Disproportionate Pulmonary Hypertension in a Patient with Early-onset Pulmonary Emphysema Treated with Specific Drugs for Pulmonary Arterial Hypertension

Shimizu, Masatoshi; Imanishi, Junichi; Takano, Takatsugu; Miwa, Yohei

doi:10.2169/internalmedicine.50.5995

Cited by 7 publications

(4 citation statements)

References 16 publications

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“…Early studies of the effects of intravenous prostaglandin E1 (PGE1) observed decreases in pulmonary vascular resistance with increased cardiac output but were associated with worsened V/Q mismatch and increased hypoxemia [110]. In a recent case study Shimizu et al [111] reported significant haemodynamic improvement in a patient with disproportionate PH using long-term intravenous infusion of epoprostenol for over 3 years. A more recent study in patients with advanced IPF and PH showed that PH-targeted therapy with trepostinil may improve right heart haemodynamics and echocardiographic function without affecting systemic oxygen saturation [112].…”

Section: Treatmentmentioning

confidence: 99%

Pulmonary Hypertension and Right Heart Dysfunction in Chronic Lung Disease

Zangiabadi

Pasquale

Sajkov

2014

BioMed Research International

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Group 3 pulmonary hypertension (PH) is a common complication of chronic lung disease (CLD), including chronic obstructive pulmonary disease (COPD), interstitial lung disease, and sleep-disordered breathing. Development of PH is associated with poor prognosis and may progress to right heart failure, however, in the majority of the patients with CLD, PH is mild to moderate and only a small number of patients develop severe PH. The pathophysiology of PH in CLD is multifactorial and includes hypoxic pulmonary vasoconstriction, pulmonary vascular remodeling, small vessel destruction, and fibrosis. The effects of PH on the right ventricle (RV) range between early RV remodeling, hypertrophy, dilatation, and eventual failure with associated increased mortality. The golden standard for diagnosis of PH is right heart catheterization, however, evidence of PH can be appreciated on clinical examination, serology, radiological imaging, and Doppler echocardiography. Treatment of PH in CLD focuses on management of the underlying lung disorder and hypoxia. There is, however, limited evidence to suggest that PH-specific vasodilators such as phosphodiesterase-type 5 inhibitors, endothelin receptor antagonists, and prostanoids may have a role in the treatment of patients with CLD and moderate-to-severe PH.

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Section: Treatmentmentioning

confidence: 99%

Pulmonary Hypertension and Right Heart Dysfunction in Chronic Lung Disease

Zangiabadi

Pasquale

Sajkov

2014

BioMed Research International

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“…An early small trial of prostacyclin in COPD patients in acute respiratory failure did not demonstrate improvement [83], but animal models have suggested that prostacyclin may have a protective effect in development of pulmonary hypertension in COPD [84]. Additionally, there has been a case-report of prostacycline being used in a COPD patient to treat disproportionate PH [85]. A clinical trial of Bosentan, an endothelin antagonist FDA approved for treatment of pulmonary arterial hypertension, did not show improvement in hemodynamics, functional capacity or quality of life [86].…”

Section: Treatment Of Pulmonary Vascular Disease In Copdmentioning

confidence: 99%

Cardiopulmonary Coupling in Chronic Obstructive Pulmonary Disease

Rahaghi¹,

Beek²,

Washko³

2014

Journal of Thoracic Imaging

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“…A favorable response to IV EPO was cited in a single case report of a 40-year-old male with emphysema and "out of proportion" PH. 260 Acute inhalation of iloprost in PH-COPD patients may improve exercise capacity and CPET parameters while preserving gas exchange. 261 However, this form of therapy is exceptionally expensive and long-term benefit has not been established.…”

Section: Treatment Of Ph In Copdmentioning

confidence: 99%

Pulmonary Hypertension Complicating Interstitial Lung Disease and COPD

Shino

Lynch

Saggar

et al. 2013

Semin Respir Crit Care Med

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Pulmonary hypertension (PH) may complicate parenchymal lung disease, specifically interstitial lung diseases and chronic obstructive pulmonary disease, and uniformly increases the mortality risk. The epidemiology and degree of PH is variable and unique to the underlying lung disease. The clinician should exercise a high index of suspicion for PH complicating parenchymal lung disease especially given the nonspecific symptomatology and the limitations of echocardiography in this patient population. In general, PH-specific therapies in this setting have been poorly studied, with concern for increased shunting and/or ventilation/perfusion (V/Q) mismatch and resultant hypoxemia. A better understanding of the mechanisms underlying PH related to parenchymal lung disease may lead to novel pharmacological targets to prevent or treat this serious complication.

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Disproportionate Pulmonary Hypertension in a Patient with Early-onset Pulmonary Emphysema Treated with Specific Drugs for Pulmonary Arterial Hypertension

Cited by 7 publications

References 16 publications

Pulmonary Hypertension and Right Heart Dysfunction in Chronic Lung Disease

Pulmonary Hypertension and Right Heart Dysfunction in Chronic Lung Disease

Cardiopulmonary Coupling in Chronic Obstructive Pulmonary Disease

Pulmonary Hypertension Complicating Interstitial Lung Disease and COPD

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