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Coronary arterial-left atrial fistula
Coronary Arterial-Left Atrial Fistula Case with Obstruction of the Inferior Vena Cava by a Giant Left Atrium
WALTER L. FLOYD, MD W . GLENN YOUNG, MD and IRWIN S . JOHNSRUDE, MD Durham, North
Carolina
A 64 year old man with a life-long history of known heart disease and irregular heart action presented with a continuous parasternal bruit, a massively enlarged heart and incomplete inferior vena can obstruction . Angiocardiograms demonstrated marked distortion of all of the heart chambers, particularly the right atrium, and partial obstruction of the inferior vena cava by a large mass presumably invading the heart . Aortogram demonstrated a large anomalous branch of the right coronary artery, but it was not possible to determine angiographically the termination of the vessel . On exploratory thoracotomy, the mass distorting the heart was determined to be a giant left atrium that was presumably the result of a coronary arteriovenous fistula between the right coronary artery and the left atrium . The angiocardiographic findings are presented in detail, and the postulated factors in the development of giant atriomegaly are discussed .
Refined angiographic techniques that allow accurate anternortem definition of a variety of coronary arterial anomalies and the successful surgical treatment of the majority of these abnormalities have led to a clearer understanding of the congenital fistulas of the coronary arteries . The clinical picture produced by these lesions varies and depends not only on the size of the communication but also on the origin and termination of the fistulous tract . We recently encountered a patient with a coronary arteriovenous fistula that had its origin in the right coronary artery and its termination in the left atrium . A giant left atrium, presumably the result of the fistula, produced a striking distortion of the other heart chambers and incomplete occlusion of the inferior vena cava . The unusual and confusing clinical and angiographic findings that led to an incomplete diagnosis before surgical exploration are described-
Case Report From the Departments of Medicine, Surgery and Radiology, Duke University Medical Center, Durham, N . C . Manuscript received December 18, 1968, accepted January30, 1969. Address for reprints ; Walter L . Floyd, MD, Department of Medicine, Division of Cardiovascular Disease, Duke University Medical Center, Durham, N . C . 27706 .
716
A 64 year old auto mechanic was first told of a heart murmur at age 18 years . In 1932 he noted irregular palpitation of his heart, and in 1934, at the time of a tonsillectomy, he was told by his physician that his heart was irregular . In 1957 he underwent a minor urologic surgical procedure and was given digitalis preoperatively . In 1958 he was seen as an out-patient at Duke Hospital primarily for complaints of arthralgia involving the hips and shoulders . The examining physician noted irregular heart rhythm and a left parasternal systolic murmur .
The American Journal of CARDIOLOGY
CORONARY ARTERIAL-LEFT ATRIAL FISTULA
The electrocardiogram showed atrial fibrillation . The chest roentgenogram was described as showing a moderately enlarged heart and a double density along the right cardiac border . In 1959 the patient underwent ligation and stripping of varicose veins for varicosities that had been present most of his adult life . In 1965 he noted postprandial abdominal bloating, increased frequency of stools and appearance of hemorrhoids that occasionally bled . He also noted increased prominence of veins over his abdomen and chest . In November 1965, on examination at the Watson Clinic in Lakeland, Fla ., he was noted to have a continuous murmur along the lower sternum and radiologic evidence of increased heart size compared with previous films made in 1958 . During the year before admission to Duke Hospital on November 28, 1966, he had noted increasing abdominal complaints accompanied by postural edema, fatigue and mild dyspnea with exertion . Physical examination demonstrated a blood pressure of 120/70 mm Hg and a grossly irregular apical pulse rate of 78 beats/min . No clubbing, spider nevi, palmar erythema nor scleral icterus was evident . The veins over the abdomen and anterior thorax were prominent and filled from below . Neck veins were not distended, and only small irregular v waves were noted in the jugular venous pulse . The apex impulse was palpable at the sixth interspace in the anterior axillary line, and retromanubrial dullness was increased by several centimeters to the right of the sternum . An early diastolic filling sound was audible at the apex . A grade 3/6 continuous murmur was heard maximally in the fourth and fifth left parasternal space . The pulmonary second sound was normal, The liver was massively enlarged and palpated a full handbreadth below the right costal margin . There was no overlying bruit. The spleen tip was palpable . No fluid wave
Figure 1 . Electrocardiogram on admission demonstrating atrial fibrillation and QRS abnormality compatible with old anterior myocardial infarction . pear elevated . The lung vascularity was normal . On fluoroscopy no pulsation of the right heart border could be discerned . The patient underwent right heart catheterization
was evident . Large varicosities extended from the lower legs up over the thighs and above the inguingl ligaments bilaterally . Stasis pigmentation of the skin and minimal edema were present in the pretibial regions and feet bilaterally . Pertinent laboratory data demonstrated a hemato-
on December 1, 1966 . Right heart pressures including pulmonary venous wedge pressures were mildly elevated, and limited oxygen saturation data suggested a significant increase in oxygen saturation in the right atrium (Table I1 . However, it was not possible to obtain blood samples from the inferior vena cave .
crit of 49 vol/100 ml, hemoglobin of 15 g, and white blood cell count of 5,100/mm8 with normal differential count. Urinalysis was normal . Total plasma protein was 7 .0 g/100 ml with albumin fraction of 4 .0/100 rnl . Prothrombin time was 28 percent of normal control .
