Tetralogy with small pulmonary arteries.
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Child, Preschool↗
Biomedical subjects
D C McGoon
Publications and source records attributed to D C McGoon.
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.
Actuarial Analysis↗
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.
Adolescent↗
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.
Adolescent↗
Caplike double-horned double-outlet right ventricle. Report of two cases.
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.
Adolescent↗
Experience with the extracardiac conduit.
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.
Adolescent↗
Surgical septation of univentricular heart with outlet chamber.
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.
Adolescent↗
Surgical considerations in treating adults with congenital heart disease.
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Adolescent↗
Modified Fontan operation for univentricular heart and complicated congenital lesions.
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Adolescent↗
Surgical management of straddling atrioventricular valve.
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.
Adolescent↗
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.
Adolescent↗
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.
Adolescent↗
Congenital pulmonary atresia with ventricular septal defect: angiographic and surgical correlates.
Of 181 patients with severe congenital pulmonary atresia and ventricular septal defect or "type IV truncus" (an obsolete term), all but 11% had true central pulmonary arteries. These arteries were demonstrable by large serial biplane angiograms using multiple selective injections into collateral vessels, frequent photographic subtraction, and occasional pulmonary vein-wedge angiograms. These techniques are extremely important for accurate diagnosis and in planning corrective or palliative surgery, which was done in 77% of patients with pulmonary arteries.
Adolescent↗
Clinical, angiographic, and hemodynamic assessment of late results after Mustard operation.
Since 1974, late results of the Mustard procedure for correcting complete transposition of the great arteries have been evaluated by cardiac catheterization, electrocardiography, roentgenography, history, and physical examination of 48 Mayo Clinic patients. Of these, 15 were studied 1 month to 2 years postoperatively because of clinical deterioration. The other 33 had been asymptomatic but were asked to return for hemodynamic reevaluation one-half to 11 years postoperatively. Of the asymptomatic group, 19 underwent exercise electrocardiography prior to catheterization and eight performed supine exercise during catheterization. Cardiac catheterization proved the most effective mode of study. Significant cardiovascular abnormalities (caval obstruction, residual pulmonary stenosis, etc.) were found in 35 of the 47 patients--including 20 of the 33 who were asymptomatic. Eight of the symptomatic group and three of the others have died since this restudy. These poor results warrant renewed effort to devise better methods for correcting complete transposition,
Cardiac Catheterization↗
Vulnerability of coronary arteries in surgery for transposition of the great arteries.
A detailed anatomic study of 82 hearts from patients operated upon for complete or congenitally corrected transposition of the great arteries revealed 12 instances of intraoperative injury to a major coronary artery. Because the coronary arterial anatomy is so variable in transposition of the great arteries, there is a significant risk of injury to them during operation. This is especially so if epicardial adhesions and scarring from previous operations obscure the surface anatomy of the heart. The most common site of injury was adjacent to incisions in the anterior surface of the right-sided ventricle, particularly in congenitally corrected transposition. Without a meticulous examination, these complications of cardiac surgery may be easily overlooked even when the hearts are examined at autopsy.
Blood Vessel Prosthesis↗
Surgical management of double-outlet right ventricle associated with atrioventricular discordance.
Twenty corrective operations for this unusual syndrome have been performed since 1965. All but two patients also had pulmonary stenosis; one of the exceptions had had pulmonary arterial banding. Two patients had situs inversus of the atria and viscera. In only three of the 20 patients (15 percent) was the apex of the heart positioned normally. Six patients had an entirely intracardiac repair; two early and two late deaths occurred in this group. The other technique, performed in 14 patients, involved insertion of an extracadiac conduit between a ventriculotomy in the morphologically left ventricle and the distal end of the proximally oversewn pulmonary artery; one operative and two late deaths occurred in this group. Heart block occurred in six patients, including two among the eight who had technically satisfactory intraoperative mapping of the His bundle. The intraventricular course of the bundle was not consistently positioned, being anterior to the septal defect in four and posterior in four. No hospital deaths have occurred in the last 13 operations; all except one of the survivors are in New York Heart Association Class I or II. Thus correction currently provides a good early results but leaves the ventricles in an inverted relationship both functionally and anatomically.
Adolescent↗
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.
Female↗