Syncope is an independent predictor of poor prognosis and stratifies adult group 1 PAH patients into a high risk category. However, in children with IPAH, while serious, it may be a marker for a highly reactive pulmonary vascular bed and lead to earlier diagnosis and treatment of PAH.
A single center analysis of presenting symptoms in children with group 1 PAH to determine their correlation with hemodynamics and outcomes. To compare hemodynamics and outcomes in children with and without syncope as a symptom of PAH.
A retrospective chart review of children with group 1 PAH seen at a large Pediatric Pulmonary Hypertension Center from January 2005 through October 2018 was performed. Kaplan Meier analysis was done to assess survival in children with PAH who had syncope versus those who did not.
Of 442 pediatric patients with pulmonary hypertension seen at our center during the study period, 185 had group 1 PAH. 133 (71.9%) complained of exertional dyspnea, 30 (16.2% all IPAH) syncope, 15(8.1%) failure to thrive and 7(3.8%) chest pain. A KM curve representing a 10 year follow up of patients with IPAH and syncope, IPAH without syncope and all PAH without syncope is shown below. Survival in patients who presented with syncope was not significantly different from those who presented with other symptoms. However, 8/30 (27%) of children with syncope were robust acute vasoresponders versus 7/156 (4.4%) of children without syncope as a symptom.
Among the patients with PAH seen at our institution, those who had syncope had similar outcomes as those without syncope. Further, children with syncope were more likely to be robustly responsive to acute vasoreactivity testing which may indicate a more malleable vascular bed.