Biplane angiocardiograms were obtained, and the inferior vena cava was angiographically visualized by
Serum bilirubin was 2 .2 mg/100 ml with direct acting fraction of 1 .9 mg/100 ml . The electrocardiogram (Fig . 1) showed atrial fibrillation (ventricular rate 50/min), a Q wave in leads V, through V3 compatible with old anterior myocardial infarction, and S-T segment changes of digitalis effect . Chest roentgenograms in posteroanterior, left lateral, left and right oblique views (Fig . 2) demonstrated massive generalized cardiomegaly with predominant enlargement to the right of the spine . The esophagus was displaced posteriorly and was shown by fluoroscopy to be deviated to the left . The left main stem bronchus did not ap-
VOLUME 25, JUNE 1970
TABLE I Right Heart Catheterization Data
Site Superior vena cava Right atrium Right ventricle Pulmonary artery Pulmonary artery "wedge" Radial artery
Pressure (mm Hg)
Oxygen Saturation (y$)
13/3(8)' 35/4-11 35/10 (20)
59 72 74 74
(12) 120/80
46
* Mean pressures indicated by parentheses .
717
FLOYD ET AL .
Figure 2 . Chest roentgenograms on admission . A, posteroanterior view ; 8, left lateral view ; C, right anterior oblique view; D, left anterior oblique view . percutaneous puncture of the right femoral vein . The superior vena cava and right atrium, visualized by injection of contrast material into the superior vena cava, were enlarged and displaced anteriorly (Fig . 3, A and B) . The right atrium appeared displaced to such a degree that it was superimposed on the right ventricle in the lateral projection . A large unopaeified density surrounded the right lateral wall of the right atrium and extended from the superior vena cava caudad to the region of the inferior vena eava (Fig . 3A, small arrows) . This mass appeared to cause the anterior displacement of the superior vena cava and
right atrium and also appeared to invade the chamber causing a large filling defect within the body of the right atrium (Fig . 3A, black arrows) . On the right inferior lateral border of the right atrium there appeared an irregular outpouching that was thought possibly to represent an irregularity in the mass contour (Fig- 3A, open arrow) . Similar irregularity was noted on a lateral angiocardiogram in the inferior anterior aspect of the right atrium (Fig . 3B, open arrow) and in the nonopacified mass displacing the junction of the atrium and superior vena cava (Fig . 3B, closed arrows) .
Figure 3 . Angiocardiographic visualization of right atrium following injection into the superior vena cava : A, anterioposterior view. Note massively enlarged atrium with large central filling defect (black arrows) . A large unopacified density surrounds the lateral atrial wall (white arrows) and an irregular outpouching of the inferior atrial border (large open arrow). 8, lateral view . The right atrium is displaced anteriorly, and irregular filling defects are demonstrated at the junction of the superior vena cava (closed arrows) and the inferior atrial border (open arrow) .
71 8
The American Journal of CARDIOLOGY
CORONARY ARTERIAL-LEFT ATRIAL FISTULA
Figure 4. Angiographic visualization of the inferior vena cava . A, anteroposterior view. Incomplete obstruction by the nonopacified mass has produced marked dilatation and lateral displacement of dye into the atrium . B, lateral view . Arrows indicate dilated azygos vein . Angiographic visualization of the inferior venua cava (Fig . 4, A and B) showed that the vessel was markedly dilated and partially obstructed at its junction with the right atrium . Contrast material entered the atrium through a sliver-like curvilinear area, that appeared to lie laterally and to the left in the anteriorly displaced atrium (Fig . 4A) . Serial films showed filling of the right ventricle from this compartment . In comparing films made from the superior vena caval injection, it appeared that the area within the atrium, which was filling from the inferior vena cava, lay more inferiorly and laterally to the left than the area opacified from the superior vena cava . These observations indicated that the right atrium had been divided into compartments by a tumor-like mass . The azygos vein was dilated and tortuous, presumably as a result of accommodating the flow of the partially obstructed inferior vena cava (Fig . 4B, closed arrows) . Visualization of the left heart chambers and aorta was achieved by injection of contrast material into the main pulmonary artery . The left ventricle was densely opacified and moderately enlarged (Fig . 5), but it was considered to be posteriorly displaced when viewed in the lateral projection . The left atrium was not visualized in its entirety, and only the left border of the atrium seemed opacified by contrast material (Fig . 5, closed arrows) . This observation suggested that the left atrium was likewise involved in a nonopacifying tumor-like mass . Aortic filling by contrast material showed a large right coronary artery (Fig . 5, open arrow) An aortogram. on the day after right heart catheterization was obtained by passing a Gensini catheter into the aortic root from the right brachial artery . On aortic root injection, the left coronary artery was normal . The right coronary artery was noted to arise from the right coronary cusp and was of very large diameter (Fig . 6, A and B, open arrow) . It coursed anteriorly and to the right and faded away fairly abruptly ; this finding made us suspect that it was
VOLUME 25, JUNE 1970
emptying into a large chamber . A descending branch of this vessel-presumably the right coronary artery -appeared of normal caliber and coursed downward to the diaphragmatic surface of the heart (Fig. 6B, closed arrow) . The patient had a chill shortly after contrast injection which necessitated termination of the procedure and prevented further visualization of the coronary artery by selective catheterization . At this point, our initial clinical impression of coronary arteriovenous fistula seemed confirmed ; even though we had riot visualized the termination of the fistulous tract, we believed that it emptied into the right atrium because of the oxygen saturation data and the statistical likelihood of its termination in this chamber . However, we were perplexed by the marked distortion of the heart chambers and occlusion of the inferior vena cava . The presence of an invasive to-
Figure 5 . Left heart and aorta visualized by injection into the pulmonary artery . Note opacification of left lateral and inferior border of the left atrium (closed arrows) . A large right coronary artery (open arrow) is seen to arise from the aorta .
