Kongenitalne konorarne arterijske fistule: kliničko i terapijsko razmatranje [Congenital coronary artery fistulas: clinical and therapeutic consideration]

Malčić, Ivan and Bertić, Mia and Eicken, Andreas and Kniewald, Hrvoje and Kašnar-Samprec, Jelena and Šarić, Dalibor and Schreiber, Christian and Bartoniček, Dorotea and Dilber, Daniel (2014) Kongenitalne konorarne arterijske fistule: kliničko i terapijsko razmatranje [Congenital coronary artery fistulas: clinical and therapeutic consideration]. Liječnički vjesnik, 136 (9-10). pp. 261-269. ISSN 0024-3477

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Abstract

A coronary artery fistula is a link between one or more coronary arteries with another heart cavity or a segment of systemic or pulmonary circulation. Arterial blood from a coronary vessel enters another segment via myocardial capillary bed. These are very rare anomalies which constitute approximately 0.2 - 0.4% of all congenital heart defects. Still, they are clinically significant if they are of medium or large size and are manifested with a series of clinical symptoms such as angina pectoris, arrhythmias, myocardial infarction, endocarditis, progressive dilatation, heart failure and cardiomyopathy, pulmonary hypertension, thrombosis of the fistula and formation of aneurysms with possible ruptures. We present six patients with a coronary arterial fistula, their history, diagnostic procedures and outcomes. Therapeutic closure of coronary artery fistulas is recommended in all symptomatic, but also in asymptomatic patients, if there are significant roentgenographic, electrocardiographic and other abnormalities. In recent times transcatheter closure of coronary fistulas has become a possible alternative to surgery and is becoming increasingly used thanks to improved diagnostic possibilities and technology. If possible, interventional closure of fistulas is precisely the method preferred in pediatric patients. The choice of method depends on the anatomy of the fistula, presence or absence of additional defects, and on the experience of an interventional cardiologist or a heart surgeon. If performed well, the effects of both methods are good. This paper presents two children with a fistula between the right coronary artery and the right ventricle (RV), one child with a fistula between LAD and RV, one child with a fistula between the main tree of the left coronary artery (LCA) and RV, one child with a fistula between LCA and the right ventricular outflow tract (RVOT), and one child with a fistula between LCA and the right atrium (RA). The last one (LCA-RA) is not described in the latest classification of anomalies of coronary blood vessels in children based on MSCT coronarography, so we consider our presentation to be a contribution to the new classification. Along with the descriptions of fistulas and presentations of interventional and cardiosurgical interventions, we are also presenting a rare case of spontaneous closing of the fistula within the first six months and of a reopening of the fistula between the right coronary artery and the right ventricle after six years.

Abstract in Croatian

Koronarna je arterijska fistula komunikacija između jedne ili više koronarnih arterija s nekom drugom šupljinom u srcu ili segmentom sistemskog ili pulmonalnog optoka. Arterijska krv iz koronarne krvne žile ulazi u drugi segment preko miokardijalnoga kapilarnog bazena. To su vrlo rijetke anomalije, a učestalost im se procjenjuje na 0,2 – 0,4% svih prirođenih srčanih grješaka. Ipak, one su klinički važne ako su srednje velike ili velike te se očituju nizom kliničkih simptoma kao što su angina pectoris, aritmije, miokardijalni infarkt, endokarditis, progresivna dilatacija, srčana insuficijencija s kardiomiopatijom, plućna hipertenzija, tromboza fistula i razvoj aneurizme s mogućom rupturom. Ovdje prikazujemo šest bolesnika s koronarnom arterijskom fistulom uz opis toka bolesti, dijagnostičkih postupaka i ishoda bolesti. Terapijsko zatvaranje koronarnih fistula preporučuje se kod svih simptomatskih, ali i kod asimptomatskih bolesnika, i to ako postoje značajni laboratorijski znakovi bolesti, u prvom redu rendgenske ili elektrokardiografske abnormalnosti. Transkatetersko zatvaranje koronarnih fistula u novije je vrijeme moguća zamjena kardiokirurškom pristupu i sve se više rabi zahvaljujući većim dijagnostičkim mogućnostima i sve boljim tehnološkim rješenjima. Danas se kod pedijatrijskih bolesnika daje prednost intervencijskom zatvaranju fistula ako je to moguće. Odabir metode ovisi o anatomiji fistule, prisutnosti ili odsutnosti dodatnih defekata te o iskustvu intervencijskog kardiologa ili kardijalnog kirurga. Učinak obiju metoda je dobar ako se uspješno izvedu. U našem je radu prikazano dvoje djece s fistulom između desne koronarne arterije i desne klijetke (RV), jedno dijete s fistulom između LAD-a i RV-a, jedno dijete s fistulom između glavnog stabla lijeve koronarne arterije (LCA) i RV-a, jedno dijete s fistulom između LCA i izlaznog trakta desne klijetke (RVOT-a) i jedno dijete s fistulom između LCA i desne pretklijetke (RA). Ova posljednja (LCA – RA) fistula nije opisana u najnovijoj klasifikaciji anomalija koronarnih krvnih žila kod djece na osnovi MSCT koronarografije pa svoj prikaz smatramo doprinosom novoj klasifikaciji. Uz opise različitih fistula i prikaze intervencijske i kardiokirurške intervencije prikazujemo i rijedak slučaj spontanog zatvaranja tijekom prvih šest mjeseci i ponovnog otvaranja fistule nakon 6 godina između desne koronarne arterije i desne klijetke.

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