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Multiple coronary arteriosystemic fistulas
Multiple Coronary Arteriosystemic
Fistulas
A 58 year old man presented with angina pectoris and no heart murmur. On selective coronary angiography, multiple coronary arteriosystemic fistulas involving all three major coronary arteries were found. This is the first such case reported. It is speculated that this vascular anomaly represents persistence of embryonic intertrabecular vessels that permit the coronary arteries to communicate with the left ventricular chamber through the Thebesian vessels.
KOTA REDDY, MD MOHINDER GUPTA, MD ROBERT I. HAMBY, MD, FACC New Hyde Park, Jamaica and Stony Brook, New York
Congenital coronary fistula is not a rare anomaly.’ The majority of these fistulas form an arteriovenous connection between a coronary artery and the right heart chambers. In most reported cases, the abnormal fistula involves only one coronary artery. This report describes a unique case in which multiple arteriosystemic fistulas connected each of the coronary arteries with the left ventricle. Case Report
From the Department of Medicine, Cardiology Division, Long Island Jewish-Hillside Medical Center, New Hyde Park, N. Y.. Queens Hospital Center Affiliation, Jamaica, N. Y. and the School of Medicine, Health Sciences Center, State University of New York at Stony Brook, Stony Brook, N. Y. Manuscript accepted January 10, 1973. Address for reprints: Robert I. Hamby, MD, Cardiology Division, Long Island Jewish-Hillside Medical Center, New Hyde Park, N. Y. 11040.
304
February
1974
The American
Journal
A 58 year old man was admitted to the hospital for evaluation of recurrent chest pain of more than 10 years’ duration. Retrosternal pain occurred with physical exertion and was relieved by rest and administration of nitroglycerin. For the previous 2 years, the pain had increased in frequency and duration. occurring at rest and after meals. The patient entered another hospital 3 weeks before this admission because of chest pain lasting 20 minutes. After acute mvocardial infarction was ruled out. he was transferred for cardiac catheterization and selective coronary angiography. There was no history of dyspnea on exertion. paroxysmal nocturnal dyspnea, orthopnea, ankle swelling, high blood pressure or heart murmur. Past medical history and family history were negative. Physica/ examination revealed a blood pressure of’ 130/$0 mm Hg and a regular pulse of 70/min. The neck veins were flat and the lungs clear. Examination of the heart revealed no abnormal heaves or thrills. The point of maximal impulse was felt in the fifth left intercostal space and midclavicular line. A fourth heart sound was heard along the lower left sternal border. No murmurs were audible. Multiple blood tests, which included complete b!ood*count, lipid profile and cholesterol, urea, uric acid and serial enzyme determinations, were within normal limits. A glucose tolerance test indicated the presence i)f diabetes mellitus. Roentgenographic studies revealed no evidence of chamber enlargement. The electrocardiogram disclosed normal sinus rhythm and small R waves in leads V, to Vi1 and nonspecific abnormal S-T and T waves in the left precordial leads. Cardiac cathetuization revealed a normal cardiac output and increased left ventricular end-diastolic pressure (19 mm Hg). Use of a platinum tip electrode and hydrogen inhalation studies ruled out intracardiac left to right shunts. A left ventricular angiogram demonstrated a normal end-diastolic chamber and contractile pattern. Selective coronary angiography revealed that all the coronary arteries were dilated, elongated and tortuous (Fig. 1). Calcification involved the main left coronary artery and the proximal left anterior descending and left circumflex arteries. Studies of the right coronary artery demonstrated a markedly dilated posterior descending branch. The contrast material streamed into and opacified the left ventricular chamber
of CARDIOLOGY
Volume
33
CDRWARY ARTWIOSYSTEMII: FISNtAS-REDDY
FIGURE 1. Selective coronary angiographic study. Actual tine frames are shown at left and a sketch of the same frame is shown at right. A, selective right coronary angiogram in the left anterior oblique projection. B and C, selective left coronary angiograms in the left anterior oblique (B) and right anterior oblique (C) projections. These frames were taken during diastole. During systole, the contrast material in the left ventricular chamber was washed out in the aorta. Note that all the major arteries are dilated and elongated.
ET AL.
rick
through a maze of fine vessels (Fig. 1A). Similarly, opacification of the left anterior descending and circumflex arteries delineated a maze or plexus of small, fine vessels surrounding these arteries, with contrast material streaming into the left ventricular chamber and completely opacifying the cavity (Fig. 1, B and C). This opacification of the left ventricle occurred only during diastole with injection of contrast material into the right or left coronary artery; during systole, the contrast material was washed out into the aorta.
