Conference: 2012 International PHA Conference and Scientific Sessions
Release Date: 06.22.2012
Presentation Type: Abstracts
Hanaa H. Banjar, MD, FRCPC.
King Faisal Specialist Hospital and Research Center, Riyadh, Kingdom of Saudi Arabia.
BACKGROUND: Pulmonary Arterial hypertension (PAH) has been described frequently in the Pediatric population. Mortality is extremely elevated. Once diagnosis has been confirmed mean survival is less than 1 year among children.
OBJECTIVE: To identify the different causes of PH and treatment modalities in referred cases to the Pediatric Pulmonary clinic in a tertiary care center in Saudi Arabia and their outcomes.
METHODS: Retrospective chart review of all referred patients to pulmonary clinic with documented PH based on cardiac catheterization and or Echocardiogram during one year period (March 2009- February 2010).
RESULTS: A total of 114 patients with confirmed Pulmonary hypertension (PH). Mean age at diagnosis 3.1 up to 3.8 years. 55 (48%) males, 59 (52%) females. The most common causes of Pulmonary hypertension were found to be: Congenital Heart Disease (CHD) in 100 (87.7%) patients, Congenital anomalies in 100 (87.7%), Down Syndrome in 44 (38.5%).
Other congenital anomalies as CHARGE association, Skeletal Dysplasia, and 32 patients with unknown syndrome. 11 patients (10%) due to congenital lung anomalies as Diaphragmatic hernia in association with lung hypoplasia, and congenital lobar emphysema. 11 patients (10%) due to Chronic Lung Disease (CLD), 2 patients due to living in high altitude, 2 patients with obesity, 2 patients with Alagile syndrome, and 2 patients with idiopathic Pulmonary Arterial hypertension (PAH) . Obstructive Sleep Apnea (OSA) was detected on 31 (27%). Asthma 33 (30%), Recurrent Chest Infection 41 (36%), 32 (28%) required O2, Gastroesophageal Reflux (GER) in 36 (32%).
Sixty of 114 patients (53%) were started on vasodilators. Sildenafil (Revatio) was the most common drug used in 35 patients (30%) alone or in combination with Bosentan (Tracleer) or inhaled Ventavis (Iloprost). Bosentan alone or in combination was used in 21 patients (18%) and Inhaled Ventavis alone or in combination in 8 patients (4%).
Seventy five patients (66%) continued to have Pulmonary hypertension (PH) at Follow up, and the factors that contributed to persistence of Pulmonary Arterial hypertension (PAH) at Follow up were: presence Congenital Heart Disease (CHD) P value = 0.05, un-closed ASD P value = 0.03.
CONCLUSIONS: Pulmonary hypertension is a common disease and should be diagnosed and treated early before it becomes irreversible and resistant to vasodilators. Early closure of Atrial Septal Defect (ASD) and Congenital Heart Disease (CHD) defect repair improves Pulmonary Arterial hypertension (PAH).