Regression of tricuspid regurgitation after two-stage arterial switch operation for failing systemic ventricle after atrial inversion operation

AbstractThe cases of five patients with previous Senning ( n = 4) or Mustard ( n = 1) operations and failing systemic ventricles in whom banding of the pulmonary artery was performed as an interim step toward an arterial switch procedure are reported. The rise in the ratio of left to right mean systolic ventricular pressure, from 0.35 before operation to 0.90 during operation and 0.80 on the first postoperative day, caused a shift of the ventricular septum from a leftward to a midline or nearly midline position. This shift was associated with a reduction of tricuspid regurgitation. At a median interval of 5.1 months after pulmonary artery banding, the mean left ventricular posterior wall thickness had increased to 8.2 mm, versus 5 mm before operation, and the mean left ventricular myocardial mass index had increased to 90 gm/m2, versus 55.6 gm/m2 before operation. After the arterial switch operation, which was performed in four patients, the tricuspid regurgitation decreased to a trivial amount ( n = 1) or disappeared completely ( n = 3). (J THORAC CARDIOVASC SURG 1996;111:342-7

Outcomes After Bidirectional Cavopulmonary Shunt in Infants Less Than 6 Months Old

AbstractObjectives. We sought to assess the results after bidirectional cavopulmonary shunt (BCPS) in infants <6 months old and to identify risk factors for poor outcome.Background. Although BCPS is a well established procedure for the palliation of patients with a single-ventricle heart, there have been very few reports of outcomes after BCPS in young infants.Methods. Since 1990, 42 infants between 0.8 and 6.0 months of age (mean [±SD] 3.7 ± 1.4) have undergone BCPS for primary (n = 16) or secondary (n = 26) palliation of tricuspid atresia (n = 13), hypoplastic left heart syndrome (n = 10) or other forms of functional single-ventricle heart (n = 19). Accessory pulmonary blood flow was included in 18 patients. Preoperative and perioperative data were gathered on retrospective review of patient records, and follow-up was conducted by means of direct physician contact or record review.Results. The overall hospital mortality rate, including that associated with reoperations, was 4.8% (2 of 42 patients). Seven patients (17%) required reoperation related to the BCPS or pulmonary blood flow in the early postoperative period: Procedures included take-down of the BCPS in four patients, with one early death, and procedures to decrease pulmonary blood flow in three patients. Age <1 month correlated significantly with early death and with early failure of the BCPS (death or take-down). Follow-up of the 37 patients discharged with intact BCPS was obtained at a mean ± SD of 14.3 ± 11.3 months postoperatively, during which time three patients died (at 6.5 ± 2.5 months). The 2-year actuarial survival rate for patients undergoing BCPS at <6 months of age was 86%. Overall freedom from death or take-down (including early and late events) was significantly lower in patients <2 months old than in those >2 months old. Four patients have undergone successful Fontan completion (18.3 ± 2.9 months postoperatively), and one patient whose BCPS was taken down subsequently underwent successful restoration of a BCPS.Conclusions. Outcomes after BCPS in young infants are comparable to those in older infants and children. However, our current preference is to defer this procedure until after 2 months of age.(J Am Coll Cardiol 1997;29:1365–70

Induced fibrillation is equally effective as crystalloid cardioplegia in the protection of fetal myocardial function

AbstractBackgroundFetal cardiac intervention represents a potential advance in the treatment of congenital cardiac lesions that increase in complexity during development. Prenatal repair of a primary defect might prevent pathologic blood-flow patterns that can result in hypoplasia of a cardiac chamber or great vessel. However, strategies to optimize fetal myocardial protection have not been studied. A biventricular working fetal heart preparation was used to evaluate the cardioprotective properties of induced fibrillation and crystalloid cardioplegia.MethodsHearts from 16 fetal lambs at 115 to 125 days’ gestation were harvested and perfused with Krebs-Henseleit solution. The descending aorta was ligated distal to the ductal insertion and the branch pulmonary arteries were ligated to simulate the parallel circulation of the fetus. Hearts were arrested with normothermic fibrillation (n = 8) or hypothermic crystalloid cardioplegia (n = 8) before reperfusion with Krebs-Henseleit solution. Baseline and postarrest myocardial function measurements were obtained from analysis of pressure-dimension relationships.ResultsFibrillatory and cardioplegic arrest were equally effective at preserving postarrest systolic function (left ventricle, 70% ± 5% vs 68% ± 15%, P = .52; right ventricle, 68% ± 4.5% vs 65% ± 4.5%, P = .26) and preventing increased diastolic stiffness (left ventricle, 32% ± 5.3% vs 38% ± 11%, P = .24; right ventricle, 25% ± 3.3% vs 27% ± 2.1%, P = .46). Myocardial water content was unchanged in hearts arrested with fibrillation and cardioplegia (84% ± 1.5% vs 83.7% ± 0.9%, P = .71).ConclusionsNormothermic fibrillation and hypothermic crystalloid cardioplegia provide equal protection of the fetal myocardium. In the setting of diminished fetal myocardial reserve and because of the limited ability to manipulate the surrounding temperature in the fetus, normothermic fibrillation may be preferable for in utero repairs of selected congenital heart defects

