Joseph W. Rossano

The Registry of the International Society for Heart and Lung Transplantation: Thirty-second Official Adult Lung and Heart-Lung Transplantation Report—2015; Focus Theme: Early Graft Failure

This section of the 32nd official International Society for Heart and Lung Transplantation (ISHLT) Registry Report of 2015 summarizes data from 51,440 adult lung and 3,820 adult heart-lung transplants that occurred through June 30, 2014. This publication reports data for donor and recipient characteristics, transplant events, and recipient treatments and outcomes. This Registry Report focuses on an overall theme of recipient early graft failure. The Registry’s online full slide set provides more detail, additional analyses, and other information not included in this publication.

The Registry of the International Society for Heart and Lung Transplantation: Thirty-second Official Adult Heart Transplantation Report - 2015; Focus Theme: Early Graft Failure

Data are submitted to the ISHLT Registry by national and multinational organ/data exchange organizations and individual centers. Since the Registry’s inception, 418 heart transplant centers, 242 lung transplant centers and 174 heart–lung transplant centers have reported data. The Registry website (www.ishlt.org/registries) provides spread sheets that show data elements collected in the Registry. The online slide set (http://www.ishlt.org/registries/slides.asp? slides=heartLungRegistry) provides POWERPOINT slides of figures and tables that support this study. The site contains additional slides for this report and slide sets from the previous annual reports.

Mortality and Sudden Death in Pediatric Left Ventricular Noncompaction in a Tertiary Referral Center

Background— Left ventricular noncompaction is a cardiomyopathy characterized by excessive trabeculation of the left ventricle, progressive myocardial dysfunction, and early mortality. Left ventricular noncompaction has a heterogeneous clinical presentation that includes arrhythmia and sudden cardiac death. Methods and Results— We retrospectively reviewed all children diagnosed with left ventricular noncompaction at Texas Children’s Hospital from January 1990 to January 2009. Patients with congenital cardiac lesions were excluded. Two hundred forty-two children were diagnosed with isolated left ventricular noncompaction over the study period. Thirty-one (12.8%) died, and 13 (5.4%) were received a transplant. One hundred fifty (62%) presented with or developed cardiac dysfunction. The presence of cardiac dysfunction was strongly associated with mortality (hazard ratio, 11; P<0.001). ECG abnormalities were present in 87%, with ventricular hypertrophy and repolarization abnormalities occurring most commonly. Repolarization abnormalities were associated with increased mortality (hazard ratio, 2.1; P=0.02). Eighty children (33.1%) had an arrhythmia, and those with arrhythmias had increased mortality (hazard ratio, 2.8; P=0.002). Forty-two (17.4%) had ventricular tachycardia, with 5 presenting with resuscitated sudden cardiac death. In total, there were 15 cases of sudden cardiac death in the cohort (6.2%). Nearly all patients with sudden death (14 of 15) had abnormal cardiac dimensions or cardiac dysfunction. No patient with normal cardiac dimensions and function without preceding arrhythmias died. Conclusions— Left ventricular noncompaction has a high mortality rate and is strongly associated with arrhythmias in children. Preceding cardiac dysfunction or ventricular arrhythmias are associated with increased mortality. Children with normal cardiac dimensions and normal function are at low risk for sudden death.

The Registry of the International Society for Heart and Lung Transplantation: Thirty-fourth Adult Heart Transplantation Report-2017; Focus Theme: Allograft ischemic time

This year marks the 50th anniversary of the first heart transplant, performed in 1967. Since then, and in particular since the introduction of cyclosporine immunosuppression in the 1970s, heart transplantation has grown worldwide. This 34th adult heart transplant report is based on data submitted to the International Society for Heart and Lung Transplantation (ISHLT) Registry on 135,387 heart transplants in recipients of all ages (including 120,991 adult heart transplants) through June 30, 2016. With each year’s report we now also provide more detailed analyses on a particular focus theme. Since 2013, these have been donor and recipient age, retransplantation, early graft failure, indication for transplant, and in 2017, allograft ischemic time.

