Prognostic Value of Exercise Stress Echocardiography in Pediatric Cardiac Transplant Recipients.

BACKGROUND: Cardiac allograft vasculopathy (CAV) is a leading cause of long-term morbidity and mortality in pediatric heart transplant (HTx) recipients. Exercise stress echocardiography (ESE) has been shown to be useful in the detection of angiographically confirmed coronary artery disease in children. However, the prognostic utility of ESE for prediction of cardiac events in HTx survivors is unknown. OBJECTIVES: We aim to assess whether an abnormal (positive) ESE is be associated with a higher risk of future cardiovascular (CV) outcomes in pediatric HTx recipients. METHODS: We conducted a retrospective review of CV outcomes in a cohort of 95 pediatric HTx recipients who underwent 188 ESEs over a 10-year period. A composite endpoint for CV events including myocardial infarction, hospitalization for nonrejection heart failure, coronary revascularization, need for repeat transplantation, and death was used. Based on the interpretation of the ESE results, each ESE study was classified for this study as either positive (abnormal) or negative (normal) for ischemia. Results of the coronary angiograms performed near the time of ESE were also assessed and classified for this study as positive (abnormal) or negative (normal) for CAV according to standard HTx criteria for CAV. RESULTS: Fifty-one (27%) ESEs were positive for ischemia. There was a total of 35 CV events in 23 patients. A positive ESE was associated with increased risk of any CV event (hazard ratio = 3.55; 95% CI, 1.52, 8.28), as well as an increased risk of CV death (hazard ratio = 3.19; 95% CI, 1.23, 8.28). Freedom from composite CV outcome at 1, 2, and 3 years following a positive ESE was 89.9% (95% CI = 77.3%, 95.7%), 81.5% (95% CI = 65.9%, 90.5%), and 63.2% (95% CI = 41.9%, 78.5%), respectively. Freedom from composite CV outcome at 1, 2, and 3 years following a negative ESE was 99.3% (94.8, 99.9), 98.4% (93.6, 99.6), and 97.0% (90.6, 99.1), respectively. No patient died within 1 year of a negative ESE. CONCLUSIONS: In this largest study of ESE in pediatric HTx recipients, a positive or abnormal ESE is associated with increased future CV morbidity and mortality. Conversely, a negative ESE can help predict CV event-free survival. Even in the setting of a normal coronary angiogram, our pilot data show that an abnormal ESE may still be clinically important. Use of ESE in follow-up may improve risk stratification and management of pediatric HTx recipients.

Vo2peak in Adult Survivors of Hodgkin Lymphoma: Rate of Decline, Sex Differences, and Cardiovascular Events.

BACKGROUND: Adult survivors of Hodgkin lymphoma (HL) are at increased risk of cardiovascular (CV) events secondary to mediastinal radiation therapy (RT). OBJECTIVES: In this group of patients, we assessed the association between cardiopulmonary exercise testing (CPET), as determined by percent-predicted peak Vo2 (ppVo2peak), and clinical outcomes, as well as the rate of ppVo2peak decline and sex differences. METHODS: All survivors of HL who were >10 years post chest RT and who underwent ≥1 CPET were enrolled from a single center. Traditional CV and treatment risk factors, along with CV events, were ascertained. RESULTS: A total of 64 patients (67% female; median age 51 years [range 26 to 70 years]) with a median follow-up time after RT of 23 years (range 11 to 41 years), and 141 CPET studies, were included. Median initial ppVo2peak was 91% (range 58% to 138%). ppVo2peak in survivors declined by 7.5 percentage points every 10-year period after RT, as compared with age- and sex-based norms (P = 0.001), even after adjusting for hypertension and history of anthracycline. Both male and female patients had a similar rate of ppVo2peak decline. However, women had a lower ppVo2peak at all times, and they developed abnormal ppVo2peak (≤85%) on average earlier than men (24.1 years vs 47.0 years after RT). Patients with abnormal ppVo2peak vs normal ppVo2peak (>85%), had an increased risk of CV events (59% vs 16%). Abnormal ppVo2peak was independently associated with the risk of CV events (adjusted HR: 6.37; 95% CI: 2.06-19.80; P = 0.001). CONCLUSIONS: Percent-predicted Vo2peak in long-term survivors of HL who were treated with chest RT progressively declined as compared with population- and sex-based norms. Importantly, women developed abnormal ppVo2peak more than 2 decades earlier than male survivors. Abnormal ppVo2peak was associated with an increased risk of CV events in this group of patients.

