Abstract 14979: Endostatin as a Predictor of Severity and Survival in Pediatric Congenital Heart Disease Associated Pulmonary Hypertension

Introduction: Pediatric pulmonary arterial hypertension (PAH) is a multifactorial pulmonary vascular disease with high mortality. Endostatin (ES), an angiogenic inhibitor, is associated with disease severity and mortality in adults with PAH and poor lung growth in children. Hypothesis: Increased ES is associated with worse disease severity and outcomes in pediatric PAH. Methods: Serum ES levels were measured in two cohorts of pediatric PAH (IPAH, FPAH and APAH) patients; the cross-sectional CCHMC PAH Biobank (PAHB, N=175) and a longitudinal cohort from Children’s Hospital of Colorado (CHC, N=61). Outcomes included medical therapy, functional and hemodynamic measures, and survival (death, transplant, palliative shunt). Adjusted logistic and linear regressions and Kaplan-Meier analysis were used to assess the relationship between ES and clinical outcomes. Results: In both cohorts, ES was significantly higher in PAH associated with congenital heart disease (APAH-CHD, PAHB n=70, p=0.002; CHC n=29, p=0.007) and was highest in those with a ventricular shunt (n=24, p=0.001). In APAH-CHD, ES was associated with a decreased 6MWD (-108m, -187- -30, p=0.01) and increased mean right atrial pressure (mRAP 1.8mmHg, 0.3-3.3, p=0.02). Longitudinally, in the APAH-CHD subgroup when adjusted for age, sex, and multiple time points, higher ES was associated with increased PVRi and mPAP (PVRi 2.5WU*m 2 , 0.7-4.3, p=0.007; mPAP 10mmHg, 4.5-15, p<0.001). Kaplan-Meier analysis (Figure 1) demonstrated that ES was associated with worse outcomes in both cohorts, with an adjusted Cox proportional hazard ratio of 4.3 (1.13-16, p=0.03) in the PAHB. Conclusions: ES was associated with worse functional measures, pulmonary vascular hemodynamics, and survival in pediatric APAH-CHD. These observations suggest that serum ES could act as a pulmonary vascular specific biomarker to identify those who are at increased risk of developing PAH and predict poor outcomes in APAH-CHD.

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Angiostatic Peptide, Endostatin, Predicts Severity in Pediatric Congenital Heart Disease–Associated Pulmonary Hypertension

Journal of the American Heart Association ◽

10.1161/jaha.120.021409 ◽

2021 ◽

Author(s):

Caroline M. Daly ◽

Megan Griffiths ◽

Catherine E. Simpson ◽

Jun Yang ◽

Rachel L. Damico ◽

...

Keyword(s):

Congenital Heart Disease ◽

Heart Disease ◽

Pulmonary Artery ◽

Risk Stratification ◽

Disease Severity ◽

Congenital Heart ◽

Adverse Outcomes ◽

Right Atrial ◽

Free Survival ◽

Over Time

Background Endostatin, an angiogenic inhibitor, is associated with worse pulmonary arterial hypertension (PAH) outcomes in adults and poor lung growth in children. This study sought to assess whether endostatin is associated with disease severity and outcomes in pediatric PAH. Methods and Results Serum endostatin was measured in cross‐sectional (N=160) and longitudinal cohorts (N=64) of pediatric subjects with PAH, healthy pediatric controls and pediatric controls with congenital heart disease (CHD) (N=54, N=15), and adults with CHD associated PAH (APAH‐CHD, N=185). Outcomes, assessed by regression and Kaplan‐Meier analysis, included hemodynamics, change in endostatin over time, and transplant‐free survival. Endostatin secretion was evaluated in pulmonary artery endothelial and smooth muscle cells. Endostatin was higher in those with PAH compared with healthy controls and controls with CHD and was highest in those with APAH‐CHD. In APAH‐CHD, endostatin was associated with a shorter 6‐minute walk distance and increased mean right atrial pressure. Over time, endostatin was associated with higher pulmonary artery pressure and pulmonary vascular resistance index, right ventricular dilation, and dysfunction. Endostatin decreased with improved hemodynamics over time. Endostatin was associated with worse transplant‐free survival. Addition of endostatin to an NT‐proBNP (N‐terminal pro‐B‐type natriuretic peptide) based survival analysis improved risk stratification, reclassifying subjects with adverse outcomes. Endostatin was secreted primarily by pulmonary artery endothelial cells. Conclusions Endostatin is associated with disease severity, disease improvement, and worse survival in APAH‐CHD. Endostatin with NT‐proBNP improves risk stratification, better predicting adverse outcomes. The association of elevated endostatin with shunt lesions suggests that endostatin could be driven by both pulmonary artery flow and pressure. Endostatin could be studied as a noninvasive prognostic marker, particularly in APAH‐CHD.

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LVNC – A review

Journal of Clinical Images and Medical Case Reports ◽

10.52768/2766-7820/1278 ◽

2021 ◽

Vol 2 (4) ◽

Author(s):

Lisa Brandon ◽

◽

Brian Kerr ◽

Ken McDonald ◽

◽

...

