講演情報
[III-PPD3-5]The Challenges in Managing of Pulmonary Arterial Hypertension Associated with Congenital Heart Disease in China
○Hong Gu, Qiangqiang Li (The Department of Pediatric Cardiology, Beijing Anzhen Hospital, Capital Medical University, Beijing)
キーワード：
Congenital Heart Disease、Pulmonary Arterial Hypertension、targeted medical therapy
Pulmonary arterial hypertension (PAH) is a major complication in patients with congenital heart disease (CHD) and is associated with increased mortality and morbidity. PAH associated with CHD is classified into four clinical groups: Eisenmenger syndrome (ES), PAH associated with prevalent left-to-right shunts, PAH with small/coincidental defects, and postoperative PAH. Advances in neonatal screening, diagnostic techniques and cardiac surgery have led to improvement in early identification and treatment of cardiac defects at early age, preventing the development of PAH in most patients. In China, because of the large population base and regional differences in medical resources, there are still considerable number of patients with advance pulmonary vascular disease related to CHD and require efficient management. CHD’s have been the top birth defect since 2004 in China. Recently, data from a prospective multi-center registry study revealed that PAH-CHD accounts for 45.2% of all patients being the most common etiology in Chinese PAH patients. Our center reported 507 patients with PAH-CHD, of whom 235 cases (46.4%) were diagnosed with ES, 193 cases (38.1%) were postoperative PAH, which is related to the late diagnosis and surgery time. The management strategies are different among the 4 clinical groups of PAH-CHD, there is no doubt that the defect closure shouldn’t be considered in patients with Eisenmenger syndrome. For patients with CHD-PAH (including ES、PAH with small/coincidental defects, and postoperative PAH), targeted medical therapy such as Bosentan may be beneficial in improving the clinical symptoms and long-term survival. Special clinical issues such as the management of ACHD and pregnancy complicated CHD and PAH are also particularly noteworthy and challenging.