Value of Exercise Stress Echocardiography in Children with Hypertrophic Cardiomyopathy.

BACKGROUND: Exercise stress echocardiography (ESE) is a valuable diagnostic and prognostic tool in adults with hypertrophic cardiomyopathy (HCM). Inducible and resting left ventricular outflow tract gradients are important predictors of heart failure. However, there are minimal data on the utility of this modality in children. METHODS: Retrospective review of all pediatric HCM patients who underwent ESE at Boston Children's Hospital (January 2007-June 2018) was carried out. Patients were assigned to one of three categories based on left ventricular outflow tract gradients: group 1: <30 mm Hg at rest and exercise; group 2: <30 mm Hg at rest and ≥30 mm Hg with exercise; and group 3: ≥ 30 mm Hg at rest and exercise. Records were reviewed for earliest occurrence of composite endpoint of any one of the following: cardiac syncope, chest pain, nonsustained and sustained ventricular tachycardia, aborted cardiac arrest, heart failure class ≥ II, or HCM-related death/transplantation. RESULTS: A total of 91 children (67% males) with median age 12 years (6-24 years) at first ESE and median left ventricle wall thickness of 20 mm formed the cohort. Median follow-up duration was 3 years. During ESE, only one child experienced an event and was resuscitated. Of the 91 children, 25 were classified as group 1, 40 as group 2, and 26 as group 3. Twenty-six patients met the composite endpoint, including two heart transplant, one aborted cardiac arrest, and one sudden cardiac death. Group 3 patients had a 5 times higher risk of developing symptoms and/or serious clinical outcome at any age (hazard ratio = 5.18; 95% CI, [1.39-19.2]; P = .014). During our short follow-up time, group 2 patients had a higher risk of outcome, but this did not achieve statistical significance (hazard ratio = 1.95; 95% CI, [0.5-7.6]; P = .33). CONCLUSIONS: In this large series of pediatric patients with HCM, ESE can be performed safely and served as an effective tool to identify the lowest risk patients for cardiac outcome.

Heart Transplantation in Muscular Dystrophy Patients: Is it a Viable Option?

BACKGROUND: Cardiomyopathy is a common complication among muscular dystrophy (MD) patients and often results in advanced heart failure and premature death. In spite of this, there is hesitancy to consider heart transplantation (HTx). This study describes the HTx outcomes in patients with MD in the United States. METHODS AND RESULTS: All HTx in the United Network for Organ Sharing database from October 1, 1987, to March 31, 2016, were identified. Two patient groups were created: MD cohort (n=81), and a cohort of all other cardiomyopathies, called cardiomyopathy-unmatched (n=41 317). Propensity score matching (ratio 1:2) was performed on transplant age, gender transplant year, renal function, and inotropic support at transplant to form a cardiomyopathy-matched cohort (n=162). Patient characteristics and posttransplant outcomes were compared. In the 81 patients with MD, Becker was the most common type (42%-52%). All the analyzed preoperative characteristics did not statistically differ between the MD and cardiomyopathy-matched cohorts except ventricular assist device use (16% versus 30%; P=0.017), ventilator support (0% versus 6%; P=0.031), and donor race mismatch (30% versus 55%; P<0.001). Median time on waitlist was not statistically different between the 2 groups (52 versus 59 days; P=0.12). Posttransplant survival of MD cohort was not statistically different compared with cardiomyopathy-matched cohort (P=0.18; hazard ratio [95% CI], 0.71 [0.42-1.18]) and was better than the cardiomyopathy-unmatched cohort (P=0.004; hazard ratio [95% CI], 0.53 [0.34-0.82]). Among the types of MD, no statistical difference was observed in posttransplant survival of Becker MD versus non-Becker MD (P=0.12; hazard ratio [95% CI], 2.17 [0.79-6.01]). CONCLUSIONS: Patients with MD undergoing HTx had similar long-term posttransplant survival compared with matched cardiomyopathy-related HTx recipients. HTx appears to be an effective treatment for a select group of muscular dystrophy patients with end-stage heart failure.

Mechanical Support for Patients With Congenitally Corrected Transposition of the Great Arteries and End-Stage Ventricular Dysfunction.

