Changes in ET-1, Plasma Neuropeptide Y, and CGRP in Child Patients With Congenital Heart Disease Complicated With Pulmonary Hypertension Before and After Operation

2020 ◽  
Vol 60 (1) ◽  
pp. 56-63
Author(s):  
Zheng Liu ◽  
Mingming Zhang ◽  
Qiang Huo ◽  
Tao Zhu

This study aims to explore the changes in endothelin-1 (ET-1), plasma neuropeptide Y, and calcitonin gene–related peptide (CGRP) in child patients before and after operation. A total of 80 child patients with congenital heart disease (CHD) complicated with pulmonary hypertension (PH) were enrolled and divided into control group (n = 40, conservative treatment for various reasons) and observation group (n = 40, active preoperative preparation and timely operative intervention) according to different treatments. There were positive correlations between systolic pulmonary arterial pressure (sPAP) and ET-1, plasma neuropeptide Y, while negative correlation between sPAP and CGRP. In conclusion, our data demonstrate that the levels of ET-1, plasma neuropeptide Y, and CGRP in PH-CHD were significantly changed after interventions, which provides new leads as alternative biomarkers to assess the efficacy of treatments against PH-CHD.

ESC CardioMed ◽  
2018 ◽  
pp. 781-784
Author(s):  
Shahin Moledina ◽  
Bejal Pandya

Congenital heart disease is a major cause of pulmonary arterial hypertension (PAH) and this can largely be prevented by early repair. PAH in the presence of systemic-to-pulmonary communication, leads to shunt reversal and cyanosis, with multiple systemic consequences (Eisenmenger syndrome). Congenital heart disease patients with PAH are vulnerable and are at high risk from non-cardiac surgery, pregnancy, and inappropriate medical treatment (e.g. excessive venesection). Survival is reduced, but is better than in idiopathic PAH. Recommendations for surgery should be based on careful assessment by experts. Modern PAH pharmacotherapy is showing promise in improving quality of life.


2020 ◽  
pp. 3695-3710
Author(s):  
Nicholas W. Morrell

Symptoms of unexplained exertional breathlessness or symptoms out of proportion to coexistent heart or lung disease should alert the clinician to the possibility of pulmonary hypertension, and the condition should be actively sought in patients with known associated conditions, such as scleroderma, hypoxic lung disease, liver disease, or congenital heart disease. Heterozygous germ-line mutations in the gene encoding the bone morphogenetic protein type II receptor (BMPR2) are found in over 70% of families with pulmonary arterial hypertension. Pulmonary hypertension is defined as a mean pulmonary arterial pressure greater than 25 mm Hg at rest, and may be due to increased pulmonary vascular resistance (e.g. pulmonary arterial hypertension), increased transpulmonary blood flow (e.g. congenital heart disease), or increased pulmonary venous pressures (e.g. mitral stenosis). Exercise tolerance and survival in pulmonary hypertension is ultimately related to indices of right heart function, such as cardiac output.


2020 ◽  
pp. 1-4
Author(s):  
Doaa El Amrousy ◽  
Amr Zoair ◽  
Doaa El Amrousy ◽  
Elham Zahran ◽  
Hesham El-Serogy

Objectives: We aimed to investigate the levels and diagnostic value of plasma growth differentiation factor15 (GDF-15) in children with pulmonary hypertension associated with congenital heart disease (PH-CHD). Methods: One hundred infants and children with CHD were included in the study; 50 of them with PHCHD and another 50 patients with CHD only. Fifty healthy infants and children of matched age and sex were included as a control group. GDF-15 levels as well as echocardiographic examination were done for all included subjects. Cardiac catheterization was performed for patients only. Results: GDF-15 levels were significantly higher in patients with PH-CHD (0.62±0.2) nmol/ml compared to those with CHD only patients (0.21±0.06) nmol/ml and to control group (0.13±0.02) nmol/ml with P<0.001. GDF-15 levels were significantly elevated according to the severity of PH; (0.4±0.1) nmol/ml in mild PH vs (0.7±0.3) nmol/ml in moderate PH vs (1.1±0.1) nmol/ml in severe PH. At a cutoff value of ≥ 0.313 nmol/ml, the sensitivity of GDF-15 as a diagnostic biomarker in PH-CHD children was 95%, the specificity was 100%, positive predictive value (PPV) was 100%, and negative predictive value (NPV) was 95.2 %. Conclusion: GDF-15 is elevated in infants and children with PH-CHD and can be a promising diagnostic biomarker in these patients.


2017 ◽  
Vol 7 (5) ◽  
pp. 459-466 ◽  
Author(s):  
Tim Van Puyvelde ◽  
Koen Ameloot ◽  
Mieke Roggen ◽  
Els Troost ◽  
Marc Gewillig ◽  
...  

