Pulmonary Arterial Hypertension: a Pharmacotherapeutic Update

Coons, James C.; Pogue, Kristen; Kolodziej, Andrew; Hirsch, Glenn A.; George, M Patricia

doi:10.1007/s11886-019-1235-4

Cited by 61 publications

(41 citation statements)

References 64 publications

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“…In addition, soluble guanylate cyclase (sGC) stimulators such as riociguat also act on the very same signaling pathway by increasing the enzymatic activity of sGC independent from endogenous NO leading to increased cGMP levels. Finally, the endothelin receptor antagonists (ERAs) bosentan, ambrisentan, and macitentan counterbalance the vasoconstrictive mode of action of endothelin (ET-1), which is a known vasoconstrictor [30]. In addition to this PAH-specific therapy, anticoagulants and diuretics are commonly used in practice.…”

Section: Pathogeneses and Clinical Manifestationmentioning

confidence: 99%

“…Each category consists of the aforementioned number of items which are rated by points, adding up to a sore of a total maximum of 32 points. Nevertheless, based on the percentage of the total score reached in the Downs and Black [70] scale, human studies were rated of low (<55%; ≤17), moderate (55-79%; 18-25), and high (≥80%; [26][27][28][29][30][31][32] quality. This rating of study quality follows other reviews, which used the same tool, but was adjusted according to more general grading systems with very good, good, and satisfactory classification.…”

Section: Study Quality Assessmentmentioning

confidence: 99%

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Effects of Different Types of Exercise Training on Pulmonary Arterial Hypertension: A Systematic Review

Waller

Krüger

Conrad

et al. 2020

JCM

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Pulmonary arterial hypertension (PAH) represents a chronic progressive disease characterized by high blood pressure in the pulmonary arteries leading to right heart failure. The disease has been a focus of medical research for many years due to its worse prognosis and limited treatment options. The aim of this study was to systematically assess the effects of different types of exercise interventions on PAH. Electronic databases were searched until July 2019. MEDLINE database was used as the predominant source for this paper. Studies with regards to chronic physical activity in adult PAH patients are compared on retrieving evidence on cellular, physiological, and psychological alterations in the PAH setting. Twenty human studies and 12 rat trials were identified. Amongst all studies, a total of 628 human subjects and 614 rats were examined. Regular physical activity affects the production of nitric oxygen and attenuates right ventricular hypertrophy. A combination of aerobic, anaerobic, and respiratory muscle training induces the strongest improvement in functional capacity indicated by an increase of 6 MWD and VO2 peak. In human studies, an increase of quality of life was found. Exercise training has an overall positive effect on the physiological and psychological components of PAH. Consequently, PAH patients should be encouraged to take part in regular exercise training programs.

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Section: Pathogeneses and Clinical Manifestationmentioning

confidence: 99%

Section: Study Quality Assessmentmentioning

confidence: 99%

Effects of Different Types of Exercise Training on Pulmonary Arterial Hypertension: A Systematic Review

Waller

Krüger

Conrad

et al. 2020

JCM

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“…Pulmonary hypertension (PH) is defined as a mean pulmonary artery pressure (mPAP) ≥ 20 mmHg at rest as assessed by right heart catheterization [1], and pulmonary artery sarcoma (PAS) along with chronic thromboembolic pulmonary hypertension (CTEPH) is categorized as Group 4 PH because of the similar hemodynamic index [2] and a pulmonary artery wedge pressure (PAWP) ≤ 15 mmHg. Transthoracic and transesophageal echocardiography (TTE and TEE), computed tomography pulmonary angiography (CTPA), cardiac magnetic resonance imaging (MRI), endobronchial ultrasound transbronchial needle aspiration (EBUS-TBNA) and fluorodeoxyglucose positron emission tomography/CT (FDG-PET/CT) are necessary for the diagnosis of PAS [3][4][5][6].…”

