The paradox of pulmonary arterial hypertension in Italy in the COVID-19 era: is risk of disease progression around the corner?

Badagliacca, Roberto; Papa, Silvia; Golino, Paolo; Ghio, Stefano; Agostoni, Piergiuseppe; Ameri, Pietro; Argiento, Paola; Brunetti, Natale Daniele; Casamassima, Vito; Casu, Gavino; Cedrone, Nadia; Confalonieri, Marco; Corda, Marco; Correale, Michele; D’Agostino, Carlo; Michele, Lucrezia De; Famoso, Giulia; Giudice, Giuseppe; Greco, Alessandra; Lombardi, Carlo; Manzi, Giovanna; Madonna, Rosalinda; Mercurio, Valentina; Mulè, Massimiliano; Paciocco, Giuseppe; Romaniello, A.; Romeo, Emanuele; Serra, Walter; Stolfo, Davide; Toma, Matteo; Vatrano, Marco; Vitulo, Patrizio; Vizza, Carmine Dario

doi:10.1183/13993003.02276-2021

Cited by 9 publications

(15 citation statements)

References 28 publications

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“…The risk of death due to COVID‐19 among PAH/CTEPH patients is much higher compared to the general population. In the first months of the pandemic, March–May 2020, the mortality rate due to COVID‐19 among PAH/CTEPH patients was reported to be around 45% 13,29 ; in subsequent months the rate was 8%–22% 8–12 . According to various authors, 30%–81% of PAH/CTEPH patients required hospitalization due to COVID‐19 8–13,29 .…”

Section: Discussionmentioning

confidence: 99%

“…Chronic thromboembolic pulmonary hypertension (CTEPH) belongs to group 4 of clinical PH classification and is characterized by chronic pulmonary artery obstructions by thromboembolic material 1,4,5 . Following the announcement of SARS‐CoV‐2 infection as a pandemic on March 11, 2020 by the World Health Organization (WHO), 6 different PH centers in Europe and the United States reported higher hospitalization and mortality rates due to COVID‐19 among PAH/CTEPH patients than in the general populations 7–13 . This may be due to the underlying medical conditions—namely chronic lung diseases and/or coronary heart diseases—and/or the reduced visits to PH centers during the pandemic, which delayed PH diagnosis and early detection of disease progression 8,12,14 .…”

Section: Introductionmentioning

confidence: 99%

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Long COVID syndrome after SARS‐CoV‐2 survival in patients with pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension

Wieteska¹,

Kuśmierczyk‐Droszcz

Betkier-Lipińska

et al. 2023

Pulm. circ.

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Pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) patients have a more severe COVID‐19 course than the general population. Many patients report different persistent symptoms after SARS‐CoV‐2 infection. The aim of our study is to analyze the prevalence of long COVID‐19 symptoms and assess if COVID‐19 affects pulmonary hypertension (PH) prognosis. PAH/CTEPH patients who survived COVID‐19 for at least 3 months before visiting the PH centers were included in the study. The patients were assessed for symptoms in acute phase of SARS‐CoV‐2 infection and persisting in follow‐up visit, WHO functional class, 6‐min walk distance, NT‐proBNP concentration. The COMPERA 2.0 model was used to calculate 1‐year risk of death due to PH at baseline and at follow‐up. Sixty‐nine patients—54 (77.3%) with PAH and 15 (21.7%) with CTEPH, 68% women, with a median age of 47.5 years (IQR 37–68)—were enrolled in the study. About 17.1% of patients were hospitalized due to COVID‐19 but none in an ICU. At follow‐up (median: 155 days after onset of SARS‐CoV‐2 symptoms), 62% of patients reported at least 1 COVID‐19‐related symptom and 20% at least 5 symptoms. The most frequently reported symptoms were: fatigue (30%), joint pain (23%), muscle pain (17%), nasal congestion (17%), anosmia (13%), insomnia (13%), and dyspnea (12%). Seventy‐two percent of PH patients had a low or intermediate‐low risk of 1‐year death due to PH at baseline, and 68% after COVID‐19 at follow‐up. Over 60% of PAH/CTEPH patients who survived COVID‐19 suffered from long COVID‐19 syndrome, but the calculated 1‐year risk of death due to PH did not change significantly after surviving mild or moderate COVID‐19.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Long COVID syndrome after SARS‐CoV‐2 survival in patients with pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension

Wieteska¹,

Kuśmierczyk‐Droszcz

Betkier-Lipińska

et al. 2023

Pulm. circ.

