山村 健一郎¹

Kenichiro YAMAMURA¹

¹九州大学病院小児科, ²ハートセンター成人先天性心疾患外来

¹Department of Pediatrics, Adult Congenital Heart Disease Clinic, Kyushu University Hospital, ²

キーワード :

Cyanotic congenital heart diseases have a wide variation, including Eisenmenger syndrome, single ventricle without Fontan procedure, and complex congenital heart diseases without intracardiac repair. Major points to be assessed by echocardiography are wall motion of the ventricle, valve regurgitation, pulmonary artery hypertension, thrombosis, and vegetation, etc. It is also important to understand the anatomical features, intrinsic complications of the disease, and therapeutic targets.
Re-defining cardiac anatomy is very important especially at the first visit of the patients to the institute. Communication between systemic and pulmonary circulation at atrial, ventricular, and arterial levels should be checked. It is important to assess the shunt direction and pressure gradient with M-mode color Doppler and pulse Doppler. Evaluation of the ventricular size and function（ejection fraction, fractional area change, and tricuspid/mitral annular motion）is mandatory. Presence and degree of the pulmonary outflow tract obstruction and atrioventricular valve regurgitation are also necessary. Thrombosis and vegetation are important points in cyanotic congenital heart diseases. Bubble contrast echo is useful to detect minor right to left shunt.
Cyanotic congenital heart disease has impacts on multiple organ systems, including hematology, coagulation system, nervous system, kidneys, gastrointestinal tract, uric acid metabolism, pulmonic and systemic circulation（endothelium）, renal function, etc. Other ultrasonography is also important in the management of cyanotic congenital heart disease. A systematic clinical approach is needed to treat this multisystem disorder.