Section: Introductionmentioning

confidence: 99%

Surgical treatment of primary pulmonary artery sarcoma

Han

Zhen

Liu

et al. 2020

Gen Thorac Cardiovasc Surg

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Objective Primary pulmonary artery sarcoma (PAS) is a rare tumor that originates from the intimal layer of the pulmonary artery or pulmonary valve and has a poor prognosis. The standard treatment for this devastating disease remains unclear. This study aimed to summarize the current standard treatments for PAS. Methods From September 2015 to January 2020, six patients were diagnosed with PAS and underwent pulmonary endarterectomy (PEA) at our department. Their medical records were retrospectively reviewed to analyze the clinical characteristics, histopathological features, and postoperative outcomes. Fourteen articles, each reporting at least 6 cases, identified 201 patients diagnosed with PAS, and 158 patients had detailed treatments and follow-up data. Results All of the patients who successfully underwent PEA were alive at follow-up, with a mean survival duration of 11.6 months (7–28 months), and one patient developed recurrence in the right upper lobe lung. Two patients received postoperative chemotherapy. In one patient, the tumor invaded the pulmonary valve. Conclusions PAS resection combined with PEA via the aid of cardiopulmonary bypass and deep hypothermic circulatory arrest could achieve maximal tumor resection in patients without metastatic lesions. An individualized surgery strategy relies on a precise preoperative imaging examination. Moreover, postoperative adjuvant therapy could yield improved survival outcomes.

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“…Although the use of targeted drugs (such as endothelin receptor antagonists, prostacyclin inhibitors, etc.) has increased the survival rate of PAH patients and significant improvements in PAH outcome have been realized in the modern era (median survival 7 years) compared to the National Institutes of Health registry from the early 1980s (median survival 2.8 years) [2], its long-term prognosis remains poor. The aetiology of PAH involves environmental and genetic factors, and its pathogenesis is complex and has not been fully elucidated to date..…”

mentioning

confidence: 99%

Gender differences in pulmonary arterial hypertension patients with BMPR2 mutation: a meta-analysis

Zhu

Zhang

et al. 2020

Respir Res

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Objective: To investigate the differences in the proportions of BMPR2 mutations in familial hereditary pulmonary arterial hypertension (HPAH) and idiopathic pulmonary arterial hypertension (IPAH) between males and females and the relationship between BMPR2 mutation and PAH severity. Methods: A computer was used to search the electronic Cochrane Library, PubMed/MEDLINE, and EMBASE databases for clinical trials containing information on the relationship between PAH prognosis and BMPR2 mutations through March 2019. After obtaining the data, a meta-analysis was performed using Review Manager Version 5.3 and Stata. Results: A meta-analysis was performed on 17 clinical trials (2198 total patients: 644 male, 1554 female). The results showed that among patients with HPAH and IPAH, the BMPR2 mutation rate is higher in male than in female patients [male group (224/644, 34.78%), female group (457/1554, 29.41%), OR = 1.30, 95% CI: 1.06~1.60, P = 0.01, I 2 = 10%]. Furthermore, haemodynamic and functional parameters were more severe in IPAH and HPAH patients with BMPR2 mutations than in those without, and those with BMPR2 mutation were diagnosed at a younger age. The risk of death or transplantation was higher in PAH patients with BMPR2 mutations than in those without (OR = 2.51, 95% CI: 1.29~3.57, P = 0.003, I 2 = 24%). Furthermore, the difference was significant only in male patients (OR = 5.58, 95% CI: 2.16~14.39, P = 0.0004, I 2 = 0%) and not in female patients (OR = 1.41, 95% CI: 0.75~2.67, P = 0.29, I 2 = 0%). Conclusion: Among patients with HPAH and IPAH, men are more likely to have BMPR2 mutations, which may predict more severe PAH indications and prognosis.

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Pulmonary Arterial Hypertension: a Pharmacotherapeutic Update

Cited by 61 publications

References 64 publications

Effects of Different Types of Exercise Training on Pulmonary Arterial Hypertension: A Systematic Review

Effects of Different Types of Exercise Training on Pulmonary Arterial Hypertension: A Systematic Review

Surgical treatment of primary pulmonary artery sarcoma

Gender differences in pulmonary arterial hypertension patients with BMPR2 mutation: a meta-analysis

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