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Coronary Artery Fistula as Source of Pulmonary Circulation in Pulmonary Atresia with Ventricular Septal Defect A. J. J. C. Boqers', J. Rohmer', S. A. E. Wolski/. 1. M. Ouoeqebeur' , and 11. A. Huqsman s ' Departmen ts of 'Fhoractc Surgery. Tho ra xcent er. Univers ity Ilospi tal Dijkzigt, Heuerdem . 2Pcdiatric Cardiolog y. "Thcracic Surge ry. University Hospital Leiden, Thc Nethe rlan ds

Pour patients a re described with pulmona ry atresia and vontricular septa l defect. in whom the pulmona ry circulation was dcpcndent on a fistula from the left corona ry ar ter y to the pulmona ry artery. The issue in this complex anom aly is eomplcte prcoperative diagnosis. including a na tomie inform ation on the corona ry arter y fistula a nd the pulmona ry vasculature. This was achieved in thc last 2 pa tients. In the last pa tient cchocardiogra phy turnc d out to be a n important diagnostic tool in this rar e anomaly and faeilitated selective a ngioea rdiogra phy. All 4 pa tlent s were successfully oper ated by closing the Ilstula. closing other aor top ulmonary connections an d inserting a valved eonduit betwecn right vent ricle and pulmon a ry a rtery. The ventricula r septal defect was closed in 3 patlcnts with a pateh. In the sett ing of an already existing pulmona ry hyperten sion a nd a possibly inadequate pulmo na ry a rterial system at surgery, a perforated patch was inserted in the ventricular septal defect oft he remaining pat ient. Key words Corona ry ar tcry flstula - Pulmonar y at resia with ventricular septal defeet

Inlrodu cti on Pulmonary circulatio n dep ending on a co ronary artery fistula to the pu lmonary a rtery, in associ ati on with pu lm on ary atresia with vent ricula r septal defect , is a very rare co mplex of cong en ital anomalies (I , 4-7. 10) . In th e few cases rep orted previous ly, preope rati ve diagn osis of the corona ry a rt ery fistu la raised a prob lem and desp ite mu ltip le ca rdiac ca the te rizations , definitive diagn osis was often only to be es ta blished a t su rgery (4 -7). We report our exper ience in 4 additional cases, in which we expe rienced com pa rable problem s . However , we would like to point at th e role of ec hoca rdiogra phy in es ta blis hin g th e di agno sis. Patie n ts Pati ent 1 was a boy of 12 years of age a t ope ra tio n in 19 69 , Echoca rdiogra phy was not yet availa ble. Pr eop erati ve car-

Thorac. cardiovasc. Surgeon 38 (1990) 30 -32 © Georg Thieme Verlag Stuttga rt · New York

Kcron a ra rt erlen flstel als Quelle des Lunge nkre isla ufs bei Pulmonal a tresie mit VSlJ Es wird über vier Patienten - im Alter von 10. 12, 14, und 19 Jah ren - mit einer Pulmonalat resie und VSD berichtet , deren Lungenkreisla uf über eine linkskoronar e Fistel ges peist wurde. Es werd en die Schwierigkeiten, eine definitive prä oper ative Diagnose zu stellen, dargestellt. Dies gela ng bei den beiden zuletzt operierten Patient en (1983 und 1984). Es wird a uf die diagnostische Bedeutun g der Echoka rdiogra phie hingew iesen . die eine selektive Angioka rdiogra phie erleichtert. Die Operation besta nd im Verschluß der Koronarfiste l un d an derer ao rtopulmonaler Verbindungen, dem Einsetze n eines Ilomografts oder eines Ha ncock-Cond utts mit Bioprothese und dem Patch-Verschluß des VSD. Beim ersten Patienten wur de 5 J ahr e nach der Oper atio n der verkalkte Homogruft durch ein Ila ncock-Conduit erse tzt. Nach weiteren 5 Jahr en versta rb diese r Pat ient plötzlich, oh ne daß die Todes urs ache geklärt we rde n konnte. Der zweite Pa tient lebte 8 Jah re nach der Operation, der dr itte Pat ient starb 3 Jahre nach der Operation plötzlich und der vierte Patient lebt 5 J ahre postoperativ in gutem Gesu ndheitszusta nd. In der Diskuss ion werden die diagnostischen und ther apeutischen Problem e diese r sehr seltene n kongenitalen Anomalie beleuchtet.

