Long-term outcome in patients undergoing surgical repair of tetralogy of Fallot.

BACKGROUND: Although corrective surgery for tetralogy of Fallot has been available for more than 30 years, the occurrence of late sudden death in patients in whom surgery was apparently successful remains worrisome. METHODS: We studied long-term survival among 163 patients who survived 30 days after complete repair of tetralogy of Fallot, examining follow-up hospital records and death certificates when relevant. RESULTS: The overall 32-year actuarial survival rate among all patients who survived surgery was 86 percent, as compared with an expected rate of 96 percent in a control population matched for age and sex (P < 0.01). Thirty-year actuarial survival rates were calculated for the patient subgroups. The survival rates among patients less than 5 years old, 5 to 7 years old, and 8 to 11 years old were 90, 93, and 91 percent, respectively--slightly less than the expected rates (P < 0.001, P = 0.06, and P = 0.02). Among patients 12 years old or older at the time of surgery, the survival rate was 76 percent, as compared with an expected rate of 93 percent (P < 0.001). The performance of a palliative Blalock-Taussig shunt procedure before repair, unlike the performance of a Waterston or Potts shunt procedure, was not associated with reduced long-term survival, nor was the need for a trans-annular patch at the time of surgery. Independent predictors of long-term survival were older age at operation (P = 0.02) and a higher ratio of right ventricular to left ventricular systolic pressure after surgery (P = 0.008). Late sudden death from cardiac causes occurred in 10 patients during the 32-year period. CONCLUSIONS: Among patients with surgically repaired tetralogy of Fallot, the rate of long-term survival after the postoperative period is excellent but remains lower than that in the general population. The risk of late sudden death is small.

Long-term outcome after surgical repair of isolated atrial septal defect. Follow-up at 27 to 32 years.

BACKGROUND: Atrial septal defects have been surgically correctable for more than 30 years. The long-term survival rates among patients treated in the early era of cardiac surgery are poorly documented, but such data are of critical importance to the future medical care, employability, and insurability of these patients. METHODS: To determine the natural history of surgically corrected atrial septal defects, we studied all 123 patients who underwent repair of an isolated defect (ostium secundum or sinus venosus) at the Mayo Clinic between 1956 and 1960, 27 to 32 years after the procedure. The follow-up status of all patients was determined by written questionnaires and telephone interviews. Hospital records and death certificates were obtained if interim hospitalization or death had occurred. RESULTS: The overall 30-year actuarial survival rate among survivors of the perioperative period was 74 percent, as compared with 85 percent among controls matched for age and sex. The perioperative mortality was 3.3 percent (four deaths). Actuarial 27-year survival rates among patients in the younger two quartiles according to age at operation (less than or equal to 11 years and 12 to 24 years) were no different from rates among controls--97 percent and 93 percent, respectively. In the two older quartiles (25 to 41 years and greater than 41 years), 27-year survival rates were significantly less (P less than 0.001)--84 percent and 40 percent, respectively--than in controls (91 and 59 percent). Independent predictors of long-term survival according to multivariate analysis were age at operation (P less than 0.0001) and systolic pressure in the main pulmonary artery before operation (P less than 0.0027). When repair was performed in older patients, late cardiac failure, stroke, and atrial fibrillation were significantly more frequent. CONCLUSIONS: Among patients with surgically repaired atrial septal defects, those operated on before the age of 25 have an excellent prognosis, but older patients require careful, regular supervision.

The potential contribution of cardiac replacement to the control of cardiovascular diseases. A population-based estimate.

The potential number of individuals who might benefit from a cardiac replacement procedure (either cardiac transplantation or insertion of a total artificial heart) was retrospectively estimated from medical records for residents of Olmsted County, Minnesota, who had died during a 5-year period. Residents were divided into two age groups: those younger than 15 years (pediatric) and those 15 to 69 years (adult). During the 5-year period of observation, cardiac disease led to death in 17 of the 8342 live births in Olmsted County. Cardiac disease also caused the deaths of 248 adults meeting the age criteria. Five children and 35 adults met all criteria for cardiac replacement. Extrapolation to the total population of the United States suggests that 2167 children (a 95% confidence interval of 361 to 3972) and 16,500 adults (a 95% confidence interval of 11,456 to 22,959) per year could potentially benefit from cardiac replacement.