Discussion Coronary arterial fistulas usually arise from a single artery, most frequently the right coronary artery, and usually communicate with the right ventricle, right atrium or pulmonary artery.1 A review’ of 172 reported cases revealed only 4 in which the fistula involved both the right and left coronary arteries. In the case described here, all three major coronary arteries had fistulous connections with the left ventricle. A coronary arterial fistula emptying into the left side of the heart is uncommon; when it occurs, the fistulous connection tends to involve the left atrium rather than the left ventricle.’
In nine reported casesiof a coronary fistula emptying into the left ventricle, the right coronary artery was involved in three,1-3 the left anterior descending artery in one4 and the left circumflex artery in one.1 The involved coronary artery was not specified5 in one patient, and in three cases described by Abbott et al.,6 only the presence of an aberrant vessel was indicated, with no mention of involvement of the major coronary arteries. Thus, the present case is unique in that all three major coronary arteries formed an arteriosystemic fistula. Selective coronary angiograms demonstrated marked dilation of the right, left anterior descending and circumflex arteries. The diameter of these arteries at their origin (6, 7 and 6 mm, respectively) was almost twice that reported as normal by Baroldi and Scomazzoni.7 Coronary fistulas may be formed by a well defined vessel emptying into a cardiac chamber or, as in our case, by a plexus of multiple fine vessels. This vascular maze probably represents persistence of embryonic sinusoids that are usually obliterated in normal development. The myocardial vessels in the
February 1974
The American Journal of CARDIOLOGY
Volume 33
305
CORONARY
ARTERtOSYSTEMlC
FISTULAS-REDDY
ET AL.
adult heart are derived embryologically from both endothelial protrusions into the myocardium and coronary arteries and veins.8 This endothelial protrusion into the myocardium extends to the epicardial surface, forming an intertrabecular network. The outermost intertrabecular network becomes almost completely obliterated and forms a capillary network as the myocardium grows, whereas the innermost intertrabecular vessels retain their communication with the chambers of the heart, forming the Thebesians vessels of the adult heart. Investigators4T”,Y reporting earlier cases, in which sinusoidal communication existed between involved coronary arteries and the heart chamber, ascribed the anomaly to persistence of the outermost intertrabecular space, thereby permitting a communication between the coronary artery and heart chamber through the Thebesian vessels. A similar mechanism can explain the angiographic findings and diffuse involvement in our case. The reason for abnormal communication only with the left ventricle is not clear.
In our case, no murmur was heard suggesting a coronary artery fistula. Such fistulas have been associated with continuous’J or purely diastolic murmurs.Z In the case reported by Lovitt and Lutz,“ no heart murmur was mentioned and the fistula was discovered only on postmortem examination. The cause of death in that patient was not related to the fistula. The symptoms of angina pectoris in our case can be attributed to the coronary fistulas. The anticipated hemodynamic burden of arteriosystemic fistulas would be diastolic overload of the left ventricle. The normal pulse pressure and the absence of left ventricular hypertrophy in our case suggest t,hat there was no hemodynamically significant volume overload on the left ventricle. The patient’s symptoms should be treated medically. Acknowledgment Dr. Irwin Hoffman for reviewing
this manuscript and express gratitude for the assistance given by Mrs. Miriam Posner, Mr. Joshua Hamby and Mrs. Brenda Hamby. We thank
References 1. McNamara JJ, Gross RE: Congenital coronary artery fistula. Surgery 6559-69, 1969 2. Eguchi S, Nitta H, Asano K, et al: Congenital fistula of the right coronary artery to the left ventricle. The third case in the literature. Am Heart J 80:242-246. 1970 3. Tanabe T, lsomatsu T, Ota S, et al: Tortuous right coronary artery to the left ventricle. Jap J Thorac Surg .20:646-651. 1967 4. Levitt WV Jr, Lutz S Jr: Embryological aneurysm of the myocardial vessels. Arch Pathol 57:163-167, 1954 5. Bellet S, Gouley BA: Congenital heart disease with multiple cardiac anomalies. Report of a case showing aortic atresia, fibrous
306
February
1974
The American
Journal of CARDIOLOGY
6.
7.
8. 9.
scar in myocardium and embryonal sinusoidal remains. Am J Med Sci 183:459-465, 1932 Abbott OA, Rivarola CH, Logue RB: Surgical correction of coronary arteriovenous fistula. J Thorac Cardiovasc Surg 42:660672, 1961 Baroldl G, Scomazzonl G: Coronary Circulation in the Normal and the Pathologic Heart. Office of the Surgeon General, Depariment of the Army, Washington, DC, 1967, p 6. 10, 18 Grant RT, Regnier M: The comparative anatomy of the cardiac coronary vessels. Heart 13:285-317, 1926 Grant RT: An unusual anomaly of the coronary vessels in the malformed heart of a child. Heart 13:273-284, 1926
Volume 33
Fistulas
A 58 year old man presented with angina pectoris and no heart murmur. On selective coronary angiography, multiple coronary arteriosystemic fistulas involving all three major coronary arteries were found. This is the first such case reported. It is speculated that this vascular anomaly represents persistence of embryonic intertrabecular vessels that permit the coronary arteries to communicate with the left ventricular chamber through the Thebesian vessels.