719
FLOYD ET AL .
Figure 6 . Aortogram obtained by aortic root injection . A, anteroposterior projection . Large right coronary artery is indicated by open arrows . B, Lateral projection . Large right coronary artery indicated by open arrow, and smaller normal-sized branch is identified by closed arrows . mor mass was the most likely explanation, and we considered the possibility of a hemangiosarcoma arising in the angiomatous malformation of the anteriovenous fistula . Exploratory thoracotomy was performed on December 19, 1966, through a median sternotomy incision . It was immediately apparent that all of the distortion was being produced by a . massively enlarged left atrium that displaced the right atrium anteriorly and to the left and extended inferiorly to produce partial occlusion of the inferior vena cava (Fig. 7) . Digital exploration of the right atrium failed to demonstrate an intra-atrial mass . There was a continuous thrill over the left atrium to the left of the interatrial groove at the entrance of right pulmonary veins . The right coronary artery was approximately
Figure 7. Artists drawing of distortion of right heart by giant left atrium . This illustration is best compared with angiographic findings shown in Figure 3B . LV.C . = inferior vena cava ; S .V .C . = superior vena cava ; V . =vein .
720
15 uim in diameter and gave off a normal-sized branch that coursed along the atrioventricular groove . A large anomalous branch went superiorly from this junction posteriorly to the right atrium and emptied into the left atrium near the interatrial groove . When this large coronary branch was compressed, the thrill disappeared, and the left atrium became less tense . Temporary occlusion was well tolerated so the vessel was ligated with multiple sutures . The postoperative course was uncomplicated . In an attempt to prevent thrombus formation in the huge, fibrillating left atrium which was no longer accepting the large shunt volume of the coronary arteriovenous fistula, anticoagulation with warfarin was initiated and maintained after discharge . The patient was able to return to part-time work in January 1967, and he felt well until April 1967 when he again became aware of postprandial bloating and abdominal swelling that was particularly prominent in the right upper abdomen . He noted increased frequency of bowel movements and small volume liquid stools that on occasion were streaked with blood . Subsequently, he observed further increase in the size of the veins on his abdomen and chest . His weight and-exercise tolerance remained unchanged . In June 1967, when he was last examined at Duke Hospital, the striking finding was a marked increase in the degree of dilatation of the veins over the abdomen, anterior thorax and the lateral thoracic veins . The varicosities over the legs were unchanged . The liver was massively enlarged ; its size on percussion was 20 .0 cm from superior to inferior border . Over the right thorax and extending to the midsternal region there was a continuous low-pitched bruit that was interpreted as being venous in origin, The neck veins were not distended . As before, there was a striking increase in retromanubrial cardiac dullness, but the continuous bruit was no longer audible . A soft holosystolic murmur was heard in the xiphoid region . The apical protodiastolic gallop noted preoperatively was not heard . Although chest X-ray films again demonstrated massive atriomegaly, it was of less degree than before surgery .
The American Journal of CARDIOLOGY
CORONARY ARTERIAL-LEFT ATRIAL FISTULA
Discussion
block .6 Although left atrial pressure is not normal in
The varieties of anomalous communications between the coronary arteries and the cardiac chambers
complete heart block, the presence of prolonged and severe elevation of pressure is unlikely . The occurrence of significant left atrial enlargement in patients
or pulmonary trunk have been reviewed by Neufeld, et al? The involvement is relatively equally divided between the right and left coronary arteries . The sites of termination in order of frequency are the right atrium, right ventricle and pulmonary trunk . The fistulas arising from the. right, coronary
artery more
frequently enter the right atrium, and those originating from the left coronary artery are more apt to terminate in the right ventricle or pulmonary trunk . Agusti et al .- noted that only 6 cases of arteriovenous communication between left coronary artery and left atrium have been reported, and before their case report in 1967 the existence of a communication between the right coronary artery and the left atrium had not been observed . The failure to visualize completely the entire left atrium angiographically when the left ventricle was densely opacified by contrast material was a major factor in our failure to make a complete diagnosis of the lesion preoperatively . It also led us to postulate in error a space-occupying tumor invading the heart . The only explanation for the phenomenon appears to be some type of preferential streaming of a portion of the injected contrast material from the entrance of the pulmonary veins across the mitral valve, whereas that portion of the dye which became mixed in the voluminous rightward expansion of the atrium became so diluted as to be invisible . Factors causing giant left atrium : The occurence of a giant left atrium has been the subject of a number of reports, and there has been much speculation about its genesis, but no single causative factor has been defined . The majority of eases have been associated with mitral valve disease, and it is generally accepted that the giant left atrium is more likely to occur in association with mitral insufficiency' However, that a large regurgitant jet is not a prerequisite to its development is demonstrated by the well-documented occurrence in patients with predominant mitral stenosis 4 By the same token, there appears to be no clear correlation with elevation of left atrial pressure . Marked elevation of left atrial pressure without striking left atrial enlargement may exist for long periods in many patients with mitral stenosis or insufficiency secondary to isolated chordae tendineae rupture, 5 whereas those patients with giant atria usually have relatively low intraatrial pressures Indeed, progressive enlargement of the atrium in the presence of normal pressure has been documented 7 Furthermore, giant left atrium has been reported in the absence of valvular disease in a patient with complete heart
