Jonathan R. Kaltman

Impact of congenital heart disease on cerebrovascular blood flow dynamics in the fetus.

Neurological abnormalities are present in some children after repair of congenital heart disease (CHD). Recently, structural brain abnormalities have been identified in infants prior to cardiac surgery. By altering in utero blood flow patterns, the type of CHD may impact upon cerebrovascular flow dynamics prior to birth. We sought to determine whether left‐ and right‐sided obstructive congenital heart lesions modify cerebrovascular flow dynamics in the fetus.

Report of the Pediatric Heart Network and National Heart, Lung, and Blood Institute Working Group on the Perioperative Management of Congenital Heart Disease

The survival of patients with congenital heart disease (CHD) has significantly improved during the past several decades (the Figure). This accomplishment is directly attributable to the net effect of individual therapeutic successes from the innovative work of surgeons and cardiologists and the courageous devotion of patients and families. However, as the field moves forward, the need for evidence-based medicine derived from multi-institutional collaborations to address more subtle and complex questions such as functional outcome and quality of life and to provide data to guide individual practitioners has become paramount. Recognizing this, the National Heart, Lung, and Blood Institute (NHLBI) established the Pediatric Heart Network (PHN) in 2001 to provide a collaborative platform for conducting clinical studies. The need for evidence-based care and the ability of the PHN to provide this evidence were demonstrated by recent developments in the surgical management of hypoplastic left heart syndrome. Hypoplastic left heart syndrome was a uniformly fatal defect before Norwood’s innovative surgical approach in which the diminutive aorta is reconstructed using the pulmonary artery trunk and pulmonary blood flow is provided by a Blalock-Taussig shunt. 2 Recently, a previously described modification to this procedure in which pulmonary blood flow is provided by a right ventricle to pulmonary artery shunt has seen renewed interest. 3 This modification has been widely adopted but has yielded conflicting results. 4–7 In May 2005, the PHN launched the Single Ventricle Reconstruction (SVR) trial, a randomized trial comparing these 2 surgical strategies. 8 In just over 3 years, 555 infants were

Modeled Economic Evaluation of Alternative Strategies to Reduce Sudden Cardiac Death Among Children Treated for Attention Deficit/Hyperactivity Disorder

Background— Stimulants are widely used to treat children with attention deficit/hyperactivity disorder and may increase the risk for sudden cardiac death (SCD). We examined the cost-effectiveness of pretreatment screening with ECG for reducing SCD risk in children diagnosed with attention deficit/hyperactivity disorder who are candidates for stimulant medication. Method and Results— We constructed a state-transition Markov model with 10 annual cycles spanning 7 to 17 years of age. Taking a societal perspective, we compared the cost-effectiveness of 3 screening strategies: (1) performing a history and physical examination with cardiology referral if abnormal (current standard of care); (2) performing a history and physical examination plus ECG after negative history and physical examination, with cardiology referral if either is abnormal; and (3) performing a history and physical examination plus ECG, with cardiology referral only if ECG is abnormal. Children identified with SCD-associated cardiac abnormalities would be restricted from stimulants and from playing competitive sports. The expected incremental cost-effectiveness over strategy 1 was

Prior Publication Productivity, Grant Percentile Ranking, and Topic-Normalized Citation Impact of NHLBI Cardiovascular R01 Grants

39 300 and

Assessment of Quality of Life in Young Patients with Single Ventricle after the Fontan Operation

27 200 per quality-adjusted life-year for strategies 2 and 3, respectively. Monte Carlo simulation found that the chance of incremental cost-effectiveness was 55% for strategy 2 and 71% for strategy 3 (willingness to pay ≤

Closing in on the PumpKIN Trial of the Jarvik 2015 Ventricular Assist Device

50 000 per quality-adjusted life-year). Both strategies 2 and 3 would avert 13 SCDs per 400 000 children seeking stimulant treatment for ADHD, for a cost of

Risk factors for prolonged length of stay after the stage 2 procedure in the single-ventricle reconstruction trial

1.6 million per life for strategy 2 and

Cardiovascular disease in adult and pediatric HIV/AIDS.

