Pediatric Cardiology

, Volume 4, Issue 4, pp 265–272 | Cite as

Reduced left ventricular size and endocardial fibroelastosis as correlates of mortality in newborns and young infants with severe aortic valve stenosis

  • R. Mocellin
  • U. Sauer
  • B. Simon
  • M. Comazzi
  • F. Sebening
  • K. Bühlmeyer
Article

Summary

30 patients with severe aortic valve stenosis presented in severe congestive heart failure within the first 2 months of life. In 25 of them, left ventricular volume and contractility were assessed; five of them had a left ventricle of normal size, in 11 left ventricular size was diminished, and in nine patients it was enlarged. Eleven of the infants had extensive endocardial fibroelastosis (EFE) evidenced angiographically by myocardial sinusoids in ten of them and established at autopsy in six. The presence of EFE correlated with the size of the left ventricle; eight of 11 with a small left ventricle, two of five with a normal-sized left ventricle, and one of nine with an enlarged left ventricle displayed EFE. The severe depression of left ventricular function associated with EFE was documented by left ventricular volume determinations on exclusion of the myocardial sinusoids. Of 30 patients, 12 (including eight of 26 who underwent surgery) did not survive. Mortality, severity, and early onset of symptoms were associated mainly with small size of the left ventricle and with the severe left ventricular dysfunction associated with EFE.

Key words

Aortic valve stenosis Endocardial fibroelastosis Neonates Left ventricular dysfunction 

