Tetralogy with small pulmonary arteries.
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Biomedical subjects
Publications and source records attributed to D C McGoon.
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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 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.
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.
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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)
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.
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.
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)
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.
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.
Two patients with a rare form of double-chambered, double-outlet right ventricle (DORV) are described. Both patients had associated pulmonary stenosis, ventricular septal defect, and a large tricuspid valve whose chordal attachments crossed the right ventricular outflow tract. The angiocardiographic, echocardiographic, and operative findings were distinctive. The right ventricle was positioned as a "cap" sitting atop the ventricular septal defect at the cephalad end of the left ventricle. The right ventricle, with its two "horns," was likened to a rider straddling a saddle. Tricuspid chordal attachments extended into each of the two right ventricular "horn." No repair was performed in the asymptomatic patient. The other repair involved closure of the tricuspid orifice and insertion of a valved extracardiac conduit from the right atrium to the pulmonary artery.
A review is made of all Mayo Clinic cases wherein 468 patients have received 516 extracardiac conduits in the repair of congenital heart defects. All patients had complex defects, which are classified in 10 basic diagnostic categories. The early mortality rate (which ranged from 4% to 49%, according to diagnostic group) averaged 25%, improving with experience. The postoperative complication rate was 70%. In 1% of operations, compression of the conduit was encountered at chest closure. The conduits placed in 333 patients contained a porcine valve. The conduit diameter averaged 22 mm. At completion of the operation, the mean gradient across the conduit was 22.8 mm Hg. At late study this mean gradient remained at 26 mm Hg in patients who had received the currently employed porcine-valved conduit. The average ratio of intraoperative postrepair ventricular (RV/LV) systolic pressures was 0.68. It was lower among the group who were to survive but was not ov prognostic value in individual cases. Among patients who survived the postoperative period, the late mortality rate (based on a mean of 3.6 years' follow-up) averaged 3.5% per year. Of the long-term survivors, 18% have required reoperation, primarily for replacement of calcified aortic homograft conduits used in the earlier part of the experience. Only 0.8% of porcine-valved conduits have required reoperation. Unrestricted life-styles were possible for 90% of survivors.
From April 1973 through March 1978, 34 patients with univentricular heart and outlet chamber underwent repair by septation of the ventricle. Three had normally related great arteries in which the pulmonary artery arose from the outlet chamber. Six patients had an anterior aorta arising from an anterior or right-sided outlet chamber and 25 patients had an anterior aorta arising from a left-sided outlet chamber. The ages ranged from 3 to 36 years. Fourteen patients had had previous palliative operations. There were 13 hospital deaths and four late deaths. Four patients have a fair result and the remaining 13 patients are in good health clinically. For the more favorable types of univentricular heart with outlet chamber on the left and natural pulmonary stenosis, the risk of corrective operation is less than 20 percent. The improved clinical course of those patients who have survived and done well gives encouragement that continued refinements in the operative repair of univentricular heart should ultimately provide many of these patients with a good result.
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Corrective operations were attempted in 10 patients with straddling atrioventricular valve (SAVV), and successful palliative operations were performed in another nine patients. SAVV occurred as a part of five different cardiac complexes, and three anatomic types were encountered. The SAVV was a tricuspid valve in eight patients, right atrioventricular valve (in univentricular hearts) in five, and mitral valve in six. Four types of corrective procedures were used: (1) the Rastelli operation, (2) isolated closure of the universally present ventricular septal defect by a patch deviating around the straddling portion of the SAVV, (3) replacement of the SAVV with or without insertion of an extracardiac conduit, and (4) closure of the SAVV plus right atrium-pulmonary artery anastomosis (modified Fontan procedure). All three hospital deaths occurred in patients managed by replacement of SAVV and extracardiac conduit insertion (technique 3). This experience allows preliminary speculation about preferred methods of surgical management.