Lionel M. Bargeron

Late survival and symptoms after repair of tetralogy of Fallot.

The long-term results of 414 patients who underwent repair of tetralogy of Fallot between 1967 and 1977 were studied and correlated with the results of others. There were nine late deaths (8-year actuarial survival 95.8%). Six of the deaths were directly related to the malformation or its treatment. Eight patients (2.4%) required reoperation. Ten patients (4.8%) had arrhythmic symptoms. Eight (3.1%) had congestive heart failure that required treatment. The risk factors associated with late events of all types, including death, were: older age at repair, a high mean ratio of peak systolic right-to-left ventricular pressures (PLV/RV) immediately after repair, and the presence of a Potts anastomosis. Neither a transannular patch nor a previous Blalock-Taussig or Waterson anastomosis was an incremental risk factor. Bacterial endocarditis was not observed. Three hundred seven patients underwent repair primarily or after a single Blalock-Taussig or Waterston shunt and had a PRV/LV of 0.85 or less after repair. Among these selected patients, the actuarial survival was 98.1%, which is still lower than that for the general population (p = 0.12), and freedom from events was 95.9%. Late after repair, PRV/LV was lower by 6 ± 28% (± SD) than PRV/LV immediately after repair (p = 0.03) in the 33 restudied patients with such data. The higher the PRV/LV immediately after repair, the greater the percent reduction.

Congenital pulmonary vein stenosis

Congenital pulmonary vein stenosis is a rare and serious form of congenital heart disease. Between 1969 and 1982 10 patients with this lesion were studied. In 2 patients the condition was diagnosed at autopsy; these patients died before the presence of congenital heart disease was suspected. Of the 8 in whom the condition was diagnosed during life, it was suspected clinically in 6 and found unexpectedly at cardiac catheterization in 2. All underwent operation, and 5 were hospital survivors. In all survivors rapid and progressive restenosis of the pulmonary veins occurred over the next several months. Three of the 5 underwent reoperation, but progressive restenosis recurred and all eventually died of this condition. Thus, despite partial surgical relief of pulmonary vein stenosis, the lesion is apparently one of relentless progression. No surgical repair has been successful in the cure or long-term palliation of this lethal lesion.

Surgical Results and Protocols in the Spectrum of Tetralogy of Fallot

Between 1967 and July 1982, 1103 operations were performed for the tetralogy of Fallot of all types with 116 (10.5%) hospital deaths. Eighty-eight hospital deaths (10.5%) occurred in the 836 patients undergoing repair. The incremental risk factors for hospital death after repair include pulmonary arterial problems (p = 0.0002), major associated cardiac anomalies (p less than 0.0001), small size (young age) (p less than 0.0001), and more than one previous operation (p = 0.0004). Absent pulmonary valve is a risk factor (p = 0.04). In patients with pulmonary stenosis, the hospital mortality has decreased with time (p = 0.08), but the incremental risk of a high hematocrit (p = 0.0003) and of transannular patching (p = 0.05) has persisted. In the current era, the risk of repair in patients with pulmonary stenosis is estimated to be 1.6% (70% confidence limits [CL] 0.7% to 3.5%) at age 5 years, and at age 12 months to be 4.1% (CL 2.7% to 6.3%) without a transannular patch and 7.7% (CL a5.3% to 11%) with one. When pulmonary atresia is present, the probability of hospital death after repair when a valved extracardiac conduit is used is estimated to be lowest (5%; CL 2% to 8%) between 5 1/2 and 16 years of age. No deaths occurred among 53 patients with pulmonary stenosis receiving a primary palliative Blalock-Taussig or Gore-Tex shunt, and six deaths (12%) occurred in 51 patients with pulmonary atresia. Serious interim complications (sudden death, brain abscess) after these shunts occurred in two (1.9%; CL 0.6% to 4.5%) of the patients operated on. No iatrogenic pulmonary arterial problems have been recognized. Protocols based on these results are presented. Many of these selective recommendations may become unnecessary if the damaging effects of cardiopulmonary bypass are overcome by future research.

Cardiac Repair in Anatomically Corrected Malposition of the Great Arteries

Anatomically corrected malposition of the great arteries is a congenital cardiac malformation in which the atria and ventricles are in concordant relation, as are the ventricles and great arteries, but the aorta is to the left of the pulmonary artery (1-position). The reported cases and our two patients have had situs solitus of viscera and atria. The aortic and pulmonary circulations are in series. We report successful surgical repair in 2 patients with associated large ventricular septal defects and pulmonary stenosis. In one the defect was posterior and in relation to the pulmonary artery which slightly overrode it. In the other the defect was anterior and the aorta overrode it. The developmental basis of these two examples of the same entity is probably different. An appropriate terminology and categorization that is useful surgically is presented.

The enlargement of small pulmonary arteries by preliminary palliative operations.

r. SUMMARY Four patients with tetralogy of Fallot, three of whom had congenital pulmonary atresia, were treated by initial palliative operations to enlarge left and right pulmonary arteries which were toconsidered too small for complete repair. Two to four years later the right and left pulmonary arteries had enlarged sufficiently to allow complete repair.

Intracardiac repair of cardiac malformations with atrioventricular discordance.

