Heart 1997;78:91-93

CASE REPORT

Multiple coronary artery-left ventricular fistulae:haemodynamic quantification by intracoronaryDoppler ultrasound

Achim Meissner, Markus Lins, Gunhild Herrmann, Rudiger Simon

AbstractMultiple coronary artery-left ventricularfistulae involving all three major coronaryarteries are extremely rare. Clinical find-ings are heterogeneous but include a his-tory of typical or atypical angina pectorisin most cases. Coronary arteriography in a65 year old woman who presented withchest pain at rest revealed multiple finefistulae arising from the left anteriordescending, left circumflex, and rightcoronary arteries. Left-to-left shunt wasestimated by measurements of coronaryartery flow velocity with intravascularDoppler ultrasound.

(Heart 1997;78:91-93)

Keywords: coronary fistulae; intracoronary ultrasound;coronary anomaly

Figure I Selective coronary arteiography (30° right anterior oblique view) showing theleft anterior descending (LAD) and circumflex (CX) arteries. Multiple fine fistulae arisingfrom the first diagonal branch (D1) and the first marginal branch (MI) communicatewith the left ventricular cavity.

Congenital coronary artery fistulae are a rarefinding in about 0-2% of patients undergoingcardiac catheterisation."2In more than 90% ofthese cases a single fistula is draining into rightheart chambers or the pulmonary artery withan ensuing left-to-right shunt. Multiple fistu-lous communications to the left ventricleinvolving all three major coronary arteries area rare anomaly with fewer than 20 docu-mented cases in the literature.3 We report thefirst case in which a quantitative estimate ofleft-to-left shunt was obtained by measure-ment of coronary artery flow velocity withintravascular Doppler ultrasound.

Case reportA 65 year old woman was admitted to ourintensive care unit with chest pain at rest. Shehad a 10 year history of non-insulin dependentdiabetes mellitus, hypertension, and hypercho-lesterolaemia. The patient smoked 20 ciga-rettes per day and reported no previousepisodes of angina pectoris or dyspnoea.Physical examination revealed a normal firstand second heart sound and an uncharacteris-tic systolic murmur grade 2/6 in the secondright intercostal space. Murmurs were alsofound over the femoral and carotid arteriespulses on both sides. Blood pressure was150/80 mm Hg. There were no pulmonaryrales and no ankle oedema. The electrocardio-gram showed sinus rhythm (65 beats/min)with minor (0- 1 mV) ST segment elevations inleads II, III, and aVF. This appeared sugges-tive of acute myocardial infarction andprompted thrombolytic treatment with intra-venous recombinant tissue plasminogen acti-vator (rtPA). In addition, the patient receivedintravenous heparin, aspirin, and nitrates.Serum enzyme activities of creatinine kinase(CK) and CK-MB reached a maximum of 266and 35 U/1, respectively. Chest x ray appearednormal. The clinical course was uneventfuland the patient was transferred to the wardafter two days.

In the bicycle exercise test a submaximalworkload (150 W) was achieved after oneweek. No clinical or electrocardiographicalsigns of myocardial ischaemia were inducible.Heart catheterisation and coronary angiogra-phy were performed on day 14. Left ventricularangiography revealed a normal sized hyper-

Department ofCardiology, 1s'Medical Clinic,University of Kiel,Schittenhelmstrasse12, D-24105 Kiel,GermanyCorrespondence to:Dr Meissner.Accepted for publication24 March 1997

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Figure 2 Selective coronary arteriography (300 right anterior oblique view) showing theright coronary artery (RCA). A maze offine vessels arise from the posterior descendingartery (PDA) and communicate with the left ventnicular cavity.

trophic left ventricle (end diastolic volumeindex 76 mlm7) without regional wall motionabnormalities, and an ejection fraction of69%. Selective arteriography of the left coro-nary artery was perturbed by the precipitousdilution of the contrast medium resulting fromaccelerated flow. The proximal part of the leftanterior descending artery (LAD), the firstdiagonal branch, and the first posterolateral

