Pulmonary hypertension and obstructive sleep apnea syndrome -Marmara Universitypediatric sleep laboratory experience

Background: Obstructive sleep apnea syndrome (OSAS) is an uncommon cause of pulmonary hypertension (PHT). Our aim is to evaluate the frequency of OSAS in children with PHT. Methods: The study included 11 patients who were referred to identify the underlying etiology in the last one year. Demographic and clinical features of the patients, sleep-related symptoms, PSG results and the etiology of PHT were evaluated. Results: Mean age of the patients was 7,2±6,1 years (54.5 % female). Median age at diagnosis was 4.1 years and onset of semptoms was 44 months. Snoring, open mouth sleeping and witnessed apnea was present in 45.4%, 45.4% and 36.3 % of the patients, respectively. Hypercarbia was present in 3 patients. Primary PHT was detected in 8 patients (72.7%), while secondary PHT was detected in 3 patients (27.3%). One of the patients with secondary PHT had arteriovenous malformation in the lung, one had cardiac anomaly and one had diaphragm hernia and right lung hypoplasia. Adenotonsillectomy was performed in the follow-up of two patients. Conclusions: OSAS should be kept in mind in the situation that the etiology of PHT can not be explained. Patients with PHT should be questioned about sleep-related symptoms. Sleep studies are methods without alternative to determine etiology.