lnternal Mammary Arteriovenous MaFormation with Communication to the Pulmonary Vessels
EDWARD M. COHEN, MD’ DONALD E. LOEW, MD JOSEPH V. MESSER, MD, FACC+z Boston, Massachusetts
A 25 year old asymptomatic man with a past history of pulmonary tuberculosis presented with a continuous murmur. Selective arteriography revealed a left internal mammary arteriovenous malformation in communication with vessels in the left upper pulmonary lobe. No significant hemodynamic abnormalities were detected. This is the 26th reported case of Internal mammary arteriovenous fistula and the 6th with a pulmonary communication. Review of the data in previous cases suggests that surgical indications are limited to symptomatic relief, heart failure durtng infancy or the possible risk of endarteritis, proximal arterial degeneration or rupture.
Arteriovenous fistulas involving the internal mammary vessels are rare. Only 25 cases have previously been reported1-21 and only 23 described in some detaiLI-19 In five cases the malformation communicase reprecated with the pulmonary vessels. %17-lg. The following sents the sixth report of this phenomenon.
Case Report
From the Cardiovascular Unit, the Circulation and Thorndike Memorial Laboratories, Boston City Hospital, and the Departments of Medicine at Tufts and Harvard Medical Schools, Boston, Mass. This work was supported in part by Training Grants HE 5687 and 5 TO1 HL 05244 from the National Heart and Lung Institute, National Institutes of Heaith, Bethesda, Md., and a grant from the Medical Care and Education Foundation (Tri-State Regional Medical Program). Manuscript accepted April 24, 1974. Present address: Probst Professional Building, Suite 317, Eisenhower Medical Center, 39000 Bob Hope Dr., Palm Desert, Calif. 92260. t Recipient of National Heart and Lung Institute Research Career Development Award 1 K3ME 8719. t Present address and address for reprints: Joseph V. Messer, MD, Section of CardioRespiratory Diseases, Rush-Presbyterian-St. Luke’s Medical Center, 1753 West Congress Parkway, Chicago, Ill. 60612. l
A 25 year old male laundry worker was admitted to the Boston City Hospital for evaluation of a continuous precordial murmur. At age 18, during a bout of “pneumonia and pleurisy,” he was told he had tuberculosis and underwent 8 months of treatment with isoniazid at another institution. At age 23 he underwent tonsillectomy at that institution. He was not informed of a murmur during either hospitalization. The patient smoked 20 cigarettes daily and admitted having taken heroin intravenously two to four times a month for the past 18 months. His exercise tolerance was excellent, and he denied all cardiovascular and respiratory symptoms. Physical examination revealed a robust, muscular young male. The vital signs were within normal limits, and no clubbing, cyanosis or telangiectasis was observed. The lungs were clear to auscultation, and the heart sounds were normal. A grade 316 high pitched continuous murmur was heard at the &third and fourth intercostal spaces along the left sternal border. The murmur appeared to be close to the ear of the examiners. Chest roentgenogran (Fig. 1) revealed small areas of calcification and linear densities in both upper pulmonary lobes. This finding as well as blunting of the left costophrenic angle was thought to represent old granulomatous disease. Results of a cardiac X-ray series with fluoroscopy and the electrocardiogram were normal. The hematocrit was 43 percent and several blood cultures yielded no growth. At cardiac catheterization (Table I), intracardiac and pulmonary arterial pressures, cardiac output, and vascular resistance were normal except for slightly increased right ventricular systolic and end-diastolic pressures. An appropriate response followed leg-raising exercise. Measurement of blood oxygen saturation revealed no evidence of intracardiac or intrapulmonary shunting. The partial pressures of oxygen in systemic arterial blood responded to breathing of 100 percent oxygen by rising in normal fashion to a level of 650 mm Hg. Indocyanine green dye-dilution curves obtained by injection into the main pulmonary artery and aortic root and sampling, respectively, from the left brachial artery and main pulmonary arteries did not demonstrate abnormal initial appearance or early recirculation of dye. Main
January 1975
The American Journal of CARDIOLOGY
Volume 35
103
INTERNAL MAMMARY-PULMONARY
ARTERIOVENOUS
MALFORMATION-COHEN
TABLE
ET AL.
