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A strange complex of diffuse congenital cardiovascular disease and cardiomyopathy, with localised myocardial calcification

Published online by Cambridge University Press:  19 August 2008

L. M. Gerlis  and
J. Somerville
L. M. Gerlis*
Affiliation:
Departments of Paediatric Cardiac Morphology and the Jane Somerville Grown-up Congenital Heart Unit, The Royal Brompton Hospital, Imperial College School of Medicine, London, UK
J. Somerville
Affiliation:
Departments of Paediatric Cardiac Morphology and the Jane Somerville Grown-up Congenital Heart Unit, The Royal Brompton Hospital, Imperial College School of Medicine, London, UK
*
Dr L. M. Gerlis, Imperial College School of Medicine at The National Heart and Lung Institute, Dovehouse Street, London SW3 6LY, UK. Tel: 0171 351 8750; Fax: 0171 351 8230
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Abstract

This unique case is of a man, followed clinically since infancy, who had a ventricular septal defect which closed spontaneously, a small arterial duct, and a minor degree of aortic coarctation, all without obvious symptoms. He later developed progressive cardiac failure which was attributed to some obscure form of congenital cardiomyopathy. He died at the age of 45 years. Necropsy showed a grossly abnormal arrangement of ventricular myocardial fascicles and bands, with absence of the papillary muscles causing tricuspid and mitral regurgitation. The various malformations are considered to be a gross example of a diffuse congenital cardiovascular disease complex, to the best of our knowledge previously undescribed.

Keywords

Absent papillary musclescongenital cardiomyopathybifoliate aortic valvecoarctation of aortacongenital cardiac malformationmyocardial calcificationmycordial fascicular disarrayventricular septal defect
Type
Clinco-Pathological Correlation
Information
Cardiology in the Young , Volume 9 , Issue 4 , July 1999 , pp. 348 - 356
Copyright
Copyright © Cambridge University Press 1999

