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

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.

scholarly journals Echocardiography for the Assessment of Pulmonary Hypertension and Congenital Heart Disease in the Young

Diagnostics ◽  
2020 ◽  
Vol 11 (1) ◽  
pp. 49
Author(s):  
Katharina Meinel ◽  
Martin Koestenberger ◽  
Hannes Sallmon ◽  
Georg Hansmann ◽  
Guido E. Pieles
Keyword(s):  
Congenital Heart Disease ◽  
Pulmonary Hypertension ◽  
Heart Disease ◽  
Congenital Heart ◽  
Delayed Diagnosis ◽  
Diagnostic Procedures ◽  
Pulmonary Vascular Disease ◽  
Special Focus ◽  
Catheterization Laboratory ◽  
Complex Anatomy

While invasive assessment of hemodynamics and testing of acute vasoreactivity in the catheterization laboratory is the gold standard for diagnosing pulmonary hypertension (PH) and pulmonary vascular disease (PVD) in children, transthoracic echocardiography (TTE) serves as the initial diagnostic tool. International guidelines suggest several key echocardiographic variables and indices for the screening studies when PH is suspected. However, due to the complex anatomy and special physiological considerations, these may not apply to patients with congenital heart disease (CHD). Misinterpretation of TTE variables can lead to delayed diagnosis and therapy, with fatal consequences, or–on the other hand-unnecessary invasive diagnostic procedures that have relevant risks, especially in the pediatric age group. We herein provide an overview of the echocardiographic workup of children and adolescents with PH with a special focus on children with CHD, such as ventricular/atrial septal defects, tetralogy of Fallot or univentricular physiology. In addition, we address the use of echocardiography as a tool to assess eligibility for exercise and sports, a major determinant of quality of life and outcome in patients with PH associated with CHD.

Pulmonary hypertension associated with congenital heart disease

2020 ◽  
pp. 313-332
Author(s):  
Ryan Coleman ◽  
Corey Chartan ◽  
Nidhy Varghese
Keyword(s):  
Congenital Heart Disease ◽  
Pulmonary Hypertension ◽  
Heart Disease ◽  
Trisomy 21 ◽  
Congenital Heart ◽  
Pulmonary Arteries ◽  
Pulmonary Vascular Disease ◽  
Single Ventricle Physiology ◽  
Vascular Bed ◽  
Therapeutic Considerations

The spectrum of pulmonary hypertension associated with congenital heart disease (PH-CHD) is broad and complex. The most common aetiologies are related to left-to-right shunts, resulting in volume loading of the pulmonary circulation (precapillary pulmonary hypertension) and left-sided obstructive disease (post-capillary pulmonary hypertension). However, in addition to these basic classifications are patients with absent pulmonary arteries, large septal defects, duct-dependent lesions, cardiomyopathies, and, perhaps the most challenging, patients with single ventricle physiology lacking a subpulmonic ventricle. It is therefore imperative that physicians taking care of children with PH-CHD are well versed not only in the different physiologies present in these children, but also in those that result from their respective surgical palliations as well and how these flows and pressures relate to their pulmonary vascular disease burden. This chapter presents the case of a child with trisomy 21 and congenital heart disease who presented late to medical care and required significant modulation of her pulmonary vascular bed to successfully undergo surgical correction. The child’s clinical course, an overview of PH-CHD and its diagnosis and management, and important therapeutic considerations are presented.

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.

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