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Obstructive calcification of the thoracic aorta revealed by dilated cardiomyopathy
International Elsevier
Journal of Cardiology,
371
19 (1988) 371-374
IJC 07011
Brief Reports
Obstructive calcification of the thoracic aorta revealed by dilated cardiomyopathy X. Marchand, D. Zannier and J.C. Kahn Department (Received
of Cardiology, Poissy Hospital, Paris West University, France 28 September
1987; revision
accepted
20 October
1987)
ObstrucGve calcification of the thoracic aorta appears as acquired coarctation of the aorta. The case we present is of note because it presented as a dilpted cardiomyopathy. The diagnosis, suspected on chest X-ray, was confiied by catheterization, aortic angiography and thoracic computer tomography. The patient onderwent aortic endarterectomy, but died in the post-operative period. Review of the literature shows that the pathogenesis of the condition remains UIlCl~. Key words: Dilated cardiomyopathy;
Aortic coarctation;
Obstructive calcification of aorta
Introduction We describe a case of obstructive calcification of the thoracic aorta which presented as a dilated cardiomyopathy with congestive heart failure. Case Report A 57-year-old man, with no previous history of cardiovascular disease or of fever, malaise or arthralgia, presented with left ventricular failure of one months duration. The physical examination revealed a normal blood pressure and no significant pressure difference between the right and left arms. There was a systolic murmur in the left interscapular region. The chest radiograph revealed an enlarged left ventricle, aortic knob calcification but no rib notching. The electrocardiogram revealed marked left ventricular strain. M-mode and cross-sectional echocardiography revealed only a dilated poorly contracting left ventricle with normal ventricular wall thickness. The patient underwent cardiac catheterization. A gradient of 20 mm Hg was found in the thoracic aorta between the left subclavian artery and the aortic isthmus. Because cardiac output was low the calculated orifice area of the obstruction was 0.60 cm2. Coronary
Correspondence Poissy, France.
0167-5273/88/$03.50
to: Dr. J.C. Kahn,
Dept. of Cardiology,
0 1988 EIsevier Science Publishers
Poissy Hospital,
B.V. (Biomedical
Paris West University,
Division)
78303
372
Fig. 1. Subtraction angiographyof aorta. Left oblique anterior
view.
arteriography was normal. An aortogram showed appearances similar to “pseudo-coarctation” above the origin of the left-subclavian artery which was stenosed (Fig. 1). Thoracic computer tomography with contrast (Fig. 2) showed a calcified stenosis of the lumen of the mid and posterior segments of the aortic arch with a normal external diameter. Diffuse calcification of the intima extended to the origin of the left subclavian artery. There were no signs of aortic dissection. Surgical repair was carried out. Aortic lumen was the site of heavy calcium deposition. Operation consisted of an endarterectomy of the aortic arch and the initial segment of descending aorta. The post-operative course was complicated by low cardiac output and the patient died on the fourth day after operation. Histological study of thoracic aorta revealed hyahne intimal fibrosis with marked calcification, absence of the internal elastic layer and calcified mural thrombus. Discussion The pathogenesis of this disease is unknown. The different hypotheses are unsatisfactory. The location of the stenosis, the absence of notching of the ribs and hypertension and the
Fig. 2. Computerized tomography through the aortic arch. Calcifications are visible.
normal external diameter of the aorta do not support the hypothesis [l] of an old true congenital coarctation which had calcified. Type I Takayasu’s arteritis [2,3] is worthy of consideration in this case because the left subclavian artery was involved. But aortographic appearances of this disease are characteristic with post-stenotic dilatation, saccular aneurysms and the typical “rattail” appearance, not seen in this case. Finally, the wall of the aorta, in the few cases of obstructive calcification of the thoracic aorta in the literature, did not have involvement of the media and adventitia, thus invalidating an arteritic aetiology. However, in our case absence of interval elastic layer was noticed. A localized aortic dissection with secondary calcification of a hematoma in the false channel is not a viable aetiology, because aortic dissection is characterized by typical aortic medial degeneration which was not present in this case. Furthermore, no dissection was found on thoracic computer tomography. Obstructive calcification of the thoracic aorta may develop on the basis of preexisting aortic atheroma [4]. Since atheroma was present in other arteries in our case this could be a possible cause. However, Borromee et al. [5] found no other atheromatous lesions in their case and no atheromatous lesion was noticed at the site of the coarctation.