Figure 3 Phasic flow velocity spectrum in the right coronary artery. Top tracing, ECG;Middle tracing, aortic pressure; Bottom tracing, Doppler signal. Vertical axis, flow velocityin cm/s. Peak flow velocity amounted to 180 cm/s and time average ofspectral peakvelocity to 85 cm/s giving a coronaryflow rate of 960 mllmin (vessel diameter 6-8 mm).

branch of the left circumflex artery appearedtortuous and dilated with multiple fine fistulaeto the cavity of the left ventricle (fig 1). Thedominant right coronary artery (RCA) alsopresented a dilated, tortuous morphology withmultiple fine communications to the leftventricular cavity via the posterior descendingartery (fig 2). Atherosclerotic irregularitieswith a narrowing of less than 50% of the vesseldiameter were visible in the middle segment ofthe RCA.

Coronary artery flow velocity was measuredwith a commercially available Dopplerguidewire technique (0-018 inch diameterFlowire and FloMap system, CardiometricsInc, Mountain View, California, USA) (fig 3).Coronary artery luminal diameters were deter-mined by computer assisted quantitative coro-nary angiography and amounted to 6-8 and6-6 mm in the proximal RCA and LAD,respectively. An estimate of coronary arteryflow rate was obtained by the following equa-tion:4

QD = (it x D2) x 0-25 x 0.5 x APVwhere QD = Doppler derived time-averageflow, D = vessel diameter, and APV = timeaverage of the spectral peak velocity.Accordingly, coronary flow rate was calculatedas 700 ml/min and 960 ml/min in the proximalLAD and RCA, respectively. Measurementsof cardiac output with the right ventricle topulmonary artery thermodilution bolus tech-nique gave an average of 4S8 I/min.The patient was discharged on an oral anti-

hypertensive (diltiazem), an antidiabetic(glibenclamide), aspirin, and a cholesterol-lowering drug (lovastatin). During three yearsof follow up no further clinical episodes ofmyocardial ischaemia or heart failureoccurred.

DiscussionMultiple coronary artery-left ventricular fistu-lae are an uncommon anomaly. According to arecent review, microfistulae originating fromall three major coronary arteries and drainingin the left ventricle have been reported in only16 cases.3 The pathogenetic origin of the mal-formation is obscure. Morphological studiessuggest a partial persistence of embryonicmyocardial sinusoids that arise from endo-thelial protrusions into the intertrabecularspaces.56 Fetal regression of these structuresresults in the formation of the Thebesian ves-sels of the adult heart. Thus, interference withdevelopmental changes might produce anabnormally prominent Thebesian system withthe morphological appearance of multiplecoronary microfistulae.Most patients in whom this anomaly is diag-

nosed present with typical or atypical anginapectoris in adult life. The clinical syndromehas been attributed to a coronary steal situa-tion due to the shunting of blood via the lowresistance fistulae.7 Surprisingly, ischaemicresponses during exercise stress testing andthallium scintigraphy were not unanimouslyobserved in the reported cases including the

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Multiple coronary artery-left ventricularfistulae

present one. In retrospect, the singular appear-ance of an acute coronary syndrome in ourpatient most probably resulted from a transi-tory thrombogenic transformation of the ath-erosclerotic plaque in the middle segment ofthe RCA. According to the literature, allpatients experienced their first anginal attacksduring advanced adulthood (older than 40years) despite the assumed congenital origin ofthe malformation. Therefore, the clinical andprognostic relevance of the suspectedischaemic syndrome in multiple coronaryartery-left ventricular fistulae appears to beuncertain.5Haemodynamic quantification of shunt flow

might provide further insight into the patho-physiology of this anomaly. In the presentcase, total flow through the left and right epi-cardial coronary artery was calculated as1660 ml/min by using a Doppler guidewiretechnique. In comparison, total myocardialblood flow in healthy adults has been deter-mined as 150-300 ml/min at rest8 Thus, totalleft ventricular output might be estimated asthe sum of shunt flow (1400 ml/min) plus sys-temic flow (4800 ml/min). Accordingly, left-to-left shunt flow would amount toapproximately 23% of total left ventricularoutput. It has been speculated that the consid-erable shunt volume draining into the left ven-tricle might result in diastolic overloadimitating the haemodynamic situation of aor-tic regurgitation.9 In contrast, the estimatedshunt fraction of 23% in our patient cannot beexpected to have any negative impact on left

ventricular function for it would be classifiedas being mild even in subjects with aorticregurgitation. Accordingly, no left ventricularenlargement or reduction in ejection fractionwere found. The clinical course of the patientwas completely uneventful before and after thefirst anginal episode that led to coronaryangiography and diagnosis. In view of thesehaemodynamic and clinical findings, the ques-tion remains whether this entity has anypathophysiological significance or has to beconsidered as a normal variation due to aprominent Thebesian system.5 10