VOLUME 25, JUNE 1970
with a large blood flow through the left atrium and ventricle as a result of left to right shunts through a ventricular septal defect or patent ductus art .eriosus is also well recognized, but these atria are seldom more than modestly enlarged . Turbulent flow is recognized as causing enlargement of vascular structures in the presence of normal pressure,9 and vibration of the floor and walls of the atria has been postulated as a causative factor .4 The possibility that inherent or acquired differences in compliance and distensibility of the atrial walls play a role has been discussed? but such a hypothesis as yet eludes objective measurement . In all probability, the development of giant left atrium is not dependent on one factor alone . For instance, our patient unquestionably had a large turbulent flow of blood into the atrium with chronic, moderate elevation of left atrial pressure ; in addition, his age and the abnormal electrocardiogram indicate that he probably had myocardial disease which would be a factor in altering the distensibility characteristics of the myocardium . Of the previously reported cases of coronary arteriovenous fistulas terminating in the left atrium, significant left atriomegaly is mentioned in only 1, a 29 year old woman who had communications with both the pulmonary artery and left atrium ." The marked distortion of the heart by massive left atrial enlargement such as occurred in this case is not a phenomenon that we have previously observed . However, distortion of other structures in the chest and mediastinum by the enlarging left atrium may occur . Symptoms referable to esophageal compression and bronchial obstruction are the most frequent complications" but involvement of other structures more remote from the heart such as systolic pulsation of the right chest wall by the rightward enlargement of the left atrium 12 and erosion of the spine 13 have been reported . Surgical indications : Coronary arteriovenous fistulas are well tolerated by many patients who survive into adult life with little or no symptoms . However, these patients are subject to complications including congestive heart failure, bacterial endocarditis, distal embolization, angiopathic anemia or glomerulonephritis . This morbidity is believed to justify the ligation of all coronary arteriovenous fistulas whether or not they are symptomatic .'* In spite of massive cardiomegaly, our patient tolerated ligation without obvious ill effect, but he has suffered continued symptoms, primarily as a result of obstruction of the inferior vena cava by the massively enlarged left atrium . Had
721
FLOYD ET AL .
the patient undergone ligation in previous years, the atriomegaly perhaps would not have reached such proportions and the complication causing continued disability could have been avoided. Thus, massive
atriomegaly may be added to the list of complications of this rare anomaly of the coronary arteries an ii constitutes one of the indications for surgery in otherwise asymptomatic patients .
References 1 . Neufeld HN, Lester RG, Adams P Jr, et al : Congenital communication of a coronary artery with a cardiac chambar or the pulmonary trunk. Circulation 24:171-179, 1961 2 . Agusti R, Liebman J, Ankeney J, et al : Congenital right coronary artery to left atrium fistula . Amer J Cardiol 19:428-433, 1967 3 . DeSanctis RW, Dean DC, Bland EF : Extreme left atrial enlargement-some characteristic features . Circulation 29 :14-23, 1964 4 . Kent EM, Fisher DL, Ford WS, et al : Mitral valve surgery and left heart catheterization in giant left atrium . Arch Burg (Chicago) 73 :503-507, 1956 5 . Seltzer A, Kelly JJ, Vannitomby M, et al : The syndrome of mitral insufficiency due to isolated rupture of the chordae tendineae . Amer J Med 43 :822-836, 1967 6. Roberts WC, Ross RS, Eggleston JC, et al : Chronic mitral regurgitation of unresolved etiology in the elderly . Johns Hopkins Med J 122 :26-28, 1968 7. Braunwald E, Awe WC : The syndrome of severe mitral regurgitation with normal left atrial pressure .
7 22
Circulation 27 :29-35, 1963 8 . Peele AAF : Dilatation of the auricles resulting from heart block . Lancet 2 :1248-1249, 1929 9 . Holman EF : The dynamic phenomena of the large vessels and the mechanism of post-stenotic dilatation, Vol I in Cardiology (Luisada AA, ed), New York, McGraw-Hill, p 2-146, 1959 10 . Sloman G, Macphee A, Fairley K : An unusual arterio . cameral fistula . Amer J Cardiol 15 :856, 1965 11 . Nichols cr, Ostrum HW : Unusual dilatation of the left auricle . Amer Heart J 8 :205-216, 1932 12 . Dressler W : Pulsations of the wall of the chest . V . Pulsations associated with mitral regurgitation and aneurysmal dilatation of the left auricle . Arch Intern Med (Chicago) 60 :663-667, 1937 13 . Ashworth J, Morgan JA : Aneurysmal dilatation of the left auricle with erosion of the spine . Brit Heart J 8 :207-211, 1946 14 . Sabiston DC : Direct surgical management of congenital and acquired lesions of the coronary circulation . Progr Cardiovasc Dis 6:299-316, 1963
The American Journal of CARDIOLOGY
WALTER L. FLOYD, MD W . GLENN YOUNG, MD and IRWIN S . JOHNSRUDE, MD Durham, North
Carolina
A 64 year old man with a life-long history of known heart disease and irregular heart action presented with a continuous parasternal bruit, a massively enlarged heart and incomplete inferior vena can obstruction . Angiocardiograms demonstrated marked distortion of all of the heart chambers, particularly the right atrium, and partial obstruction of the inferior vena cava by a large mass presumably invading the heart . Aortogram demonstrated a large anomalous branch of the right coronary artery, but it was not possible to determine angiographically the termination of the vessel . On exploratory thoracotomy, the mass distorting the heart was determined to be a giant left atrium that was presumably the result of a coronary arteriovenous fistula between the right coronary artery and the left atrium . The angiocardiographic findings are presented in detail, and the postulated factors in the development of giant atriomegaly are discussed .