Geometric mismatch of pulmonary and aortic anuli in children undergoing the Ross procedure: Implications for surgical management and autograft valve function

AbstractBackground: There is often substantial mismatch between the diameters of the pulmonary and aortic anuli in young patients with systemic outflow tract disease. To implant the autologous pulmonary valve in the aortic position under such circumstances, it is necessary to adapt the geometry of the systemic outflow tract. The effects of such adaptations on autograft function in children are not well known. Methods: To determine factors predictive of autograft regurgitation, we analyzed 41 cases of children who have undergone the Ross procedure. The diameter of the pulmonary valve was greater (by at least 3 mm) than that of the aortic valve in 20 cases, equal (within 2 mm) in 12 cases, and less (by at least 3 mm) in nine cases, with differences ranging from +10 to –12 mm. In 12 patients with a larger pulmonary anulus, aortoventriculoplasty was used to correct the mismatch. In patients with a larger aortic anulus, the mismatch was corrected by gradual adjustment along the circumference of the autograft, rather than by tailoring of the native aortic anulus. Results: At follow-up (median 31 months), two patients had undergone reoperation on the neoaortic valve for moderate regurgitation. In the remaining 38 cases, autograft regurgitation was as follows: none or trivial in 30, mild in seven, and moderate in one. There was no correlation between regurgitation and age, geometric mismatch, or previous or concurrent procedures. Conclusions: Subtle technical factors that may result in distortion of the valve complex are probably more important determinants of autograft regurgitation than are indication for repair, geometric mismatch, or previous or concomitant outflow tract procedures. Significant mismatch of the semilunar anuli is not a contraindication to the Ross procedure in children. (J Thorac Cardiovasc Surg 1998;115:1255-63

Long-term follow-up of truncus arteriosus repaired in infancy: A twenty-year experience

AbstractBackground: There have been few reports of long-term follow-up after truncus arteriosus repair in infancy. Methods: A retrospective review was performed to assess long-term outcomes among 165 patients who survived the initial hospital stay after complete repair of truncus arteriosus since 1975. The median age at truncus repair over this 20-year experience was 3.5 months (range 2 days to 36 years), and 81% of patients were less than 1 year of age. Previous pulmonary artery banding had been performed in 15 patients, and two patients had undergone prior repair of interrupted aortic arch. Significant procedures performed along with truncus repair included truncal valve replacement (n = 10) or repair (n = 5) and repair of interrupted aortic arch (n = 4). Results: Patients were followed up for up to 20.4 years (median 10.5 years). Twenty-five patients were lost at cross-sectional follow-up, with a total of 67 patient-years of follow-up available on these patients. There have been 23 late deaths, eight of which occurred within 6 months of repair and 13 of which occurred within 1 year. Ten of the late deaths were related to reoperations. Actuarial survival among all hospital survivors was 90% at 5 years, 85% at 10 years, and 83% at 15 years and was essentially identical for infants alone. A significant independent risk factor for poorer long-term survival was truncus with moderate to severe truncal valve insufficiency before repair. During the follow-up period, 107 patients underwent 133 conduit reoperations. Median time to conduit reoperation was 5.5 years, and the only factor significantly associated with shorter time to conduit replacement was smaller conduit size at initial repair. In addition, 26 patients underwent 30 truncal valve replacements. Six patients required truncal valve replacement before any conduit-related reintervention, with two associated deaths. Actuarial freedom from truncal valve replacement among patients with no prerepair truncal valve insufficiency was 95% at 10 years. Actuarial freedom from truncal valve replacement was significantly lower among patients with truncal insufficiency before initial repair (63% at 10 years). At follow-up, all patients except three were in New York Heart Association functional class I. Conclusions: Ten- to 20-year survival and functional status are excellent among infants undergoing complete repair of truncus arteriosus. Conduit replacement or revision is almost inevitably necessary in this group of patients. (J Thorac Cardiovasc Surg 1997;113:869-79

Results of 102 cases of complete repair of congenital heart defects in patients weighing 700 to 2500 grams

