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Hypoplastic left heart syndrome

Key sources
The following summarized guidelines for the evaluation and management of hypoplastic left heart syndrome are prepared by our editorial team based on guidelines from the Association for European Paediatric and Congenital Cardiology (AEPC/EACTS 2020) and the American Heart Association (AHA 2018).
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Guidelines

1.Screening and diagnosis

Prenatal diagnosis: obtain an obstetrical fetal anomaly scan at 18-22 weeks of gestation. Include at minimum a four-chamber view, outflow tract views, and three-vessel tracheal view in the cardiac imaging during obstetrical fetal anomaly scan.
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  • Postnatal diagnosis

2.Diagnostic investigations

Cardiac imaging: obtain echocardiographic evaluation of HLHS to define the HLHS type, including the situs and cardiac variants, and assess the left ventricle and its function, ventricular septal defect, atrial septum, pulmonary veins (anatomy and Doppler flow patterns), aortic arch including flow patterns (antegrade, retrograde), RV function, and tricuspid valve. Obtain cardiac MRI as the standard for measurement of right ventricle volume and function.
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  • ECG

  • CXR

  • Genetic testing

3.Medical management

Setting of delivery: allow delivery of a fetus with HLHS, in the absence of risk factors, to occur spontaneously up to 40 weeks. Offer elective delivery planning (induction of labor or Cesarean delivery) no earlier than 39 weeks as long as there are no obstetrical risk factors.
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  • Initial resuscitation

  • Prostaglandin E1

  • Hemodynamic support

4.Perioperative care

Preoperative imaging: obtain TTE for planning initial surgery in patients with classical HLHS. Consider obtaining 3D echocardiography to assist in imaging the tricuspid valve to assess morphology and regurgitation.
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  • Intraoperative imaging

  • Postoperative imaging

5.Surgical interventions

Prenatal interventions
Consider performing fetal intervention to augment the size of the atrial septal communication to improve perinatal and longer-term outcomes in the fetus with a restrictive foramen ovale.
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Consider performing fetal intervention to improve antegrade flow across the aortic valve in an effort to achieve a biventricular outcome in fetuses with critical aortic stenosis and features of emerging HLHS.
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  • Immediate postnatal interventions

  • Pre-stage 1 interventions

  • Norwood stage 1 palliation

  • Hybrid stage 1 palliation

  • Considerations for anesthesia

6.Specific circumstances

Patients with tricuspid regurgitation: recognize that tricuspid regurgitation moderately increases mortality and morbidity rates and prevents completion of the single ventricle pathway.
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  • Patients with restrictive atrial septal defect

  • Patients with pulmonary vein anomalies

  • Patients with coronary anomalies

7.Follow-up and surveillance

Early postoperative care: ensure compliance with an operating room checklist and a rigorous handover to the ICU team based on an algorithm.
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  • Interstage 1 monitoring

8.Quality improvement

Analysis of outcomes
Use the International Paediatric and Congenital Cardiac Code in all registries, databases, and research studies in pediatric and congenital cardiac care, including cases of HLHS.
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Ensure that all pediatric and congenital cardiac teams routinely assess their data against national and international benchmarks using multi-institutional databases and registries.
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