Despite great advances in caring for patients with congenitally corrected transposition of the great arteries (ccTGA), a high proportion of these patients go on to develop heart failure and death in early adulthood. Adults with congenital heart disease (ACHD) only comprise a small number of patients receiving ventricular assist devices (VAD), but ccTGA accounted for 36% of ACHD patients in the INTERMACS database. Review of the literature describing ccTGA patients receiving VAD therapy shows promising results. With newer devices and the assistance of advanced imaging, mechanical circulatory support is becoming a desirable option for this population of patients and has the potential to provide significant long-term support, relieving them of heart failure symptoms and delaying and perhaps in the future avoiding, the need for cardiac transplantation.

Simulation Training Improves Resuscitation Team Leadership Skills of Nurse Practitioners.

INTRODUCTION: In the current era of limited physician trainee work hours, limited nurse practitioner orientation times, and highly specialized care settings, frontline providers have limited opportunities for mentored resuscitation training in emergency situations. We aimed to evaluate the effectiveness of a pilot program to improve resuscitation team leadership skills of nurse practitioners using simulation-based training. METHODS: Seven nurse practitioners underwent a 4-hour simulation course in pediatric cardiac emergencies. Pre- and post-course surveys were conducted to evaluate previous emergency leadership experience and self-reported comfort in the team lead role. The time to verbalization of a shared mental model to the team was tracked during the simulations. RESULTS: The increases in self-reported comfort level in team leading, sharing a mental model, and differential diagnosis were statistically significant. Average time to shared mental model significantly decreased between simulations. DISCUSSION: Simulation can improve code leadership skills of nurse practitioners. These preliminary findings require confirmation in larger studies.

Sequence of refusals for donor quality, organ utilization, and survival after lung transplantation.

BACKGROUND: Lung donor utilization rates remain low, with many organs refused for donor quality. However, some centers have successfully transplanted these organs despite multiple refusals for donor quality (RDQs) by other centers. We hypothesized that the number of refusals due to donor quality does not impact post-transplant outcomes. METHODS: Lung transplants (LTxs) from 2006 to 2015, identified using the United Network for Organ Sharing (UNOS) database, were matched against the potential transplant recipient (PTR) data set by donor identification. Transplants were categorized into 2 groups: low RDQ (0 to 3 RDQs) and high RDQ (>3 RDQs). Post-transplant survival and predictors for high RDQ were observed using Kaplan‒Meier and logistic regression analyses, respectively. RESULTS: Of 10,126 adult (>18 years) LTxs, 77% had at least 1 RDQ, with a median of 4 RDQs. Post-transplant 1-year survival was similar for both the low and high RDQ groups (p = 0.49). Furthermore, groups of recipients who received donors with an increasing number of RDQs (>3, >6, or >10) also had similar post-transplant 1-year survival (p = 0.77). Treatment for rejection within 1 year and intubation at 72 hours post-transplant were higher in the high RDQ group (p < 0.01). An inverse relationship was identified between the number of RDQs and likelihood of utilization. After 10 RDQs, the likelihood of utilization varied significantly by donor characteristics. CONCLUSIONS: Lung transplant survival is not associated with number of refusals due to donor quality. When determining whether an organ is suitable for transplant, the number of refusals due to donor quality should not influence one's decision, especially in this era of limited donor supply.

Listing Low-Weight or Ill Infants for Heart Transplantation: Is It Prudent?

