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Congestive heart failure due to aortic incompetence with intestinal infarction due to endarteritis obiliterans
356 4 Yater WM. Variations and anomalies of the venous valves of the right atrium of the human heart. Arch Pathol 1929; 7:418-441. 5 Kinney EL, Wright RJ. Efficacy of treatment of patients with echocardiographically detected right-sided heart thrombi: a meta-analysis. Am Heart J 1989;118:569-573.
Farfel 2. Shechter M, Vered Z, Rath S, Goor D, Gafni J. Review of echocardiographically diagnosed right heart entrapment of pulmonary emboli-in-transit with emphasis on management. Am Heart J 1987;113:171-178. Covarrabias EA. Shekh MU, Fox LM. Echocardiography and pulmonary embolism. AM Intern Med 1977;87:720721.
International Journal of Cardiology, 30 (1990) 356-358 0 1991 Elsevier Science Publishers B.V. 0167-5273/91/$03.50 ADONIS 016752739100088Q
CARD10
12164
Congestive heart failure due to aortic incompetence with intestinal infarction due to endarteritis obiliterans J.A. Purvis ‘, A.A.J. Adgey 1 and M.D. O’Hara
2
’ Regional Medical Cardiology Centre, and ’ Queen’s University of Beljast. Department of Pathology, Royal Victoria Hospital, Belfast, N. Ireland (Received
8 September
1990; revision
accepted
28 September
1990)
A 27-year-old man with congestive heart failure due to aortic incompetence and subsequent intestinal infarction was found at laparotomy to have extensive necrosis of the bowel due to proliferative endarteritis. Symptoms resolved following treatment with prednisolone and cyclophosphamide, and replacement of the aortic valve. The sub-total occlusion produced by endarteritis obliterans may lead to acute end-organ infarction if cardiac output is reduced. Key words:
Congestive
heart failure;
Aortic incompetence;
Endarteritis
obliterans;
Intestinal
infarction
Introduction
Case Report
Polyarteritis nodosa can present as end-organ infarction due to thrombosis of medium-sized arteries, with acute inflammatory changes and fibrinoid necrosis of the internal elastic lamina. Our patient had classical prodromal symptoms and developed intestinal infarction during a period of diminished cardiac output secondary to congestive heart failure complicating significant aortic incompetence. Histologic examination of the culprit arteries revealed vessels compromised by fibro-intimal hyperplasia without acute vasculitis.
A 27-year-old man was admitted from a referring hospital with a 2-week history of increasing breathlessness and 2 days of pyrexia, malaise, vomiting, abdominal pain and blood-stained diarrhoea. He gave a 3-year history of progressive weight loss, myalgia and hypertension. Two years prior to admission he had had polyneuropathy. Medication on admission was atenolol 100 mg and enalapril 5 mg orally daily for hypertension. He was pyrexic, 38.5 “C, underweight 52 kg and tachypnoeic. The pulse was at the rate of lOO/min, regular, and collapsing in character. The blood pressure was 160/90 mmHg and the jugular venous pressure was elevated 6 cm. A harsh aortic systolic murmur was audible and a
Correspondence
to: Dr. A.A.J. Adgey M.D., Regional
Medi-
cal Cardiology Centre, Royal Victoria Hospital, Belfast BT12 6BA. N. Ireland.
Fig. 1. Marked fibrointimal hyperplasia has caused significant narrowing of the lumen. The adventitial tissues are uninvolved. (Haematoxylin and eosin. x 60.)
