LETTERS

COMMENT: I

We have a great deal to learn about coronary spasm. At this time, the prevalence of spasm in patients with chest pain with or without anatomic coronary lesions is unknown. Aside from scattered case reports, the lethality of spasm as well as its role in converting coronary disease with stable angina pectoris into acute myocardial infarction is also unknown. That provocative testing for spasm carries risk is certain. The question of whether the risk is worthwhile will ultimately be answered in a general way by the collection of more information about the natural history of coronary spasm. In an individual case, a decision to attempt to provoke a potentially life-threatening entity will (and should) always be a difficult one, made with the patient’s full understanding and agreement and carried out with appropriate caution.

Catherine Nelson, MD Stanley A. Forwand, MD, FACC Harvard Medical School Mount Auburn Hospital Cambridge, Massachusetts

COMMENT: II

Dr. Bauman reports on a man with probable Prinzmetal’s angina and relatively normal coronary arteries who experi- enced severe unrelenting chest pain and arrhythmias due to near-occlusive right coronary arterial spasm in response to provocative administration of ergonovine maleate. This case emphasizes the need for a careful and systematic approach to such induction of coronary spasm in patients with suspected Prinzmetal’s angina. The points to be learned from this case are as follows:

1. Reproduction of the coronary spasm and clinical syn- drome of Prinzmetal’s angina by ergonovine maleate is certain to be accompanied occasionally by arrhythmias because of their large incidence during spontaneous episodes.‘J

2. The route of administration of ergonovine maleate may be important. Reported series have utilized only intravenous administration, allowing substantial dilution before coronary arterial perfusion. 3-5 The intraaortic or intracoronary route should be reserved for investigational purposes.

3. Methodic sequential administration of increasing doses of ergonovine maleate may be important. Reports of series have generally utilized doses of 0.1 to 0.4 mg. Because patients with Prinzmetal’s angina appear to be sensitive to a certain dose of ergonovine maleate, we urge a sequential increase in dosage based on reported experience, that is, 0.1 mg initially, 0.15 mg at 3 and 0.2 mg at 3 to 5 minute intervals with serial angiograms to document effect. If the patient has had docu- mented spontaneous episodes of S-T elevation 0.05 mg should be given as the initial dose.

4. Repeat coronary arteriography, at least of the suspect coronary artery, should be performed after each injection because coronary spasm may well occur without complete occlusion or reproduction of symptoms. Nonocclusive spasm of the right coronary artery may well have occurred after the second or third dose of 0.05 mg of ergonovine maleate without reproduction of symptoms in the case report by Bauman. Repeat coronary angiography at that time might have averted the final larger dose of 0.2 mg that precipitated the unrelenting right coronary spasm.

5. The primary goal of therapy, after reproduction of Prinzmetal’s angina and coronary spasm and its documen- tation, should be reversal of the coronary spasm because it is the presumed cause of the arrhythmias. We suggest admin-

istration of l/100 grain of nitroglycerin sublingually, repeated once or twice over 5 minutes for unrelenting spasm. Intrave- nous nitroglycerin or nitroprusside can also be given if the spasm fails to resolve after 5 to 10 minutes.

6. Finally, we believe that reproduction of the clinical syndrome of Prinzmetal’s angina during coronary angiography is worth the small risk because of the implications for long- term therapy with vasodilators. This is particularly true in view of the reported marked efficacy of calcium-antagonist drugs for this syndrome.6-*

John Speer Schroeder, MD, FACC Coronary Care Unit Cardiology Division Stanford University School of Medicine Stanford, California

References

1. Prlnzmetal M, Kennamer R, K&as R, et al: Angina pectoris. I. A variant form of angina pectoris. Preliminary report. Am J Med 27:375-37,8, 1959

2. Hiaalnr C. Wexler L. Silverman J. et al: Clinical and arterioaraphic features of P&teta~s variant angina: docu&ntation of etiolcgic factors. &n J Cardiol 37: 831-839. 1976

3. Nelson C, Nowak B, Chllds H, et al: Provocative testing for coronary arterial spasm: rationale, risk and clinical illustrations. Am J Cardiol 40:624-629, 1977

4. Curry RC Jr. Peplne CJ, Sabom MB, et al: Effects of ergonovine an patients with and without coronary artery disease. Circulation 56:803-809. 1977

5. Heuplsr F. Proudflt W, Siegel W, et al: The ergonovine maleate test for the diagnosis of coronary artery spasm. Circulation 51, 52:Suppl ll:ll-11. 1975

6. Bosserf F, Valor W: Dihydropyridine. eine neue Gruppe start wirksamer Coronarth- eraoeutika. Naturwissen 58:578. 1971

7. H&da S, Klmura E: In. proceedings, First International Nifedipine (Ad&t) Symposium, Tokyo 1973 (Hashimato K, Kimura E. Kobayashi T. ed). Tokyo, University of Tokyo Press, 1974, p 175

