Pacing threshold spikes months and years after implant.

To determine patterns of variation in chronic pacing thresholds, we made 4,942 threshold measurements in 257 patients with 312 leads, at times from implant to 295 months (median 17 months) including 1,053 determinations in 46 children less than 12 years old. Motivation was late sudden death in two single-ventricle pacemaker-dependent children with multiple possible death causes. At stimulus duration 0.

Complete transposition of the great arteries: treatment in the current era.

Transposition of the great arteries usually can be diagnosed by fetal echocardiography, which is important because the arterial switch operation, currently the treatment of choice, must be performed very early in life. The results of the arterial switch operation have been good to date. The dilemma remains, however, whether a fetus in whom a diagnosis of transposition is made should be aborted or treated surgically soon after delivery.

Corrective repair of complete atrioventricular canal defects and major associated cardiac anomalies.

Twenty-nine patients with balanced forms of complete atrioventricular (AV) canal defects and tetralogy of Fallot in 12, double-outlet right ventricle (DORV) in 10, and additional anomalies associated with atrial isomerism in 7 underwent corrective repair. Age ranged from 39 days to 24 years (median, 3.7 years).

Prediction of severe obstruction to right ventricular outflow after repair of tetralogy of Fallot and pulmonary atresia.

Cineangiograms were available for a quantitative retrospective study along with complete clinical information in 96 patients who underwent intracardiac repair of tetralogy of Fallot with pulmonary atresia. Multivariate analysis determined that the risk factors for too high a ratio (greater than or equal to 1) between the peak pressure in the right ventricle and that in the left, in the operating room about 30 minutes after repair, were as follows: size of the patient, small size of the right and left pulmonary arteries, and a larger number of large aortopulmonary collateral arteries. When, according to the multivariate equation, the predicted probability of this ratio being equal to or greater than 1 is 50% or more, consideration may be given to preliminary operations before repair; when the predicted probability is 70% or more, complete repair at that stage may be unwise.

Survival, functional status, and reoperations after repair of tetralogy of Fallot with pulmonary atresia.

Data: Among 139 patients who underwent repair of tetralogy with pulmonary atresia, survival rates at 1 month and at 1, 5, 10, and 20 years were 85%, 82%, 76%, 69%, and 58%, respectively. The hazard function (instantaneous risk of dying) was greatest immediately after operation and declined thereafter, but a low constant hazard persisted for as long as the patients were followed up. Multivariately, the postrepair ratio between peak right ventricular and left ventricular pressures measured in the operating room provided the most information relative to the probability of death after repair, and cardiopulmonary bypass time the next.

The structure of the pulmonary circulation in tetralogy of Fallot with pulmonary atresia. A quantitative cineangiographic study.

Unlabelled: Cineangiograms of 172 patients with tetralogy of Fallot and pulmonary atresia were qualitatively and quantitatively analyzed retrospectively.

Findings: Twenty-eight (16%) patients had congenital absence of the central and unbranched hilar portions of the left or right pulmonary artery or both, and thus had congenitally nonconfluent pulmonary arteries. Five additional patients (3%) with originally confluent pulmonary arteries had nonconfluent ones because of an end-to-end surgical shunt, as did seven others (4%) because of thrombosis of one pulmonary artery.

Subaortic stenosis and sudden death.

An obese white boy, 16 years old, collapsed and suddenly died while walking with friends. Since early childhood he had been known to have mild subaortic stenosis. At age 6 he exhibited first-degree heart block during an electrocardiographic exercise test.

Transatrial-transpulmonary repair of tetralogy of Fallot.

Classical tetralogy of Fallot was repaired by a transatrial-transpulmonary approach in 61 of 70 patients treated surgically on one surgical service between July 1981 and December 1985. Their ages ranged from 2.5 months to 57 years (mean 52.

Pericardial cyst diagnosed by two-dimensional echocardiography and computed tomography in a newborn.

A one-day-old full-term female infant with massive "cardiomegaly" developed marked respiratory distress soon after birth. The two-dimensional (2D) echocardiogram demonstrated a 4 X 5-cm cystic mass adjacent to the left ventricular free wall. Computed tomography (CT) confirmed the presence and dimension of the cyst and CT number indicated the fluid within the cyst to be serous.

The repair of atrioventricular septal defects in infancy.

