Neonatal Repair of Total Anomalous Pulmonary Venous Connections (TAPVC)- Cardiac Type

This is an 8-day old, 3.4 kg female neonate presented with cyanosis and failed the neonatal screening at 24 hours due to tachypnea and desaturation. She was born at 39 weeks gestation via a normal vaginal delivery, and apart from gestational diabetes, pregnancy was otherwise uncomplicated. Echocardiography showed total anomalous pulmonary venous connection (TAPVC) of the cardiac type, where all pulmonary veins come to a confluence that connects to a large dilated coronary sinus which then drains into the right atrium. There was a moderate size patent ductus arteriosus. Computed tomography scan confirmed the diagnosis.

TAPVC is a rare cardiac anomaly and it represents about 1.5 % of all congenital heart defects. Cardiac type of TAPVC represents about 20% of all cases of TAPVC. This type was thought to be a non-obstructed type; however, there is evidence of almost 20% risk of obstruction that occurs at the connection site between the pulmonary venous confluence and the coronary sinus. This should be ruled out with certainty either by echocardiography or cross sectional imaging. Surgery is mostly on elective basis in this type of TAPVC in absence of obstruction. Several techniques have been described, however in absence of obstruction; the most straightforward strategy is to unroof the coronary sinus into the left atrium and close the newly created atrial septal defect such as it was done in the current case. This drainage of the coronary sinus into the left atrium will drop the patient oxygen saturation by few points only but it should not be problematic.

Our patient underwent the above described technique and was extubated in the operating room and discharged home three days later. Echocardiography revealed widely patent communication between the pulmonary venous confluence/coronary sinus and the left atrium and no residual shunts. The patient oxygen saturation at time of discharge was 95-98% on room air.

References

1. Herlong JR, Jaggers JJ, Ungerleider RM. Congenital Heart Surgery Nomenclature and Database Project: pulmonary venous anomalies. Ann Thorac Surg. 2000;69(suppl):S56–S69
2. St Louis JD, Harvey BA, Menk JS, Raghuveer G, O’Brien JE, Bryant R, Kochilas L. Repair of “simple” total anomalous pulmonary venous connection: a review from the Pediatric Cardiac Care Consortium. Ann Thorac Surg. 2012;94:133–137. doi: 10.1016/j.athoracsur.2012.03.006
3. Hancock Friesen CL, Zurakowski D, Thiagarajan RR, Forbess JM, del Nido PJ, Mayer JE, Jonas RA. Total anomalous pulmonary venous connection: an analysis of current management strategies in a single institution. Ann Thorac Surg. 2005;79:596–606.

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