Dvostruka (pregrađena) desna klijetka - prikriveni pratitelj ventrikularnoga septalnog defekta

Scientific paper - Case study

• Title (english): Double chambered right ventricle – concealed companion of ventricular septal defect
• Author: Ivan Malčić
• Author: Višnja Tokić Pivac
• Author: Darko Anić
• Author: Stanko Težak
• Author: Dorotea Bartoniček
• Author: Dalibor Šarić
• Author: Dražen Belina
• Author's institution: University of Zagreb; School of Medicine
• Scientific / art field, discipline and subdiscipline: BIOMEDICINE AND HEALTHCARE; Clinical Medical Sciences

Abstract (english)

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 (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.

• Language: croatian
• Publication type: Scientific paper - Case study
• Publication status: Published
• Peer review: Peer review
• Publication version: Published version
• Journal title: Liječnički vjesnik
• Numbering: vol. 140, no. 11-12, pp. 307-313
• p-ISSN: 0024-3477
• DOI: https://doi.org/doi:10.26800/LV-140-11-12-41
• URN:NBN: urn:nbn:hr:105:773002
• Publication: 2018
• Document URL: http://doi.org/10.26800/LV-140-11-12-41
• Type of resource: Text
• Access conditions: Open access
• Created on: 2024-03-28 10:17:40