Medical Management of Pulmonary Hypertension with Unclear and/or Multifactorial Mechanisms (Group 5): Is There a Role for Pulmonary Arterial Hypertension Medications?

2017 ◽  
Vol 19 (11) ◽  
Author(s):  
Jason Weatherald ◽  
Laurent Savale ◽  
Marc Humbert
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Medical Management ◽  
Pulmonary Arterial ◽  
Group 5

Medical Management of Pulmonary Arterial Hypertension

2018 ◽  
Author(s):  
Inderjit Singh ◽  
Aaron B Waxman
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Medical Management ◽  
Soluble Guanylate Cyclase ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Endothelin Receptor ◽  
Prostacyclin Receptor ◽  
Thromboembolic Pulmonary Hypertension ◽  
Pulmonary Arterial

The medical management of pulmonary arterial hypertension (PAH) has advanced considerably over the years. Once the diagnosis of PAH is made, the medical management includes both conventional and PAH-pathway specific therapies. Five different classes of drugs are now available targeting the endothelin, prostacyclin, and nitric oxide pathways (i.e. endothelin receptor antagonists, phosphodiesterase-5 inhibitors, soluble guanylate cyclase stimulators, prostacyclin analogues, and prostacyclin receptor agonists). These targeted therapies are approved for treatment of patients with PAH and chronic thromboembolic pulmonary hypertension (CTEPH) only. Long-term and event-driven studies of novel drugs have led to further improvement in the medical management of PAH and CTEPH. In this review, we will focus on the medical management of patients with PAH and CTEPH. This review contains 4 figures, 6 Tables and 30 references Keywords: Pulmonary arterial hypertension, Chronic thromboembolic pulmonary hypertension, Medical management, Pulmonary vasodilators, Phosphodiesterase inhibitor, Soluble guanylate cyclase stimulator, Endothelin receptor antagonist, Prostacyclin receptor agonist

scholarly journals Combination therapy with pulmonary arterial hypertension targeted drugs and immunosuppression can be a useful strategy for sarcoidosis-associated pulmonary hypertension: a case report

2020 ◽  
Author(s):  
Keiko Sumimoto ◽  
Yu Taniguchi ◽  
Noriaki Emoto ◽  
Ken-Ichi Hirata
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Functional Class ◽  
Targeted Drugs ◽  
Appropriate Treatment ◽  
Pulmonary Arterial ◽  
Group 5 ◽  
Underlying Mechanisms

Abstract Background Pulmonary hypertension (PH) is one of the significant complications of sarcoidosis. In the clinical classification of PH proposed in the recent world symposium of PH 2018, sarcoidosis-associated PH is classified in Group 5. The mechanisms of sarcoidosis-associated PH are very heterogeneous. There is no evidence of effective treatment for this condition. Case summary We report a case of a 46-year-old female who developed severe pulmonary hypertension due to sarcoidosis. Her haemodynamics, exercise capacity, and functional class remarkably improved after the treatment with pulmonary arterial hypertension (PAH) targeted drugs including macitentan and tadalafil and secondary immunosuppressive therapy with corticosteroids. Discussion This case emphasizes the importance of considering the underlying mechanisms of sarcoidosis-associated PH in order to choose appropriate treatment.

Medical Management of Pulmonary Arterial Hypertension

2018 ◽  
Author(s):  
Inderjit Singh ◽  
Aaron B Waxman
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Medical Management ◽  
Soluble Guanylate Cyclase ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Endothelin Receptor ◽  
Prostacyclin Receptor ◽  
Thromboembolic Pulmonary Hypertension ◽  
Pulmonary Arterial

The medical management of pulmonary arterial hypertension (PAH) has advanced considerably over the years. Once the diagnosis of PAH is made, the medical management includes both conventional and PAH-pathway specific therapies. Five different classes of drugs are now available targeting the endothelin, prostacyclin, and nitric oxide pathways (i.e. endothelin receptor antagonists, phosphodiesterase-5 inhibitors, soluble guanylate cyclase stimulators, prostacyclin analogues, and prostacyclin receptor agonists). These targeted therapies are approved for treatment of patients with PAH and chronic thromboembolic pulmonary hypertension (CTEPH) only. Long-term and event-driven studies of novel drugs have led to further improvement in the medical management of PAH and CTEPH. In this review, we will focus on the medical management of patients with PAH and CTEPH. This review contains 4 figures, 6 Tables and 30 references Keywords: Pulmonary arterial hypertension, Chronic thromboembolic pulmonary hypertension, Medical management, Pulmonary vasodilators, Phosphodiesterase inhibitor, Soluble guanylate cyclase stimulator, Endothelin receptor antagonist, Prostacyclin receptor agonist

