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Congenital coronary arteriovenous fistula∗
Case Reports Congenital Coronary Arteriovenous Fistula* DAVID B . CARMICHAEL, M .D ., F .A .C .C .
and
DOUGLAS G . DAVIDSON, M .D .
La Jolla, California
L
Roentgenogram of the chest showed normal pulmonary vascularity, slight fullness of the main pulmonary arterial segment and left ventricular enlargement of moderate degree . The electrocardiogram was within normal limits . The phonocardiogram revealed a continuous systolic and diastolic murmur of approximately equal intensity in the left and right fourth intercostal spaces . Cardiac catheterization provided the following data :
NC BEFORE the problem of coronary athero-
sclerosis attained its present recognition, a
considerable body of literature had accumulated concerning aneurysm and rupture of the coronary arteries .' In 1912 the relationship between coronary arterial aneurysm and fistulous connection with the venous system of the heart was initially reported .' Congenital coronary arteriovenous fistulas remain rare developmental lesions, but their recognition is increasing, stimulated by reports of successful surgical corrections .
Hemoglobin 13,8 gm . per cent Hematocrit 38 per cent pH, direct 7 .44 Ventilation 3 .78 L . per minute per Ms Oxygen uptake 166 cc . pCO 31 .5 mm . Hg Oxygen capacity 18 .45 volume per cent Oxygen uptake 117 ml . per minute per M' Respiration quotient 0 .76
CASE REPORTS CASE 1 .
A 38 year old housewife had a known heart murmur since she was 4 years of age . During her school years the patient was active in sports until she entered college . In her freshman year exhaustion developed and she was advised to terminate her college career . The patient did clerical work until her marriage at age 29 . A normal pregnancy occurred at age 30, with an uncomplicated delivery . Exercise fatigue ensued at age 34, and during the year prior to surgery, exercise fatigue was progressively more severe . The patient denied edema of the ankles, orthopnea or paroxysmal nocturnal dyspnea . On physical examination, her height was 62 inches, weight 100 pounds, and vital capacity 3,200 cc . No cyanosis or clubbing was noted . There was no precordial asymmetry and the heart size was within normal limits on percussion . The apical thrust was normal . A faint systolic thrill was noted in the fourth left intercostal space at the left sternal border . A grade 4 to-and-fro machinery murmur was audible widely over the anterior precordium, maximal in the fourth left intercostal space and well heard in the fourth right intercostal space . The murmur had a superficial, rather high frequency quality that examiners described as being "distant from the heart sounds ." The peripheral vessels were not remarkable . There was a regular sinus mechanism at 60 per minute . The blood pressure was 125/70 mm . Hg.
The pressure readings were within normal limits but there was a distinct step-up in oxygen content at the right ventricular level (Table i) . Retrograde aortography showed aneurysmal vessels with tortuous branches of the right coronary artery (Figs. I and 2) . Surgical Findings: Exploratory thoracotomy was done by Dr . John Chambers with the pump-oxygenator in readiness . An aneurysmal dilatation of the right coronary artery was found, measuring 2 by 3 cm ., approximately 2 cm . from the origin of the right coronary artery. A thrill was palpable over the aneurysm . Tortuous, dilated, serpentine branches of the right coronary artery and the veins over the surface of the heart were noted (Fig . 3) . Oximetry revealed a rise in saturation at both right atrial and right ventricular levels . The aneurysm was freed by dissection (Fig . 4) and the right coronary artery temporarily clamped with continuous electrocardiographic monitoring . No injury effect appeared and the rhythm remained stable . After a period of 5 minutes of occlusion of the right coronary artery the vessel was doubly ligated . Repeat oximetry continued to reveal a step-up in oxygen saturation at the
' Supported in part by the Mr. and Mrs . F . H . Tewksbury grant administered by the San Diego Bio-Medical Research Institute . 846
THE AMERICAN JOURNAL OF CARDIOLOGY
Coronary Arteriovenous Fistula TABLE I
Cathcterization Data in Case t Pressure (mm . Hg)
Measurement
Superior versa cava Inferior versa cava Right atrium (mid) Right atrium (low) Right ventricle (mid) Main pulmonary artery Pulmonary wedge Coronary sinus Aorta Systemic blood flow Pulmonary blood flow A-V shunt Cardiac index Pulmonary index Pulmonary arteriolar resistance Total pulmonary resistance Total peripheral res stance
18/0 (4) 18/8 (12) (3) 7 .5/0 (4) 107/66 (83)
02 Content
(volume per cent) 12 .13 13 .03 12 .87 13 .40 14 .76 15 .03 6 .14 17 .41 (94 .4)
3 .55 L ./min . 6 .98 L ./min . 49 per cent 2 .50 L,/min ./M° 4 .92 L ./min ./M2 103 dynes sec- cm . -' .-5 m 138 dynes sec. c I
1870 dynes s ee . cm . -5
right ventricular level . Additional dissection disclosed several branches of the right coronary artery distal to the aneurysmal mass plunging into the right ventricular cavity . These were ligated and divided .
to . 1 . DECEMBER
1961
Case 1 .
847
Repeat oximetry verified stable levels of oxygenation at the right atrial, right ventricular, and main pulmonary artery levels . The postoperative course was benign . Eighteen months after surgery the physical examination is entirely within normal limits and the patient carries on tier normal duties without symptoms . CASE 2 . This was a 43 year old Caucasian woman whose birth and early development were reported to be unremarkable . At 10 years of age migratory polyarthralgias appeared without objective arthritis . A tonsillectomy was performed and all symptoms cleared . No comment was made about the heart ; childhood was otherwise within normal limits . Exertional dyspnea occurred at age 26 . 1' his became progressively more incapacitating and at age 30, edema of the ankles appeared and a heart murmur was initially described . The patient was digitalized and activities were restricted to the sedentary activities of a religious cloister . At age 36 orthopnea, intractable ankle edema, cyanosis and hepatic enlargement necessitated continuous medical care. Death occurred at age 43 after 17 years of chronic cardiac invalidism . On physical examination the patient was emaciated and pale . The heart was markedly enlarged on percussion, with an increased apical thrust displaced to the left. No thrill was palpable. A to-and-fire, machinery murmur of grade 4 intensity and a peculiar superficial quality was audible in the third and fourth interspaces with equal intensity at the right and left sternal borders . There was wide radiation of both systolic and diastolic components . The
Retrograde aortogram demonstrates aneurysmal mass with dilated branches .