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References

  1. 1.
    Bellet S, Gouley BA (1932) Congenital heart disease with multiple cardiac anomalies: report of a case showing aortic atresia, fibrous scar in myocardium and embryonal sinusoidal remains.Am J Med Sci 183:458Google Scholar
  2. 2.
    Bernhard WF, Keane JF, Fellows KE, Litwin SB, Gross RE (1973): Progress and problems in the surgical management of congenital aortic stenosis.J Thorac Cardiovasc Surg 66:404PubMedGoogle Scholar
  3. 3.
    Bühlmeyer K, Simon B, Mocellin R, Sauer U (1979) Clinical, angiocardiographic and functional studies in the assessment of critical valvular aortic stenosis: In: Goddman MJ, Marquis RM (eds)Paediatric cardiology, vol 2:Heart disease in the newborn. Churchill Livingstone, EdinburghGoogle Scholar
  4. 4.
    Blakeway H (1917/18) A hitherto undescribed malformation of the heart.J Anat 52:354Google Scholar
  5. 5.
    Bryan CS, Oppenheimer EH (1969) Ventricular endocardial fibroelastosis: basis for its presence or absence in cases of pulmonic and aortic atresia.Arch Pathol 87:82PubMedGoogle Scholar
  6. 6.
    Chiarello L, Vlad P, Subramanian S (1976) Surgical treatment of congenital valvular aortic stenosis.Thorax 31:398PubMedGoogle Scholar
  7. 7.
    Coran AG, Bernhard WF (1969) The surgical management of valvular aortic stenosis during infancy. J Thorac Cardiovasc Surg58:401PubMedGoogle Scholar
  8. 8.
    Dodge HT, Baxley WA (1969) Left ventricular volume and mass and their significance in heart disease.Am J Cardiol 23:528CrossRefPubMedGoogle Scholar
  9. 9.
    Edmunds LH, Wagner HR, Heymann MA (1980) Aortic valvulotomy in neonates.Circulation 61:421PubMedGoogle Scholar
  10. 10.
    Fishman NH, Hof R, Rudolph AM, Heymann MA (1972) Effect of banding ascending aorta on cardiovascular function and development in fetal lambs.Circulation (Suppl 2)46:5PubMedGoogle Scholar
  11. 11.
    Gaasch WH, Battle WE, Obolev AA, Banas JS, Levine HJ (1972) Left ventricular stress and compliance in man.Circulation 45:746PubMedGoogle Scholar
  12. 12.
    Graham TP, Jarmakani JM, Canent RV, Morrow MN (1971) Left heart volume estimation in infancy and childhood: reevaluation of methodology and normal values.Circulation 43:895PubMedGoogle Scholar
  13. 13.
    Hallmann K, Mocellin R, Gössner W, Bühlmeyer K (1983) Ungewöhnliche Kontrastmittelverteilung im linksventrikulären Myokard bei Säuglingen mit hochgradiger valvulärer Aortenstenose und Endokardfibroelastose. Pathologisch-morphologische Befunde.Z Kardiol (in press)Google Scholar
  14. 14.
    Hastreiter AR, Oshima M, Miller RA, Lev M, Paul M (1962) Congenital aortic stenosis syndrome in infancy.Circulation 28:1084Google Scholar
  15. 15.
    Keane JF, Bernhard WF, Nadas AS (1975) Aortic stenosis surgery in infancy.Circulation 52:1138PubMedGoogle Scholar
  16. 16.
    Kennedy JW, Twiss RD, Blackmon JR, Dodge HT (1968) Quantitative angiography: 3. Relationships of left ventricular pressure, volume and mass in aortic valve disease.Circulation 38:838PubMedGoogle Scholar
  17. 17.
    Lakier JB, Lewis AB, Heymann MA, Stanger P, Hoffman JIE, Rudolph AM (1974) Isolated aortic stenosis in the neonate: natural history and hemodynamic considerations.Circulation 50:801PubMedGoogle Scholar
  18. 18.
    Liedke AJ, Gentzler RD, Babb JD, Hunter AS, Gault JH (1976) Determinants of cardiac performance in severe aortic stenosis.Chest 69:192PubMedGoogle Scholar
  19. 19.
    Mody MR, Nadas AS, Bernhard WF (1967) Aortic stenosis in infants.N Engl J Med 276:832PubMedGoogle Scholar
  20. 20.
    Nakazawa M, Marks RA, Isabel-Jones J, Jarmakani JM (1976) Right and left ventricular volume characteristics in children with pulmonary stenosis and intact ventricular septum.Circulation 53:884PubMedGoogle Scholar
  21. 21.
    Neutze JM (1973) Congenital heart disease in infancy [panel discussion]. In: Barratt-Boyes BG, Neutze JM, Harris EA (eds)Heart disease in infancy: diagnosis and surgical treatment. Churchill Livingstone, London, p. 303Google Scholar
  22. 22.
    Raghib G, Bloemendaal RD, Kanjah VI, Edwards JE (1965) Aortic atresia and premature closure of the foramen ovale: myocardial sinusoids and coronary arteriovenous fistula serving as outflow channel.Am Heart J 70:476CrossRefPubMedGoogle Scholar
  23. 23.
    Simon B, Sauer U, Mocellin R, Sebening F (1977) Aortenklappenstenose im Säuglingsalter: Diagnostik und Behandlungsergebnisse.Herz 2:420Google Scholar
  24. 24.
    Weber KT, Janicke JS (1975) The calculation of the mean pressure gradient in aortic valve stenosis: a simplified approach.Am Heart J 89:126CrossRefPubMedGoogle Scholar
  25. 25.
    Worms AM, Stehlin H, Didier F, Plenat F, Neveux JY, Pernot C (1977) La sténose aortique valvulaire à expression néonatale: a propos de 8 observations.Arch Mal Coeur 70:329A0946016 00004 CS-SPJRNPDF [HEADSUP]PubMedGoogle Scholar

Copyright information

© Springer-Verlag 1983

Authors and Affiliations

  • R. Mocellin
    • 1
    • 2
  • U. Sauer
    • 1
    • 2
  • B. Simon
    • 1
    • 2
  • M. Comazzi
    • 1
    • 2
  • F. Sebening
    • 1
    • 2
  • K. Bühlmeyer
    • 1
    • 2
  1. 1.Clinic for Cardiovascular Diseases in Children, and Clinic for Cardiovascular SurgeryGerman Heart CenterMunich
  2. 2.Department of Pediatric CardiologyFreiburgFederal Republic of Germany

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