Advances in Pulmonary Hypertension
Latest Publications


TOTAL DOCUMENTS

671
(FIVE YEARS 78)

H-INDEX

8
(FIVE YEARS 2)

Published By Pulmonary Hypertension Association

1933-088x

2021 ◽  
Vol 20 (5) ◽  
pp. 150-156
Author(s):  
Rachel T Sullivan ◽  
Eric D Austin

There has been significant advancement in the understanding of the genetics of pulmonary hypertension (PH), particularly in those with heritable or idiopathic pulmonary arterial hypertension. In addition to genetic variants with a primarily pulmonary vascular disease phenotype, the prevalence of PH in other genetic syndromes is increasingly recognized. We will review the current knowledge of PH associated with multisystem genetic syndromes. There is high prevalence of coexisting cardiac and pulmonary disease, making it challenging to discern whether PH is secondary to these processes or underlying genetic makeup. There is a paucity of data on response to PH-targeted therapy and implications on overall prognosis.


2021 ◽  
Vol 20 (5) ◽  
pp. 141-141
Author(s):  
Usha S. Krishnan ◽  
Greg Elliott
Keyword(s):  

2021 ◽  
Vol 20 (5) ◽  
pp. 168-175
Author(s):  
Greg Elliott ◽  
Usha S. Krishnan ◽  
Wendy K. Chung ◽  
Paul Yu ◽  
Eric D Austin

2021 ◽  
Vol 20 (5) ◽  
pp. 142-149
Author(s):  
Carrie Lynn Welch ◽  
Wendy K. Chung

Background - Pulmonary hypertension (PH), defined by mean pulmonary artery pressure >20 mmHg, is a common physiologic manifestation of many diseases. Pulmonary arterial hypertension (PAH) represents a smaller subgroup of patients who have PH, and PAH causes significant cardiorespiratory morbidity and premature mortality. PH can manifest across the lifespan, with similar incidence for both pediatric- and adult-onset disease. However, pediatric-onset disease is particularly challenging because it is frequently associated with a more severe clinical course and comorbidities including lung and heart developmental anomalies. For PH Group 1/pulmonary arterial hypertension, causal genetic variants can be identified in ~13% of adults and ~43% of children. Clinical implications – Education about the option for genetic testing is strongly recommended for all pediatric and adult HPAH/IPAH patients. Both gene panel and exome/genome sequencing tests can be useful in diagnosis, but exome/genome sequencing provides a comprehensive dataset for reanalysis over time for cases without an initial diagnosis. Knowledge of genetic diagnoses can immediately impact clinical management of PH, including multimodal medical treatment, surgical intervention, transplantation decisions, and screening for associated conditions. Conclusions - There is a need for large, diverse, international consortia with ever-improving analytical pipelines to confirm previously implicated genes, identify additional genes/variants, assess penetrance, and clinically characterize each genetic subtype for natural history, prognosis and response to therapies to inform more precise clinical management.


2021 ◽  
Vol 20 (4) ◽  
pp. 119-122
Author(s):  
Eileen M. Harder ◽  
Aaron B. Waxman

Pulmonary hypertension associated with interstitial lung disease signifies worse outcomes. Given previous negative clinical trials, the use of pulmonary vasodilators in pulmonary hypertension associated with interstitial lung disease has traditionally been on a case-by-case basis; however, the recent INCREASE study has led to the first and milestone approval of inhaled treprostinil for this population. This review discusses the management of pulmonary hypertension associated with interstitial lung disease from the pulmonary vascular perspective, with an emphasis on clinical trials in this population.


2021 ◽  
Vol 20 (4) ◽  
pp. 102-102
Author(s):  
Deborah Jo Levine

Sign in / Sign up

Export Citation Format

Share Document