848
Carmichael and Davidson
ro . 2 . Ca . Later sta c in retrograde aortogram more clearly outlining the aneurysmal vessel .
Fro . 4 . Case 1 . artery (arrow) . Fin . 3 . Case 1 . Tortuous . dilated vessels over the surface of the heart . blood pressure was 125/30 mm . Hg . Duroziez' sign was positive . Sinus rhythm with occasional premature contractions was maintained until immediately before death when atria]l fibrillation supervened . The electrocardiogram showed left axis deviation, left ventricular enlargement ("strain" pattern) and atrial enlargement . A roentgenogram of the chest showed marked, generalized cardiac enlargement with bilateral hilar congestion . Autopsy Findings: The heart weighed 1,200 gm . with dilatation of all chambers and marked left ventricular hypertrophy . A tortuous, cirsoid left anterior de-
eurysm of the right coronary
scending branch of the left coronary artery with fistulotts communication through the great cardiac vein to the right heart was noted . The right coronary artery was similarly tortuous and markedly enlarged . A small branch of the right coronary artery terminated in a huge aneurysmal mass measuring 5 .5 by 9 cm . which connected directly with the right atrium and with several anterior cardiac veins . The aneurysm was partially filled with laminated antemortem clot . CASE 3 . A 16 year old girl had a heart murmur discovered as an incidental finding during an episode of pneumonia at 18 months of age . As a young child she would not play as vigorously as her fellows and THE AMERICAN JOURNAL OF CARDIOLOGY
Coronary Arteriovenous Fistula
849 TABLE 11
Catheterization Data in Case 2
Measurement
Fro . 5 . Case 3 . Posteroanterior chest roentgenogram demonstrating widening of the base of the heart . voluntarily sought more rest . Respiratory infections were frequent . At 37 months of age she underwent operation with a preoperative diagnosis of patent do( tus arteriosus . A patent vessel 1 .5 cm . in length and 0.3 cut. i n diameter was triply ligated . This procedure did not obliterate a pronounced thrill over the base of the heart . The pericardium was opened and the surgeon described "dilation of the pulmonary artery as well as both auricles and a vigorous systolic thrill in the region of the root of the pulmonary artery and over the right auricle ." Postoperatively, a toand-ho murmur remained audible . For 1 year the child remained on digitalis therapy . At the time of examination she was experiencing exercise fatigue, recurrent palpitation, recurrent pain in the left anterior portion of the chest and an increased susceptibility to respiratory infection . On physical examination her height was 67 inches, weight 128 pounds, and vital capacity 3,000 cc . No cyanosis, clubbing, or precordial asymmetry were present . The heart was enlarged to percussion . 'I he retromanubrial pulsation was markedly increased and a systolic thrill was present over the left carotid artery . A continuous precordial thrill was maximal at the third left interspace at the left sternal border. A grade 5 to 6 to-and-fro machinery murmur was maximal at this point and was widely audible over the entire precnrdium . There was sinus rhythm at 80 per minute and the blood pressure was 130/ 45 mm . Hg . The electrocardiogram presented voltage evidence of left ventricular enlargement. Chest roentgenogram and fluoroscopy (Fig . 5) demonstrated cardiac enlargement with an enlarged, widely pulsatile aorta . Dilation of the pulmonary arteries with an expansile pulsation bilaterally was noted . A phonocardiogram (Fig . 6) revealed a continuous systolic and diastolic DECEMBER
1961
Superior versa eava Right atrium (high) Right atrium (mid) Right atrium (low) Right ventricle (inflow) Right ventricle (mid) Right ventricle (infundibulum) Main pulmonary artery Right pulmonary artery Pulmonary wedge Brachial artery
Pressure (mm . Hg)
5
O s Content (volume per cent) 12 .29 12 .66 12 .80 12 .55
40/0--5 (17) 43/0-5 (17)
14 .95 15 .59
29/0-5
16 .15
2 7 /13 (20)
15 .64
29/13 (20) (10) 114/69 (88)
15 .50
System blood flow Pulmonary blood flow A-V shunt Cardiac index Pulmonary index Pulmonary arteriolar resistance Total pulmonary resistance Total peripheral resistance
17 .61 (95 .6)
5 .34 L ./min . 13 .40 L./min . 60 per cent 3 .20 L ./min ./Mr 8 .02 L ./min ./M2 60 dynes s ec . c m. -6 119 dynes s ec . cm . -5 1318 dynes sec . c m .'
murmur, maximal in the third and fourth left intercostal spaces at the left sternal border . Cardiac catheterization provided the following data : Hemoglobin Hematocrit . . . . . . Ventilation . . . . . pCOs Oxygen capacity Oxygen uptake Respiratory quotient
13.7 mg. per cent 44 per cent 7,43 4 .0 L . per minute per Ms 264 cc . 37 .0 mm . Hg 18 .42 volume per cent 158 ml. per minute per M2 0 .7
A moderate elevation in right ventricular pressure and a huge left to right shunt at the ventricular level were observed (Table it) . Retrograde aortography (Fig. 7) demonstrated opacification of a vascular structure apparently arising from the root of the aorta, progressing superiorly and describing an arc to the right . This structure was considered to represent a markedly dilated right coronary artery . Surgical Findings : Exploratory thoracotomy was done by Dr . Glen Gibbons, with the pump-oxygenator in readiness . A single, enlarged, thin-walled vessel was found at the base of the aorta representing
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Carmichael and Davidson
Fro . 6 .
