Pulmonary hypertension: epidemiology

ESC CardioMed ◽  
2018 ◽  
pp. 2484-2487
Author(s):  
Jose Leonidas Alves ◽  
Susana Hoette ◽  
Rogério Souza
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Health System ◽  
Heart Diseases ◽  
Developed Countries ◽  
Older Age ◽  
Disease Awareness ◽  
The World ◽  
Pulmonary Arterial

The epidemiology of pulmonary hypertension has been better studied in the last decade. Registries coming from all parts of the world allowed not only the recognition of a change in pulmonary hypertension demographics in developed countries but also the existence of regional peculiarities in less developed regions that need to be accounted for in the development of appropriate health policies. Although left-sided heart diseases and lung diseases are believed to be the most prevalent causes of pulmonary hypertension, most of the available data are related to causes of pulmonary arterial hypertension. In this particular group, patients are now diagnosed at an older age, as compared with the data from the first national registries, from about 20 years ago. The reasons for such changes are not clear and might be related to factors such as disease awareness, diagnostic approach, treatment availability, and health system accessibility rather than to a change in the disease itself. This chapter summarizes the available data regarding the current epidemiology of pulmonary hypertension.

Pulmonary Arterial Hypertension: Epidemiology and Registries

2014 ◽  
Vol 13 (1) ◽  
pp. 21-26 ◽  
Author(s):  
Michael D. McGoon ◽  
Marc Humbert
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Task Force ◽  
The World ◽  
Important Means ◽  
Pulmonary Arterial

Registries of pulmonary arterial hypertension (PAH) are important means by which to characterize the presentation and outcome of patients and to provide a basis for predicting the course of the disease. This article summarizes the published conclusions of the World Symposium of Pulmonary Hypertension task force that addressed registries and epidemiology of PAH.

scholarly journals Acute vasoreactivity testing in pediatric idiopathic pulmonary arterial hypertension: an international survey on current practice

2019 ◽  
Vol 9 (4) ◽  
pp. 204589401985753
Author(s):  
Lina Caicedo ◽  
Rachel Hopper ◽  
Humberto Garcia Aguilar ◽  
Dunbar Ivy ◽  
Dora Haag ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Functional Class ◽  
Idiopathic Pulmonary Arterial Hypertension ◽  
Clinical Criteria ◽  
First Line Therapy ◽  
Specific Protocol ◽  
The World ◽  
Pulmonary Arterial

The aim of this study was to determine practice patterns and inter-institutional variability in how acute vasoreactivity testing (AVT) is performed and interpreted in pediatrics throughout the world. A survey was offered to physicians affiliated with the Pediatric & Congenital Heart Disease Taskforce of the Pulmonary Vascular Research Institute (PVRI), the Pediatric Pulmonary Hypertension Network (PPHNET), or the Spanish Registry for Pediatric Pulmonary Hypertension (REHIPED), from February to December 2016. The survey requested data about the site-specific protocol for AVT and subsequent management of pediatric patients with idiopathic pulmonary arterial hypertension (IPAH) or heritable PAH (HPAH). Twenty-eight centers from 13 countries answered the survey. AVT is performed in most centers using inhaled nitric oxide (iNO). Sitbon criteria was used in 39% of the centers, Barst criteria in 43%, and other criteria in 18%. First-line therapy for positive AVT responders in functional class (FC) I/II was calcium channel blocker (CCB) in 89%, but only in 68% as monotherapy. Most centers (71%) re-evaluated AVT-positive patients hemodynamics after 6–12 months; 29% of centers re-evaluated based only on clinical criteria. Most centers (64%) considered a good response as remaining in FC I or II, with near normalization of pulmonary arterial pressure and pulmonary vascular resistance, but a stable FC I/II alone was sufficient criteria in 25% of sites. Protocols and diagnostic criteria for AVT, and therapeutic approaches during follow-up, were highly variable across the world. Reported clinical practice is not fully congruent with current guidelines, suggesting the need for additional studies that better define the prognostic value of AVT for pediatric IPAH patients.

Pulmonary hypertension due to chronic lung diseases

ESC CardioMed ◽  
2018 ◽  
pp. 2547-2550
Author(s):  
Ari Chaouat ◽  
Anne Guillaumot ◽  
Emmanuel Gomez ◽  
Olivier Huttin ◽  
Christine Selton-Suty ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Lung Diseases ◽  
Heart Diseases ◽  
Severe Pulmonary Hypertension ◽  
Therapeutic Implications ◽  
Chronic Lung Diseases ◽  
Pulmonary Haemodynamics ◽  
Pulmonary Arterial