Refined angiographic techniques that allow accurate anternortem definition of a variety of coronary arterial anomalies and the successful surgical treatment of the majority of these abnormalities have led to a clearer understanding of the congenital fistulas of the coronary arteries . The clinical picture produced by these lesions varies and depends not only on the size of the communication but also on the origin and termination of the fistulous tract . We recently encountered a patient with a coronary arteriovenous fistula that had its origin in the right coronary artery and its termination in the left atrium . A giant left atrium, presumably the result of the fistula, produced a striking distortion of the other heart chambers and incomplete occlusion of the inferior vena cava . The unusual and confusing clinical and angiographic findings that led to an incomplete diagnosis before surgical exploration are described-
Case Report From the Departments of Medicine, Surgery and Radiology, Duke University Medical Center, Durham, N . C . Manuscript received December 18, 1968, accepted January30, 1969. Address for reprints ; Walter L . Floyd, MD, Department of Medicine, Division of Cardiovascular Disease, Duke University Medical Center, Durham, N . C . 27706 .
716
A 64 year old auto mechanic was first told of a heart murmur at age 18 years . In 1932 he noted irregular palpitation of his heart, and in 1934, at the time of a tonsillectomy, he was told by his physician that his heart was irregular . In 1957 he underwent a minor urologic surgical procedure and was given digitalis preoperatively . In 1958 he was seen as an out-patient at Duke Hospital primarily for complaints of arthralgia involving the hips and shoulders . The examining physician noted irregular heart rhythm and a left parasternal systolic murmur .
The American Journal of CARDIOLOGY
CORONARY ARTERIAL-LEFT ATRIAL FISTULA
The electrocardiogram showed atrial fibrillation . The chest roentgenogram was described as showing a moderately enlarged heart and a double density along the right cardiac border . In 1959 the patient underwent ligation and stripping of varicose veins for varicosities that had been present most of his adult life . In 1965 he noted postprandial abdominal bloating, increased frequency of stools and appearance of hemorrhoids that occasionally bled . He also noted increased prominence of veins over his abdomen and chest . In November 1965, on examination at the Watson Clinic in Lakeland, Fla ., he was noted to have a continuous murmur along the lower sternum and radiologic evidence of increased heart size compared with previous films made in 1958 . During the year before admission to Duke Hospital on November 28, 1966, he had noted increasing abdominal complaints accompanied by postural edema, fatigue and mild dyspnea with exertion . Physical examination demonstrated a blood pressure of 120/70 mm Hg and a grossly irregular apical pulse rate of 78 beats/min . No clubbing, spider nevi, palmar erythema nor scleral icterus was evident . The veins over the abdomen and anterior thorax were prominent and filled from below . Neck veins were not distended, and only small irregular v waves were noted in the jugular venous pulse . The apex impulse was palpable at the sixth interspace in the anterior axillary line, and retromanubrial dullness was increased by several centimeters to the right of the sternum . An early diastolic filling sound was audible at the apex . A grade 3/6 continuous murmur was heard maximally in the fourth and fifth left parasternal space . The pulmonary second sound was normal, The liver was massively enlarged and palpated a full handbreadth below the right costal margin . There was no overlying bruit. The spleen tip was palpable . No fluid wave
Figure 1 . Electrocardiogram on admission demonstrating atrial fibrillation and QRS abnormality compatible with old anterior myocardial infarction . pear elevated . The lung vascularity was normal . On fluoroscopy no pulsation of the right heart border could be discerned . The patient underwent right heart catheterization
was evident . Large varicosities extended from the lower legs up over the thighs and above the inguingl ligaments bilaterally . Stasis pigmentation of the skin and minimal edema were present in the pretibial regions and feet bilaterally . Pertinent laboratory data demonstrated a hemato-
on December 1, 1966 . Right heart pressures including pulmonary venous wedge pressures were mildly elevated, and limited oxygen saturation data suggested a significant increase in oxygen saturation in the right atrium (Table I1 . However, it was not possible to obtain blood samples from the inferior vena cave .
crit of 49 vol/100 ml, hemoglobin of 15 g, and white blood cell count of 5,100/mm8 with normal differential count. Urinalysis was normal . Total plasma protein was 7 .0 g/100 ml with albumin fraction of 4 .0/100 rnl . Prothrombin time was 28 percent of normal control .
