Joshua M. Friedland-Little

Survival Through Staged Palliation: Fate of Infants Supported by Extracorporeal Membrane Oxygenation After the Norwood Operation

BACKGROUND Infants supported by extracorporeal membrane oxygenation (ECMO) after a Norwood operation face in-hospital mortality rates of 60% to 70%. There are limited data on completion of staged palliation for the subset of patients who survive to hospital discharge. METHODS We performed a retrospective case-control study of 64 sequential patients at a single institution supported by ECMO after a Norwood operation. Primary endpoints were survival to hospital discharge, stage II palliation, and stage III palliation. Predictors of non-survival to each endpoint were identified with logistic regression. Survival was compared with a 3:1 era-matched group of control patients who underwent a Norwood operation but did not require ECMO. RESULTS Survival to hospital discharge, stage II palliation, and stage III palliation was 43.8%, 35.9%, and 25.4%, respectively for ECMO cases. Factors independently associated with non-survival to hospital discharge included female gender, ECMO 7 days or greater, and need for renal replacement therapy on ECMO. Non-Caucasian race and ECMO 7 days or greater were independently associated with non-survival to stage II, while non-Caucasian race, lower birth weight, and ECMO 7 days or greater were independently associated with non-survival to stage III. Extracorporeal membrane oxygenation was associated with decreased survival at each endpoint. Patients who survived ECMO had increased interstage mortality between hospital discharge and stage II palliation. CONCLUSIONS Extracorporeal membrane oxygenation after a Norwood operation can be life-saving but ultimate survival through staged palliation remains suboptimal. The elevated mortality risk for patients supported by ECMO persists after hospital discharge. Both socioeconomic factors and ECMO-related morbidity may contribute to midterm mortality.

Risk factors for requiring extracorporeal membrane oxygenation support after a Norwood operation.

BACKGROUND Patients requiring extracorporeal membrane oxygenation (ECMO) support after a Norwood operation constitute an extremely high-risk group. Data regarding risk factors for the requirement for ECMO post-Norwood are limited, however. We retrospectively assessed risk factors for requiring ECMO support after a Norwood operation during a 10-year period in a high-volume center. METHODS Retrospective case-control study of 64 consecutive patients requiring ECMO support after a Norwood operation at a single institution during a 10-year period (January 2001-December 2010), with a 3:1 era-matched control group of patients who underwent a Norwood but did not require ECMO. RESULTS In univariate analysis, ascending aorta less than 2.0 mm, longer cardiopulmonary bypass (CPB) time, intraoperative shunt revision, and right ventricle to pulmonary artery conduit were associated with the need for postoperative ECMO. A single left ventricle was protective compared with single right ventricle anatomy. By multivariate logistic regression, birth weight less than 2.5 kg and longer CPB time were independently associated with the need for postoperative ECMO. Receiver-operating characteristic curve analysis identified a peak lactate of 9 mmol/L and a peak vasoactive inotrope score (VIS) of 27 within 48 hours of surgery as most prognostic of the need for ECMO. CONCLUSIONS Birth weight less than 2.5 kg and longer CPB time are independently associated with the need for ECMO after a Norwood operation. Peak serum lactate and peak VIS may be useful in stratifying risk for ECMO. Risk factors for ECMO post-Norwood appear to be similar to the risk factors for early mortality post-Norwood.

Differential effect of body mass index on pediatric heart transplant outcomes based on diagnosis

The impact of nutritional status on HTx waitlist mortality in children is unknown, and there are conflicting data regarding the role of nutrition in post‐HTx survival. This study examined the influence of nutrition on waitlist and post‐HTx outcomes in children. Children 2–18 yr listed for HTx from 1997 to 2011 were identified from the OPTN database and stratified by BMI percentile. Multivariable logistic regression evaluated the influence of BMI on waitlist mortality. Cox proportional hazard regression assessed the impact of BMI on post‐HTx mortality. When all 2712 patients were analyzed, BMI did not impact waitlist, one‐, or five‐yr mortality. However, when stratified by diagnosis, BMI > 95% (AOR 1.96; 95% CI 1.24, 3.09) and BMI < 1% (AOR 2.17; 95% CI 1.28, 3.68) were independent risk factors for waitlist mortality in patients with CM. BMI did not impact waitlist mortality in CHD and did not impact post‐HTx outcomes, regardless of diagnosis. BMI > 95% and BMI < 1% are independent risk factors for waitlist mortality in patients with CM, but not CHD. This suggests differing risk factors based on disease etiology, and an individualized approach to risk assessment based on diagnosis may be warranted.

