Author + information
- Fatih Yilmaz,
- Ozgur Akbal,
- Aykun Hakgor,
- Hacer Ceren Tokgoz Demirel,
- ibrahim Halil Tanboga,
- Canan Elif Yildiz,
- Sevim Turkday,
- Nertila Poci,
- Tugba Aktemur,
- Durmus Demir,
- Seda Tanyeri,
- Nihal Ozdemir and
- Cihangir Kaymaz
Background: Pulmonary arterial hypertension (PAH) associated with congenital heart disease (APAH-CHD) is a subgroup of PAH including four different forms sharing similar clinical and pathological findings. However, attention have been focused on the Eisenmenger syndrome (ES) and predictors of survival (SV) in other patients (pts) with APAH-CHD remain to be determined.
Methods: We investigated clinical, biochemical, neurohumoral, echocardiographic and hemodynamic predictors of SV in 170 pts with APAH-CHD (Female 95, age 39.1±16.6 years) out of 481 pts included in our single-center study. Subsets of APAH-CHD were as follows;1-ES (n=97), 2-APAH with prevalent systemic-to-pulmonary shunts (n=31), 3-APAH with small defects (n=4),and 4-APAH after defect correction (n=34).
Results: Baseline six-minute walk distance (6MWD) was 320m (240-395m). Pulmonary arterial systolic and mean pressures (PASP, PAMP) were 102±33 and 65.4±23.8 mmHg, transpulmonary and diastolic pressure gradients (TPG,DPG) were 53.5±24 and 34.8±19.6 mmHg, respectively. Pulmonary and systemic vascular resistance (PVR, SVR; in WU) and PVR/SVR ratio were 9 (3.4-14.8), 19.4 (15.7-27.2) and 0.46 (0.18-0.80), respectively. Targeted PAH treatments (TT) were noted in 99 (59.6%) pts. Median follow-up was 20 (3- 144) months. APAH-CHD compared to idiopathic PAH and PAH associated with connective tissue diseases was associated with a better SV (99.2 vs 61.5 and 42.6 months, p=0.010) while SV across APAH-CHD subgroups were comparable (p=0.868). Pericardial effusion (PE) (p=0,008), interatrial septum bulging to left side (IASB) (p=0.003), right atrial pressure (RAP, mmHg) (p=0,002), functional class (FC) (<0.001), 6MWD (p=0.026), C-reactive protein (CRP) (p=0.002) and brain natriuretic peptide (BNP) (p=0.025), but not other baseline measures, TT choices or clinical worsening episodes were associated with SV in pts with APAH-CHD.
Conclusions: APAH-CHD compared to other PAH subgroups was associated with a better SV and baseline FC, 6MWD, PE, IASB, RAP, CRP and BNP seem to discriminate high-risk pts in this setting.
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: 1100-002
- 2017 American College of Cardiology Foundation