Biplane angiocardiograms were obtained, and the inferior vena cava was angiographically visualized by
Serum bilirubin was 2 .2 mg/100 ml with direct acting fraction of 1 .9 mg/100 ml . The electrocardiogram (Fig . 1) showed atrial fibrillation (ventricular rate 50/min), a Q wave in leads V, through V3 compatible with old anterior myocardial infarction, and S-T segment changes of digitalis effect . Chest roentgenograms in posteroanterior, left lateral, left and right oblique views (Fig . 2) demonstrated massive generalized cardiomegaly with predominant enlargement to the right of the spine . The esophagus was displaced posteriorly and was shown by fluoroscopy to be deviated to the left . The left main stem bronchus did not ap-
VOLUME 25, JUNE 1970
TABLE I Right Heart Catheterization Data
Site Superior vena cava Right atrium Right ventricle Pulmonary artery Pulmonary artery "wedge" Radial artery
Pressure (mm Hg)
Oxygen Saturation (y$)
13/3(8)' 35/4-11 35/10 (20)
59 72 74 74
(12) 120/80
46
* Mean pressures indicated by parentheses .
717
FLOYD ET AL .
Figure 2 . Chest roentgenograms on admission . A, posteroanterior view ; 8, left lateral view ; C, right anterior oblique view; D, left anterior oblique view . percutaneous puncture of the right femoral vein . The superior vena cava and right atrium, visualized by injection of contrast material into the superior vena cava, were enlarged and displaced anteriorly (Fig . 3, A and B) . The right atrium appeared displaced to such a degree that it was superimposed on the right ventricle in the lateral projection . A large unopaeified density surrounded the right lateral wall of the right atrium and extended from the superior vena cava caudad to the region of the inferior vena eava (Fig . 3A, small arrows) . This mass appeared to cause the anterior displacement of the superior vena cava and
right atrium and also appeared to invade the chamber causing a large filling defect within the body of the right atrium (Fig . 3A, black arrows) . On the right inferior lateral border of the right atrium there appeared an irregular outpouching that was thought possibly to represent an irregularity in the mass contour (Fig- 3A, open arrow) . Similar irregularity was noted on a lateral angiocardiogram in the inferior anterior aspect of the right atrium (Fig . 3B, open arrow) and in the nonopacified mass displacing the junction of the atrium and superior vena cava (Fig . 3B, closed arrows) .
Figure 3 . Angiocardiographic visualization of right atrium following injection into the superior vena cava : A, anterioposterior view. Note massively enlarged atrium with large central filling defect (black arrows) . A large unopacified density surrounds the lateral atrial wall (white arrows) and an irregular outpouching of the inferior atrial border (large open arrow). 8, lateral view . The right atrium is displaced anteriorly, and irregular filling defects are demonstrated at the junction of the superior vena cava (closed arrows) and the inferior atrial border (open arrow) .
71 8
The American Journal of CARDIOLOGY
CORONARY ARTERIAL-LEFT ATRIAL FISTULA
Figure 4. Angiographic visualization of the inferior vena cava . A, anteroposterior view. Incomplete obstruction by the nonopacified mass has produced marked dilatation and lateral displacement of dye into the atrium . B, lateral view . Arrows indicate dilated azygos vein . Angiographic visualization of the inferior venua cava (Fig . 4, A and B) showed that the vessel was markedly dilated and partially obstructed at its junction with the right atrium . Contrast material entered the atrium through a sliver-like curvilinear area, that appeared to lie laterally and to the left in the anteriorly displaced atrium (Fig . 4A) . Serial films showed filling of the right ventricle from this compartment . In comparing films made from the superior vena caval injection, it appeared that the area within the atrium, which was filling from the inferior vena cava, lay more inferiorly and laterally to the left than the area opacified from the superior vena cava . These observations indicated that the right atrium had been divided into compartments by a tumor-like mass . The azygos vein was dilated and tortuous, presumably as a result of accommodating the flow of the partially obstructed inferior vena cava (Fig . 4B, closed arrows) . Visualization of the left heart chambers and aorta was achieved by injection of contrast material into the main pulmonary artery . The left ventricle was densely opacified and moderately enlarged (Fig . 5), but it was considered to be posteriorly displaced when viewed in the lateral projection . The left atrium was not visualized in its entirety, and only the left border of the atrium seemed opacified by contrast material (Fig . 5, closed arrows) . This observation suggested that the left atrium was likewise involved in a nonopacifying tumor-like mass . Aortic filling by contrast material showed a large right coronary artery (Fig . 5, open arrow) An aortogram. on the day after right heart catheterization was obtained by passing a Gensini catheter into the aortic root from the right brachial artery . On aortic root injection, the left coronary artery was normal . The right coronary artery was noted to arise from the right coronary cusp and was of very large diameter (Fig . 6, A and B, open arrow) . It coursed anteriorly and to the right and faded away fairly abruptly ; this finding made us suspect that it was
VOLUME 25, JUNE 1970
emptying into a large chamber . A descending branch of this vessel-presumably the right coronary artery -appeared of normal caliber and coursed downward to the diaphragmatic surface of the heart (Fig. 6B, closed arrow) . The patient had a chill shortly after contrast injection which necessitated termination of the procedure and prevented further visualization of the coronary artery by selective catheterization . At this point, our initial clinical impression of coronary arteriovenous fistula seemed confirmed ; even though we had riot visualized the termination of the fistulous tract, we believed that it emptied into the right atrium because of the oxygen saturation data and the statistical likelihood of its termination in this chamber . However, we were perplexed by the marked distortion of the heart chambers and occlusion of the inferior vena cava . The presence of an invasive to-
Figure 5 . Left heart and aorta visualized by injection into the pulmonary artery . Note opacification of left lateral and inferior border of the left atrium (closed arrows) . A large right coronary artery (open arrow) is seen to arise from the aorta .