Sudden death after repair of double-outlet right ventricle.

The outlook for patients with double-outlet right ventricle has improved since the development of corrective operations. Late arrhythmic deaths after successful procedures have been reported; however, the magnitude remains unknown. This study was undertaken to identify the magnitude of late sudden death and the significant factors associated with it. From 1965 through 1985, 118 patients underwent corrective operation for double-outlet right ventricle; of these, 23 died in the hospital and six were lost to follow-up. The 89 remaining patients (52 male and 37 female) made up the study population. Their mean age (+/- SD) was 10.3 +/- 7.8 years at the time of repair. The mean duration of follow-up was 82 months. Of the 22 late deaths, 16 (73%) were sudden. Eight (50%) of the sudden deaths occurred within 1 year of operation. Cox proportional hazards multivariate analysis revealed the following significant risk factors for late sudden death: older age at the time of operation, perioperative or postoperative ventricular tachyarrhythmias, and third-degree atrioventricular block. Factors not associated with late sudden death included year of operation, sex, type and number of associated cardiac anomalies, preoperative functional class, previous palliative procedures, surgical technique, perioperative or postoperative single premature ventricular contractions, and postoperative left or right bundle branch block with or without fascicular block. We conclude that the incidence of late sudden death after successful surgical repair of double-outlet right ventricle is very high. Complete corrective operation at an early age and aggressive diagnosis and treatment of arrhythmias and conduction defects after operation are warranted.

Coarctation of the aorta. Long-term follow-up and prediction of outcome after surgical correction.

The long-term clinical course was studied in 646 patients, who underwent isolated operative repair of coarctation of the aorta at the Mayo Clinic from 1946 to 1981. There were 17 perioperative deaths, and 58 patients were lost to follow-up. Of the 571 patients with long-term follow-up, 11% required subsequent cardiovascular surgery, and 25% developed hypertension. There were 87 late deaths. The mean age at death was 38 years (range, 0-67 years). Estimated survival analysis revealed 91% of patients alive at 10, 84% at 20, and 72% at 30 years after operative repair. The most common cause of late death was coronary artery disease in 32 patients, followed by sudden death, heart failure, cerebrovascular accidents, and ruptured aortic aneurysm. Age, sex, and postoperative systolic blood pressure were found to be independently predictive of survival. For patients less than 14 years of age at the time of initial coarctectomy, survival to 20 years was 91%, and for patients 14 years or older at the time of operation, survival was 79%. The best survivorship was observed in patients operated on at 9 years of age or less. The higher the postoperative systolic pressure, the higher the probability of death. This study has the largest population undergoing repair of coarctation of the aorta with a median follow-up of as long as 20 years. Four main points emerged. 1) Age at the time of initial repair is the most important predictor of long-term survival. Surgery should be offered to patients after age 1 year or sooner if hypertension is severe. 2) Coronary artery disease is the most common cause of late death.(ABSTRACT TRUNCATED AT 250 WORDS)

Long-term outcome of patients undergoing surgical repair of isolated pulmonary valve stenosis. Follow-up at 20-30 years.

In a group of 191 consecutive patients who underwent operation at the Mayo Clinic for isolated pulmonary valve stenosis between 1956 and 1967, late results were excellent. In patients surviving operation before the age of 21 years, long-term survival was similar to that in an age- and sex-matched control population. In the other patients, late survival and functional status were good, but survival was poorer than in the control population. This late attrition, most likely due to the sequelae of long-standing right ventricular hypertrophy, suggests the need for continued surveillance. Late sudden death occurred in only three patients, between 10 and 21 years postoperatively. The finding of normal life expectancy for such patients who survive operation before age 21 is important for actuarial purposes and for the patients' career and employment plans.