I
Results of Cardiac Catheterization Oxygen Saturatron Site
81 80
(6) 3218
80 81 82
(14)
97
(104)
Exercrse
(15)
119/4
Heart rate (beats/min) Oxygen consumption (cc/min) Cardiac output (liters/min) Cardiac index (liters/min per m2) Pulmonary vascular resistance (dynes set cm-“) Systemic vascular resistance (dynes set cm-“)
TABLE
Rest
(%)
Right atrium Right ventricle Pulmonary artery Main Right Left Left ventricle Brachial artery
FIGURE 1. Chest roentgenogram, posteroanterior view. The internal mammary artery-pulmonary vessel fistula is seen immediately above the left pulmonary artery.
Pressure (mm Hg)*
66 199 6.3 3.2
(115) 120 850 12.8 6.6
127
69
1321
719
* Figures in parentheses indicate mean pressure; phasic pressures are expressed as peak systolic and end-diastolic values.
II
Five Previously
Reported
Authors Bjork et al.1’
Cases of Internal Patient’s Age (yr) & Sex 23M 25M
Voll et al.‘* Kiphart et al.‘9 Stafford et al.13
32F 23M 21F
* In all cases the lesion was congenital
Mammary
(IM) Fistulas with Communication
to the Pulmonary
Anatomic Description Left IM, phrenic, pericardiophrenic arteries to pulmonary veins Left IM, phrenic, intercostal arteries to pulmonary veins Right IM artery to right pulmonary artery Right IM artery to right pulmonary artery Left IM artery to left pulmonary vessels in origin and surgery
Vessels*
Presenting Data Continuous
murmur
Continuous
murmur
Continuous Continuous Continuous
murmur murmur, murmur,
chest pain chest pain
was performed.
FIGURE 2. Selective left internal mammary arteriogram, anteroposterior view. 1, catheter in the dilated proximal left internal mammary artery. 2, fistula from the arteriovenous malformation to the left upper pulmonary lobe and site of entry into the pulmonary circulation. 3, catheter in the left pulmonary artery. 4, systemic arteriovenous malformation. 5, intercostal branches of the let? internal mammary artery.
104
January 1975
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Volume 35
!NTERNAL
MAMMARY-PULMONARY
and left pulmonary arteriograms revealed a 1 cm nodular area of opacification filling from the proximal left upper pulmonary artery and draining after a slight delay into the corresponding pulmonary vein. An aortic root arteriogram demonstrated a dilated proximal portion of the left internal mammary artery. A selective left internal mammary arteriogram (Fig. 2 and 3) revealed an extensive arteriovenous malformation at the level of the fourth rib with a late filling vessel coursing superiorly through the anterior mediastinum opacifying the same left upper lobe pulmonary nodule. Small pulmonary arterial radicles, identical to those visualized in the pulmonary arteriogram, emanated from this nodular area and coursed to the lung periphery. Drainage was by the same left upper lobe pulmonary vein, which was in direct communication with this nodule. Despite the considerable risk of infectious endarteritis as a result of his heroin habit, the patient refused surgical intervention. Because of his lack of symptoms and relatively normal hemodynamic profile, he was discharged to followup in the clinic.
Discussion Clinical and hemodynamic features: Thoracic arteriovenous fistulas are most commonly located in the lungs and cause left to right shunts that are sometimes symptomatic and associated with cyanosis, clubbing or telangiectasis.22 Fistulas involving the vessels of the chest wall are rare, possibly because of the protected location of the vessels behind the bone structures of the thorax. Although systemic arteriovenous fistulas have been associated with increased blood volume, increased cardiac output and congestive heart failure when the involved vessels have been of large caliber, thoracic fistulas involving the internal mammary arteries have seldom caused severe symptoms in the adult. In the 16 adult cases without pulmonary communication previously reported in sufficient detail for evaluation, 5 patients had vague chest pain, 5 had various degrees of dyspnea and 3 had a pulsatile or expanding mass. There are two reports of congestive heart failure in infants with an internal mammary artery-ductus venosus communication,5J4 but no adult has been reported to
ARTERIOVENOUS
MALFORMATION-COHEN
ET
AL.