314
Conclusion Obstructive calcification of the thoracic aorta may be a rare cause of an unexplained cardiomyopathy. The pathogenesis of the aortic calcification is unknown.
References Courbier R, Jausseran JM, Reggi M. Hypoplasie de l’aorte abdominale. Chirurgie 1974;100:284-290. Sen PK, Kinare SG, Engineer SD, Papulkar GB. The middle aortic syndrome. Br Heart J 1963;25:610-615. Chin& LA, Kronson I, Treman N, Kang JA. Total occlusion of the abdominal aorta in a patient with Takayasu’s arteritis. The importance of lower rib notching in the differential diagnosis. Cathet Cardiovasc Diagn 1986;12:405-408. Guihnet D, Tawil N, Bachet J, et al. Maladie obstructive calcifiante de l’aorte thoracique descendante. J Chir (Paris) 1982;119:157-160. Borromee L, Frija G, Baghn A, et al. Pseudo-coarctation due B une masse intra-aortique calcifiee. Sem Hap 1983;59:2827-2830.
International Elsevier
Journal of Cardiology,
19 (1988) 374-378
IJC 07012
Non-invasive diagnosis of ruptured sinus of Valsalva aneurysm by pulsed Doppler echocardiography S. Radhakrishnan, Department
of Cardiology (Received
V.K. Bahl and S. Shrivastava
All India Institute of Medical Sciences, Ansari Nagar, New Delhi, India 17 July 1987; revision accepted
3 November
1987)
Six cases of ruptured aneurysm of the sinus of Vakalva into the right ventricle, including two post-operative patients with residual murmurs have heen reported. Complete diagnosis, including that of residual and associated defects, was made non-invasively using cross-sectional and pulsed Doppler echauudiographic studies. ‘Ike exact site of rupture was localized by a continuous flow signal in the right ventricle. The diagnosis was subsequently confirmed by cardiac catheterization and angiocardiography in each case. Key words: Sinus of Valsalva aneurysm; Right ventricle; Pulsed Doppler echocardiogaphy
Correspondence to: S. Shrivastava, Dept. Ansari Nagar, New Delhi-110029, India.
of Cardiology,
0167-5273/88/$03.50
Publishers
0 1988 Elsevier Science
All India
B.V. (Biomedical
Institute
of Medical
Division)
Sciences,
Journal of Cardiology,
371
19 (1988) 371-374
IJC 07011
Brief Reports
Obstructive calcification of the thoracic aorta revealed by dilated cardiomyopathy X. Marchand, D. Zannier and J.C. Kahn Department (Received
of Cardiology, Poissy Hospital, Paris West University, France 28 September
1987; revision
accepted
20 October
1987)
ObstrucGve calcification of the thoracic aorta appears as acquired coarctation of the aorta. The case we present is of note because it presented as a dilpted cardiomyopathy. The diagnosis, suspected on chest X-ray, was confiied by catheterization, aortic angiography and thoracic computer tomography. The patient onderwent aortic endarterectomy, but died in the post-operative period. Review of the literature shows that the pathogenesis of the condition remains UIlCl~. Key words: Dilated cardiomyopathy;
Aortic coarctation;
Obstructive calcification of aorta
Introduction We describe a case of obstructive calcification of the thoracic aorta which presented as a dilated cardiomyopathy with congestive heart failure. Case Report A 57-year-old man, with no previous history of cardiovascular disease or of fever, malaise or arthralgia, presented with left ventricular failure of one months duration. The physical examination revealed a normal blood pressure and no significant pressure difference between the right and left arms. There was a systolic murmur in the left interscapular region. The chest radiograph revealed an enlarged left ventricle, aortic knob calcification but no rib notching. The electrocardiogram revealed marked left ventricular strain. M-mode and cross-sectional echocardiography revealed only a dilated poorly contracting left ventricle with normal ventricular wall thickness. The patient underwent cardiac catheterization. A gradient of 20 mm Hg was found in the thoracic aorta between the left subclavian artery and the aortic isthmus. Because cardiac output was low the calculated orifice area of the obstruction was 0.60 cm2. Coronary
Correspondence Poissy, France.
