Abstract
The diagnosis of pulmonary arterial hypertension (PAH) associated with pretricuspid shunts (PTS) is often made in adulthood. The characteristics of this subgroup of CHD-PAH are not well established.
Objective: To review and analyze patients with PAH and PTS enrolled in the second prospective French PAH registry, initiated in 2006.
Results: CHD-PAH (n=255) accounted for 9.8% of PAH. 100 patients had PTS: atrial septal defects (ASD) ostium secundum type (n=93), ASD sinus venosus type (n=3), ASD ostium primum type (n=1), and partial abnormal pulmonary venous return without ASD (n=3). 72% of patients were female. At diagnosis, mean age was 38 years (range, 0-81 years). 17 patients were diagnosed before 18 years and 28 after 60 years. In 15 patients CHD was repaired before PAH diagnosis. In patients with open shunts, PAH diagnosis was made at time of diagnosis of CHD in 56% of cases, whereas the diagnosis of CHD followed the diagnosis of PAH in 16% of cases. PAH appeared during the follow-up of the CHD in 28% of cases. At inclusion, 5% of patients were in functional class (FC) IV, 61% were in FC III and 34% in FC I or II. 6MWD was 360±107m. Pulmonary hemodynamics were: mPAP=53±16mmHg, CO=5.02±1.7L/min and PVR=11±8.3WU. 50% of patients in FC II or III received PAH specific therapy. During the 3-year follow-up period, 9 patients died and 3 underwent heart-lung transplantation.
Conclusion: In this registry, the proportion of CHD-PAH due to PTS compared to other forms of CHD is higher than in previously reported series. The natural history of PTS remains poorly understood as PAH can be diagnosed throughout life. PAH specific therapies are not widely used in the PAH associated with PTS population.
- © 2011 ERS