have cardiac decompensation attributable to a systemic internal mammary arteriovenous fistula. In the adult patient of Massumi12 the shunt carried only 15 percent of the cardiac output. Intracardiac pressures, when measured, have invariably been normal.asJO This relative lack of hemodynamic significance is in marked contrast to the frequently striking physical finding of a continuous thoracic bruit occasionally mistaken for the murmur of a patent ductus arteriosus.s,s,17 The normal resting Fick cardiac output value in our case indicates a small shunt volume through the systemic internal mammary arteriovenous malformation. The normal response to 100 percent oxygen inhalation, the normal systemic tion and the lack of an oxygen
arterial step-up
oxygen saturain intracardiac
and pulmonary blood samples also imply insignificant or no pulmonary venoarterial, intracardiac or pulmonary-cardiac shunting. The slightly elevated values for pulmonary arterial systolic and right ventricular end-diastolic pressures may be due to our utilization of one half of the anteroposterior thoracic diameter as zero pressure reference point. This procedure has been observed to overestimate right heart pressure by as much as 3 mm Hg.23 The arteriographic evidence that the internal mammary vessel fistula communicated predominantly with the left upper lobe pulmonary vein suggests the presence of a left to left shunt. The indicator-dilution curve, obtained by injection into the main pulmonary artery and sampling from the brachial artery, failed to demonstrate an early recirculation contour, thus attesting to the small volume of any such shunt. Further efforts at quantification, such as injection of indicator into the superior vena cava with simultaneous sampling from the pulmonary and brachial arteries,24 were therefore not performed. As in our case, the hemodynamic alterations were insignificant in the five previously reported cases of internal mammary arteriovenous malformations in communication with the pulmonary vessels (Table II). Although two of the patients experienced vague
FIGURE 3. Selective left internal mammary arteriogram. lateral view. 1, dilated proximal left internal mammary artery. 2, arteriovenous malformation. 3, distal internal mammary artery and its intercostal branches. 4, fistula from the arteriovenous malformation to the left upper pulmonary lobe. 5. entry site of the fistula into the pulmonary circulation. 6, tortuous anterior mediastinal branch of the internal mammary artery. 7, catheter to the internal mammary artery. 8, catheter in the left pulmonary artery.
January 1975
The American Journal of CARDIOLOGY
Volume 35
105
INTERNAL MAMMARY-PULMONARY
ARTERIOVENOUS
MALFORMATION-COHEN
chest pain, and the patients of Voll et a1.18 and Kiphart et al.lg had small oxygen increases at the pulmonary arterial level indicative of left to right shunting, no patient was reported to have cardiomegaly, increased intracardiac pressures13J8Jg or increased cardiac output. l3 There have been a few reports of systemic artery-pulmonary vessel shunts, similar to the shunts in the aforementioned cases, invascular adhesions,25 anomalous aortic volving branches,26J7 intercostal arteries,27T28bronchial arteries,27 coronary arteries2g and other arteries of the chest wa11.30,31Except in a few cases with large shunt volumes, these fistulas have been of little hemodynamic significance. Etiology: The cause of these vascular anomalies is not always clear. Congenital, traumatic and inflammatory origins have been suggested. According to the early postulate of Lewis,32 and on the basis of experience gained in previous cases, it is possible that con-
ET AL.
genital arteriovenous fistulas may not become manifest until adult life; this may have been the case in our patient, who gave no history of chest trauma. In view of the history and roentgenographic signs of left pulmonary and pleural tuberculosis, it is possible that the systemic-pulmonary communication had an inflammatory origin, similar to that in the patient described by Wolarsky.28 Surgical indications: The long-term prognosis of internal mammary arteriovenous fistulas with or without communication to the pulmonary vessels is unknown. Most reported cases have involved surgical ligation and excision. Despite the usually small shunt volume, complaints of local symptoms, or the risk of infectious endarteritis, rupture with intrathoracic or intrapulmonary hemorrhage or proximal arterial degeneration,33 may justify surgical intervention. Medical follow-up of our patient will offer unique information on the natural history of this lesion.
