BS tuyen hoi chung thieu san tim trai

HYPOPLASTIC LEFT HEART SYNDROME Dr Lê Kim Tuyến Dr Đào Hữu Trung Pr.Phạm Nguyễn Vinh Review by text Pathology Management Video Conclusion  This term defines a spectrum of malformation which share underdevelopment of the left heart, including the left atrium, mitral valve, left ventricle, aortic valve and aortic arch Circulation depends on the patency of ductus arteriosus, utilizing the right ventricle as the combined systemic and pulmonary ventricle HLHS: definition “Complete ductus dependence of the systemic circulation due to mitro-aortic atresia or hypoplasia with hypoplasia of the left ventricle” SITUS SOLITUS D- VENTRICULAR LOOP CONCORDANT A-V and V-ARTERIAL CONNECTION HLHS: epidemiology Prevalence Natural Hystory 0,2-0,3/1000 live births 3,5% of CHD - 55% male 95% mortality in the 1° month 80% mortality in the 1° week Autosomal recessive or multifactorial inheritance Concordant lesions (Bicuspid aortic valve in 1st degree relatives) Chromosomal 10% Extracardiac Major malformations 10% Anomalies 30% Other 10% Familiar recurrence Ao Ao - PA SPTI DPTI LV Ao RV - PA SPTI DPTI LV Good Luck! ... Extracardiac anomalies Ao ≤ mm Mitro-aortic atresia Age > m Weight < kg Cardiopulmonary by-pass time Neonatal diagnosis ↑creatinine J H Kern Am J Cardiol 1997 Restrictive FO W Tworetsky Circulation...     Better QP for the same PA sys pr No PA regurgitation despite less pulsatility that may stimulate growth? For RV function: RV/PA better than Ao/PA   Better coronary diastolic perfusion