Pulmonary Arterial Hypertension In the Modern Era: The Intersection of Genotype and Phenotype

Pulmonary arterial hypertension (PAH) is a severe cardiopulmonary disease that is characterized by increased vascular resistance and early progression to right heart failure clinically, which occurs due to plexigenic, hypertrophic, and fibrotic effacement of distal pulmonary arterioles without systemic vascular involvement.1 There are several features that distinguish PAH from many other vascular disorders. First, the prevalence of PAH is ∼20 per 1,000,000 individuals, and, thus, it is classified as rare.
Source: The Journal of Heart and Lung Transplantation - Category: Transplant Surgery Authors: Source Type: research

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Fight Aging! publishes news and commentary relevant to the goal of ending all age-related disease, to be achieved by bringing the mechanisms of aging under the control of modern medicine. This weekly newsletter is sent to thousands of interested subscribers. To subscribe or unsubscribe from the newsletter, please visit: https://www.fightaging.org/newsletter/ Longevity Industry Consulting Services Reason, the founder of Fight Aging! and Repair Biotechnologies, offers strategic consulting services to investors, entrepreneurs, and others interested in the longevity industry and its complexities. To find out m...
Source: Fight Aging! - Category: Research Authors: Tags: Newsletters Source Type: blogs
Despite advances in treatment of idiopathic pulmonary arterial hypertension (IPAH), there remains no medical cure and patients can experience disease progression leading to right heart failure, progressive exercise intolerance and death. The reversed Potts shunt (left pulmonary artery to descending aorta) was re-introduced for treatment of end-stage IPAH to permit decompression of the suprasystemic right ventricle by right to left shunting, with preservation of upper body oxygenation. The shunt has the potential to delay the need for lung transplantation and offer a treatment for those who are transplant ineligible.
Source: The Journal of Thoracic and Cardiovascular Surgery - Category: Cardiovascular & Thoracic Surgery Authors: Source Type: research
In conclusion, high-dose NR induces the onset of WAT dysfunction, which may in part explain the deterioration of metabolic health. Towards a Rigorous Definition of Cellular Senescence https://www.fightaging.org/archives/2019/11/towards-a-rigorous-definition-of-cellular-senescence/ The accumulation of lingering senescent cells is a significant cause of aging, disrupting tissue function and generating chronic inflammation throughout the body. Even while the first senolytic drugs capable of selectively destroying these cells already exist, and while a number of biotech companies are working on the productio...
Source: Fight Aging! - Category: Research Authors: Tags: Newsletters Source Type: blogs
This report is the first instance in which a patient with severe PAH, survived a successful atrial septal defect (ASD) repair and bilateral lung transplantation during puerperium. Patient concerns: A 42-year-old pregnant woman with congenital heart disease (CHD) and severe PAH was admitted to our hospital for the management of pregnancy and delivery. The patient was diagnosed with severe PAH in 2013, and no significant improvements or deteriorations were found until this pregnancy-related hospital admission. Diagnosis: The patient was diagnosed with CHD and severe PAH in 2013 with color Doppler echocardiography, righ...
Source: Medicine - Category: Internal Medicine Tags: Research Article: Clinical Case Report Source Type: research
Cystic fibrosis (CF) is the most common genetically inherited disease in the Caucasian population [1]. Respiratory manifestations of CF include reduction of mucus clearance, chronic pulmonary infections, and bronchiectasis, causing a progressive respiratory failure that is the primary cause of death in CF patients. Advanced CF might be complicated by pulmonary hypertension, right ventricular hypertrophy, and right heart failure [2]. Bilateral lung transplantation (LUTX) is a viable option for CF, providing a significant survival benefit [3].
Source: Journal of Cystic Fibrosis - Category: Respiratory Medicine Authors: Source Type: research
ConclusionsIn these patients, PAC can be a potential prognostic predictor and patients with a higher comorbidity burden have worse outcomes.
Source: Heart and Lung: The Journal of Acute and Critical Care - Category: Respiratory Medicine Source Type: research
Abstract Pulmonary hypertension is a term used to describe a complex multifactorial group of conditions diagnosed by an elevated mean pulmonary artery pressure of 20 mm Hg or higher on right heart catheterization. The diagnosis of pulmonary hypertension in pregnancy is important, as it is associated with high rates of maternal morbidity and mortality, even with modern management. Diagnostic testing is important for establishing the diagnosis, type, and severity of pulmonary hypertension, which in turn, dictates treatment options. Echocardiographic assessment is the first step in diagnosis and the gold standard for...
Source: Obstetrics and Gynecology - Category: OBGYN Authors: Tags: Obstet Gynecol Source Type: research
In this study, researchers studied 438,952 participants in the UK Biobank, who had a total of 24,980 major coronary events - defined as the first occurrence of non-fatal heart attack, ischaemic stroke, or death due to coronary heart disease. They used an approach called Mendelian randomisation, which uses naturally occurring genetic differences to randomly divide the participants into groups, mimicking the effects of running a clinical trial. People with genes associated with lower blood pressure, lower LDL cholesterol, and a combination of both were put into different groups, and compared against those without thes...
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We examined human lung tissue from COPD patients and normal control subjects, and found a substantial increase in p16-expressing alveolar cells in COPD patients. Using a transgenic mouse deficient for p16, we demonstrated that lungs of mice lacking p16 were structurally and functionally resistant to CS-induced emphysema due to activation of IGF1/Akt regenerative and protective signaling. Fat Tissue Surrounds Skeletal Muscle to Accelerate Atrophy in Aging and Obesity https://www.fightaging.org/archives/2019/09/fat-tissue-surrounds-skeletal-muscle-to-accelerate-atrophy-in-aging-and-obesity/ Researchers her...
Source: Fight Aging! - Category: Research Authors: Tags: Newsletters Source Type: blogs
Publication date: July 2019Source: The Annals of Thoracic Surgery, Volume 108, Issue 1Author(s): Ji-ming Sha, Yi Cao, Sheng-song XuAnomalous origin of the pulmonary artery from the ascending aorta can lead to congestive heart failure in infancy, and with advancing age many patients will experience severe pulmonary hypertension. Surgical intervention has high mortality and morbidity risks if this happens. Strategies to manage these patients seem only limited to heart–lung transplantation or lung transplantation. Here, we successfully performed surgical intervention in an adult patient who had anomalous origin of the r...
Source: The Annals of Thoracic Surgery - Category: Cardiovascular & Thoracic Surgery Source Type: research
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