References 1. Maier HC, Stout AP: Congenital arteriovenous fistulas of the thoracic wall. Circulation 1:809-812, 1950 2. Glenn F, Steinberg I: Arteriovenous fistula of the right internal mammary vessels following radical mastectomy: visualization by angiocardiography. J Thorac Surg 33:719-722, 1957 3. Wells BG, Hurt RL: Congenital arteriovenous fistula of the internal mammary vessels. Br Heart J 19:135-137, 1957 4. Brownlee WE, McGannon PT: Arteriovenous fistula between the internal mammary artery and vein following stab wound of the chest. A case report. J Thorac Cardiovasc Surg 38:271273, 1959 5. Glass IH, Rowe RD, Duckworth JWA: Congenital arteriovenous fistula between the left internal mammary artery and the ductus venosus: unusual cause of congestive heart failure in the newborn infant. Pediatrics 26:604-610, 1960 6. Holland RH: Arteriovenous fistula of the lefl internal mammary vessels simulating a patent ductus arteriosus. J Thorac Cardiovast Surg 39:767-769, 1960 7. Mercado HR, Santin HI: Aneurisma traum&o de la arteria mammaria interna: hemotorax crbnico organizato. Plensa Med Argent 47129 15-2918, 1960 8. Greenfield LJ, Hatcher CR: Post-traumatic internal mammary arteriovenous fistula. Diagnostic considerations and report of a case. Ann Surg 158:129-130, 1963 9. Shirkey AL, Baay JEW, Law SW, et al: Arteriovenous fistula of the internal mammary vessels. Report of a case and review of the literature. J Thorac Cardiovasc Surg 48:49-53, 1964 10. Chevrier JL, de la Fuente A: A propos de deux cas d’anbvrisme arterio-veineux developp& sur le trajet de I’arthre mammaire interne. J Chir (Parig) 90:441-444, 1965 11. Sautot J, Duquesnel J: A propos d’un an(?vrisme art&rio-veineux congenital dependent de la mammaire interne droite. Lyon Chir 62: 82-85, 1966 12. Massuml RA: Internal mammary arteriovenous fistula. Report of a case. Med Ann DC 36:163-176, 1967 13. Stafford RW, Kroneberg MW, Dunbar JD, et al: Continuous precordial murmurs due to internal mammary artery fistulas including internal mammary to pulmonary artery fistulas. Am J Cardiol 241414-419, 1969 14. Stanford W, Fixler DE, Armstrong RG, et al: Congenital arteriovenous fistula between the left internal mammary artery and the ductus venosus. J Thorac Cardiovasc Surg 60:248-252, 1970 15. Boontje AH, Kruyswljk HH: Arteriovenous fistula of the internal mammary vessels. J Thorac Cardiovasc Surg 62:618-623. 1971 16. Rublno PJ, Milnes RF: Internal mammary arteriovenous fistula. Report of a case and brief review of the literature. Int Surg 55:
106
January 1975
The American Journal of CARDIOLOGY
404-410.1971 17. Bjork VO, lntontl F, Aletras H, et al: Varieties of pulmonary arteriovenous aneurysms. Acta Chir Stand 125:69-76, 1963 18. Voll A, Marstrander F, Wexels P: Systemic-pulmonary shunt. Dis Chest 45:396-401, 1964 19. Kiphart RJ, Mackenzie JW, Templeton AW, et al: Systemicpulmonary arteriovenous fistula of the chest wall and lung. A report of a case and review of the literature. J Thorac Cardiovasc Surg 54:113-120, 1967 20. Schumaker HB, Carter KL: Arteriovenous fistula and arterial aneurysms in military personnel. Surgery 20:9-25, 1946 21. Elkln DC: Vascular Surgery. Washington, DC, Office of the Surgeon General, Department of the Army, 1966, p 156,162 22. Moyer JH, Glantz G, Best AN: Pulmonary arteriovenous fistula. Am J Med 32:417-435, 1962 23. Debrunner F, Buhler F: Normal central venous pressure-significance of reference point and normal range. Br Med J 3: 148-150, 1969 24. Frltts HW, Harris P, Chidsey CA, et al: Estimation of flow through bronchial-pulmonary vascular anastomoses with use of T-1824 dye. Circulation 23:390-398, 1961 25. Burchell HB, Clagett OT: The clinical syndrome associated with pulmonary arteriovenous fistulas. Including a case report and a surgical cure. Am Heart J 34:151-162, 1947 26. Claiborne TS, Hopklns WA: Aorta-pulmonary artery communication through the lungs. Report of a case. Circulation 14: 1090-1092, 1956 27. Gomes MR, Bernatz PE, Dines DE: Pulmonary arteriovenous fiitulas. Ann Thorac Surg 7:582-593, 1969 28. Wolarsky ER, Humphreys GH: Systemic-pulmonary arteriovenous fistula. A case report. J Thorac Cardiovasc Surg 59:859863, 1970 29. Schultz J: Coronary arteriovenous aneurysm: review of the literature. Am Heart J 56:431-442, 1958 30. Cox PA, Keshlshlan JM, Blades BB: Traumatic arteriovenous fist& of the chest wall and lung secondary to insertion of an intercostal catheter. J Thorac Cardiovasc Surg 54: 109- 112, 1967 31. Prutzman LD, Flock JB: Pulmonary arteriovenous fistula with extensive thoracic wall collateral circulation. Bull Ayer Clin Lab 4:23-29, 1954 32. Lewis DD: Congenital arteriovenous fistulae. Lancet 2:621-628, 1930 HB: Aneurysm development and degenerative 33. Shumaker changes in dilated artery proximal to arteriovenous fist&. Surg Gynecol Obstet 1301636-640, 1970
Volume 35
EDWARD M. COHEN, MD’ DONALD E. LOEW, MD JOSEPH V. MESSER, MD, FACC+z Boston, Massachusetts
A 25 year old asymptomatic man with a past history of pulmonary tuberculosis presented with a continuous murmur. Selective arteriography revealed a left internal mammary arteriovenous malformation in communication with vessels in the left upper pulmonary lobe. No significant hemodynamic abnormalities were detected. This is the 26th reported case of Internal mammary arteriovenous fistula and the 6th with a pulmonary communication. Review of the data in previous cases suggests that surgical indications are limited to symptomatic relief, heart failure durtng infancy or the possible risk of endarteritis, proximal arterial degeneration or rupture.