Case 3 . Pre- and postoperative phonocardiographic tracings .
the entire right coronary artery medially placed from its normal position (Fig . 8) . The structure was 3 cm . in length with the initial 1 .5 cm. directed superiorly, where it described a tight arc and plunged inferiorly into the outflow tract of the right ventricle just proximal to the pulmonary valve . No branches were identified . The aneurysmal vessel was exposed until it could be clamped proximally and distally . Oximetry confirmed the rise in saturation at the ventricular level . The electrocardiogram was monitored for five minutes after clamping the vessel and no change occurred . The aneurysm was then divided in its midportion and the two ends oversewn (Fig . 9) . The patient tolerated the procedure well and her postoperative course has been uncomplicated . Phono-
7. Case 3 . Retrograde aortogram with arrow pointing to dilated right coronary artery . FIG .
cardiography (Fig . 6B) demonstrated obliteration of the continuous murmur . COMMENTS
The lesion represents a fistulous tract between the coronary arterial circulation and either the coronary venous system, right atrium, right ventricle or pulmonary artery . Steinberg et al .' postulate two forms of embryologic development . One represents an arrest in the normal development of the coronary vessels with retention of primitive myocardial sinusoids . A second variation is composed of relatively mature vessels which can he identified as arteries and veins . Secondary changes in the vessels entering and leaving the area of fistulous communication result in the majority of the gross changes observed at surgery and many of the physical and laboratory findings which ease the clinical identification of the fistula . The importance of the abnormality to the afflicted patient has been a source of dispute . In a review of the relevant literature, 2-" data from which one may draw conclusions are available in thirty-three cases . In this group, twenty-five patients either died as a result of the lesion, gave objective evidence of increased cardiac work or had symptoms indicative of cardiac insufficiency . In a significant number of cases the lesion is important and factors dictating importance are the age of the patient and the size of the shunt . Davison ct al .'22 point out that congenital A-V aneurysms are known to increase gradually in size over the course of years and this necessitates a progressive rise in cardiac output in order to maintain an adequate THE AMERICAN JOURNAL OF CARDIOLOGY
Coronary Arteriovenous Fistula
851
Case 3 . The two clamps identify the oversewn ends of the aneurysmal right coronary artery . FIG . 9.
Case 3. The clamp identifies the aneurysmal right coronary artery arising from the root of the aorta . Fin . 8 .
orthodox circulation in the presence of an increasing shunt . Steinberg et al ." succinctly state : "Given sufficient shunt-volume acting over a long enough period of time, left ventricular hypertrophy and failure must inevitably occur ." This situation results in a high output failure with all of its usual consequences . Of interest in light of current thought is the fact that in spite of the high flow, high gradient and turbulence, little or no mention has been made of atherosclerotic plaques in the afferent arteries or efferent veins . Isolated subjects survive to old age . Colbeck and Shawt° report the survival of a patient who reached the advanced age of 85 years with only 18 months of cardiac insufficiency . The patient reported by White and Paul'" • 4 ' in 1949 remains asymptomatic in spite of the fact that his surgery was limited to an exploratory procedure with with no attempt at definitive correction . The opposite is more likely to prevail . Large shunt volumes have been described in several cases in which physiologic data are available . Valdivia" described a patient who died in chronic cardiac insufficiency at age 25 years . The heart weight at autopsy was 1,800 gm . In nine autopsy cases 3 the mean heart weight was 700 gm . Clinical Diagnosis : The lesion is usually discovered in one of two ways . In the analysis of a continuous murmur, an unusual location of the murmur and a peculiarly superficial quality suggest coronary arteriovenous fistula . A sizaDECEMBER
1961
ble number of cases have been found during an exploratory thoracotorny for presumed patent ductus artcriosus . Apart from the murmur, other physical findings are those anticipated with a left to right shunt . Suspicion must be aroused during auscultation or the diagnosis will be overlooked . The roentgenogram of the chest and the electrocardiogram customarily offer no assistance . Right heart catheterization demonstrating a left to right shunt is supportive evidence and the combination of a to-and-fro cardiac murmur in an unusual location coupled with this laboratory finding strongly suggests the lesion . Dye studies utilizing the technic of retrograde aortography or controlled coronary angiography present the greatest opportunity for definitive preoperative diagnosis . These findings are summarized by Fell et al ."' as a "diagnostic triad" of (1) abnormal location of a to-and-fro machinery murmur, (2) left-to-right shunt at the atrial or ventricular level and (3) a large, tortuous coronary artery seen by angiocardiography or aortography . Indications for and Results of Surgery : The complications dictate the necessity for an effort at corrective treatment . These include cardiac insufficiency, bacterial endocarditis, myocardial ischemia and thrombosis in the anomalous vessel and aneurysm . Including the three cases presented in this review the world literature includes a total of forty-six verified cases . Since the relationship between congenital aneurysm of the coronary arterial system and fistulous connection with the venous system has been appreciated for such a short period of time, surgical correction of the problem has been a relatively recent innovation . Including Cases 1 and 3 in this report, fifteen
852
Carmichael and Davidson
patients have had successful ligation
of
the fistu-
lous vessels . One surgical death occurred in a child who had a concomitant ligation of a patent ductus arteriosus and was assumed to have died as a result of pulmonary hypertension 3 4
Five
other operations were limited to exploration without attempted ligation . The fear of myocardial ischemic changes with ligation
of
the
shunt-bearing vessels as postulated by Davison et a1
22
has not proved to be a problem with the
exception of the case described by Michel and Herbst 2 5 Our own experience emphasizes the value of careful oximetry following ligation of all apparent communications with the right heart chambers, as multiple shunts may be present .
SUMMARY Three verified cases of congenital coronary arteriovenous fistula have been described, bringing the total reported cases to forty-six . The combination
of
a machinery murmur in an
unusual location, catheterization evidence of a left to right shunt, and opacification of a dilated coronary artery by angiography confirms the clinical diagnosis . Surgical correction has proved to be safe and effective. The number of surgical cures (including two cases reported herein) now totals fifteen cases with one death due to surgery . REFERENCES 1 . GRIPEITH, T . W . Remarks on aneurism of the coronary arteries of the heart . Brit . M. .7„ 1 : 266, 1901 . 2 . TREVOR, R. S . Aneurysm of the descending branch of the right coronary artery, situated in the wall of the right ventricle and opening into the cavity of the ventricle, associated with great dilatation of the right coronary artery and non-valvular infective endocarditis . Proc . Roy . Soc . Med., 5 :20,1911-12 . 3 . STEINBERG, I ., BALDWIN, J. S . and DOTTER, C . T . Coronary arterio-venous fistula. Circulation, 17 : 372,1958 . 4 . BLAREWAY, H . A hitherto undescribed malformation of the heart . J. Anat ., 52 : 354, 1917 . 5 . WILSON, J . G. and GRANT, R, T . A case of congenital malformation of the heart in an infant associated with partial heart block . Heart, 12 : 295,1925-26 . 6 . HALPERT, B . Arteriovenous communication between the right coronary artery and the coronary sinus . Heart, 15 : 129, 1930 . 7 . LOWENHEIM, I . Eine seltene Missbildung der Coronargefasse. Frankfurt. Ztschr. Path ., 43 : 63,1932 . 8 . NAGOYA, M . and TAKAHASHI, H. Aneurysma Serpentinum de linken Doron Arterie . Tr. Japanese Path . Soc ., 22 : 583, 1932 .