Abnormal nutrition affects waitlist mortality in infants awaiting heart transplant

BACKGROUND Although nutritional status affects survival after heart transplant (HTx) in adults and older children, its effect on outcomes in young children is unknown. This study aimed to assess the effect of pre-HTx nutrition on outcomes in this population. METHODS Children aged 0 to 2 years old listed for HTx from 1997 to 2011 were identified from the Organ Procurement and Transplantation Network database. Nutritional status was classified according to percentage of ideal body weight at listing and at HTx. Logistic regression analysis evaluated the risk of waitlist mortality. Cox proportional hazard models assessed the effect of nutrition on post-HTx survival. RESULTS Of 1,653 children evaluated, 899 (54%) had normal nutrition at listing, 445 (27%) were mildly wasted, 203 (12%) were moderate or severely wasted, and 106 (6%) had an elevated weight-to-height (W:H) ratio. Moderate or severe wasting (adjusted odds ratio, 1.9; 95% confidence interval, 1.3-2.7) and elevated W:H (adjusted odds ratio, 1.7; 95% confidence interval, 1.1-2.6) were independent risk factors for waitlist mortality. HTx was performed in 1,167 patients, and 1,016 (87%) survived to 1-year post-HTx. Nutritional status at listing or at HTx was not associated with increased post-HTx mortality. Nutritional status did not affect the need for early reoperation, dialysis, or the incidences of infection, stroke, or rejection before hospital discharge. CONCLUSIONS Moderate or severe wasting and an elevated W:H are independent risk factors for waitlist mortality in patients aged < 2 years but do not affect post-HTx mortality. Optimization of pre-HTx nutritional status constitutes a strategy to reduce waitlist mortality in this age range.

Outcomes of third heart transplants in pediatric and young adult patients: Analysis of the United Network for Organ Sharing database

BACKGROUND Repeat heart transplantation (re-HTx) is standard practice in many pediatric centers. There are limited data available on outcomes of third HTx after failure of a second graft. We sought to compare outcomes of third HTx in pediatric and young adult patients with outcomes of second HTx in comparable recipients. METHODS All recipients of a third HTx in whom the primary HTx occurred before 21 years of age were identified in the United Network for Organ Sharing database (1985 to 2011) and matched 1:3 with a control group of second HTx patients by age, era and re-HTx indication. Outcomes including survival, rejection and cardiac allograft vasculopathy (CAV) were compared between groups. RESULTS There was no difference between third HTx patients (n = 27) and control second HTx patients (n = 79) with respect to survival (76% vs 80% at 1 year, 62% vs 58% at 5 years and 53% vs 34% at 10 years, p = 0.75), early (<1 year from HTx) rejection (33.3% vs 44.3%, p = 0.32) or CAV (14.8% vs 30.4%, p = 0.11). Factors associated with non-survival in third HTx patients included mechanical ventilation at listing or HTx, extracorporeal membrane oxygenation support at listing or HTx, and elevated serum bilirubin at HTx. CONCLUSIONS Outcomes among recipients of a third HTx are similar to those with a second HTx in matched patients, with no difference in short- or long-term survival and comparable rates of early rejection and CAV. Although the occurrence of a third HTx remains relatively rare in the USA, consideration of a third HTx appears reasonable in appropriately selected patients.

An apparent case of undiagnosed donor Kawasaki disease manifesting as coronary artery aneurysm in a pediatric heart transplant recipient.

We present a case of coronary ectasia and LAD coronary artery aneurysm with angiographic characteristics of Kawasaki disease in a three‐yr‐old girl two‐yr status post‐orthotopic heart transplant. Coronary anomalies were noted during initial screening coronary angiography two yr after transplant. Subsequent review of the donor echocardiogram revealed that the LMCA had been mildly dilated prior to transplant. In the absence of any symptoms consistent with Kawasaki disease in the transplant recipient, this appears to be a case of Kawasaki disease in the organ donor manifesting with coronary anomalies in the transplant recipient. The patient has done well clinically, and repeat coronary angiography has revealed partial regression of coronary anomalies. Given multiple reports in the literature of persistent abnormalities of coronary artery morphology and function after Kawasaki disease, close monitoring is warranted, with consideration of potential coronary protective medical therapies.