Arteriovenous fistulas involving the internal mammary vessels are rare. Only 25 cases have previously been reported1-21 and only 23 described in some detaiLI-19 In five cases the malformation communicase reprecated with the pulmonary vessels. %17-lg. The following sents the sixth report of this phenomenon.
Case Report
From the Cardiovascular Unit, the Circulation and Thorndike Memorial Laboratories, Boston City Hospital, and the Departments of Medicine at Tufts and Harvard Medical Schools, Boston, Mass. This work was supported in part by Training Grants HE 5687 and 5 TO1 HL 05244 from the National Heart and Lung Institute, National Institutes of Heaith, Bethesda, Md., and a grant from the Medical Care and Education Foundation (Tri-State Regional Medical Program). Manuscript accepted April 24, 1974. Present address: Probst Professional Building, Suite 317, Eisenhower Medical Center, 39000 Bob Hope Dr., Palm Desert, Calif. 92260. t Recipient of National Heart and Lung Institute Research Career Development Award 1 K3ME 8719. t Present address and address for reprints: Joseph V. Messer, MD, Section of CardioRespiratory Diseases, Rush-Presbyterian-St. Luke’s Medical Center, 1753 West Congress Parkway, Chicago, Ill. 60612. l
A 25 year old male laundry worker was admitted to the Boston City Hospital for evaluation of a continuous precordial murmur. At age 18, during a bout of “pneumonia and pleurisy,” he was told he had tuberculosis and underwent 8 months of treatment with isoniazid at another institution. At age 23 he underwent tonsillectomy at that institution. He was not informed of a murmur during either hospitalization. The patient smoked 20 cigarettes daily and admitted having taken heroin intravenously two to four times a month for the past 18 months. His exercise tolerance was excellent, and he denied all cardiovascular and respiratory symptoms. Physical examination revealed a robust, muscular young male. The vital signs were within normal limits, and no clubbing, cyanosis or telangiectasis was observed. The lungs were clear to auscultation, and the heart sounds were normal. A grade 316 high pitched continuous murmur was heard at the &third and fourth intercostal spaces along the left sternal border. The murmur appeared to be close to the ear of the examiners. Chest roentgenogran (Fig. 1) revealed small areas of calcification and linear densities in both upper pulmonary lobes. This finding as well as blunting of the left costophrenic angle was thought to represent old granulomatous disease. Results of a cardiac X-ray series with fluoroscopy and the electrocardiogram were normal. The hematocrit was 43 percent and several blood cultures yielded no growth. At cardiac catheterization (Table I), intracardiac and pulmonary arterial pressures, cardiac output, and vascular resistance were normal except for slightly increased right ventricular systolic and end-diastolic pressures. An appropriate response followed leg-raising exercise. Measurement of blood oxygen saturation revealed no evidence of intracardiac or intrapulmonary shunting. The partial pressures of oxygen in systemic arterial blood responded to breathing of 100 percent oxygen by rising in normal fashion to a level of 650 mm Hg. Indocyanine green dye-dilution curves obtained by injection into the main pulmonary artery and aortic root and sampling, respectively, from the left brachial artery and main pulmonary arteries did not demonstrate abnormal initial appearance or early recirculation of dye. Main
January 1975
The American Journal of CARDIOLOGY
Volume 35
103
INTERNAL MAMMARY-PULMONARY
ARTERIOVENOUS
MALFORMATION-COHEN
TABLE
ET AL.
I
Results of Cardiac Catheterization Oxygen Saturatron Site
81 80
(6) 3218
80 81 82
(14)
97
(104)
Exercrse
(15)
119/4
Heart rate (beats/min) Oxygen consumption (cc/min) Cardiac output (liters/min) Cardiac index (liters/min per m2) Pulmonary vascular resistance (dynes set cm-“) Systemic vascular resistance (dynes set cm-“)
TABLE
Rest
(%)
Right atrium Right ventricle Pulmonary artery Main Right Left Left ventricle Brachial artery
FIGURE 1. Chest roentgenogram, posteroanterior view. The internal mammary artery-pulmonary vessel fistula is seen immediately above the left pulmonary artery.
