Aortic and mitral valve incompetence: long-term follow-up (10 to 19 years) of patients treated with the Starr-Edwards prosthesis.

The long-term course (mean 15 years) of 336 patients with valvular incompetence who underwent Starr-Edwards ball valve implantation between 1962 and 1971 was reviewed. Eighteen patients (10%) with aortic valve replacement and 24 (16%) with mitral valve replacement died early postoperatively. Mortality remained high (31%) in the first 3 years after aortic valve replacement; it was highest (13%) in the first year after mitral valve replacement and then approached the normal rate. The most common mode of death was sudden death after aortic and cardiac failure after mitral valve replacement. At follow-up, 76% of survivors had improved symptomatically. Three instances of primary valve malfunction occurred. The probability of freedom from thromboembolism at 15 years postoperatively was 56% for aortic valve replacement and 52% for mitral valve replacement. The Starr-Edwards valve prosthesis is durable over prolonged follow-up period, but thromboembolism remains a persistent problem. Survival may be normal for patients surviving the early postoperative years.

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.

Surgical repair of univentricular heart (double inlet left ventricle) with obstructed anterior subaortic outlet chamber.

The results of operation in all patients with univentricular heart and an obstructed anterior subaortic outlet chamber who were operated on utilizing extracorporeal circulation at the Mayo Clinic from 1973 through 1983 were reviewed. Ten of the 18 patients died during the immediate postoperative period and there was one late death. Factors significantly related to operative and immediate postoperative mortality were age at operation, cardiothoracic ratio on X-ray examination, degree of ST depression on electrocardiogram and pressure gradient across the outlet foramen at catheterization. Autopsy in eight cases revealed significant hypertrophy of ventricular myocardium and a small outlet foramen that was considered stenotic relative to either body surface area or aortic root area. The ventricular myocardium showed histologic changes of chronic ischemia that predated the surgical procedure.

Long-term effects of prosthetic materials.

The surgical repair of the various types of congenital cardiac defects often requires the use of prosthetic materials, both bioprosthetic and synthetic. Patches have proved highly effective in many applications and locations. All prosthetic valves have significant limitations, and particularly disappointing was the observation that bioprosthetic valves undergo accelerated degeneration in younger patients. The extracardiac conduit allowed corrective operation for several complex types of anomalies, but it suffers the double jeopardy of premature degeneration of its integral tissue valve (if any), and progressive peel formation within its lumen. A threat of infection exists in the presence of prosthetic materials, especially valves. These known limitations of prosthetic devices as well as possible potential late complications not yet fully elucidated are sufficient indication for periodic, perpetual surveillance of patients who have received cardiac prostheses.

Surgical correction of atrioventricular canal during the first year of life.

Eight infants less than 1 year of age with an atrioventricular (A-V) canal defect (five with the complete form and three with the partial form) had definitive surgical correction performed because of intractable congestive heart failure. At operation, the infants weighed 2.9 to 6.5 kg. There was one operative death and one late death. The six surviving children were all doing well when followed up 10 months to more than 4 years postoperatively. This small series demonstrates that complete connection of A-V canal is possible even in very small infants and that chances of success are excellent. The infant with this defect who is not progressing with medical management should undergo definitive surgical correction, and a strong argument can be made for elective complete repair in all patients with this lesion, who have severe pulmonary hypertension, during the first 1 or 2 years of life.

Surgical significance of the coronary arterial anatomy in truncus arteriosus communis.

In truncus arteriosus communis, as in other anomalies of conotruncal development, the coronary arterial anatomy not only differs from that found in the normal heart but also is subject to unpredictable variations. A consistently distinctive pattern was found in roughly two thirds of 31 cases, a frequency approximating that with which a distinctive coronary arterial pattern occurs in complete transposition of the great vessels. Surgical injury to a major coronary artery was identified in 2 of the 30 hearts from patients with truncus arteriosus communis who died in the early postoperative period. Of particular significance are large branches of the right coronary artery crossing the upper anterior surface of the right ventricle to supply the anterobasal surface of both ventricles and the upper part of the interventricular septum. These arteries are at special risk in surgical procedures utilizing a conduit anastomosed to a right ventriculotomy.

