BS tuyen hoi chung thieu san tim trai

HYPOPLASTIC LEFT HEART
SYNDROME

Dr. Lê Kim Tuyến
Dr. Đào Hữu Trung
Pr.Phạm Nguyễn Vinh


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Review by text
Pathology
Management
Video
Conclusion


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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

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HLHS: hemodynamics
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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%)
•33 gestations continuing after 28° week

interruption
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
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Fetal diagnosis doesn’t improve
the pre and post operatory mortality

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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