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Hypoplastic Left Heart Syndrome (HLHS)

A complex congenital heart disease in which the left heart structures are underdeveloped and that is fatal if untreated.

Written by: Saygı Hospital Health Guide Editorial Board
Last updated:

This content has been compiled by the Saygı Hospital Health Guide Editorial Board and is periodically reviewed by a specialist physician.

References (5)

This content is for informational purposes only and does not constitute medical advice. You can book an appointment at our Çocuk Sağlığı ve Hastalıkları department. Book Appointment →

What is Hypoplastic Left Heart Syndrome (HLHS)?

Hypoplastic left heart syndrome (HLHS) is a severe congenital heart disease in which the left-sided cardiac structures are developmentally small and non-functional. The right ventricle has to provide the entire systemic circulation, and systemic blood flow is dependent on a patent ductus arteriosus.

It can largely be detected by antenatal ultrasonography. After birth, when the ductus begins to close, systemic perfusion decreases and hypothermia, acidosis, hypotension and shock develop. It can also be detected by pulse oximetry screening.

Treatment keeps the ductus open with prostaglandin E1. The condition is treated with a three-stage palliative surgical scheme: Norwood, Glenn and Fontan procedures. In some centers a hybrid approach or heart transplantation may also be an option.

Symptoms

Gray-pale appearance after birth
Respiratory distress and acidosis
Weak femoral pulses
Shock and hypothermia
Cyanosis
Hepatomegaly
Feeding difficulty

Risk Factors

Chromosomal anomalies (Turner, trisomy 13/18)
Family history of congenital heart disease
Maternal drug use
Male sex (slightly more common)
Pre-gestational diabetes
Teratogen exposure

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

  • Suspicion of left heart hypoplasia on antenatal ultrasonography
  • Pale-gray color and weak pulse at birth
  • Low pulse oximetry readings
  • When findings of shock appear
  • Prenatal referral for fetal echocardiography decision

Treatment Methods

01
Prostaglandin E1 infusion
02
Atrial septostomy (if needed)
03
Norwood operation (stage I, neonatal)
04
Glenn shunt (stage II, 4-6 months)
05
Fontan operation (stage III, 2-4 years)
06
Heart transplantation (selected cases)

Which Department to Visit?

You can visit our Çocuk Sağlığı ve Hastalıkları department for these complaints. Our specialist physicians will create the most suitable treatment plan for you.

Learn About Çocuk Sağlığı ve Hastalıkları Department

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Health Disclaimer: The information on this page is prepared for general informational purposes only. It does not replace medical diagnosis and treatment. Please consult your physician for your complaints. Saygı Hospital does not accept responsibility for actions taken based on the information on this page.