Risk factors and mode of death in isolated hypertrophic cardiomyopathy in children.

OBJECTIVES This study was designed to review outcomes of pediatric isolated hypertrophic cardiomyopathy (HCM) managed uniformly at a single institution and assess whether reported adult risk factors for sudden death are predictive in pediatric HCM. BACKGROUND Cardiac death in HCM occurs suddenly (SCD) or may be nonsudden (non-SCD). Little data exists on non-SCD in children. Risk factors for SCD in adult HCM are characterized and consensus management strategies detailed. Their application to children is uncertain and treatment strategies vary. METHODS A retrospective cohort study of children with HCM was performed. Primary end points were cardiac death and transplantation. Frequency and outcomes of known adult risk factors were assessed. Outcomes analysis was performed using Kaplan-Meier curves and Cox regression analysis. RESULTS Ninety-six patients were included. The average age at diagnosis was 10.6 +/- 5.4 years, and mean follow-up was 6.4 +/- 5.2 years. Primary end points occurred in 11 patients over the 20-year follow-up (11%), 4 underwent cardiac transplant and 7 died (3 suddenly). Extreme left ventricular hypertrophy (z-score: >6) and an abnormal blood pressure response to exercise were predictive of non-SCD (p < 0.02 and p < 0.03, respectively). Kaplan-Meier survival analysis predicts an 82% survival over a 20-year period. CONCLUSIONS In children with isolated HCM managed primarily with exercise restriction and medication, cardiac death occurred infrequently. Non-SCD or transplant was at least as common as SCD. Extreme left ventricular hypertrophy and blunted blood pressure response to exercise were associated with an increased risk of non-SCD.

The Registry of the International Society for Heart and Lung Transplantation: Eighteenth Official Pediatric Heart Transplantation Report—2015; Focus Theme: Early Graft Failure

Data are submitted to the ISHLT Registry by national and multinational organ/data exchange organizations and individual centers. Since the Registry’s inception, 418 heart transplant centers, 242 lung transplant centers and 174 heart–lung transplant centers have reported data. The Registry website (www.ishlt.org/registries) provides spread sheets that show data elements collected in the Registry. The online slide set (http://www.ishlt.org/registries/slides.asp? slides=heartLungRegistry) provides POWERPOINT slides of figures and tables that support this study. The site contains additional slides for this report and slide sets from the previous annual reports.

Outcomes of pediatric patients supported by the HeartMate II left ventricular assist device in the United States

OBJECTIVE The HeartMate II (HMII; Thoratec, Pleasanton, CA) continuous-flow left ventricular assist device (LVAD) is an established treatment modality for advanced heart failure in adults. The objective of this study was to evaluate outcomes of pediatric patients supported by the HMII LVAD. METHODS This was a retrospective review of the Interagency Registry for Mechanically Assisted Circulatory Support (INTERMACS) of patients supported with a HMII from April 2008 to September 2011. The primary cohort comprised pediatric patients aged 11 to 18 years. Outcomes were compared with a group of young adults aged 19 to 39 years who underwent HMII implant during the same period. Ischemic etiologies for heart failure were excluded. RESULTS There were 28 pediatric patients, of whom 19 (68%) were males, 14 (46%) were African American, and 7 (25%) underwent device placement in a pediatric hospital. Competing outcomes analysis showed that at 6 months of follow-up, the composite of survival to transplantation, ongoing support, or recovery was 96% for the pediatric group, which was not significantly different from the young adult group (96%, p = 0.330). The 2 groups had similar INTERMACS profiles but differed in diagnosis, weight, and morbidities. Bleeding complications requiring surgical intervention were more common in the pediatric group. CONCLUSIONS Pediatric outcomes with a HMII LVAD are comparable to that of young adults. As we continue to monitor this growing group, more sophisticated characterization and comparisons will be possible. Also, as technology progress and second- and third-generation devices are introduced, the number of children who will benefit from mechanical support will continue to grow.

