ORIGINAL ARTICLE
Dutch survey of congenital coronary artery fistulas in adults
S.A.M. Said, T. van der Werf
Aims. This Dutch survey focused on the clinical presentation, noninvasive and invasive diagnostic methods, and treatment modalities of adult patients with congenital coronary artery fistulas
(CAFs).
Methods. Between 1996 and 2003, the initiative was taken to start a registry on congenital CAFs in adults. In total 71 patients from a diagnostic coronary angiographic population of 30,829 at 28 hospitals were collected from previously developed case report forms. Patient demographic data, clinical presentation, noninvasive and invasive techniques and treatment options were retrospectivdy collected and analysed. Results. Out of 71 patients with angiographically proven CAFs, 51 (72%) had 63 congenital solitary fistulas and 20 (28%) had 31 congenital coronaryventricular multiple microfistulas. Patients with pseudofistulas were exduded from the registry. Coronary angiograms were independently reanalwd for morphology and specific fistula details. The majority (72%) of the fistulas were unilateral, 24% were bilateral and only 4% were multilateral. The morphological characteristics of these 94 fistulas were as follows: the origin was multiple in 47% and single in 53%; the termination was multple in 52% and single in 48%; and the pathway of the fistulous vessels was tortuous/multiple in 66%, tortuous/single in 28%, straight/multiple in 3% and straight/single in 3%. Percutaneous translutminal embolisation (PTE) was performed in two (3%) patients; surgical ligation was undertaken in 13 (18%) patients. The overwhelming majority of the patients (56; 79%) were treated with conS.A.M. Said Departrnent of Cardiology, Regional Hospital, Twente Hospital Group, Hengelo, the Netherlands T. van der Wed Radboud University Medical Centre, N#megen, the Netherlands
Correspondence to: S.A.M. Said Geerdinksweg 141, 7555 DL Hengelo, the Netherlands E-mail: [email protected]
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servative medical management. The total mortality was 6% (4/71) at a mean follow-up period of approximately five years. Cardiac mortality accounted for 4% (3/71); in all three patients, death could possibly be attributed to the presence of the fistula. Conclusion. Registry of congenital coronary artery fistulas in adults in the Netherlands is feasible. In spite of restrictions imposed by the Dutch Privacy Law, it was possible to include 71 adult patients with congenital coronary artery fistulas who were eligible for thorough evaluation. (Neth Heart J
2006;14:5-13.)
Keywords: coronary artery, fistula, survey, adults, coronary arteriography, morphology
fongenital coronary artery fistulas (CAFs) in adults Ware infrequent anomalies. They have been reported by several authors since the first description by Krause some 140 years ago.' CAFs are considered to belong to the group of congenital coronary artery anomalies of termination associated with serious cardiac complications such as angina pectoris, myocardial infarction, congestive heart failure, ventricular and supraventricular arrhythmias, syncope and sudden death.2 According to large angiographic series, CAFs are found in 0.1 to 0.2% of the patients.2'3 A recent survey estimated the cumulative prevalence of CAFs in the Netherlands at 0.22%.4
Angiographically, two major types of fistulas are recognised: solitary coronary artery fistulas and coronary-ventricular multiple microfistulas. They are infrequent coincidental angiographic findings that represent the most common haemodynamically significant congenital coronary artery anomalies. The Dutch survey focuses on adult patients with congenital CAFs, diagnosed by coronary angiographic examination. Twenty-eight catheterisation laboratories contributed to the Dutch survey from 1996 to 2003. Only patients with congenital solitary CAFs or multiple coronary-ventricular multiple microfistulas were included in this report. 5
Dutch survey of congenital coronary artery fistulas in adults
For further details and fistula characteristics, coronary angiographic studies (CAG) were independently reevaluated. Morphological characteristics ofthe different components (origin, pathway and termination) ofthe angiographically detected congenital CAFs are described in detail.
ly for the study of fistula description (singularity, multiplicity, tortuosity of origin/termination/pathway, diameter of fistula-related artery and aneurysmal
Methods
Solitary CAFs Solitary CAFs are defined as an abnormal direct communication between a coronary artery and a cardiac cavity or any part of the pulmonary or systemic circulation where there is an increase in the diameter of the vessel or cardiac chamber receiving the fistulous communication associated with volume overload. The origin, termination and pathway may be identified. These may have congenital or acquired aetiology.5 7
Patients Between 1996 and 2003, details of 71 adult patients out of a diagnostic coronary angiographic population of 30,829 at 28 hospitals were acquired from previously developed case report forms. Patient demographic data, clinical presentation, noninvasive and invasive techniques used and treatment choices were retrospectively collected and analysed. The angiograms were independently reviewed for further details of fistula morphology. The extent of coronary artery disease was recorded. A blood vessel with a high-grade stenosis was considered significantly diseased. Recorded patient characteristics included age, gender and comorbidity. Also recorded were the results ofthe physical examination, noninvasive diagnostic methods and whether a left or right heart catheterisation with shunt assessment was performed. Also noted was information on whether or not the patient had had a penetrating or nonpenetrating chest injury and previous valve or coronary artery bypass surgery. Clinical data were collected retrospectively and angiograms were reviewed and reanalysed independent-
formation). Definitions
Coronary-ventricular mulfiple microfistulas These are anomalies described as an abnormal communication between coronary arteries and a cardiac chamber, characterised by multiple fistulas of small calibre opacifying the ventricular cavities, mostly left ventricle (LV). The origin, termination and pathway may be visualised. The fistulas are mainly congenital in origin, and are also known as generalised myocardial microfistulas.8 Pseudofistulas Pseudofistulas are recognised by the rapid passage of dye from an artery to the corresponding draining venous area, after inadvertent superselective injection .~~~~~~~~~~~~~~~~~~~~~!
Figure 1. Pseudofltuas. Pseudofistula of the rigbt coronary artery (RCA) in kft anterior oblique (LAO) projections: A) Injection of contrast through a 5-Frencb Judkins coronary cathetr. Paralkl to the main RCA (large arrow) a small branch (small arrow) can be identified whichfilkd very rapidly and drained into the coronary sinus/right atrium (open arrow). There is no specific origin, pathway or termination. B) Demonstrates the same normal RCA (large arrow). In the proximal segment of the vessel a small side branch can be seen (small arrow). It was this branch that was inadvertently supersekctively entered by the 5-French cathetr.
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1/
_4
J9
Em.F.,_ . l _
Figure 2. Congenital solitaty CAFs. Angiographic frames ofproximally located fistulas (LAD --+P, double arrows) demonstrating the different morphological components (origin, termination and pathway) of congenital solitary CAPs: A) Right aniteior oblique (RAO) view, ori'gin is multiple, termination is multiple and pathway is tortuous/multiple; B) Left lateral view, origin is single termination is single and pathway is tortwuoussingle; C) RAO view, origin is single, termination is single and pathway is tortuous/multiple with an aneutysm D) RAO view, origin is multiple, termination is single and pathway is tortuous/multiple; Persistence offistulousfiow (double arw)during left ventriculogram in RAO0 view, in both E) systole andF) diastole; G) RCA angiogram in LAO0 view, showing elongatied; dilated and serpentine RCA fistulating (double arrows) into the RV, origin is multiple, termnination is multiple and pathway is tortuusmultiple; H) Angulated LAO projection of RCA, depicting afistulous vessel (double arrows) originatingfrom a side branch of the RCA draining into the PA,, origin is single, termination is single and pathway is tortuous/single. into a side branch or the main coronary artery itself, subsequent to forceful injection of a large amount of contrast, causing prsure damping and/or arrhyxthmias, without the recognition of origin, termination and pathway of an abnormal fistulous communication.9
Aneurysm An aneurysm is angiographically defined as a coronary artery dilatation that exceeds the diameter of the normal adjacent segments or the diameter of the patient's largest coronary vessel by a factor of 1. 5. 10 Resufts Patients were recruited for the Dutch survey on congenital CAFs in adults from a databank of 28 catheterisation laboratories. Participating hospitals represented both academic (7%) and nonacademic (93%) centres (appendix 1). Patients with pseudofistulas (n=10) (figures LA and B)9 were identified and excluded from the registry and from firther analysis. of27 patients with angiographically proven CAFs, Out 5 1 (72%) had congenital solitary (figures 2A to H) and 20 (28%) had congenital coronary-ventricular multiple microfistulas (figure 3). The clinical and angiographic data of these 71 patients were collected from 30,829 subjects who
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Figure 3. Congenital coronaty artery left ventricular multiple microfistulas Angiographic frame of a distally located fistula in RAO view illustrating the morphological aspects of a coronarye ventricular multiple microfistula (doub arrows) originating from the dilated LAD (single arrow) and terminating into the LV. Theorigin is multiple, termination is multiple and pathway is multipe(.
