RT Journal Article
SR Electronic
T1 Prognostic impact of follow-up pulmonary vascular resistance in pulmonary arterial hypertension
JF Open Heart
JO Open Heart
FD British Cardiovascular Society
SP e002054
DO 10.1136/openhrt-2022-002054
VO 9
IS 1
A1 Sho Suzuki
A1 Ryotaro Asano
A1 Tatsuo Aoki
A1 Sayuri Nakayama
A1 Jin Ueda
A1 Akihiro Tsuji
A1 Teruo Noguchi
A1 Takeshi Ogo
YR 2022
UL http://openheart.bmj.com/content/9/1/e002054.abstract
AB Objective Pulmonary arterial hypertension (PAH), caused by pulmonary artery remodelling and increased pulmonary vascular resistance (PVR) due to an unknown mechanism, is an intractable disease with a poor prognosis. The recent development of PAH-specific treatment medications may allow for higher PVR reduction than previously achieved. This study aimed to identify the prognostic significance of follow-up PVR levels achieved shortly after the initiation of targeted treatment in patients with idiopathic/heritable pulmonary arterial hypertension (I/H-PAH).Methods We analysed the data of all patients with I/H-PAH admitted to our hospital between 1998 and 2019. We collected data at baseline and during the first invasive haemodynamic evaluation. The primary outcome was death or lung transplantation.Results Of the 133 treatment-naïve patients enrolled in this study, 47 experienced adverse events during a median follow-up period of 6.4 (IQR 3.5–11.5) years. The median time interval to first follow-up from diagnosis was 162 (IQR 117–253) days. Incidence of the primary outcome was significantly lower in patients who achieved low PVR at follow-up. Of risk factors evaluated at follow-up, the multivariate Cox regression analysis revealed PVR as an independent predictor of the primary outcome (HR 1.103, 95% CI 1.029 to 1.183; p=0.006). The results were consistent across risk profiles according to the simplified risk stratification recommended by the European Society of Cardiology and European Respiratory Society guidelines.Conclusion Follow-up PVR was an independent predictor of transplant-free survival in patients with I/H-PAH. Evaluation of haemodynamic status shortly after initiating treatment may help predict long-term prognosis.Data are available upon reasonable request.