Recovery of Echocardiographic Function in Children with Idiopathic Dilated Cardiomyopathy: Results from the Pediatric Cardiomyopathy Registry

OBJECTIVES This study sought to determine the incidence and predictors of recovery of normal echocardiographic function among children with idiopathic dilated cardiomyopathy (DCM). BACKGROUND Most children with idiopathic DCM have poor outcomes; however, some improve. METHODS We studied children <18 years of age from the Pediatric Cardiomyopathy Registry who had both depressed left ventricular (LV) function (fractional shortening or ejection fraction z-score <-2) and LV dilation (end-diastolic dimension [LVEDD] z-score >2) at diagnosis and who had at least 1 follow-up echocardiogram 30 days to 2 years from the initial echocardiogram. We estimated the cumulative incidence and predictors of normalization. RESULTS Among 868 children who met the inclusion criteria, 741 (85%) had both echocardiograms. At 2 years, 22% had recovered normal LV function and size; 51% had died or undergone heart transplantation (median, 3.2 months), and 27% had persistently abnormal echocardiograms. Younger age (hazard ratio [HR]: 0.92; 95% confidence interval [CI]: 0.88 to 0.97) and lower LVEDD z-score (HR: 0.78; 95% CI: 0.70 to 0.87) independently predicted normalization. Nine children (9%) with normal LV function and size within 2 years of diagnosis later underwent heart transplantation or died. CONCLUSIONS Despite marked LV dilation and depressed function initially, children with idiopathic DCM can recover normal LV size and function, particularly those younger and with less LV dilation at diagnosis. Investigations related to predictors of recovery, such as genetic associations, serum markers, and the impact of medical therapy or ventricular unloading with assist devices are important next steps. Longer follow-up after normalization is warranted as cardiac failure can recur. (Pediatric Cardiomyopathy Registry; NCT00005391).

Impact of antibodies against human leukocyte antigens on long-term outcome in pediatric heart transplant patients: An analysis of the United Network for Organ Sharing database

OBJECTIVES Controversy exists regarding the importance of circulating antibodies as determined by panel-reactive antibody screening as a risk factor for graft failure in pediatric patients undergoing heart transplantation. This study sought to determine the association of elevated anti-human leukocyte antibodies with long-term survival in pediatric heart transplant patients. METHODS The United Network for Organ Sharing registry was queried for pediatric patients (aged < 18 years at listing) with panel-reactive antibody levels obtained before heart transplantation from 1987 through 2004. Survival analysis methods were used to assess the association of elevated panel-reactive antibodies with long-term graft and patient survival. RESULTS Panel-reactive antibodies were obtained before transplantation from 3534 patients, median age 4 years (interquartile range 0-12 years). Most, 2711 (77%), had no detectable panel-reactive antibodies, 436 (12%) had panel-reactive antibodies of 1% to 10%, and 387 (11%) had panel-reactive antibodies greater than 10%. Patients with panel-reactive antibodies greater than 10% were more likely to be older (P = .04), have congenital heart disease (P < .001), and have a longer wait list time (P = .006). Patients with panel-reactive antibodies greater than 10% had significantly worse graft survival and patient survival than did patients with undetectable panel-reactive antibodies and panel-reactive antibodies of 1% to 10% (P < .05 for all). Controlling for confounding variables, elevated panel-reactive antibodies as a continuous variable and panel-reactive antibodies greater than 10% as a categorical variable were independently associated with decreased graft survival (P = .04 and P = .02, respectively). CONCLUSIONS Elevated panel-reactive antibodies are independently associated with worse long-term graft survival in pediatric patients undergoing heart transplantation. Further study is needed to determine the optimal management of this high-risk population.

Successful Use of the Total Artificial Heart in the Failing Fontan Circulation

Typical left ventricular assist devices are often ineffective for the failing Fontan circulation. We report the first successful use of a total artificial heart as a bridge to transplant in a patient who had previously undergone a Fontan operation.