appear. This is the most acceptable theory in the pathogenesis of EMF. Death can occur at any stage, and a long survival is followed by fibrous scarring and thickening. Even when it reaches this stage, the disease has a poor prognosis, often in patients in functional class III or IV.4J So, the usual clinical picture of the disease is characterized by fibrosis as the end stage of a probable eosinophilic heart disease, leading to deformation of the ventricles. Balakrishnan et a1,7in 1986, suggested the possibility of progression or new appearance of angiographic evidence of fibrosis in the unaffected ventricle of 2 patients. Thii raised the possibility of a slowly progressive disease in a few cases. Lepley et al8 thought that new formation of fibrotic tissue would not occur because fibrosis is a scarring process. Gupta et a1,9in 1989, showed angiographic progression of the disease in 8 patients, with repeated cardiac catheterization over a period of 2 to 3 years. They showed progression of the disease in the same ventricle and new involvement of the previously uninvolved ventricle. If the patients are still subjected to the environmental factors responsible for the disease, the recurrence could theoretically occur. Metras et allo and Davies et al” disagree with this point. They noticed no recurrence of the fibrosis despite the persistence of the predisposing factors in their series. Dubost et al,‘* in 1990, did not observe recurrence of the fibrosis in 6 patients who died in the late postoperative period and in 12 patients who underwent repeat operation because of bioprosthetic dysfunction. In the last 12 years we examined 142 patients with EMF in our institution. From this group, we selected 22

Incidence of Renal Heart Disease

Dysfunction

Michael F. Flanagan, MD, Maribeth

Hourihan,

patients that, in the follow-up, had undergone to L2 cineventriculographies. In these series, no patient had worsening of the disease. These results suggest that this stage of EMF is not evolutionary in character, confirming the hypothesis that ventricular fibrosis is only the consequence of a previously healed disease, and not a changing process.

1. Roberts WC. Liegler DG, Carbone PP. Endomyocardial disease and eosinophilia. A clinical and pathologic spectrum. Am J bled 196%46:28-4X 2. Oakley CM. Olsen EGJ. Eosinophilia and heart disease. Br Heart J 1977;39:233-237. 3. Davies JNP. Pathology and pathogen&s of endocardial disease. Cardiologia 1963;42:161-175. 4. Mady C, Pereira Barrett0 AC, Oliveira SA, Stolf NAG, Bellotti G, Jatene AD, Pileggi F. Effectiveness of operative and nonoperative therapy in endomyccardial fibrosis. Am J Cardiol 1989;63:1281~1282. 5. Pereira Barrett0 AC, Luz PL, Oliveira SA, Stolf NAG, Mady C, Bellotti G, Jatene AD, Pileggi F. Determinants of survival in endomyocardial fibrosis. Circulation 1989;90:1-77--I-82. 6. Oliveira SA, Pereira Barrett0 AC, Mady C, Dallan LAO, Luz PL, Jatene AD, Pileggi F. Surgical treatment of endomyocardial fibrosis: a new approach. J Am Co11 Cardiol 1990;16:1246-1251. 7. Balakrishnan KG. Venkitachalam CG, Pillai VRK, Subramanian R, Valiathan MS. Postoperative evaluation of endomyocardial fibrosis. Cardiobgy 1986:73:73-84. 8. Lepley D Jr, Aria A, Kerns ME, Walker 5.4, O’Cunha RM. Endomyccardial fibrosis. A surgical approach. Ann Tborac Surg 1974;18:626-633. 9. Gupta PN, Valiathan M, Balakrishnan KG. Kartha CC, Ghosh MK. Clinical course of endomyocardial fibrosis. Br Heart J 1989;61:450-~454. 10. Metras D, Coulibaly AO, Ouattara K, Chauvet J. Traitement chirurgical de la fibrose endomyocardique. A propos de 45 cas. Chirurgie 1983;109:598-607. 11. Davies J, Sapsford R, Brooksby I, Olsen EGJ. Spry CJF, Oakley CM, Goodwin JF. Successful surgical treatment of two patients with eosinophilic endomyocardial disease. Br Heart J 1981;46:438-445. 12. Dubost C. Chapelon 0. De&he A, Piette JC, Chauvaud S, Fabiani JN, Carpentier A, Chirurgie des fibroses endomyocardiques. Apropos de 32 cas. Arch Mal Coeur 1990;83:481-486.

in Adults

with Cyanotic

Congenital

MD, and John F. Keane, MD

ephropathy has long been recognized as a potential complication of cyanotic congenital heart disease (CHD).’ Previous studies have demonstrated that glomerular enlargement, mesangial hypercellularity, glomerular capillary congestion and segmental sclerosis occur in some patients with cyanotic CHD. l Functional abnormalities including decreased re-

N

From the Department of Cardiology, Children’s Hospital, and the Department of Pediatrics, Harvard Medical School, 25 Shattuck Street, Boston, Massachusetts 02115, and the Department of Maternal and Child Health, Dartmouth-Hitchcock Medical Center, Hanover, New Hampshire 03756. This study was supported in part by Clinician Scientist Award 89-0411 from the American Heart Association, Dallas, Texas.

nal plasma flow and glomerular filtration rate, azotemia, abnormal uric acid secretion, proteinuria and nephrotic syndrome also occur in some patients with cyanotic CHD.2-5 The incidence of renal abnormalities increases with the degree of cyanosis and accompanying erythrocytosis.’ Previous studies2,3 and our clinical experience suggest that the incidence of renal abnormalities may also increase with increasing duration of cyanosis. Owing to advances in cardiovascular surgery, growing numbers of patients with cyanotic CHD are now surviving longer into adulthood and may be at risk for developing renal abnormalities that may significantly influence their clinical course.” To our knowledge, the incidence of renal

BRIEF

REPORTS

403

TABLE

I Diagnoses

Cyanotic Cyanotic Repaired

with abnormal UA with normal UA CHD

Tetralogy of Fallot (56)

Transposition of Great Arteries (%)

Complex Cyanotic (%)

(26)* 12/49 (25)* 38/42 (90)

ii/34 (18) 7/49 (14) 4/42 (10)

16/34 (47)* 23149 (47F O/42 (0)

9/34

*p 10.05 wrsus repaired congenital heart disease. CHO = congenital heartdisease; CV = cardiovascular;

TABLE II Abnormalities and Unrepaired Cyanotic

Patients

Proteinuria (z 100 mg/dl) Erythrocytes r3/HPF Leukocytes >YHPF Casts Any of above HPF = high-powerfield;

25/83

(%) (30)

9/83 (11) 14/83

(17)

14/83 (17) 34/83 (41)

Acyanotic

O/42 (0) O/42 (0) 4/42

(10)

O/42 (0) 4/42 (10)

(%)

p Value p