Pressure (mm Hg)*
66 199 6.3 3.2
(115) 120 850 12.8 6.6
127
69
1321
719
* Figures in parentheses indicate mean pressure; phasic pressures are expressed as peak systolic and end-diastolic values.
II
Five Previously
Reported
Authors Bjork et al.1’
Cases of Internal Patient’s Age (yr) & Sex 23M 25M
Voll et al.‘* Kiphart et al.‘9 Stafford et al.13
32F 23M 21F
* In all cases the lesion was congenital
Mammary
(IM) Fistulas with Communication
to the Pulmonary
Anatomic Description Left IM, phrenic, pericardiophrenic arteries to pulmonary veins Left IM, phrenic, intercostal arteries to pulmonary veins Right IM artery to right pulmonary artery Right IM artery to right pulmonary artery Left IM artery to left pulmonary vessels in origin and surgery
Vessels*
Presenting Data Continuous
murmur
Continuous
murmur
Continuous Continuous Continuous
murmur murmur, murmur,
chest pain chest pain
was performed.
FIGURE 2. Selective left internal mammary arteriogram, anteroposterior view. 1, catheter in the dilated proximal left internal mammary artery. 2, fistula from the arteriovenous malformation to the left upper pulmonary lobe and site of entry into the pulmonary circulation. 3, catheter in the left pulmonary artery. 4, systemic arteriovenous malformation. 5, intercostal branches of the let? internal mammary artery.
104
January 1975
The American Journal of CARDIOLOGY
Volume 35
!NTERNAL
MAMMARY-PULMONARY
and left pulmonary arteriograms revealed a 1 cm nodular area of opacification filling from the proximal left upper pulmonary artery and draining after a slight delay into the corresponding pulmonary vein. An aortic root arteriogram demonstrated a dilated proximal portion of the left internal mammary artery. A selective left internal mammary arteriogram (Fig. 2 and 3) revealed an extensive arteriovenous malformation at the level of the fourth rib with a late filling vessel coursing superiorly through the anterior mediastinum opacifying the same left upper lobe pulmonary nodule. Small pulmonary arterial radicles, identical to those visualized in the pulmonary arteriogram, emanated from this nodular area and coursed to the lung periphery. Drainage was by the same left upper lobe pulmonary vein, which was in direct communication with this nodule. Despite the considerable risk of infectious endarteritis as a result of his heroin habit, the patient refused surgical intervention. Because of his lack of symptoms and relatively normal hemodynamic profile, he was discharged to followup in the clinic.
Discussion Clinical and hemodynamic features: Thoracic arteriovenous fistulas are most commonly located in the lungs and cause left to right shunts that are sometimes symptomatic and associated with cyanosis, clubbing or telangiectasis.22 Fistulas involving the vessels of the chest wall are rare, possibly because of the protected location of the vessels behind the bone structures of the thorax. Although systemic arteriovenous fistulas have been associated with increased blood volume, increased cardiac output and congestive heart failure when the involved vessels have been of large caliber, thoracic fistulas involving the internal mammary arteries have seldom caused severe symptoms in the adult. In the 16 adult cases without pulmonary communication previously reported in sufficient detail for evaluation, 5 patients had vague chest pain, 5 had various degrees of dyspnea and 3 had a pulsatile or expanding mass. There are two reports of congestive heart failure in infants with an internal mammary artery-ductus venosus communication,5J4 but no adult has been reported to
ARTERIOVENOUS
MALFORMATION-COHEN
ET
AL.