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“…Increased susceptibility to PAH may only occur in those who survive severe COVID-19, or in those who develop mild or no symptoms of SARS-CoV-2 infection. The results of an Italian study showed that the incidence of COVID-19 in patients with PAH was comparable to that of the general population but consistent with other chronic diseases and a higher risk of death [ 75 ]. Researchers reported a severe case of SARS-CoV-2 infection without the underlying conditions for PAH who developed PAH two months after being discharged [ 70 ].…”

Section: Role Of Sars-cov-2mentioning

confidence: 95%

Human Endogenous Retrovirus, SARS-CoV-2, and HIV Promote PAH via Inflammation and Growth Stimulation

Wang

Gomes

et al. 2023

IJMS

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Pulmonary arterial hypertension (PAH) is a pulmonary vascular disease characterized by the progressive elevation of pulmonary arterial pressures. It is becoming increasingly apparent that inflammation contributes to the pathogenesis and progression of PAH. Several viruses are known to cause PAH, such as severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2), human endogenous retrovirus K(HERV-K), and human immunodeficiency virus (HIV), in part due to acute and chronic inflammation. In this review, we discuss the connections between HERV-K, HIV, SARS-CoV-2, and PAH, to stimulate research regarding new therapeutic options and provide new targets for the treatment of the disease.

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“…The Italian Pulmonary Hypertension Network noted 100% mortality in their hospitalized cohort early in the pandemic, while the French PH network demonstrated 41.3% in hospital mortality and 24.6% overall mortality. 115 116 Both mRNA and viral vector type COVID-19 vaccines are noted to be safe in patients with PAH. 117…”

Section: Adjunctive and Supportive Therapiesmentioning

confidence: 99%

Management of Pulmonary Arterial Hypertension

Weatherald

Varughese

Liu

et al. 2023

Semin Respir Crit Care Med

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Pulmonary arterial hypertension (PAH) is a rare pulmonary vascular disease characterized by progressive pulmonary arterial remodeling, increased pulmonary vascular resistance, right ventricular dysfunction, and reduced survival. Effective therapies have been developed that target three pathobiologic pathways in PAH: nitric oxide, endothelin-1, and prostacyclin. Approved therapies for PAH include phosphodiesterase type-5 inhibitors, soluble guanylate cyclase stimulators, endothelin receptor antagonists, prostacyclin analogs, and prostacyclin receptor agonists. Management of PAH in the modern era incorporates multidimensional risk assessment to guide the use of these medications. For patients with PAH and without significant comorbidities, current guidelines recommend two oral medications (phosphodiesterase type-5 inhibitor and endothelin receptor antagonist) for low- and intermediate-risk patients, with triple therapy including a parenteral prostacyclin to be considered in those at high or intermediate-high risk. Combination therapy may be poorly tolerated and less effective in patients with PAH and cardiopulmonary comorbidities. Thus, a single-agent approach with individualized decisions to add-on other PAH therapies is recommended in older patients and those with significant comorbid conditions. Management of PAH is best performed in multidisciplinary teams located in experienced centers. Other core pillars of PAH management include supportive and adjunctive treatments including oxygen, diuretics, rehabilitation, and anticoagulation in certain patients. Patients with PAH who progress despite optimal treatment or who are refractory to best medical care should be referred for lung transplantation, if eligible. Despite considerable progress, PAH is often fatal and new therapies that reverse the disease and improve outcomes are desperately needed.

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The paradox of pulmonary arterial hypertension in Italy in the COVID-19 era: is risk of disease progression around the corner?

Cited by 9 publications

References 28 publications

Long COVID syndrome after SARS‐CoV‐2 survival in patients with pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension

Long COVID syndrome after SARS‐CoV‐2 survival in patients with pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension

Human Endogenous Retrovirus, SARS-CoV-2, and HIV Promote PAH via Inflammation and Growth Stimulation

Management of Pulmonary Arterial Hypertension

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