Pulmonary arterial hypertension (PAH) associated with congenital heart disease (APAH-CHD) is classified as a subgroup of PAH that includes four different forms sharing a similar clinical and pathological findings. However, attention has been focused on the Eisenmenger syndrome (ES) and predictors of the survival in other patients (pts) with APAH-CHD remain to be determined.
In this study, we investigated clinical, biochemical, neurohumoral, echocardiographic and hemodynamic predictors of the survival in 134 pts with APAH-CHD (F72 M 62, age 40.7±17.5 years) out of the 341 pts included into the our single-centre, prospective study. Subgroups of APAH-CHD were as follows: 1.ES (n=71) 2.PAH associated with prevalent systemic-to-pulmonary shunts (n=37), and 4. PAH after defect correction (n=26). Baseline six-minute walk distance (6MWD) was 301.5±127.8 m, and pulmonary arterial systolic and mean pressures (PASP, PAMP, mm Hg) were 99.6±32.9 and 64.7±22.85, and transpulmonary and diastolic pressure gradients (TPG,DPG,mm Hg) were 51.8±22.9 and 29.6±20.3, respectively. Pulmonary and systemic vascular resistance (PVR,SVR,WU) and PVR/SVR ratio were 10.6±9.3, 21.8±9.6 and 0.52±0.37, respectively. Targeted PAH treatments were noted in 100 (75%) pts. Mean follow-up was 30.9 ± 28 (0,5- 122,6) months. The APAH-CHD compared with idiopathic PAH(IPAH) and PAH-associated with connective tissue disease (APAH-CTD) was associated with a better 8-year survival (83 vs 54,4 and 61%, p<0,05), and APAH-CHD subgroups showed comparable survival (p>0,05). The age, sex, functional class, haemoglobin, oxyhaemoglobin saturation %, C-reactive protein, uric acid and brain natriuretic peptide were not related with survival(p>0,05). Neither tertile definition (<190,190-330,>331), nor pre-defined cut-off values of 6MWD (m) (<165, 165-440,> 440) related with APAH-CHD survival(p>0,05). Similarly, tertiles of TPG,DPG, PVR and PVR/SVR ratio showed comparable survival (p>0,05). However, pericardial effusion(p=0,0001) and the highest tertile of right atrial pressure (RAP,mm Hg ) (> 11 vs 9-11 and < 9, p<0.01) were found to discriminate the high-risk pts.
In conclusion, APAH-CHD compared with other PAH subgroups is associated with a better survival. However, only pericardial effusion and RAP predict the worst clinical outcome in these patients.