Modified Fontan procedure for biventricular hearts with complex forms of double-outlet right ventricle.

The surgical management of patients with biventricular hearts and complex double-outlet right ventricle with noncommitted ventricular septal defect, hypoplastic ventricles, common atrioventricular orifice, straddling tricuspid valve, and straddling mitral valves is controversial. More recently at our institution, 23 of these patients underwent Fontan-type procedures as an alternative to the intraventricular tunnel repair. In all cases, the presence of more than one associated anomaly would have prevented adequate biventricular correction. The ventricular septal defects were of the inlet type in 14 patients, inlet and subpulmonary in one, multiple in one, subpulmonary in three, and subaortic in four. Twenty-one patients had atrioventricular junction anomalies, and 14 had hypoplastic ventricles (left ventricle in 11 and right ventricle in three). Pulmonary stenosis was present in 13 patients and pulmonary atresia in two. At operation, an extracardiac conduit was used in two patients and an atriopulmonary direct anastomosis in 21. There were six hospital deaths (26%). Seventeen patients had a mean follow-up period of 25 months. At the follow-up evaluation, nine patients were assigned to New York Heart Association Class I and seven to Class II; one patient assigned to Class IV died at 9 months postoperatively. Cumulative mortality was 30.4% (seven deaths). Univariate analysis identified hypoplastic left ventricle with any type of ventricular septal defect and atrioventricular junction as the only incremental risk factor for early and late outcome (p less than 0.01), which also influenced functional status and use of drug therapy (p less than 0.01) at the time of follow-up.(ABSTRACT TRUNCATED AT 250 WORDS)

Palliative transposition of venous return. Long-term follow-up.

A palliative operation to create intraatrial transposition of venous return without concomitant closure of an associated ventricular septal defect (palliative Mustard or Senning operation) has been used primarily for patients with a combination of transposition of the great arteries, ventricular septal defect, and pulmonary vascular obstructive disease. We evaluated the results in all 15 patients (median age 13 years) who had this procedure at the Mayo Clinic between 1973 and 1979. There were two (13%) operative deaths. One patient was lost to long-term follow-up. Of the 12 operative survivors for whom follow-up data were available, 11 reported subjective improvement in exercise tolerance and activity level. There were seven late deaths: six from cardiovascular problems and one from an accident. There are five survivors currently alive (average 10.8 years after operation). Patients who survived the operation for more than 5 years had a significantly (p less than 0.05) lower pulmonary resistance preoperatively than those who survived less than 5 years. The hemoglobin level decreased from 21.1 gm/dl before operation to 18.4 gm/dl, and arterial saturation was increased from 64% to 85% at an average of 9 years after operation. We conclude that this operation produces subjective improvement and lessens hypoxemia and polycythemia. In some patients, palliation may be achieved for 10 years or longer.

Isolated atrial septal defect with pulmonary vascular obstructive disease--long-term follow-up and prediction of outcome after surgical correction.

UNLABELLED: We examined the cases of 702 patients found to have isolated atrial septal defect of the secundum or sinus venosus type at catheterization from 1953 to 1978. Forty patients (6%), 34 women and six men, had pulmonary vascular obstructive disease, with a total pulmonary resistance greater than 7 U/m2; of these patients 26 (mean age 47 years) underwent surgical closure and 14 (mean age 44 years) received medical treatment. All patients were followed for at least 4 years, with a median follow-up of 12 years. At the most recent follow-up, 17 of the 40 patients were dead. Of the 22 surgically treated patients with total pulmonary resistance less than 15 U/m2, 19 were alive with significant regression of symptoms. All four surgically treated patients with total pulmonary resistance greater than or equal to 15 U/m2 were dead. Of the five medically treated patients with total pulmonary resistance less than 15 U/m2, four had died, and one was alive with significant progression of symptoms. Of the nine medically treated patients with total pulmonary resistance greater than or equal to 15 U/m2, six had died and the three survivors had progression of symptoms. In the surgically treated group, the following variables correlated with survival: total pulmonary resistance (p less than .00001), pulmonary arteriolar resistance (p less than .00001), pulmonary-to-systemic resistance ratio (p = .004), systemic arterial oxygen saturation (p = .005), and pulmonary arterial oxygen saturation (p = .007). IN CONCLUSION: (1) Atrial septal defect with high total pulmonary resistance is uncommon and predominates in adult female patients.(ABSTRACT TRUNCATED AT 250 WORDS)

