Pre Operative Diagnosis:
• Pentology of Cantrell – Hallor – Ravitch.
• DORV, VSD, PAH + LV apical diverticulum.
Comments:
Karolina is a 3 months old baby girl from Tashkent , presenting with anterior parietal wall defected involving lower thorax & upper abdomen. CT angiogram and Echocardiography reveled the above diagnosis. USG brain was done to screen for any associated midline intracranial defects which were normal. Mother and maternal grandmother were counselled regarding the risk / benefits of this procedure, the palliative nature of the PA banding and need for intracardiac tunneling in the future.
 
Procedure Performed:
• Pulmonary artery banding and LV diverticulectomy.
• PTFE patch repair, repair of pericardial, Diaphragmatic and anterior abdominal wall defects.
Findings:
• Mesocardia.
• Absent Xiphisternum, well formed Gladiollus & manubrium.
• Defect in pericardium, diaphragm and upper anterior abdominal wall.
• Omphalocete contained frond – like LV apical diverticulum with all the cardiac wall components, Colon & small. • Normal coromary distribution.
• MPA dilated, smallish Asc Aorta, Extremely dextroverted position of RA.  
Operative Steps:
• Midline sternotomy, Rt thymic lobe excised and pericardium slit in the miline upto the defect in the pericardium and diaphragm through which LV apical Diverticulum was herniating into the ophalocele.
• Diverticulum was clamped and amputed. The stump was suture closed using 4-0 prolene and felt pledgets.
• Skin over the omphalocele was incised in continuity with the midline thoracic incision and hernia sac was opened. A PTFE pathc (Duomesh) was sutured to the edges of the pericardial, diaphragmatic defect and the most superior aspect of the anterior abdominal wall defect. The whole length of gut was inspected. Rest of the midline defect was closed by mobilizing the anterior rectussheath approximated by 3-0 PDS suture in an interrupted fashion.
• Subcutaneous closure over the abdominal incision done after creating an umbilicus by infolding of the redundant skin .
• MPA looped and banded to circum ference of 26mm (22 + 1mm/kg) using mersiline tape.
• Stable hemodynamics and adequate blood gasses achieved.
2V + 2A pacing wires placed and Rt pleuropericardial drain placed.
• Hemostasis achieved.
• Pericardium closed using 0.1mm thick PTFF pericardial membrane.
• Sternum approximated with steel wires. Subutaneous closure done using 3-0 PDS.
• Skin close using subcuticular 4-0 vicry.
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