Volume 105

Number 1 Brief Communications 161

defined by debrisoquinet and the high metabolic ratio in the patient described suggests that impaired drug oxida- tion may be the inborn metabolic disorder predisposing an individual to perhexiline accumulation and the associated neurotoxic sequelae.

REFERENCES

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Pilcher J, Chandrasekhar KP, Russell Rees J, Boyce MJ, Pence TH, Ikram H: Long-term assessment of perhexiline maleate in angina pectoris. Postgrad Med J 49(suppl 31:115, 1973. L’Hermitte F, Fardeau M, Chedru F, Mallecourt J: Polyneu- ropathy after perhexiline maleate therapy. Br Med J 1:1256, 1976. Pessayre D, Bichara M, Feldman G, Degott C, Potet F, Benhamou J: Perhexiline maleate-induced cirrhosis. Gastro- enterology 76:170, 1979. Wright GJ, Leeson GA, Zeiger AV, Lang JF: The absorption, excretion and metabolism of perhexiline maleate by the human. Postgrad Med J 49(suppl 3):8, 1973. Idle JR, Oates NS, Ritchie JC, Shah RR, Sloan TP, Smith RL: New perspectives of genetic polymorphism in drug metabolism. In Bellingham AJ, editor: Advanced Medicine. Vol. 16. Bath, 1980, Pitman Medical, p 227. Bertilsson L, Mellstriim B, Sjijqvist F, Martensson B, Asberg M: Slow hydroxylation of nortriptyline and concomitant poor debrisoquine hydroxylation: Clinical implications. Lancet 1:561, 1981. Singlas E, Goujet MA, Simon P: Pharmacokinetics of perhex- iline maleate in angina1 patients with and without peripheral neuropathy. Eur J Clin Pharmacol 14:195, 1978.

Fulminant acute rheumatic fever with multisystem involvement

Jorge Escudero, M.D., Estanislao Stanislawsky, M.D., and Xavier Escudero, M.D. Mexico City, Mexico

A 14-year-old boy admitted to our institution had no history of pharyngitis or rheumatic fever. Four months prior to admission he developed malaise, arthritis, and weight loss without fever. Shortly thereafter, exercise- related dyspnea occurred, progressing to orthopnea, noc- turnal paroxysmal episodes, cough with blood-streaked sputum, pain in the right hypochondrium, and malleolar edema. At physical examination he was afebrile with a heart rate of lOO/min and blood pressure of 140/O mm Hg, cachectic, dehydrated, and jaundiced. The jugular veins were distended with hepatojugular reflux. Cardiac exami- nation revealed a hyperdynamic apex, gallop rhythm, pericardial rub, systolic and diastolic apical murmurs, systolic murmur at the tricuspid area, ejection systolic

From the Hospital de Cardiologia y Neumologia, National Medical Center, Mexican Institute of Social Security.

Received for publication April 6, 1981; revision received Nov. 1, 1981; accepted Dec. 3, 1981.

Reprint requests: Jorge Escudero, M.D., Hospital de Cardiologia y Neumo- logia, National Medical Center, Mexican Institute of Social Security, Avenida Cuauhtemoc 330, Mexico 7. D.F., Mexico.

Fig. 1. Chest x-ray film shows cardiomegaly with pre- dominance of left-heart chambers, signs of pulmonary and venocapillary hypertension, and an area of consolidation at the right base with pleural effusion.

Fig. 2. Trivalvular rheumatic valvulitis as viewed from above with the atria removed. The aortic valve is in the midcenter field, the mitral valve in the upper right, and the tricuspid valve in the upper left. The mitral and aortic valves are thickened with commissural fusion and small vegetations. The tricuspid valve displays similar, although milder, changes.

murmur with soft diastolic murmur at the base, and a reinforced or loud pulmonary second sound. There were pulmonary rales, painful moderate hepatomegaly, periph- eral pulses of the Corrigan type, and marked malleoiar edema. An ECG showed four-chamber enlargement. Chest x-ray films revealed marked cardiomegaly, pulmonary and venocapillary hypertension, and right pleural effusion (Fig. 1). Laboratory findings were as follows: hemoglobin,

0002-8703/83/010161 + 02$00.20/O L, 1983 The C.V. Mosby Co.

