Pulmonary Arterial Hypertension

ABC

Editorial

Pulmonary Arterial Hypertension and Cardioprotective Interventions

Elida Paula Benquique Ojopi et al. Carolina Rodrigues Tonon Katashi Okoshi Marina Politi Okoshi

The normal pulmonary vasculature is a low-pressure system compared with the systemic vasculature. Pulmonary hypertension is characterized by a mean pulmonary artery pressure higher than 25 mmHg. The primary pathophysiological process of pulmonary hypertension is a restriction of blood flow through the pulmonary circulation, which leads to increased pulmonary vascular resistance and eventual failure of the right ventricle. Pulmonary hypertension may be idiopathic (PAH) or aconsequence of chronic diseases, including left-sided heart failure, parenchymal lung diseases, and thromboembolic disease.

Pathological structural and functional changes of the ventricles are known as cardiac remodeling, which is associated with a poor prognosis in PAH. Currently, no specific treatment is recommended for PAH-induced cardiac remodeling.

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ABC

Case Report

Pulmonary Arterial Hypertension with Features of Venous Involvement: A Detective's Task

Daniel Inácio Cazeiro et al. Rui Plácido Miguel Azeredo Raposo Joana Brito … Alexandra Borba Tatiana Guimarães Eugénia Pinto Pedro Freitas Fausto J. Pinto

Abstract

Pulmonary veno-occlusive disease (PVOD) and pulmonary capillary hemangiomatosis are rare types of histopathological substrates within the spectrum of pulmonary arterial hypertension (PAH) with a very poor prognosis. They are characterized by a widespread fibroproliferative process of the small caliber veins and/or capillaries with sparing of the larger veins, resulting in a pre-capillary pulmonary hypertension phenotype. Clinical presentation is unspecific and similar to other PAH etiologies. Definitive diagnosis is obtained through histological analysis, although lung biopsy is not advised due to a higher risk of complications. However, some additional findings may allow a presumptive clinical diagnosis of PVOD, particularly a history of smoking, chemotherapy drug use, exposure to organic solvents (particularly trichloroethylene), low diffusing capacity for carbon monoxide (DLCO), exercise induced desaturation, and evidence of venous congestion without left heart disease on imaging, manifested by a classical triad of ground glass opacities, septal lines, and lymphadenopathies. Lung transplant is the only effective treatment, and patients should be referred at the time of diagnosis due to the rapid progression of the disease and associated poor prognosis.

We present a case of a 58-year-old man with PAH with features of venous/capillary involvement in which clinical suspicion, prompt diagnosis, and early referral for lung transplantation were determinant factors for the successful outcome.

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ABCIC

Original Article

Relationship Between Echocardiographic Parameters and The Systemic Immune-Inflammation Index in Patients With Pulmonary Arterial Hypertension

Sefa Erdi Ömür et al. Gulsen Genc Tapar Mustafa Yilmaz Abdullah Emre Bektaş Osman Demir

Abstract

Background:

Pulmonary arterial hypertension (PAH) is a chronic disease with high morbidity and mortality. The diagnosis of PAH is mainly made based on echocardiographic parameters and natriuretic peptide levels. However, given the low incidence of PAH worldwide, the diagnosis of PAH can be challenging.

Objectives:

To evaluate the relationship between systemic immune-inflammation (SII) index and PAH.

Methods:

This was a retrospective, cross-sectional study of 110 patients (43 PAH patients and 67 controls). The SII index was compared between the PAH and control groups. A probability(p) <0.05 were deemed to indicate statistical significance.

Results:

The findings of this study indicated that SII index was significantly higher in the PAH group than in the control group (1054.15±439.99 vs. 506.7±180.55, p<0.001). The correlation analysis between SII index and echocardiographic parameters revealed that SII index was moderately correlated with right ventricular fractional area change (FAC) (r:-0.567,p<0.001), systolic pulmonary artery pressure (sPAP) (r:0.593, p<0.001), and tricuspid regurgitation velocity (TRV) (r:0.662,p<0.001). Additionally, the SII index was strongly correlated with right atrial (RA) area (r:0.822,p<0.001), pulmonary artery (PA) diameter (r:0.819, p<0.001), left atrium (LA) diameter (r:0.937,p<0.001), inferior vena cava diameter (r:0.869,p<0.001), tricuspid annular plane systolic excursion (TAPSE) (r:-0.902,p<0.001), TAPSE/sPAP (r:-0.831,p<0.001). In addition, the SII index significantly increased as patients' functional capacity (FC) decreased.

Conclusion:

The SII index is as a simple, inexpensive, noninvasive and easily accessible biochemical parameter that may be useful in the diagnosis and follow-up of PAH patients, especially in centers where echocardiography (ECHO) is not available.