Partial atrioventricular canal defect in elderly patients (aged 60 years or older).

Partial atrioventricular canal defect in elderly patients (aged 60 years or older) is extremely rare, and surgical results in this select group have not been reported. This report describes in detail the clinical profile of six such patients and the surgical results in four of the six. There was no operative mortality. This experience, albeit based on small numbers, suggests that operation can be performed at low risk with gratifying symptomatic improvement and that surgical repair in symptomatic elderly patients with partial atrioventricular canal detect can be justified.

Long-term evaluation (12 to 22 years) of open heart surgery for tetralogy of Fallot.

Four hundred seventy-five patients underwent repair of tetralogy of Fallot from 1955 to 1964; 396 of these were hospital survivors and were followed up for 12 to 22 years. An excellent late clinical result was maintained by 87 percent of the 396 hospital survivors. A less than excellent result in the remaining 13 percent of hospital survivors was caused by late mortality in 7 percent (sudden death in 3 percent, death due to cardiac causes in 2 percnt and death due to noncardiac causes in 2 percent), required reoperation in 4 percent (mainly because of residual ventricular septal defect) and development of symptoms in 2 percent. Postoperative cardiomegaly (cardiothoracic ratio greater than 0.55) was observed in 60 (25 percent) of 246 patients who had a follow-up chest roentgenogram, and was more common among those who died late or remained symptomatic. Among the few patients with inadequate surgical relief of right ventricular hypertension who did not have transanular patch repair, the hypertension did not tend to decrease progressively, whereas it did decrease in patients who had patch repair. No late sudden deaths were encountered in 20 patients shown to have postoperative right bundle branch block plus left axis deviation (bifascicular block pattern). Pulmonary valve incompetence appeared to have relatively little harmful influence on the late result, causng cardiac disability in 1 percent of the patients and appeared to be the main contributing factor of postoperative cardiomegaly in 13 (5 percent) of the 246 patients who had a follow-up chest roentgenogram. Most late deaths and complicatins appeared within 2 years of operation, and accelerating deterioration in late results did not occur as the follow-up extended beyond 2 decades.

Clinical follow-up study of paroxysmal supraventricular tachyarrhythmias after operative repair of a secundum type atrial septal defect in adults.

Adults with isolated secundum atrial septal defect (ASD) may present with paroxysmal atrial tachyarrhythmias. The clinical course of these rhythmic disturbances after correction of the ASD is unknown. Of 188 patients aged 44 years old or older with isolated ASD, 27 (14%) (mean age 52.7 years, range 44 to 71) had documented paroxysmal atrial tachyarrhythmias preoperatively: 16 had paroxysmal atrial fibrillation, 2 had atrial flutter, and 9 had supraventricular tachycardia. Among these 3 groups, there were no differences in age, sex, New York Heart Association class, duration of preoperative symptoms, mean right atrial and pulmonary arterial pressures, shunt size, atrial size, or follow-up period. After operative repair, follow-up data were available in all patients for a mean of 12 years (range 1.5 to 25). Of the 16 patients with preoperative paroxysmal atrial fibrillation, 14 (88%) continued to have increasingly frequent paroxysmal atrial fibrillation, culminating in sustained atrial fibrillation. In 1 of the 2 patients with paroxysmal atrial flutter, sustained atrial fibrillation developed. Of the 9 patients with preoperative paroxysmal supraventricular tachycardia, 5 had no further episodes of this arrhythmia postoperatively.

Long-term endocardial atrial pacing in children with postoperative bradycardia-tachycardia syndrome and limited ventricular access.

Permanent pacing in children, including those with postoperative bradycardia-tachycardia syndrome has been compromised by the availability of pulse generators, electrode leads and implantation techniques designed for the adult patient. Recent technologic improvements and simplified implantation techniques have reduced many of these barriers and have made endocardial as well as epicardial ventricular pacing more feasible. However, in some children, ventricular pacing may be impeded by anatomic abnormalities due to congenital anomalies or prior cardiac operations. In these instances, endocardial atrial pacing may provide an alternative therapeutic approach in selected patients. This report describes the use of endocardial atrial demand pacing in four children with postoperatively bradycardia-tachycardia syndrome and restricted ventricular access. This approach controls symptomatic bradycardia, helps prevent and convert paroxysmal intraatrial tachycardia and overcomes the problems of limited ventricular access.