162 brief Communica1ion.s January 1983

American Heart Journal

Fig. 3. AV node with replacement of nodal fibers by nonspecific inflammatory cells. The nodal artery (left upper field) shows no changes. (Hematoxylin-eosin stain; magnification x250.1

10.2 g/dl; hematocrit, 34 mm/dl; leukocytes, 9800/mm,‘; sedimentation rate, 20 mm/hr; C-reactive protein 4+, antistreptolysin 0 titer, 1250 units; direct and indirect bilirubin, 3.2/2 mg/dl; albumin/globulin ratio, 2.813.5 pm/ dl; prothrombin time, 28.5 seconds; serum glutamic pyru- vie transaminase, 100 units, serum glutamic oxaloacetic transaminase, 252 units; serum cholesterol, 162 mg/dl; blood glucose, 80 mg/dl; blood urea, 100 mg/dl; serum creatinine, 2 mg/dl; culture of pharynx positive for Strep- tococcus viridans; and blood cultures negative.

Two weeks after admission the patient developed a Stokes-Adams episode with the ECG showing intermit- tent complete AV block, for which an endocardial pace- maker was inserted. However, the clinical course worsened and the patient died. At necropsy, the heart was enlarged and weighed 590 gm. The mitral, tricuspid, and aortic valves were damaged (Fig. 2). The mitral cusps were grossly thickened with commissural fusion and chordal thickening; there were rows of small vegetations along the edge of the cusps. The tricuspid valve displayed similar, although milder, changes. The aortic valve showed thick- ening, shortening, rolled edges, inversion of the free margins, and vegetations. Histologically, the normal struc- ture of the three damaged valves was destroyed with increased vascularity. Aschoff bodies’ were present and the vegetations consisted of platelet and fibrin thrombi with some fibroblasts. Numerous Aschoff bodies were also present in the ventricular and atria1 myocardium.” The conduction system, mainly the AV node and the bundle of His, showed chronic nonspecific inflammatory cell infi-

rration with occasional Aschoff bodies (Fig. 3). The aorta, coronary and systemic arteries, as well as the pulmonary and venous vessels revealed vasculitis manifested by lesions similar 1.0 those found in the heart. The lungs exhibited rheumatic pneumonitis, bronchopneumonia, and infarction. The kidneys showed acute postinfectious glomerulonephritis with endocapillary proliferation and exudation. The systemic organs were severely congested.

Rheumatic fever has been more severe in Mexico than in other countries, causing active pancarditis and refracto- ry heart failure. Rheumatic encephalopathy was described hy Costero et al.,’ and rheumatic pneumonitis was also reported in Mexico by Cuellar et al.” Infarct of the lung was considered to be rare in children with active rheumat- ic cardiopathy as well as involvement of blood vessels. The association of active rheumatic heart disease with acute glomerulonephritis is an exceptional occurrence. The presence of all these conditions makes the case described here extremely rare.

REFERENCES

1. Gross L, Ehrlich JC: Studies on the myocardial Aschoff body. 1. Descriptive classification of lesions. Am J Path01 10:467, 1934.

2. Gross L, Ehrlich .JC: Studies on the myocardial Aschoff body. II. Life cycle, sites of predilection and relation to clinical course of rheumatic fever. Am J Path01 l&489, 1934.

:1. Costero I, Rarroso R, De Gortari A, Pellbn R: Encefalopatia de1 reumitico. II. Cuadro histopatol6gico de1 enkfalo jugoso. Arch Inst Cardiol Mex 17:488, 1947.

4. Cuellar A, Pkrez-Tamayo R: Estudio anatomoclinico de la neumonitis reumbtica. Arch Inst Cardiol Mex 21:594, 1951.