scholarly journals Sequence Variants inBMPR2and Genes Involved in the Serotonin and Nitric Oxide Pathways in Idiopathic Pulmonary Arterial Hypertension and Chronic Thromboembolic Pulmonary Hypertension: Relation to Clinical Parameters and Comparison with Left Heart Disease

Respiration ◽  
2010 ◽  
Vol 79 (4) ◽  
pp. 279-287 ◽  
Author(s):  
Silvia Ulrich ◽  
Justyna Szamalek-Hoegel ◽  
Martin Hersberger ◽  
Manuel Fischler ◽  
Jesus Solera Garcia ◽  
...  
Keyword(s):  
Nitric Oxide ◽  
Pulmonary Hypertension ◽  
Heart Disease ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Idiopathic Pulmonary Arterial Hypertension ◽  
Sequence Variants ◽  
Clinical Parameters ◽  
Left Heart ◽  
Thromboembolic Pulmonary Hypertension ◽  
Pulmonary Arterial

Pulmonary hypertension: definition

ESC CardioMed ◽  
2018 ◽  
pp. 2482-2484
Author(s):  
Stephan Rosenkranz
Keyword(s):  
Pulmonary Hypertension ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Left Heart ◽  
Chronic Lung Diseases ◽  
Common Causes ◽  
Thromboembolic Pulmonary Hypertension ◽  
Underlying Causes ◽  
Pulmonary Arterial ◽  
Clinical Phenotyping ◽  
Global Population

Pulmonary hypertension (PH) in general is characterized by an elevation of pulmonary artery pressure which may result from various underlying causes. PH is a common disorder affecting approximately 1% of the global population, and up to 10% of patients older than 65 years of age. The most common causes are diseases of the left heart and chronic lung diseases, whereas disorders primarily originating from the pulmonary vessels including pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension remain relatively rare conditions. PH is primarily defined by haemodynamic variables, and subdivided into pre- and post-capillary PH. Further information obtained from imaging, pathology, genetics, and clinical phenotyping may be required for accurate subclassification of PH.

Abstract 2305: Pulmonary Vascular Reactivity And Prognosis In Patients With Chronic Thromboembolic Pulmonary Hypertension

Circulation ◽  
2007 ◽  
Vol 116 (suppl_16) ◽  
Author(s):  
Nika Skoro-Sajer ◽  
Nicklas Hack ◽  
Roela Sadushi ◽  
Johannes Jakowitsch ◽  
Diana Bonderman ◽  
...  
Keyword(s):  
Nitric Oxide ◽  
Pulmonary Hypertension ◽  
Arterial Pressure ◽  
Vascular Reactivity ◽  
Pulmonary Arterial Pressure ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Mean Pulmonary Arterial Pressure ◽  
Thromboembolic Pulmonary Hypertension ◽  
Pulmonary Arterial ◽  
Relative Change

Hemodynamic responder status defined as an acute decrease of mean pulmonary arterial pressure (mPAP)>10mmHg and below 40mmHg is associated with improved outcome in patients (pts) with pulmonary arterial hypertension (PAH). Pulmonary vascular reactivity to nitric oxide (NO) is controversial in chronic thromboembolic pulmonary hypertension (CTEPH). We speculated that the magnitude of the acute decrease in mean pulmonary artery pressure (mPAP) after exposure to NO might reflect the degree of small vessel disease in CTEPH and thus, affect long-term outcome. Methods: Right heart catheterization was performed in 62 (55  ± 15 years, 32 female) pts with major-vessel CTEPH, at baseline and during inhalation of 40ppm NO. Within 25±15 days patients underwent pulmonary endarterectomy (PEA). Pts were followed for 11.3±26 months. Predictors of survival were analyzed by Cox regression analysis, and survival was described by Kaplan-Meier curves. Results: Significant reductions in mean pulmonary arterial pressure (mPAP; p<0.001), pulmonary vascular resistance (PVR; p<0.001) and an increase in mixed venous oxygen saturation following NO inhalation were demonstrated (p<0.001) by a paired t-test. Stepwise multivariate analysis revealed the relative change of PVR after NO inhalation as a predictor of survival. Patients whose PVR during NO inhalation declined below 789.8 dynes.s.cm-5 had significantly better outcome than patients with above median PVR. There was a strong negative correlation between the relative change of PVR under NO and recurrent pulmonary hypertension after PEA (p=0.02). Conclusions: Patients with operable CTEPH demonstrated acute pulmonary vascular reactivity, mostly not corresponding to a complete responder status, but accounting for a wide range of decreases of mPAP [change of mPAP (%) (−10.9±14)] and PVR [change of PVR (%) (−17 ±15)]. Reduction of PVR under 800 dynes.s.cm-5 after inhalation of NO was associated with better outcome. Responsiveness to inhaled nitric oxide is a predictor for mid-term survival in adult patients with CTEPH undergoing PEA.

scholarly journals The importance of deep phenotyping PH registries with a focus on the PVRI-GoDeep registry

2020 ◽  
Vol 2020 (1) ◽  
Author(s):  
Paul A Corris
Keyword(s):  
Pulmonary Hypertension ◽  
Heart Disease ◽  
Pulmonary Arterial Hypertension ◽  
Pharmaceutical Industry ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Rare Condition ◽  
Erroneous Belief ◽  
Thromboembolic Pulmonary Hypertension ◽  
Pulmonary Arterial ◽  
Deep Phenotyping

[No abstract. Showing first paragraph of article]Although pulmonary hypertension has long been recognised to complicate many common diseases, especially left-sided heart disease and lung disease, most basic, translational and clinical scientists together with the pharmaceutical industry have,to date, focused predominantly on pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension when developing effective treatments . Both entities are rare, leading to the erroneous belief that pulmonary hypertension in general is a rare condition, and not worthy of major global focus.

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