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Dutch survey of congenital coronary artery fistulas in adults
Table 1. Noninvasive techniques used in patients in the Dutch registry (n=71) with 94 fistulas. Characteristics
Solitary (n=51)
Multiple (n=20)
Mean age (years) (range) Gender Age distribution range - 20-50 - 51-70 ->70
60 (range 29-85) 33 M (65%), 18 F (35%)
67 (range 49-82) 7 M (35%), 13 F (65%)
16 (31%) (11 M, 5 F) 21 (41%) (12 M, 9 F) 14 (28%) (10 M, 4 F)
1 (5%) (1 F) 9 (45%) (3 M, 6 F) 10 (50%) (4 M, 6 F)
25 (49%) 11 (22%) 13 (25%) 1 (2%) 26 (51%)
7
(35%)
7
(35%)
22 (43%) 29 (57%)
5 (25%) 15 (75%)
47 (92%) 4 (8%) 8 (16%) 1 (2%) 7 (14%) 36 (71%) 8 (16%) 28 (55%) 20 5 3
19 (95%) 1 (5%) 5 (25%) 3 (15%) 2 (10%) 11 (55%) 3 (15%) 8 (40%) 6
Noninvasive techniques Cardiac murmur - Continuous - Systolic - Diastolic No audible cardiac murmur ECG - Normal - Pathological* Rhythm - Sinus rhythm - Atrial fibrillation Myocardial infarction - Ipsilateral - Contralateral TTE - Normal - Abnormal - Mild valvular disease - Severe valvular disease - Wall motion abnormalities - LVH ETT - Normal - Positive for coronary insufficiency MIBI scintigraphy - Normal - Positive for coronary insufficiency
13 (65%)
1 1 7 (35%) 5 (25%) 2 (10%) 6 (30%) 3 (15%) 3 (15%)
32 (63%) 23 (45%) 9 (18%) 20 (39%) 9 (18%) 11 (21%)
M=male, F=female, TTE=transthoracic echocardiography, LVH=leIt ventricular hypertrophy, ETI=exercise tolerance test. *Pathological ECG=ST-T segment abnormalities, pathological Q waves, LVH and healed Ml.
underwent coronary angiographic examination in Dutch centres between 1996 and 2003. Angiograms were recorded on various media (35 mm films, super VHS video tapes and compact disks). In some hospitals Dutch Privacy Law regulations restricted the inclusion of more patients. The data of the Dutch registry on CAFs has been described earlier in detail: clinical data and coronary angiographic findings of 51 patientswith 63 congenital solitary CAFs (figure 4A)" and 20 subjects with 31 coronary artery-left ventricular multiple microfistulas (figure 4B)'2 were included. The characteristic findings of both patient groups are presented in tables 1 and 2.
8
Suspected coronary artery disease was the most frequent indication for performing angiography (62%), followed by analysis of heart murmur (13%) and congestive heart failure (6%) (table 3). Clinical presentation Angina pectoris occurred in 41 (58%) patients. Of these, 22 (31%) had significant atherosclerotic coronary artery disease and 19 (27%) showed a normal coronary arterial tree (table 4). Myocardial infarction (MI) occurred in 13 (18%) patients. The MI was located on the same side of the shunt in four (6%) and on the contralateral side in nine (12%) of these patients.
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Table 2. Angiographic features and fistula characteristics of patients in the Dutch registry (n=71) with 94 fistulas. InvasIve methods
Solitary (n=51)
Multiple (n=20)
Description of CAFs Unilateral Bilateral Multilateral
41(80%) 8 (16%) 2 (4%)
10 (50%) 9 (45%)
63 (67%)
31 (33%)
17 (27%) 46 (73%) 29 21 11 2 Right 93%, left 7% 21 (33%) 42 (67%) 46 (73%) 1 (2%) 3 (5%) 4 (6%)
27 (87%) 4 (13%) 16 9 6
Fistula characterstIcs Origin - Multiple - Single LAD RCA -
-
-
Cx
LMCA Termination (right or left heart) - Multiple - Single - PA - LV -RA
-
- SCV
RV -LA - RVOT
Left 100% 28 (90%) 3 (10%) 20x
3(5%) 3(5%)
-
-
1(5%)
1 (2%) 2(3%)
CS
Pathway Tortuous/multiple
56% (35 fistulas) 41% (26 fistulas) None 3% (2 fistulas) 29% of fistulas (35% of patients) 9% 3%
-
-
Tortuous/single
-
Straight/multiple
Straight/single - Aneurysmal formation - Dilatation of fistula-related artery - CalcIfication -
87% (27 fistulas) 10% (3 fistulas) 3% (1 fistula) 3% (5% of patients)
CAF=coronary artery fistula, CS=coronary sinus, Cx=circumflex coronary artery, LA=left atrium, LAD=left anteror descending coronary artery, LCA=left coronary artery, LMCA=left main coronary artery, LV=left ventricle, PA=pulmonary artery, RA=right atrum, RCA=nght coronary artery, RV=right ventricle, RVOT=right ventricular outflow tract, SCV=superior caval vein.
Tabe 3. Indications for cathetersation in 71 patients in the Dutch registry with congenital CAFs.
Indication Assessment of suspected coronary heart disease Analysis of cardiac murmur and presence of echocardiographic turbulent flow Dyspnoea and congestive heart failure Risk stratification postinfarction Evaluation of valvular heart disease Ventricular and supraventricular arrhythmias Preoperative assessment for noncardiac surgery Pathological rest ECG Prior to lung transplantation Atypical chest pain Congenital heart disease
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No of cases 44 9 4 3 2 2 2 2 1 1 1
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RCA I
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B
A
Figure 4. Origin offistulas in the Dutch registry ofcongenital CAFs. A. In solitary CAPFs: Coronary arterial tree demonstrating the origin ofthefistulous vessels in 51 patients with 63 congenital solitary CAFs in adults; B. Coronary arterial tree illustrating the origin ofthefistous vessels in 20 patients with 31 congenital coronary artery-left ventricular multiple microfistulas in adults.
During a follow-up period of approximately five years there was no recurrence. One patient (1%) in the present cohort developed infective endocarditis. Initially, he was medically treated with an intravenous antibiotic and the fistula was later surgically ligated. Noninvasive diagnostic methods Some aspects of the noninvasive methods used are described below and further details are summarised in table 1. Sinus rhythm was predominant in 66 (93%) patients and atrial fibrillation was present in only five (7%) patients. Exercise tolerance test (ETT) was performed in 39 (55%) ofthe 71 patients ofthe Dutch contingent. The results were judged to be normal in 28 subjects (39%) and positive for myocardial ischaemia in 11 (16%). Exercise Sesta-MIBI scintigraphy was performed in 26 (36%) patients. The scan was normal in 12 (17%) of these patients, and positive, showing reversible myocardial defects, in 14 (19%). A total of 47 (66%) patients underwent transthoracic echocardiography (TTE). Normal findings were reported in 11 (15%) and abnormalities were reported in 36 (51%) ofthese patients. Mild and severe valvular heart disease were present in 26 (37%) and five (7%) patients, respectively. Furthermore, wall motion abnormality and left ventricular hypertrophy
Table 4. Presence or absence of angina pectoris with or without significant coronary artery disease (CAD). Angina pectoris No angina pectoris Total
10
Patients 41 30 71
CAD 22 18 40
No CAD 19 12 31
were seen in five (7%) patients. Cardiovascular magnetic resonance (CMR) was performed in 13 patients, as part of a feasibility study on solitary congenital CAFs.
