Surgical Correction of Pentalogy of Cantrell

This is a case presentation of a full-term infant female who was prenatally diagnosed with pentalogy of Cantrell and a cardiac diagnosis of ectopia cordis, a Tet-type double outlet right ventricle (DORV) with two large subaortic and inlet ventricular septal defects (VSD), left superior vena cava (LSVC) to left atrium (LA) without a bridging vein, an unroofed coronary sinus, and a large secundum atrial septal defect (ASD). The abdominal viscera were situs solitus. The patient was a planned delivery via C-section, which was uncomplicated, with saturations in the mid-80s. The interprofessional team discussed the proper timing of her concomitant procedures, as cardiac physiology was well balanced. She was presented at 21 months of age for a complete repair of her ectopia cordis, Tet-type DORV, and omphalocele. To further delineate the intracardiac anatomy, a 3D model was constructed for surgical planning. Note the secundum ASD and LSVC draining into the LA, as well as the two VSD’s and the DORV.  
 
The interprofessional team provided a time-out to discuss the cadence of the operation between team members. Prior to heparinization, the general surgery team began their dissection by entering through the epithelialized omphalocele sac into the peritoneum. A well-developed rectus sheath was identified circumferentially and was mobilized laterally to minimize tension. Once these flaps were mobilized, the cardiac surgery team began their portion of the case. A median sternotomy was performed in the standard fashion, extending the skin incision into the superior aspect of the omphalocele. Note the patient’s long flat ventricle extended beyond the diaphragm. The patient was placed on cardiopulmonary bypass in the standard manner, with the exception of an additional LSVC cannula, and the heart was arrested with 15 mg/kg of del Nido cardioplegia. Attention was then directed to the elongated right ventricular outflow tract obstruction. A longitudinal incision was made in the right ventricle (RV) and stay sutures were placed. The subaortic VSD was enlarged—as is common with DORV—to avoid outflow tract obstruction from baffle augmentation. The pulmonary valve was inspected and measured to be normal, so no issues were addressed. The baffling of the subaortic VSD patch was completed with a 5-0 polypropylene suture. The right atrium was then entered, stay sutures were placed, and the inlet VSD was identified and closed with Cormatrix and 5.0 polypropylene suture in a running fashion.  

Attention was then directed to the LSVC. A 5 mm probe was used to identify the LSVC ostium entering into the left atrium. A Cormatrix patch was fashioned in the shape of a Russian doll to baffle the LSVC to the right atrium. The narrow part of the patch was anastomosed to the floor of the LSVC ostium, and the patch was folded to extend into and close the ASD. Finally, the patch was folded upon itself to close the secundum ASD in a one-patch technique, and a 5 mm probe was utilized to ensure the patency of the LSVC baffle. All was closed with 5-0 polypropylene in a running fashion. Next, a Cormatrix patch was used to augment the infundibulum. To address the ectopia cordis, the left pleural space was entered, and the pericardial reflection was released laterally. The patient was successfully weaned off cardiopulmonary bypass, and after adequate hemostasis, the general surgery team was called to complete the procedure. Adhesions were noted and taken down with electrocautery between the liver and the rectus muscle, which allowed for adequate mobilization to remove the hernia sac. No diaphragmatic defect was noted laterally or posteriorly, and the diaphragm edges were sewn to the fascia anteriorly and to the midline fascial closure medially. Drains were placed anteriorly to the rectus fascia and brought out laterally. Excess portions of the omphalocele sac were excised, and the skin was reapproximated at the midline in an attempt to recreate the umbilicus and closed with 5.0 Monocryl sutures. 
 
Cross-clamp time was 63 minutes, and cardiopulmonary bypass time was 86 minutes. The patient was sent to the CVICU intubated and sequentially paced. Postoperative TEE demonstrated normal left and right ventricular function, no residual VSD, no gradient across the LSVC baffle to the right atrium (RA), no residual secundum ASD, and no gradient across the right ventricular outflow tract (RVOT). On postoperative day three, she was discontinued from AV pacing with a return of normal sinus rhythm. On postoperative day seven, chest tubes were removed.

References

1. RASTELLI GC, ONGLEY PA, KIRKLIN JW. SURGICAL CORRECTION OF COMMON ATRIUM WITH ANOMALOUSLY CONNECTED PERSISTENT LEFT SUPERIOR VENA CAVA: REPORT OF CASE. Mayo Clin Proc. 1965;40:528-532.
2. Rudrappa, S.C., et al., Extracardiac Rerouting of Left Superior Vena Cava to Right Atrium. Seminars in Thoracic and Cardiovascular Surgery, 2019. 31(3): p. 571-572.
3. Williams AP, Marayati R, Beierle EA. Pentalogy of Cantrell. Semin Pediatr Surg. 2019;28(2):106-110. doi:10.1053/j.sempedsurg.2019.04.006

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