long immediate early diastolic murmur radiated from the aortic area to the apex of the heart. There were bilateral basal crepitations in the lung fields. Guarding and rebound tenderness were present in the right iliac fossa, while dark-red blood was noted in the stools on rectal examination. There were no abnormal neurological signs. Blood counts were also unremarkable, except the white cell count at 21,900/ mm3. The chest X-ray showed left ventricular prominence and pulmonary oedema, while the echocardiogram showed a dilated left ventricle with poor systolic function. There was moderately severe aortic incompetence. No vegetations were seen on the leaflets of the aortic valve. Congestive heart failure responded well to intravenous diuretics and digitalis, but his abdominal pain worsened. Laparotomy performed 3 days after admission showed that the ileo-colic, right, and middle colic arteries were pulseless, with gangrene of the terminal
ileum, caecum and right hemicolon. A sub-total colectomy with ileostomy was performed. Pathological examination revealed ischaemic necrosis of the bowel. The medium-sized mesenteric arteries showed gross fibro-intimal hyperplasia, with subtotal occlusion in some vessels and overlying thrombus in others. The internal elastic lamina was preserved (Fig. 1). There was no evidence of an active arteritis or involvement of adjacent veins or nerves. He required further abdominal surgery one month after ileostomy for division of adhesions. Digital subtraction aortic arteriography demonstrated fusiform aneurysms in the internal mammary (Fig. 2) hepatic, splenic, renal and superior mesenteric arteries. Prednisolone given 20 mg thrice daily, and cyclophosphamide 100 mg orally daily, were commenced, with subsequent resolution of abdominal symptoms and weight gain. Cardiac failure resolved with oral frusemide and digoxin.
Fig. 2. Digital subtraction arteriogram demonstrating the right subelavian artery (RSA) and aneurysmal dilatation (An) of the right internal mammary artery (RIMA).
the vessel wall. By strict definition, all three layers of the vessel wall should be involved [2]. In patients seen in the United Kingdom, prodromal symptoms of myalgia and loss of weight occur in up to 70%, intestinal infarction in 19%, whilst only 6% develop valvitis [3]. In our patient, microscopy demonstrated an unusual but non-specific fibro-intimal hyperplasia with no evidence of fibrinoid necrosis or infiltration of inflammatory cells. Buerger’s disease, and homocystinuria, were considered in the differential diagnosis but discounted after arteriography showed the presence of fusiform aneurysms characteristic of polyarteritis nodosa. Biochemical screening for homocystinuria proved negative. Obliterative endarteritis is a recognised intimal response to chronic inflammatory diseases such as syphilis and tuberculosis [4] and, with sub-total arterial occlusion, can result in significant mortality and morbidity. McCurley and Collins have reported three cases of intestinal infarction due to endarteritis obliterans in sero-positive rheumatoid arthritis [5]. In all three cases: infarction followed periods of decreased cardiac output. In our patient, the decrease in mesenteric blood supply resulting from cardiac failure (in vessels already compromised by proliferative intimal changes) may have led to intestinal infarction. References
Replacement of the aortic valve was carried out 7 months later after severe aortic incompetence was confirmed at cardiac catheterisation. Histology of the excised aortic wall and valvar leaflets showed myxomatous degeneration with no evidence of active valvitis or aortitis. Since discharge he has maintained good health on cyclophosphamide and prednisolone. Discussion Current theories regarding the aetiology of polyarteritis nodasa suggest an inflammatory response to deposition of immune complex in vascular tissue [l]. The two main components of polyarteritis nodosa are fibrinoid necrosis and inflammatory cell’ infiltration of
Scott DGI. Vasculitis. In: Scott JT, ed. Copeman’s textbook of the rheumatic diseases, vol. 2.6th ed. Edinburgh: Churchill Livingstone, 1986;1292-1324. Neild GH, Williams DG. Polyarteritis and related syndromes. In: Weatherall DJ, Ledingham JGG, Warrell DA, eds. Oxford textbook of medicine, vol. 2. 2nd ed. Oxford: Oxford University Press, 1987; 16.28-34. Scott DGI, Bacon PA, Elliott PJ, Tribe CR, Wallington TB. Systemic vasculitis in a district general hospital 1972-1980: clinical and laboratory features, classification and prognosis of 80 cases. Q J Med 1982;203:292-311. Lindop G. Blood vessels and lymphatics. In: Anderson, JR, ed. Muirs textbook of pathology. 12th ed. London: Edward Arnold, 1985;14.2. McCurley TL, Collins RD. Intestinal infarction in rheumatoid arthritis. Three cases due to unusual obliterative vascular lesions. Arch Pathol Lab Med 1984;108:125-128.
