Abstract 14116: Favorable Outcomes for Heart Transplantation in Barth Syndrome as Demonstrated by Multicenter/multiregistry Analysis

Circulation ◽  
2020 ◽  
Vol 142 (Suppl_3) ◽  
Author(s):  
Yu Li ◽  
Justin Godown ◽  
carolyn taylor ◽  
Anne I Dipchand ◽  
Brian Feingold
Keyword(s):  
Acute Rejection ◽  
Heart Transplant ◽  
Mechanical Circulatory Support ◽  
Case Reports ◽  
Barth Syndrome ◽  
Circulatory Support ◽  
Transplant Recipients ◽  
Morbidity Data ◽  
Pediatric Heart Transplant

Introduction: Barth Syndrome (BTHS) is a rare (~1/350,000), X-linked mitochondrial disease characterized by cardioskeletal myopathy and neutropenia. Reported outcomes after heart transplant (HT) are limited to case reports. We sought to identify a large cohort of BTHS HT recipients to describe clinical outcomes. Hypothesis: HT in BTHS is associated with non-inferior survival, acute rejection (AR), infection, and vasculopathy (CAV) relative to non-BTHS dilated cardiomyopathy (DCM). Methods: We analyzed data from the Barth Syndrome Registry and Repository (BRR), Pediatric Heart Transplant Society (PHTS), and Scientific Registry of Transplant Recipients (SRTR). To avoid recounting patients occurring in >1 source, we used years of birth, listing, and HT and listing/HT city to link records across registries. BTHS HT recipients were matched 1:4 on age, era, urgency status, and use of ECMO/VAD to male, non-BHTS, DCM HT recipients in PHTS. Survival was analyzed for all BTHS HT recipients. Because BRR and SRTR morbidity data are limited, AR, infection, malignancy, and CAV were analyzed only for those with PHTS data. Results: Forty-seven BTHS patients with 51 listings and 43 HTs (including 2 re-transplants) were identified; 29 BTHS HTs (1 re-transplant) had data in PHTS. Median age at HT was 1.9 yrs (IQR: 0.6-5.8) with 35% <1 years-old at HT. Median follow up was 4.2 yrs (2.1-9.1). Mechanical circulatory support at HT was common (VAD 29%, ECMO 5%). We found no difference in survival between BTHS and non-BTHS HT recipients (HR 0.93, 95% CI 0.40-2.14). Freedom from infection (HR 0.63, 0.33-1.20), bacterial infection (HR 0.76, 0.29-1.99), malignancy (HR 0.22, 0.02-2.02), and CAV (HR 0.55, 0.15-1.97) were also similar between the groups. BTHS HT recipients had greater freedom from AR (HR 0.39, 0.17-0.87). Use of induction therapy (62 vs 74%, p=0.21), steroids at 30 days (76 vs 62%, p=0.17), and dual or triple immunosuppression at 1 year (80 vs 84%, p=0.58) were similar for BTHS and non-BTHS recipients. Conclusions: In this largest cohort yet reported, BTHS HT recipients show equivalent survival and freedom from infection, malignancy, and CAV, with a lower risk of acute rejection. Thus, individuals with BTHS should not be excluded from HT solely based on the diagnosis of BTHS.

Advantages of C2 Monitoring to Avoid Acute Rejection in Pediatric Heart Transplant Recipients

2006 ◽  
Vol 25 (6) ◽  
pp. 619-625 ◽  
Author(s):  
S SCHUBERT ◽  
H ABDULKHALIQ ◽  
H LEHMKUHL ◽  
M HUBLER ◽  
M ABDELRAHMAN ◽  
...  
Keyword(s):  
Acute Rejection ◽  
Heart Transplant ◽  
Transplant Recipients ◽  
C2 Monitoring ◽  
Heart Transplant Recipients ◽  
Pediatric Heart Transplant

scholarly journals Does cardiac transplant protect against broken hearts?

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
A Isath ◽  
S Perembeti ◽  
A Correa ◽  
S Rao ◽  
A Chahal ◽  
...  
Keyword(s):  
Heart Transplant ◽  
Average Length ◽  
Myocardial Dysfunction ◽  
Case Reports ◽  
Fold Increase ◽  
Circulatory Support ◽  
Cardiac Transplant ◽  
Transplant Recipients ◽  
The Mean ◽  
Heart Transplant Recipients

