Association of delayed diagnosis of pulmonary arterial hypertension with its prognosis.

BACKGROUND: Currently, pulmonary hypertension-targeted therapy has been shown to improve the survival of patients with pulmonary artery hypertension (PAH). However, the importance of early diagnosis has not been investigated. Therefore, this study aimed to investigate whether a delayed diagnosis of PAH is associated with its prognosis. METHODS AND RESULTS: A total of 66 consecutive untreated patients were diagnosed with PAH from January 2008 to December 2021 at the Kagoshima University Hospital. The time from symptom onset to diagnosis correlated with brain natriuretic peptide levels (p < 0.001), right ventricle (RV) Tei index (p < 0.001), and the tricuspid annular plane systolic excursion/systolic pulmonary artery pressure ratio (p = 0.003). These findings suggest that in patients with PAH, RV function declines with increasing time from symptom onset to diagnosis. Furthermore, older patients with PAH appeared to have a longer time from symptom onset to diagnosis. Next, patients were divided into delayed diagnosis (>3 months) and early diagnosis (≤3 months) groups based on the time from symptom onset to diagnosis. Patients were categorized into three groups according to the European Society of Cardiology (or the European Respiratory Society) risk stratification guidelines. Patients diagnosed with PAH within 3 months of symptom onset were significantly in the low- or intermediate-risk groups (p < 0.001). A Kaplan-Meier analysis revealed that the cumulative event-free rate was significantly lower (p < 0.01) in the delayed diagnosis group than in the early diagnosis group. A delayed diagnosis was significantly associated with a worse outcome than an early diagnosis, after adjusting for different sets of confounding factors. CONCLUSIONS: A delayed PAH diagnosis is associated with a poor prognosis. Early diagnosis of PAH may lead to a low-risk treatment. Furthermore, older patients need more careful screening for PAH.

Pulmonary artery hypertension-associated with human immunodeficiency virus infection with attenuated effect of subcutaneous treprostinil injection during long-term observation: A case report.

Pulmonary artery hypertension associated with human immunodeficiency virus infection (PAH-HIV) is known to be caused by HIV infection. Antiretroviral therapy and PAH-specific drugs improve the prognosis of patients with PAH-HIV, but the pathophysiology of PAH-HIV remains unclear. We report a case of PAH-HIV treated with upfront combination therapy including subcutaneous injection of treprostinil. One year after treatment initiation, the patient's PAH improved significantly. However, it worsened over time due to reduced efficacy of subcutaneous injection of treprostinil. Learning objective: The etiology and pathophysiology of pulmonary artery hypertension associated with human immunodeficiency virus infection (PAH-HIV) remain unclear, and there are few case reports of PAH-HIV in Japan because the HIV prevalence is low. We encountered a case of PAH-HIV in which the efficacy of subcutaneous treprostinil injection was attenuated. It was unclear whether the reduced efficacy was associated with the pathophysiology of PAH-HIV. When PAH control deteriorates, early alteration of medication choice and administration route is important.