Background: Outcome after cardiopulmonary resuscitation (CPR) in patients with underlying congenital heart disease is uncertain. This study aimed at evaluating outcome after CPR in patients with underlying congenital heart disease, factors related to worse outcome after CPR and whether survivors of sudden cardiac death (SCD) have a worse outcome when compared to an age, gender and disease-matched control population. Methods: Between 1984 and 2015, all patients with congenital heart disease who received in or out-of-hospital CPR were identified from the database of congenital heart disease from the University Hospitals Leuven. Postoperative and neonatal (<6 months of age) CPR was excluded. For each survivor of SCD, two control patients matched for gender, age and underlying heart defect were included in the study. Results: Thirty-eight patients (66% men; median age 25 years (interquartile range 9–40); 68% out-of-hospital) were identified, of which 27 (66%) survived the event. The main cause of SCD was ventricular tachycardia or fibrillation ( n=21). Heart defect complexity (odds ratio (OR) 5.1; 95% confidence interval (CI) 1.2–21.9; P=0.027), pulmonary hypertension (OR 13.8; 95% CI 2.1–89.5; P=0.006) and time to return of spontaneous circulation (OR 1.1; 95% CI 1.0–1.1; P=0.046) were related to worse outcome. Survivors of SCD had a worse prognosis when compared to an age, gender and disease-matched control group (5-year survival 76% vs. 98%; P=0.002). Conclusions: The complexity of underlying heart defect, pulmonary hypertension and time to return of spontaneous circulation are related to worse outcome in the case of CPR. Survivors of SCD have a worse outcome when compared to matched controls, indicating the need for adequate implantable cardioverter defibrillator indication assessment and for stringent follow-up of patients with worsening haemodynamics.


2020 ◽  
Vol 48 (11) ◽  
pp. 030006052096437
Author(s):  
Yang Zi-yang ◽  
Zhao Kaixun ◽  
Luo Dongling ◽  
Yin Zhou ◽  
Zhou Chengbin ◽  
...  

Objective Pulmonary artery hypertension (PAH) is a severe complication of congenital heart disease (CHD). Monitoring of pulmonary arterial pressure (PAP) and pulmonary vascular resistance (PVR) is essential during follow-up. This retrospective study aimed to examine carcinoembryonic antigen (CEA) as an additional marker for evaluation by investigating the correlation between CEA levels and hemodynamics in CHD-PAH. Methods Seventy-six patients with CHD-PAH (mean PAP [mPAP] >25 mmHg and PVR >3 Wood units, group A), 71 patients with CHD and pulmonary hypertension (CHD-PH, mPAP >25 mmHg and PVR ≤3 Wood units, group B), and 102 patients with CHD without PH (mPAP ≤25 mmHg, group C) were enrolled. Serum CEA levels and the relationships between CEA levels and hemodynamic data were assessed. Results Mean serum CEA levels were 1.99±1.61, 2.44±1.82, and 1.58±1.07 ng/mL, mPAP was 58.66±20.21, 30.2±4.83, and 17.31±4.51 mmHg, and PVR was 10.12±7.01, 2.19±0.56, and 2.2±1.1 Wood units in groups A, B, and C, respectively. Mean pulmonary output (PO) was 7.24±3.07, 15.79±5.49, 10.18±4.72 L/minute, respectively. CEA levels were positively correlated with PO and negatively correlated with PVR in all of the patients. Conclusion CEA levels are increased with PO and decreased with PVR in CHD-PH.


2013 ◽  
Vol 11 (4) ◽  
pp. 189-195 ◽  
Author(s):  
Jamil A. Aboulhosn

This manuscript is intended to provide a brief overview of the indications for and outcomes of surgical and transcatheter interventions for congenital heart disease and pulmonary hypertension (PH). Pulmonary hypertension is frequently encountered in children and adults with congenital heart disease and is most commonly related to large “central” shunts, ie, those occurring at the ventricular or great arterial level (Figure 1). If uncorrected early in infancy or childhood, large central shunts result in increased pulmonary blood flow, left heart volume overload, PH, and heart failure. If the child survives this initial period of volume overload and heart failure, they will very likely develop effacement of the normal pulmonary arterial architecture and severe elevations in pulmonary arterial resistance, eventually resulting in cyanosis and Eisenmenger syndrome.1


2007 ◽  
Vol 6 (3) ◽  
pp. 136-141 ◽  
Author(s):  
Michael J. Landzberg

Pulmonary arterial hypertension associated with congenital heart disease (CHD-PAH), as discussed throughout this issue of Advances in Pulmonary Hypertension, is one of the most commonly occurring causes of significant or severe morbidity and untimely mortality in CHD patients.1 Without surgical therapy, it is suggested that some 30% of CHD patients will develop PAH.2 Early recognition and treatment of CHD is often discussed, therefore, as the most effective preventive treatment measure for patients at risk for pulmonary vascular disease. Epidemiologic estimates of occur-rence of CHD-PAH have targeted approximately 15% of all CHD survivors as having PAH.3


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