9 . HARRIS, P . N . Aneurysmal dilatation of the cardiac coronary arteries : Review of the literature and report of a case . .4m . J. Path ., 13 : 1937, 10 . BJoRK, C . and CRArooRD, C . Artcriovenous aneurysm of the pulmonary artery simulating patent ductus arteriosus Botalli . Thorax, 2 : 65, 11 . VALDIVIA, E ., ROWE, G . C . and ANGLVTNE, D . M. Large congenital aneurysm of the right coronary artery . Arch. Path ., 63 : 168, 1947 . 12 . EMMINGER, E . Arterio-venoses Aneurysma de rechten Herzkranzschlagader . Klin, med, 2 : 652,1947 . 13 . SCOTT, D . H ., Aneurysm of the coronary arteries. Am . Heart J., 36 : 403, 1948 . 14 . PAUL, 0., SWEET, R . H . and WHITE, P. D . Coronary arteriovenous fistula : Case report . Am . Heart J., 37 : 441, 1949 . 15 . BROWN, R . C . and BURNETT, J . D . Anomalous channel between aorta and right ventricle. Report of a case . Pediatrics, 3 : 597, 1949 . 16 . ESPINO VELA, J ., VELAZQUEZ, 'F. and FUENMAYOR, A. Amplia communication congenita de la aorta con el ventrie to derecho a traves de la arteria coronaria derecha anomala. Arch . Inst. cardiol . Mexico, 21 : 686, 1951 . 17 . WILLIAMS, R . R ., KENT, G . B ., JR . and EDWARDS, J. F . Anomalous cardiac blood vessel communicating with right ventricle . Observations in case of pulmonary atresia with intact ventricular septum . Arch . Path ., 52 : 480, 1951 . 18 . ALEXANDER, W . S . and GREEN, H . C . Coronary blood vessel arising from cardiac ventricle . Report of case showing other cardiac anomalies . Arch . Path., 53 : 187, 1952 . 19 . BAYLISS, J . H . and CAMPBELL, M . An unusual cause for a continuous murmur . Guy's Hasp . Rep ., 101 : 174, 1952 . 20 . COLBECK, J . C . and SHAW, J . M . Coronary aneurysm with arteriovenous fistula . Am . Heart J., 48 : 270, 1954 . 21 . LoviTT, W . V ., JR . and LUTZ, S ., JR . Embryological aneurysm of the myocardial vessels . Arch . Path., 57 : 163, 1954 . 22 . DAVISON, P . H ., MCCRACKEN, B . H . and MeILVEEN, D . J. S . Congenital coronary arteriovenous aneurysm . Brit. Heart J., 17 : 569, 1955 . 23 . SONDERGAARD, T. Henry Ford Hospital International Symposium in Cardiovascular Surgery, p .490, Philadelphia, 1955 . W. B. Saunders Co . 24 . DAvis, C ., DILLON, R . F ., FELL, E . G. and GASUL, B. M. Anomalous coronary artery simulating patent ductus arteriosus. J.A.M.A . . 160 :1047, 1956 . 25 . KNOBLICH, R . and RAWSON, A . J . Arteriovenous fistula of the heart . Am . Heart .1., 52 :474, 1956 . 26 . MozEN, H . E . Congenital cirsoid aneurysm of a coronary artery with associated arterio-atrial fistula, treated by operation : A case report . Ann . Surg., 144 : 215, 1956 . 27 . COOLEY, R . M . and SLOAN, R. B . Radiology of the Heart and Great Blood Vessels, p . 226 . Baltimore, 1956 . Williams & Wilkins. 28 . MICHEL, D . and HERBST, M . ZGSdm. Kreislaufrmsch ., 46 : 538, 1957 . THE AMERICAN JOURNAL OF CARDIOLOGY
Coronary Arteriovenous Fistula 29 . WALTHER, R . J ., STARKEY, G . y'V. B ., ZERVOPOLUS, E . and GTRAONS, G . A . Coronary arteriovenous fistula . Clinical and physiologic report on two patients, with review of the literature . Am . J . Med., 22 : 213, 1957 . 30 . KITTLE, C . F . In ; discussion of paper by GASUL, B . M ., FELL . E . G., MOREANO, M . and WEINBERO, M ., JR . Congenital coronary arteriovenous aneurysm . Arch . Surg., 78 :203, 1958 . 31 . FELL, E., WEINBERG . M ., GORDON, A . S ., GASUL, B . M . and .foaesos, F . R . Surgery for congenital coronary artery arteriovenous fistulae . Arch . Sing ., 77 : 331, 1958 . 32 . EDWARDS, J . E ., CLADDING, T . C . and WEIR, A . B ., JR . Congenital communication between the right coronary artery and the right atrium . J. Thoraric Surg ., 35 : 662, 1958 . 33 . SANGER, P . W ., TAYLOR, F . H . and RoBarsEK, F . Cited in reference 34 .
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853
34. Bosun, L . H ., VASLI, S ., MOUE, C . M . and BELTER, L . F . Congenital coronary arteriovenous fistula associated with large patent ductus . Circulation, 20 : 254, 1959 . 35 . GASUL, B . M ., ARCILLA, R . A ., LYNFIELU, J ., BICOFF, J . P . and LOAN, L . L . Congenital coronary arteriovenous fistula : Clinical, angiocardiographic, and physiologic findings in five patients . Abstracts of the 32nd Scientific Sessions, American Heart Association . Circulation, 20 : 700, 1959 (abs.) . 36 . GR06, M . and Korn . E . Congenital aneurysm of the coronary artery . Arch . Dis, Childhood, 34 : 8, 1959 . 37 . GROSS, R. E. Cited in reference 14 . 38 . GAROMELLA, J . J . Cited in reference 32 . 39 . MoRRow, A . G . Cited in reference 32 . 40. JOHNSON, J . Cited in reference 24 . 41 . WHITE, P . I) . Personal communication, 1961 .
and
DOUGLAS G . DAVIDSON, M .D .
