[II-JCKAP3-3] Characterization and Therapeutic precision of Pulmonary Hypertension related to Congenital Heart Disease
キーワード：pulmonary hypertension, congenital heart disease, selective pulmonary vasodilator
Pulmonary hypertension (PH) is defined by a mean pulmonary arterial pressure (mPAP)>20 mmHg at rest according to updated from the 6th World Symposium of PH (WSPH) in 2018. Four WSPH pathogenetic groups except group 3 include forms of PH with CHD:Group 1 PAH related with CHD, precapillary PH with 4 described clinical and hemodynamic profiles: Eisenmenger syndrome(ES), PAH with CHD with left to right shunt, PAH with small defect, PAH after CHD correction. Many genes are also being reported in PAH with CHD, including BMPR2, SOX17, TBX4 and others. Group 2 PH with left sided HD include pulmonary vein stenosis, ventricular dysfunction (systolic or diastolic), valve disease, and left heart obstructive disease. Group 4 PH with CHD is due to PA obstructions with congenital PA stenosis. Group 5 PH with multifactorial mechanisms; single ventricle physiology, segmental PH, and scimitar syndrome. The goals of therapy are to improve hemodynamics and quality of life; treat to close, treat after repair of the defects; improve quality of life in ES; or further lower PAP and PVR in PH with CHD and Fontan circulations to improve forward PA flow. In group 2, 4, 5 PH with CHD, targeted PH therapy is only partially effective after the Glenn or Fontan procedure and usually ineffective. Ultimately, understanding the cause and pathophysiology of PH-CHD within the various subgroups will allow targeted drug development and effective treatment strategies.