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 Heart and lung transplantation in pulmonary arterial hypertension related to congenital heart disease: an unusual indication

2020 ◽  
Vol 4 (S1) ◽  
Author(s):  
Rosaria Barracano ◽  
Heba Nashat ◽  
Andrew Constantine ◽  
Konstantinos Dimopoulos
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Lung Transplantation ◽  
Atrial Septal Defect ◽  
Septal Defect ◽  
Pulmonary Vascular Disease ◽  
Eisenmenger Syndrome ◽  
Pulmonary Arterial ◽  
Recurrent Haemoptysis

Abstract Background Eisenmenger syndrome is a multisystem disorder, characterised by a significant cardiac defect, severe pulmonary hypertension and long-standing cyanosis. Despite the availability of pulmonary hypertension therapies and improved supportive care in specialist centres, Eisenmenger patients are still faced with significant morbidity and mortality. Case presentation We describe the case of a 44-year-old woman with Eisenmenger syndrome secondary to a large secundum atrial septal defect. Her pulmonary vascular disease was treated with pulmonary vasodilators, but she experienced a progressive decline in exercise tolerance, increasing atrial arrhythmias, resulting in referral for transplantation. Her condition was complicated by significant recurrent haemoptysis in the context of extremely dilated pulmonary arteries and in-situ thrombosis, which prompted successful heart and lung transplantation. She made a slow recovery but remains well 3 years post-transplant. Conclusions Patients with Eisenmenger syndrome secondary to a pre-tricuspid lesion, such as an atrial septal defect have a natural history that differs to patients with post-tricuspid shunts; the disease tends to present later in life but is more aggressive, prompting early and aggressive medical intervention with pulmonary arterial hypertension therapies. This case illustrates that severe recurrent haemoptysis can be an indication for expediting transplantation in Eisenmenger syndrome patients.

scholarly journals Sex Dimorphism in Pulmonary Hypertension: The Role of the Sex Chromosomes

Antioxidants ◽  
2021 ◽  
Vol 10 (5) ◽  
pp. 779
Author(s):  
Daria S. Kostyunina ◽  
Paul McLoughlin
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Pulmonary Vascular Resistance ◽  
Vascular Resistance ◽  
Sex Chromosomes ◽  
Bone Morphogenetic Protein 2 ◽  
Sex Dimorphism ◽  
Pulmonary Arterial ◽  
The Impact

Pulmonary hypertension (PH) is a condition characterised by an abnormal elevation of pulmonary artery pressure caused by an increased pulmonary vascular resistance, frequently leading to right ventricular failure and reduced survival. Marked sexual dimorphism is observed in patients with pulmonary arterial hypertension, a form of pulmonary hypertension with a particularly severe clinical course. The incidence in females is 2–4 times greater than in males, although the disease is less severe in females. We review the contribution of the sex chromosomes to this sex dimorphism highlighting the impact of proteins, microRNAs and long non-coding RNAs encoded on the X and Y chromosomes. These genes are centrally involved in the cellular pathways that cause increased pulmonary vascular resistance including the production of reactive oxygen species, altered metabolism, apoptosis, inflammation, vasoconstriction and vascular remodelling. The interaction with genetic mutations on autosomal genes that cause heritable pulmonary arterial hypertension such as bone morphogenetic protein 2 (BMPR2) are examined. The mechanisms that can lead to differences in the expression of genes located on the X chromosomes between females and males are also reviewed. A better understanding of the mechanisms of sex dimorphism in this disease will contribute to the development of more effective therapies for both women and men.

scholarly journals Symptoms, impacts, and suitability of the Pulmonary Arterial Hypertension – Symptoms and Impact (PAH-SYMPACT™) questionnaire in patients with chronic thromboembolic pulmonary hypertension (CTEPH): a qualitative interview study

2021 ◽  
Vol 5 (1) ◽  
Author(s):  
Brooke Currie ◽  
Evan Davies ◽  
Amélie Beaudet ◽  
Larissa Stassek ◽  
Leah Kleinman
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Shortness Of Breath ◽  
Response Options ◽  
Qualitative Interview Study ◽  
Thromboembolic Pulmonary Hypertension ◽  
Pulmonary Arterial ◽  
The Impact