Definitive operation for pulmonary atresia with intact ventricular septum. Results in twenty patients.

Definitive operation was performed on 20 patients (aged 25 to 178 months) with pulmonary atresia and intact ventricular septum. All patients had one or more prior preliminary palliative procedures. Right ventricular outflow tract reconstruction with atrial septal defect closure and shunt removal was done on 10 patients. Tricuspid annular circumference was at least 70% of normal in seven patients and between 55% and 70% in three patients. Two patients died during hospitalization. The eight surviving patients were asymptomatic 3 to 145 months after operation. The modified Fontan operation was performed on 10 patients. None of these patients had a tricuspid annular circumference greater than 70% of normal; the circumference was less than 55% in nine patients and between 55% and 70% in one patient. One patient died during hospitalization and one died later. Follow-up 6 to 48 months after operation showed that six patients were in the New York Heart Association Class I and two were in Class II. After effective preliminary palliation of pulmonary atresia with intact ventricular septum, definitive operation can be done with an operative risk of 15% (three of 20 patients) and excellent late results. Right ventricular outflow tract reconstruction can be done as a complete repair for patients who have adequate tricuspid annular size. The modified Fontan operation is the only option for definitive repair when the tricuspid anulus is severely hypoplastic.

Artificial heart and assist devices: directions, needs, costs, societal and ethical issues.

A Working Group appointed by the Director of the National Heart, Lung, and Blood Institute (NHBLI) has reviewed the current status of mechanical circulatory support systems (MCSS), and has examined the potential need for such devices, their cost, and certain societal and ethical issues related to their use. The media have reported the limited clinical investigative use of pneumatically energized total artificial hearts (which actually replace the patient's heart) and left ventricular assist devices (which support or replace the function of the left ventricle by pumping blood from the left heart to the aorta with the patient's heart in place). However, electrically energized systems, which will allow full implantation, permit relatively normal everyday activity, and involve battery exchange or recharge two or three times a day, are currently approaching long-term validation in animals prior to clinical testing. Such long-term left ventricular assist devices have been the primary goal of the NHLBI targeted artificial heart program. Although the ventricular assist device is regarded as an important step in the sequence of MCSS development, the Working Group believes that a fully implantable, long-term, total artificial heart will be a clinical necessity and recommends that the mission of the targeted program include the development of such systems. Past estimates of the potential usage of artificial hearts have been reviewed in the context of advances in medical care and in the prevention of cardiovascular disease. In addition, a retrospective analysis of needs was carried out within a defined population. The resulting projection of 17,000-35,000 cases annually, in patients below age 70, falls within the general range of earlier estimates, but is highly sensitive to many variables. In the absence of an actual base of data and experience with MCSS, projection of costs and prognoses was carried out using explicit sets of assumptions. The total cost of a left ventricular assist device, its implantation and maintenance for a projected average of 4 1/2 years of survival might be approximately $150,000 (in 1983 dollars). The gross annual cost to society could fall in the range of $2.5-$5 billion. Ethical issues associated with use of the artificial heart are not unique. For individual patients these relate primarily to risk-benefit, informed consent, patient selection, and privacy. However, for society as a whole, the larger concern relates to the distribution of national resources.(ABSTRACT TRUNCATED AT 400 WORDS)

Outcome of the Fontan procedure in patients with tricuspid atresia.