diac ca th ete riza tio n show ed pu lm on ary a tr es ia with ve ntricul ar septal defect, maj or aorto pulmona ry collateral a rte ries a n d a coro na ry a rtery fistu la from th e left main coro nary a rte ry to th e pu lmo n ary a rte ry. Th e fist ula wa s m ad e visible through aort ic roo t inj ection. wh ich was in ad equ at e to accurat ely visua lise th e pulmon ary a rt e ry. At su rge ry explora tion revealed a central pu lmonary arte rial system of normal size. Correctio n cons iste d of liga tion of aorto pulm onary collate ral a rteries an d th e coro na ry a rterial fistu la . pat ch -closure oft he ven tr icula r septal de fect a nd insertion of a n aortic root homograft from right ventricle to th e pu lm on ary artery. At follow -up the pati ent was in function al class 2 acco rding to th e New York Heart Associati on until1 7 yea rs of age , when the hom ogr aft showed calcified obstructi on a nd fals e aneurysm formation at its proxim al a nastomo sis. Th e homo graft was replaced with a Ha ncock bioprost hetic valved conduit. After this reop eration the pati en t was again in fun ctional d ass 2 , un til sudden death at 22 yea rs , ca use unknow n .

Re icei ved for Publication : J un e J:l . 1989

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Summary

Coronary Artery Fistula as Source 0/ Pulmonary Circulation in Pulmonary Atresia

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artery to the pulmonary arte ry, see Fig. 1. Preoperative ca rdiac cathete rization confirmed the echoca rdiographic findings and showed majo r aortopulmonary co!lat erals as we!l. Aort ic root injection was insufficient to completely visualise the pulmonary artery . By selective injection of the left coro na ry arte ry th e pulmonary arte ry filled ad equ at ely through the coro nary arterial fistula , see Fig. 2. Surg ery consiste d on ligation ofthe aorto pulmo na ry co!late rals, elosure of th e fistula through th e opened pulm on ary artery, pat ch-elosure of th e ventricular se ptal defect a nd ins ertion of an ao rtic root hom ograft between the right ventriele and pulm onary artery. Th e pulm onary conOue ns was reconst ructed with Gor e-Tex. At follow-up the patien t is in norm al health, functional elass 1 accord ing to the New York Heart Association.

Fig. 1 Coronaryarteryfistula(CAF) framthe lettcoronary artery(l CA) tothe pulmonary arterydemonstratedbyechocardiographyinpanent 4.Aortic(AG) crigin (AG) ofthe l CA is shown asweil

Fig.2 Coronary artery fistula (cat) tothepulmonaryartery (pu),both visualised byselective coronaryangiography intheleftcoronaryartery (lca) inpatient 4. Branchesof theIca areshownasweil

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Patient 2 is a girl of 14 yea rs at operation in 1978. Preoperative echoca rdiog ra phy showed pulmonary atres ia with ventricular septal defect and secundum at ria l septal defect. The coronary arterial fistula to th e pulmonary a rtery was initia!ly not identifi ed and only in retrospect recognized as su ch. Preop erative ca rdia c catheterization confirme d th e pulmonary atre sia and th e ventricular and atrial septal defects . In addition major aortopulmonary co!late ra l arteri es were identifi ed as we!l as a coronary arteri al fistula from the left main corona ry arte ry to th e pulmonary artery . The pulmonary artery was being only indirectly a nd inad equately visualised through th e fistula by aorti c root injection . At su rgery explora tion reveal ed a normal-si zed centra l pulmon ary arte rial syste m. Cerreetion cons isted of ligati on of th e aortopulmonary co!late rals. elosu re of the fistul a through th e opened pulmonary a rte ry a nd open ed left main corona ry artery , pat ch -elosure of th e ventricular septal defect, direct elosure of the atrial septal defect, a nd ins ertion of a Hanco ck biop rosth etic valved conduit between th e right ventriele a nd the pulmonary artery . At follow-u p th e pati ent was in functional elass 1 according to th e New York Hea rt Association unti l staphyloccocus aure us endocard itis at 21 years. With continued antibiotic treatment the pat ient remains in fun ctional elass 2. She refuses surgical therapy for the destroyed condu i!. At pr esen t she is 22 yea rs ofage. Patient 3 was a ma le of 19 years at ope ra tion in 198 3. Preoperative echocard iography showed pulmonary at resia with ventricular septal defect, major ao rto pulmona ry co!laterals and pat ent arte rial duct, No coronary arterial fistu la was recognised . Preoperative cardiac cathete rization confirm ed the echocardiogra phic dia gno sis and add itiona !ly showe d a coro nary arte rial fistu la from the left ma in coronary artery and the left an te rio r descend ing coro nary a rte ry to the pulmon ary artery . The fistula was visualised by ao rtic root injection , which was insufficient for adequat e dernonstration of the pulmo nary artery . ln a second cathe terization through th e patent arterial duct, mod erate pu lmonary hyp ertension was shown and the pulmona ry artery was visualised and conside re d to be only ma rgina!ly ad equate to a!low cor rective su rgery . Beca use ofthese finding s, th e decision was preop erativ ely made to perform cor rective surgery in case an ad equate pulmonary arterial system was found at surgery or to leave a residual ventricula r septal defect in cas e of an inadequate pulmonary arterial system, with th e option of elosure of the remaining defect in ca se of furth er development of the pulm onary arte ries and no progression of the pulm onary hypertension. At surgery however, th e pu lmonary artery was conside red to be inadequate for corrective surgery . The palliativ e pro cedure consisted of ligation of th e aortopulmonary co!late ra ls and th e coro nary arte rial fistu la. Th e arte rial duct was left open . Aperforated patch was ins erted in th e ventricular septal defect and a Hancock bioprosthetic valved conduit inserted betw een right ventriele and pulmon ary artery . At follow-up , 1 year after su rgery, the pulmonary arteria l syst em was of comparable size as befor e surgery and the pulmo nary hypertension had further increased. Surgery was not further cons idered . The patient was less cyanotic and in functio na l elass 2 accor ding to th e New York Heart Association un ti! sudden death at 22 yea rs ofage. Patient 4 is a girl of 10 yea rs at operation in 1984. Preoperat ive echocardiogra phy sho we d pulmo nary atresia with ventricular septal defect, The coro na ry arte rial fistul a was identifi ed and was localised from the left main coro nary

