A False Diagnosis of Hypoplastic Left Heart Syndrome in a Fetus

태아기에 좌심형성부전증후군으로 잘못 진단되었던 1례

Typical hypoplastic left heart syndrome(HLHS) is a distinct pathologic entity with aortic atresia, mitral atresia, very hypoplastic or absent left ventricle and thread like ascending aorta. Occasionally, the lesser degree of hypoplasia is found and is called hypoplastic left heart complex(HLHC) by some authors. This HLHC is often associated with critical aortic stenosis. Fetal echocardiography has enabled us to observe human fetal heart in-utero and to diagnose congenital heart disease prenatally over the last 20 years. The diagnosis of HLHS in fetal echocardiography is based on 2-dimensional echocardio -graphic evidence of a diminutive ascending aorta, aortic atresia, mitral atresia or severe stenosis and a hypoplastic left ventricle. Abnormal flow direction through atrial septum or through isthmus greatly aids the diagnosis. This report shows a fetal case who showed hypoplastic left side chambers and retrograde isthmic flow and was diagnosed with hypoplastic left heart syndrome. After birth, although the baby had tachy-dyspnea for the first 3 weeks, she finally recovered without any intervention and showed catch up growth of left side chambers. This case illustrates the extreme difficulty of assessing left ventricle in a fetus.

저자들은 재태 $31^{+5}$주 산전 심초음파검사에서 좌심형성부전 및 대동맥축삭으로 진단되어 추적 관찰한 태아가 출생 후 전부하의 증가에 의해 단기간 내에 좌심실 용적의 증가를 보임으로 생후 최소 며칠은 심근세포 증식의 가능성이 있음을 경험하였기에 문헌고찰과 함께 보고하는 바이다.