Patricia M. Clarkson

Results after repair of coarctation of the aorta beyond infancy: A 10 to 28 year follow-up with particular reference to late systemic hypertension☆

The late outcome in 160 patients aged 1 to 54 years who had surgical repair of coarctation of the aorta was examined 10 to 28 years postoperatively. Twenty years postoperatively the probability of survival of patients discharged from the hospital aged 1 to 19 years at operation was a little less than that of the general population (95% versus 97%). The discrepancy between patients and the general population was more marked in those aged 20 to 39 years and was grossly different when surgical repair was undertaken beyond age 40. There were 19 late deaths (12%), 79% due to cardiovascular disease. Thirteen patients had a poor result because of recoarctation (11 patients) or the development of complications at the site of repair (2 patients). Most patients were hypertensive before operation. The frequency of hypertension decreased markedly in the first few postoperative years. Blood pressure was normal in most patients 5 to 10 years after operation, but when followed up for longer periods the proportion of patients with hypertension increased. Hypertension was more common in patients operated on after 20 years of age than in those aged 5 to 19 years at operation (p = 0.007). The likelihood of being alive without complications and with a normal blood pressure was 69% at 10 years, 55% at 15 years, and 20% at 25 years postoperatively.

Long-term outcome after the mustard repair for simple transposition of the great arteries: 28-year follow-up

OBJECTIVES This study examines the late outcome in patients with simple transposition of the great arteries (TGA) after a Mustard operation. BACKGROUND Continuing medical follow-up for patients after the Mustard procedure, now extending to three decades, is required. The quality of life of adult survivors has not been well documented. METHODS Survival and quality of life among 113 hospital survivors of the Mustard operation performed for simple TGA between 1964 and 1982 were assessed by medical review and a lifestyle questionnaire. The incidence of right ventricular failure and echocardiographic right ventricular dysfunction (RVD) were determined. A measure of lifestyle, the ability index, was determined. RESULTS Actuarial survival was 90%, 80%, and 80% at 10, 20, and 28 years, respectively, with 76% of survivors being New York Heart Association class 1. Sudden death, with an incidence of 7% without identifiable risk factors, was the most common cause of late demise. RVD was identified in 18% of patients who had echocardiography, but there was right ventricular failure in only two patients. Seventy-five percent of current survivors lead a normal life, 20% have some symptoms or lifestyle modification, and 5% are unable to work. CONCLUSIONS The survival of patients to 28 years with the Mustard repair has been good. Late sudden death is the most worrisome feature. There is a 97% freedom from right ventricular failure to date. The quality of life of late survivors is good, most achieving a normal level of education and employment.

Outcome of pregnancy after the mustard operation for transposition of the great arteries with intact ventricular septum

OBJECTIVES This study examined the outcome of pregnancy in patients with transposition of the great arteries and an intact ventricular septum after a Mustard operation. BACKGROUND Before the introduction of surgical treatment, most children with transposition of the great arteries died in early infancy. A number of these patients have now reached their reproductive years. There is little information about the effect of pregnancy on cardiovascular status, particularly the ability of the right ventricle to adjust to the hemodynamic changes of pregnancy. The outcome for the offspring and their risk of congenital heart disease are also unknown. METHODS Twenty-three female late survivors after the Mustard operation > 15 years of age were reviewed in relation to the occurrence of pregnancy and its outcome. Serial echocardiographic estimates of right ventricular volume during pregnancy were made in three local patients. RESULTS Nine women had 15 pregnancies. They were asymptomatic before pregnancy and remained free from cardiac symptoms during each pregnancy. Right ventricular volume in the three patients studied increased during pregnancy but returned to normal at 8 to 11 weeks postpartum. There were 12 live births, 2 spontaneous abortions and 1 intrauterine death. None of the liveborn infants had evidence of congenital heart disease. CONCLUSIONS In this small group of women with good quality late survival after a Mustard operation, pregnancy was well tolerated. We suspect that the incidence of congenital heart disease in infants of mothers with transposition of the great arteries will be at the lower end of the range for mothers with different types of congenital heart disease, but further data will be needed to confirm this.