Twenty-six patients with atrioventricular discordance and a variety of ventriculo-arterial connections have had appropriate intracardiac repairs. Seven of 26 patients (26.9%) died early postoperatively, but only two (11.1%) of 18 patients operated upon since May 1972 have died. Ten patients (40%) developed permanent complete heart block. Two patients died late postoperatively; 16 (84.1% of those surviving the early postoperative period) were in NYHA Class I or II at late follow-up.Seventeen of the patients had “corrected transposition.” Six developed tricuspid incompetence with regurgitation into the left atrium after repair. Two patients with atrioventricular discordance and double outlet right ventricle survived operation and are asymptomatic but have permanent pacemakers. One patient died after repair of double outlet left ventricle with atrioventricular discordance. Three of four patients with atrioventricular discordance and “pseudotruncus arteriosus” survived repair with valved external conduits and are well. One of two patients is well after repair of “isolated ventricular inversion” and ventricular septal defect.

Investigation and management of primary cardiac tumors in infants and children.

Seven children with primary cardiac tumors are discussed. The diagnosis was achieved by cardiac catheterization in all seven patients. In four patients, surgical excision was undertaken with one long-term good result. One of the other three surgical patients died of acute heart failure at the discontinuation of cardiopulmonary bypass. One of the other two surgical patients died suddenly late postoperatively, presumably from arrhythmia. The other, affected by fibrosarcoma, also died suddenly while undergoing chemotherapy. The histologic findings on the tumor masses were available in six patients. There were three rhabdomyomas, one fibrosarcoma, one fibroma and one hamartomatous mass. In one patient with tuberous sclerosis, a presumptive diagnosis of rhabdomyoma was made clinically on the basis of multiple masses infiltrating the left ventricle. Two-dimensional echocardiography is the best noninvasive tool to determine the extent and location of the tumor and suitability for surgery. When surgery is indicated, cardiac catheterization for pressure data is also required. Surgery is recommended in symptomatic solitary lesions that most likely are fibromas and have an overall good surgical outcome.

Corrective Repair of Complete Atrioventricular Canal Defects and Major Associated Cardiac Anomalies

Twenty-nine patients with balanced forms of complete atrioventricular (AV) canal defects and tetralogy of Fallot in 12, double-outlet right ventricle (DORV) in 10, and additional anomalies associated with atrial isomerism in 7 underwent corrective repair. Age ranged from 39 days to 24 years (median, 3.7 years). Repair included reconstruction of the common AV valve in 25 patients and prosthetic valve replacement in 4. Construction of an intraventricular tunnel connecting the left ventricle with the aorta was accomplished in 23 of 25 patients with ventriculoarterial concordant or double-outlet connections. In 2 patients with situs inversus and associated DORV and in 4 patients in the isomerism group who had ventriculoarterial discordant connections, repair included intraatrial venous diversion by a modified Senning procedure in 2, a modified Mustard venous switch in 1, and a tailored atrial baffle in 3. A valved extracardiac conduit was required for repair in 4 patients. Partial anomalous systemic venous connection was repaired in 7 patients and total anomalous pulmonary venous connection, in 5. There was 1 hospital death (3%) and 3 late deaths (11%). There was 1 (3.6%) reoperation to replace an incompetent right (systemic) AV valve 17 months postoperatively, and each of the 25 surviving patients was in New York Heart Association Functional Class I (80%) or II (20%) at follow-up.

Surgical treatment of straddling tricuspid valves.

Six patients whose cardiac malformations included a large ventricular septal defect (VSD) in the inlet portion of the septum and straddling tricuspid valve underwent corrective intracardiac operations. In four patients, both the tricuspid valve annulus and its tensor apparatus occupied part of each ventricle. In one, only the annulus overrode the ventricular septum and in one the annulus was normally positioned, but a portion of the tensor apparatus crossed through the VSD from its origin in the left ventricle. This last patient had related mitral valve incompetence. One patient had an isolated VSD and one had isolated, multiple VSDs and a previously banded pulmonary artery. Associated malformations in the other four included tetralogy of Fallot, double outlet right ventricle, transposition of the great arteries with severe tricuspid incompetence, and corrected transposition. Hypoplasia of the sinus portion of the right ventricle was present to some degree in four patients. Preoperatively the diagnosis was definitively established by axial cineangiocardiograms in three and suspected in one.The straddling tricuspid valve was preserved in the repair in five patients, but tricuspid valve replacement was used in the patient with severe tricuspid incompetence. In one case, intraoperative electrophysiologic mapping studies were performed and showed abnormal conduction pathways.Five patients survived the hospital period. Postoperative cardiac catheterization studies were performed in four, one of whom died 16 months postoperatively from chronic congestive heart failure. The remaining four survivors have had a good clinical result.

Intracardiac Surgery in Infants Under Age 3 Months: Predictors of Postoperative In-Hospital Cardiac Death

Data are reported on 142 infants less than 3 months old who left the operating room alive after an open intracardiac operation during the 13 years from January 1967 to July 1980. The probability of postoperative in-hospital cardiac death for acute postoperative heart failure (the most common mode of death in these infants) was found by multivariate logistic analysis to be significantly related only to the strength of pedal pulses, the pedal skin temperature and the cardiac index in the first 5 postoperative hours. When cardiac index was not analyzed and cold cardioplegic myocardial preservation methods used, only pedal pulses and pedal skin temperature were significant predictors of hospital death. Blood pressure and heart rate were not related to this mode of hospital death. Oliguria occurred in 23 percent of patients; it was related primarily to inadequate cardiac performance and increased the probability of hospital death. Treatment protocols are derived based on these facts.