After initial blind review, CMRconfimned the diagnosis in ten patients. In two patients the diagnosis was initially overlooked, but visualised in a second inspection. In one patient (fistula diameter 1 mm), the fistula was not visualised by CMR The fistulas could, after a second revision, be detected by CMR in 92% of the patients.13 Invasive diagnostic methods Invasive diagnostic methods are summarised in table 2. Coronary arteriography (CAG) was assumed to be the gold standard. Morphology of the fistulas Dilatation ofthe fistula-related artery was demonstrated in five (7%) patients and aneurysmal formation of the fistulous vessel in 19 (27%) patients. Further analysis ofthe angiograms ofthe 71 patients with a total of 94 fistulas demonstrated six straight pathways (6%) in five patients (7%) with a mean age of 53 years (range 30 to 70, three females and two males) with two bilateral and three unilateral fistulas, respectively. In the current study, pathways were found to be tortuous (94%) in the majority of the patients (93%). We found only six straight pathways (6%) in five patients (7%) with a mean age of 53 years (range 30 to 70), with two bilateral and three unilateral fistulas. Coronary artery disease No significant atherosclerotic coronary artery disease (CAD) was found in 42 patients (59%). Single, dual and triple vessel disease was present in 18 (25%), five (7%) and six (9%), respectively.
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Figure 5. Materiak used for transcatheter permanent embolic occlusion ofsolitary CAFs. A. Tbrombogenic polyvinyl alcohol foam, B. Detachable silicone balloons, C Microcoils, D. Gianturco-Grifta vascular occlusion device (GGVOD), E. Amplatzer duct occluder.
Treatment
Ofthe treatment modalities, percutaneous transluminal embolisation (PTE) was performed in two (3%) patients and surgical ligation (SL) was undertaken in 13 (18%) patients. The overwhelming majority of the patients (56; 79%) were treated with conservative medical management (CMM). Of those, six patients were under close clinical follow-up (watchful waiting) and 50 patients were treated pharmacologically. Betablockers (38 patients), calcium-channel blockers (16 patients), oral anticoagulation/antiplatelet (29 patients), angiotensin-converting enzyme inhibitors (8 patients), angiotensin-receptor blockers (3 patients), statins (15 patients), diuretics (10 patients), short and long-acting nitrates (21 patients), antiarrhythmic drugs (3 patients) and antidiabetic therapy (3 patients) were among the medications used. Follow-up and mortality During a follow-up period of approximately five years there was no recurrence of myocardial infarction in any ofthe patients. The total mortality was 6% (4/71) and cardiac mortality accounted for three of the four deaths. Two patients died of terminal heart failure. They both had cardiomegaly on chest X-ray and one had permanent atrial fibrillation. The third patient died suddenly at home. He had documented paroxysmal atrial fibrillation and a normal coronary arterial tree. In all three patients (age >72 years, two females and one male) death could possibly have been related to the presence of the fistula. None of the patients demonstrated aneurysmal formation of the fistulous pathways.
Discussion This is a report on the first survey on congenital CAFs in adults in the Netherlands, for the purpose of creating a registry. In the Dutch registry, suspected coronary artery disease was the primary indication for diagnostic angiography in 62% ofall cases with either solitary or multiple microfistulas. All 71 patients were collected from a total of 30,829 diagnostic coronary arteriography cohorts at 28 Dutch catheterisation suits. Population data for comparison from other countries is scarce. Urratia-S et al. reported that congenital CAFs were found in approximately one of every 50,000 patients Netherlands Heart Journal, Volume 14, Nulmber 1, January 2006
with congenital heart disease and in one of every 500 patients who undergo coronary angiographic examination.'4 It has been reported that multiple CAFs (bilateral, multilateral or both) were identified in 16% of the patients and fusiform aneurysmal formation in 19%.'4 Recently saccular aneurysm was reported by Kinoshita et al."5 In the present registry, we found that bilateral and multilateral fistulas were detected in 24 and 4% of the patients, respectively (together they may both be classified as multiple CAFs). Furthermore, aneurysmal formation was found in 27% of the patients and dilatation of the fistula-related coronary artery was recognised in 7% of the patients. Tortuosity and multiplicity of the pathway has an inverse relationship to the PTE therapeutic option, since accessibility and safe cannulation for percutaneous closure of the fistula may be limited.'6 For symptomatic patients with a sizeable left-toright shunt, invasive therapeutic options (surgical ligation and percutaneous transluminal embolisation) rely heavily on the morphological appearance and characteristics ofthe fistulas. Multilaterality, multiplicity and complexity of the fistulous tract determine the choice of the appropriate invasive therapeutic option. Surgical ligation and percutaneous closure (figure 5) are the two modalities currently available for permanent fistula abolition. Surgical ligation is indicated if multilaterality, multiplicity or extreme tortuosity ofthe components (origin, pathway and termination) is present with or without aneurysmal formation. On the other hand, PTE is chosen in the case of proximal localisation of the fistula or for patients of advanced age. For symptomatic patients, with a large-sized left-toright shunt, surgery or percutaneous catheter-based closure of CAFs is generally accepted. Coil embolisation is reserved for patients who satisfy the following criteria: safe accessibility to the fistula-related coronary artery, a single narrow drainage site, absence of large side branch, and absence of multiple fistulas. On the other hand, surgical ligation could be performed for large fistulas characterised by high fistulous flow, multiple origins or terminations, extreme tortuous pathways, and aneurysmal formation.'7 This retrospective survey revealed that surgical ligation accounted for 18% and percutaneous trans11
Dutch survey of congenital coronary artery fistulas in adults
luminal embolisation for only (3%) of the patients, possibly suggesting the need for longer learning curves for the latter therapeutic procedure. Because the morphological appearance of the CAFs and the choice of appropriate invasive therapeutic option is closely linked, in this study, the part played by PTE was very modest. This might be due to the presence of a high percentage of tortuosity of the different fistula components. The diagnosis of CAFs requires a high index of scepticism and alertness while taking the history and performing the physical examination. Nonspecific presentations are common, although dramatic presentations such as myocardial infarction or sudden death may occur. Typically the results of the physical examination, ECG and the chest X-ray film are normal except for sizeable coronary artery fistulas. In a substudy of 13 patients with congenital solitary CAFs, CMR demonstrated the feasibility of fistula detection. CMR confirmed the angiographic findings revealing the fistulous communications in 12 of 13 patients. In addition, the fistula characteristics were recognised in most of these patients. It has been previously reported that magnetic resonance imaging (MRI) could demonstrate the fistulous communications and their anatomy.'8"19 It should also be pointed out that CMRoffers the advantage of not requiring the injection of contrast materials and that there is excellent contrast between flowing blood and adjacent cardiovascular structures. Cine-CMR clearly delineates the relation between the fistulous jet and the cardiac valves. Sato et al. reported the detection of solitary CAFs by multislice CT scan.20 In the case ofthe current survey all data had been acquired prior to the availability of advanced multislice computed tomography. Increased availability of this diagnostic method may facilitate noninvasive detection and determination of CAFs. Although coronary-ventricular multiple microfistulous communications are rare,2' some cases of symptomatic female patients were reported in the 1980s by Martens et al.,22 and Vachon,23 and also recently by Duran et al.8 The typical patient with coronary artery-left ventricular multiple microfistulas is a female ofaround 50 years of age presenting with angina pectoris, without audible cardiac murmur, and with inconclusive noninvasive diagnostic tests. This invariably leads to investigation of suspected obstructive coronary artery disease. The diffuse character of the microfistulous communications renders them neither amenable for PTE or for surgical ligation, but instead requires conservative medical treatment. On the other hand, from a therapeutic point of view, solitary CAFs with multiple communications have recently been successfully treated by surgical closure.24 In addition, PTE has been successfully performed, after good cannulation, in patients with narrow and nonbranching fistulous communications.2' 12
