There is currently limited data available regarding COVID-19 infection in patients with pulmonary hypertension (PH), resulting in poor evidence-based guidance to manage this specific patient population and to reliably predict the clinical course. According to the US Centers for Disease Control and Prevention (CDC), patients with underlying health conditions, including all types of lung and cardiovascular diseases, have an increased risk of developing serious disease when infected by SARS-CoV-2 [1]. Based on prior publications on the effects of acute right heart failure superimposed on systemic infection [2-5], RYAN et al. [6] suggested that right heart failure and concomitant COVID-19 infection may result in increased mortality in pulmonary arterial hypertension (PAH) patients. Surprisingly, the number of hospitalised PAH-COVID-19 patients remained rather low in Italy and the USA so far [7]. In late March 2020, experts from over 32 US PH expert centres answered a query endorsed by the US Pulmonary Hypertension Association. COVID-19 infection was reported in 13 PAH patients, among whom three required intubation and one died. This is consequently raising the question whether and why PAH patients appear to be at lower risk of developing severe COVID-19 [7]. Considering that more evidence was needed, an international survey was launched from 17 April 2020 to 10 May 2020, in the middle of the pandemic surge in Europe, to evaluate the impact of COVID-19 infection in patients with rare forms of PH, i.e. PAH and chronic thromboembolic pulmonary hypertension (CTEPH). The aim of the survey was to collect data on the clinical course, treatment, hospitalisations, intensive care unit (ICU) admissions and outcomes of COVID positive patients with PAH and CTEPH. The SurveyMonkey platform was used for data collection. Numbers of available data are listed in brackets. 47 PH centres, from 28 countries worldwide, responded to the survey (figure 1a). During the pandemic period, most consultations for patients with PAH and CTEPH occurred remotely (80%), either by tele-(68%) or video-consultation (12%), whereas 9% were followed live, with normal outpatient consultation, 8% exclusively when the patients called and 3% were not followed at all. In total, COVID-19 infections were reported in 70 PAH or CTEPH patients by 19 of the participating centres. 13 of these centres reported more than one case and 28 did not report any case. Most patients had idiopathic or heritable PAH (31%), followed by PAH due to connective tissue diseases (23%), CTEPH (20%), PAH associated with congenital heart diseases (10%), porto-pulmonary hypertension (4%), and other causes (11%, including pulmonary veno-occlusive disease, PAH associated with HIV infection or with intake of drugs and toxic oil) (data available for 70 patients). Median age of the cohort was 50-59 years (n=70; figure 1b). Most of the patients were under specific PAH combination @ERSpublications This international survey highlights that a limited number of PAH and CTEPH patients suffered from sev...

Arrhythmia is common in PAH-CHD and is associated with an adverse long-term outcome, even when managed in a specialist centre.

Atrial septal defects (ASD) are a common congenital heart defect. The majority of patient with ASDs often follow an uncomplicated course of events. However, a proportion of patients with ASDs, may have their condition complicated by pulmonary hypertension (PH), with a subsequent significant impact on management, morbidity and mortality. The presence of PH, influences the suitability for defect closure. In this review we describe the different types of ASDs, the classification of PH related to congenital heart disease (CHD), when ASD closure is contraindicated and the management of patients who develop pulmonary arterial hypertension (PAH), including the most extreme form, Eisenmenger syndrome (ES).