Home

Loading...

Table of contents

Hypoplastic left heart syndrome

Guidelines

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)....
Show more

Screening and diagnosis

Prenatal diagnosis: as per AEPC/EACTS 2020 guidelines, 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.
B
Show 4 more
Create free account

More topics in this section

  • Postnatal diagnosis

Diagnostic investigations

Cardiac imaging: as per AEPC/EACTS 2020 guidelines, 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.
B

More topics in this section

  • ECG

  • CXR

  • Genetic testing

Medical management

Setting of delivery: as per AEPC/EACTS 2020 guidelines, 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.
B
Show 2 more

More topics in this section

  • Initial resuscitation

  • Prostaglandin E1

  • Hemodynamic support

Perioperative care

Preoperative imaging: as per AEPC/EACTS 2020 guidelines, 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.
B
Show 3 more

More topics in this section

  • Intraoperative imaging

  • Postoperative imaging

Surgical interventions

Prenatal interventions
As per AEPC/EACTS 2020 guidelines:
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.
C
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.
C

More topics in this section

  • Immediate postnatal interventions

  • Pre-stage 1 interventions

  • Norwood stage 1 palliation

  • Hybrid stage 1 palliation

  • Considerations for anesthesia

Specific circumstances

Patients with tricuspid regurgitation: as per AEPC/EACTS 2020 guidelines, Recognize that tricuspid regurgitation moderately increases mortality and morbidity rates and prevents completion of the single ventricle pathway.
B
Show 5 more

More topics in this section

  • Patients with restrictive atrial septal defect

  • Patients with pulmonary vein anomalies

  • Patients with coronary anomalies

Follow-up and surveillance

Early postoperative care: as per AEPC/EACTS 2020 guidelines, Ensure compliance with an operating room checklist and a rigorous handover to the ICU team based on an algorithm.
A
Show 8 more

More topics in this section

  • Interstage 1 monitoring

Quality improvement

Analysis of outcomes
As per AEPC/EACTS 2020 guidelines:
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.
B
Ensure that all pediatric and congenital cardiac teams routinely assess their data against national and international benchmarks using multi-institutional databases and registries.
B