Our current series, reporting on an adult population of 20 patients with congenital coronary-ventricular multiple microfistulas and 51 patients with congenital solitary CAFs, is the largest known to us. A prospective study with a stringent protocol is warranted with a larger number ofpatients, perhaps European-wide, in order to clarify several aspects of these infrequent congenital entities (solitary CAFs and coronary arteryleft ventricular multiple microfistulas) in adult patients. Limitations The limitations of this study are those inherent to observational surveys involving retrospective data collection. Lack of some data, as commonly encountered in a registry format, was a common finding. U
Acknowledgment We are indebted to our colleagues (appendix 1) who provided the patient data which formed the solid base for this paper. On www.cardiologie.nl you will find an example of the national registration form for coronary artery fistula in adult population in the Netherlands. References 1 2
3 4
5 6 7 8
9 10 11 12
13
14
Krause W. Uber den Ursprung einer akzessorischen A. Coronaria aus der A. Pulmonalis. ZRationell Med 1865;24:225-7. Yamanaka 0, Hobbs RE. Coronary artery anomalies in 126,595 patients undergoing coronary arteriography. Cathet Cardiovasc Diagn 1990;21:28-40. Gilebert C, van Hoof R, van de Werf F, Piessens J, De Geest H. Coronary artery fistula in an adult population. Eur Heart J
1986;7:437-43. Said SAM, Relik-van Wely L, van der Werf T. Angiographically diagnosed congenital coronary artery fistulas in an adult population. Cardiologie 1998;2:71-3. Levin DC. Anomalies and anatomic variations ofthe coronary arteries. In: Abrams HL, editor. Coronary arteriopathy. A practical approach. Little, Brown Co, Boston, 1983; pp 283-99. Angeini P. Coronary-to-pulmonary fistulae: What are they? What are their causes? What are their functional consequences? TexHeart InstJ2000;27:327-9. Angelini P. Coronary artery anomalies-current clinical issues. Definitions, classification, incidence, clinical relevance, and treatment guidelines. Tex Heart InstJ2002;29:271-8. Duran DA, Michelis V, Diaz P, Lujambio M, Kuster F, Lluberas R, et al. Evaluacion de pacientes portadores de fistulas coronarioventriculares multiples. Rev Med Uruguay 2003;19:237-41. Said SAM, Crijns HJGM, van der Werf T. Prerequisite conditions for identification of true coronary artery fistula. Cardiologie 2000;7:379-83. Aintablian A, Hamby RI, Hoffman I, Kramer RJ. Coronary ectasia: Incidence and results of coronary bypass surgery. Am Heart J 1978;96:309-15. Said SAM, van derWerfT. Dutch survey ofcoronary artery fistulas in adults: Congenital solitary fistulas. Int J Cardiol [in press]. Said SAM, van derWerfT. Dutch survey ofcoronary artery fistulas in adults: Congenital coronary artery-left ventricular multiple micro-fistulas. Int J Cardiol [in press]. Said SAM, Hofman MBM, BeekAM, van derWerfT, van Rossum AC. Feasibility of cardiovascular magnetic resonance imaging of angiographically diagnosed congenital solitary coronary artery fistulas [Submitted]. Urrutia-S CO, Falaschi G, Ott DA, Cooley DA. Surgical management of 56 patients with congenital coronary artery fistulas. Ann Thorac Surg 1983;35:300-7.
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15 Kinoshita 0, Ogiwara F, Hanaoka T, Tomita T, Yokozeki 0, Kai R, et al. Large saccular aneurysm in a coronary arterial fistula. A case report. Angiology2005;56:233-5. 16 Hsieh KS, Huang TC, Lee CL. Coronary artery fistulas in neonates, infants, and children: Clinical findings and outcome. Pediatr CardioI2002;23:415-9. 17 Mavroudis C, Backer CL, Rocchini AP, Muster AJ, Gevitz M. Coronary artery fistulas in infants and children: a surgical review and discussion of coil embolization. Ann Thorac Surg 1997;63: 1235-42. 18 Bolognesi R, Tsialtas D, Barbaresi F, Manga C. Single coronaryright ventricular fistula with a partially thrombosed large aneurysm of its proximal tract in a 66-year-old man. Eur HeartJ 1994;15: 1720-4. 19 Parga JR, Ikari NM, Bustamante LNP, Rochitte CE, De Avila LF, Oliveira SA. MRI evaluation of congenital coronary artery fistulae. BrJRadiol2004;77:508-1 1. 20 Sato Y, Mitsui M, Takahashi H, Miyazawa T, Okabe H, Inoue F, et al. A giant left circumflex coronary artery-right atrium arteriovenous fistula detected by multislice spiral computed tomography. Heart Vessels2004;19:55-6.
21 Chia BL, Chan ALK, Tan LKA, Ng RAL, Chiang SP. Coronary artery-left ventricular fistula. Cardiology 1981;68:167-79. 22 Martens J, Haseldoncks C, van de Werf F, De Geest H. Silent left and right coronary artery-left ventricular fistulas: An unusual prominent thebesian system. Acta Cardiologica 1983;38:139-42. 23 Vachon JM. Les fistules congenitales coronaro-ventriculaires gauches. Revue de la litterature. Ann de Cardiologie et D' angeiologie 1983;32:21-5. 24 Muller D, Wimmer-Greinecker G, Fichtescherer S, Moritz A. Symptomatic coronary artery-pulmonary artery fistulae. IJTCVS 2004;20:192-3. 25 Boccalandro F, Awadalla H, Smalling RW. Percutaneous transcatheter coil embolization of two coronary fistulas originating from the left main ostium and left anterior descending artery. Cath Cardiovasc Intervent2002;57:221-3.
Appendix 1: Participating Dutch centres J.O.J. Peels, Alkmaar Medical Centre RJ. Lionarons, B.J.L. de Rode, Twente Hospital, location Almelo W.L. Mosterd, Eemland Hospital, Amersfoort C.C. de Cock, VU University Hospital Medical Centre, Amsterdam W.G. de Voogt, R-G.E.J. Groutars, B. Ilmer, A.R. Wlllems, J. Visser, St Lucas-Andreas Hospital, Amsterdam J.KM. Peters, Maas Hospital, Boxmeer B.J. van den Berg, IJsselland Hospital, Capelle a/d IJssel F.C.W. Tietge, Deventer Hospitals, Deventer L. Relik-van Wely, C. de Nooyer, Diaconessenhuis, Eindhoven E.M.C.J. Wajon, J.C. Poortermans, J.W. Louwerenburg, Medical Spectrum Twente Hospital, Enschede W.A.A.J. van Ekelen, P.E. Polak, St Anna Hospital, Geldrop J.A.J. de Boo, A.H. Liem, H.W.O. Roeters van Lennep, Oosterschelde Hospital, Goes B. van Vlies, A.J. Funke Kiipper, Kennemer Gasthuis, Haarlem K Dijkgraaf, St Jansdal Hospital, Harderwijk M.F. Scholten, RR Lalisang, Elkerliek Hospital, Helmond A. Derks, H.T. Droste, J.H. Fast, Twente Hospital Group, location Hengelo C.G.K.M. Fauser, Bethesda Hospital, Hoogeveen C.J. de Vries, Leeuwarden Medical Centre, Leeuwarden A.G. Boehmer, Atrium Medical Centre, Kerkrade L.H.R Bouwels, Canisius Wilhelmina Hospital, Nijmegen T. van der Werf, J. Timmermans, Radboud University Medical Centre, Nijmegen D.G. de Waal, Waterland Hospital, Purmerend G.J. de Weerd, Maasland Hospital, Sittard D.E.P. de Waard, Antonius Hospital Sneek C.L.J.M. van Engelen, De Heel Hospital, Zaans Medical Centre, Zaandam A.C. Tans, Het Spittaal Hospital, Zutphen W.J. Louridtz, H.A. Oude Luttikhuis, Isala Hospital, Zwolle
Dq
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Dutch survey of congenital coronary artery fistulas in adults
S.A.M. Said, T. van der Werf
Aims. This Dutch survey focused on the clinical presentation, noninvasive and invasive diagnostic methods, and treatment modalities of adult patients with congenital coronary artery fistulas
(CAFs).