Abstract Background Takotsubo cardiomyopathy (TC) is a reversible stress-induced myocardial dysfunction with increased sympathetic activity caused by excessive release of catecholamines playing a central role in its pathophysiology. The occurrence of TC in transplanted hearts is rare given the complete denervation done during transplantation. However, it has been demonstrated that 40% of transplant recipients undergo sympathetic re-innervation. There have only been case reports describing TC in post-transplant recipients. Purpose To evaluate the incidence, baseline characteristics and outcomes of TC occurring in heart transplant recipients using Healthcare Cost and Utilization Project (HCUP) National (nationwide) Inpatient Sample (NIS) in United States from 2009 to 2014. Methods Using NIS data, we identified patients who underwent cardiac transplantation using ICD9 procedure codes 37.5 and 33.6. Among these patients, we identified those admitted to the hospital with diagnosis of TC based on ICD-9-CM code 429.83. We presented categorical data as percentages and continuous data as mean or median as appropriate. Results We identified 257 hospitalizations for TC in heart transplant recipient patients. There was an approximately 9-fold increase in admissions from 11 in 2009 to 95 in 2014. Among patients with TC, the mean age was 65.3±1.8 years and majority were female (76.6%). A majority of patients were Caucasians (63%) followed by smaller proportion of African-Americans (13.2%). In-patient mortality in patients admitted with TC following heart transplant was 11.3% (n=29). During the hospitalization, 4.8% of patients had cardiogenic shock and 2.8% required mechanical circulatory support. The average length of stay for patients with TC was 16.6±3.3 days. The mean cost of hospitalization for these patients when adjusted for inflation were 237248±55709 dollars. Conclusion TC can still occur in substantially in heart transplant recipients and should be considered one of the differential diagnosis in transplant patients presenting to the hospital. Funding Acknowledgement Type of funding source: None

Abstract 2965: QT Interval Prolongation during Severe Acute Rejection is Predictive of Sudden Cardiac Death in Heart Transplant Recipients

Circulation ◽  
2008 ◽  
Vol 118 (suppl_18) ◽  
Author(s):  
Bojan Vrtovec ◽  
Francois Haddad ◽  
Vivian Tsai ◽  
Amin Al-Ahmad ◽  
Tobias Deuse ◽  
...  
Keyword(s):  
Acute Rejection ◽  
Heart Transplant ◽  
Qt Interval ◽  
Cardiac Death ◽  
Rejection Episode ◽  
Transplant Recipients ◽  
Interval Prolongation ◽  
Qt Interval Prolongation ◽  
Heart Transplant Recipients

Background. QT interval prolongation is considered a risk factor for sudden cardiac death (SCD) in various non-transplant populations. Since acute allograft rejection is associated with a prolonged QT interval, we sought to investigate the effects of rejection-induced QT interval changes on SCD in heart transplant recipients. Methods. Of all patients who underwent heart transplantation between 1998 and 2007, we enrolled those with severe acute cellular rejection episodes (ISHLT grade 3A or higher, accompanied with hemodynamic compromise). In this cohort, baseline ECGs were obtained within 7 days after transplantation; follow-up ECGs were recorded at the time of the rejection episode. On all ECGs, QT interval was defined as the mean duration QT interval measurements in all leads, and corrected for heart rate with Bazett formula. A significant increase of QTc during rejection (dQTc) was defined as a relative change in QTc ≥10%. Patients were followed for SCD for 1 year after the rejection episode. Results. Of 80 patients with a severe rejection episode, 9 (11%) were excluded because of the inadequate quality of ECG recordings. Within the 1-year follow-up, 21 of 71 (30%) patients died. Of these, 14 (67%) died of SCD, and 7 (23%) died of other causes. Patients who died of SCD and survivors did not differ with regards to age (44 ± 12 years in SCD group vs. 46 ± 16 years in survivors, P = 0.61), gender (male: 83% vs. 58%, P = 0.06), incidence of infections (0.21 ± 0.43 vs. 0.20 ± 0.40, P = 0.91), malignancy (0% vs. 8%, P = 0.84), or allograft vasculopathy (29% vs. 26%, P = 0.85). No difference in Qtc prolonging drug was noted. During acute rejection, QTc interval was significantly longer in SCD group than in survivors (475 ± 57 ms vs. 437 ± 36 ms, P = 0.01), and the same was true for the incidence of dQTc > 10% (50% vs. 16%, P = 0.008). SCD-free survival as evaluated by Kaplan-Meier analysis was significantly lower in patients with dQTc > 10% (P = 0.013). On multivariate analysis, dQTc > 10% was the only independent predictor of SCD (P = 0.02). The multivariate model included left ventricular dysfunction (LVEF < 30%) and dQTc. Conclusion. Heart transplant recipients who display a significant (> 10%) prolongation of QT interval during a severe acute rejection episode may be at increased risk for SCD.

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