a right ventricular systolic pressure > 60 mm Hg and all had elevated right ventricular end-diastolic pressure. We conclude that 1) clinical assessment alone is nonpredictive of the hemodynamic result and thus cardiac catheterization is indicated in all patients; 2) the combination of persistent elevation of right ventricular systolic pressure above 60 mm Hg and VPDs placed the patient at risk for sudden death; and 3) over 80% of adults with repaired TOF can lead a normal life without impairment of intellect, exercise tolerance or fertility.

Electrophysiological abnormalities after Mustard's operation for transposition of the great arteries.

Because of the frequent occurrence of significant dysrhythmias after Mustards operation for transposition of the great arteries, electrophysiological studies were performed in i6 patients during postoperative cardiac catheterization. Intracavitary electricalpotentials were recordedfrom the conduction system in all i6patients. In 9 of these patients, the stability of the atrioventricular conduction system during rapid atrial pacing was tested, as well as the subsequent promptness ofpacemaker recovery. The pacemaker recovery time, corrected for cycle length, was significantly prolonged in 4 of 9 patients studied. After atrialpacing, an escapejunctional rhythmpreceded the resumption of sinus rhythm in 3 patients. No intra-atrial block was found between the upper and lower intra-atrial electrograms. In 4 patients, an early electrical activity was recorded inside the pulmonary venous (new left) atrium, above the tricuspid valve. This electrical activity occurred during the T-P interval and was unrepresented on the surface electrocardiogram. No significant abnormalities ofconduction were observed in the atrioventricular node or the His Purkinje system. Furthermore, rapid atrial pacing caused no abnormal atrioventricular block. The abnormal pacemaker recovery time and the absence of conduction disturbances in the atria, atrioventricular node, and His-Purkinje system confirm the hypothesis that injury to the sinoatrial node is the main

Comparison of resection versus patch aortoplasty for repair of coarctation in infants and children.

To evaluate two surgical techniques of treatment for coarctation of the aorta in infants and children, we retrospectively compared our long-term experience with resection and end-to-end anastomosis (resection) vs Dacron patch aortoplasty in patients operated upon when younger than 16 years of age.Since 1967, 184 patients underwent either resection (n = 96) or patch aortoplasty (n = 88). These groups were comparable in terms of preoperative systolic blood pressure, systolic pressure gradient across the coarctation, the year of operation, age and weight at operation, prevalence of associated cardiovascular defects, and duration of postoperative clinical follow-up (1-12.3 years, median 4.3 years).There was no statistically significant difference in operative mortality between surgical groups. Among 104 patients with follow-up longer than 1 year, there was no statistically significant difference between the two operations in the appearance of a residual gradient greater than 10 mm Hg, but for both groups this degree of residual gradient was more frequent in infants (22 of 43, 51%) than in older children (18 of 61, 31%; p < 0.025). Late postoperative systolic hypertension was noted in 20 of 104 patients (19%), divided equally between the two operative groups. Most late hypertensive patients (13 of 20, 65%) also had residual coarctation; otherwise, the occurrence of postoperative hypertension was not influenced by the type of repair or age at repair.We conclude that resection and patch aortoplasty are equivalent operations for coarctation of the aorta in infants and children, and that long-term relief of gradient is related to age at operation rather than to technique of repair.

The absent pulmonary valve syndrome. Considerations of management.

The absent pulmonary valve syndrome consists of a severely hypoplastic pulmonary valve with anular stenosis, aneurysmal dilatation of the main pulmonary artery with one or both pulmonary branches also dilated, and a ventricular septal defect. From 1955 to 1975, 15 patients were seen at Texas Childrens Hospital with this syndrome. Although anatomically similar to tetralogy of Fallot, the most significant symptoms during early infancy were secondary to bronchial compression resulting from the dilated pulmonary arteries and enlarged left atrium. Intracardiac repair performed on two infants at four months of age failed to ameliorate the respiratory symptoms and both died. Seven patients from 2.3 to 16 years of age were operated upon, with one late postoperative death. Five other patients have not yet received surgical intervention. We recommend vigorous continual respiratory therapy for infants and small children and intracardiac repair in older children since repair in infancy does not relieve respiratory symptoms. We do not recommend a prosthetic pulmonary valve unless there is persistently elevated main pulmonary artery pressure.

Late development of pulmonary venous obstruction following Mustard's operation using a dacron baffle.

Tbree patients developed late pulmonary vein obstruction (PVO) following Mustards operation for transposition of the great arteries. In all three the absence of PVO bad been documented by an earlier postoperative cardiac catheterization. At reoperation shrinkage and kinking of the dacron baffle was evident. After Mustards operation, patients should be observed cloey for symptoms and signs of PVO since it can occur insidiously despite previously proven absence of this complication.

