Late Status of Fontan Patients With Persistent Surgical Fenestration

ObjectivesThis study was undertaken to determine the effects of creating a systemic-to-pulmonary venous atrial-level communication (fenestration) at the time of the Fontan procedure on late outcomes.BackgroundFenestrations are frequently performed during Fontan procedures, but late consequences are not well described.MethodsPatient characteristics were compared between those with and without surgical fenestration among 536 subjects (mean age 11.9 years) enrolled in the Pediatric Heart Network Fontan Cross-Sectional Study. The status of the fenestration and the association of a currently patent fenestration with health status and measures of ventricular performance were investigated.ResultsFenestration was performed in 361 patients (67%), and frequency differed by year and center (p < 0.001 for each). After adjustment for center, age at Fontan, year of Fontan, and prior superior cavopulmonary surgery, the fenestrated group had shorter length of Fontan hospital stay. At the time of cross-sectional testing 8 ± 3 years after Fontan, the fenestration remained open in 19% of subjects. Among those with confirmed fenestration closure, 59% were by catheter intervention and 1% by surgical intervention, and 40% had apparent spontaneous closure. Compared with those without evidence of a fenestration, subjects with a current fenestration were taking more medications (p = 0.02) and had lower resting oxygen saturation (median 89% vs. 95%, p < 0.001). Functional health status, exercise performance, echocardiographic variables, prevalence of post-Fontan stroke or thrombosis, and growth did not differ by current fenestration status.ConclusionsSurgical fenestration is associated with well-demonstrated early post-operative benefits. This cross-sectional study found few associations between a persistent fenestration and deleterious later outcomes

Noncoronary Cardiac Abnormalities Are Associated With Coronary Artery Dilation and With Laboratory Inflammatory Markers in Acute Kawasaki Disease

ObjectivesWe explored the association of noncoronary cardiac abnormalities with coronary artery dilation and with laboratory inflammatory markers early after Kawasaki disease (KD) diagnosis.BackgroundLeft ventricular (LV) dysfunction, mitral regurgitation (MR), and aortic root dilation occur early after diagnosis; their associations with coronary artery dilation and inflammatory markers have not been well-described.MethodsCentrally interpreted echocardiograms were obtained at KD diagnosis and 1 and 5 weeks after diagnosis on 198 subjects in the National Institutes of Health-sponsored Pediatric Heart Network KD pulsed steroid trial. Regression models were constructed to investigate the relationships among early LV dysfunction, MR, and aortic root dilation with coronary artery dilation and laboratory inflammatory markers.ResultsAt diagnosis, LV systolic dysfunction was present in 20% of subjects and was associated with coronary artery dilation, seen in 29% (p = 0.004). Although LV dysfunction improved rapidly, LV dysfunction at diagnosis predicted greater odds of coronary artery dilation at 1 and 5 weeks after diagnosis (5-week odds ratio: 2.7, 95% confidence interval: 1.2 to 6.3). At diagnosis, MR was present in 27% of subjects and aortic root dilation was present in 8%; each was associated with larger coronary artery size at diagnosis. Left ventricular dysfunction was associated with higher erythrocyte sedimentation rate and, at diagnosis only, lower serum albumin; MR was associated with higher erythrocyte sedimentation rate and lower albumin at all times. Aortic root size had little association with inflammatory markers.ConclusionsNoncoronary cardiac abnormalities are associated with coronary artery dilation and laboratory evidence of inflammation in the first 5 weeks after KD, suggesting a shared inflammatory mechanism. (Trial of Pulse Steroid Therapy in Kawasaki Disease [A Trial Conducted by the Pediatric Heart Network]; NCT00132080

Markers of Bone Mineral Metabolism and Cardiac Structure and Function in Perinatally HIV-Infected and HIV-Exposed but Uninfected Children and Adolescents