The palliative Mustard operation: rationale and results.

The tendency toward development of severe pulmonary vascular obstructive disease in patients with complete transposition of the great arteries and associated large ventricular septal defect has been well documented. The physiologic consequence of this process is a significant reduction in intercirculatory mixing, resulting in increasingly severe levels of systemic arterial hypoxemia, compensatory polycythemia and an associated significant increase in symptoms. Data indicate that definitive surgical correction in patients with pulmonary arteriolar resistance of more than 10.0 units m2 is associated with a prohibitive operative mortality. However, the "palliative" Mustard operation (that is, intraatrial baffle repair, leaving the ventricular septal defect open) can be accomplished with relatively low risk and substantial hemodynamic benefits to the patient. It is erroneous to assume that the result of the operation will be a mere "reversal" of the preoperative systemic arterial and pulmonary arterial saturations, as shown by a review of the hemodynamic changes attributable to the operation. After the operation, systemic arterial saturations of 80 to 93 percent have been achieved, with a concomitant significant reduction in hemoglobin concentration, to a near normal level in some. Although the long-term prognosis in such patients is unknown, the reduction in symptoms and increase in exercise tolerance soon after operation are most gratifying.

Truncus arteriosus and previous pulmonary arterial banding: clinical and hemodynamic assessment.

Twenty-seven patients with truncus arteriosus and previous pulmonary arterial banding were evaluated 1 1/2 to 14 years (mean 7 years) after banding. Ages at the time of cardiac catheterization ranged from 3 to 18 years (mean 9 years). Current symptoms were severe in five patients and were related to truncal valve incompetence or decreased pulmonary blood flow (or both) rather than to age, duration of palliation or band location. Twenty-one of 22 patients with two pulmonary arteries were considered to be in a hemodynamically operable state at the time of study. The condition of three of five patients with a single pulmonary artery was subsequently found inoperable because of severe pulmonary vascular disease in the lung supplied by the single pulmonary artery. In patients with two pulmonary arteries, demonstration of low pressure in at least one normal-sized pulmonary artery established operability. Postoperative pressure measurements correlated well with preoperative prediction of operability, with 19 of 20 patients having a pulmonary arterial pressure less than 70 percent of systemic levels after repair. Bilateral pulmonary arterial binding may be more effective than central arterial banding (which frequently produces severe obstruction to the right pulmonary artery) in preventing pulmonary vascular obstructive disease in patients with truncus arteriosus who have two pulmonary arteries. Patients with truncus arteriosus and a single pulmonary artery with pulmonary arterial banding remain at high risk for the development of pulmonary vascular obstructive disease.

Hypertrophic obstructive cardiomyopathy: ten- to 21-year follow-up after partial septal myectomy.

This study reviews the outcome in 36 consecutive patients who survived partial septal myectomy for hypertrophic obstructive cardiomyopathy operated on between 1960 and 1972. All patients were followed up until death or until June 1981 (mean 13.4 years). Of the 26 survivors, 17 had been more than mildly symptomatic preoperatively, but only 1 remained so postoperatively. The operation was effective in relieving the obstruction (peak systolic pressure gradient reduced from 79 to 8 mm Hg [p less than 0.001]), and mitral regurgitation was relieved. No survivor's symptoms worsened, but 10 died late--4 suddenly, 5 from congestive heart failure, and 1 from a malignancy. The 10-year survival rate was 77%. No correlation with outcome was found with respect to age, surgical approach, preoperative functional class, pressure gradient, left ventricular end-diastolic pressure, or presence of atrial fibrillation, but atrial fibrillation occurring late postoperatively (12 patients) was associated with an increased frequency of late death (7 of 10 late deaths) or continuing New York Heart Association functional class III status. Early or late postoperative complete heart block occurred in 1 patient each. Thus, these results suggest a favorable effect of operation and support continued surgical intervention for appropriate patients.

Long-term follow-up of isolated replacement of the aortic or mitral valve with the Starr-Edwards prosthesis.

