Increased mortality, morbidities, and costs after heart transplantation in heterotaxy syndrome and other complex situs arrangements.

OBJECTIVES: Identify pediatric heart transplant (HT) recipients with heterotaxy and other complex arrangements of cardiac situs (heterotaxy/situs anomaly) and compare mortality, morbidities, length of stay (LOS), and costs to recipients with congenital heart disease without heterotaxy/situs anomaly. METHODS: Using linked registry data (2001-2016), we identified 186 HT recipients with heterotaxy/situs anomaly and 1254 with congenital heart disease without heterotaxy/situs anomaly. We compared post-HT outcomes in univariable and multivariable time-to-event analyses. LOS and cost from HT to discharge were compared using Wilcoxon rank-sum tests. Sensitivity analyses were performed using stricter heterotaxy/situs anomaly group inclusion criteria and through propensity matching. RESULTS: HT recipients with heterotaxy/situs anomaly were older (median age, 5.1 vs 1.6 years; P < .001) and more often black, Asian, Hispanic, or "other" nonwhite (54% vs 32%; P < .001). Heterotaxy/situs anomaly was independently associated with increased mortality (hazard ratio, 1.58; 95% confidence interval, 1.19-2.09; P = .002), even among 6-month survivors (hazard ratio, 1.86; 95% confidence interval, 1.09-3.16; P = .021). Heterotaxy/situs anomaly recipients more commonly required dialysis (odds ratio, 2.58; 95% confidence interval, 1.51-4.42; P = .001) and cardiac reoperation (odds ratio, 1.91; 95% confidence interval, 1.17-3.11; P = .010) before discharge. They had longer ischemic times (19.2 additional minutes [range, 10.9-27.5 minutes]; P < .001), post-HT intensive care unit LOS (16 vs 13 days; P = .012), and hospital LOS (26 vs 23 days; P = .005). Post-HT hospitalization costs were also greater ($447,604 vs $379,357; P = .001). CONCLUSIONS: Heterotaxy and other complex arrangements of cardiac situs are associated with increased mortality, postoperative complications, LOS, and costs after HT. Although increased surgical complexity can account for many of these differences, inferior late survival is not well explained and deserves further study.

Experience with bidirectional cavopulmonary anastomosis and modified Fontan operation in patients with single ventricle and concomitant visceral heterotaxy.

The object of our study was to analyze the results of bidirectional cavopulmonary anastomosis (BCPA) and modified Fontan operations (MFO) in patients with a functionally single ventricle and heterotaxy syndrome and to reveal risk factors for these surgical interventions. During 1983-2010, 681 patients underwent BCPA or MFO. Thirty-nine had heterotaxy syndrome. The median follow-up period after BCPA and MFO was nine and 1.5 years, respectively. Risk factors for lethal outcomes were determined by logistic regression analysis. Hospital mortality after BCPA and MFO was 7.9% and 12.5%, respectively and did not significantly differ from patients without heterotaxy. The most frequent hospital complications were heart failure, pleural effusions, and arrhythmias. Late mortality after BCPA and MFO was 8.7% and did not significantly differ from patients without heterotaxy. Late deaths were caused by congestive heart failure or pulmonary thromboembolism. The main non-lethal complication was arrhythmia. Patients have significantly improved their functional class at follow-up. The independent risk factor for lethal outcomes after BCPA and MFO was preoperative regurgitation at atrioventricular valves (P=0.012). BCPA and MFO in patients with a functionally single ventricle and heterotaxy syndrome allow to significantly improves their quality of life. Preoperative regurgitation at atrioventricular valves worsens surgical results.

Increased postoperative and respiratory complications in patients with congenital heart disease associated with heterotaxy.

OBJECTIVE: Patients with heterotaxy and complex congenital heart disease underwent cardiac surgery with high mortality and morbidity. Recent studies have revealed an association among heterotaxy, congenital heart disease, and primary ciliary dyskinesia. We undertook a retrospective review of patients undergoing cardiac surgery at Children's National Medical Center between 2004 and 2008 to explore the hypothesis that there is increased mortality and respiratory complications in heterotaxy patients. METHODS: Retrospective review was performed on postsurgical outcomes of 87 patients with heterotaxy and congenital heart disease exhibiting the full spectrum of situs abnormalities associated with heterotaxy. As controls patients, 634 cardiac surgical patients with congenital heart disease, but without laterality defects, were selected, and surgical complexities were similar with a median Risk Adjustment in Congenital Heart Surgery-1 score of 3.0 for both groups. RESULTS: We found the mean length of postoperative hospital stay (17 vs 11 days) and mechanical ventilation (11 vs 4 days) were significantly increased in the heterotaxy patients. Also elevated were rates of tracheostomies (6.9% vs 1.6%; odds ratio, 4.6), extracorporeal membrane oxygenation support (12.6% vs 4.9%: odds ratio, 2.8), prolonged ventilatory courses (23% vs 12.3%; odds ratio, 2.1) and postsurgical deaths (16.1% vs 4.7%; odds ratio, 3.9). CONCLUSIONS: Our findings show heterotaxy patients had more postsurgical events with increased postsurgical mortality and risk for respiratory complications as compared to control patients with similar Risk Adjustment in Congenital Heart Surgery-1 surgical complexity scores. We speculate that increased respiratory complications maybe due to ciliary dysfunction. Further studies are needed to explore the basis for the increased surgical risks for heterotaxy patients undergoing cardiac surgery.

Biventricular repair of atrioventricular septal defect with common atrioventricular valve and double-outlet right ventricle.