Methods. Between 1996 and 2003, the initiative was taken to start a registry on congenital CAFs in adults. In total 71 patients from a diagnostic coronary angiographic population of 30,829 at 28 hospitals were collected from previously developed case report forms. Patient demographic data, clinical presentation, noninvasive and invasive techniques and treatment options were retrospectivdy collected and analysed. Results. Out of 71 patients with angiographically proven CAFs, 51 (72%) had 63 congenital solitary fistulas and 20 (28%) had 31 congenital coronaryventricular multiple microfistulas. Patients with pseudofistulas were exduded from the registry. Coronary angiograms were independently reanalwd for morphology and specific fistula details. The majority (72%) of the fistulas were unilateral, 24% were bilateral and only 4% were multilateral. The morphological characteristics of these 94 fistulas were as follows: the origin was multiple in 47% and single in 53%; the termination was multple in 52% and single in 48%; and the pathway of the fistulous vessels was tortuous/multiple in 66%, tortuous/single in 28%, straight/multiple in 3% and straight/single in 3%. Percutaneous translutminal embolisation (PTE) was performed in two (3%) patients; surgical ligation was undertaken in 13 (18%) patients. The overwhelming majority of the patients (56; 79%) were treated with conS.A.M. Said Departrnent of Cardiology, Regional Hospital, Twente Hospital Group, Hengelo, the Netherlands T. van der Wed Radboud University Medical Centre, N#megen, the Netherlands
Correspondence to: S.A.M. Said Geerdinksweg 141, 7555 DL Hengelo, the Netherlands E-mail: [email protected]
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servative medical management. The total mortality was 6% (4/71) at a mean follow-up period of approximately five years. Cardiac mortality accounted for 4% (3/71); in all three patients, death could possibly be attributed to the presence of the fistula. Conclusion. Registry of congenital coronary artery fistulas in adults in the Netherlands is feasible. In spite of restrictions imposed by the Dutch Privacy Law, it was possible to include 71 adult patients with congenital coronary artery fistulas who were eligible for thorough evaluation. (Neth Heart J
2006;14:5-13.)
Keywords: coronary artery, fistula, survey, adults, coronary arteriography, morphology
fongenital coronary artery fistulas (CAFs) in adults Ware infrequent anomalies. They have been reported by several authors since the first description by Krause some 140 years ago.' CAFs are considered to belong to the group of congenital coronary artery anomalies of termination associated with serious cardiac complications such as angina pectoris, myocardial infarction, congestive heart failure, ventricular and supraventricular arrhythmias, syncope and sudden death.2 According to large angiographic series, CAFs are found in 0.1 to 0.2% of the patients.2'3 A recent survey estimated the cumulative prevalence of CAFs in the Netherlands at 0.22%.4
Angiographically, two major types of fistulas are recognised: solitary coronary artery fistulas and coronary-ventricular multiple microfistulas. They are infrequent coincidental angiographic findings that represent the most common haemodynamically significant congenital coronary artery anomalies. The Dutch survey focuses on adult patients with congenital CAFs, diagnosed by coronary angiographic examination. Twenty-eight catheterisation laboratories contributed to the Dutch survey from 1996 to 2003. Only patients with congenital solitary CAFs or multiple coronary-ventricular multiple microfistulas were included in this report. 5
Dutch survey of congenital coronary artery fistulas in adults
For further details and fistula characteristics, coronary angiographic studies (CAG) were independently reevaluated. Morphological characteristics ofthe different components (origin, pathway and termination) ofthe angiographically detected congenital CAFs are described in detail.
ly for the study of fistula description (singularity, multiplicity, tortuosity of origin/termination/pathway, diameter of fistula-related artery and aneurysmal
Methods
Solitary CAFs Solitary CAFs are defined as an abnormal direct communication between a coronary artery and a cardiac cavity or any part of the pulmonary or systemic circulation where there is an increase in the diameter of the vessel or cardiac chamber receiving the fistulous communication associated with volume overload. The origin, termination and pathway may be identified. These may have congenital or acquired aetiology.5 7
Patients Between 1996 and 2003, details of 71 adult patients out of a diagnostic coronary angiographic population of 30,829 at 28 hospitals were acquired from previously developed case report forms. Patient demographic data, clinical presentation, noninvasive and invasive techniques used and treatment choices were retrospectively collected and analysed. The angiograms were independently reviewed for further details of fistula morphology. The extent of coronary artery disease was recorded. A blood vessel with a high-grade stenosis was considered significantly diseased. Recorded patient characteristics included age, gender and comorbidity. Also recorded were the results ofthe physical examination, noninvasive diagnostic methods and whether a left or right heart catheterisation with shunt assessment was performed. Also noted was information on whether or not the patient had had a penetrating or nonpenetrating chest injury and previous valve or coronary artery bypass surgery. Clinical data were collected retrospectively and angiograms were reviewed and reanalysed independent-
formation). Definitions
Coronary-ventricular mulfiple microfistulas These are anomalies described as an abnormal communication between coronary arteries and a cardiac chamber, characterised by multiple fistulas of small calibre opacifying the ventricular cavities, mostly left ventricle (LV). The origin, termination and pathway may be visualised. The fistulas are mainly congenital in origin, and are also known as generalised myocardial microfistulas.8 Pseudofistulas Pseudofistulas are recognised by the rapid passage of dye from an artery to the corresponding draining venous area, after inadvertent superselective injection .~~~~~~~~~~~~~~~~~~~~~!
Figure 1. Pseudofltuas. Pseudofistula of the rigbt coronary artery (RCA) in kft anterior oblique (LAO) projections: A) Injection of contrast through a 5-Frencb Judkins coronary cathetr. Paralkl to the main RCA (large arrow) a small branch (small arrow) can be identified whichfilkd very rapidly and drained into the coronary sinus/right atrium (open arrow). There is no specific origin, pathway or termination. B) Demonstrates the same normal RCA (large arrow). In the proximal segment of the vessel a small side branch can be seen (small arrow). It was this branch that was inadvertently supersekctively entered by the 5-French cathetr.
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1/
_4
J9
Em.F.,_ . l _
Figure 2. Congenital solitaty CAFs. Angiographic frames ofproximally located fistulas (LAD --+P, double arrows) demonstrating the different morphological components (origin, termination and pathway) of congenital solitary CAPs: A) Right aniteior oblique (RAO) view, ori'gin is multiple, termination is multiple and pathway is tortuous/multiple; B) Left lateral view, origin is single termination is single and pathway is tortwuoussingle; C) RAO view, origin is single, termination is single and pathway is tortuous/multiple with an aneutysm D) RAO view, origin is multiple, termination is single and pathway is tortuous/multiple; Persistence offistulousfiow (double arw)during left ventriculogram in RAO0 view, in both E) systole andF) diastole; G) RCA angiogram in LAO0 view, showing elongatied; dilated and serpentine RCA fistulating (double arrows) into the RV, origin is multiple, termnination is multiple and pathway is tortuusmultiple; H) Angulated LAO projection of RCA, depicting afistulous vessel (double arrows) originatingfrom a side branch of the RCA draining into the PA,, origin is single, termination is single and pathway is tortuous/single. into a side branch or the main coronary artery itself, subsequent to forceful injection of a large amount of contrast, causing prsure damping and/or arrhyxthmias, without the recognition of origin, termination and pathway of an abnormal fistulous communication.9
Aneurysm An aneurysm is angiographically defined as a coronary artery dilatation that exceeds the diameter of the normal adjacent segments or the diameter of the patient's largest coronary vessel by a factor of 1. 5. 10 Resufts Patients were recruited for the Dutch survey on congenital CAFs in adults from a databank of 28 catheterisation laboratories. Participating hospitals represented both academic (7%) and nonacademic (93%) centres (appendix 1). Patients with pseudofistulas (n=10) (figures LA and B)9 were identified and excluded from the registry and from firther analysis. of27 patients with angiographically proven CAFs, Out 5 1 (72%) had congenital solitary (figures 2A to H) and 20 (28%) had congenital coronary-ventricular multiple microfistulas (figure 3). The clinical and angiographic data of these 71 patients were collected from 30,829 subjects who
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Figure 3. Congenital coronaty artery left ventricular multiple microfistulas Angiographic frame of a distally located fistula in RAO view illustrating the morphological aspects of a coronarye ventricular multiple microfistula (doub arrows) originating from the dilated LAD (single arrow) and terminating into the LV. Theorigin is multiple, termination is multiple and pathway is multipe(.
