Hypoplastic Left Heart Syndrome (HLHS)

Present by 4A Ri 田馥綿

History

1952, Lev's : congenital cardiac malformations associated with underdevelopment of the chambers on the left side and a small ascending aorta and arch.

1958, Noonan and Nadas: describe the morphologic features of combined aortic and mitral atresia

Introduction

Marked hypoplasia of the left ventricle and ascending aorta

The aortic and mitral valves are atretic, hypoplastic, or stenostic

A large patent ductus arteriosus supplies blood to the systemic circulation

Epidemiology

CHD: 4.9 per 1,000 livebirths HLHS: 1.8 per 10,000 livebirths Account for 3.8% of all CHD(8th)

Hypothesis

A late phenomenon after embryogenesis, eg. Viral illness, hypoxemic event.

Diminished flow from foramen ovale LV hypoplasia

History

Routine obstetrical ultrasound examination

2-5%: respiratory symptoms and systemic cyanosis at birth

First 24-48 hrs: cyanosis, tachypnea, respiratory distress (closure of ductus arteriosus)

Clinical menifestations Heart failure: dyspnea, hepatomegaly, lo

w C.O. Weak or absent peripheral pulse Palpable RV parasternal lift, systolic mur

mur

Prognosis & complications

Morality: 90% in the 1st month, usually during 1st wk or two.

80-90% survival rates for the first-stage Norwood operation

Heart transplantation 1/3: CNS abnormality

Pre-op management

Correct acidosis and hypoglycemia Support systemic blood flow: PGE1 for P

DA Avoid excessive pul. blood flow

Surgical therapy

1979, Norwood: first successful palliation on a neonate.

3 stages: 1. The Norwood procedure (first 2 wks) --reconstruction of Aorta

2. Hemi-Fontan (4-6 months) 3. Fontan (18-36 months) --separate pulmonary and systemic flow

Norwood procedure

Atrial septectomy Ductus arteriosus ligation Form a neoaorta Aortopulmonary shunt

Hemi-Fontan: Connect SVC to pulmonary arteries Fontan: Connect IVC to pulmonary arteries

Complications s/p Norwood procedure

Bleeding, low cardiac output syndrome, and arrhythmia

Aortic arch obstruction at the site of surgical anastomosis

Progressive cyanosis caused by limited blood flow through the shunt

Complications s/p Fontan procedure Transient superior vena cava

syndrome Persistent pleural or pericardial

effusion

Prognosis of surgical treatment

Success rate (survival to discharge):

Stage1:75%

Stage2:95%

Stage3:70%

5-year survival: 70%

Quality-of-life outcomes

Behavioural abnormalities, learning disabilities, lower intelligence scores

Multifactors:1. Possible associated abnormalities of CNS2. Hemodynamic instability in the pre-op perio

d3. Intra-op perfusion --> neurologic injury

Risk factors with HLHS

Prematurity/low birth weight Chromosomal and other extracardiac an

omalies Additional intracardiac lesions/anatomic

variants Obstructed pulmonary venous return

Summary

Early diagnosis, operative techniques for HLHS: progress in the care of these children

Define the optimal strategies to improve survival and quality of life

Thanks for your attention!!!