Visualization of the pulmonary arteries in pseudotruncus by pulmonary vein wedge angiography.

SUMMARY Pulmonary venous wedge angiography revealed the anatomy of the mediastinal and parenchymal pulmonary arteries in 22 patients with obstruction of the right ventricular outflow tract, proximal pulmonary arteries or branch pulmonary arteries, when conventional angiographic methods had failed. Nine of these patients had pseudotruncus-type anatomy and had no previous surgery. One pulmonary artery was not visualized in 13 of the 22 patients because of postoperative occlusion in 10, congenital absence of the left pulmonary artery in two, and suspected sequestration of the left lower lobe in one.Retrograde pulmonary wedge angiograms were obtained by weding an end-hole catheter in a pulmonary vein and slowly injecting, by hand, 0.2 to 0.45 ml/kg of contrast material followed by a flush of dextrose solution (1-2 ml/kg). In seven of the nine patients without previous surgery, the confluence of the pulmonary arteries in the mediastinum could be seen clearly by this method. Nine patients had a previous systemic-to-pulmonary anastomosis, and only the ipsilateral pulmonary arteries were visualized by aortography. Venous wedge angiography filled the pulmonary arteries of the nonopacified lung up to the point of obstruction in the mediastinum in each patient. Eight patients were able to have a systemic-to-pulmonary shunt because of the visualization of the previously unseen pulmonary arteries.We conclude that pulmonary venous wedge angiography is a valuable adjunct to the current angiographic methods of investigating patients with pulmonary atresia and related conditions. In some cases, it may be the only way to determine the presence of surgically accessible pulmonary arteries.

Balloon atrial septostomy for total anomalous pulmonary venous return.

During the 2-year period July I970 to July I972, balloon atrial septostomy was performed in I2 patients with total anomalous pulmonary venous return, ranging in age from 4 days to I year, and in weight from 2 27 to 6 8o kg. After septostomy, 9 of the I2 patients were improved both clinically and haemodynamically. Of these 9 patients, I died inexplicably I week after the septostomy. In the remaining 8 patients, improvement was maintained during follow-up ranging from 7 months to 2 years. Repeat cardiac catheterization in 4 patients documented continued haemodynamic improvement. Of the I2 patients, 3 were not helped by the septostomy and required operation within a few days. In 2 of these infants, the opening created by the septostomy was found at operation to be too small to have been considered beneficial, yet both of these patients had had less than a i mmHg mean atrial pressure gradient before septostomy. The third patient, who had infradiaphragmatic return, had a large interatrial communication confirmed at operation. Based on the results achieved in these patients, balloon atrial septostomy is now recommended at the time of the initial cardiac catheterization in all infants with total anomalous pulmonary venous return. The procedure is less likely to be successful in infants over 3 months of age, and in those with infradiaphragmatic pulmonary venous return. The haemodynamic improvement obtained by successful septostomy in the young infant should allow delay of operation until a more optimal age.

Anomalous origin of left coronary artery from the pulmonary artery with ventricular septal defect.

Only two cases have been reported previously of the association of ventricular septal defect (VSD) with anomalous origin of the left coronary artery (ALCA) arising from the pulmonary artery. The purpose of this paper is to present two additional cases, to describe the pathophysiology, and to emphasize how the clinical course of this combination of defects differs from that of isolated ALCA. Patients with both of these anomalies present in infancy with manifestations only of a large left-right ventricular shunt and pulmonary hypertension. Initially the ALCA is well perfused from the high pressure in the pulmonary artery. In these instances in which the pulmonary artery pressure subsequently decreased because of spontaneous reduction in size of the VSD, the left coronary arterial system became less well perfused. Because of this decreased perfusion in association with the left ventricular myocardial stress initially caused by volume overload, myocardial ischemia and ultimately infarction occurred. Early identification and repair of the anatomic abnormality could prevent irreversible myocardial damage.

Congenital coronary artery obstruction associated with aortic anomalies in children: report of two cases.

Coronary arterial obstruction associated with congenital aortic valve disease is rare in children. We studied two children with aortic valve disease and symptoms of coronary insufficiency. Cineangiography revealed localized obstruction of the proximal left coronary artery. In one patient, a membrane-like structure partially covered the left sinus of Valsalva, almost isolating the ostium of the left coronary artery; the other patient had severe stenosis of the proximal left coronary artery and a congenitally hypoplastic left coronary cusp. Surgical repair was successful in both cases. Symptoms of myocardial ischemia in children with aortic valve disease should be investigated promptly to exclude obstruction of the coronary arteries.