Review of 1,684 cases of isolated aortic or mitral valve replacement with a Starr-Edwards prosthesis demonstrated that the procedure provides improved life expectancy over that found in the natural history of valvular heart disease. Further improvement in results depends on continued reduction in operative and late mortality and in the incidence of thromboembolism. Advanced preoperative functional class, atrial or ventricular enlargement, a history of prior heart surgery, advanced age at operation and untreated valvular disease were among the factors related to increased early or late mortality. The data suggest that adequate anticoagulation, earlier performance of valve replacement and more complete repair of valvular dysfunction may increase survival rates.

Anatomy of coronary arteries in univentricular hearts and its surgical implications.

The coronary arterial anatomy in 26 univentricular hearts, its relation to the morphologic characteristics of the ventricles and rudimentary chambers, and its surgical implications were analyzed. All of the hearts except two had been operated on; 18 had septation with or without an extracardiac conduit and 6 had had palliative procedures. Twenty-one univentricular hearts with a left ventricular type main chamber had an anterior outlet chamber (17 left-sided subaortic, 3 right-sided subaortic and 1 right-sided subpulmonary). Right and left delimiting arteries outlined the outlet chamber in 16 hearts (76 percent). In 20 of the 21 hearts, large delimiting parallel branches of the right coronary artery course over the anterior wall of the heart; 13 of these vessels had been injured surgically with resultant ischemic myocardial necrosis. Five univentricular hearts did not have an outlet chamber; two had a left ventricular type main chamber and three had a morphologically right ventricular main chamber. Three of the five hearts had rudimentary pouches, located anteriorly in one and posteriorly in two. The two rudimentary pouches lying posteriorly were not outlined by delimiting arteries. Two of the five univentricular hearts without an outlet chamber also had injured coronary arteries. Thus, the identification of outlet chambers and rudimentary pouches in univentricular hearts is facilitated by the determination of coronary anatomy. The presence of major delimiting parallel branches over the usually favored ventriculotomy sites renders them vulnerable to surgical injury; such mishaps occurred in 15 of the 24 hearts that had either corrective or palliative operations.

Idiopathic hypertrophic subaortic stenosis in the young.

Thirty-six young patients with idiopathic hypertrophic subaortic stenosis were studied. Twenty-seven patients were male and 9 female, and their mean age was 11.3 years (range 5 months to 20 years). Twenty-three patients (64 percent) had symptoms, the most common being dyspnea, angina and syncope. Diagnostic difficulties were encountered frequently in younger patients, especially those with right heart involvement, and in asymptomatic patients with murmurs suggestive of other cardiac defects. Patients were classified retrospectively into three groups on the basis of management. The first group consisted of 16 patients who were operated on; 4 of these patients died, 1 operatively and 3 suddenly late postoperatively (at 1.6, 2 and 10 years). The 12 long-term survivors (average follow-up period 6.2 years) have had good relief of symptoms. The second group comprised seven patients treated with propranolol; none of these died. The 13 patients in the third group received no therapy; 7 of these patients died, 6 suddenly and 1 from congestive cardiac failure. Idiopathic hypertrophic subaortic stenosis is a serious disorder that may present at any age and that may be difficult to diagnose. All patients with this disorder should be treated with propranolol; surgical intervention, although it does not totally abolish the risk of sudden death, appears to offer symptomatic improvement in most cases over a long-term follow-up period.

Late results after extracardiac conduit repair for congenital cardiac defects.

Analysis of the late results in 352 patients surviving insertion of an extracardiac conduit before mid 1977 has provided a mean follow-up interval of 65 months. Three fourths of the patients remain in improved condition after operation. Serial measurements of transconduit gradient are available in 90. The median change was +7 mm Hg and the mean +21 mm Hg. Reoperation was required in 16 percent of patients (mortality rate 9 percent), most commonly (77 percent) because of progressive conduit stenosis, more commonly for transposition of the great arteries than for other types of anomalies, and more commonly after use of a homograft aortic conduit than a Hancock conduit. The side of the aorta on which the conduit was placed exerted no significant influence. The postrepair transconduit gradient did not affect the need for reoperation. Late survival was 95 percent at 1 year, 85 percent at 5 years, and 73 percent at 10 years and was significantly better (probability [p] less than 0.006) for patients with pulmonary atresia than for the others. The hospital mortality rate was highest, and the late mortality rate lowest, for children less than 5 years of age; the overall survival rate in this age group was lower. The postrepair right ventricular to left ventricular pressure ratio, together with age, was a principal prognostic indicator of late survival, being less good when more than 0.73. THe most frequent causes of late death were progressive congestive heart failure and sudden death.