Keyword(s):

Heart Failure ◽

Congenital Heart Disease ◽

Heart Disease ◽

Gold Standard ◽

Congenital Heart ◽

Imaging Techniques ◽

Disease Modifying Therapies ◽

Increased Risk ◽

Disease Modifying ◽

No Gold Standard

LVNC is a relatively new clinical entity, with a significant increase in awareness and diagnosis in recent years. Currently the aetiology and pathogenesis of LVNC remains uncertain, alongside prevalence, however the diagnosis of LVNC appears to be increasing with improving imaging techniques. For educational purposes involving a rare clinical condition, we present the case of a 52 year old gentleman who was diagnosed with LV non compaction via ECHO and CMR. Interestingly it was noted two of his children had congenital heart disease, one daughter had Tetralogy of Fallot, and a second daughter had both an ASD and VSD. Challenges facing LVNC involve difficulty of diagnosis with no gold standard yet available, uncertainty of benefit with standard disease modifying therapies for HF-REF, and apparent increased risk of arrhythmias suggesting early ICD placement may be warranted for patients. Keywords: Hr-Ref; heart failure; lv non compaction; arrhythmias; lcd Risk.

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Nutritional assessment and associated factors in children with congenital heart disease in Ethiopia.

10.1101/2021.04.12.21255315 ◽

2021 ◽

Author(s):

Temesgen Tsega Desta

Keyword(s):

Congenital Heart Disease ◽

Pulmonary Hypertension ◽

Heart Disease ◽

Associated Factors ◽

Congenital Heart ◽

Nutritional Assessment ◽

Failure To Thrive ◽

Cross Sectional ◽

Care Givers ◽

Increased Risk

ABSTRACT Infants and children with congenital heart disease exhibit a range of delays in weight gain and growth. In some instances, the delay can be relatively mild, whereas in other cases, cause the failure to thrive. OBJECTIVES To determine the nutritional status and associated factors of pediatric patients with congenital heart disease. MATERIAL AND METHODS A cross sectional analytical study was done over a period of 6months (Feb to Jul 2020). A total of 228 subjects with congenital heart disease that come to the cardiac center during the study period where included until the calculated sample size was attained. Data was collected from patient card and care givers of the children included in the study after obtaining their informed consent using data inquiry sheet. RESULTS A total of 228 children from age 3month to 17yrs. Most of the subjects had acyanotic heart disease accounting for 87.7%. The overall prevalence of wasting, underweight and stunting were 41.3%, 49.1% and 43% respectively. Among this children with congenital heart disease those with PAH were found more likely have wasting compared to those without PAH with an odds of 1.9 (95% CI: 1.0-3.4) and also greater chance of being stunted with an odds of 1.9 (95% CI: 1.0-3.4). children above 5years of age were 2.3 times more likely to be underweight. CONCLUSION Malnutrition is a major problem of patients with CHD. Pulmonary hypertension and older age are associated with increased risk of undernutrition. KEYWORDS: Acyanotic, cyanotic, Pulmonary hypertension, underweight, wasting and stunting.

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The impact of actual and perceived disease severity on pre-operative psychological well-being and illness behaviour in adult congenital heart disease patients

Cardiology in the Young ◽

10.1017/s1047951113000218 ◽

2013 ◽

Vol 24 (2) ◽

pp. 275-282 ◽

Cited By ~ 14

Author(s):

Edward Callus ◽

Elisabeth M. W. J. Utens ◽

Emilia Quadri ◽

Cristian Ricci ◽

Mario Carminati ◽

...

Keyword(s):

Congenital Heart Disease ◽

Heart Disease ◽

Disease Severity ◽

Cardiac Disease ◽

Congenital Heart ◽

Well Being ◽

Illness Behavior ◽

Psychological State ◽

Illness Behaviour ◽

Psychological Well Being

AbstractObjectives: The purpose of this study was to investigate whether the objective medical parameters related to congenital heart disease and patients’ ratings of cardiac disease severity were related to psychological well-being and illness behaviour during the pre-operative period.Methods: A total of 143 patients (63 male patients; 16–73 years old) with congenital heart disease evaluated the severity of their cardiac condition using a numerical rating scale ranging from 0, indicating the least severe condition, to 100, indicating the most severe condition. Psychological well-being was assessed using the Psychological General Well-Being Index (total score ≤60 indicating severe distress) and illness behaviour using the Illness Behavior Questionnaire.Results: Pre-operative psychological well-being was not related to the objective medical parameters reflecting cardiac disease severity. In contrast, total psychological well-being scores correlated significantly with patients’ subjective ratings of disease severity (p < 0.001). When compared with the reference values from the Italian population, the mean scores of the patients on psychological well-being were similar. As regards the Illness Behavior Questionnaire, the scores on denial were higher and those on hypochondria were lower compared with other hospitalised patients.Conclusions: This study shows that the perception of cardiac disease severity, and not the medical parameters in congenital heart disease, is related to the patients’ pre-operative psychological state. Thus, more importance needs to be given to assessing the patients’ pre-operative perception and psychological state independently of cardiac severity. Targeted interventions with regard to the cardiac condition are recommended.