0167-5273/88/$03.50
to: Dr. J.C. Kahn,
Dept. of Cardiology,
0 1988 EIsevier Science Publishers
Poissy Hospital,
B.V. (Biomedical
Paris West University,
Division)
78303
372
Fig. 1. Subtraction angiographyof aorta. Left oblique anterior
view.
arteriography was normal. An aortogram showed appearances similar to “pseudo-coarctation” above the origin of the left-subclavian artery which was stenosed (Fig. 1). Thoracic computer tomography with contrast (Fig. 2) showed a calcified stenosis of the lumen of the mid and posterior segments of the aortic arch with a normal external diameter. Diffuse calcification of the intima extended to the origin of the left subclavian artery. There were no signs of aortic dissection. Surgical repair was carried out. Aortic lumen was the site of heavy calcium deposition. Operation consisted of an endarterectomy of the aortic arch and the initial segment of descending aorta. The post-operative course was complicated by low cardiac output and the patient died on the fourth day after operation. Histological study of thoracic aorta revealed hyahne intimal fibrosis with marked calcification, absence of the internal elastic layer and calcified mural thrombus. Discussion The pathogenesis of this disease is unknown. The different hypotheses are unsatisfactory. The location of the stenosis, the absence of notching of the ribs and hypertension and the
Fig. 2. Computerized tomography through the aortic arch. Calcifications are visible.
normal external diameter of the aorta do not support the hypothesis [l] of an old true congenital coarctation which had calcified. Type I Takayasu’s arteritis [2,3] is worthy of consideration in this case because the left subclavian artery was involved. But aortographic appearances of this disease are characteristic with post-stenotic dilatation, saccular aneurysms and the typical “rattail” appearance, not seen in this case. Finally, the wall of the aorta, in the few cases of obstructive calcification of the thoracic aorta in the literature, did not have involvement of the media and adventitia, thus invalidating an arteritic aetiology. However, in our case absence of interval elastic layer was noticed. A localized aortic dissection with secondary calcification of a hematoma in the false channel is not a viable aetiology, because aortic dissection is characterized by typical aortic medial degeneration which was not present in this case. Furthermore, no dissection was found on thoracic computer tomography. Obstructive calcification of the thoracic aorta may develop on the basis of preexisting aortic atheroma [4]. Since atheroma was present in other arteries in our case this could be a possible cause. However, Borromee et al. [5] found no other atheromatous lesions in their case and no atheromatous lesion was noticed at the site of the coarctation.
314
Conclusion Obstructive calcification of the thoracic aorta may be a rare cause of an unexplained cardiomyopathy. The pathogenesis of the aortic calcification is unknown.
References Courbier R, Jausseran JM, Reggi M. Hypoplasie de l’aorte abdominale. Chirurgie 1974;100:284-290. Sen PK, Kinare SG, Engineer SD, Papulkar GB. The middle aortic syndrome. Br Heart J 1963;25:610-615. Chin& LA, Kronson I, Treman N, Kang JA. Total occlusion of the abdominal aorta in a patient with Takayasu’s arteritis. The importance of lower rib notching in the differential diagnosis. Cathet Cardiovasc Diagn 1986;12:405-408. Guihnet D, Tawil N, Bachet J, et al. Maladie obstructive calcifiante de l’aorte thoracique descendante. J Chir (Paris) 1982;119:157-160. Borromee L, Frija G, Baghn A, et al. Pseudo-coarctation due B une masse intra-aortique calcifiee. Sem Hap 1983;59:2827-2830.
International Elsevier
Journal of Cardiology,
19 (1988) 374-378
IJC 07012
Non-invasive diagnosis of ruptured sinus of Valsalva aneurysm by pulsed Doppler echocardiography S. Radhakrishnan, Department
of Cardiology (Received
V.K. Bahl and S. Shrivastava
All India Institute of Medical Sciences, Ansari Nagar, New Delhi, India 17 July 1987; revision accepted
3 November
1987)
Six cases of ruptured aneurysm of the sinus of Vakalva into the right ventricle, including two post-operative patients with residual murmurs have heen reported. Complete diagnosis, including that of residual and associated defects, was made non-invasively using cross-sectional and pulsed Doppler echauudiographic studies. ‘Ike exact site of rupture was localized by a continuous flow signal in the right ventricle. The diagnosis was subsequently confirmed by cardiac catheterization and angiocardiography in each case. Key words: Sinus of Valsalva aneurysm; Right ventricle; Pulsed Doppler echocardiogaphy
Correspondence to: S. Shrivastava, Dept. Ansari Nagar, New Delhi-110029, India.
of Cardiology,
0167-5273/88/$03.50
Publishers
0 1988 Elsevier Science
All India
B.V. (Biomedical
Institute
of Medical
Division)
Sciences,