Author + information
- Mehmet Serdar Küçükoglu,
- Cihangir Kaymaz,
- Gul Sagin Saylam,
- Dursun Alehan,
- Serdar Kula,
- Atif Akcevin,
- Alpay Celiker,
- Sertac Cicek and
- Sadberk Lale Tokgozoglu
Background: Baseline data from the national, multicenter, prospective, observational THALES Registry providing information regarding demographic and clinical characteristics and treatment patterns had previously reported. In this analysis, we aim to assess survival in association with clinical, laboratory and hemodynamic characteristics and targeted treatment patterns.
Methods: Kaplan Meier estimates and Breslow Chi-Square tests were used for survival analysis and intergroup differences, respectively. We compared observed survival with baseline survival estimates according to National Institutes of Health (NlH) and Pulmonary Hypertension Connection (PHC) equations.
Results: Follow-up (FU) data were available for 506 (59% female, median age 13.6 years (y) out of 1034 patients enrolled. Median FU from diagnosis was 39 months. The patients consisted of Eisenmenger Syndrome (46.9%), left to right shunt (44%), small defects (1.8%) and residual PAH after defect correction (7.3%). Patients with FU data had a higher risk profile as indicated by significantly worse mean pulmonary artery pressure, pulmonary-systemic flow ratio (Qp / Qs), pulmonary vascular resistance (PVR), age and functional class as compared to patients without FU data. Overall survival at 140 months was estimated as 79%. Patients who had six-minute walking distance (6MWD) over 440 m (p=0.009), brain natriuretic peptide (BNP) levels <50 pg/mL (p<0.001) and who were treated with PAH-specific treatment (p=0.004) significantly lived longer. Targeted treatments, either as mono or combination, have provided improved survival (p=0.012, p=NS, respectively). Baseline survival estimates predicted by PHC equation (1y 94,7%, 2y 89,8%, 3y 85,1%) provided comparable results with observed survival (1y 96,3%, 2y 93,9%, 3y 92,6%) whereas NlH equation (1y 77,3%, 2y 67,1%, 3y 58,6%) significantly underestimated.
Conclusions: Our results have confirmed the findings provided in the current literature in terms of survival predictors (6MWD, BNP), treatment effect and also suggest the consistency of PHC equation in estimating survival in APAH-CHD population.
Poster Hall, Hall C
Friday, March 17, 2017, 10:00 a.m.-10:45 a.m.
Session Title: Imaging Pulmonary Vascular Disease Plus the Impact of Congenital Heart Disease
Abstract Category: 35. Pulmonary Hypertension and Pulmonary Thrombo-embolic Disease
Presentation Number: 1100-001
- 2017 American College of Cardiology Foundation