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IJCS

Case Report

A Challenging Case Of Pulmonary Arterial Hypertension: Never Judge By Appearances

Pedro Silvério António et al. Manuel Silvério António Catarina Lopes Resende Tatiana Guimarães … Daniel Cazeiro Yolanda Sá Pereira Fausto José Pinto Rui Plácido

Abstract

We report the case of a patient with symptomatic pulmonary hypertension (PH) associated with diffuse systemic sclerosis (SSc) whose initial assessment suggested a group 3 (clinical classification) PH. The patient had a history of drugs/toxins consumption, which contributed to the development of intrinsic pulmonary vascular disease. This changed the panorama towards the diagnosis of pulmonary arterial hypertension (PAH), with important therapeutic and prognostic implications. In fact, the excellent clinical, laboratory and hemodynamic response to therapy confirmed the hypothesis of a case of drug-associated PAH (DPAH) in a patient with diffuse SSc and lung disease. Considering the presence of DPAH, it was deemed necessary to assess acute vasoreactivity during right heart catheterization (RHC). If criteria were met, the clinical scenario may change towards a favorable and sustained clinical and hemodynamic response with oral calcium channel blockers. However, the response to inhaled nitric oxide was negative in our patient and the therapeutic strategy with dual oral combination therapy with tadalafil and ambrisentan was continued. After six-months of therapy the patient significantly improved, from a high to a low risk of one-year mortality.

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ABC

Editorial

Cardioprotective Effect of Resistance Exercise on Left Ventricular Remodeling Associated with Monocrotaline-Induced Pulmonary Arterial Hypertension

Silvio A. Oliveira-Junior et al. Alex Y. Ogura Marianna R. Carvalho Paula F. Martinez

Pulmonary arterial hypertension (PAH) is characterized by progressive pulmonary vascular resistance, affecting several arteries and arterioles. These changes are associated with increased right ventricular afterload and remodeling, characterized by severe hypertrophy, initially adaptive and derived from vascular pressure gradient.^, Afterward, these alterations are accompanied by right ventricular dilation and impaired contractile performance, resulting in reduced ejection fraction and ventricular failure.^, Clinically, right ventricular size and function parameters alterations have a recognized association with bad prognosis in pulmonary arterial hypertension.

During the PAH development, when right ventricular filling pressure has been increased, and cardiac output is deteriorating, there are concomitant volumetric and pressoric overload into the right ventricle. These effects make the interventricular septum move to the left side, sustaining a paradoxical movement. Next, left ventricle (LV) ejection fraction and diastolic performance are affected, facing impaired early diastolic filling, reduced end-diastolic volume, and adverse remodeling.^, Therefore, LV dysfunction configures a secondary and important effect from PAH onset.^{, ,}

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ABC

Original Article

Soluble Guanylate Cyclase Stimulators (Riociguat) in Pulmonary Hypertension: Data from Real-Life Clinical Practice in a 3-Year Follow-Up

Fernanda Brum Spilimbergo et al. Taís Silveira Assmann Marcelo Bellon Laís Machado Hoscheidt … Cássia Ferreira Braz Caurio Márcia Puchalski Bruno Hochhegger Gabriela Roncato Gisela Martina Bohns Meyer

Abstract

Background

Pulmonary hypertension (PH) is a rare and complex disease with poor prognosis, which requires lifelong treatment.

Objective

To describe 3-year follow-up real-life data on treatment with soluble guanylate cyclase stimulators (Riociguat) of patients with PH, measuring current risk assessment parameters.

Methods

This study retrospectively collected clinical and epidemiological data of patients with PH of group 1 (pulmonary arterial hypertension) and group 4 (chronic thromboembolic PH). Non-invasive and invasive parameters corresponding to the risk assessment were analyzed at baseline and follow-up. Statistical analyses were performed using the SPSS 18.0 software, and p-values < 0.050 were considered statistically significant.

Results

In total, 41 patients receiving riociguat were included in the study. Of them, 31 had already completed 3 years of treatment and were selected for the following analysis. At baseline, 70.7% of patients were in WHO functional class III or IV. After 3 years of treatment, the WHO functional class significantly improved in all patients. In addition, the median of the 6-minute walk test (6MWT) significantly increased from 394 ± 91 m at baseline to 458 ± 100 m after 3 years of follow-up (p= 0.014). The three-year survival rate was 96.7%.

Conclusion

In our real-life cohort, most patients with PH treated with riociguat showed stable or improved risk parameters, especially in the 6MWT, at 3 years of follow-up.

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ABC

Review Article

Update on the Treatment of Pulmonary Arterial Hypertension

Caio J. Fernandes et al. Daniela Calderaro Ana Paula Luppino Assad William Salibe-Filho … Luciana Tamie Kato-Morinaga Susana Hoette Bruna Piloto Marcela Araújo Castro Roberta Pontes Lisboa Taysa Antonia Felix da Silva Murillo de Araújo Martins Jose L. Alves-Jr Carlos Jardim Mario Terra-Filho Rogerio de Souza

Abstract

In the last decades, important advances have been made in the treatment of pulmonary arterial hypertension (PAH), a severe, progressive, incurable, and potentially fatal disease. For an adequate therapy, correct hemodynamic diagnosis and etiology classification are fundamental. Many etiologies – rheumatic disease, portal hypertension, congenital heart diseases, schistosomiasis – require specific measures, in addition to drug therapy for PAH. The specific therapy for PAH is based on medications that act on three pathophysiological pathways – prostacyclin, endothelin, and nitric oxide pathways. These drugs have multiple presentations (oral, intravenous, subcutaneous, and inhaled) and have changed the history of PAH. This review presents an overview of drug therapy strategies and different forms and peculiarities of PAH.

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