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Dutch survey of congenital coronary artery fistulas in adults
Table 1. Noninvasive techniques used in patients in the Dutch registry (n=71) with 94 fistulas. Characteristics
Solitary (n=51)
Multiple (n=20)
Mean age (years) (range) Gender Age distribution range - 20-50 - 51-70 ->70
60 (range 29-85) 33 M (65%), 18 F (35%)
67 (range 49-82) 7 M (35%), 13 F (65%)
16 (31%) (11 M, 5 F) 21 (41%) (12 M, 9 F) 14 (28%) (10 M, 4 F)
1 (5%) (1 F) 9 (45%) (3 M, 6 F) 10 (50%) (4 M, 6 F)
25 (49%) 11 (22%) 13 (25%) 1 (2%) 26 (51%)
7
(35%)
7
(35%)
22 (43%) 29 (57%)
5 (25%) 15 (75%)
47 (92%) 4 (8%) 8 (16%) 1 (2%) 7 (14%) 36 (71%) 8 (16%) 28 (55%) 20 5 3
19 (95%) 1 (5%) 5 (25%) 3 (15%) 2 (10%) 11 (55%) 3 (15%) 8 (40%) 6
Noninvasive techniques Cardiac murmur - Continuous - Systolic - Diastolic No audible cardiac murmur ECG - Normal - Pathological* Rhythm - Sinus rhythm - Atrial fibrillation Myocardial infarction - Ipsilateral - Contralateral TTE - Normal - Abnormal - Mild valvular disease - Severe valvular disease - Wall motion abnormalities - LVH ETT - Normal - Positive for coronary insufficiency MIBI scintigraphy - Normal - Positive for coronary insufficiency
13 (65%)
1 1 7 (35%) 5 (25%) 2 (10%) 6 (30%) 3 (15%) 3 (15%)
32 (63%) 23 (45%) 9 (18%) 20 (39%) 9 (18%) 11 (21%)
M=male, F=female, TTE=transthoracic echocardiography, LVH=leIt ventricular hypertrophy, ETI=exercise tolerance test. *Pathological ECG=ST-T segment abnormalities, pathological Q waves, LVH and healed Ml.
underwent coronary angiographic examination in Dutch centres between 1996 and 2003. Angiograms were recorded on various media (35 mm films, super VHS video tapes and compact disks). In some hospitals Dutch Privacy Law regulations restricted the inclusion of more patients. The data of the Dutch registry on CAFs has been described earlier in detail: clinical data and coronary angiographic findings of 51 patientswith 63 congenital solitary CAFs (figure 4A)" and 20 subjects with 31 coronary artery-left ventricular multiple microfistulas (figure 4B)'2 were included. The characteristic findings of both patient groups are presented in tables 1 and 2.
8
Suspected coronary artery disease was the most frequent indication for performing angiography (62%), followed by analysis of heart murmur (13%) and congestive heart failure (6%) (table 3). Clinical presentation Angina pectoris occurred in 41 (58%) patients. Of these, 22 (31%) had significant atherosclerotic coronary artery disease and 19 (27%) showed a normal coronary arterial tree (table 4). Myocardial infarction (MI) occurred in 13 (18%) patients. The MI was located on the same side of the shunt in four (6%) and on the contralateral side in nine (12%) of these patients.
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Dutch survey of congenital coronary artery fistulas in adults
Table 2. Angiographic features and fistula characteristics of patients in the Dutch registry (n=71) with 94 fistulas. InvasIve methods
Solitary (n=51)
Multiple (n=20)
Description of CAFs Unilateral Bilateral Multilateral
41(80%) 8 (16%) 2 (4%)
10 (50%) 9 (45%)
63 (67%)
31 (33%)
17 (27%) 46 (73%) 29 21 11 2 Right 93%, left 7% 21 (33%) 42 (67%) 46 (73%) 1 (2%) 3 (5%) 4 (6%)
27 (87%) 4 (13%) 16 9 6
Fistula characterstIcs Origin - Multiple - Single LAD RCA -
-
-
Cx
LMCA Termination (right or left heart) - Multiple - Single - PA - LV -RA
-
- SCV
RV -LA - RVOT
Left 100% 28 (90%) 3 (10%) 20x
3(5%) 3(5%)
-
-
1(5%)
1 (2%) 2(3%)
CS
Pathway Tortuous/multiple
56% (35 fistulas) 41% (26 fistulas) None 3% (2 fistulas) 29% of fistulas (35% of patients) 9% 3%
-
-
Tortuous/single
-
Straight/multiple
Straight/single - Aneurysmal formation - Dilatation of fistula-related artery - CalcIfication -
87% (27 fistulas) 10% (3 fistulas) 3% (1 fistula) 3% (5% of patients)
CAF=coronary artery fistula, CS=coronary sinus, Cx=circumflex coronary artery, LA=left atrium, LAD=left anteror descending coronary artery, LCA=left coronary artery, LMCA=left main coronary artery, LV=left ventricle, PA=pulmonary artery, RA=right atrum, RCA=nght coronary artery, RV=right ventricle, RVOT=right ventricular outflow tract, SCV=superior caval vein.
Tabe 3. Indications for cathetersation in 71 patients in the Dutch registry with congenital CAFs.
Indication Assessment of suspected coronary heart disease Analysis of cardiac murmur and presence of echocardiographic turbulent flow Dyspnoea and congestive heart failure Risk stratification postinfarction Evaluation of valvular heart disease Ventricular and supraventricular arrhythmias Preoperative assessment for noncardiac surgery Pathological rest ECG Prior to lung transplantation Atypical chest pain Congenital heart disease
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No of cases 44 9 4 3 2 2 2 2 1 1 1
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RCA I
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B
A
Figure 4. Origin offistulas in the Dutch registry ofcongenital CAFs. A. In solitary CAPFs: Coronary arterial tree demonstrating the origin ofthefistulous vessels in 51 patients with 63 congenital solitary CAFs in adults; B. Coronary arterial tree illustrating the origin ofthefistous vessels in 20 patients with 31 congenital coronary artery-left ventricular multiple microfistulas in adults.
During a follow-up period of approximately five years there was no recurrence. One patient (1%) in the present cohort developed infective endocarditis. Initially, he was medically treated with an intravenous antibiotic and the fistula was later surgically ligated. Noninvasive diagnostic methods Some aspects of the noninvasive methods used are described below and further details are summarised in table 1. Sinus rhythm was predominant in 66 (93%) patients and atrial fibrillation was present in only five (7%) patients. Exercise tolerance test (ETT) was performed in 39 (55%) ofthe 71 patients ofthe Dutch contingent. The results were judged to be normal in 28 subjects (39%) and positive for myocardial ischaemia in 11 (16%). Exercise Sesta-MIBI scintigraphy was performed in 26 (36%) patients. The scan was normal in 12 (17%) of these patients, and positive, showing reversible myocardial defects, in 14 (19%). A total of 47 (66%) patients underwent transthoracic echocardiography (TTE). Normal findings were reported in 11 (15%) and abnormalities were reported in 36 (51%) ofthese patients. Mild and severe valvular heart disease were present in 26 (37%) and five (7%) patients, respectively. Furthermore, wall motion abnormality and left ventricular hypertrophy
Table 4. Presence or absence of angina pectoris with or without significant coronary artery disease (CAD). Angina pectoris No angina pectoris Total
10
Patients 41 30 71
CAD 22 18 40
No CAD 19 12 31
were seen in five (7%) patients. Cardiovascular magnetic resonance (CMR) was performed in 13 patients, as part of a feasibility study on solitary congenital CAFs.