La Jolla, California
L
Roentgenogram of the chest showed normal pulmonary vascularity, slight fullness of the main pulmonary arterial segment and left ventricular enlargement of moderate degree . The electrocardiogram was within normal limits . The phonocardiogram revealed a continuous systolic and diastolic murmur of approximately equal intensity in the left and right fourth intercostal spaces . Cardiac catheterization provided the following data :
NC BEFORE the problem of coronary athero-
sclerosis attained its present recognition, a
considerable body of literature had accumulated concerning aneurysm and rupture of the coronary arteries .' In 1912 the relationship between coronary arterial aneurysm and fistulous connection with the venous system of the heart was initially reported .' Congenital coronary arteriovenous fistulas remain rare developmental lesions, but their recognition is increasing, stimulated by reports of successful surgical corrections .
Hemoglobin 13,8 gm . per cent Hematocrit 38 per cent pH, direct 7 .44 Ventilation 3 .78 L . per minute per Ms Oxygen uptake 166 cc . pCO 31 .5 mm . Hg Oxygen capacity 18 .45 volume per cent Oxygen uptake 117 ml . per minute per M' Respiration quotient 0 .76
CASE REPORTS CASE 1 .
A 38 year old housewife had a known heart murmur since she was 4 years of age . During her school years the patient was active in sports until she entered college . In her freshman year exhaustion developed and she was advised to terminate her college career . The patient did clerical work until her marriage at age 29 . A normal pregnancy occurred at age 30, with an uncomplicated delivery . Exercise fatigue ensued at age 34, and during the year prior to surgery, exercise fatigue was progressively more severe . The patient denied edema of the ankles, orthopnea or paroxysmal nocturnal dyspnea . On physical examination, her height was 62 inches, weight 100 pounds, and vital capacity 3,200 cc . No cyanosis or clubbing was noted . There was no precordial asymmetry and the heart size was within normal limits on percussion . The apical thrust was normal . A faint systolic thrill was noted in the fourth left intercostal space at the left sternal border . A grade 4 to-and-fro machinery murmur was audible widely over the anterior precordium, maximal in the fourth left intercostal space and well heard in the fourth right intercostal space . The murmur had a superficial, rather high frequency quality that examiners described as being "distant from the heart sounds ." The peripheral vessels were not remarkable . There was a regular sinus mechanism at 60 per minute . The blood pressure was 125/70 mm . Hg.
The pressure readings were within normal limits but there was a distinct step-up in oxygen content at the right ventricular level (Table i) . Retrograde aortography showed aneurysmal vessels with tortuous branches of the right coronary artery (Figs. I and 2) . Surgical Findings: Exploratory thoracotomy was done by Dr . John Chambers with the pump-oxygenator in readiness . An aneurysmal dilatation of the right coronary artery was found, measuring 2 by 3 cm ., approximately 2 cm . from the origin of the right coronary artery. A thrill was palpable over the aneurysm . Tortuous, dilated, serpentine branches of the right coronary artery and the veins over the surface of the heart were noted (Fig . 3) . Oximetry revealed a rise in saturation at both right atrial and right ventricular levels . The aneurysm was freed by dissection (Fig . 4) and the right coronary artery temporarily clamped with continuous electrocardiographic monitoring . No injury effect appeared and the rhythm remained stable . After a period of 5 minutes of occlusion of the right coronary artery the vessel was doubly ligated . Repeat oximetry continued to reveal a step-up in oxygen saturation at the
' Supported in part by the Mr. and Mrs . F . H . Tewksbury grant administered by the San Diego Bio-Medical Research Institute . 846
THE AMERICAN JOURNAL OF CARDIOLOGY
Coronary Arteriovenous Fistula TABLE I
Cathcterization Data in Case t Pressure (mm . Hg)
Measurement
Superior versa cava Inferior versa cava Right atrium (mid) Right atrium (low) Right ventricle (mid) Main pulmonary artery Pulmonary wedge Coronary sinus Aorta Systemic blood flow Pulmonary blood flow A-V shunt Cardiac index Pulmonary index Pulmonary arteriolar resistance Total pulmonary resistance Total peripheral res stance
18/0 (4) 18/8 (12) (3) 7 .5/0 (4) 107/66 (83)
02 Content
(volume per cent) 12 .13 13 .03 12 .87 13 .40 14 .76 15 .03 6 .14 17 .41 (94 .4)
3 .55 L ./min . 6 .98 L ./min . 49 per cent 2 .50 L,/min ./M° 4 .92 L ./min ./M2 103 dynes sec- cm . -' .-5 m 138 dynes sec. c I
1870 dynes s ee . cm . -5
right ventricular level . Additional dissection disclosed several branches of the right coronary artery distal to the aneurysmal mass plunging into the right ventricular cavity . These were ligated and divided .
to . 1 . DECEMBER
1961
Case 1 .
847
Repeat oximetry verified stable levels of oxygenation at the right atrial, right ventricular, and main pulmonary artery levels . The postoperative course was benign . Eighteen months after surgery the physical examination is entirely within normal limits and the patient carries on tier normal duties without symptoms . CASE 2 . This was a 43 year old Caucasian woman whose birth and early development were reported to be unremarkable . At 10 years of age migratory polyarthralgias appeared without objective arthritis . A tonsillectomy was performed and all symptoms cleared . No comment was made about the heart ; childhood was otherwise within normal limits . Exertional dyspnea occurred at age 26 . 1' his became progressively more incapacitating and at age 30, edema of the ankles appeared and a heart murmur was initially described . The patient was digitalized and activities were restricted to the sedentary activities of a religious cloister . At age 36 orthopnea, intractable ankle edema, cyanosis and hepatic enlargement necessitated continuous medical care. Death occurred at age 43 after 17 years of chronic cardiac invalidism . On physical examination the patient was emaciated and pale . The heart was markedly enlarged on percussion, with an increased apical thrust displaced to the left. No thrill was palpable. A to-and-fire, machinery murmur of grade 4 intensity and a peculiar superficial quality was audible in the third and fourth interspaces with equal intensity at the right and left sternal borders . There was wide radiation of both systolic and diastolic components . The
Retrograde aortogram demonstrates aneurysmal mass with dilated branches .
