Discussion
A double ductal system is produced by the persistence of the distal segment of the sixth left and right aortic arch,6presenting as an isolated bilateral duct or associated with congenital heart disease, such as congenital absence of one of the pulmonary arteries, among others.3
Due to its presentation at different ages and with different stages of pulmonary hypertension, it is important to determine the appropriate therapeutic approach, without a consensus on its management. Thereby, Batlivala et al.7 highlight the importance of an early diagnosis and intervention to optimize long-term results, recommending the initial diagnosis with cardiac catheterization or magnetic resonance imaging to adequately identify the anatomy and perform pulmonary unifocalization early, coinciding with Varghese et al.8 that recognition of the anatomy is essential since it proposes reconnection in patients with origin of RPA from the brachiocephalic trunk with a flap from the MPA.
Krammoh et al.5 propose a two-stage strategy, with the placement of an intraductal stent followed by direct surgical reconnection or a prosthetic graft, coinciding with the series by Mery et al,9 where they performed centralization, a surgical connection between the MPA and RPA, in a single stage in 2 patients. With the remaining 8 cases, an intraductal stent was placed in 2 of the cases and 6 were treated with a systemic-to-pulmonary shunt. In a second surgical procedure 7 patients were later corrected with centralization, highlighting the rehabilitation of the pulmonary artery branches as an effective measure to restore the affected blood flow.
The critical factor that determines the treatment strategy is the size of the affected pulmonary artery, as determined by Trivedi et al.10 who found that the best strategy for patients with hypoplasia of RPA and a wide left ductus arteriosus and pulmonary hypertension, was the hybrid therapy with two stages: right intraductal stent and closure with a ductal occluder device of the left ductus arteriosus, facilitating the growth of RPA with normalization of pulmonary pressures and finally performing surgical centralization with a vascular graft with cardiopulmonary bypass similar to the proceedings in our center.
In conclusion, due to the low incidence of this defect, and its delay in diagnosis and treatment, it is important to determine the best therapeutic strategy, a situation that in our center carried the therapeutic success of the two patients presented.
References
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