Ivan Malčić, Višnja Tokić Pivac, Darko Anić, Stanko Težak, Dorotea Bartoniček, Dalibor Šarić, Dražen Belina
Objective: The primary objective is to present the diagnostic and treatment experiences with double chambered right ventricle (DChRV) as a possible consequence of the ventricular septal defect (VSD). Results:The intracavitary gradient as a consequence of the remodeling with aberrant muscle bundles in the right ventricle was diagnosed in five children (four females) at the age of 2.3–11.3 years (mean age 6.3 years). All patients had VSD with hemodynamically nonsignificant L-R shunts (Qp/Qs 10–31%). In three children DChRV developed with primary VSD, in one with a residual defect after patch repair of perimembranous VSD, and in one four years after adequate VSD closure. Intracavitary gradient measured by an end-hole catheter was 50–90 mmHg. Two patients had no cardiac symptoms, while three of them had exercise intolerance.In two patients VSD was located proximally to the obstructive band (perimembranous) and in two patients distally (subaortic-supracristal, conal-doubly committed). One patient had two VSDs – one located proximally and one distally to the obstructive band. The defect was so far surgically corrected in three patients with VSD patch and resection of the obstructive aberrant muscle tissue at the age ranging from two years and seven months to the age of six years and three months. One patient waits for the same surgical procedure, and the other one for an isolated aberrant band resection. There were no postoperative complications, no postoperative symptoms were reported, and maximal intracavitary gradient was 20 mmHg. Conclusion: Remodeling of RV (DChRV) is probably caused by the presence of VSD, independent of its size and location; it can develop in primary, residual VSD, as well as after successful VSD closure. Therefore, patients with VSD, especially with perimembranous and doubly committed VSD need a continuous follow up even after operation. Long-term follow up is necessary due to possible obstruction recurrence, subaortic membrane and/or aortic valve insufficiency appearance. Surgical removal of the hypertrophic and aberrant tissue is an adequate way of treatment with relatively rare serious complications. Regarding the expected progressive course of the disease, the operative treatment in significant DChRV obstruction even in the absence of symptoms is justified.