848
Carmichael and Davidson
ro . 2 . Ca . Later sta c in retrograde aortogram more clearly outlining the aneurysmal vessel .
Fro . 4 . Case 1 . artery (arrow) . Fin . 3 . Case 1 . Tortuous . dilated vessels over the surface of the heart . blood pressure was 125/30 mm . Hg . Duroziez' sign was positive . Sinus rhythm with occasional premature contractions was maintained until immediately before death when atria]l fibrillation supervened . The electrocardiogram showed left axis deviation, left ventricular enlargement ("strain" pattern) and atrial enlargement . A roentgenogram of the chest showed marked, generalized cardiac enlargement with bilateral hilar congestion . Autopsy Findings: The heart weighed 1,200 gm . with dilatation of all chambers and marked left ventricular hypertrophy . A tortuous, cirsoid left anterior de-
eurysm of the right coronary
scending branch of the left coronary artery with fistulotts communication through the great cardiac vein to the right heart was noted . The right coronary artery was similarly tortuous and markedly enlarged . A small branch of the right coronary artery terminated in a huge aneurysmal mass measuring 5 .5 by 9 cm . which connected directly with the right atrium and with several anterior cardiac veins . The aneurysm was partially filled with laminated antemortem clot . CASE 3 . A 16 year old girl had a heart murmur discovered as an incidental finding during an episode of pneumonia at 18 months of age . As a young child she would not play as vigorously as her fellows and THE AMERICAN JOURNAL OF CARDIOLOGY
Coronary Arteriovenous Fistula
849 TABLE 11
Catheterization Data in Case 2
Measurement
Fro . 5 . Case 3 . Posteroanterior chest roentgenogram demonstrating widening of the base of the heart . voluntarily sought more rest . Respiratory infections were frequent . At 37 months of age she underwent operation with a preoperative diagnosis of patent do( tus arteriosus . A patent vessel 1 .5 cm . in length and 0.3 cut. i n diameter was triply ligated . This procedure did not obliterate a pronounced thrill over the base of the heart . The pericardium was opened and the surgeon described "dilation of the pulmonary artery as well as both auricles and a vigorous systolic thrill in the region of the root of the pulmonary artery and over the right auricle ." Postoperatively, a toand-ho murmur remained audible . For 1 year the child remained on digitalis therapy . At the time of examination she was experiencing exercise fatigue, recurrent palpitation, recurrent pain in the left anterior portion of the chest and an increased susceptibility to respiratory infection . On physical examination her height was 67 inches, weight 128 pounds, and vital capacity 3,000 cc . No cyanosis, clubbing, or precordial asymmetry were present . The heart was enlarged to percussion . 'I he retromanubrial pulsation was markedly increased and a systolic thrill was present over the left carotid artery . A continuous precordial thrill was maximal at the third left interspace at the left sternal border. A grade 5 to 6 to-and-fro machinery murmur was maximal at this point and was widely audible over the entire precnrdium . There was sinus rhythm at 80 per minute and the blood pressure was 130/ 45 mm . Hg . The electrocardiogram presented voltage evidence of left ventricular enlargement. Chest roentgenogram and fluoroscopy (Fig . 5) demonstrated cardiac enlargement with an enlarged, widely pulsatile aorta . Dilation of the pulmonary arteries with an expansile pulsation bilaterally was noted . A phonocardiogram (Fig . 6) revealed a continuous systolic and diastolic DECEMBER
1961
Superior versa eava Right atrium (high) Right atrium (mid) Right atrium (low) Right ventricle (inflow) Right ventricle (mid) Right ventricle (infundibulum) Main pulmonary artery Right pulmonary artery Pulmonary wedge Brachial artery
Pressure (mm . Hg)
5
O s Content (volume per cent) 12 .29 12 .66 12 .80 12 .55
40/0--5 (17) 43/0-5 (17)
14 .95 15 .59
29/0-5
16 .15
2 7 /13 (20)
15 .64
29/13 (20) (10) 114/69 (88)
15 .50
System blood flow Pulmonary blood flow A-V shunt Cardiac index Pulmonary index Pulmonary arteriolar resistance Total pulmonary resistance Total peripheral resistance
17 .61 (95 .6)
5 .34 L ./min . 13 .40 L./min . 60 per cent 3 .20 L ./min ./Mr 8 .02 L ./min ./M2 60 dynes s ec . c m. -6 119 dynes s ec . cm . -5 1318 dynes sec . c m .'
murmur, maximal in the third and fourth left intercostal spaces at the left sternal border . Cardiac catheterization provided the following data : Hemoglobin Hematocrit . . . . . . Ventilation . . . . . pCOs Oxygen capacity Oxygen uptake Respiratory quotient
13.7 mg. per cent 44 per cent 7,43 4 .0 L . per minute per Ms 264 cc . 37 .0 mm . Hg 18 .42 volume per cent 158 ml. per minute per M2 0 .7
A moderate elevation in right ventricular pressure and a huge left to right shunt at the ventricular level were observed (Table it) . Retrograde aortography (Fig. 7) demonstrated opacification of a vascular structure apparently arising from the root of the aorta, progressing superiorly and describing an arc to the right . This structure was considered to represent a markedly dilated right coronary artery . Surgical Findings : Exploratory thoracotomy was done by Dr . Glen Gibbons, with the pump-oxygenator in readiness . A single, enlarged, thin-walled vessel was found at the base of the aorta representing
850
Carmichael and Davidson
Fro . 6 .