After initial blind review, CMRconfimned the diagnosis in ten patients. In two patients the diagnosis was initially overlooked, but visualised in a second inspection. In one patient (fistula diameter 1 mm), the fistula was not visualised by CMR The fistulas could, after a second revision, be detected by CMR in 92% of the patients.13 Invasive diagnostic methods Invasive diagnostic methods are summarised in table 2. Coronary arteriography (CAG) was assumed to be the gold standard. Morphology of the fistulas Dilatation ofthe fistula-related artery was demonstrated in five (7%) patients and aneurysmal formation of the fistulous vessel in 19 (27%) patients. Further analysis ofthe angiograms ofthe 71 patients with a total of 94 fistulas demonstrated six straight pathways (6%) in five patients (7%) with a mean age of 53 years (range 30 to 70, three females and two males) with two bilateral and three unilateral fistulas, respectively. In the current study, pathways were found to be tortuous (94%) in the majority of the patients (93%). We found only six straight pathways (6%) in five patients (7%) with a mean age of 53 years (range 30 to 70), with two bilateral and three unilateral fistulas. Coronary artery disease No significant atherosclerotic coronary artery disease (CAD) was found in 42 patients (59%). Single, dual and triple vessel disease was present in 18 (25%), five (7%) and six (9%), respectively.
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Figure 5. Materiak used for transcatheter permanent embolic occlusion ofsolitary CAFs. A. Tbrombogenic polyvinyl alcohol foam, B. Detachable silicone balloons, C Microcoils, D. Gianturco-Grifta vascular occlusion device (GGVOD), E. Amplatzer duct occluder.
Treatment
Ofthe treatment modalities, percutaneous transluminal embolisation (PTE) was performed in two (3%) patients and surgical ligation (SL) was undertaken in 13 (18%) patients. The overwhelming majority of the patients (56; 79%) were treated with conservative medical management (CMM). Of those, six patients were under close clinical follow-up (watchful waiting) and 50 patients were treated pharmacologically. Betablockers (38 patients), calcium-channel blockers (16 patients), oral anticoagulation/antiplatelet (29 patients), angiotensin-converting enzyme inhibitors (8 patients), angiotensin-receptor blockers (3 patients), statins (15 patients), diuretics (10 patients), short and long-acting nitrates (21 patients), antiarrhythmic drugs (3 patients) and antidiabetic therapy (3 patients) were among the medications used. Follow-up and mortality During a follow-up period of approximately five years there was no recurrence of myocardial infarction in any ofthe patients. The total mortality was 6% (4/71) and cardiac mortality accounted for three of the four deaths. Two patients died of terminal heart failure. They both had cardiomegaly on chest X-ray and one had permanent atrial fibrillation. The third patient died suddenly at home. He had documented paroxysmal atrial fibrillation and a normal coronary arterial tree. In all three patients (age >72 years, two females and one male) death could possibly have been related to the presence of the fistula. None of the patients demonstrated aneurysmal formation of the fistulous pathways.
Discussion This is a report on the first survey on congenital CAFs in adults in the Netherlands, for the purpose of creating a registry. In the Dutch registry, suspected coronary artery disease was the primary indication for diagnostic angiography in 62% ofall cases with either solitary or multiple microfistulas. All 71 patients were collected from a total of 30,829 diagnostic coronary arteriography cohorts at 28 Dutch catheterisation suits. Population data for comparison from other countries is scarce. Urratia-S et al. reported that congenital CAFs were found in approximately one of every 50,000 patients Netherlands Heart Journal, Volume 14, Nulmber 1, January 2006
with congenital heart disease and in one of every 500 patients who undergo coronary angiographic examination.'4 It has been reported that multiple CAFs (bilateral, multilateral or both) were identified in 16% of the patients and fusiform aneurysmal formation in 19%.'4 Recently saccular aneurysm was reported by Kinoshita et al."5 In the present registry, we found that bilateral and multilateral fistulas were detected in 24 and 4% of the patients, respectively (together they may both be classified as multiple CAFs). Furthermore, aneurysmal formation was found in 27% of the patients and dilatation of the fistula-related coronary artery was recognised in 7% of the patients. Tortuosity and multiplicity of the pathway has an inverse relationship to the PTE therapeutic option, since accessibility and safe cannulation for percutaneous closure of the fistula may be limited.'6 For symptomatic patients with a sizeable left-toright shunt, invasive therapeutic options (surgical ligation and percutaneous transluminal embolisation) rely heavily on the morphological appearance and characteristics ofthe fistulas. Multilaterality, multiplicity and complexity of the fistulous tract determine the choice of the appropriate invasive therapeutic option. Surgical ligation and percutaneous closure (figure 5) are the two modalities currently available for permanent fistula abolition. Surgical ligation is indicated if multilaterality, multiplicity or extreme tortuosity ofthe components (origin, pathway and termination) is present with or without aneurysmal formation. On the other hand, PTE is chosen in the case of proximal localisation of the fistula or for patients of advanced age. For symptomatic patients, with a large-sized left-toright shunt, surgery or percutaneous catheter-based closure of CAFs is generally accepted. Coil embolisation is reserved for patients who satisfy the following criteria: safe accessibility to the fistula-related coronary artery, a single narrow drainage site, absence of large side branch, and absence of multiple fistulas. On the other hand, surgical ligation could be performed for large fistulas characterised by high fistulous flow, multiple origins or terminations, extreme tortuous pathways, and aneurysmal formation.'7 This retrospective survey revealed that surgical ligation accounted for 18% and percutaneous trans11
Dutch survey of congenital coronary artery fistulas in adults
luminal embolisation for only (3%) of the patients, possibly suggesting the need for longer learning curves for the latter therapeutic procedure. Because the morphological appearance of the CAFs and the choice of appropriate invasive therapeutic option is closely linked, in this study, the part played by PTE was very modest. This might be due to the presence of a high percentage of tortuosity of the different fistula components. The diagnosis of CAFs requires a high index of scepticism and alertness while taking the history and performing the physical examination. Nonspecific presentations are common, although dramatic presentations such as myocardial infarction or sudden death may occur. Typically the results of the physical examination, ECG and the chest X-ray film are normal except for sizeable coronary artery fistulas. In a substudy of 13 patients with congenital solitary CAFs, CMR demonstrated the feasibility of fistula detection. CMR confirmed the angiographic findings revealing the fistulous communications in 12 of 13 patients. In addition, the fistula characteristics were recognised in most of these patients. It has been previously reported that magnetic resonance imaging (MRI) could demonstrate the fistulous communications and their anatomy.'8"19 It should also be pointed out that CMRoffers the advantage of not requiring the injection of contrast materials and that there is excellent contrast between flowing blood and adjacent cardiovascular structures. Cine-CMR clearly delineates the relation between the fistulous jet and the cardiac valves. Sato et al. reported the detection of solitary CAFs by multislice CT scan.20 In the case ofthe current survey all data had