Between 1973 and 1983, 90 patients with tricuspid atresia underwent the Fontan operation at the Mayo Clinic. Operative mortality was 12% overall and decreased to 7% during the second 5 year period. At postoperative follow-up of 65 patients (1 to 10 years, mean 4), 50 (86%) of 58 present survivors were in excellent condition. There had been seven late deaths. Catheterization 0.5 to 8 years (mean 2.7) postoperatively in 25 patients disclosed a mean right atrial pressure of 14 mm Hg (range 6 to 21) and a mean cardiac index at rest of 3.0 (range 1.8 to 4.2). Mean left ventricular end-diastolic pressure was 8.5 mm Hg postoperatively compared with 16.5 mm Hg preoperatively; the decrease was secondary to a decreased ventricular volume load (mean volume load index 10.2 preoperatively, 3.0 postoperatively). We recommend that patients with tricuspid atresia have the Fontan operation by age 6 years to protect myocardial function.

Results of triple valve replacement in 91 patients: perioperative mortality and long-term follow-up.

Between 1961 and 1984, 91 patients underwent simultaneous triple valve replacement at the Mayo Clinic. Of the 273 prosthetic valves used, 77% were Starr-Edwards. Perioperative (30 day) mortality was 24% to 27% between 1962 and 1974 and 7% between 1975 and 1983 (p = .17). In patients with NYHA class IV symptoms, perioperative mortality was 44%, and in those with milder symptoms, it was 8% (p less than .0001). The median follow-up was 7.5 years (range, 6 weeks to 20 years). Cumulative survival, which was calculated taking into consideration perioperative mortality, was 64% at 1 year, 55% at 5 years, 40% at 10 years, and 25% at 15 years. Multivariate analysis identified preoperative functional class and age as predictors of late survival. Among causes of late mortality were sudden death in 32.5%, congestive heart failure in 15%, thromboembolism in 12.5%, prosthetic valve dysfunction in 7.5%, and infective endocarditis in 5%. Late complications included systemic emboli in 42% (embolic rate, 12.3 events per 100 patient-years), bleeding in 22%, myocardial infarction in 16%, and infective endocarditis in 6%. Eight patients required reoperation for prosthetic valve dysfunction, and 12 patients had permanent pacemakers. Of the 29 patients still alive, 79% are in NYHA class I or II. In summary, perioperative mortality after triple valve replacement appears to be declining; long-term survival in 30 day survivors is similar to that after single valve replacement and excellent symptomatic improvement can be obtained, although morbidity is high.

Truncus arteriosus with single pulmonary artery: influence of pulmonary vascular obstructive disease on early and late operative results.

Nineteen patients with truncus arteriosus and single pulmonary artery had corrective operations at the Mayo Clinic from 1969 to 1983. At operation, their ages ranged from 4 months to 20 years (mean 8.1 years). The preoperative pulmonary resistance divided by 2 was used to predict the degree of pulmonary vascular obstructive disease at operation. The influence of elevated pulmonary resistance and the intraoperative postrepair ratio of pulmonary artery to left ventricular pressure on operative and late mortality were examined. The outcome of patients with single pulmonary artery was compared with the outcome of 148 patients with truncus arteriosus and two pulmonary arteries operated on during the same period. Patients with a single pulmonary artery had an operative mortality similar to that of patients with two pulmonary arteries (32 versus 28%, p greater than 0.05). Late mortality was, however, significantly greater (p less than 0.001) for patients with a single pulmonary artery. Elevated ratios of intraoperative postrepair pulmonary artery to left ventricular pressure were associated with significantly higher (p less than 0.02) operative and late mortality, but elevated preoperative pulmonary resistance was not (p greater than 0.10). Truncus arteriosus with single pulmonary artery is associated with poor postoperative survival, and although elevated pulmonary resistances preoperatively did not predict outcome, elevated intraoperative postrepair pulmonary artery to left ventricular pressure ratios were associated with increased operative and late survival, suggesting a deleterious role of pulmonary hypertension.