Abstract
We report a 10-month-old male infant who was admitted to our hospital with a history of failure to thrive and bulky stools. On examination, he was dystrophic and had a protruding abdomen, but he was well oxygenated and his lungs were clear on auscultation. A tachycardia of 145 beats per min and radiological evidence of cardiomegaly indicated involvement of the heart, but an ECG failed to show signs of myocarditis or cardiac hypertrophy. An elevated sweat chloride concentration of 141 mEq/l confirmed the diagnosis of cystic fibrosis (CF). Molecular analysis revealed heterozygosity for the common mutation delta F508. He died unexpectedly of a sudden cardiac arrest 2 days later. Autopsy revealed scattered myocardial necrosis and fibrosis. Some 50 documented cases of myocardial fibrosis in infants with CF have been reported. Suggested causes such as malnourishment and hypovitaminosis remain speculative as systematic studies have yet to be done.
This is a preview of subscription content, log in via an institution to check access.
Similar content being viewed by others
References
Batisse A, Fermont L, Kachaner J (1961) Myocardiopathies “degeneratives” de l'enfant. Ann Pediatr 28: 351–357
Boat TS (1992) Cystic fibrosis. In: Behrman RE, et al (eds) Nelson textbook of pediatrics. WB Saunders Company, Philadelphia, pp 1106–1116
Boivin MJ, Guillot F, Juvin AM, Gire R, Casasoprana A (1982) Fibrose myocardique au cours de la mucoviscidose. Arch Fr Pediatr 39: 27–29
Bragdon J, Levine HD (1949) Myocarditis in vitamin E-deficient rabbits. Am J Pathol 25: 265–271
Chéron G, Paradis K, Stéru D, Demay G, Lenoir G (1984) Cardiac involvement in cystic fibrosis revealed by a ventricular arrhythmia. Acta Paediatr Scand 73: 697–700
Cutting GR, Kasch LM, Rosenstein BJ, Zielinski J, Tsui L-C, Antonorakis SE, Kazazian HH jr (1990) A cluster of cystic fibrosis mutations in the first nucleotide-binding fold of the cystic fibrosis conductance regulator protein. Nature 346: 366–369
Danihel I, Zaviacic M, Janik P, Porubsky J, Babal P, Nagy V (1989) Myokardschädigung als seltene Komplikation der Mukoviszidose. Zentralbl Allg Pathol 135: 457–461
Dean M, White MB, Amos J, Gerrard B, Stewart C, Khaw K-T, Leppert M (1990) Multiple mutations in highly conserved residues are found in mildly affected cystic fibrosis patients. Cell 61: 863–870
Deschamp J-P, Didier F, Eloquet J (1971) Mucoviscidose révélée et dominée par ses manifestations cardiaques. Arch Fr Pediatr 28: 417–424
Feer W (1952) Xerophthalmie und Myokardschaden bei Pankreasfibrose. Schweiz Med Wochenschr 82: 102–106
Friedman KJ, Highsmith WE jr, Silverman LM (1991) Detecting multiple cystic fibrosis mutations by polymerase chain reaction-mediated site-directed mutagenesis. Clin Chem 37: 753–755
Guesnu M, Feigelson J, Travert G, Vidailhet M, Navarro J (1982) Taux élevé de trypsine immuno-réactive circulante lors du'une myocardiopathie primitive de mucoviscidose. Arch Fr Pediatr 39: 127–128
Hide DW, Martlew R (1977) Cystic fibrosis and myocardial fibrosis. Arch Dis Child 52: 163–165
Linhartová A (1962) Myokardveränderung bei Pankreasfibrose. Zentralbl Allg Pathol 103: 405–413
MacLusky I, Levison H (1990) Cystic fibrosis. In: Chernick V (ed) Kendig's disorders of the respiratory tract in children. WB Saunders Company, Philadelphia, pp 692–730
McGiven AR (1962) Myocardial fibrosis in fibrocystic disease of the pancreas. Arch Dis Child 37: 656–660
Meschan I (1981) Röntgenologie des Herzens. In: Meschan I (ed) Analyse der Röntgenbilder: klin. Radiologie in 3 Bd. Enke, Stuttgart, p 390
Moss AJ (1982) The cardiovascular system in cystic fibrosis. Pediatrics 70: 728–741
Mouy A, Tordjmann M (1968) Fibrose myocardique au cours de la mucoviscidose. Arch Fr Pediatr 25: 687–701
Nezelof C, Lesec G (1979) Multifocal myocardial necrosis and fibrosis in pancreatic diseases of children. Pediatrics 63: 361–368
Nivelon JL, Michiels R, Martres-Lassaunière MN, Passavy-Trouche AM, Petit A, Lorenzini JL (1978) Fibrose myocardique compliquant un syndrome de Shwachman. Pédiatrie 33: 461–469
Niznikowska-Marks J, Okninska A, Kaminski Z (1971) Fibrose myocardique au cours de la mucoviscidose. Helv Paediatr Acta 26: 56–62
Oppenheimer EH, Esterly JR (1973) Myocardial lesions in patients with cystic fibrosis of the pancreas. Hopkins Med J 133: 252–261
Reis LD (1963) Visceral lesions in acute pancreatitis. An experimental study. Arch Surg 87: 604–608
Rinehart JF, Greenberg LD (1949) Effect of experimental thiamine deficiency on the heart of the rhesus monkey. Arch Pathol 48: 89–95
Riordan JR, Rommens JM, Kerem B-S, Alon N, Rozmahel R, Grzelczak Z, Zielinski J, Lok S, Plavsic N, Chou J-L, Drumm ML, Ianuzzi MC, Collins FS, Tsui L-C (1989) Identification of the cystic fibrosis gene: cloning and characterization of complementary DNA. Science 245: 1066–1073
Sullivan MM, Moss RB, Hindi RD, Lewiston NJ (1986) Supraventricular tachycardia in patients with cystic fibrosis. Chest 90: 239–242
Wissler H, Zollinger H (1945) Die familiäre kongenitale zystische Pankreasfibrose mit Bronchiektasen. Helv Paediatr Acta 1 [Suppl 1]: 3–88
Zimmermann A, Stocker F, Torriani R, Chassot J, Weber JW (1982) Cardiomyopathy in cystic fibrosis: lymphoedema of the heart with focal myocardial fibrosis. Helv Paediatr Acta 37: 183–192
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Wiebicke, W., Artlich, A. & Gerling, I. Myocardial fibrosis — a rare complication in patients with cystic fibrosis. Eur J Pediatr 152, 694–696 (1993). https://doi.org/10.1007/BF01955251
Received:
Accepted:
Issue Date:
DOI: https://doi.org/10.1007/BF01955251