KOTA REDDY, MD MOHINDER GUPTA, MD ROBERT I. HAMBY, MD, FACC New Hyde Park, Jamaica and Stony Brook, New York
Congenital coronary fistula is not a rare anomaly.’ The majority of these fistulas form an arteriovenous connection between a coronary artery and the right heart chambers. In most reported cases, the abnormal fistula involves only one coronary artery. This report describes a unique case in which multiple arteriosystemic fistulas connected each of the coronary arteries with the left ventricle. Case Report
From the Department of Medicine, Cardiology Division, Long Island Jewish-Hillside Medical Center, New Hyde Park, N. Y.. Queens Hospital Center Affiliation, Jamaica, N. Y. and the School of Medicine, Health Sciences Center, State University of New York at Stony Brook, Stony Brook, N. Y. Manuscript accepted January 10, 1973. Address for reprints: Robert I. Hamby, MD, Cardiology Division, Long Island Jewish-Hillside Medical Center, New Hyde Park, N. Y. 11040.
304
February
1974
The American
Journal
A 58 year old man was admitted to the hospital for evaluation of recurrent chest pain of more than 10 years’ duration. Retrosternal pain occurred with physical exertion and was relieved by rest and administration of nitroglycerin. For the previous 2 years, the pain had increased in frequency and duration. occurring at rest and after meals. The patient entered another hospital 3 weeks before this admission because of chest pain lasting 20 minutes. After acute mvocardial infarction was ruled out. he was transferred for cardiac catheterization and selective coronary angiography. There was no history of dyspnea on exertion. paroxysmal nocturnal dyspnea, orthopnea, ankle swelling, high blood pressure or heart murmur. Past medical history and family history were negative. Physica/ examination revealed a blood pressure of’ 130/$0 mm Hg and a regular pulse of 70/min. The neck veins were flat and the lungs clear. Examination of the heart revealed no abnormal heaves or thrills. The point of maximal impulse was felt in the fifth left intercostal space and midclavicular line. A fourth heart sound was heard along the lower left sternal border. No murmurs were audible. Multiple blood tests, which included complete b!ood*count, lipid profile and cholesterol, urea, uric acid and serial enzyme determinations, were within normal limits. A glucose tolerance test indicated the presence i)f diabetes mellitus. Roentgenographic studies revealed no evidence of chamber enlargement. The electrocardiogram disclosed normal sinus rhythm and small R waves in leads V, to Vi1 and nonspecific abnormal S-T and T waves in the left precordial leads. Cardiac cathetuization revealed a normal cardiac output and increased left ventricular end-diastolic pressure (19 mm Hg). Use of a platinum tip electrode and hydrogen inhalation studies ruled out intracardiac left to right shunts. A left ventricular angiogram demonstrated a normal end-diastolic chamber and contractile pattern. Selective coronary angiography revealed that all the coronary arteries were dilated, elongated and tortuous (Fig. 1). Calcification involved the main left coronary artery and the proximal left anterior descending and left circumflex arteries. Studies of the right coronary artery demonstrated a markedly dilated posterior descending branch. The contrast material streamed into and opacified the left ventricular chamber
of CARDIOLOGY
Volume
33
CDRWARY ARTWIOSYSTEMII: FISNtAS-REDDY
FIGURE 1. Selective coronary angiographic study. Actual tine frames are shown at left and a sketch of the same frame is shown at right. A, selective right coronary angiogram in the left anterior oblique projection. B and C, selective left coronary angiograms in the left anterior oblique (B) and right anterior oblique (C) projections. These frames were taken during diastole. During systole, the contrast material in the left ventricular chamber was washed out in the aorta. Note that all the major arteries are dilated and elongated.
ET AL.
rick
through a maze of fine vessels (Fig. 1A). Similarly, opacification of the left anterior descending and circumflex arteries delineated a maze or plexus of small, fine vessels surrounding these arteries, with contrast material streaming into the left ventricular chamber and completely opacifying the cavity (Fig. 1, B and C). This opacification of the left ventricle occurred only during diastole with injection of contrast material into the right or left coronary artery; during systole, the contrast material was washed out into the aorta.
