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

Abstract

ObjectiveThe coronavirus disease-2019 (COVID-19) oubreak has led to significant restrictions on routine medical care. We conducted a multicenter nationwide survey of PAH patients aiming at determining the consequences of the Governance measures on PAH management and risk of poor outcome in patients with COVID-19.Meterials and MethodsDemographic data, number of in-person visits, 6-min walk and echocardiographic tests, BNP/NT-proBNP tests, WHO functional class assessment, presence of elective and non-elective hospitalisation, need for treatment escalation/initiation, newly diagnosed PAH, incidence of COVID-19 and mortality rates were considered in the present study including 25 Italian centers. Data were collected, double checked and tracked by institutional records, between the 1st March and 1st May 2020 to coincide with the first peak of COVID-19 and compared with the same time-period in 2019.ResultsAmong 1922 PAH patients the incidence of SARS- CoV-2 infection and COVID-19 was 1.0% and 0.46%, respectively, the latter comparable to the overall Italian population (0.34%), but associated with 100% mortality. Less systematic activities were converted into more effective remote interfacing between clinicians and PAH patients allowing lower rates of hospitalisation and related death compared with 2019 (1.2% and 0.3% versus 1.9% and 0.5%, respectively; p<0.001). High level of attention is needed to avoid the potential risk of disease progression related to less aggressive escalation of treatment and the reduction in new PAH diagnosis compared with 2019.ConclusionCohesive partnership of health care providers with regional public health officials is needed to prioritise PAH patients for remote monitoring by dedicated tools.

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

“…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 .…”

“…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 .…”

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

“…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 ].…”

Management of Pulmonary Arterial Hypertension

“…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…”