Farfel 2. Shechter M, Vered Z, Rath S, Goor D, Gafni J. Review of echocardiographically diagnosed right heart entrapment of pulmonary emboli-in-transit with emphasis on management. Am Heart J 1987;113:171-178. Covarrabias EA. Shekh MU, Fox LM. Echocardiography and pulmonary embolism. AM Intern Med 1977;87:720721.
International Journal of Cardiology, 30 (1990) 356-358 0 1991 Elsevier Science Publishers B.V. 0167-5273/91/$03.50 ADONIS 016752739100088Q
CARD10
12164
Congestive heart failure due to aortic incompetence with intestinal infarction due to endarteritis obiliterans J.A. Purvis ‘, A.A.J. Adgey 1 and M.D. O’Hara
2
’ Regional Medical Cardiology Centre, and ’ Queen’s University of Beljast. Department of Pathology, Royal Victoria Hospital, Belfast, N. Ireland (Received
8 September
1990; revision
accepted
28 September
1990)
A 27-year-old man with congestive heart failure due to aortic incompetence and subsequent intestinal infarction was found at laparotomy to have extensive necrosis of the bowel due to proliferative endarteritis. Symptoms resolved following treatment with prednisolone and cyclophosphamide, and replacement of the aortic valve. The sub-total occlusion produced by endarteritis obliterans may lead to acute end-organ infarction if cardiac output is reduced. Key words:
Congestive
heart failure;
Aortic incompetence;
Endarteritis
obliterans;
Intestinal
infarction
Introduction
Case Report
Polyarteritis nodosa can present as end-organ infarction due to thrombosis of medium-sized arteries, with acute inflammatory changes and fibrinoid necrosis of the internal elastic lamina. Our patient had classical prodromal symptoms and developed intestinal infarction during a period of diminished cardiac output secondary to congestive heart failure complicating significant aortic incompetence. Histologic examination of the culprit arteries revealed vessels compromised by fibro-intimal hyperplasia without acute vasculitis.
A 27-year-old man was admitted from a referring hospital with a 2-week history of increasing breathlessness and 2 days of pyrexia, malaise, vomiting, abdominal pain and blood-stained diarrhoea. He gave a 3-year history of progressive weight loss, myalgia and hypertension. Two years prior to admission he had had polyneuropathy. Medication on admission was atenolol 100 mg and enalapril 5 mg orally daily for hypertension. He was pyrexic, 38.5 “C, underweight 52 kg and tachypnoeic. The pulse was at the rate of lOO/min, regular, and collapsing in character. The blood pressure was 160/90 mmHg and the jugular venous pressure was elevated 6 cm. A harsh aortic systolic murmur was audible and a
Correspondence
to: Dr. A.A.J. Adgey M.D., Regional
Medi-
cal Cardiology Centre, Royal Victoria Hospital, Belfast BT12 6BA. N. Ireland.
Fig. 1. Marked fibrointimal hyperplasia has caused significant narrowing of the lumen. The adventitial tissues are uninvolved. (Haematoxylin and eosin. x 60.)
