Left Ventricular Aneurysm Secondary to Behget’s Disease G. Di Eusanio, MD, A. Mazzola, MD, R. Gregorini, MD, G. Esposito, MD, W. Di Nardo, MD, G. Di Manici, MD, and C. Villani, MD Divisione Di Cardiochirurgia, Ospedale Civile G. Mazzini, Teramo, Italy
A 16-year-old boy with Behqet‘s disease who was seen with thrombophlebitis of the leg was found to have coronary artery occlusion with postinfarction left ventricular aneurysm. Recurrent femoral artery aneurysms and orogenital ulceration developed in him. The diagnostic features together with successful treatment of this patient and a discussion of Behqet’s syndrome are presented. (Ann Thorac Surg 7991;51:131-2)
B
ehcet’s syndrome is a systemic disease of unknown origin in which the cardiovascular system is involved in 7% to 29% of cases, with recurrent superficial thrombophlebitis, aneurysms of the pulmonary and systemic arteries, pericarditis, myocarditis, endocardia1 fibrosis, conduction defects, and aortic regurgitation [l, 21. We present a case of Behcet’s disease with thrombophlebitis, thrombotic occlusion of the left anterior descending coronary artery with infarction and aneurysmal formation, and arteritis of the femoral artery with recurrent aneurysms. A 16-year-old boy was admitted to the hospital with thrombophlebitis of the right leg. The laboratory test showed a fibrinolysis defect, and oral anticoagulant therapy was started. Routinely performed electrocardiography showed old anterior myocardial infarct: the history of the patient in fact showed a previous episode of severe chest pain with vomiting that was undervalued in the first instance. Cardiac catheterization showed proximal occlusion of the left anterior descending coronary artery and the left ventricular aneurysm. Three months after cardiac catheterization, pulsatile aneurysm developed at the puncture site of the femoral artery and was resected; the arterial wall was repaired with a saphenous vein patch. The aneurysm recurred again a month later, requiring a graft interposition to reconstruct the artery. At that time, Doppler echocardiography was performed, showing a new aneurysmatic ventricular pouch with a very thin wall in free communication with the left ventricular aneurysm previously noted (Fig 1). Surgical treatment was then advised. At operation, performed using cardiopulmonary bypass, a biloculated aneurysm was Accepted for publication June29, 1990. Address reprint requests to Dr Di Eusanio, Divisione di Cardiochirurgia, Ospedale Civile G. Mazzini, Villa Mosca 64100, Italy.
0 1991 by The Society of Thoracic Surgeons
found in which the proximal part was probably the result of the anterior myocardial infarct whereas the distal one appeared to be of mycotic origin (Fig 2). The aneurysm was resected, and samples were sent for bacteriological and histological studies. The postoperative course was uneventful, but a month later, a pulsatile aneurysm recurred in the femoral artery, initially requiring iliacfemoral bypass and then, because of its recurrence in the distal anastomotic site, requiring reconstruction of the artery using a 7-mm Gore-Tex graft. The macroscopic appearance of the femoral aneurysm was of inflammatory origin, but all the cultures were sterile. During the patient’s hospital stay, oral and genital ulcerations were noted and Behcet’s disease was then suspected. The histological slides taken from the left ventricular and femoral artery aneurysms were then looked at again, and the diagnosis was confirmed by recognizing the typical appearance of Behset’s disease. Corticosteroid and immunosuppressive therapy was given; the boy recovered completely, and he is well after 16 months.
Comment A syndrome characterized by the triad of recurrent orogenital ulcerations, relapsing iridocyclitis, and erythema nodosum was described by Behfet [3] in 1937. Since then many other clinical manifestations of the same disease were recognized, conferring the character of a systemic disease to Behcet’s syndrome [4]. Its cause is unknown, but the pathogenesis is probably due to an immunopathologic disorder because of the characteristic vasculitis with early mononuclear and late polymorphonuclear cell infiltration that is always present in the involved organs and systems. This hypothesis is also supported by the good response to corticosteroid and immunosuppressive ther-
~ P [41. Y
The disease affect young adults especially in Japan, the Middle East, Cyprus, Turkey, and Greece with a prevalence of 10/100,000 population in Japan [4, 51. Oral and genital ulcerations are nearly constant features. The eye may be involved resulting in inflammation of the anterior chamber, conjunctivitis, scleritis, retinal vasculitis, and optic neuritis [4]. Erythema nodosum, the third element of the syndrome, has been reported in about 80% of patients [6]. Inflammatory polyarthritis is present in about half of the patients and most often involves the superficial layers of the cartilages of the knees and ankles [4]. Neurological manifestations are related to meningoencephalitis, cranial nerve palsies, or pseudotumor cerebri, 0003-4975/91/$3.50
132
Ann Thorac Surg
CASE REPORT DI EUSANIO ET AL LV ANEURYSM IN BEHCET’S DISEASE
1991;51:131-2
Fig 2. lntraoperative aspect of left ventricular aneurysm. Fig I . Preoperative two-dimensional echocardiogram demonstrating left ventricular aneu y s m . Arrows indicate the communication between the aneurysm and the left ventricle. (AD = right atrium; AS = left atrium; VD = right ventricle; VS = left ventricle.)
