Tài liệu Bs tuyen hoi chung thieu san tim trai

HYPOPLASTIC LEFT HEART SYNDROME Dr. Leâ Kim Tuyeán Dr. Ñaøo Höõu Trung Pr.Phaïm Nguyeãn Vinh 1. 2. 3. 4. 5. 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 HLHS: 4 chamber view HLHS: long axis view HLHS: ascending aorta HLHS: aortic coartation * HLHS: hemodynamics        Obligatory L-R shunt at atrial level (complete mixing) Mild Ao hypoxiemia Higher PO2 in PA Critical flow at DA level Role of PA resistences Critical flow at FO level RV function HLHS: fetal diagnosis 105 cases 1983-1988 •Diagnosis < 24° week →72/77 (93,5%) →interruption •33 gestations continuing after 28° week →all death “…that the introduction of a nationwide protocol for fetal ultrasound scanning after 18 weeks of gestation would strikingly reduce the numbers of newborn babies with hypoplastic left heart syndrome.” L.D. Allan, Lancet 1991 HLHS: impact of fetal diagnosis  Fetal diagnosis doesn’t improve the pre and post operatory mortality  Fetal diagnosis improves pre-operatory conditions but not the surgical outcome J.H. Kern Am J Cardiol 1997 L.D.Allan Heart 1998 R.K Kumar Am J cardiol 1999 HLHS: impact of fetal diagnosis 88 cases 1992-1999 (33 fetal diagnosis) Fetal Diagnosis 11/33 (33%) → interruption 8/22 (24%)→ compassionate care 14 (42%) surgery → all alive Neonatal Diagnosis 17/55 (31%)→compassionate care 38 (69%) surgery → 25 alive (66%) Alive after fetal diagnosis 42% Alive after neonatal diagnosis 45% W.Tworetzky Circulation 2001 HLHS: surgical risk factors Norwood Transplantation Restrictive FO Pre-operatory acidosis Technical issue Extracardiac anomalies Ao ≤ 2 mm Mitro-aortic atresia Age > 1 m Weight < 3 kg Cardiopulmonary by-pass time Neonatal diagnosis ↑creatinine J. H. Kern Am J Cardiol 1997 Restrictive FO W. Tworetsky Circulation 2001 P.C.Jenkins JACC 2000 D.C. Oliveira Circulation 2004