Prosthetic repair of coarctation of the aorta with particular reference to Dacron onlay patch grafts and late aneurysm formation.

Prosthetic material was used in the repair of coarctation of the aorta in 72 patients followed for 8 months to 24 years after operation. False aneurysms occurred late postoperatively in 1 of 17 instances when a tube graft was used to replace the resected aorta and in 1 of 2 patients when the graft bypassed the obstructed area. A Dacron onlay patch graft (DOPG) was used in 52 patients as the primary method of repair, to revise a previous repair or as an adjunct to another procedure at the time of initial operation. True or false late aneurysm occurred between 6 and 18 years postoperatively in 5 of the 38 patients in whom a DOPG was used for primary repair of the coarctation or to revise an earlier repair, an actuarial incidence of 38% at 14 years. Diagnosis of aneurysm formation late postoperatively depends on awareness of this complication and careful assessment of lateral as well as posteroanterior chest radiographs. DOPG should be avoided whenever possible.

Developmental progress after cardiac surgery in infancy using hypothermia and circulatory arrest.

Seventy-two of 76 long-term survivors who had surgical repair of congenital heart lesions at 11 days to 26 months of age using profound hypothermia and circulatory arrest underwent psychometric testing (Stanford-Binet) late postoperatively. The mean IQ of the 72 patients was 92.9 ± 16.5 (SD). Stanford-Binet scores bore no relationship to the duration of circulatory arrest or other aspects of surgical technique. Scores were significantly lower in those who had a low birth weight for gestational age, important neurologic problems preoperatively or were in the lower socioeconomic classes. An “ideal” control group of 69 children randomly selected from patients satisfying certain criteria based on birth and neonatal characteristics had a mean IQ of 106.2 ± 11.6. Twenty-five patients who had surgical treatment of congenital heart disease met the criteria for the control group except for their heart lesions. Their mean IQ was 101.4 ± 15.0 (NS). We could not demonstrate any significant deleterious effect that could be attributed to the surgical methods. Rather, the postoperative IQ scores reflected characteristics related to individual patients.

The pulmonary vascular bed in patients with complete transposition of the great arteries.

Histological material obtained at autopsy from 35 patients over three months of age with complete transposition of the great arteries (TGA) was examined. Two of six patients less than a year of age with an intact ventricular septum and closed ductus arteriosus were found to have pulmonary vascular changes of at least grade 3 severity; in addition, two of seven patients with a large ventricular septal defect in this age group showed changes of similar severity. The reported low incidence of marked pulmonary vascular changes in patients with only an interatrial communication dying during the first year of life would appear to be due in part to the high proportion of cases less than three months old in whom there was not sufficient time for such changes to develop. Although the incidence is not as high as that found beyond a year of age, it is sufficiently high to influence the management of infants beyond three months of age. Hemodynamic studies were performed following atrial baffle repair in 34 patients. All but one of the 16 patients operated upon prior to two years of age had a pulmonary arteriolar resistance (Rpa) less than 3 units M2 at the time of review, including three with a large communication at ventricular or great vessel level who underwent surgery before two months of age. Repair at an older age did not preclude a low Rpa on follow-up, but in 11 of the 18 patients who were two or more years of age at the time of repair it was greater than 3 units m2. None of these latter patients had a large ventricular septal defect or patent ductus arteriosus.Comparison of postoperative hemodynamic data with autopsy studies would suggest that advanced pulmonary vascular disease is associated with earlier death among patients with complete transposition of the great arteries.