Discussion Coronary arterial fistulas usually arise from a single artery, most frequently the right coronary artery, and usually communicate with the right ventricle, right atrium or pulmonary artery.1 A review’ of 172 reported cases revealed only 4 in which the fistula involved both the right and left coronary arteries. In the case described here, all three major coronary arteries had fistulous connections with the left ventricle. A coronary arterial fistula emptying into the left side of the heart is uncommon; when it occurs, the fistulous connection tends to involve the left atrium rather than the left ventricle.’
In nine reported casesiof a coronary fistula emptying into the left ventricle, the right coronary artery was involved in three,1-3 the left anterior descending artery in one4 and the left circumflex artery in one.1 The involved coronary artery was not specified5 in one patient, and in three cases described by Abbott et al.,6 only the presence of an aberrant vessel was indicated, with no mention of involvement of the major coronary arteries. Thus, the present case is unique in that all three major coronary arteries formed an arteriosystemic fistula. Selective coronary angiograms demonstrated marked dilation of the right, left anterior descending and circumflex arteries. The diameter of these arteries at their origin (6, 7 and 6 mm, respectively) was almost twice that reported as normal by Baroldi and Scomazzoni.7 Coronary fistulas may be formed by a well defined vessel emptying into a cardiac chamber or, as in our case, by a plexus of multiple fine vessels. This vascular maze probably represents persistence of embryonic sinusoids that are usually obliterated in normal development. The myocardial vessels in the
February 1974
The American Journal of CARDIOLOGY
Volume 33
305
CORONARY
ARTERtOSYSTEMlC
FISTULAS-REDDY
ET AL.
adult heart are derived embryologically from both endothelial protrusions into the myocardium and coronary arteries and veins.8 This endothelial protrusion into the myocardium extends to the epicardial surface, forming an intertrabecular network. The outermost intertrabecular network becomes almost completely obliterated and forms a capillary network as the myocardium grows, whereas the innermost intertrabecular vessels retain their communication with the chambers of the heart, forming the Thebesians vessels of the adult heart. Investigators4T”,Y reporting earlier cases, in which sinusoidal communication existed between involved coronary arteries and the heart chamber, ascribed the anomaly to persistence of the outermost intertrabecular space, thereby permitting a communication between the coronary artery and heart chamber through the Thebesian vessels. A similar mechanism can explain the angiographic findings and diffuse involvement in our case. The reason for abnormal communication only with the left ventricle is not clear.
In our case, no murmur was heard suggesting a coronary artery fistula. Such fistulas have been associated with continuous’J or purely diastolic murmurs.Z In the case reported by Lovitt and Lutz,“ no heart murmur was mentioned and the fistula was discovered only on postmortem examination. The cause of death in that patient was not related to the fistula. The symptoms of angina pectoris in our case can be attributed to the coronary fistulas. The anticipated hemodynamic burden of arteriosystemic fistulas would be diastolic overload of the left ventricle. The normal pulse pressure and the absence of left ventricular hypertrophy in our case suggest t,hat there was no hemodynamically significant volume overload on the left ventricle. The patient’s symptoms should be treated medically. Acknowledgment Dr. Irwin Hoffman for reviewing
this manuscript and express gratitude for the assistance given by Mrs. Miriam Posner, Mr. Joshua Hamby and Mrs. Brenda Hamby. We thank
References 1. McNamara JJ, Gross RE: Congenital coronary artery fistula. Surgery 6559-69, 1969 2. Eguchi S, Nitta H, Asano K, et al: Congenital fistula of the right coronary artery to the left ventricle. The third case in the literature. Am Heart J 80:242-246. 1970 3. Tanabe T, lsomatsu T, Ota S, et al: Tortuous right coronary artery to the left ventricle. Jap J Thorac Surg .20:646-651. 1967 4. Levitt WV Jr, Lutz S Jr: Embryological aneurysm of the myocardial vessels. Arch Pathol 57:163-167, 1954 5. Bellet S, Gouley BA: Congenital heart disease with multiple cardiac anomalies. Report of a case showing aortic atresia, fibrous
306
February
1974
The American
Journal of CARDIOLOGY
6.
7.
8. 9.
scar in myocardium and embryonal sinusoidal remains. Am J Med Sci 183:459-465, 1932 Abbott OA, Rivarola CH, Logue RB: Surgical correction of coronary arteriovenous fistula. J Thorac Cardiovasc Surg 42:660672, 1961 Baroldl G, Scomazzonl G: Coronary Circulation in the Normal and the Pathologic Heart. Office of the Surgeon General, Depariment of the Army, Washington, DC, 1967, p 6. 10, 18 Grant RT, Regnier M: The comparative anatomy of the cardiac coronary vessels. Heart 13:285-317, 1926 Grant RT: An unusual anomaly of the coronary vessels in the malformed heart of a child. Heart 13:273-284, 1926
Volume 33