Case 3 . Pre- and postoperative phonocardiographic tracings .
the entire right coronary artery medially placed from its normal position (Fig . 8) . The structure was 3 cm . in length with the initial 1 .5 cm. directed superiorly, where it described a tight arc and plunged inferiorly into the outflow tract of the right ventricle just proximal to the pulmonary valve . No branches were identified . The aneurysmal vessel was exposed until it could be clamped proximally and distally . Oximetry confirmed the rise in saturation at the ventricular level . The electrocardiogram was monitored for five minutes after clamping the vessel and no change occurred . The aneurysm was then divided in its midportion and the two ends oversewn (Fig . 9) . The patient tolerated the procedure well and her postoperative course has been uncomplicated . Phono-
7. Case 3 . Retrograde aortogram with arrow pointing to dilated right coronary artery . FIG .
cardiography (Fig . 6B) demonstrated obliteration of the continuous murmur . COMMENTS
The lesion represents a fistulous tract between the coronary arterial circulation and either the coronary venous system, right atrium, right ventricle or pulmonary artery . Steinberg et al .' postulate two forms of embryologic development . One represents an arrest in the normal development of the coronary vessels with retention of primitive myocardial sinusoids . A second variation is composed of relatively mature vessels which can he identified as arteries and veins . Secondary changes in the vessels entering and leaving the area of fistulous communication result in the majority of the gross changes observed at surgery and many of the physical and laboratory findings which ease the clinical identification of the fistula . The importance of the abnormality to the afflicted patient has been a source of dispute . In a review of the relevant literature, 2-" data from which one may draw conclusions are available in thirty-three cases . In this group, twenty-five patients either died as a result of the lesion, gave objective evidence of increased cardiac work or had symptoms indicative of cardiac insufficiency . In a significant number of cases the lesion is important and factors dictating importance are the age of the patient and the size of the shunt . Davison ct al .'22 point out that congenital A-V aneurysms are known to increase gradually in size over the course of years and this necessitates a progressive rise in cardiac output in order to maintain an adequate THE AMERICAN JOURNAL OF CARDIOLOGY
Coronary Arteriovenous Fistula
851
Case 3 . The two clamps identify the oversewn ends of the aneurysmal right coronary artery . FIG . 9.
Case 3. The clamp identifies the aneurysmal right coronary artery arising from the root of the aorta . Fin . 8 .
orthodox circulation in the presence of an increasing shunt . Steinberg et al ." succinctly state : "Given sufficient shunt-volume acting over a long enough period of time, left ventricular hypertrophy and failure must inevitably occur ." This situation results in a high output failure with all of its usual consequences . Of interest in light of current thought is the fact that in spite of the high flow, high gradient and turbulence, little or no mention has been made of atherosclerotic plaques in the afferent arteries or efferent veins . Isolated subjects survive to old age . Colbeck and Shawt° report the survival of a patient who reached the advanced age of 85 years with only 18 months of cardiac insufficiency . The patient reported by White and Paul'" • 4 ' in 1949 remains asymptomatic in spite of the fact that his surgery was limited to an exploratory procedure with with no attempt at definitive correction . The opposite is more likely to prevail . Large shunt volumes have been described in several cases in which physiologic data are available . Valdivia" described a patient who died in chronic cardiac insufficiency at age 25 years . The heart weight at autopsy was 1,800 gm . In nine autopsy cases 3 the mean heart weight was 700 gm . Clinical Diagnosis : The lesion is usually discovered in one of two ways . In the analysis of a continuous murmur, an unusual location of the murmur and a peculiarly superficial quality suggest coronary arteriovenous fistula . A sizaDECEMBER
1961
ble number of cases have been found during an exploratory thoracotorny for presumed patent ductus artcriosus . Apart from the murmur, other physical findings are those anticipated with a left to right shunt . Suspicion must be aroused during auscultation or the diagnosis will be overlooked . The roentgenogram of the chest and the electrocardiogram customarily offer no assistance . Right heart catheterization demonstrating a left to right shunt is supportive evidence and the combination of a to-and-fro cardiac murmur in an unusual location coupled with this laboratory finding strongly suggests the lesion . Dye studies utilizing the technic of retrograde aortography or controlled coronary angiography present the greatest opportunity for definitive preoperative diagnosis . These findings are summarized by Fell et al ."' as a "diagnostic triad" of (1) abnormal location of a to-and-fro machinery murmur, (2) left-to-right shunt at the atrial or ventricular level and (3) a large, tortuous coronary artery seen by angiocardiography or aortography . Indications for and Results of Surgery : The complications dictate the necessity for an effort at corrective treatment . These include cardiac insufficiency, bacterial endocarditis, myocardial ischemia and thrombosis in the anomalous vessel and aneurysm . Including the three cases presented in this review the world literature includes a total of forty-six verified cases . Since the relationship between congenital aneurysm of the coronary arterial system and fistulous connection with the venous system has been appreciated for such a short period of time, surgical correction of the problem has been a relatively recent innovation . Including Cases 1 and 3 in this report, fifteen
852
Carmichael and Davidson
patients have had successful ligation
of
the fistu-
lous vessels . One surgical death occurred in a child who had a concomitant ligation of a patent ductus arteriosus and was assumed to have died as a result of pulmonary hypertension 3 4
Five
other operations were limited to exploration without attempted ligation . The fear of myocardial ischemic changes with ligation
of
the
shunt-bearing vessels as postulated by Davison et a1
22
has not proved to be a problem with the
exception of the case described by Michel and Herbst 2 5 Our own experience emphasizes the value of careful oximetry following ligation of all apparent communications with the right heart chambers, as multiple shunts may be present .
SUMMARY Three verified cases of congenital coronary arteriovenous fistula have been described, bringing the total reported cases to forty-six . The combination
of
a machinery murmur in an
unusual location, catheterization evidence of a left to right shunt, and opacification of a dilated coronary artery by angiography confirms the clinical diagnosis . Surgical correction has proved to be safe and effective. The number of surgical cures (including two cases reported herein) now totals fifteen cases with one death due to surgery . REFERENCES 1 . GRIPEITH, T . W . Remarks on aneurism of the coronary arteries of the heart . Brit . M. .7„ 1 : 266, 1901 . 2 . TREVOR, R. S . Aneurysm of the descending branch of the right coronary artery, situated in the wall of the right ventricle and opening into the cavity of the ventricle, associated with great dilatation of the right coronary artery and non-valvular infective endocarditis . Proc . Roy . Soc . Med., 5 :20,1911-12 . 3 . STEINBERG, I ., BALDWIN, J. S . and DOTTER, C . T . Coronary arterio-venous fistula. Circulation, 17 : 372,1958 . 4 . BLAREWAY, H . A hitherto undescribed malformation of the heart . J. Anat ., 52 : 354, 1917 . 5 . WILSON, J . G. and GRANT, R, T . A case of congenital malformation of the heart in an infant associated with partial heart block . Heart, 12 : 295,1925-26 . 6 . HALPERT, B . Arteriovenous communication between the right coronary artery and the coronary sinus . Heart, 15 : 129, 1930 . 7 . LOWENHEIM, I . Eine seltene Missbildung der Coronargefasse. Frankfurt. Ztschr. Path ., 43 : 63,1932 . 8 . NAGOYA, M . and TAKAHASHI, H. Aneurysma Serpentinum de linken Doron Arterie . Tr. Japanese Path . Soc ., 22 : 583, 1932 .