but more often to brainstem involvement with dysfunction resembling minor strokes [4].Decreased activity of the fibrinolytic system is responsible for thrombotic occlusion of the arteries and veins [6]. Cardiovascular involvement is present in 7% to 29% of the patients [l]and is characterized by recurrent thrombophlebitis; arterial occlusion; aneurysm of the aorta, its major branches, and peripheral arteries especially at the site of arterial punctures; aneurysm formation of the pulmonary artery; pericarditis; myocarditis; endocardia1 fibrosis; conduction defects; and aortic regurgitation [I, 6, 7-91. In our patient, most of the cardiovascular manifestations of Behqet’s syndrome were present; however, the macroscopic appearance of the myocardium involved in the ventricular aneurysm and of the aneurysm in the femoral artery were misleading in that they were strongly suggestive of infective process. The late appearance of orogenital ulceration led to the right diagnosis.
References 1. James DG, Thompson A. Recognition of diverse cardiovascular manifestation in Behqet disease. Am Heart J 1982;45:595602. 2. Rae SA, Vandemburg M, Scholtz CL. Aortic regurgitation and false aortic aneurysm formation in Behcet disease. Postgrad Med J 1980;56:438-9. 3. Behqet H. Ueber residivierente, aphthoese durch ein virus verurdachte Geschwure am Mund am Auge und an der Genitalien. Dermatol Monatsschr 1937;105:1152-7. 4. James DG. Behqet’s syndrome. New Engl J Med 1979;308: 431-2. 5. Chamberlain MA. Behqet disease. Br Med J 1978;2:1369-0. 6. Chajek T, Fainaru M. Behqet disease with decreased fibrinolysis and superior vena cava obstruction. Br Med J 1973;l: 7824. 7. Salamon F, Weinberger A, Nili M, et al. Massive hemoptysis complicating Behqet’s syndrome: the importance of early pulmonary angiography and operation. Ann Thorac Surg 1988;45:566-7. 8. Kingston M, Ratcliffe JR, Alltree M, Merendino KA. Aneurysm after arterial puncture in Behcet disease. Br Med J 1979;1:1766-7. 9. Bowles CA, Nelson AM, Hammil SC, ODufYy JD. Cardiac involvement in Behqet’s disease. Arthritis Rheum 1985;28: 345-8.
A 16-year-old boy with Behqet‘s disease who was seen with thrombophlebitis of the leg was found to have coronary artery occlusion with postinfarction left ventricular aneurysm. Recurrent femoral artery aneurysms and orogenital ulceration developed in him. The diagnostic features together with successful treatment of this patient and a discussion of Behqet’s syndrome are presented. (Ann Thorac Surg 7991;51:131-2)
B
ehcet’s syndrome is a systemic disease of unknown origin in which the cardiovascular system is involved in 7% to 29% of cases, with recurrent superficial thrombophlebitis, aneurysms of the pulmonary and systemic arteries, pericarditis, myocarditis, endocardia1 fibrosis, conduction defects, and aortic regurgitation [l, 21. We present a case of Behcet’s disease with thrombophlebitis, thrombotic occlusion of the left anterior descending coronary artery with infarction and aneurysmal formation, and arteritis of the femoral artery with recurrent aneurysms. A 16-year-old boy was admitted to the hospital with thrombophlebitis of the right leg. The laboratory test showed a fibrinolysis defect, and oral anticoagulant therapy was started. Routinely performed electrocardiography showed old anterior myocardial infarct: the history of the patient in fact showed a previous episode of severe chest pain with vomiting that was undervalued in the first instance. Cardiac catheterization showed proximal occlusion of the left anterior descending coronary artery and the left ventricular aneurysm. Three months after cardiac catheterization, pulsatile aneurysm developed at the puncture site of the femoral artery and was resected; the arterial wall was repaired with a saphenous vein patch. The aneurysm recurred again a month later, requiring a graft interposition to reconstruct the artery. At that time, Doppler echocardiography was performed, showing a new aneurysmatic ventricular pouch with a very thin wall in free communication with the left ventricular aneurysm previously noted (Fig 1). Surgical treatment was then advised. At operation, performed using cardiopulmonary bypass, a biloculated aneurysm was Accepted for publication June29, 1990. Address reprint requests to Dr Di Eusanio, Divisione di Cardiochirurgia, Ospedale Civile G. Mazzini, Villa Mosca 64100, Italy.