long immediate early diastolic murmur radiated from the aortic area to the apex of the heart. There were bilateral basal crepitations in the lung fields. Guarding and rebound tenderness were present in the right iliac fossa, while dark-red blood was noted in the stools on rectal examination. There were no abnormal neurological signs. Blood counts were also unremarkable, except the white cell count at 21,900/ mm3. The chest X-ray showed left ventricular prominence and pulmonary oedema, while the echocardiogram showed a dilated left ventricle with poor systolic function. There was moderately severe aortic incompetence. No vegetations were seen on the leaflets of the aortic valve. Congestive heart failure responded well to intravenous diuretics and digitalis, but his abdominal pain worsened. Laparotomy performed 3 days after admission showed that the ileo-colic, right, and middle colic arteries were pulseless, with gangrene of the terminal
ileum, caecum and right hemicolon. A sub-total colectomy with ileostomy was performed. Pathological examination revealed ischaemic necrosis of the bowel. The medium-sized mesenteric arteries showed gross fibro-intimal hyperplasia, with subtotal occlusion in some vessels and overlying thrombus in others. The internal elastic lamina was preserved (Fig. 1). There was no evidence of an active arteritis or involvement of adjacent veins or nerves. He required further abdominal surgery one month after ileostomy for division of adhesions. Digital subtraction aortic arteriography demonstrated fusiform aneurysms in the internal mammary (Fig. 2) hepatic, splenic, renal and superior mesenteric arteries. Prednisolone given 20 mg thrice daily, and cyclophosphamide 100 mg orally daily, were commenced, with subsequent resolution of abdominal symptoms and weight gain. Cardiac failure resolved with oral frusemide and digoxin.
Fig. 2. Digital subtraction arteriogram demonstrating the right subelavian artery (RSA) and aneurysmal dilatation (An) of the right internal mammary artery (RIMA).
the vessel wall. By strict definition, all three layers of the vessel wall should be involved [2]. In patients seen in the United Kingdom, prodromal symptoms of myalgia and loss of weight occur in up to 70%, intestinal infarction in 19%, whilst only 6% develop valvitis [3]. In our patient, microscopy demonstrated an unusual but non-specific fibro-intimal hyperplasia with no evidence of fibrinoid necrosis or infiltration of inflammatory cells. Buerger’s disease, and homocystinuria, were considered in the differential diagnosis but discounted after arteriography showed the presence of fusiform aneurysms characteristic of polyarteritis nodosa. Biochemical screening for homocystinuria proved negative. Obliterative endarteritis is a recognised intimal response to chronic inflammatory diseases such as syphilis and tuberculosis [4] and, with sub-total arterial occlusion, can result in significant mortality and morbidity. McCurley and Collins have reported three cases of intestinal infarction due to endarteritis obliterans in sero-positive rheumatoid arthritis [5]. In all three cases: infarction followed periods of decreased cardiac output. In our patient, the decrease in mesenteric blood supply resulting from cardiac failure (in vessels already compromised by proliferative intimal changes) may have led to intestinal infarction. References
Replacement of the aortic valve was carried out 7 months later after severe aortic incompetence was confirmed at cardiac catheterisation. Histology of the excised aortic wall and valvar leaflets showed myxomatous degeneration with no evidence of active valvitis or aortitis. Since discharge he has maintained good health on cyclophosphamide and prednisolone. Discussion Current theories regarding the aetiology of polyarteritis nodasa suggest an inflammatory response to deposition of immune complex in vascular tissue [l]. The two main components of polyarteritis nodosa are fibrinoid necrosis and inflammatory cell’ infiltration of
Scott DGI. Vasculitis. In: Scott JT, ed. Copeman’s textbook of the rheumatic diseases, vol. 2.6th ed. Edinburgh: Churchill Livingstone, 1986;1292-1324. Neild GH, Williams DG. Polyarteritis and related syndromes. In: Weatherall DJ, Ledingham JGG, Warrell DA, eds. Oxford textbook of medicine, vol. 2. 2nd ed. Oxford: Oxford University Press, 1987; 16.28-34. Scott DGI, Bacon PA, Elliott PJ, Tribe CR, Wallington TB. Systemic vasculitis in a district general hospital 1972-1980: clinical and laboratory features, classification and prognosis of 80 cases. Q J Med 1982;203:292-311. Lindop G. Blood vessels and lymphatics. In: Anderson, JR, ed. Muirs textbook of pathology. 12th ed. London: Edward Arnold, 1985;14.2. McCurley TL, Collins RD. Intestinal infarction in rheumatoid arthritis. Three cases due to unusual obliterative vascular lesions. Arch Pathol Lab Med 1984;108:125-128.