8. Muller JE, Gunther BJ: Nifedipine therapy for Prinzmetal’s angina. Circulation 57: 137-139. 1978

“NORMAL” CHOLESTEROL AND ATHEROSCLEROSIS

There is reason to question the basis of the definition of nor- mal lipids used by Lie et al.’ The issue is more than one of semantics, and potentially leads to an underestimate of the role of nutrition in the prevention of atherosclerosis. Choles- terol values for the group designated as “normolipemic” ranged from 214 to 291 mg/dl, whereas “hyperlipemic” pat- ients had cholesterol values greater than 308 mg/dl. Using “normal” in a biologic sense,2 not as an average or a statistical term, an international perspective would suggest that a cho- lesterol level much above 180 mg/dl is “abnormal” and leads to atherosclerosis on a wide scale.3$4 It is worth noting that the lowest cholesterol value in a patient who came to surgery in this series was 214 mg/dl. Obviously the risk increases as cholesterol increases, but it would appear that a threshold can be defined. Lie et al. conclude that “the observation that mi- croscopic changes of atherosclerosis may occur in saphenous vein grafts in some patients without hyperlipemia also em- phasizes the importance of effective control not only of blood lipids but also of the other recognized risk factors for athero- sclerosis.” Although control of other risk factors is crucial in the hyperlipemic population in the United States, perhaps 214 to 291 mg/dl is not yet an optimal level of serum cholesterol, and should not be accepted as normal.

Richard Cooper, MD Department of Community Health ‘Northwestern University Chicago, Illinois

References

1. Lie JT. Lawrle GM, Morris GC: Aortocoronary bypass saphenous vein graft athero- sclerosis: anatomic study of 99 vein grafts from ncfmal and hyperlipoproteinemic patients up to 75 months postoperatively. Am J Cardiol 40: 906-914, 1977

2. Wynder EL, Hill P: Blood lipids: how normal is normal? Prev Med 1:161-166. 1972

October 1978 The American Journal of CARDIOLOGY Volume 42 895

LETTERS

3. Connor WE, Connor SL: The key role of nutritional factors in the prevention of coronary heart disease. Prev Med 1:49-83. 1972

4. Coronary Heart Disease in Seven Countries (Keys A, ed). Circulation 41:Suppl I, 1970

REPLY

The point is well taken. Blood cholesterol and triglyceride concentrations of patients in our study were determined ac- cording to the procedures of the Lipid Research Clinics Pro- gram.’ The “cut-off” values of cholesterol for patients in the 3rd, 4th, 5th and 6th decades were 210, 240, 260, and 280 mg/dl, respectively, as reported in a recent article by Gotto et al2 Cooper may be right in suggesting that perhaps 214 to 291 mg/dl is not yet an optimal level of cholesterol and should not be accepted as “normal” when regarded as a risk factor for atherosclerosis.

JT Lie, MD, FACC Mayo Clinic Rochester, Minnesota

Gerald M. Lawrie, MD, FACC George C. Morris, MD, FACC Baylor College of Medicine Houston, Texas

References

1. Manual of Laboratory Operations. Lipld Research Clinic Program, vol I. Washington DC, Department of Health, Education, and Welfare, Publication no NIH-75-628. 1974

2. Gotto AM, Gerry GA, Thompson JR, et al: Relationship between plasma lipid concen- trations and coronary artery disease in 496 patients. Circulation 56:875-883, 1977

VENTRICULAR INVERSION WITHOUT TRANSPOSITION

OF GREAT ARTERIES

Dunkman et a1.l refer to “d-(‘complete’) and l-(‘corrected’)” transposition. No doubt, they are employing the widely used terminology coined by Van Praagh et a1.*p3 However, the ac- cepted definition of “transposition” by Van Praagh et a1.2 is now “the anatomic condition in which the aorta arises from the morphologic right ventricle and pulmonary trunk from the morphologic left ventricle.” This is not only the literal meaning of the term, but also the original meaning, as used by John Farre4 of London in 1814. This definition is also ac- cepted by the authors. While discussing the “loop rule,” Dunkman et al. rightly point out that exceptions to it are not uncommon. Van Praagh5 himself recognized its fallibility. Complete transposition with d- and l-transposition is well documented.6T7 Conversely, corrected transposition with d- and l-transposition has been described.sTsTo add to the con- fusion, situs inversus with D-ventricular loop and l-transpo- sition,ic and many such complex anomalies have been de- scribed. Therefore, the terms d- (‘complete’) and I-(‘correct- ed’) transposition appear illogical to me.