9 . HARRIS, P . N . Aneurysmal dilatation of the cardiac coronary arteries : Review of the literature and report of a case . .4m . J. Path ., 13 : 1937, 10 . BJoRK, C . and CRArooRD, C . Artcriovenous aneurysm of the pulmonary artery simulating patent ductus arteriosus Botalli . Thorax, 2 : 65, 11 . VALDIVIA, E ., ROWE, G . C . and ANGLVTNE, D . M. Large congenital aneurysm of the right coronary artery . Arch. Path ., 63 : 168, 1947 . 12 . EMMINGER, E . Arterio-venoses Aneurysma de rechten Herzkranzschlagader . Klin, med, 2 : 652,1947 . 13 . SCOTT, D . H ., Aneurysm of the coronary arteries. Am . Heart J., 36 : 403, 1948 . 14 . PAUL, 0., SWEET, R . H . and WHITE, P. D . Coronary arteriovenous fistula : Case report . Am . Heart J., 37 : 441, 1949 . 15 . BROWN, R . C . and BURNETT, J . D . Anomalous channel between aorta and right ventricle. Report of a case . Pediatrics, 3 : 597, 1949 . 16 . ESPINO VELA, J ., VELAZQUEZ, 'F. and FUENMAYOR, A. Amplia communication congenita de la aorta con el ventrie to derecho a traves de la arteria coronaria derecha anomala. Arch . Inst. cardiol . Mexico, 21 : 686, 1951 . 17 . WILLIAMS, R . R ., KENT, G . B ., JR . and EDWARDS, J. F . Anomalous cardiac blood vessel communicating with right ventricle . Observations in case of pulmonary atresia with intact ventricular septum . Arch . Path ., 52 : 480, 1951 . 18 . ALEXANDER, W . S . and GREEN, H . C . Coronary blood vessel arising from cardiac ventricle . Report of case showing other cardiac anomalies . Arch . Path., 53 : 187, 1952 . 19 . BAYLISS, J . H . and CAMPBELL, M . An unusual cause for a continuous murmur . Guy's Hasp . Rep ., 101 : 174, 1952 . 20 . COLBECK, J . C . and SHAW, J . M . Coronary aneurysm with arteriovenous fistula . Am . Heart J., 48 : 270, 1954 . 21 . LoviTT, W . V ., JR . and LUTZ, S ., JR . Embryological aneurysm of the myocardial vessels . Arch . Path., 57 : 163, 1954 . 22 . DAVISON, P . H ., MCCRACKEN, B . H . and MeILVEEN, D . J. S . Congenital coronary arteriovenous aneurysm . Brit. Heart J., 17 : 569, 1955 . 23 . SONDERGAARD, T. Henry Ford Hospital International Symposium in Cardiovascular Surgery, p .490, Philadelphia, 1955 . W. B. Saunders Co . 24 . DAvis, C ., DILLON, R . F ., FELL, E . G. and GASUL, B. M. Anomalous coronary artery simulating patent ductus arteriosus. J.A.M.A . . 160 :1047, 1956 . 25 . KNOBLICH, R . and RAWSON, A . J . Arteriovenous fistula of the heart . Am . Heart .1., 52 :474, 1956 . 26 . MozEN, H . E . Congenital cirsoid aneurysm of a coronary artery with associated arterio-atrial fistula, treated by operation : A case report . Ann . Surg., 144 : 215, 1956 . 27 . COOLEY, R . M . and SLOAN, R. B . Radiology of the Heart and Great Blood Vessels, p . 226 . Baltimore, 1956 . Williams & Wilkins. 28 . MICHEL, D . and HERBST, M . ZGSdm. Kreislaufrmsch ., 46 : 538, 1957 . THE AMERICAN JOURNAL OF CARDIOLOGY
Coronary Arteriovenous Fistula 29 . WALTHER, R . J ., STARKEY, G . y'V. B ., ZERVOPOLUS, E . and GTRAONS, G . A . Coronary arteriovenous fistula . Clinical and physiologic report on two patients, with review of the literature . Am . J . Med., 22 : 213, 1957 . 30 . KITTLE, C . F . In ; discussion of paper by GASUL, B . M ., FELL . E . G., MOREANO, M . and WEINBERO, M ., JR . Congenital coronary arteriovenous aneurysm . Arch . Surg., 78 :203, 1958 . 31 . FELL, E., WEINBERG . M ., GORDON, A . S ., GASUL, B . M . and .foaesos, F . R . Surgery for congenital coronary artery arteriovenous fistulae . Arch . Sing ., 77 : 331, 1958 . 32 . EDWARDS, J . E ., CLADDING, T . C . and WEIR, A . B ., JR . Congenital communication between the right coronary artery and the right atrium . J. Thoraric Surg ., 35 : 662, 1958 . 33 . SANGER, P . W ., TAYLOR, F . H . and RoBarsEK, F . Cited in reference 34 .
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34. Bosun, L . H ., VASLI, S ., MOUE, C . M . and BELTER, L . F . Congenital coronary arteriovenous fistula associated with large patent ductus . Circulation, 20 : 254, 1959 . 35 . GASUL, B . M ., ARCILLA, R . A ., LYNFIELU, J ., BICOFF, J . P . and LOAN, L . L . Congenital coronary arteriovenous fistula : Clinical, angiocardiographic, and physiologic findings in five patients . Abstracts of the 32nd Scientific Sessions, American Heart Association . Circulation, 20 : 700, 1959 (abs.) . 36 . GR06, M . and Korn . E . Congenital aneurysm of the coronary artery . Arch . Dis, Childhood, 34 : 8, 1959 . 37 . GROSS, R. E. Cited in reference 14 . 38 . GAROMELLA, J . J . Cited in reference 32 . 39 . MoRRow, A . G . Cited in reference 32 . 40. JOHNSON, J . Cited in reference 24 . 41 . WHITE, P . I) . Personal communication, 1961 .