BACKGROUND: Infants awaiting heart transplantation (HTx) have the highest waitlist mortality than other HTx patients. This study analyzed the impact of weight and other factors on waitlist and post-HTx outcomes in infants. METHODS: All infants on the HTx waitlist in the United Network for Organ Sharing database from October 1987 to June 2016 were divided into the following weight groups: less than 2.5 kg, 2.5 to 3.9 kg, and more than 4 kg. Survival from listing regardless of transplantation was compared by using Kaplan-Meier analysis. RESULTS: Of 4,711 infants listed for HTx, 250 (5.3%) weighed less than 2.5 kg, 1,993 (42%) weighed 2.5 to 3.9 kg, and 2,468 (52%) weighed more than 4 kg. Median time on the waitlist was similar between the groups weighing less than 2.5 kg and 2.5 to 3.9 kg (28 days versus 31 days, p = 0.423), whereas the group weighing more than 4 kg waited longer (42 days; p = 0.027 and p < 0.001). Infants weighing less than 2.5 kg (1 year, 43%) had the worst survival from listing regardless of transplantation compared with other groups (versus weighing 2.5 to 3.9 kg: 1 year, 54%, p = 0.001; versus weighing >4 kg: 1 year, 66%, p < 0.001). For infants weighing less than 2.5 kg on ventilator support, the 1 year-survival was 35%, on extracorporeal membrane oxygenation (ECMO) it was 10%. For infants weighing 2.5 to 3.9 kg on ventilator support, the 1 year-survival was 45%, on ECMO it was 23%. An infant with congenital heart disease (CHD) on ventilator support awaiting transplantation has a 1 year-survival rate of 37% to 42% regardless of weight. Multivariate analysis found that weighing less than 2.5 kg and being on ventilator support, ECMO, or having CHD were associated with 1-year mortality. CONCLUSIONS: Survival of infants after being listed for HTx is substantially affected by weight but also by illness at listing. Therefore, going into a transplantation ill or too small, as seen in the present study, may not be prudent, given the limited resource of infant donors.

Transplant Outcomes for Congenital Heart Disease Patients Bridged With a Ventricular Assist Device.

BACKGROUND: Ventricular assist device (VAD) use as a bridge to transplant (BTT) for children with end-stage heart failure and congenital heart disease (CHD), although challenging, has increased, but its effect on posttransplant outcome is unknown. This study describes posttransplant outcomes of CHD patients BTT with a VAD. METHODS: All heart transplant recipients identified in United Network of Organ Sharing database from 2006 to 2015 (n = 21,865) were divided into four groups by those with (+) and without (-) a diagnosis of CHD and with (+) and without (-) VAD support at transplant: +CHD/+VAD, +CHD/-VAD, +VAD/-CHD, and -VAD/-CHD. Posttransplant survival of +CHD/+VAD was compared with +CHD/-VAD, -CHD/+VAD, and -CHD/-VAD in addition to pretransplant characteristics comparison between +CHD/+VAD and +CHD/-VAD. RESULTS: Of 1,871 patients (8.6%) with CHD, 1,348 (72%) were younger than 18 years old, and 143 (7.6%) were BTT with a VAD (+CHD/+VAD). At transplant, +CHD/+VAD compared with +CHD/-VAD were more likely to have worse functional status (<50%: 60% vs 46%, p = 0.004), infections (29% vs 14%, p < 0.001), to be sensitized (47% vs 30%, p < 0.001) and on ventilator support (20% vs 13%, p = 0.029) and dialysis (13% vs 2.5%, p < 0.001). Overall, 1-year (84% vs 87%) and 5-year (72% vs 75%) survival was similar for +CHD/+VAD and +CHD/-VAD (p = 0.694). Survival was also similar when +CHD/+VAD were compared with -CHD/+VAD (n = 7,363; p = 0.529) and -CHD/-VAD (n = 12,613; p = 0.097). CONCLUSIONS: Although more ill pretransplant, CHD patients BTT with a VAD have similar posttransplant survival compared with CHD patients without a VAD and with other non-CHD heart transplant patients. VAD support may mitigate certain risk factors for poor posttransplant outcomes in the challenging CHD cohort.

Pediatric Heart Donor Assessment Tool (PH-DAT): A novel donor risk scoring system to predict 1-year mortality in pediatric heart transplantation.

BACKGROUND: In this study we sought to quantify hazards associated with various donor factors into a cumulative risk scoring system (the Pediatric Heart Donor Assessment Tool, or PH-DAT) to predict 1-year mortality after pediatric heart transplantation (PHT). METHODS: PHT data with complete donor information (5,732) were randomly divided into a derivation cohort and a validation cohort (3:1). From the derivation cohort, donor-specific variables associated with 1-year mortality (exploratory p-value < 0.2) were incorporated into a multivariate logistic regression model. Scores were assigned to independent predictors (p < 0.05) based on relative odds ratios (ORs). RESULTS: The final model had an acceptable predictive value (c-statistic = 0.62). The significant 5 variables (ischemic time, stroke as the cause of death, donor-to-recipient height ratio, donor left ventricular ejection fraction, glomerular filtration rate) were used for the scoring system. The validation cohort demonstrated a strong correlation between the observed and expected rates of 1-year mortality (r = 0.87). The risk of 1-year mortality increases by 11% (OR 1.11 [1.08 to 1.14]; p < 0.001) in the derivation cohort and 9% (OR 1.09 [1.04 to 1.14]; p = 0.001) in the validation cohort with an increase of 1-point in score. Mortality risk increased 5 times from the lowest to the highest donor score in this cohort. Based on this model, a donor score range of 10 to 28 predicted 1-year recipient mortality of 11% to 31%. CONCLUSION: This novel pediatric-specific, donor risk scoring system appears capable of predicting post-transplant mortality. Although the PH-DAT may benefit organ allocation and assessment of recipient risk while controlling for donor risk, prospective validation of this model is warranted.