Background: Disordered bone mineral metabolism and low vitamin D concentrations are associated with cardiovascular abnormalities; few studies have evaluated this relationship in HIV-infected youth. Setting: Adolescent Master Protocol (AMP) is a Pediatric HIV/AIDS Cohort Study (PHACS) network study conducted across 14 United States sites. Methods: Among perinatally HIV-infected (PHIV) and HIV-exposed uninfected (PHEU) youth enrolled in AMP, we evaluated associations of vitamin D (measured as 25-hydroxyvitamin D [25OHD]), parathyroid hormone (PTH), calcium, phosphate, and fibroblast growth factor-23 (FGF-23) concentrations with echocardiographic measures of left ventricular (LV) structure, function and concentrations of NT-proBNP, a biomarker of cardiac damage. Results: Among 485 participants (305 PHIV, 180 PHEU) with echocardiograms and bone mineralization measures, low 25OHD ( 65 pg/mL) was identified more often among PHIV than PHEU participants (9% vs 3%, p=0.02). After adjusting for HIV status and demographic covariates, both low 25OHD and elevated PTH were associated with lower mean LV mass z-scores, while elevated PTH was associated with higher mean fractional shortening z-scores. Participants with low 25OHD also had slightly higher mean LV end-systolic wall stress z-scores, but differences were more pronounced in PHEU than in PHIV participants. FGF-23 was inversely related to end-diastolic septal thickness both overall and among PHIV participants. Conclusion: In this cohort of PHIV and PHEU youth, we observed associations of 25OHD, PTH, and FGF-23 with both structural and functional cardiac parameters, supporting links between bone mineral metabolism and cardiac status

Survival Data and Predictors of Functional Outcome an Average of 15 Years after the Fontan Procedure: The Pediatric Heart Network Fontan Cohort

ObjectiveMulticenter longitudinal outcome data for Fontan patients surviving into adulthood are lacking. The aim of this study was to better understand contemporary outcomes in Fontan survivors by collecting follow‐up data in a previously well‐characterized cohort.DesignBaseline data from the Fontan Cross‐Sectional Study (Fontan 1) were previously obtained in 546 Fontan survivors aged 11.9 ± 3.4 years. We assessed current transplant‐free survival status in all subjects 6.8 ± 0.4 years after the Fontan 1 study. Anatomic, clinical, and surgical data were collected along with socioeconomic status and access to health care.ResultsThirty subjects (5%) died or underwent transplantation since Fontan 1. Subjects with both an elevated (>21 pg/mL) brain natriuretic peptide and a low Child Health Questionnaire physical summary score (<44) measured at Fontan 1 were significantly more likely to die or undergo transplant than the remainder, with a hazard ratio of 6.2 (2.9–13.5). Among 516 Fontan survivors, 427 (83%) enrolled in this follow‐up study (Fontan 2) at 18.4 ± 3.4 years of age. Although mean scores on functional health status questionnaires were lower than the general population, individual scores were within the normal range in 78% and 88% of subjects for the Child Health Questionnaire physical and psychosocial summary score, and 97% and 91% for the SF‐36 physical and mental aggregate score, respectively. Since Fontan surgery, 119 (28%) had additional cardiac surgery; 55% of these (n = 66) in the interim between Fontan 1 and Fontan 2. A catheter intervention occurred in 242 (57%); 32% of these (n = 78) after Fontan 1. Arrhythmia requiring treatment developed in 118 (28%) after Fontan surgery; 58% of these (n = 68) since Fontan 1.ConclusionsWe found 95% interim transplant‐free survival for Fontan survivors over an average of 7 years of follow‐up. Continued longitudinal investigation into adulthood is necessary to better understand the determinants of long‐term outcomes and to improve functional health status.Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/110738/1/chd12193.pd

Superior outcomes for repair in infants and neonates with tetralogy of Fallot with absent pulmonary valve syndrome

ObjectivePrimary repair of tetralogy of Fallot with absent pulmonary valve syndrome has been associated with significant mortality, particularly for neonates in respiratory distress. Controversy persists regarding the method of establishing right ventricle–pulmonary artery continuity.MethodsAnatomic and demographic parameters were evaluated for patients undergoing repair of tetralogy of Fallot with absent pulmonary valve syndrome from 1990 to 2005, as were perioperative and late postoperative parameters (airway complications, reoperation or catheter-based intervention, and mortality).ResultsTwenty-three patients underwent repair. Median age was 15 days (range 2-1154 days). Patients were followed up for 5.3 ± 3.9 years. Seventeen (85%) required preoperative ventilatory assistance. One patient died within 24 hours; 1 patient died 8 months postoperatively. Four patients received valved homografts, and the remainder had valveless connections. All patients underwent reduction pulmonary arterioplasty and mobilization, unifocalization (in 3), and ventricular septal defect closure. Valveless connection recipients had a transannular hood. No patient underwent a Lecompte maneuver. Four patients underwent reoperation for conversion to valveless connection (n = 1), reduction arterioplasty (n = 1), and repair of pulmonary stenosis (n = 2). Three patients required catheter-based intervention, with balloon angioplasty (n = 3) and stent placement (n = 1); 2 now demonstrate equal quantitative lung perfusion. No patient has had significant debility from airway compromise. All patients demonstrate free pulmonary insufficiency and good biventricular function.ConclusionsWe report excellent overall survival (89%) and low postoperative morbidity for neonates and infants undergoing primary repair of tetralogy of Fallot with absent pulmonary valve syndrome. Our recent experience supports the use of a valveless right ventricle–pulmonary artery connection, which, combined with catheter-based intervention, reduces the likelihood of reoperation necessitated by homograft placement