Mild-to-moderate pulmonary hypertension is a common complication of chronic lung diseases. Very few patients have severe pulmonary hypertension; if that is the case, it may be due to co-morbidities or correspond to a particular phenotype. Although pulmonary hypertension has little or no involvement in exercise limitation, it is an independent prognostic factor for survival. The first stage of diagnosis of pulmonary hypertension in chronic lung disease is to establish a level of high, intermediate, or low probability using Doppler echocardiography according to the European Society of Cardiology/European Respiratory Society Guidelines. To determine the cause of pulmonary hypertension, it may be necessary to perform a comprehensive search for the most frequent respiratory and cardiovascular diseases. There are few, but important, indications for right heart catheterization in chronic lung diseases. The main indications are candidates for lung transplantation, suspicion of pulmonary arterial hypertension or chronic thromboembolic pulmonary hypertension, and when pulmonary haemodynamics are likely to have significant therapeutic implications. The underlying respiratory disease or associations of diseases should be treated according to current guidelines before concluding that a patient has severe pulmonary hypertension. Non-invasive ventilation and long-term oxygen therapy in hypercapnic and hypoxaemic patients, respectively, improve pulmonary haemodynamics. With the exception of cardiovascular co-morbidities, pharmacotherapies for high blood pressure and for left heart diseases are not recommended to treat pulmonary hypertension due to chronic lung diseases. Pulmonary arterial hypertension-approved therapies are not recommended in pulmonary hypertension due to chronic lung diseases.

scholarly journals Correlation of Echocardiographic Variables with Exercise Capacity in Patients with Pulmonary Hypertension due to Congenital Heart Diseases

QJM ◽  
2020 ◽  
Vol 113 (Supplement_1) ◽  
Author(s):  
B H A Elsayed ◽  
A M M Zaky ◽  
A M Roshdy ◽  
H M Attia
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Functional Capacity ◽  
Congenital Heart ◽  
Independent Predictor ◽  
Heart Diseases ◽  
Congenital Heart Diseases ◽  
Pulmonary Arterial ◽  
The Right

Abstract Background Pulmonary arterial hypertension (PAH) is a disease of the pulmonary circulation with resultant effects on the right heart. Conceptually, left heart anatomy and function should be normal in patients with PAH. However, the RV and left ventricle (LV) do not function in isolation, sharing a common pericardial sac and inter-ventricular septum which in turn refers to the significance of assessment of both ventricles which has implications for patient prognosis with PAH. Aim Study the correlation between echocardiographic hemodynamic parameters and functional capacity in patients with pulmonary hypertension due to congenital heart disease and detection which parameter is the most independent predictor of functional capacity in patients with PAH-CHD. Methodology The study was conducted in the period from November 2016 to September 2017 including 34 patients who had been diagnosed with pulmonary arterial hypertension associated with congenital heart diseases (PAH-CHD). All of them were recruited from the congenital heart diseases unit at Ain Shams University hospital. The studied group was divided into two groups in a cross-sectional study: Group I: This included 21 patients with functional capacity according the 6MWT less than 330 m as an average for all subjects. Group II: This included 13 patients with functional capacity more than 330 m. Patients’ participation was voluntary and the subject had the right to withdraw from the study at any time without affecting his/her further medical care. Results The whole study population was divided according to the 6MW distance into two groups less than 330 and more than or equal 330 m. Comparing the 6MWD of each patient with the different Echocardiographic variables of systolic & diastolic dysfunction revealed that the group of shorter 6MW distance had higher RVSP, MPAP, E/e’ ratio, LVMPI and RVMPI, while they had lower LV e’ TDI, LV GRS, RV FAC and RV GLS (less negative value). Multivariate analysis of these Echocardiographic variables revealed that MPAP is independent predictor of 6MWD & thus an independent predictor of functional capacity in PAH-CHD patients. Conclusion Assessment of RV and LV function in PAH-CHD patients is important. Strain measurement by STE provides valuable information on the quantitative RV and LV functional assessment in the setting of volume and pressure overload. The MPAP and saturation were found to be the most independent predictors affecting the functional capacity of the patients with PAH-CHD. Recommendations Assessment of functional capacity in patients with PH using the 6MWT is recommended as a routine investigation from diagnosis and during follow-up and correlated with the echocardiographic variables, it has been shown to be practical and feasible and provides an easy and not expensive tool for clinical and prognostic assessment.

Pulmonary hypertension in ACHD

2017 ◽  
Author(s):  
Sara Thorne ◽  
Sarah Bowater
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Connective Tissue ◽  
Arterial Hypertension ◽  
Connective Tissue Disease ◽  
Lower Risk ◽  
Developed Countries ◽  
Common Complication ◽  
Risk Patients ◽  
Pulmonary Arterial

Pulmonary arterial hypertension (PAH) is a common complication in ACHD patients, with a prevalence of around 10% in developed countries. It is most commonly due to large, uncorrected left-to-right shunts, although a permissive genotype might explain its development in other, seemingly lower-risk, patients. Histologically, PAH in ACHD is the same as PAH due to other aetiologies, such as idiopathic and connective tissue disease.

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