Hypoplastic left heart syndrome

Case contributed by Mr. Jijeesh K Meethal


Known hypoplastic left heart syndrome with mitral stenosis/aortic stenosis ( MS /AS ) with endocardial fibroelastosis (EFE), restrictive patent foramen ovale (PFO) and large patent ductus arteriosus. Post atrial septectomy and bilateral pulmonary banding.

Patient Data

Age: 20 days
Gender: Male
  • hypoplastic left aortic arch with its branches and large PDA
  • aberrant right subclavian artery
  • hypoplastic left atrium and left ventricle are noted and post-surgical atrial septectomy
  • dilated right atrium, right ventricle, and main pulmonary artery are noted
  • large ductus connected to the descending aorta narrowed at its aortic connection
  • normal coronal arteries  

Special Thanks to

Dr. Ajay Prashanth Dsouza (consultant & Head of Radiology), Dr. Muhammed Anwar (consultant Radiologist),Jukha Shater Ali Ali AL Badawi( Radiology Manager) and Radiology / Cardiology colleagues. Al Jalila Children's Specialty Hospital. Dubai.

Case Discussion

Hypoplastic left heart syndrome (HLHS) is a rare congenital heart defect in which the left side of the heart is severely underdeveloped. It may affect the left ventricle, aorta, aortic valve, or mitral valve.

Babies born with hypoplastic left heart syndrome usually are seriously ill soon after birth. Hypoplastic left heart syndrome symptoms include:

  • grayish-blue skin color (cyanosis)
  • rapid, difficult breathing
  • poor feeding
  • cold hands and feet
  • weak pulse
  • unusually drowsy or inactive.

Hypoplastic left heart syndrome can be diagnosed prenatally or after birth via echocardiography. Typical findings include a small left ventricle and aorta, abnormalities of the mitral and aortic valves, retrograde flow in the transverse arch of the aorta, and left-to-right flow between the atria. It is often recognized during the second trimester of pregnancy, between 18 and 24 weeks' gestation.

Surgical operations to assist with hypoplastic left heart are complex and need to be individualized for each patient and must to assess all medical and surgical options on a case-by-case basis

  1. Norwood procedure
  2. Hybrid procedure
  3. Glenn procedure
  4. Fontan procedure

Infants undergo either the staged reconstructive surgery (Norwood or Sano procedure within a few days of birth, Glenn or Hemi-Fontan procedure at 3 to 6 months of age, and the Fontan procedure at 1 1/2 to 5 years of age) or cardiac transplantation.

PlayAdd to Share

Case information

rID: 82886
Published: 8th Dec 2020
Last edited: 8th Dec 2020
Inclusion in quiz mode: Included
Institution: Al Jalila Children's Speciality Hospital

Updating… Please wait.

 Unable to process the form. Check for errors and try again.

 Thank you for updating your details.