1 Gillebert C, Van Hoof R, Van de Werf F, De Geest H.Coronary artery fistulas in an adult population. Eur HeartJ 1986;7:437-42.

2 Hobbs RE, Millit HD, Raghavan PV, Moodie DS, SheldonWC. Coronary artery fistulae: a 10-year review. ClevelandClin Q 1982;49:191-7.

3 Coussement P, De Geest H. Multiple coronary artery-leftventricular communications: an unusual prominentThebesian system. Acta Cardiologica 1994;49:165-73.

4 Coucette JW, Corl D, Payne HM, Flynn AE, Goto M,Nassi M, et al. Validation of a Doppler guide wire forintravascular measurement of coronary artery flow veloc-ity. Circulation 1992;85:1899-911.

5 Cha SD, Maranhao V, Goldberg H. Silent coronary artery-left ventricular fistula: a disorder of the Thebesian sys-tem? Angiology 1978;29:169-73.

6 Black IW, Loo CKC, Allan RM. Multiple coronary artery-left ventricular fistulae: clinical, angiographic, and patho-logic findings. Cathet Cardiovasc Diagn 1991;23:133-5.

7 Duckworth F, Mukharji J, Vetrovec GW. Diffuse coronaryartery to left ventricular communications: an unusualcause of demonstrable ischaemia. Cathet CardiovascDiagn 1987;13:133-7.

8 Simon R. Coronary circulation. In: Lentner C, ed. Geigyscientific tables, volume 5, heart and circulation. Basel: CibaGeigy Ltd, 1990:173-81.

9 Reddy K, Gupta M, Hamby RI. Multiple coronary arteri-oventricular fistulas. Am J Cardiol 1974;33:304-6.

10 Sheikhzadeh A, Stierle U, Langbehn AF, Thoran P,Diederich KW. Generalized coronary arterio-systemic(left ventricular) fistula. 7pn HeartJ7 1986;27:533-44.

SHORT CASES IN CARDIOLOGY

Intractable vasospastic angina

C Kurata, A Shimane

Department ofMedicine III,HamamatsuUniversity School ofMedicine,Hamamatsu, JapanC KurataA ShimaneCorrespondence to:Dr C Kurata, Department ofMedicine m, HamamatsuUniversity School ofMedicine, 3600 Handa-cho,Hamamatsu 431-31, Japan.Accepted for publication29 April 1997

A 48 year old woman with drug refractoryvasospastic angina had been in our hospital fornine months. Electrocardiograms recordedduring angina attacks showed marked eleva-tion of ST segments in the precordial, inferior,and/or lateral leads, often with ventriculararrhythmia or atrioventricular block (fig 1).Coronary angiography revealed no atheroscle-rotic stenosis, and intracoronary injection ofacetylcholine induced total occlusion of theleft anterior descending and right coronaryarteries despite continuation of high dose cal-cium antagonists (fig 2).

Calcium antagonists (nifedipine, nisoldip-ine, amlodipine, benidipine, diltiazem, andverapamil), nitrates (isosorbide dinitrate,isosorbide mononitrate, and nitroglycerin),

nicorandil or various combinations of drugscould not prevent her attacks. Blood concen-trations of drugs such as diltiazem and nico-randil were much higher than normaltherapeutic values. She did not have signs of ageneralised vasospastic disorder such asRaynaud's phenomenon nor a family historyof coronary artery disease or sudden death.The patient had stopped smoking after admis-sion. Her attacks occurred not only betweenmidnight and early morning but also in thedaytime. The trigger of attacks such as emo-tional distress, exercise, cold, alcohol, or cof-fee could not be specified. Her regularmenstrual cycle with normal variation of bloodoestrogen concentrations was not related tothe incidence of attacks.

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