Abstract Background Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare form of pulmonary hypertension caused by blood clots and scar tissue in the blood vessels of the lungs. Health-related quality of life is often significantly impaired in patients with CTEPH. However, a better understanding of how CTEPH symptoms affect patients’ lives is needed to optimally assess the impact of the disease and treatment. Objectives This qualitative study aimed to better understand the symptoms of CTEPH and how they affect patients’ lives, as well as to determine the appropriateness of the Pulmonary Arterial Hypertension – Symptoms and Impact (PAH-SYMPACT™) questionnaire for use in this patient population. Methods Adults diagnosed with CTEPH, recruited from two clinical sites in the US, participated in one-to-one qualitative telephone interviews. They described their experience of CTEPH symptoms and the impact these symptoms have on their lives. They also provided feedback on the comprehensibility and relevance of the PAH-SYMPACT™‘s instructions, items, and response options. Results Participants (N = 12) had a mean age of 62.5 years. Two thirds were female and most (83%) had undergone pulmonary endarterectomy and/or balloon pulmonary angioplasty. The most frequently endorsed symptoms were shortness of breath (endorsed by all 12 participants), fatigue (11 participants), and lightheadedness (10 participants). All participants identified shortness of breath as an “extremely important” symptom, and seven participants rated fatigue as “extremely important.” The most frequent impacts of CTEPH were on ability to walk quickly (endorsed by all 12 participants), ability to walk up inclines or stairs (11 participants), and ability to carry things (11 participants). The PAH-SYMPACT™ items were relevant to most participants and reflected their experience of CTEPH. All participants indicated that no important CTEPH symptoms were missing from the PAH-SYMPACT™. Overall, the instructions, items, and response options of the PAH-SYMPACT™ were clear and easy to understand. Conclusions The symptoms and impacts experienced by patients with CTEPH align with items included in the PAH-SYMPACT™. The PAH-SYMPACT™ appears to be fit for purpose for assessing disease status in patients with CTEPH.

Prognostic relevance of sleep apnea syndrome in patients with pulmonary arterial hypertension

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
J Precek ◽  
K Vykoupil ◽  
F Kovacik ◽  
M Hutyra
Keyword(s):  
Pulmonary Hypertension ◽  
Sleep Apnea ◽  
Pulmonary Arterial Hypertension ◽  
General Population ◽  
Arterial Hypertension ◽  
Left Ventricular ◽  
Sleep Study ◽  
Prognostic Relevance ◽  
Obstructive Sleep ◽  
Pulmonary Arterial

Abstract Introduction Sleep disordered breathing (SDB) is a group of ventilatory disorders during sleep which includes obstructive sleep apnea (OSA), central sleep apnea (CSA), and sleep related hypoventilation. In patients with SDB, the prevalence of pulmonary hypertension (PH) ranges from 17% to 52%. While SDB is prevalent in the general population with recent estimates of 20% to 30%, in those with cardiovascular disease, particularly left ventricular failure, there is a higher reported prevalence of 47%. Aims The aims of this study were to determine the prevalence and prognostic relevance of sleep apnea in a cohort of patients with newly diagnosed pulmonary arterial hypertension (ESC/WHO Group 1 pulmonary hypertension). Methods We evaluated prospectively 76 patients with the pulmonary arterial hypertension (mean age 54±16 years; 45% male). All patients underwent right heart catheterisation, clinical assessments, sleep study, standard laboratory testing and evaluation of subjective sleepiness by the Epworth Sleepiness Scale. Sleep test was provided with an ApneaLink Plus, consisting of nasal pressure sensor, respiratory effort band, and pulse oximeter worn on the finger. Subjects previously treated for or diagnosed with SDB were excluded from the study. Results Sleep apnea (SA) – defined as apnea-hypopnea index (AHI) ≥5/h – was found in 59 (77.6%) of the pulmonary arterial hypertension (PAH) patients. Mean AHI in the cohort of PAH patients with SA was 26.1±16.6/h. Mean follow-up was 24 months, during which 15 (19.7%) patients died. Characteristics of parameters related to SA in groups of survivors and deceased are in table 1. From the sleep apnea-related parameters, only time with O2Sat <90% – T90 was significantly associated with mortality (AUC 0.856; 95% CI 0.693 – 1.019; p<0.001). Conclusions The presence of sleep apnea in pulmonary arterial hypertension patients is high. The prevalence of sleep apnea is higher in PAH patients than in the general population. The presence of sleep apnea in patients with PAH was not associated with worse prognosis, but noctural hypoxemia (time with O2Sat <90%) was related to poor prognosis. Sleep apnea in patients with PAH should be screened for systematically. Funding Acknowledgement Type of funding source: None

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