Pulmonary Hypertension Due to Common Respiratory Conditions: Classification, Evaluation and Management Strategies

Fein, Daniel G.; Zaidi, Ali N.; Sulica, Roxana

doi:10.3390/jcm5090075

Cited by 13 publications

(12 citation statements)

References 63 publications

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“…LV diastolic dysfunction, characterized by a reduction of transmitral E/A ratio and e' mitral annular velocity and increase of E/e' ratio, was already observed in IPF patients [9]. Our data confirm this impairment of LV filling (lower E/A ratio) and of myocardial relaxation (lower tissue Doppler septal and lateral e' velocity) and the higher E/e' ratio in IPF than in the healthy controls.…”

Section: Diastolic and Systolic Functionsupporting

confidence: 86%

“…Respiratory function declines along with disease progression, and changes in lung diffusion capacity of carbon monoxide (DLCO) and forced vital capacity (FVC) are both independent predictors of worse prognosis [1]. Pulmonary arterial hypertension (PAH) is frequently found in the early stages of IPF and the outcome is directly related to the capacity of right ventricular (RV) function to adapt to the elevated afterload [8,9]. The combination of severe vascular and fibrotic abnormalities induces changes in right ventricular (RV) structure and function until heart failure onset [1].…”

Section: Introductionmentioning

confidence: 99%

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Impaired Right and Left Ventricular Longitudinal Function in Patients with Fibrotic Interstitial Lung Diseases

Buonauro

Santoro

Galderisi

et al. 2020

JCM

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Background: Left ventricular (LV) and right ventricular (RV) dysfunction is recognized in idiopathic pulmonary fibrosis (IPF). Little is known about cardiac involvement in non-idiopathic pulmonary fibrosis (no-IPF). This issue can be explored by advanced echocardiography. Methods: Thirty-three clinically stable and therapy-naive fibrotic IPF and 28 no-IPF patients, and 30 healthy controls were enrolled. Exclusion criteria were autoimmune systemic diseases, coronary disease, heart failure, primary cardiomyopathies, chronic obstructive lung diseases, pulmonary embolism, primary pulmonary hypertension. Lung damage was evaluated by diffusion capacity for carbon monoxide (DLCOsb). All participants underwent an echo-Doppler exam including 2D global longitudinal strain (GLS) of both ventricles and 3D echocardiographic RV ejection fraction (RVEF). Results: We observed LV diastolic dysfunction in IPF and no-IPF, and LV GLS but not LV EF reduction only in IPF. RV diastolic and RV GLS abnormalities were observed in IPF versus both controls and no-IPF. RV EF did not differ significantly between IPF and no-IPF. DLCOsb and RV GLS were associated in the pooled pulmonary fibrosis population and in the IPF subgroup (β = 0.708, p < 0.001), independently of confounders including pulmonary arterial systolic pressure. Conclusion: Our data highlight the unique diagnostic capabilities of GLS in distinguishing early cardiac damage of IPF from no-IPF patients.

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Section: Diastolic and Systolic Functionsupporting

confidence: 86%

Section: Introductionmentioning

confidence: 99%

Impaired Right and Left Ventricular Longitudinal Function in Patients with Fibrotic Interstitial Lung Diseases

Buonauro

Santoro

Galderisi

et al. 2020

JCM

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“…Drug treatment and pulmonary endarterectomy may help people with PAH [4] and CTEPH [5], respectively, but benefit is often limited. For people with PH secondary to cardiac or pulmonary disease (groups 2 & 3), there are no treatments of proven benefit [6,7].…”

Section: Introductionmentioning

confidence: 99%

“…Recent recommendations support a conservative approach, under the careful supervision of appropriately skilled practitioners [3,9]. However, exercise rehabilitation has not been tested in those with the most common forms of PH (groups 2 & 3), or in an out-patient setting in the UK [6,7,10]. For people living with chronic heart failure (CHF) or chronic obstructive pulmonary disease (COPD), without coexisting PH, exercise rehabilitation is recommended by NICE [11], and the British Thoracic Society [12], supported by a considerable evidence base [13].…”

Section: Introductionmentioning

confidence: 99%

Supervised pulmonary hypertension exercise rehabilitation (SPHERe): study protocol for a multi-centre randomised controlled trial

et al. 2020

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Background: Supervised cardio-pulmonary rehabilitation may be safe and beneficial for people with pulmonary hypertension (PH) in groups 1 (pulmonary arterial hypertension) and 4 (chronic thromboembolic disease), particularly as a hospital in-patient. It has not been tested in the most common PH groups; 2 (left heart disease), 3 (lung disease), or 5 (other disorders). Further it has not been evaluated in the UK National Health Service (NHS) outpatient setting, or with long-term follow-up. The aim of this randomised controlled trial (RCT) is to test the clinical and cost-effectiveness of a supervised exercise rehabilitation intervention with psychosocial support compared to best practice usual care for people with PH in the community/outpatient setting.

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“…It is well accepted that chronic hypoxia stimulates vascular structural changes and lumen narrowing, leading to the alteration of pulmonary vascular responsiveness and contributing to the development of PH 7 . This occurs clinically in more than 30% of patients suffering from chronic obstructive pulmonary diseases (COPD), with a sharply rising death rate subsequently 8 9 . Actually, hypoxic rodent is one of the most commonly used models to dissect the molecular mechanisms and to identify potential therapeutic targets for PH 10 .…”

mentioning

confidence: 99%

Follistatin-like 1 protects against hypoxia-induced pulmonary hypertension in mice

Zhang

Wang

Liu

et al. 2017

Sci Rep

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Pulmonary hypertension (PH) remains a life-limiting disease characterized by pulmonary vascular remodelling due to aberrant proliferation and migration of pulmonary artery smooth muscle cells (PASMCs), thus leading to raised pulmonary arterial pressure and right ventricular hypertrophy. Secreted glycoprotein follistatin-like 1 (FSTL1) has been reported to ameliorate tissue remodelling in cardiovascular injuries. However, the role of FSTL1 in deranged pulmonary arteries remains elusive. We found that there were higher serum levels of FSTL1 in patients with PH related to chronic obstructive pulmonary diseases (COPD) and in mice model of hypoxia-induced PH (HPH). Haploinsufficiency of Fstl1 in mice contributed to an exacerbated HPH, as demonstrated by increased right ventricular systolic pressure, pulmonary arterial muscularization and right ventricular hypertrophy index. Conversely, FSTL1 administration attenuated HPH. In cultured human PASMCs, hypoxia-promoted cellular viability, DNA synthesis and migration were suppressed by exogenous FSTL1 but enhanced by small interfering RNA targeting FSTL1. Additionally, FSTL1 inhibited the proliferation and migration of PASMCs via extracellular regulated kinase (ERK) signal pathway. All these findings indicate that FSTL1 imposed a protective modulation on pulmonary vascular remodelling, thereby suggesting its role in the regulation of HPH.

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Pulmonary Hypertension Due to Common Respiratory Conditions: Classification, Evaluation and Management Strategies

Cited by 13 publications

References 63 publications

Impaired Right and Left Ventricular Longitudinal Function in Patients with Fibrotic Interstitial Lung Diseases

Impaired Right and Left Ventricular Longitudinal Function in Patients with Fibrotic Interstitial Lung Diseases

Supervised pulmonary hypertension exercise rehabilitation (SPHERe): study protocol for a multi-centre randomised controlled trial

Follistatin-like 1 protects against hypoxia-induced pulmonary hypertension in mice

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