A well known textbook of cardiology11 defines l- and d- transposition thus: “d-transposition signifies that the aorta lies to the right of the pulmonary artery; I-transposition places the aorta to the left of the pulmonary artery.” This does not take into account the connections of the great vessels to their respective chambers. In contrast, the term transposition in- dicates precisely that. It does not take into account the in- terrelation of the great vessels themselves. Use of the same term to indicate such vastly different meanings creates un- necessary confusion.

I believe this confusion can be avoided if the term trans- position is strictly reserved to describe the ventriculo-arterial connection (discordance). To describe the relation between

the pulmonary artery and aorta, d- and l-positions (not transposition) or d- and l-malpositions might be used (as D- and L-loop are used to describe the ventricular position). The term “anatomically corrected malposition” becomes more meaningful in this context. Similarly, the terms o-position, when the aortic valve is anterior12 and p-position, when the aortic valve is posterior”xls may also be used.

In an otherwise excellent paper by Shinebourne et a1.14 and in the editorial by the same group15 arguing forcefully in favor of a nomenclature based on connections rather than relations, the terms I- and d-transpositions are used. It is important to realize that connections and relations are different and to describe them separately. It was shown that an aorta normally related to the pulmonary artery can have an abnormal con- nection to the right ventricle. 2,13 A differentiation between transposition and position will increase understanding of the connections and relations.

Finally, I point out that a case of “anatomically corrected malposition” with an entirely subpulmonary conus has been reported.‘6

K. A. Chacko, MD Department of Cardiology Sree Chitra Tirunal Medical Centre Trivandrum, India

References

1. Dunkman WB, Perloff JK, Roberts WC: Ventricular inversion without transposition of the great arteries. A rarity found in association with atresia of the left-sided (tricuspid) atrioventricular valve. Am J Cardiol 39:226-231. 1977

2. Van Praagh R, Perez-Trevlno G, Lopez-Cuellar M, et 81: Transposition of the great arteries with posterior aorta. anterior pulmonary artery. subpulmonary conus and fibrous continuity between aortic and atrioventricular valves. Am J Cardiol 28~621-631. 1971

3. Van Praagh R, Van Praagh S, Wad P, et aI: Anatomical types of congenital dextrocardia: diagnostic and embryologic implications. Am J Cardiol 13:510, 1964

4. Van Praagh R: The story of anatomically corrected m&position of great arteries. Chest 69:2-4. 1976

5. Van Praagh R: The segmental approach to diagnosis in congenital heart disease. Birth Defects: Oriainal Article Series 8:4-23. 1972

6. Bracia A, K&aid OW, Davis GD, et al: Transposition of great arteries: an angiocar- diooraohic studv. Am J Rcentoenol Radium Ther Nucl t&d 100:249-261. 1967

7. C& I: Tynan i, Aberdeen 6 et al: Predictive accuracy of the “loop &lea’ in 109 children with classic complete transposition of the great arteries (TGA). Circulation 37. 38; Suppl VI:VI-52. 1968

8. Esplno-Vela J: On a variety of the “corrected” type of transposition of the great vessels associated with dextrocardia: a study of two cases with autopsy report. Am Heart J 58:250-261. 1959

9. Anderson RC, Llllahei CW, Lester RG: Corrected transposition of great ve?.sels of the heart A review of 17 cases. Pediatrics 20:626-635. 1957

10. Anderson RH, Arnold R, James RS: D-bulboventricular loop with L transposition in situs inversus. Circulation 46:173-185. 1972

11. binsfield DE, Edwards FK: Clinical recognition and medical management of congenital heart disease, chap 40. In. The Heart (Hurst JW, ed), third edition. Tokyo, MC Graw Hill Kogakusho. 1974, p 733

12. Squarcla U, RHler DG, Klncald 0: Dextrocardia: angicgraphic study and classification. Am J Cardiol 32:965-977. 1973

13. Wilkinson JL. Arnold R. Anderson RH. et al: ‘Posterior’ transposition reconsidered. Br Heart J 37:757-766:1975

14. Shinebourne EA. Macatlney FJ, Anderson RH: Sequential chamber localisation. Br Heart J 38:327-340, 1976

15. Macartney FJ. Shinebourne EA, Anderson RH: Connections, relations, discordance and distortions. Br Heart J 38:323-326. 1976

16. Zakhelm R, Maiiloli L, Vasseenon T, et al: Anatomically correct&i malposition of the great arteries (S.L.D). Chest 69:101-104. 1976

REPLY

If we interpret Chacko’s letter correctly, he seems to take issue with our use of the term transposition and may suggest that the case we reported should be considered an example of anatomically corrected malposition of the great arteries.

We believe that our use of terms is in accord with the principles he endorses. Perhaps part of his concern arises from the fact that in both the original first page of his letter and its references [now corrected], he referred to the title of our article as “Ventricular inversion r&h (rather than c&ho&) trans- position. .”

696 October 1976 The American Journal of CARDIOLOGY Volume 42