Dvostruka (pregrađena) desna klijetka - prikriveni pratitelj ventrikularnoga septalnog defekta [Double chambered right ventricle – concealed companion of ventricular septal defect]

Malčić, Ivan and Tokić Pivac, Višnja and Anić, Darko and Težak, Stanko and Bartoniček, Dorotea and Šarić, Dalibor and Belina, Dražen (2018) Dvostruka (pregrađena) desna klijetka - prikriveni pratitelj ventrikularnoga septalnog defekta [Double chambered right ventricle – concealed companion of ventricular septal defect]. Liječnički vjesnik, 140 (11-12). pp. 307-313. ISSN 0024-3477

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Abstract

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.

Abstract in Croatian

CILJ: Cilj je ovog rada prikazati kliničke karakteristike i rezultate kirurškog liječenja petero bolesnika s dvostrukom (pregrađenom) desnom klijetkom (engl. Double-Chambered Right Ventricle – DChRV ) kao mogućom posljedicom ventrikularnoga septalnog defekta (VSD). ----- REZULTATI ISTRAŽIVANJA: U petero djece (4 djevojčice i 1 dječak) u dobi od 2,3 do 11,3 godine (srednja dob 6,3 godine) dijagnosticiran je intrakavitarni gradijent desne klijetke zbog pregradnje aberantnim mišićnim snopovima. Svi su bolesnici imali VSD s hemodinamski nevažnim lijevo-desnim pretokom (Qp/Qs od 10 do 31%). U troje djece DChRV razvio se s primarnim VSD-om, kod jednog s ostatnim defektom nakon zatvaranja perimembranoznog VSD-a zakrpom, a u jednog se djeteta desna klijetka pregradila približno 4 godine nakon primarno zatvorenog VSD-a. Intrakavitarni gradijent mjeren je s pomoću katetera end-hole u rasponu od 50 do 90 mmHg. Dva bolesnika nisu imala subjektivnih tegoba srčane naravi, a u troje se opisuje umor pri pojačanim naporima. Kod dva bolesnika VSD je bio smješten proksimalno od pregradnje (perimembranozni), a kod dva distalno (subaortalni- suprakristalni, konalni – doubly committed). Jedna je bolesnica imala dva VSD-a, jedan smješten proksimalno, a drugi distalno od pregradne stenoze. Dosad je grješka kardiokirurški korigirana u tri bolesnika patch-plastikom VSD-a i uklanjanjem zapreke u desnoj klijetki resekcijom aberantne muskulature, sve u dobi od 2 godine i 7 mjeseci do 6 godina i 3 mjeseca. Isti zahvat planiran je u još jednog bolesnika, dok se u drugoga planira izolirana resekcija aberantnog snopa. Postoperativnih komplikacija i subjektivnih simptoma srčane naravi u naših bolesnika nije bilo, a maksimalni intrakavitarni gradijent bio je 20 mmHg. ----- ZAKLJUČAK: Pregradnja desne klijetke vjerojatno je uzrokovana postojanjem VSD-a, neovisno o veličini i lokalizaciji, a može se razviti kod primarnog i rezidualnog VSD-a te nakon njegova uspješnog zatvaranja. Stoga pacijente s VSD-om, osobito perimembranoznim i suprakristalnim, valja dugotrajno kontrolirati u duljim vremenskim razmacima i nakon operacije. To je osobito važno zbog mogućnosti recidiva pregrađivanja, pojave subaortalne membrane i/ili insuficijencije aortalne valvule. Kirurško uklanjanje hipertrofičnog i aberantnog tkiva prikladan je oblik liječenja s relativno rijetkim teškim komplikacijama, što, s obzirom na očekivani progresivan tijek bolesti, opravdava operaciju pri znatnoj opstrukciji DChRV -a i odsutnosti simptoma.

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