ABSTRACT
The Saudi Association for Pulmonary Hypertension [previously called Saudi Advisory Group for Pulmonary Hypertension] has published the first Saudi Guidelines on Diagnosis and Treatment of Pulmonary Arterial Hypertension back in 2008. [1] That guideline was very detailed and extensive and reviewed most aspects of pulmonary hypertension [PH]. One of the disadvantages of such detailed guidelines is the difficulty that some of the readers who just want to get a quick guidance or looking for a specific piece of information might face. All efforts were made to develop this guideline in an easy-to-read form, making it very handy and helpful to clinicians dealing with PH patients to select the best management strategies for the typical patient suffering from a specific condition. This Guideline was designed to provide recommendations for problems frequently encountered by practicing clinicians involved in management of PH. This publication targets mainly adult and pediatric PH-treating physicians, but can also be used by other physicians interested in PH
ABSTRACT
Congenital heart disease [CHD] with intracardiac/extracardiac shunts is an important etiology of pulmonary arterial hypertension [PAH]. The majority of children with congenital cardiac shunts do not develop advanced pulmonary vasculopathy, as surgical repair of the anomalies is now performed early in life. However, if not repaired early, some defects will inevitably lead to pulmonary vascular disease [truncus arteriosus, transposition of the great arteries associated with a ventricular septal defect [VSD], atrioventricular septal defects remarkably in Down syndrome, large, nonrestrictive VSDs, patent ductus arteriosus and related anomalies]. The majority of patients are now assigned to surgery based on noninvasive evaluation only. PAH becomes a concern [requiring advanced diagnostic procedures] in about 2-10% of them. In adults with CHD, the prevalence of advanced pulmonary vasculopathy [Eisenmenger syndrome] is around 4-12%. [1] This article will discuss the diagnostic and management approach for PAH associated with CHD [PAH-CHD]