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Figure 6 . Aortogram obtained by aortic root injection . A, anteroposterior projection . Large right coronary artery is indicated by open arrows . B, Lateral projection . Large right coronary artery indicated by open arrow, and smaller normal-sized branch is identified by closed arrows . mor mass was the most likely explanation, and we considered the possibility of a hemangiosarcoma arising in the angiomatous malformation of the anteriovenous fistula . Exploratory thoracotomy was performed on December 19, 1966, through a median sternotomy incision . It was immediately apparent that all of the distortion was being produced by a . massively enlarged left atrium that displaced the right atrium anteriorly and to the left and extended inferiorly to produce partial occlusion of the inferior vena cava (Fig. 7) . Digital exploration of the right atrium failed to demonstrate an intra-atrial mass . There was a continuous thrill over the left atrium to the left of the interatrial groove at the entrance of right pulmonary veins . The right coronary artery was approximately
Figure 7. Artists drawing of distortion of right heart by giant left atrium . This illustration is best compared with angiographic findings shown in Figure 3B . LV.C . = inferior vena cava ; S .V .C . = superior vena cava ; V . =vein .
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15 uim in diameter and gave off a normal-sized branch that coursed along the atrioventricular groove . A large anomalous branch went superiorly from this junction posteriorly to the right atrium and emptied into the left atrium near the interatrial groove . When this large coronary branch was compressed, the thrill disappeared, and the left atrium became less tense . Temporary occlusion was well tolerated so the vessel was ligated with multiple sutures . The postoperative course was uncomplicated . In an attempt to prevent thrombus formation in the huge, fibrillating left atrium which was no longer accepting the large shunt volume of the coronary arteriovenous fistula, anticoagulation with warfarin was initiated and maintained after discharge . The patient was able to return to part-time work in January 1967, and he felt well until April 1967 when he again became aware of postprandial bloating and abdominal swelling that was particularly prominent in the right upper abdomen . He noted increased frequency of bowel movements and small volume liquid stools that on occasion were streaked with blood . Subsequently, he observed further increase in the size of the veins on his abdomen and chest . His weight and-exercise tolerance remained unchanged . In June 1967, when he was last examined at Duke Hospital, the striking finding was a marked increase in the degree of dilatation of the veins over the abdomen, anterior thorax and the lateral thoracic veins . The varicosities over the legs were unchanged . The liver was massively enlarged ; its size on percussion was 20 .0 cm from superior to inferior border . Over the right thorax and extending to the midsternal region there was a continuous low-pitched bruit that was interpreted as being venous in origin, The neck veins were not distended . As before, there was a striking increase in retromanubrial cardiac dullness, but the continuous bruit was no longer audible . A soft holosystolic murmur was heard in the xiphoid region . The apical protodiastolic gallop noted preoperatively was not heard . Although chest X-ray films again demonstrated massive atriomegaly, it was of less degree than before surgery .
The American Journal of CARDIOLOGY
CORONARY ARTERIAL-LEFT ATRIAL FISTULA
Discussion
block .6 Although left atrial pressure is not normal in
The varieties of anomalous communications between the coronary arteries and the cardiac chambers
complete heart block, the presence of prolonged and severe elevation of pressure is unlikely . The occurrence of significant left atrial enlargement in patients
or pulmonary trunk have been reviewed by Neufeld, et al? The involvement is relatively equally divided between the right and left coronary arteries . The sites of termination in order of frequency are the right atrium, right ventricle and pulmonary trunk . The fistulas arising from the. right, coronary
artery more
frequently enter the right atrium, and those originating from the left coronary artery are more apt to terminate in the right ventricle or pulmonary trunk . Agusti et al .- noted that only 6 cases of arteriovenous communication between left coronary artery and left atrium have been reported, and before their case report in 1967 the existence of a communication between the right coronary artery and the left atrium had not been observed . The failure to visualize completely the entire left atrium angiographically when the left ventricle was densely opacified by contrast material was a major factor in our failure to make a complete diagnosis of the lesion preoperatively . It also led us to postulate in error a space-occupying tumor invading the heart . The only explanation for the phenomenon appears to be some type of preferential streaming of a portion of the injected contrast material from the entrance of the pulmonary veins across the mitral valve, whereas that portion of the dye which became mixed in the voluminous rightward expansion of the atrium became so diluted as to be invisible . Factors causing giant left atrium : The occurence of a giant left atrium has been the subject of a number of reports, and there has been much speculation about its genesis, but no single causative factor has been defined . The majority of eases have been associated with mitral valve disease, and it is generally accepted that the giant left atrium is more likely to occur in association with mitral insufficiency' However, that a large regurgitant jet is not a prerequisite to its development is demonstrated by the well-documented occurrence in patients with predominant mitral stenosis 4 By the same token, there appears to be no clear correlation with elevation of left atrial pressure . Marked elevation of left atrial pressure without striking left atrial enlargement may exist for long periods in many patients with mitral stenosis or insufficiency secondary to isolated chordae tendineae rupture, 5 whereas those patients with giant atria usually have relatively low intraatrial pressures Indeed, progressive enlargement of the atrium in the presence of normal pressure has been documented 7 Furthermore, giant left atrium has been reported in the absence of valvular disease in a patient with complete heart