Cardiac and inflammatory biomarkers in perinatally HIV-infected and HIV-exposed uninfected children

To compare distributions of serum cardiac and inflammatory biomarkers between perinatally HIV-infected (PHIV) and perinatally HIV-exposed uninfected (PHEU) children, to evaluate their associations with echocardiographic measures, and among PHIV youth, with antiretroviral therapy (ART) and HIV disease severity measures. Cross-sectional analysis of temporally paired serum samples for biomarkers and echocardiograms in a prospective multicenter cohort study of PHIV and PHEU youth. Serum samples were analyzed among 402 youth in the PHACS Adolescent Master Protocol (AMP) for high-sensitivity cardiac troponin-T (hs-cTnT, a cardiomyocyte injury marker), N-terminal-pro-brain natriuretic peptide (NT-proBNP, a myocardial stress marker), and inflammatory markers [high-sensitivity C-reactive protein, interleukin (IL)-1, IL-6, IL-8, IL-10, IL-18, tumor necrosis factor-α (TNF-α), and soluble TNF receptor II (sTNF-RII)]. Echocardiograms were centrally measured and parameters converted to z cores to account for differences in age and body size. Compared with PHEU (N = 156), PHIV youth (N = 246) more often had detectable hs-cTnT and higher levels of sTNF-RII and IL-18. Higher inflammatory biomarkers were generally associated with higher left ventricular (LV) wall stress and lower LV function and LV mass in the two groups. Among PHIV youth, the biomarkers were more strongly associated with current rather than historical immunologic and virologic status. PHEU and PHIV have modest, significant differences in serum levels of specific inflammatory and active myocardial injury biomarkers. Higher biomarker levels were associated with lower LV mass and shifts in LV structure. Further study is warranted on the longitudinal role of cardiac and inflammatory biomarkers for targeting interventions among PHIV and PHEU youth

Abstract 2556: A Comparative Analysis of Outcomes for Pediatric Patients with Biopsy-Proven Myocarditis, Clinically-Diagnosed Myocarditis and Idiopathic Dilated Cardiomyopathy

Background: Data on outcomes in children with myocarditis is sparse and challenging to interpret given no ``gold standard” for diagnosis. The NHLBI Pediatric Cardiomyopathy Registry provides a large, multi-center cohort with clinical and echocardiographic follow-up of up to 16 years. Methods/Results : We compared cases of biopsy-confirmed myocarditis (BCM, N=119) and clinically-diagnosed myocarditis (CDM, N=253), with idiopathic dilated cardiomyopathy (IDCM, N=1123). BCM and CDM patients were older at diagnosis than those with IDCM (median age 1.6, 1.9, 1.2 yr, respectively). For BCM, heart failure symptoms (86%) and admission (97%) at diagnosis were more common (p2) in 71% with myocarditis and 86% with IDCM. Systolic function was impaired in all groups, with a median fractional shortening (FS) Z score of -9 for myocarditis and -10 for IDCM; 23% of myocarditis patients had impaired function without dilation. Two-year death/transplant rates were 24% (BCM), 24% (CDM), and 47% (IDCM). Of myocarditis survivors, 46% had normalization of both EDD and FS Z-scores at 2 years vs. 25% for IDCM (p<.0001). Baseline echo status was strongly associated with later echo status for IDCM pts. Recovery from myocarditis at 2 years was not strongly associated with baseline echocardiographic characteristics, but transplant-free survival was lower in myocarditis patients when both baseline FS and EDD were abnormal (logrank p=.03). When therapeutic data were available, the BCM group received IVIG/steroids most often (44% vs. 23% CDM, 6% IDCM); this was not significantly associated with 2-year echo recovery or transplant-free survival, but power was limited. Conclusions : In this dataset, children with BCM and CDM have similar profiles and a better outcome than those with IDCM, with 76% surviving without transplant at 2 years. Nearly 1 / 2 have normalization of LV size and function, but the presence of both dilation and poor function at presentation is associated with increased risk for death or transplant. Echocardiographic normalization occurs in 25% of IDCM survivors by 2 years. This study is limited by being an analysis of available information in a database collected for other purposes