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References

1.Somerville, J. Congenital cardiovascular disease or congenital heart disease? – a time for change in concepts. Paediatric cardiology Vol. 3. Eds Becker, AE, Losekoot, TG, Marcelletti, C, Anderson, RH. Churchill Livingstone, London 1981; pp 324–345.Google Scholar
2.Shone, JD, Sellers, RD, Anderson, RC, Adams, P, Lillelei, CW, Edwards, JE. The developmental complex of “Parachute Mitral Valve”, supravalvar ring of the left atrium, subaortic stenosis and coarctation of the aorta. Amer J Cardiol 1963; 11: 714–725.CrossRefGoogle Scholar
3.Becker, AE, Becker, MJ, Edwards, JE. Anomalies associated with coarctation of the aorta. Circulation 1970; 41: 1067–1075.CrossRefGoogle Scholar
4.Goldberg, SJ, Gerlis, LM, Ho, SY, Penilla, MB. Location of the left papillary muscles in juxtaductal aortic coarctation. Amer J Cardiol 1995; 75: 746–750.CrossRefGoogle ScholarPubMed
5.Kappetein, AP, Gittenberger-de-Groote, AC, Zwinderman, AH, Poelmann, RE, Huysmans, HA. The neural crest as a possible factor in coarctation of the aorta and bicuspid aortic valve. J Thorac Cardiovasc Surg 1991; 102: 830–836.CrossRefGoogle ScholarPubMed
6.Flege, JB, Vlad, P, Ehrenhaft, JL. Congenital mitral incompetence. J Cardiovasc Surg 1967; 53: 138–144.Google ScholarPubMed
7.Terzaki, AK, Leachman, RD, Ali, MK, Hallman, GL, Cooley, DA. Congenital mitral incompetence and coarctation of the aorta. Thorax, 1972; 27: 729–737.CrossRefGoogle Scholar
8.Rosenquist, GG. Congenital mitral valve disease associated with coarctation of the aorta. Circularion 1974; 49: 985–993.Google ScholarPubMed
9.Davachi, F, Moller, JH, Edwards, JE. Diseases of the mitral valve in infancy. An anatomic analysis of 55 cases. Circulation 1971; 43: 565–579.CrossRefGoogle ScholarPubMed
10.Roberts, WC, Cohen, LS. Left ventricular papillary muscles. Circulation 1972; 46: 138–154.CrossRefGoogle ScholarPubMed
11.Bett, JNH, Stovin, PGI. Parachute deformity of the mitral valve. Thorax 1969; 24: 632–636.CrossRefGoogle ScholarPubMed
12.Edwards, JE, Burchell, HB. Pathological anatomy of mitral insufficiency. Proc Staff Meet. Mayo Clin 1958; 33: 497–509.Google ScholarPubMed
13.Lamers, WH, Viragh, S, Wessels, A, Moorman, AFM, Anderson, RH. Formation of the tricuspid valve in the human heart. Circulation 1995; 91: 111–121.CrossRefGoogle ScholarPubMed
14.Robb, JS, Robb, RC. Abnormal distribution of the superficial muscle bundles in the human heart. Am Heart J 1938; 15: 597–603.CrossRefGoogle Scholar
15.MacCullum, JB. On the muscular architecture and growth of the ventricles of the heart.Google Scholar
16.John, Hopkins Hosp Rep 1900; 9: 307–335.CrossRefGoogle Scholar
17.Kirk, RS, Russell, JGB. Spiral calcification in the ventricular myocardium. Br Heart J 1966; 28: 342–349.CrossRefGoogle ScholarPubMed
18.Becker, AE, Caruso, G. Congenital heart disease – a morphologists view on myocardial dysfunction. In Paediatric Cardiology Vol. 3. Eds. Becker, AE, Losekoot, TG, Marcelletti, C, Anderson, RH. Churchill Livingstone, Edinburgh, 1981; pp 307–323.Google Scholar
19.Robb, JS, Robb, RC. The normal heart. Anatomy and physiology of the strucrural units. Am Heart J 1942; 23: 455–467.CrossRefGoogle Scholar
20.Lowe, TE. Some principles governing the supply of blood to the myocardium in occlusive arterial disease. Am Heart J 1941; 21: 326–344.CrossRefGoogle Scholar
21.Robb, JS, Robb, RC. Localisation of cardiac infarcts in man: A comparison of interior – posterior with muscle bundle nodes of localisation. Am J Med Sci 1939; 197: 7–17.CrossRefGoogle Scholar
22.Wartman, WB, Sonders, JC. Localisation of myocardial infarcts with respect to the muscle bundles of the heart. Arch Path 1950; 50: 329–346.Google Scholar
23.Becker, AE, Becker, MJ, Edwards, JE. Anomalies associated with coarctation of the aorta.Google Scholar
24. Circulation 1970; 41: 1067–1075.CrossRefGoogle Scholar
25.Rosenquist, GC. Congenital mitral valve disease associated with coarctation of the aorta. Circulation 1974; 49: 985–993.CrossRefGoogle ScholarPubMed
26.Esterly, JR, Oppenheimer, EH. Some aspects of cardiac pathology in infancy and childhood IV. Myocardial and coronary lesions in cardiac malformations. Pediatrics 1967; 39: 896–903.CrossRefGoogle ScholarPubMed
27.Franciosi, RA, Blanc, WA. Myocardial infarcts in infants and children. IA necropsy study in congenital heart disease. J Pediat 1968; 73: 309–319.CrossRefGoogle ScholarPubMed
28.Somerville, J, Becu, L. Congenital heart disease associated with hypertrophic cardiomyopathy. Br Heart J 1978; 40: 1034–1039.CrossRefGoogle ScholarPubMed
29.Becu, L, Somerville, J, Gallo, A. “Isolated” pulmonary valve stenosis as part of a more widespread cardiovascular disease. Br Heart J 1976; 38: 472–482.CrossRefGoogle ScholarPubMed