Two (3%) of 72 patients undergoing repair of atrioventricular septal defects, 1984-September 1985, died in hospital, and among the 58 patients with isolated atrioventricular septal defect, one (2%) died in hospital. One (3%) of the 30 patients with isolated atrioventricular septal defects undergoing repair in the first year of life died. The absence of an incremental risk of young age is a continuation of the trend demonstrated in a parametric analysis of the earlier 1967-1982 experience (n = 310).

The Fontan operation. Ventricular hypertrophy, age, and date of operation as risk factors.

One hundred two patients, aged 0.7 to 38 years, with a wide variety of cardiac malformations underwent the Fontan operation (1975 to April, 1985). Several different techniques were used.

Current risks and protocols for operations for double-outlet right ventricle. Derivation from an 18 year experience.

Recent follow-up of 127 patients having repair of double-outlet right ventricle during an 18 year experience (1967 to July, 1984) indicated an overall actuarial survival rate at 12 years of 38%. However, multivariate analysis supported by contingency tables indicated that currently the early (2 week) survival rate after the intraventricular tunnel repair for double-outlet right ventricle with subaortic ventricular septal defect in 6-month-old infants is 99% and the 10 year survival rate 97%. Reoperation of the tunnel repair was rarely required (one of 56 patients), and the functional results were excellent.

Intraventricular tunnel repair for Taussig-Bing heart and related cardiac anomalies.

Uncertainty currently exists as to the optimal definitive procedure for treatment of patients with the Taussig-Bing heart and related cardiac anomalies. Between December 1, 1981 and October 1, 1985, we have used a completely intraventricular tunnel repair, without the use of an extracardiac conduit, in nine patients between 1 and 33 months of age. There was one hospital death from low cardiac output in a 1-month-old infant and one late death 11 months after repair from preexisting and progressive right ventricular cardiomyopathy.

Spectrum of hearts with one underdeveloped and one dominant ventricle.

Hearts with one underdeveloped and one dominant ventricle form a spectrum of anomalies extending from the heart with two clearly adequate chambers to those with a true single ventricle. An angiographic concept of grouping such hearts is presented. 129 patients with unequal ventricles underwent catheterization and cineangiography between 1974 and 1983.

Death and other events after cardiac repair in discordant atrioventricular connection.

The 1 month, 1 year, and 10 year actuarial survival rates after operation among 99 consecutive patients with discordant atrioventricular connection who underwent intracardiac repair are 86%, 75%, and 68%, respectively. The hazard function of death declines rapidly after operation to reach a low level 6 months postoperatively, and it becomes indistinguishable from that of a matched general population by 4 years. Risk factors for premature death include double-outlet right ventricle, complete heart block preoperatively or developing perioperatively, very young or older (greater than 25 years) age, a high hematocrit value, and earlier date of operation.

Overriding atrioventricular valves. An angiographic-anatomical correlate.

An analysis of 6 patients with overriding atrioventricular valve was performed in order to correlate the anatomic and angiographic features of this malformation. The atrial arrangement was usual (situs solitus) in each patient. The ventricular chambers were normally related in 4 and the mirror image of normal in 2.

Angiographic anatomy of hearts with one ventricular chamber: the true single ventricle.

Fifteen patients with an isolated ventricular chamber were studied using angiography. These patients constitute 7% of the 218 patients with single ventricle who were examined at our institution over a 10-year period. The atrial situs was solitus in 6 and isomeric in 9.

Secondary left atrioventricular valve replacement in atrioventricular septal (AV canal) defect: a method to avoid left ventricular outflow tract obstruction.

Patients with atrioventricular septal defects are morphologically predisposed to subaortic obstruction. Some individuals require secondary left atrioventricular valve replacement for severe incompetence persisting after repair, and they are especially susceptible to left ventricular outflow tract obstruction. A surgical technique is described for replacement of the atrioventricular valve which seems to avoid the complication of left ventricular outflow tract obstruction by the prosthesis.

Repair of congenital pulmonary venous stenosis with living autologous atrial tissue.

Repair of localized bilateral congenital pulmonary venous stenosis with living autologous atrial tissue was accomplished in two patients, 3 and 4 months of age. The tip of the left atrial appendage was opened and used as an onlay patch to widen the stenotic area of the left pulmonary veins, leaving a double pathway for venous return. A flap of interatrial septum was evaginated through an incision in the right side of the left atrium and used as an onlay patch to widen the stenotic area of the right pulmonary veins.