Screening for pulmonary arterial hypertension

ESC CardioMed ◽  
2018 ◽  
pp. 2506-2507
Author(s):  
John Coghlan
Keyword(s):  
Congenital Heart Disease ◽  
Pulmonary Hypertension ◽  
Portal Hypertension ◽  
Congenital Heart ◽  
Diagnostic Tools ◽  
Primary Screening ◽  
Mutation Carriers ◽  
Pulmonary Arterial ◽  
Hypertension Screening ◽  
Low Prevalence

Screening programmes for pulmonary hypertension are justifiable in some circumstances but not others. The inherent inaccuracies of the diagnostic tools and the low prevalence of pulmonary hypertension renders screening programmes ineffective unless the population evaluated with the primary screening tool (echocardiography) can be enriched. At the recent World Symposium of Pulmonary Hypertension screening programmes were recommended in asymptomatic systemic sclerosis, BMPR2 mutation carriers, patients with portal hypertension, and some groups with HIV, hereditary telangiectasis(HHT) or mutation carriers for HHT and congenital heart disease.

Screening for pulmonary arterial hypertension

ESC CardioMed ◽  
2018 ◽  
pp. 2506-2507
Author(s):  
John Coghlan
Keyword(s):  
Pulmonary Hypertension ◽  
Portal Hypertension ◽  
Systemic Sclerosis ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Diagnostic Tools ◽  
Primary Screening ◽  
Class 1 ◽  
Pulmonary Arterial ◽  
Low Prevalence

Screening programmes for pulmonary hypertension are justifiable in some circumstances but not others. The inherent inaccuracies of the diagnostic tools and the low prevalence of pulmonary hypertension renders screening programmes ineffective unless the population evaluated with the primary screening tool (echocardiography) can be enriched. Nevertheless, significant progress has been made over the past 5 years and screening programmes are now strongly recommended (class 1 recommendation) in asymptomatic systemic sclerosis, BMPR2 mutation carriers, first-degree relatives of patients with hereditable pulmonary hypertension, and patients with portal hypertension referred for transplantation.

Pulmonary hypertension

ESC CardioMed ◽  
2018 ◽  
pp. 781-784
Author(s):  
Shahin Moledina ◽  
Bejal Pandya
Keyword(s):  
Quality Of Life ◽  
Congenital Heart Disease ◽  
Pulmonary Hypertension ◽  
Cardiac Surgery ◽  
Heart Disease ◽  
Congenital Heart ◽  
Eisenmenger Syndrome ◽  
Pulmonary Arterial ◽  
Careful Assessment

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.

Pulmonary hypertension

2020 ◽  
pp. 3695-3710
Author(s):  
Nicholas W. Morrell
Keyword(s):  
Congenital Heart Disease ◽  
Pulmonary Hypertension ◽  
Heart Disease ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Lung Disease ◽  
Congenital Heart ◽  
Heart Function ◽  
Gene Encoding ◽  
Pulmonary Arterial

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.

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
Keyword(s):  
Congenital Heart Disease ◽  
Pulmonary Hypertension ◽  
Heart Disease ◽  
Neuropeptide Y ◽  
Congenital Heart ◽  
Control Group ◽  
Child Patients ◽  
Before And After ◽  
Pulmonary Arterial ◽  
Positive Correlations

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.

scholarly journals Carcinoembryonic antigen levels are increased with pulmonary output in pulmonary hypertension due to congenital heart disease

2020 ◽  
Vol 48 (11) ◽  
pp. 030006052096437
Author(s):  
Yang Zi-yang ◽  
Zhao Kaixun ◽  
Luo Dongling ◽  
Yin Zhou ◽  
Zhou Chengbin ◽  
...  
Keyword(s):  
Congenital Heart Disease ◽  
Pulmonary Hypertension ◽  
Heart Disease ◽  
Carcinoembryonic Antigen ◽  
Congenital Heart ◽  
Pulmonary Artery Hypertension ◽  
Group A ◽  
Serum Cea ◽  
Pulmonary Arterial ◽  
Group B

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.

The Role of Catheter-Based and Surgical Treatments in Patients With Congenital Heart Disease and Pulmonary Hypertension

2013 ◽  
Vol 11 (4) ◽  
pp. 189-195 ◽  
Author(s):  
Jamil A. Aboulhosn
Keyword(s):  
Heart Failure ◽  
Congenital Heart Disease ◽  
Pulmonary Hypertension ◽  
Heart Disease ◽  
Congenital Heart ◽  
Initial Period ◽  
Volume Overload ◽  
Heart Volume ◽  
Pulmonary Arterial ◽  
Transcatheter Interventions

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

scholarly journals Emerging Therapy of Congenital Heart Disease Associated with Pulmonary Hypertension

2007 ◽  
Vol 6 (3) ◽  
pp. 136-141 ◽  
Author(s):  
Michael J. Landzberg
Keyword(s):  
Congenital Heart Disease ◽  
Pulmonary Hypertension ◽  
Heart Disease ◽  
Congenital Heart ◽  
Early Recognition ◽  
Preventive Treatment ◽  
Pulmonary Vascular Disease ◽  
Patients At Risk ◽  
Pulmonary Arterial ◽  
Emerging Therapy

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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