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Correspondence

Clinical Problem-Solving: Getting the Story Right

N Engl J Med 1993; 329:1128-1129October 7, 1993

Article

To the Editor:

We enjoyed reading Dr. Duffy's comments (May 6 issue),1 particularly with regard to the mechanisms of chest pain in a patient with systemic mastocytosis. We disagree with the statement that the exacerbation of chest pain after the administration of drugs that typically ameliorate angina argues against coronary disease. Variant angina can be aggravated by beta-blocker therapy2. This deleterious effect of beta-blockers could be ascribed to the fact that in human coronary arteries, beta-adrenergic blockade potentiates the vasoconstrictor effect of catecholamines3. Moreover, positive exercise tests have been reported in patients with variant angina4. Since there is general agreement that coronary angiography is justified when variant angina is suspected,5 we think that angiography should have been performed, contrary to what the commentator suggested.

Philippe Unger, M.D.
Guy Berkenboom, M.D., Ph.D.
Universite Libre de Bruxelles, B-1070 Brussels, Belgium

5 References
  1. 1

    Duffy TP. Getting the story right. N Engl J Med 1993;328:1333-1336
    Full Text | Web of Science | Medline

  2. 2

    Robertson RM, Wood AJ, Vaughn WK, Robertson D. Exacerbation of vasotonic angina pectoris by propranolol. Circulation 1982;65:281-285
    CrossRef | Web of Science | Medline

  3. 3

    Berkenboom G, Fontaine J, Desmet J-M, Degre S. Comparison of the effect of beta adrenergic antagonists with different ancillary properties on isolated canine and human coronary arteries. Cardiovasc Res 1987;21:299-304
    CrossRef | Web of Science | Medline

  4. 4

    Bashour TT. Vasotonic myocardial ischemia. Am Heart J 1991;122:1701-1722
    CrossRef | Web of Science | Medline

  5. 5

    Guidelines for coronary angiography: a report of the American College of Cardiology/American Heart Association Task Force on Assessment of Diagnostic and Therapeutic Cardiovascular Procedures (Subcommittee on Coronary Angiography). J Am Coll Cardiol 1987;10:935-950
    CrossRef | Web of Science | Medline

To the Editor:

In his commentary Duffy suggested that the patient's chest pain might be caused by coronary-artery spasm due to mediators secreted by mast cells. Recently, we evaluated a 57-year-old man in whom an exercise test was immediately followed by elevation of the ST segment in the inferior leads and ventricular fibrillation1. A coronary angiogram showed normal coronary arteries. However, spasms of the proximal right and left anterior descending coronary arteries could be provoked by intravenous ergonovine. After the arteriography, a capillary-leak syndrome developed. Recovery was uneventful. After a subsequent exercise test, severe hypotension (50/30 mm Hg) and a generalized skin rash developed. Exercise-induced anaphylaxis was diagnosed when plasma histamine levels were found to be elevated after exercise (>150 nmol per liter; normal, <10) and the results of biopsies of bone marrow, stomach, and skin were normal. The increase in plasma histamine levels (from 1.7 to 93.5 nmol per liter) as well as serum tryptase levels (from 1.2 to 9.1 U per liter; normal, <2) after exercise was again documented under a therapeutic regimen with H1 and H2 blockers and aspirin (3 g daily), but the patient was only mildly symptomatic. Our observations can confirm the laboratory data of Ginsburg et al.2 with regard to the pathogenesis of chest pain in mast-cell disorders.

Rudolf Speich, M.D.
Christine Attenhofer, M.D.
Franz Wolfgang Amann, M.D.
University Hospital, 8091 Zurich, Switzerland

2 References
  1. 1

    Attenhofer C, Speich R, Salomon F, Burkhard R, Amann FW. Ventricular fibrillation in a patient with exercise-induced anaphylaxis, normal coronary arteries, and a positive ergonovine test. Chest (in press).

  2. 2

    Ginsburg R, Bristow MR, Kantrowitz N, Baim DS, Harrison DC. Histamine provocation of clinical coronary artery spasm: implications concerning pathogenesis of variant angina pectoris. Am Heart J 1981;102:819-822
    CrossRef | Web of Science | Medline

Author/Editor Response

Dr. Duffy replies:

To the Editor: It seems likely that the variant angina in the patient described by Speich et al. was caused by mast-cell release. The inability to identify mast-cell infiltration on skin or bone marrow biopsy suggests that increased numbers of cardiac mast cells may be present, the finding in the patient with coronary spasm described by Forman et al.1.

Unger and Berkenboom have correctly pointed out that variant angina may worsen with beta-adrenergic blockade, and they use the guidelines of the American College of Cardiology2 to support the use of angiography in patients with this condition. The introduction to these guidelines specifically states that the primary purpose of coronary angiography is to define the anatomy of the coronary arteries when such information is needed for management. With the knowledge now available about mast cells and coronary spasm, it might be appropriate for the physician to consider the diagnosis of mastocytosis before proceeding to angiography. For variant angina, management of chest pain would be subsumed under treatment of the systemic condition if the cause was mastocytosis. Just as patterns in clinical reasoning need adjustment for the individual patient, set patterns in the use of technology need the same critical circumspection.

Thomas P. Duffy, M.D.
Yale University School of Medicine, New Haven, CT 06510

2 References
  1. 1

    Forman MB, Oates JA, Robertson D, Robertson RM, Roberts LJ II, Virmani R. Increased adventitial mast cells in a patient with coronary spasm. N Engl J Med 1985;313:1138-1141
    Full Text | Web of Science | Medline

  2. 2

    Guidelines for coronary angiography: a report of the American College of Cardiology/American Heart Association Task Force on Assessment of Diagnostic and Therapeutic Cardiovascular Procedures (Subcommittee on Coronary Angiography). J Am Coll Cardiol 1987;10:935-950
    CrossRef | Web of Science | Medline