Predictors of long-term survival with Ebstein's anomaly of the tricuspid valve

In patients with Ebsteins anomaly of the tricuspid valve, predictors of death and indications for surgery are poorly defined. We retrospectively reviewed 48 patients with Ebsteins anomaly, 17 (35%) of whom presented in the first week of life. Duration of follow-up extended to 32 years (greater than 10 years in 35%). Twenty of the 48 patients (42%) died, 6 in the first week of life and 1 at age 5 months. Thirteen of the 41 patients surviving to age 6 months subsequently died, 50% probability of survival being reached at 47 years. Significant (p less than or equal to 0.05) predictors of death in this group were: male sex, cardiothoracic ratio greater than or equal to 0.65, New York Heart Association class III or IV, breathlessness and the absence of Wolff-Parkinson-White syndrome. Eight patients died suddenly. A cardiothoracic ratio greater than or equal to 0.65 was a better predictor of sudden death than functional status. All who developed atrial fibrillation died within 5 years. Other atrial arrhythmias were not helpful in predicting sudden death. In view of these findings, tricuspid valve surgery is recommended before the cardiothoracic ratio reaches 0.65, regardless of the symptomatic state.

Bacterial Endocarditis Following Homograft Replacement of the Aortic Valve

Endocarditis was encountered on an aortic homograft valve in 14 of 539 patients, giving a total incidence of 2.6%. It was an early postoperative complication in five patients (0.9%), and only one of these survived. Four hundred and six hospital survivors were followed for periods up to 76 mo. Nine of these patients (2.2%) developed infection of the homograft valve 11 to 66 mo postoperatively and four survived. Late endocarditis occurred more commonly in patients with a history of endocarditis prior to operation (P=0.005). In only one of the nine patients with late endocarditis was the infection related to a peripheral leak around the graft. Most patients with late endocarditis rapidly developed severe homograft valve incompetence, which was the cause of death in four of the nine. Six patients were reoperated on and three of these survived.

Assessment and follow-up of patients with ventricular septal defect and elevated pulmonary vascular resistance

Cardiac catheterization was undertaken in 87 patients (for a total of 89 studies) with ventricular septal defects, including 58 patients with moderate or severe elevation of pulmonary arteriolar resistance. When resting resistance was less than or equal to 7.9 U . m2, it always decreased with isoproterenol and no postoperative problems were experienced with pulmonary vascular obstructive disease. In 36 patients resting resistance measured greater than or equal to 8 U . m2. In 17 of these patients it decreased to less than 7 U . m2 with isoproterenol. Fifteen patients were operated on and postoperative problems with pulmonary vascular disease were experienced only in the single patient whose repair broke down. Surgery was undertaken in 4 of 19 patients in whom resistance did not decrease to less than 7 U . m2 with isoproterenol and advanced pulmonary vascular disease was evident in the 3 patients with follow-up observation. Correlation between measured resistance and other hemodynamic parameters was only fair. A pulmonary to systemic resistance ratio greater than or equal to 0.75 always indicated high absolute resistance but resistance ratios less than 0.75 were found quite frequently in the group with limited response to isoproterenol. These data argue that a reliable estimate of resistance, less than 7 U . m2, with a vasodilator predicts a good postoperative response regardless of measurements at rest or other hemodynamic parameters. Although observations on postoperative progress of patients with resistance greater than 7 U . m2 with a vasodilator are limited, a good postoperative course is unlikely unless resistance can be lowered to a level close to 7 U . m2.

Late postoperative hemodynamic results and cineangiocardiographic findings after Mustard atrial baffle repair for transposition of the great arteries.

Forty-nine patients with transposition of the great arteries who underwent a Mustard atrial baffle repair between 1964 and 1971 were assessed late postoperatively. There have been five late deaths: two related to baffle obstruction, two from noncardiac causes, and one sudden and unexpected. Hemodynamic data were available in 42 patients and autopsy in four. Obstruction of the lower venous channel was not encountered. Three patients had severe obstruction of the upper venous channel and in four there was mild restriction. Two patients had severe pulmonary venous obstruction resulting in late death; mild asymptomatic obstruction could not be excluded with certainty in six patients. Tricuspid incompetence was infrequently encountered in patients with an essentially intact ventricular septum. Left ventricular outflow tract obstruction was found in eight patients. In three it was present after satisfactory but incomplete surgical relief and in five it had not been recognized prior to operation. In only one of the latter patients was the obstruction important.Seventeen patients were operated on prior to one year of age. While baffle obstruction was confined almost entirely to these patients, the five youngest patients, aged one to nine weeks at operation, had adequate pulmonary and caval compartments at restudy two to three years later.