Transient atrioventricular block after open-heart surgery for congenital heart disease.

The records of 22 patients with transient atrioventricular (AV) block after open-heart surgery for congenital heart disease from 1972 to 1978 were reviewed to determine the natural history of this entity. Preoperatively, no patient had AV block; 3 had right bundle branch block (BBB), 1 had left BBB and 5 had nonspecific intraventricular conduction delay. Complete AV block developed in 20 patients and Mobitz II AV block in 2. Transient AV block occurred intraoperatively in 14 patients and within 48 hours postoperatively in 8; AV block persisted for greater than or equal to 48 hours postoperatively in all patients, for a mean of 7.3 days (range 2 to 28). During a follow-up of 5.5 years (range 2.5 to 10), late AV block developed in 2 patients. None of the 18 patients whose escape QRS complex morphology during AV block was similar to the final QRS complex during normal sinus rhythm or atrial fibrillation with AV conduction had late AV block, whereas 2 of the 4 in whom it differed did (p less than 0.01). There was no difference in the escape rate between the 2 groups. Thus, late development of high-grade AV block is infrequent among patients with transient postoperative AV block. An escape QRS complex during postoperative AV block that differs from the QRS complex seen on recovery of normal sinus rhythm or atrial fibrillation with anterograde conduction may identify those at high risk of late AV block.

Long-term follow-up after palliative operation for univentricular heart.

Data are presented from 84 patients (45 males, 39 females), aged 6 days to 25 years (mean 10 years), with univentricular heart who underwent a palliative surgical procedure. The mean follow-up period was 5 years (range 1 month to 25 years). Of the 84 patients, 33 died and 51 are alive. The survivors were 1.5 to 41 years (mean 15). Systemic pulmonary artery shunts alone were performed in 43 patients, pulmonary artery banding in 15, and a combination of procedures in 26. Five years after diagnosis, 70% of patients with type A and 54% with type C univentricular heart were alive, with no statistical difference in survival between the patient groups. Because of the high attrition rate in these patients despite palliation, further attempts to perfect corrective surgery appear justified.

Trial of combined warfarin plus dipyridamole or aspirin therapy in prosthetic heart valve replacement: danger of aspirin compared with dipyridamole.

Despite the use of oral anticoagulation in patients with prosthetic heart valves, persistent thromboembolism over time warrants a search for improved methods of prevention. Thus, patients receiving 1 or more mechanical prosthetic heart valves were randomized to therapy with warfarin plus dipyridamole (400 mg/day) or warfarin plus aspirin (500 mg/day) on the basis of location and type of valve and surgeon, and followed up with a concurrent, nonrandomized control group taking warfarin alone. In 534 patients followed up 1,319 patient-years, excessive bleeding (necessitating blood transfusion or hospitalization) was noted in the warfarin plus aspirin group (23 of 170 [14%], or 6.0/100 patient-years) compared with warfarin plus dipyridamole (7 of 181 [4%], or 1.6/100 patient-years, p less than 0.001), or warfarin alone (9 of 183 [5%], or 1.8/100 patient-years, p less than 0.001). A trend was evident toward a reduction in thromboembolism in the warfarin plus dipyridamole group (2 of 181 [1%], or 0.5/100 patient-years) as compared with warfarin plus aspirin (7 of 170 [4%], or 1.8/100 patient-years), or warfarin alone (6 of 183 [4%], or 1.2/100 patient-years). Adequacy of anticoagulation (based on 12,720 prothrombin time determinations) was similar in all 3 groups with 65% of prothrombin times in the therapeutic range (1.5 less than or equal to prothrombin time/control less than or equal to 2.5), 30% too low, and 5% too high. Warfarin plus aspirin therapy resulted in excessive bleeding and is contraindicated. Longer follow-up study is needed to determine whether further separation of the incidence of thromboembolism can be detected.