The Number of Refusals for Donor Organ Quality Does Not Impact Heart Transplant Outcomes in Children.

BACKGROUND: Waitlist mortality is more than 12% for pediatric heart transplantation, with strikingly high rates of organ refusal, many of which are due to donor quality. However, some centers use these organs despite refusals by other centers for donor quality. We hypothesize that the number of refusals for donor quality (RDQ) does not affect pediatric heart transplantation outcomes. METHODS: Pediatric heart transplants from 2000 to 2015 were identified using the United Network for Organ Sharing database and were matched against the potential transplant recipients dataset with donor refusal codes. Refusals for donor quality were counted for each organ. The population was divided into two groups: RDQ-low (0 to 3 RDQs, n = 3,404) and RDQ-high (>3 RDQs, n = 1,585). Posttransplant outcomes of both cohorts were analyzed. RESULTS: Of 4,989 pediatric heart transplants, 75% (n = 3,770) had 1 or more RDQ (median 3 RDQ; interquartile range, 1 to 7). The RDQ-lower group (0 to 3 RDQs) and the RDQ-higher group (>3 RDQ) had similar posttransplant survival (p = 0.41) and freedom from retransplantation (p = 0.37). Both groups had similar posttransplant survival even for high-risk recipient cohorts: adolescents (p = 0.06), congenital heart disease (p = 0.87), retransplantation (p = 0.47), extracorporeal membrane oxygenation (p = 0.61), mechanical ventilation (p = 0.24), and poor renal function at transplant (p = 0.46). In addition, recipient subgroups who had donors with increasing number of RDQ (>6, >9, >12, and >15 RDQ) also had similar posttransplant survival compared with the RDQ-low group (p = 0.63, p = 0.62, p = 0.92, and p = 0.50, respectively). CONCLUSIONS: The outcome of pediatric heart transplantation is not affected by the number of RDQ, even for high-risk recipients. The use and interpretation of donor quality refusal code should be considered carefully while selecting or refusing donors in this era of supply and demand mismatch.

Contemporary Outcomes of Combined Heart-Liver Transplant in Patients With Congenital Heart Disease.

BACKGROUND: As more patients survive into adulthood with repaired congenital heart disease (CHD), transplant centers now have patients presenting with both end-stage cardiac and hepatic failure. An understanding of the contemporary outcomes with combined heart liver transplantation (CHLT) in patients with CHD is needed. METHODS: A retrospective review of the outcomes of CHLT in CHD was conducted from October 1, 1987, to June 30, 2015, from the United Network of Organ Sharing (UNOS) database. Propensity score matched cohorts were formed for the assessment of posttransplant outcome: CHLT with CHD, CHLT without CHD, and isolated heart transplant for CHD (HT-CHD). Cohorts were matched based on age, body mass index, inotrope use, and ventilator support at the time of transplant. We assessed 30-day, 1-, 5-, and 10-year posttransplant survivals. RESULTS: There were 61 437 heart transplants during the study period, of which 190 (0.3%) were CHLT. Among CHLT, 41(22%) patients had CHD. In 26 (63%) of these, the indication for CHLT was hepatic congestion/cirrhosis of cardiac origin. In the matched cohorts, the overall survival for CHLT with CHD at 30 days, 1, 5, and 10 years was 95%, 86%, 83%, and 83%, respectively; for CHLT without CHD, it was 100%, 92%, 92%, and 63%, respectively (vs CHLT with CHD: P = 0.49); and for HT-CHD, it was 90%, 84%, 63%, and 39% (vs CHLT with CHD: P = 0.03), respectively. CONCLUSIONS: The posttransplant outcome of CHLT, with and without CHD, is comparable. However, there is a trend toward better survival for CHLT for CHD compared with isolated heart transplant for CHD.