have cardiac decompensation attributable to a systemic internal mammary arteriovenous fistula. In the adult patient of Massumi12 the shunt carried only 15 percent of the cardiac output. Intracardiac pressures, when measured, have invariably been normal.asJO This relative lack of hemodynamic significance is in marked contrast to the frequently striking physical finding of a continuous thoracic bruit occasionally mistaken for the murmur of a patent ductus arteriosus.s,s,17 The normal resting Fick cardiac output value in our case indicates a small shunt volume through the systemic internal mammary arteriovenous malformation. The normal response to 100 percent oxygen inhalation, the normal systemic tion and the lack of an oxygen
arterial step-up
oxygen saturain intracardiac
and pulmonary blood samples also imply insignificant or no pulmonary venoarterial, intracardiac or pulmonary-cardiac shunting. The slightly elevated values for pulmonary arterial systolic and right ventricular end-diastolic pressures may be due to our utilization of one half of the anteroposterior thoracic diameter as zero pressure reference point. This procedure has been observed to overestimate right heart pressure by as much as 3 mm Hg.23 The arteriographic evidence that the internal mammary vessel fistula communicated predominantly with the left upper lobe pulmonary vein suggests the presence of a left to left shunt. The indicator-dilution curve, obtained by injection into the main pulmonary artery and sampling from the brachial artery, failed to demonstrate an early recirculation contour, thus attesting to the small volume of any such shunt. Further efforts at quantification, such as injection of indicator into the superior vena cava with simultaneous sampling from the pulmonary and brachial arteries,24 were therefore not performed. As in our case, the hemodynamic alterations were insignificant in the five previously reported cases of internal mammary arteriovenous malformations in communication with the pulmonary vessels (Table II). Although two of the patients experienced vague
FIGURE 3. Selective left internal mammary arteriogram. lateral view. 1, dilated proximal left internal mammary artery. 2, arteriovenous malformation. 3, distal internal mammary artery and its intercostal branches. 4, fistula from the arteriovenous malformation to the left upper pulmonary lobe. 5. entry site of the fistula into the pulmonary circulation. 6, tortuous anterior mediastinal branch of the internal mammary artery. 7, catheter to the internal mammary artery. 8, catheter in the left pulmonary artery.
January 1975
The American Journal of CARDIOLOGY
Volume 35
105
INTERNAL MAMMARY-PULMONARY
ARTERIOVENOUS
MALFORMATION-COHEN
chest pain, and the patients of Voll et a1.18 and Kiphart et al.lg had small oxygen increases at the pulmonary arterial level indicative of left to right shunting, no patient was reported to have cardiomegaly, increased intracardiac pressures13J8Jg or increased cardiac output. l3 There have been a few reports of systemic artery-pulmonary vessel shunts, similar to the shunts in the aforementioned cases, invascular adhesions,25 anomalous aortic volving branches,26J7 intercostal arteries,27T28bronchial arteries,27 coronary arteries2g and other arteries of the chest wa11.30,31Except in a few cases with large shunt volumes, these fistulas have been of little hemodynamic significance. Etiology: The cause of these vascular anomalies is not always clear. Congenital, traumatic and inflammatory origins have been suggested. According to the early postulate of Lewis,32 and on the basis of experience gained in previous cases, it is possible that con-
ET AL.
genital arteriovenous fistulas may not become manifest until adult life; this may have been the case in our patient, who gave no history of chest trauma. In view of the history and roentgenographic signs of left pulmonary and pleural tuberculosis, it is possible that the systemic-pulmonary communication had an inflammatory origin, similar to that in the patient described by Wolarsky.28 Surgical indications: The long-term prognosis of internal mammary arteriovenous fistulas with or without communication to the pulmonary vessels is unknown. Most reported cases have involved surgical ligation and excision. Despite the usually small shunt volume, complaints of local symptoms, or the risk of infectious endarteritis, rupture with intrathoracic or intrapulmonary hemorrhage or proximal arterial degeneration,33 may justify surgical intervention. Medical follow-up of our patient will offer unique information on the natural history of this lesion.