AbstractBackground: Published data suggest that low birth weight is a risk factor for poor outcome in corrective surgery for many cardiac defects. Congenital heart defects in low birth weight infants are typically managed with supportive therapy or palliative operations, with definitive repair delayed. The morbidity associated with such approaches is high. Methods: Since 1990 complete repair of congenital heart defects (other than patent ductus arteriosus) has been performed in 102 infants no larger than 2500 g (median 2100 g, range 700-2500 g), including 16 no larger than 1500 g. Defects included ventricular septal defect (n = 22), tetralogy of Fallot complexes (n = 20), transposition complexes (n = 13), aortic coarctation (n = 12), interrupted arch (n = 10), truncus arteriosus (n = 8), atrioventricular septal defect (n = 6), total anomalous pulmonary venous return (n = 5), and other (n = 6). Results: Preoperative morbidity was more common among patients referred late for surgical correction. There were 10 early deaths (10%) attributable to cardiac failure (n = 4), arrhythmia (n = 1), multiorgan failure (n = 1), sepsis (n = 1), idiopathic coronary artery intimal necrosis (n = 1), foot gangrene (n = 1), and pulmonary hemorrhage (n = 1). No patient had postbypass intracerebral hemorrhage. At follow-up (median 36 months) there were 8 late deaths, and 8 patients underwent 10 reinterventions. There was no evidence of neurologic sequelae attributable to the operation. Conclusions: In general, delaying repair of congenital heart defects in low birth weight infants does not confer a benefit and is associated with higher preoperative morbidity. Complete repair of both simple and complex lesions can be achieved in such cases with good results. Growth after repair approximates the normal curve for low birth weight infants without heart disease. It is recommended that such infants, especially when they have symptoms, undergo early surgical repair rather than prolonged medical management or other forms of palliation. (J Thorac Cardiovasc Surg 1999;117:324-31

Early results of the extracardiac conduit Fontan operation

AbstractBackground: Among the modifications of the Fontan operation, the extracardiac approach may offer the greatest potential for optimizing early postoperative ventricular and pulmonary vascular function, insofar as it can be performed with short periods of normothermic partial cardiopulmonary bypass and without cardioplegic arrest in most cases. In this study, we reviewed our experience with the extracardiac conduit Fontan operation, with a focus on early postoperative outcomes. Methods and results: Between July 1992 and April 1997, 51 patients (median age 4.9 years) underwent an extracardiac conduit Fontan operation. Median cardiopulmonary bypass time was 92 minutes and has decreased significantly over the course of our experience. Intracardiac procedures were performed in only 5 patients (10%), and the aorta was crossclamped in only 11 (22%). Intraoperative fenestration was performed in 24 patients (47%). There were no early deaths. Fontan failure occurred in 1 patient who was a poor candidate for the Fontan procedure. Transient supraventricular tachyarrhythmias occurred in 5 patients (10%). Median duration of chest tube drainage was 8 days. Factors significantly associated with prolonged resource use (mechanical ventilation, inotropic support, intensive care unit stay, and hospital stay) included longer bypass time and higher Fontan pressure. At a median follow-up of 1.9 years, there was 1 death from bleeding at reoperation. Conclusions: The extracardiac conduit Fontan procedure can be performed with minimal mortality and morbidity. Improved results may be related to advantages of the extracardiac approach and improved preservation of ventricular and pulmonary vascular function. (J Thorac Cardiovasc Surg 1999;117:688-96

Hemidiaphragmatic paralysis increases postoperative morbidity after a modified Fontan operation

AbstractObjectives: After a Fontan procedure, forward pulmonary blood flow is augmented during inspiration because of negative intrathoracic pressure. Total pulmonary blood flow is higher during inspiration. With hemidiaphragmatic paralysis, inspiratory augmentation of pulmonary flow is lost or diminished. The objective of this study was to compare early postoperative morbidity after the modified Fontan operation in patients with and without hemidiaphragmatic paralysis. Methods: A case-control analysis was performed comparing 10 patients with documented hemidiaphragmatic paralysis against 30 patients without paralysis who were matched for diagnosis, fenestration, and age. The following early postoperative outcomes were assessed: duration of ventilator support, duration of hospital stay, incidence of ascites, prolonged effusions, and readmission. Results: Preoperatively, there were no significant differences between the 2 groups. However, among the postoperative outcomes, the duration of hospital stay (25.4 ± 16.6 days vs 10.8 ± 6.3 days; P =.03), incidence of ascites (70% vs 3%; P ||.001), prolonged pleural effusions (60% vs 13%; P =.007), and readmission (50% vs 7%; P =.007) were significantly greater in patients with hemidiaphragmatic paralysis than in those without hemidiaphragmatic paralysis. Conclusions: Hemidiaphragmatic paralysis after the modified Fontan operation is associated with an increase in early morbidity. Care should be taken to avoid injury to the phrenic nerve. Patients with prolonged effusions should be evaluated for hemidiaphragmatic paralysis.J Thorac Cardiovasc Surg 2001;122:856-6

