A form of severest variety of pediatric heart disease.

In this complex heart disease, left sided heart components are severely small in size or may be atretic.

Clinical Presentation
At birth, neonate can have normal appearance as there is a large PDA.
In next 48 hours, when PDA is getting close then neonates can present in the form of bluishness of the body, shortness of breath or in shock.

Diagnostic Evaluation
This defect can be diagnosed antenatally also by doing fetal echocardiography and If diagnosed, then it is a clear indication for medical termination of pregnancy.

After birth, Echocardiography needs to be done so that timely management can be planned.

Treatment Modalities
Without treatment, it is fatal, but with treatment, neonate can be saved. Prostaglandin (PGE1) infusion to be started for maintaining the patency of PDA.

Open Heart Surgery
Pediatric cardiac surgeon performs multiple operations. Surgical operations are complex.

Neonates undergo 3 surgeries:

  • After birth, in the form of Norwood or Sano procedure. Sometimes, HYBRID procedurein the form of PDA stenting with PA banding can be planned instead of Norwood or Sano procedure.
  • For second & third stage surgeries: please read double outlet ventricle section in common cardiac condition of www.childrenheartcare.com for more information.