References 1. Maier HC, Stout AP: Congenital arteriovenous fistulas of the thoracic wall. Circulation 1:809-812, 1950 2. Glenn F, Steinberg I: Arteriovenous fistula of the right internal mammary vessels following radical mastectomy: visualization by angiocardiography. J Thorac Surg 33:719-722, 1957 3. Wells BG, Hurt RL: Congenital arteriovenous fistula of the internal mammary vessels. Br Heart J 19:135-137, 1957 4. Brownlee WE, McGannon PT: Arteriovenous fistula between the internal mammary artery and vein following stab wound of the chest. A case report. J Thorac Cardiovasc Surg 38:271273, 1959 5. Glass IH, Rowe RD, Duckworth JWA: Congenital arteriovenous fistula between the left internal mammary artery and the ductus venosus: unusual cause of congestive heart failure in the newborn infant. Pediatrics 26:604-610, 1960 6. Holland RH: Arteriovenous fistula of the lefl internal mammary vessels simulating a patent ductus arteriosus. J Thorac Cardiovast Surg 39:767-769, 1960 7. Mercado HR, Santin HI: Aneurisma traum&o de la arteria mammaria interna: hemotorax crbnico organizato. Plensa Med Argent 47129 15-2918, 1960 8. Greenfield LJ, Hatcher CR: Post-traumatic internal mammary arteriovenous fistula. Diagnostic considerations and report of a case. Ann Surg 158:129-130, 1963 9. Shirkey AL, Baay JEW, Law SW, et al: Arteriovenous fistula of the internal mammary vessels. Report of a case and review of the literature. J Thorac Cardiovasc Surg 48:49-53, 1964 10. Chevrier JL, de la Fuente A: A propos de deux cas d’anbvrisme arterio-veineux developp& sur le trajet de I’arthre mammaire interne. J Chir (Parig) 90:441-444, 1965 11. Sautot J, Duquesnel J: A propos d’un an(?vrisme art&rio-veineux congenital dependent de la mammaire interne droite. Lyon Chir 62: 82-85, 1966 12. Massuml RA: Internal mammary arteriovenous fistula. Report of a case. Med Ann DC 36:163-176, 1967 13. Stafford RW, Kroneberg MW, Dunbar JD, et al: Continuous precordial murmurs due to internal mammary artery fistulas including internal mammary to pulmonary artery fistulas. Am J Cardiol 241414-419, 1969 14. Stanford W, Fixler DE, Armstrong RG, et al: Congenital arteriovenous fistula between the left internal mammary artery and the ductus venosus. J Thorac Cardiovasc Surg 60:248-252, 1970 15. Boontje AH, Kruyswljk HH: Arteriovenous fistula of the internal mammary vessels. J Thorac Cardiovasc Surg 62:618-623. 1971 16. Rublno PJ, Milnes RF: Internal mammary arteriovenous fistula. Report of a case and brief review of the literature. Int Surg 55:
106
January 1975
The American Journal of CARDIOLOGY
404-410.1971 17. Bjork VO, lntontl F, Aletras H, et al: Varieties of pulmonary arteriovenous aneurysms. Acta Chir Stand 125:69-76, 1963 18. Voll A, Marstrander F, Wexels P: Systemic-pulmonary shunt. Dis Chest 45:396-401, 1964 19. Kiphart RJ, Mackenzie JW, Templeton AW, et al: Systemicpulmonary arteriovenous fistula of the chest wall and lung. A report of a case and review of the literature. J Thorac Cardiovasc Surg 54:113-120, 1967 20. Schumaker HB, Carter KL: Arteriovenous fistula and arterial aneurysms in military personnel. Surgery 20:9-25, 1946 21. Elkln DC: Vascular Surgery. Washington, DC, Office of the Surgeon General, Department of the Army, 1966, p 156,162 22. Moyer JH, Glantz G, Best AN: Pulmonary arteriovenous fistula. Am J Med 32:417-435, 1962 23. Debrunner F, Buhler F: Normal central venous pressure-significance of reference point and normal range. Br Med J 3: 148-150, 1969 24. Frltts HW, Harris P, Chidsey CA, et al: Estimation of flow through bronchial-pulmonary vascular anastomoses with use of T-1824 dye. Circulation 23:390-398, 1961 25. Burchell HB, Clagett OT: The clinical syndrome associated with pulmonary arteriovenous fistulas. Including a case report and a surgical cure. Am Heart J 34:151-162, 1947 26. Claiborne TS, Hopklns WA: Aorta-pulmonary artery communication through the lungs. Report of a case. Circulation 14: 1090-1092, 1956 27. Gomes MR, Bernatz PE, Dines DE: Pulmonary arteriovenous fiitulas. Ann Thorac Surg 7:582-593, 1969 28. Wolarsky ER, Humphreys GH: Systemic-pulmonary arteriovenous fistula. A case report. J Thorac Cardiovasc Surg 59:859863, 1970 29. Schultz J: Coronary arteriovenous aneurysm: review of the literature. Am Heart J 56:431-442, 1958 30. Cox PA, Keshlshlan JM, Blades BB: Traumatic arteriovenous fist& of the chest wall and lung secondary to insertion of an intercostal catheter. J Thorac Cardiovasc Surg 54: 109- 112, 1967 31. Prutzman LD, Flock JB: Pulmonary arteriovenous fistula with extensive thoracic wall collateral circulation. Bull Ayer Clin Lab 4:23-29, 1954 32. Lewis DD: Congenital arteriovenous fistulae. Lancet 2:621-628, 1930 HB: Aneurysm development and degenerative 33. Shumaker changes in dilated artery proximal to arteriovenous fist&. Surg Gynecol Obstet 1301636-640, 1970
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