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Abstract 13468: Effect of Listing Criteria on Transplant Rate and Early Outcomes in Adults With Congenital Heart Disease

Circulation ◽

10.1161/circ.142.suppl_3.13468 ◽

2020 ◽

Vol 142 (Suppl_3) ◽

Author(s):

Wagih m Zayed ◽

Neha Bansal ◽

Snehal R Patel ◽

Jacqueline M Lamour ◽

Daniel J GOLDSTEIN ◽

...

Keyword(s):

Congenital Heart Disease ◽

Heart Disease ◽

Congenital Heart ◽

Primary Diagnosis ◽

Organ Allocation ◽

Transplant Recipients ◽

Allocation Policy ◽

Kaplan Meier ◽

Allocation Criteria ◽

History Of

Introduction: Heart failure (HF) is the leading cause of death in adults with congenital heart disease (ACHD). Heart transplant (HT) is one of the few options for the treatment of advanced HF in this growing population. In October 2018, the United Network for Organ Sharing (UNOS) implemented a change in organ allocation criteria. The effect of this change on outcomes in ACHD patients (pts) after listing and transplant has not been evaluated. Hypothesis: Change in organ allocation criteria negatively impacts outcomes in ACHD patients. Methods: Data from the Scientific Registry of Transplant Recipients in pts age > 18 years old listed for HT between Oct. 2016 and 0ct. 2019 and followed through March 2020 were analyzed. Pts were grouped by diagnosis (ACHD and non-ACHD) and by the time of listing (pre- and post-change in allocation criteria). Differences in comorbidities, outcomes while listed, and 1-year Kaplan Meier survival post-HT were compared among groups. For comparison, post-change criteria (status 1-6) were equated to pre-change criteria (status 1A, 1B, 2). Results: Over 3 years, 11,931 patients were listed for HT; 459 had a primary diagnosis of ACHD. ACHD was present in 279/7942 pts listed in the 2 years pre-change and 180/3989 pts in the year post-change. ACHD pts listed post-change were less likely to have a history of cardiac surgery (88% vs. 79%, p=0.01) and more likely to have an abnormal BMI (p=0.015) than ACHD pts pre-change. Post-change, ACHD pts were listed at a higher priority status compared to pre-change ACHD. (Figure). The proportion of pts transplanted with ACHD increased slightly pre- and post-change (3.7% vs. 4.1%). There was no difference in 1-year survival in ACHD pts transplanted pre- and post-change (Figure). Conclusions: Recent changes to the UNOS organ allocation policy increased the proportion of ACHD patients transplanted with no change in early post-HT survival.

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Abstract 19314: Impact of Body Mass Index on Clinical Outcomes in Complex Adult Congenital Heart Disease

Circulation ◽

10.1161/circ.130.suppl_2.19314 ◽

2014 ◽

Vol 130 (suppl_2) ◽

Author(s):

Norihisa Toh ◽

Ines Uribe Morales ◽

Zakariya Albinmousa ◽

Tariq Saifullah ◽

Rachael Hatton ◽

...

Keyword(s):

Heart Failure ◽

Body Mass Index ◽

Congenital Heart Disease ◽

Heart Disease ◽

Congenital Heart ◽

Adult Congenital Heart Disease ◽

Cardiac Events ◽

Increased Risk ◽

Adult Congenital

Background: Obesity can adversely affect most organ systems and increases the risk of comorbidities likely to be of consequence for patients with complex adult congenital heart disease (ACHD). Conversely, several studies have demonstrated that low body mass index (BMI) is a risk factor for heart failure and adverse outcomes after cardiac surgery. However, there are currently no data regarding the impact of BMI in ACHD. Methods: We examined the charts of 87 randomly selected, complex ACHD patients whose first visit to our institution was at 18-22 years old. Patients were categorized according to BMI at initial visit: underweight (BMI < 18.5 kg/m 2 ), normal (BMI 18.5 - 24.9 kg/m 2 ), overweight/obese (BMI ≥ 25 kg/m 2 ). Events occurring during follow-up were recorded. Data was censured on 1/1/2014. Cardiac events were defined as a composite of cardiac death, heart transplantation or admission for heart failure. Results: The cohort included patients with the following diagnoses: tetralogy of Fallot n=31, Mustard n=28, Fontan n=17, ccTGA n=9 and aortic coarctation n=2. The median (IQR) duration of follow-up was 8.7 (4.2 - 1.8) years. See table for distribution and outcomes by BMI category. Cardiac events occurred in 17/87 patients. After adjustment for age, sex, and underlying disease, the underweight group had increased risk of cardiac events (HR=12.9, 95% CI: 2.8-61.5, p < 0.05). Kaplan-Meier curves demonstrate the poorer prognosis of underweight patients (Figure). Conclusions: Underweight was associated with increased risk of late cardiac events in ACHD patients. We were unable to demonstrate significant overweight/obesity impact.

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