Thorac. cardiovasc. Surgeo n 38 (1990)

Thorac. ca rdiovasc. ~(iurgeo Tl 3 8 (1990)

Discussion In total surgieal correction of pulmonary atresia with ventricular se ptal defect and a coronary arte ry fistu la to th e pulmonary arter y, esta blishing p reop eratively th e exact diagno sis of this complex of congenita l anomalies is an important issue. Espccially, anatomie information on the location ofthe fistula and on the pulmonary vasculature is of great impor tance. In mo st of th e few cas es reported pr eviously and in 2 of our pat ients, th e precise diagnosis was not achieved pr eop eratively (1, 4- 8). In the first 2 patien ts th e surgical procedure had to sta rt with an exploration of th e fistu la aud th e pulmouary arte rial syste m be fore th e decision to start a correction could be made. In most cases reported in th e literature the fistu la was not diagnosed or it was misinterp reted preoperatively and th e diagnosis had to be correcte d at surge ry (1, 6, 8). This may be du e to the rarity of thi s ano malaus complex and the difficulties in interp retation oft he resu lts of pre ope rative analysis, in particular angiocardiography (1, 3-7), as wa s th e case in our first 2 pati ent s. In th is regard multip le cardiac catheter iza tions in each patient we re reported in a recent study to reach a complete diagno sis only in 3 out of 4 pat ients (5). In our third patient a second ca rdiac ca theterization was done to ad equ ately visualise the pulmonary arterial syste m . At the time of treatme nt of the first pati ent echocardiogra phy was not yet availab le. In th e second pati ent the coronary arte ry fistu la was only recognised in retrospect and in the third patient not recognised . However, in the fourth patient th e corona ry arte ry fistul a was looked for and recogni sed . With this information selective left coronary arteriography was ineluded in th e cardiac cathe te rizatio n and res ulted in ade quate visualization of th e coronary a rte ry fistula as weil as the pulmonary arterial syste m . Recent rep orts da not rnen tion echoca rdiography in this regard at all (5, 10). Our experience in this last pati ent dem onstrated 2D echocard iography to be very useful in thi s regard, es pecia lly in the left parasternal transverse view of th e arte rial pole of the heart , Diagnosin g the coronary a rte ry fistul a to th e pul monary arter y was facilitated in thi s way and subs eque ntly confirmed by select ive an giogr aphy. Mor eover, thi s selective angiogra phy of the fistula ca n be used to visualise th e pulmonary artery ade qua tely. Three of the reported coro nary artery fist ulas ori gina ted fro m the left main coro nary arte ry and 1 from th e left main as weil as from the left anterior desc ending coronary artery. Following the descriptive con cep t of coronary arte ry origin in relation to the aortic sinuses facing the pulmonary arter y (2, 9), corona ry anatomy in repo rted pati ents was normal: 1 R, 2 LC. In a recen tly repo rted se ries 3 out of 4 patients were reported to have a single coro nary artery, 2 cases with the pattern 2 LCR or descriptively "from th e left sinus of valsa lva" and 1 case with the pattern 1 LCR or descriptively "from th e right sinus of valsalva" (5). The combined protocol of echocardiogra phy followed by angiocardiogra phy inelud ing selective corona ry arte riogra phy should facilitat e minimalization of surgical exploration. In pr eviously reported cases of pulmon ary atresia with ventricular se ptal defect and coronary artery fistula to the pulmonary a rte ry, thi s some times rai sed a pr oblem (1,6, 8). As a conseque nce ofl ess su rgical exploration, the fistula should not be an increm ental risk factor in correction of pulmonary atresia with ventrieular septal defect (3, 4). Th e therap y of cho ice being elosure of the