BACKGROUND: The combination of an atrioventricular septal defect with a common atrioventricular junction guarded by a common valve, and double-outlet right ventricle, is a rare lesion that presents a challenge for surgical repair. This report describes our surgical approach and results in 16 patients undergoing biventricular repair for such a combination of lesions. METHODS: A retrospective analysis was performed for all patients undergoing biventricular repair of atrioventricular septal defect with common atrioventricular valve and double-outlet right ventricle between 1991 and 2008. Patients with tetralogy of Fallot and common atrioventricular valve were excluded from analysis. Early and actuarial outcomes were evaluated using the chi(2) test for categorical variables and Wilcoxon rank sum for ordinal variables. RESULTS: The median age at operation was 16 months. Heterotaxy syndrome was present in 12 of the 16 patients (9 right isomerism and 3 left isomerism), and 6 had concurrent totally anomalous pulmonary venous connections. Primary repair was achieved in 6 patients, and 10 underwent one or more prior operations (most frequently a shunt, banding of the pulmonary trunk, or repair of the anomalous pulmonary venous connections). Enlargement of the ventricular septal defect by resection of the muscular outlet septum was required in 11 patients, in whom the ventricular septal defect emptied entirely or primarily to the inlet of the right ventricle. A conduit was placed from the right ventricle to the pulmonary arteries in 13. There was 1 death before discharge from hospital, 1 late death, and 2 episodes of heart block. Among survivors, follow-up was complete with a median follow-up of 66 months. No patient had late obstruction of the left ventricular outflow tract. The presence of heterotaxy with totally anomalous pulmonary venous connections was associated with combined mortality and significant morbidity (p = 0.008). CONCLUSIONS: Although technically challenging, the surgical repair can be accomplished with acceptable early results. Heterotaxy syndrome, with concurrent anomalous connections of the pulmonary veins, represented the strongest identified risk factor for death or significant complication.

Heterotaxy syndrome with functional single ventricle: does prenatal diagnosis improve survival?

BACKGROUND: Despite improved outcome for many single ventricle lesions, staged reconstruction for heterotaxy syndrome with a functional single ventricle continues to have a high mortality. Prenatal identification of heterotaxy syndrome may improve long-term survival. METHODS: Our database was reviewed from January 1996 to December 2004 for patients with heterotaxy syndrome. Assessment was made for prenatal diagnosis and echocardiographic characteristics of heterotaxy syndrome. We sought to assess the accuracy of fetal echocardiography in the diagnosis of heterotaxy syndrome and determine whether prenatal diagnosis and other risk factors have an impact on survival in patients with heterotaxy syndrome. RESULTS: Of 81 patients that met criteria, 43 (53%) had prenatal diagnosis. Prenatal diagnosis had high specificity and positive predictive value for all findings but had low sensitivity for anomalous pulmonary veins. Among the 70 patients born alive, survival was 60% with median follow-up of 51.4 months (range, 6.5 to 109.7 months). Prenatal diagnosis did not improve survival (p = 0.09). None of the 11 patients with complete heart block (CHB) survived past 3 months of age. Two patients underwent heart transplantation as their first intervention and have survived. CHB and anomalous pulmonary venous connection were associated with shorter duration of survival. CONCLUSIONS: Prenatal diagnosis of heterotaxy syndrome does not improve survival in patients who undergo single ventricle reconstruction. The most potent risk factors for poor outcome (CHB, anomalous pulmonary veins) are likely not impacted by identification in utero. In light of the poor outcome, cardiac transplantation as an initial therapy may be a viable option for some patients.

[Long-term surgical results of total cavopulmonary connection in children with visceral heterotaxy syndrome; comparison between lateral tunnel method and extracardiac conduit method].

We reviewed our 58 experience of total cavopulmonary connection (TCPC) for children with visceral heterotaxy syndrome from 1992 to 1999. Surgical results, pre- and post-operative hemodynamics, and the incidence of cardiac-related events were compared between lateral tunnel method (LT) and extra-cardiac conduit method (EC). Overall survival rate was 89.6% in 8 years. Freedom from cardiac-related events was significantly lower in LT method compared with EC method (70.8% vs. 91.3% in 5 years and 67.8% vs. 91.3% in 8 years, p = 0.048). Extra-cardiac conduit TCPC following bi-directional Glenn shunt is the procedure of choice for children with visceral heterotaxy syndrome and functional single ventricle.

Heart transplantation in infants and children with situs inversus.

BACKGROUND: Recipient situs inversus has always represented a technical challenge during heart transplantation. OBJECTIVE: A simplified operative strategy for heart transplantation in a recipient with atrial situs inversus is described. METHODS: Fifteen pediatric recipients with situs inversus accompanying other complex congenital heart disease or dilated cardiomyopathy having "orthotopic" heart allotransplantation in one center, between 1985 and 1997, were reviewed retrospectively. A nearly uniform, simplified technical approach to transplantation was used and is described. RESULTS: Fourteen of these recipients with complex malformations survived the transplantation. Morbidity relating to surgical technique has been limited to partial (n = 2) or complete (n = 1) late obstruction of superior vena caval drainage; each case was managed successfully by interventional cardiologic techniques. Actuarial survival after transplantation compares favorably with that among 290 infants and children with atrial situs solitus who underwent heart transplantation. CONCLUSIONS: Systemic atrial malposition, including situs inversus, does not limit successful heart transplantation by the simplified method described.

Prolonged survival with isolated levocardia and situs inversus.

Isolated levocardia with situs inversus, or ILSI, is a rare anomaly with a reported incidence of 1:22,000 in the general population and from 0.4% to 1.2% of all patients with congenital heart diseases. It is commonly associated with both congenital heart disease and splenic abnormalities, either asplenia or polysplenia. The prognosis is poor, and only about 5% to 13% of patients survive more than 5 years. The case described here, which had computed tomographic findings, is the first case reported with multiple malignancies associated with ILSI. This patient, at 73 years of age, is the second longest survivor reported in the literature.