[The current pharmacotherapy of pulmonary arterial hypertension]. / Wspólczesna farmakoterapia tetniczego nadcisnienia plucnego.

Pulmonary arterial hypertension (PAH) is a chronic, serious disease caused by remodeling of small pulmonary vessels, which leads to increase of pulmonary resistance, right heart failure and death. The 1990ths of XX century are the beginning of dynamic research into the pathophysiology and treatment of this disease. Actually, the goal oriented therapy based on three main metabolic pathways includes: prostacyclin's analogues, endothelin receptor antagonists and phosphodiesterase-5 inhibitors. Applying this therapy, according to ESC guidelines has prolonged significantly the survival in the group of patients with PAH. Due to the high cost of the therapy, there has been created the national therapeutic program in Poland. It assures the real possibility of therapy for patients with PAH from the third FC WHO.

[New directions in the therapy of pulmonary arterial hypertension]. / Nowe kierunki w leczeniu tetniczego nadcisnienia plucnego.

Recent years are the time of dynamic development of pulmonary arterial pressure pharmacotherapy. By introducing the goal oriented therapy the survival in this group of patients has significantly increased. Apart from the pharmacotherapy used according to the ESC guidelines, new attempts of interventional treatment based on denervation of pulmonary artery have also been taken. Constantly, the new clinical trials (tests?) of drugs acting via new metabolic pathways have been conducted. They include for example: soluble guanylate cyclase stimulators, tyrosine kinase inhibitors, serotonin receptors inhibitors, Rhokinase inhibitors, VIP analogues. One of the newmedicines is riociguat, the effectiveness and safety of which have been confirmed in the PATENT and CHEST study. However, the small number and clinical diversity in the group of the PAH patients cause significant difficulties with the extrapolation of the results of trials according to the guidelines of the therapy.

[Pulmonary arterial hypertension: modern diagnostics and therapy--Part 1]. / Tetnicze nadcisnienie plucne: wspólczesne mozliwosci diagnostyki i terapii--Czesc I.

Pulmonary arterial hypertension (PAH) belongs to the group of rare diseases and the morbidity rate is 15 to 50 people per million per year. Before the era of specific treatment of PAH, the prognosis was poor. The average life expectancy of the patients was 2.8 years. However, in the last years there has been a breakthrough in treating the patients with PAH. The introduction of this specific treatment has prolonged the life and improved the quality of it within the group of the patients with PAH. In Poland, since 2008 the therapy has been organized by the Pulmonary Hypertension Therapeutic Program. PAH is a recognition done by excluding more probable causes of pulmonary hypertension (PH) such as: PH due to left heart disease and lung disease, chronic thromboembolic pulmonary hypertension (CTEPH) and PH with multifactorial mechanisms. The clinical symptoms of pulmonary hypertension are non-specific, they develop for a several months and they are mainly caused by progressive right ventricular failure. The base of PAH recognition is echocardiography, which indirectly estimates the pulmonary artery systolic pressure. However, the golden standard of PAH diagnostics is right heart catheterization (RHC) with measurements of pulmonary arterial pressure (PAP), right atrial pressure (RAP), right ventricular pressure and pulmonary wedge pressure (PWP). The early PAH recognition and the correct classification of patients to the treatment organized by the Pulmonary Hypertension Therapeutic Program give them a chance for longer and more comfortable life.

[Reversible pulmonary hypertension as a consequence of dasatinib treatment in a patient with chronic myeloid leukemia and scleroderma]. / Odwracalne nadcisnienie plucne jako powiklanie leczenia dazatinibem pacjentki z przewleklq bialaczka szpikowa i twardzina ukladowa.

Pulmonary hypertension may be associated with many clinical conditions, including connective tissue diseases. We present a case of a patient with sclerodermia and chronic myeloid leukemia, who developed severe symptoms of pulmonary hypertension. Atypical clinical course of pulmonary hypertension, including complete remission of clinical symptoms and hemodynamic improvement provoked critical approach to the etiology of pulmonary hypertension. Taking into account the temporal coincidence with the use of dasatinib (a tyrosine kinase inhibitor) and a few case reports in the literature, it appears that reversible pulmonary hypertension in our patient was associated with the use of dasatinib. Sclerodermia in a previous medical history, an acknowledged risk factor for pulmonary hypertension, initially delayed the correct diagnosis. Reclassification changes the clinical prognosis of pulmonary hypertension in this specific case and allowed to terminate of specific treatment of pulmonary hypertension with good results.

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

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.

Outcomes of COVID-19 in patients vaccinated and unvaccinated against SARS-CoV-2 and suffering from pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension.

INTRODUCTION: Pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) lead to progressive right heart failure. The mortality rates in PAH and CTEPH patients due to COVID­19 are high, and vaccination against COVID­19 is recommended in this group. OBJECTIVES: We analyzed the incidence and outcomes of COVID­19in the PAH/CTEPH patients for 2 years of the pandemic, as well as the predictors of worse outcomes of COVID­19 in this group. PATIENTS AND METHODS: PAH/CTEPH patient data for this observational, cohort study were obtained from 3 pulmonary hypertension centers between March 11, 2020 and March 11, 2022. RESULTS: A total of 364 consecutive patients with PAH/CTEPH (248/122; 232 women [64%]; median [interquartile range] age, 61 years [18-92]) were included in the study. All the patients had advanced pulmonary hypertension at baseline. Eighty­five patients (23%) suffered from COVID­19. Seven of them (8%), all of whom were unvaccinated, died of COVID­19. The unvaccinated patients suffered from COVID­19 more often than the vaccinated ones (46% vs 9%; P <0.001). As many as 31% of the PAH/CTEPH patients with COVID­19 needed hospitalization, in 8% of cases in the intensive care unit. Age equal to or above 65 years and severe pulmonary hypertension defined as a World Health Organization functional class 3 or 4 were associated with severe COVID­19 in the PAH/CTEPH patients. CONCLUSIONS: The vaccinated PAH/CTEPH patients suffered from COVID­19 less frequently than the unvaccinated ones. The mortality rate and hospitalization due to COVID­19 were higher in the PAH/CTEPH patients than in the general population. All efforts should be made to convince the PAH/CTEPH patients to vaccinate against COVID­19.

Pulmonary artery aneurysm in an adult patient with idiopathic dilatation of the pulmonary artery.

Idiopathic dilatation of the pulmonary artery (IDPA) is a rare congenital heart disease. It has been described for almost one hundred years, and numerous definitions have been proposed. The IDPA diagnostic criteria have not been updated for years. Secondary to primary disease, pulmonary artery aneurism was recognised as a lethal defect; however, long-term follow-up of patients with IDPA has not been well researched. Thus, indications to medical or surgical treatment are not evidence based. Here, we present a rare case of a 54-year-old patient with IDPA, who remained under observation for 36 years without surgical intervention.