Characteristics of our pulmonary hypertension cases, survival and factors affecting survival

Background: Pulmonary hypertension (PH) is characterized by an increase in pulmonary artery pressure (PAP), and its awareness is a rare disease group that has been increasing recently. There are many recording works carried out about PH in different countries around the world. It is a disease with a high risk of mortality and many different risk scores have been created to assess the risk of mortality. Our study aims to evaluate the descriptive characteristics and survival analyses of the patients who were diagnosed with PH by right heart catheterization (RHC) following the guides at the Pulmonary Hypertension Clinic of Istanbul University from 2010 to the present day. Materials and Methods: Two hundred sixty patients were included in the study between January 2010 and January 2022 at the Department of Chest Diseases Pulmonary Hypertension Polyclinic, Istanbul Medical Faculty, Istanbul University. The files of these cases were retrospectively examined. All patients diagnose were confirmed by RHC. PH groups and subgroups of pulmonary arterial hypertension (PAH) were included in the study. The age, sex, group of PH, comorbidities, duration of symptoms to diagnosis, follow-up time, The World Health Organization functional class (WHO-FC), findings of transthoracic echocardiography as a pulmonary arterial systolic pressure (sPAP) and pericardial effusion, the value of NT-proBNP (pg/mL), RHC findings, a 6-minute walking test (6 MWT), the results of respiratory functional tests as FEV1 (%), FEV1 (mL), FVC (%), FVC (mL), DLCO (%), PH- specific treatment with side effects, anti-coagulant treatment and pulmonary endarterectomy for chronic thromboembolic pulmonary hypertension (CTEPH) were noted. The RHC time was received as diagnosis time. The survival of the patients and the factors affecting their survival were analyzed (Ethics Committee File No: 2021/2092). Results: The files of 260 patients were retrospectively examined in our study. The majority of the study population were women (75.8%). The average age was 54.6 ± 16.6 and the average time up to the diagnosis was 13 months. At diagnosis time, most of the patients were FS II and (45.4%) and FS III (48.5%). The majority of patients were in the PAH group (55%) and CTEPH group (30%). PAH due to connective tissue disease (23.1%) and idiopathic pulmonary arterial hypertension (20.8%) were the majority in PAH subgroups. One hundred forty-five (55.8%) patients had the additional disease (comorbidity). İt was observed that 35% of the cases received monotherapy, 26.2% received dual therapy, and 11.5% received triple therapy. Pulmonary endarterectomy was applied to 18 patients with CTEPH. The average survival duration was 89.5 ± 6.3 months, and as a result of the survival analysis, 1-year survival rates of 86%, 3-year survival rates of 73%, and 5-year survival rates of 65%. Survival time was 124±9.5 months for the WHO-FC II, 68.1±8.1 months for the WHO-FC III, and 31.8±6.3 months for the WHO-FC IV group. Also, survival time was 85.5±8.1 months for the PAH group and 92.7±10.6 months for the CTEPH group. The one-year survival rate of 86%, the 3-year survival rate of 70%, and 5-year survival rate of 62% for the PAH group. The one- year survival rate of 89%, the 3-year survival rate of 83%, and 5-year survival rate of 69% for the CTEPH group. The average survival duration was 87.8±12.9 months for IPAH, 71.6±11.9 months for CTD-PAH, and 101.5±8.3 months for CHD-APAH. Important parameters affecting survival in our study; age (p=0.004), WHO-FC (PS II; p<0.005, FS III; p<0.005, FS IV; p=0.029), 6 DYM (p=0.001), DLCO (p<0.005), cardiac output (p=0.002) and cardiac index (p=0.025) were found. Conclusion: Five-year survival of our PH patients showed significantly improvement due to increased awareness of the disease, early diagnosis and development of the specific drugs. In our study, important parameters affecting survival were found as WHO-FC, 6MWT, DLCO, cardiac output, and cardiac index. Keywords: Pulmonary arterial hypertension, pulmonary hypertension, survival, risk factors.