0 1991 by The Society of Thoracic Surgeons
found in which the proximal part was probably the result of the anterior myocardial infarct whereas the distal one appeared to be of mycotic origin (Fig 2). The aneurysm was resected, and samples were sent for bacteriological and histological studies. The postoperative course was uneventful, but a month later, a pulsatile aneurysm recurred in the femoral artery, initially requiring iliacfemoral bypass and then, because of its recurrence in the distal anastomotic site, requiring reconstruction of the artery using a 7-mm Gore-Tex graft. The macroscopic appearance of the femoral aneurysm was of inflammatory origin, but all the cultures were sterile. During the patient’s hospital stay, oral and genital ulcerations were noted and Behcet’s disease was then suspected. The histological slides taken from the left ventricular and femoral artery aneurysms were then looked at again, and the diagnosis was confirmed by recognizing the typical appearance of Behset’s disease. Corticosteroid and immunosuppressive therapy was given; the boy recovered completely, and he is well after 16 months.
Comment A syndrome characterized by the triad of recurrent orogenital ulcerations, relapsing iridocyclitis, and erythema nodosum was described by Behfet [3] in 1937. Since then many other clinical manifestations of the same disease were recognized, conferring the character of a systemic disease to Behcet’s syndrome [4]. Its cause is unknown, but the pathogenesis is probably due to an immunopathologic disorder because of the characteristic vasculitis with early mononuclear and late polymorphonuclear cell infiltration that is always present in the involved organs and systems. This hypothesis is also supported by the good response to corticosteroid and immunosuppressive ther-
~ P [41. Y
The disease affect young adults especially in Japan, the Middle East, Cyprus, Turkey, and Greece with a prevalence of 10/100,000 population in Japan [4, 51. Oral and genital ulcerations are nearly constant features. The eye may be involved resulting in inflammation of the anterior chamber, conjunctivitis, scleritis, retinal vasculitis, and optic neuritis [4]. Erythema nodosum, the third element of the syndrome, has been reported in about 80% of patients [6]. Inflammatory polyarthritis is present in about half of the patients and most often involves the superficial layers of the cartilages of the knees and ankles [4]. Neurological manifestations are related to meningoencephalitis, cranial nerve palsies, or pseudotumor cerebri, 0003-4975/91/$3.50
132
Ann Thorac Surg
CASE REPORT DI EUSANIO ET AL LV ANEURYSM IN BEHCET’S DISEASE
1991;51:131-2
Fig 2. lntraoperative aspect of left ventricular aneurysm. Fig I . Preoperative two-dimensional echocardiogram demonstrating left ventricular aneu y s m . Arrows indicate the communication between the aneurysm and the left ventricle. (AD = right atrium; AS = left atrium; VD = right ventricle; VS = left ventricle.)
but more often to brainstem involvement with dysfunction resembling minor strokes [4].Decreased activity of the fibrinolytic system is responsible for thrombotic occlusion of the arteries and veins [6]. Cardiovascular involvement is present in 7% to 29% of the patients [l]and is characterized by recurrent thrombophlebitis; arterial occlusion; aneurysm of the aorta, its major branches, and peripheral arteries especially at the site of arterial punctures; aneurysm formation of the pulmonary artery; pericarditis; myocarditis; endocardia1 fibrosis; conduction defects; and aortic regurgitation [I, 6, 7-91. In our patient, most of the cardiovascular manifestations of Behqet’s syndrome were present; however, the macroscopic appearance of the myocardium involved in the ventricular aneurysm and of the aneurysm in the femoral artery were misleading in that they were strongly suggestive of infective process. The late appearance of orogenital ulceration led to the right diagnosis.
References 1. James DG, Thompson A. Recognition of diverse cardiovascular manifestation in Behqet disease. Am Heart J 1982;45:595602. 2. Rae SA, Vandemburg M, Scholtz CL. Aortic regurgitation and false aortic aneurysm formation in Behcet disease. Postgrad Med J 1980;56:438-9. 3. Behqet H. Ueber residivierente, aphthoese durch ein virus verurdachte Geschwure am Mund am Auge und an der Genitalien. Dermatol Monatsschr 1937;105:1152-7. 4. James DG. Behqet’s syndrome. New Engl J Med 1979;308: 431-2. 5. Chamberlain MA. Behqet disease. Br Med J 1978;2:1369-0. 6. Chajek T, Fainaru M. Behqet disease with decreased fibrinolysis and superior vena cava obstruction. Br Med J 1973;l: 7824. 7. Salamon F, Weinberger A, Nili M, et al. Massive hemoptysis complicating Behqet’s syndrome: the importance of early pulmonary angiography and operation. Ann Thorac Surg 1988;45:566-7. 8. Kingston M, Ratcliffe JR, Alltree M, Merendino KA. Aneurysm after arterial puncture in Behcet disease. Br Med J 1979;1:1766-7. 9. Bowles CA, Nelson AM, Hammil SC, ODufYy JD. Cardiac involvement in Behqet’s disease. Arthritis Rheum 1985;28: 345-8.