been acquired prior to the availability of advanced multislice computed tomography. Increased availability of this diagnostic method may facilitate noninvasive detection and determination of CAFs. Although coronary-ventricular multiple microfistulous communications are rare,2' some cases of symptomatic female patients were reported in the 1980s by Martens et al.,22 and Vachon,23 and also recently by Duran et al.8 The typical patient with coronary artery-left ventricular multiple microfistulas is a female ofaround 50 years of age presenting with angina pectoris, without audible cardiac murmur, and with inconclusive noninvasive diagnostic tests. This invariably leads to investigation of suspected obstructive coronary artery disease. The diffuse character of the microfistulous communications renders them neither amenable for PTE or for surgical ligation, but instead requires conservative medical treatment. On the other hand, from a therapeutic point of view, solitary CAFs with multiple communications have recently been successfully treated by surgical closure.24 In addition, PTE has been successfully performed, after good cannulation, in patients with narrow and nonbranching fistulous communications.2' 12
Our current series, reporting on an adult population of 20 patients with congenital coronary-ventricular multiple microfistulas and 51 patients with congenital solitary CAFs, is the largest known to us. A prospective study with a stringent protocol is warranted with a larger number ofpatients, perhaps European-wide, in order to clarify several aspects of these infrequent congenital entities (solitary CAFs and coronary arteryleft ventricular multiple microfistulas) in adult patients. Limitations The limitations of this study are those inherent to observational surveys involving retrospective data collection. Lack of some data, as commonly encountered in a registry format, was a common finding. U
Acknowledgment We are indebted to our colleagues (appendix 1) who provided the patient data which formed the solid base for this paper. On www.cardiologie.nl you will find an example of the national registration form for coronary artery fistula in adult population in the Netherlands. References 1 2
3 4
5 6 7 8
9 10 11 12
13
14
Krause W. Uber den Ursprung einer akzessorischen A. Coronaria aus der A. Pulmonalis. ZRationell Med 1865;24:225-7. Yamanaka 0, Hobbs RE. Coronary artery anomalies in 126,595 patients undergoing coronary arteriography. Cathet Cardiovasc Diagn 1990;21:28-40. Gilebert C, van Hoof R, van de Werf F, Piessens J, De Geest H. Coronary artery fistula in an adult population. Eur Heart J
1986;7:437-43. Said SAM, Relik-van Wely L, van der Werf T. Angiographically diagnosed congenital coronary artery fistulas in an adult population. Cardiologie 1998;2:71-3. Levin DC. Anomalies and anatomic variations ofthe coronary arteries. In: Abrams HL, editor. Coronary arteriopathy. A practical approach. Little, Brown Co, Boston, 1983; pp 283-99. Angeini P. Coronary-to-pulmonary fistulae: What are they? What are their causes? What are their functional consequences? TexHeart InstJ2000;27:327-9. Angelini P. Coronary artery anomalies-current clinical issues. Definitions, classification, incidence, clinical relevance, and treatment guidelines. Tex Heart InstJ2002;29:271-8. Duran DA, Michelis V, Diaz P, Lujambio M, Kuster F, Lluberas R, et al. Evaluacion de pacientes portadores de fistulas coronarioventriculares multiples. Rev Med Uruguay 2003;19:237-41. Said SAM, Crijns HJGM, van der Werf T. Prerequisite conditions for identification of true coronary artery fistula. Cardiologie 2000;7:379-83. Aintablian A, Hamby RI, Hoffman I, Kramer RJ. Coronary ectasia: Incidence and results of coronary bypass surgery. Am Heart J 1978;96:309-15. Said SAM, van derWerfT. Dutch survey ofcoronary artery fistulas in adults: Congenital solitary fistulas. Int J Cardiol [in press]. Said SAM, van derWerfT. Dutch survey ofcoronary artery fistulas in adults: Congenital coronary artery-left ventricular multiple micro-fistulas. Int J Cardiol [in press]. Said SAM, Hofman MBM, BeekAM, van derWerfT, van Rossum AC. Feasibility of cardiovascular magnetic resonance imaging of angiographically diagnosed congenital solitary coronary artery fistulas [Submitted]. Urrutia-S CO, Falaschi G, Ott DA, Cooley DA. Surgical management of 56 patients with congenital coronary artery fistulas. Ann Thorac Surg 1983;35:300-7.
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15 Kinoshita 0, Ogiwara F, Hanaoka T, Tomita T, Yokozeki 0, Kai R, et al. Large saccular aneurysm in a coronary arterial fistula. A case report. Angiology2005;56:233-5. 16 Hsieh KS, Huang TC, Lee CL. Coronary artery fistulas in neonates, infants, and children: Clinical findings and outcome. Pediatr CardioI2002;23:415-9. 17 Mavroudis C, Backer CL, Rocchini AP, Muster AJ, Gevitz M. Coronary artery fistulas in infants and children: a surgical review and discussion of coil embolization. Ann Thorac Surg 1997;63: 1235-42. 18 Bolognesi R, Tsialtas D, Barbaresi F, Manga C. Single coronaryright ventricular fistula with a partially thrombosed large aneurysm of its proximal tract in a 66-year-old man. Eur HeartJ 1994;15: 1720-4. 19 Parga JR, Ikari NM, Bustamante LNP, Rochitte CE, De Avila LF, Oliveira SA. MRI evaluation of congenital coronary artery fistulae. BrJRadiol2004;77:508-1 1. 20 Sato Y, Mitsui M, Takahashi H, Miyazawa T, Okabe H, Inoue F, et al. A giant left circumflex coronary artery-right atrium arteriovenous fistula detected by multislice spiral computed tomography. Heart Vessels2004;19:55-6.
21 Chia BL, Chan ALK, Tan LKA, Ng RAL, Chiang SP. Coronary artery-left ventricular fistula. Cardiology 1981;68:167-79. 22 Martens J, Haseldoncks C, van de Werf F, De Geest H. Silent left and right coronary artery-left ventricular fistulas: An unusual prominent thebesian system. Acta Cardiologica 1983;38:139-42. 23 Vachon JM. Les fistules congenitales coronaro-ventriculaires gauches. Revue de la litterature. Ann de Cardiologie et D' angeiologie 1983;32:21-5. 24 Muller D, Wimmer-Greinecker G, Fichtescherer S, Moritz A. Symptomatic coronary artery-pulmonary artery fistulae. IJTCVS 2004;20:192-3. 25 Boccalandro F, Awadalla H, Smalling RW. Percutaneous transcatheter coil embolization of two coronary fistulas originating from the left main ostium and left anterior descending artery. Cath Cardiovasc Intervent2002;57:221-3.
Appendix 1: Participating Dutch centres J.O.J. Peels, Alkmaar Medical Centre RJ. Lionarons, B.J.L. de Rode, Twente Hospital, location Almelo W.L. Mosterd, Eemland Hospital, Amersfoort C.C. de Cock, VU University Hospital Medical Centre, Amsterdam W.G. de Voogt, R-G.E.J. Groutars, B. Ilmer, A.R. Wlllems, J. Visser, St Lucas-Andreas Hospital, Amsterdam J.KM. Peters, Maas Hospital, Boxmeer B.J. van den Berg, IJsselland Hospital, Capelle a/d IJssel F.C.W. Tietge, Deventer Hospitals, Deventer L. Relik-van Wely, C. de Nooyer, Diaconessenhuis, Eindhoven E.M.C.J. Wajon, J.C. Poortermans, J.W. Louwerenburg, Medical Spectrum Twente Hospital, Enschede W.A.A.J. van Ekelen, P.E. Polak, St Anna Hospital, Geldrop J.A.J. de Boo, A.H. Liem, H.W.O. Roeters van Lennep, Oosterschelde Hospital, Goes B. van Vlies, A.J. Funke Kiipper, Kennemer Gasthuis, Haarlem K Dijkgraaf, St Jansdal Hospital, Harderwijk M.F. Scholten, RR Lalisang, Elkerliek Hospital, Helmond A. Derks, H.T. Droste, J.H. Fast, Twente Hospital Group, location Hengelo C.G.K.M. Fauser, Bethesda Hospital, Hoogeveen C.J. de Vries, Leeuwarden Medical Centre, Leeuwarden A.G. Boehmer, Atrium Medical Centre, Kerkrade L.H.R Bouwels, Canisius Wilhelmina Hospital, Nijmegen T. van der Werf, J. Timmermans, Radboud University Medical Centre, Nijmegen D.G. de Waal, Waterland Hospital, Purmerend G.J. de Weerd, Maasland Hospital, Sittard D.E.P. de Waard, Antonius Hospital Sneek C.L.J.M. van Engelen, De Heel Hospital, Zaans Medical Centre, Zaandam A.C. Tans, Het Spittaal Hospital, Zutphen W.J. Louridtz, H.A. Oude Luttikhuis, Isala Hospital, Zwolle
Dq
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