Inferior Transplant Outcomes of Adolescents and Young Adults Bridged with a Ventricular Assist Device.

Adolescents, who are thought to have compliance issues, are well known to have poor heart transplant (HTx) outcomes. This "effect" has recently been demonstrated to extend to age 29. The study sought to investigate whether the poor outcomes for HTx related to adolescent age are also observed in recipients who are bridged to transplant (BTT) with a ventricular assist device (VAD) and whether this effect extends beyond the standard definition of adolescent age 12-18 years. All HTx BTT with a VAD in recipients 8-39 years were identified in the United States Organ Sharing (UNOS) database (1 January 2005 to 30 June 2016). Based on the Kaplan-Meier survival comparison for age year, patients were divided into three groups: Group 1 (8-14 years), group 2 (15-29 years), and group 3 (30-39 years). A total of 1,848 HTx were bridged with a VAD. A decline in post-HTx 5 years survival was noted after 14 years of age, which improved at around 30 years of age. Group 1 had 237 (13%) HTx, group 2 had 787 (43%) HTx, and group 3 had 823 (44%) HTx. Group 2 (15-29 years) had worse post-HTx survival compared with group 1 (p < 0.001) and group 3 (p = 0.005). On subdividing group 2 (15-29 years) into "older adolescents" (15-17 years) and "young adults" (18-29 years), post-HTx survival was similar between the two subgroups (p = 0.353). In conclusion, older adolescents and young adults, both, have similarly poor post-HTx survival when BTT with a VAD compared with other age groups. These groups are generally categorized into different broad pediatric and adult age groups; however, these similarities should be carefully considered when formulating treatment protocols for older adolescents and young adults.

Modified Aortic Uncrossing Procedure: A Novel Approach for Norwood Palliation of Complex Univentricular Congenital Heart Disease With a Circumflex Aorta.

The circumflex aorta is a rare type of true vascular ring anomaly. It consists of a retroesophageal right aortic arch, a left-sided descending thoracic aorta, and a left-sided ligamentum arteriosum. The "aortic uncrossing procedure" described by Planché and Lacour-Gayet is the procedure of choice for managing this aortic anomaly in patients with a biventricular heart. The presence of a circumflex aorta in a patient with heterotaxy syndrome and univentricular congenital heart disease requiring Norwood palliation is highly unusual. We report such a case and describe our approach to its surgical management.

Implications and outcomes of cardiac grafts refused by pediatric centers but transplanted by adult centers.

BACKGROUND: According to Organ Procurement Transplant Network policy, hearts from donors age <18 years are offered to pediatric recipients before being offered to adults of the same health status. We aimed to analyze differences in the use of adolescent donor hearts between adult and pediatric candidates and also to analyze the outcomes of pediatric candidates in which an adolescent donor heart was refused and later used in an adult recipient. METHODS: All adolescent donors (age 12-17 years) for 2000 to 2015 were identified using the standard United Network of Organ Sharing dataset and matched against the Potential Transplant Recipient dataset. RESULTS: Of the 2457 adults who received an adolescent heart, 855 (35%) received it after at least 1 refusal by a pediatric candidate (n = 844). Of the 844 pediatric candidates, 643 (76%) subsequently underwent transplantation (designated PCTs) and 201 (24%) never underwent transplantation (designated PCNTs). Among the latter group, 87 patients (43%) died or became too ill for transplantation. These 87 PCNTs refused 256 hearts that were later accepted by adult recipients. Donor quality was the most common reason for refusal. Overall, adult recipients had similar post-transplantation survival compared with PCTs, all pediatric transplants, and all adult transplants (P > .10). A breakdown of adolescent heart donors by year shows a trend toward increased use in pediatric candidates. CONCLUSIONS: A significant number of adolescent donor hearts that are refused by pediatric centers result in excellent post-transplantation outcomes in adult recipients. One in 10 pediatric candidates died on the waitlist after refusal of these hearts used by adult recipients. This warrants careful evaluation of the refusal criteria used by pediatric centers. Encouragingly, there now appears to be a trend toward an increased use of adolescent donor hearts by pediatric centers.

