The Role of Novel Transcatheter Procedures in Patients With Congenital Heart Disease.

The development of percutaneous structural interventions in patients with acquired heart disease is happening at an exponential rate, and some of this technology is being used to treat patients with congenital heart disease. This review describes the pathophysiology of valvular abnormalities specific to congenital heart disease and discusses the application of structural procedures in this population. Although the overall experience has been encouraging, especially in high-risk patients, this article will highlight the reasons that a cautious approach to adoption of this technology is necessary in these patients.

Outcomes of Adults with Congenital Heart Disease Supported with Extracorporeal Life Support After Cardiac Surgery.

Patients with adult congenital heart disease (ACHD) who undergo cardiac surgery may require extracorporeal life support (ECLS) for cardiopulmonary support, but outcomes after ECLS support have not been well described. This study aimed to identify risk factors for ECLS mortality in this population. We identified 368 ACHD patients who received ECLS after cardiac surgery between 1994 and 2016 in the Extracorporeal Life Support Organization (ELSO) database, a multicenter international registry of ECLS centers. Risk factors for mortality were assessed using multivariate logistic regression. Overall mortality was 61%. In a multivariate model using precannulation characteristics, Fontan physiology (odds ratio [OR]: 5.7; 95% CI: 1.6-20.0), weight over 100 kg (OR: 2.6; 95% CI: 1.3-5.4), female gender (OR: 1.6; 95% CI: 1.001-2.6), delayed ECLS cannulation (OR: 2.0; 95% CI: 1.2-3.2), and neuromuscular blockade (OR: 1.9; 95% CI: 1.1-3.3) were associated with increased mortality. Adding postcannulation characteristics to the model, renal complications (OR: 3.0; 95% CI: 1.7-5.2), neurologic complications (OR, 4.7; 95% CI: 1.5-15.2), and pulmonary hemorrhage (OR: 6.4; 95% CI: 1.3-33.2) were associated with increased mortality, whereas Fontan physiology was no longer associated, suggesting the association of Fontan physiology with mortality may be mediated by complications. Fontan physiology was also a risk factor for neurologic complications (OR: 8.2; 95% CI: 3.3-20.9). Given the rapid increase in ECLS use, understanding risk factors for ACHD patients receiving ECLS after cardiac surgery will aid clinicians in decision-making and preoperative planning.

Value of a flow cytometry cross-match in the setting of a negative complement-dependent cytotoxicity cross-match in heart transplant recipients.

Complement-dependent cytotoxicity cross-match (CDCXM) is used for evaluation of preformed HLA-specific antibodies in patients undergoing heart transplantation. Flow cytometry cross-match (FCXM) is a more sensitive assay and used with increasing frequency. To determine the clinical relevance of a positive FCXM in the context of negative CDCXM in heart transplantation, the United Network for Organ Sharing (UNOS) database was analyzed. Kaplan-Meier analysis and Cox proportional hazard modeling were used to assess graft survival for three different patient cohorts defined by cross-match results: T-cell and B-cell CDCXM+ ("CDCXM+" cohort), CDCXM- but T-cell and/or B-cell FCXM+ ("FCXM+" cohort), and T-cell/B-cell CDCXM- and FCXM- ("XM-" cohort). During the study period, 2558 patients met inclusion criteria (10.7% CDCXM+, 18.8% FCXM+, 65.5% XM-). CDCXM+ patients had significantly decreased graft survival compared to FCXM+ and XM- cohorts (P = .003 and <.001, respectively). CDCXM- and FCXM+ patients did not have decreased graft survival compared to XM- patients (P = .09). In multivariate analysis, only CDCXM+ was associated with decreased graft survival (HR 1.22, 95% CI 1.01-1.49). In conclusion, positive FCXM in the context of negative CDCXM does not confer increased risk of graft failure. Further study is needed to understand implications of CDCXM and FCXM testing in heart transplant recipients.

Changes in the methodology of pre-heart transplant human leukocyte antibody assessment: an analysis of the United Network for Organ Sharing database.

BACKGROUND: We sought to investigate temporal trends in the methodology of human leukocyte antibody assessment in heart transplantation. METHODS: The United Network for Organ Sharing database was queried from June 2004 to March 2013 to obtain pre-heart transplantation human leukocyte antibody results. The % panel reactive antibody for class I and II antibodies was recorded along with the methodology of assessment. Allosensitization was defined as class I and/or II panel reactive antibody of ≥ 10%. The primary outcome measure was graft survival. RESULTS: During the study period, 12,858 patients with available data underwent heart transplantation. The prevalence of allosensitization increased, with 16.8% in 2005-2006 sensitized at the time of transplantation compared to 23.1% in 2010-2011 (p < 0.001); this occurred in conjunction with an increase in the utilization of flow cytometry (77.2% in 2005-2006; 97.0% in 2010-2011, p < 0.001). Using multivariable analysis, a positive pre-heart transplantation panel reactive antibody by flow cytometry independently predicted graft loss. CONCLUSIONS: There has been a recent increase in flow cytometric assessment of human leukocyte antibodies prior to heart transplantation, which may be associated with an increase in the prevalence of pre-transplant patients being characterized as allosensitized. Flow cytometry may identify patients with the highest likelihood of graft loss.

Lung transplant waitlist mortality: height as a predictor of poor outcomes.

The LAS was designed to minimize pretransplant mortality while maximizing post-transplant outcome. Recipients <12 are not allocated lungs based on LAS. Waitlist mortality has decreased for those >12, but not <12, suggesting this population may be disadvantaged. To identify predictors of waitlist mortality, a retrospective analysis of the UNOS database was performed since implementation of the LAS. There were 16,973 patients listed for lung transplant in the United States; 12,070 (71.1%) were transplanted, and 2498 (14.7%) patients died or were removed from the wait list. Significantly more pediatric patients died or were removed compared with adults (22.0% vs. 14.4%, p < 0.01). In multivariate analysis, in addition to higher LAS at time of listing (adj. HR1.058, 1.055-1.060), shorter height (1.008, 1.006-1.010), male gender (1.210, 1.110-1.319), and requiring ECMO (1.613, 1.202-2.163) were associated with pretransplant mortality. Post-transplant survival was not affected by height. The current age cutoff may impose limitations within the current lung allocation system in the United States. Height is an independent predictor of waitlist mortality and may be a valuable factor for the development of a comprehensive lung allocation system.

Impact of congenital heart disease on outcomes of pediatric heart-lung transplantation.

HLT is reserved for children with cardiopulmonary disease not amendable to alternative therapies. Children with CHD with or without ES may be considered for HLT. Outcomes of HLT in this population are not well described. To test the hypothesis that CHD without ES is associated with worse graft survival and identify factors associated with poor outcome, a retrospective analysis of the UNOS database was performed. One hundred and seventy-eight pediatric HLTs were performed between 1987 and 2011. CHD was the diagnosis in 65 patients, of which 34 had CHD without ES. Patients with CHD without ES had decreased patient survival (median 1.31 yr) compared with CHD with ES (4.80 yr, p = 0.05). On multivariable analysis, the following were associated with graft failure: CHD without ES (adjusted HR 1.69, 95% CI 1.09-2.62), younger age (1.04, 1.01-1.08), pretransplant mechanical ventilation (1.75, 1.01-3.06), pretransplant ECMO (3.07, 1.32-7.12), pretransplant PRAs (1.53, 1.06-2.20), and transplant era (1.85, 1.16-2.94). In children with CHD who require HLT, underlying physiology influences outcomes. Those without ES have a worse prognosis. The diagnosis of CHD without ES and preoperative factors may inform decisions in a complex patient population.