Slagæðavíxlun við hjarta á Íslandi 1971-1996

Abstract

Objective: We reviewed our experience regarding D- and L-transposition of the great arteries (D- and L-TGA) in Iceland over a 26 year period, from 1971 to 1996. We looked at incidence, diagnosis, treatment and outcome and any changes in these parameters during the study period were noted. Material and methods: Data were obtained from hospital records which contained echocardiographic, cardiac catheterization- and autopsy reports. Results: There were 31 children diagnosed as having transposition of the great arteries during the study period, 29 had D-TGA. Follow-up period was from 11 months to 21 years (median 13 years). The incidence was 1:3681 births and male to female ratio 2.4:1. Cardiac catheterization was used as a diagnostic tool in 11 cases, but as of 1984 all diagnoses were made by echocardiography. Of the patients with D-TGA, 21 (72%) had no additional cardiac defects, however when these were present, a ventricular septal defect was most common. Twenty-six patients (84%) underwent a balloon atrial septostomy and it was successful in 24 (92%). Twenty-three of 31 patients (74%) have had cardiac surgery, all fully corrective. Of the 23 surgeries, 21 were done in London, England. Fifteen children had atrial switch repair, a Mustard operation was done twice and Senning in 13 patients. In five cases an arterial switch operation was performed and three patients had other surgeries. Half of the patients had a difficult post operative course, however there was only one death within the first month following surgery. Of the 31 children born with TGA from 1971 to 1996, eight (26%) have died and two are lost to follow-up. All the patients that died were born during the first half of the study period. Of these eight children, four died within the first four days of life. Corrective cardiac surgery had been done on two patients before death. There are 21 patients alive which updated information is available on. All are in good condition leading full active lives. Five patients are needing prescription medications, thereof two antiarrhythmics. Of the most recent echocardiograms, 16 are without hemo-dynamically significant sequela. In five cases however, echocardiography shows significant abnormalities, probably warranting intervention within the next few years. Conclusions: The diagnosis, treatment and outcome of patients with transposition of the great arteries has improved dramatically over the years. Close follow-up of these patients with regards to sequela of cardiac surgery is mandatory, especially since the majority of this patient population has had atrial switch repair.

Ágrip Tilgangur: Rannsóknin varðar slagæðavíxlun við hjarta (D- and L-transposition of the great arteries) á íslandi á 26 ára tímabili, 1971-1996. Skoðað var nýgengi sjúkdómsins, greining, meðferð og horfur og hvernig þessir þættir hafa breyst á rannsóknartímanum. Efniviður og aðferðir: Gagna var aflað úr sjúkraskrám sem innihéldu hjartaómskoðunar-, hjartaþræðingar- og krufningaskýrslur. Niðurstöður: Þrjátíu og eitt barn greindist með slagæðavíxlun á tímabilinu, þar af höfðu 29 fullkomna víxlun (D-TGA). Eftirlitstími er frá 11 mánuðum til 21 árs (miðgildi 13 ár). Nýgengi reyndist einn á hverjar 3681 fæðingar, kynhlutfall 2,4 drengir á hverja telpu. Í 11 tilvikum var hjartaþræðing notuð til sjúkdómsgreiningar, en frá 1984 hefur sjúkdómurinn í öllum tilvikum verið greindur með ómskoðun. Tuttugu og eitt barn hafði fullkomna slagæðavíxlun eingöngu, en þegar einnig voru til staðar aðrir hjartagallar var oftast um op í sleglaskilum (ventricular septal defect) að ræða. Gáttaskilarofsaðgerð (balloon atrial septostomy) var gerð á 26 börnum (84%) með viðunandi árangri hjá 24 (92%). Af 31 sjúklingi hafa 23 gengist undir hjartaskurðaðgerð og er um fullnaðaraðgerð að ræða í öllum tilvikum. Gerð var gáttaskiptaaðgerð (atrial switch/baffle) á 15 börnum, þar af Mustard aðgerð í tveimur tilvikum og Senning 13 sinnum. Slagæðaskiptaaðgerð (arterial switch) var gerð á fimm sjúklingum, en þrívegis aðrar aðgerðir. Í helmingi tilvika var gangur í kjölfar skurðaðgerðar erfiður, en aðeins eitt barn dó innan eins mánaðar frá aðgerð. Af 31 barni með slagæðavíxlun sem fæddist á rannsóknartímanum eru átta látin og tvö hafa tapast úr eftirliti. Öll börnin sem dóu voru fædd á fyrri 13 árum tímabilsins, þar af létust fjögur innan fjögurra sólarhringa frá fæðingu. Tvö börn höfðu gengist undir hjartaskurðaðgerð fyrir andlát. Af 21 einstaklingi sem á lífi er og upplýsingar liggja fyrir um, telst ástand viðunandi eða gott hjá öllum. Fimm sjúklingar eru á lyfjameðferð, þar af tveir vegna hjartsláttaróreglu. Við síðustu hjartaómskoðun var útlit gott hjá 16 einstaklingum, en rannsókn óeðlileg í fimm tilvikum. Ályktanir: Miklar framfarir hafa orðið í greiningu og meðferð slagæðavíxlunar við hjarta og horfur hafa batnað. Stöðugt eftirlit er mikilvægt með tilliti til síðkominna afleiðinga hjartaskurðaðgerða, en stór hluti sjúklinga í rannsóknarhópnum hefur gengist undir gáttaskiptaaðgerð.