Pediatric Heart Transplantation Long-Term Survival in Different Age and Diagnostic Groups: Analysis of a National Database.

BACKGROUND: The purpose of this study was to evaluate differences in long-term survival without the influence of early mortality, and to identify factors associated with one-year conditional ten-year survival after heart transplantation (HTx) across different age and diagnostic groups. METHODS: Organ Procurement and Transplant Network data from January 1990 to December 2005 were used. Cohort was divided according to age (infants [<1 year], children [>1-10 years], and adolescents [11-18 years]) and diagnosis (cardiomyopathy and congenital heart disease [CHD]). Factors associated with one-year conditional ten-year survival were identified using multivariable logistic regression and using a case-control design. RESULTS: One-year conditional ten-year survivors included 1,790 patients compared to 1,114 patients who died after the first posttransplant year and within ten years of transplant with a median follow-up of 4.8 years. Predictors of one-year conditional ten-year survival for infants were recipient's Caucasian race (odds ratio [OR]: 1.9, 95% confidence interval [CI]: 1.3-2.7) and donor-recipient weight ratio (OR: 0.8, 95% CI: 0.6-1); for children: Caucasian race (OR: 1.6, 95% CI: 1.2-2.1), retransplantation (OR: 0.4, 95% CI: 0.2-0.6), and transplantation after the year 2000 (OR: 1.5, 95% CI: 1.1-2.1); for adolescents only Caucasian race (OR: 2.5, 95% CI: 1.9-2.3). In both CHD and cardiomyopathy, adolescents had worse survival compared to infants and children. There was an era effect with improved survival after 2000. Male gender was a predictor of survival in cardiomyopathy group. CONCLUSION: Predictors of one-year conditional ten-year survival varied among groups. These data and analyses provide important information that may be useful to clinicians, particularly when counseling patients and families regarding expectations of survival after pediatric HTx.

Worldwide Experience with the Syncardia Total Artificial Heart in the Pediatric Population.

Individual centers have documented the use of the Syncardia Total Artificial Heart (TAH) in adolescents with heart failure; however, the number of patients at any given center is small. Herein, we describe the worldwide experience for all patients ≤21 years old supported with the TAH between May 2005 and May 2015 (n = 43). The number of patients experiencing a positive outcome at 60, 90, and 120 days were 30 (70%), 27 (63%), and 25 (58%), respectively. Successful bridge to transplantation varied by diagnosis, but outcomes reported are similar to adults supported with the TAH or biventricular assist devices.

Changing demographics and outcomes of lung transplantation recipients with cystic fibrosis.

BACKGROUND: Cystic fibrosis (CF) is one of the most common diagnoses in adult and pediatric patients undergoing lung transplantation (LTx). A changing pattern of indications for LTx among patients with CF has been noted. This study analyzes the prevalence and characteristics of patients with CF who underwent LTx in the current era. METHODS: A retrospective analysis was performed using data from the United Network of Organ Sharing database of all LTx performed from 1999 to 2013 (N = 20,345). Sub-analyses focused on children (<18 years old). Patients with CF who underwent LTx were assigned to early (1999-2003), mid (2004-2008), and current (2009-2013) eras based on the date of the procedure as well as before and after implementation of the new lung allocation score system in 2005. RESULTS: CF was the indication for LTx in 14% (2,877) of who patients underwent LTx, a decrease from >17% in the early era to <13% in the current era (p < 0.001). In the pediatric cohort, CF was the indication for LTx in 383 (53%) patients, a proportion that also decreased across eras (early, 60%; mid, 53%; current, 47%; p = 0.009). The mean age of patients with CF undergoing LTx increased across the eras (early, 28 years ± 10; mid. 28 years ± 10; current, 30 years ± 11; p < 0.001). Pre-transplant ventilator use and incidence of pan-resistant infections also increased (p < 0.001), whereas pre-transplant forced expiratory volume in 1 second and waitlist times decreased (p < 0.001) in patients with CF. Graft survival across the eras remained similar (p > 0.05) at 5.1 years overall. CONCLUSIONS: The proportion of LTx performed for CF has significantly decreased over time, a trend especially pronounced in pediatric patients. The change in pre-transplant characteristics across eras indicates a trend to perform LTx in more clinically ill and older patients with CF. The overall post-LTx survival has not changed.