Revision of previous Fontan connections to total extracardiac cavopulmonary anastomosis: A multicenter experience

AbstractBackground: Conversion to total extracardiac cavopulmonary anastomosis is an option for managing patients with dysfunction of a prior Fontan connection. Methods: Thirty-one patients (19.9 ± 8.8 years) underwent revision of a previous Fontan connection to total extracardiac cavopulmonary anastomosis at four institutions. Complications of the previous Fontan connection included atrial tachyarrhythmias (n = 20), progressive heart failure (n = 17), Fontan pathway obstruction (n = 10), effusions (n = 10), pulmonary venous obstruction by an enlarged right atrium (n = 6), protein-losing enteropathy (n = 3), right atrial thrombus (n = 2), subaortic stenosis (n = 1), atrioventricular valve regurgitation (n = 3), and Fontan baffle leak (n = 5). Conversion to an extracardiac cavopulmonary connection was performed with a nonvalved conduit from the inferior vena cava to the right pulmonary artery, with additional procedures as necessary. Results: There have been 3 deaths. Two patients died in the perioperative period of heart failure and massive effusions. The third patient died suddenly 8 months after the operation. All surviving patients were in New York Heart Association class I (n = 20) or II (n = 7), except for 1 patient who underwent heart transplantation. Early postoperative arrhythmias occurred in 10 patients: 4 required pacemakers, and medical therapy was sufficient in 6. In 15 patients, pre-revision arrhythmias were improved. Effusions resolved in all but 1 of the patients in whom they were present before revision. The condition of 2 patients with protein-losing enteropathy improved within 30 days. Conclusions: Conversion of a failing Fontan connection to extracardiac cavopulmonary connection can be achieved with low morbidity and mortality. Optimally, revision should be undertaken early in symptomatic patients before irreversible ventricular failure ensues. (J Thorac Cardiovasc Surg 2000;119:340-6

Pulmonary artery growth after bidirectional cavopulmonary shunt: Is there a cause for concern?

AbstractObjective: Our objective was to analyze changes in pulmonary artery size after bidirectional cavopulmonary shunt. Methods: All 47 patients who underwent bidirectional cavopulmonary shunt between March 1990 and May 1995 who had preoperative and postoperative angiograms available for review were selected for study. This included 24 patients who had also undergone a modified Fontan operation. Clinical records were reviewed retrospectively and cross-sectional follow-up was obtained by direct physician contact. Angiograms were reveiwed, and the right and left pulmonary artery diameters were each measured at two locations: immediately distal to their origin and at the narrowest point. In addition, the lower lobe pulmonary artery branch was measured just distal to its origin. Cross-sectional areas (left, right, and total) at each point of measurement were indexed to body surface area. Angiographic and hemodynamic data were analyzed. Results: Changes in the various measures of pulmonary artery size after bidirectional cavopulmonary shunt varied considerably. On average the absolute diameters increased for all measures, but the increase in diameter was significant only for the lower lobe arteries. All pulmonary artery indices decreased on average, but these changes did not approach significance. Patients who underwent pulmonary artery augmentation at the time of bidirectional cavopulmonary shunt had significantly smaller pulmonary artery indices before pulmonary artery augmentation, relative to those who did not undergo pulmonary artery repair, and significantly greater changes (possibly to a large extent owing to pulmonary artery repair) in the right and left pulmonary artery index after bidirectionl cavopulmonary shunt. Lower lobe indices did not differ preoperatively or exhibit different degrees of change in size between patients who did and did not undergo pulmonary artery repair. One patient died after Fontan completion (pulmonary artery index: 305 mm2/m2), and none of the factors analyzed correlated with Fontan outcomes. Conclusions: A more appropriate measure of pulmonary artery growth is the indexed cross-sectional area of the lower lobe branch of the right and left pulmonary arteries, which is less likely to be altered surgically with systemic-pulmonary shunts, pulmonary artery repair, and the bidirectional cavopulmonary anastomosis itself. Pulmonary artery indices, including the lower lobe index, do not change significantly after bidirectional cavopulmonary shunt during medium-term follow-up and do not influence the Fontan outcome. (J THORAC CARDIOVASC SURG 1996;112:1180-92