VOLUME 25, JUNE 1970
with a large blood flow through the left atrium and ventricle as a result of left to right shunts through a ventricular septal defect or patent ductus art .eriosus is also well recognized, but these atria are seldom more than modestly enlarged . Turbulent flow is recognized as causing enlargement of vascular structures in the presence of normal pressure,9 and vibration of the floor and walls of the atria has been postulated as a causative factor .4 The possibility that inherent or acquired differences in compliance and distensibility of the atrial walls play a role has been discussed? but such a hypothesis as yet eludes objective measurement . In all probability, the development of giant left atrium is not dependent on one factor alone . For instance, our patient unquestionably had a large turbulent flow of blood into the atrium with chronic, moderate elevation of left atrial pressure ; in addition, his age and the abnormal electrocardiogram indicate that he probably had myocardial disease which would be a factor in altering the distensibility characteristics of the myocardium . Of the previously reported cases of coronary arteriovenous fistulas terminating in the left atrium, significant left atriomegaly is mentioned in only 1, a 29 year old woman who had communications with both the pulmonary artery and left atrium ." The marked distortion of the heart by massive left atrial enlargement such as occurred in this case is not a phenomenon that we have previously observed . However, distortion of other structures in the chest and mediastinum by the enlarging left atrium may occur . Symptoms referable to esophageal compression and bronchial obstruction are the most frequent complications" but involvement of other structures more remote from the heart such as systolic pulsation of the right chest wall by the rightward enlargement of the left atrium 12 and erosion of the spine 13 have been reported . Surgical indications : Coronary arteriovenous fistulas are well tolerated by many patients who survive into adult life with little or no symptoms . However, these patients are subject to complications including congestive heart failure, bacterial endocarditis, distal embolization, angiopathic anemia or glomerulonephritis . This morbidity is believed to justify the ligation of all coronary arteriovenous fistulas whether or not they are symptomatic .'* In spite of massive cardiomegaly, our patient tolerated ligation without obvious ill effect, but he has suffered continued symptoms, primarily as a result of obstruction of the inferior vena cava by the massively enlarged left atrium . Had
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the patient undergone ligation in previous years, the atriomegaly perhaps would not have reached such proportions and the complication causing continued disability could have been avoided. Thus, massive
atriomegaly may be added to the list of complications of this rare anomaly of the coronary arteries an ii constitutes one of the indications for surgery in otherwise asymptomatic patients .
References 1 . Neufeld HN, Lester RG, Adams P Jr, et al : Congenital communication of a coronary artery with a cardiac chambar or the pulmonary trunk. Circulation 24:171-179, 1961 2 . Agusti R, Liebman J, Ankeney J, et al : Congenital right coronary artery to left atrium fistula . Amer J Cardiol 19:428-433, 1967 3 . DeSanctis RW, Dean DC, Bland EF : Extreme left atrial enlargement-some characteristic features . Circulation 29 :14-23, 1964 4 . Kent EM, Fisher DL, Ford WS, et al : Mitral valve surgery and left heart catheterization in giant left atrium . Arch Burg (Chicago) 73 :503-507, 1956 5 . Seltzer A, Kelly JJ, Vannitomby M, et al : The syndrome of mitral insufficiency due to isolated rupture of the chordae tendineae . Amer J Med 43 :822-836, 1967 6. Roberts WC, Ross RS, Eggleston JC, et al : Chronic mitral regurgitation of unresolved etiology in the elderly . Johns Hopkins Med J 122 :26-28, 1968 7. Braunwald E, Awe WC : The syndrome of severe mitral regurgitation with normal left atrial pressure .
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Circulation 27 :29-35, 1963 8 . Peele AAF : Dilatation of the auricles resulting from heart block . Lancet 2 :1248-1249, 1929 9 . Holman EF : The dynamic phenomena of the large vessels and the mechanism of post-stenotic dilatation, Vol I in Cardiology (Luisada AA, ed), New York, McGraw-Hill, p 2-146, 1959 10 . Sloman G, Macphee A, Fairley K : An unusual arterio . cameral fistula . Amer J Cardiol 15 :856, 1965 11 . Nichols cr, Ostrum HW : Unusual dilatation of the left auricle . Amer Heart J 8 :205-216, 1932 12 . Dressler W : Pulsations of the wall of the chest . V . Pulsations associated with mitral regurgitation and aneurysmal dilatation of the left auricle . Arch Intern Med (Chicago) 60 :663-667, 1937 13 . Ashworth J, Morgan JA : Aneurysmal dilatation of the left auricle with erosion of the spine . Brit Heart J 8 :207-211, 1946 14 . Sabiston DC : Direct surgical management of congenital and acquired lesions of the coronary circulation . Progr Cardiovasc Dis 6:299-316, 1963
The American Journal of CARDIOLOGY