1.2 million per life for strategy 3. Conclusions— Relative to current practice, adding ECG screening to history and physical examination pretreatment screening for children with attention deficit/hyperactivity disorder has borderline cost-effectiveness for preventing SCD. Relative cost-effectiveness may be improved by basing cardiology referral on ECG alone. Benefits of ECG screening arise primarily by restricting children identified with SCD risk from competitive sports.

Sports Participation and Quality of Life in Adolescents and Young Adults with Congenital Heart Disease

Rationale: We previously demonstrated absence of association between peer-review–derived percentile ranking and raw citation impact in a large cohort of National Heart, Lung, and Blood Institute cardiovascular R01 grants, but we did not consider pregrant investigator publication productivity. We also did not normalize citation counts for scientific field, type of article, and year of publication. Objective: To determine whether measures of investigator prior productivity predict a grant’s subsequent scientific impact as measured by normalized citation metrics. Methods and Results: We identified 1492 investigator-initiated de novo National Heart, Lung, and Blood Institute R01 grant applications funded between 2001 and 2008 and linked the publications from these grants to their InCites (Thompson Reuters) citation record. InCites provides a normalized citation count for each publication stratifying by year of publication, type of publication, and field of science. The coprimary end points for this analysis were the normalized citation impact per million dollars allocated and the number of publications per grant that has normalized citation rate in the top decile per million dollars allocated (top 10% articles). Prior productivity measures included the number of National Heart, Lung, and Blood Institute–supported publications each principal investigator published in the 5 years before grant review and the corresponding prior normalized citation impact score. After accounting for potential confounders, there was no association between peer-review percentile ranking and bibliometric end points (all adjusted P>0.5). However, prior productivity was predictive (P<0.0001). Conclusions: Even after normalizing citation counts, we confirmed a lack of association between peer-review grant percentile ranking and grant citation impact. However, prior investigator publication productivity was predictive of grant-specific citation impact.

Challenges and Priorities for Research A Report From the National Heart, Lung, and Blood Institute (NHLBI)/National Institutes of Health (NIH) Working Group on Thrombosis in Pediatric Cardiology and Congenital Heart Disease

OBJECTIVES To assess self-reported quality of life (QOL) in a large multicenter cohort of adolescent and young adults surviving Fontan. STUDY DESIGN Cross-sectional. The Pediatric Quality of Life Inventory (PedsQL) was administered to 408 survivors of Fontan ages 13-25 years enrolled in the Pediatric Heart Network Fontan Follow-up Study. Subjects also completed either the Child Health Questionnaire (age <19 years) or Short Form Health Survey (age ≥ 19 years). PedsQL data were compared with matched controls without a chronic health condition. Correlations between the measures were examined. RESULTS Mean PedsQL scores for subjects receiving Fontan were significantly lower than those for the control group for physical and psychosocial QOL (P < .001). Overall, 45% of subjects receiving Fontan had scores in the clinically significant impaired range for physical QOL with 30% in the impaired range for psychosocial QOL. For each 1 year increase in age, the physical functioning score decreased by an average of 0.76 points (P = .004) and the emotional functioning score decreased by an average of 0.64 points (P = .03). Among subjects ≥19 years of age, the physical functioning score decreased by an average of 2 points for each year increase in age (P = .02). PedsQL scale scores were significantly correlated with conceptually related Child Health Questionnaire (P < .001) and Short Form Health Survey scores (P < .001). CONCLUSIONS Survivors of Fontan are at risk for significantly impaired QOL which may decline with advancing age. Routine assessment of QOL is essential to inform interventions to improve health outcomes. The PedsQL allowed QOL assessment from pediatrics to young adulthood. TRIAL REGISTRATION ClinicalTrials.gov: NCT00132782.