A. J. 1. C. Bagers et al.

fistula with preserv ation of normal coronary fiow (1). closure ofthe ventricular septal de fect, discon nection of other systemic-pulmonary connections and reconstruction of a right ventricular out flow tr act, pr eferably with a preserved homograft, i. e. a cryopreserved pulmonary hornograft. We conelude th at in pulmonary atresi a with ventricular septa l defect , echo cardiography sho uld in addition be used to look for a possib le corona ry artery fistula to the pulmonary artery. Ir such a fistu la exists, it should be confirme d by selective coronary a rteriogra phy. This will exactly visu alise the fist ula as weil as allow ad equ at e demonstration of th e pulmonary arte rial syste m. Th e diagnosis es tablishe d pr eop eratively in thi s way facilitates surg ical correction .

References 1

2

3

4

Bag ers. A. 1. J. c.. 1. M. Quaegebeur. and H, A. fl uy smans : Early and late results of surgica l treatment of conge nita l coro nary artery fistula. Thora x 42 (1987) 369 -373 Gitte nbercer-de Groot. A. c. . U. Sauer. A. Oppenheimer-Dekker . and 1. M . Quaegebeu r: Co ronary arter ial anato my in transposition ofthe great arte r ies. a morphologie study. Ped . Cardio l. 4 , sup pl. 1

(1983) 15- 24 Kirklin. 1. w.. and B. G. Harrett -Boues : Cardiac Surgery. 1'( edn . New York, Wiley 1986 Kronera d. 8.. D. E. Ritter. A. lIa we. O. W. Kincaid. and /) . C.

McGoon: Pulmonary atres ia or se vere stenos is and coro nary artery 5

to pulmona ry arter y flstula. Circulation 46 (1972) 100 5- 1012 Puhl, H.. L. Fang. R. H, Anderson, S. C. Par k. and J. R. Zuberbuhler: Fistu lous communications between a so lila ry coronary arte ry and the pulmonary arteries as th e prima ry so urce of pulmonary blood su pply in tetralogy of Pallot with pulmonary atresia . Am. J. Cardiol.

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Rastetli. G. c., P. A. Ollgly. G. D. lJavis. and 1. W. Kirkltn: Surgical repair for pulmonary valve atresia wit h coronary-pulmo nary artery f lstu la: re port of a case . Maya Clin . Proc. 40 (1965) 52 1- 527 Rohmet. 1.. T. N. Huis-Liem . and J. M. Quaege beur: Oorspro ng van de arteria pulmonalis uit de linker arte ria coronaria bij pulmo na llsatresle met ventrikelse ptuni defekt Ia bstr .l. Ned . Tijdsc hr.

Genees kd. 130 (1986 ) 710 Roos. J. P.. 11. Hartma n. JI. van der Schaar, and A. G. Brom: Diagnosis and surgtcal treat me nt of corona ry artery flstula . Thorax 25 (19701259-266 Quaegebeur. J. M.. J. Rohm er. J. Ottenkump, T. N. Uuis-Liem. J. W. Kirklin. H. 11. Blackst one. and A. G. Brom: The arterial sw itch opera tion, a n e ight-yea r experience . J. Thorac. Cardiovasc. Surg. 92 (1986) 361 - 384 Vignes wura n. W. 1:. and J. C. S. Pollock: Pulmona ry atresia with ventricula r se ptal defec t and coronary artery f lstula: a la te presentation. Hr. Ileart J. 59( 988) 387- 388

A. 1. 1. C. Boqers. M. D. Department of Thoracic Surgery Thora xee nter Univers ity Hospital Dijkzigt Dr. Molewaterplein 40 NL-Rotterdam

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Coronary artery fistula as source of pulmonary circulation in pulmonary atresia with ventricular septal defect.

Four patients are described with pulmonary atresia and ventricular septal defect, in whom the pulmonary circulation was dependent on a fistula from th...
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