ABSTRACTS

THE SPECTRUM OF CARDIAC SARCOIDOSIS:A CLINICOPATHO-

LOGIC STUDY OF 83 PATIENTS. Kenneth J. Silverman, M.D.,

Grover M. Hutchins, M.D., Bernadine H. Bulkley, M.D.,

F.A.C.C. The Johns Hopkins Medical Institutions, Baltimore,Md.

Although sarcoid may involve the myocardium, there is little in-

formation on its true incidence or significance. To examine this we

studied 83 consecutive autopsied patients with sarcoidosis. The

patients ranged in age from I8 to 80 years (average 46) and 60%

were women; 22 (27%) of them had myocardial granulomas: in F/

(41%) these were clinically silent, and in I3 (59%) there was a

history of heart failure and/or arrhythmias and conduction defects.

Of the 22 patients only 4 (18%) had grossly evident, widespread

myocardial lesions: 3 of these 4 (75%) had documented ventricular

arrhythmias. All 4 had sudden unexpected death at an average age

of 36 and in only 2 had sarcoid been suspected during life. The

other I8 patients (82%) had trivial myocardial involvement with

lesions evident only microscopically. Of these, only 2 (II%) hod

ventricular arrhythmias, 7 (39%) conduction block and 4 (22%)

sudden death.

Comparing the 22 patients with and the 61 without cardiac sar-

coid, those with cardiac lesions had 4 times (x) more ventricular

arrhythmias, 3 x more heart block, and 2 x more sudden death.

Sudden death was as common, however, in patients without cardiac

sarcoid as in those with only histologically evident lesions. Congs-

tive heart failure was as frequent in patients with and without car-

diac sarcoid, and correlated more with systemic hypertension.

The results show that although myocardial involvement occurs in

over 25% of patients with sarcoid, it is most often morphologically

trivial and clinically silent. Arrhythmias and heart block are more

common inthe cardiac sarcaid group, but the findings suggest that

only that small subset of patients with severe grossly evident myo-

cardial sarcoid are at increased risk for sudden death.

ANGIOGRAPHIC APPEARANCE OF CORONARY ARTERIES IN CONSTRICTIVE PERICARDITIS Jonathan Alexander, MD; Steven Wolfson, MD, FACC; Michael J. Kelley, MD, FACC; Lawrence S. Cohen, MD, FACC; Rene A. Langou, MD, Yale University School of Medicine, New Haven, Connecticut

The motion of the coronary arteries (CA) during angio- graphy was assessed in patients (pts) with 1) constric- tive pericarditis (CP), 2) cardiomyopathy (CM), and 3) normals (NM). CP was established in 7 pts by character- istic right and left heart hemodynamics and surgical pathology. Four had calcific CP and 3 had non-calcific CP. All underwent left ventriculography (LVgram) and coronary arteriography. All had normal ejection frac- tions (EF), >55%. The 10 CM pts had abnormal EF (mean 25 +3 SEM) and normal CA. The 10 NM pts had normal hemo- dynamics, LVgram and CA.

A normal patter" of systolic foreshortening (SF) of the coronary artery branches (distal and proximal segments moving toward each other) was observed in all NM pts. Normal SF was noted in 9/10 CM pts. Absent SF, in the one CM patient occurred in association with akinesis of the corresponding left ventricular segment on LVgram. Six/seven CP pts had coronary arterial segments with no SF, despite normal LVgrams and EF. The CP patient with normal SF did not have demonstrable pericardial calcifi- cation.

Thus, absent SF of the CA with normal LVgram is sug- gestive of CP and is probably due to entrapment of the epicardial CA within the fibrotic pericardium. This angiographic sign may suggest the diagnosis in unsuspected CP.

MALIGNANCY FOLLOWING CARDIAC TRANSPLAXTATION Jeffrey L. Anderson, MD; John G. Krikorian, MD; Charles P. Bieber, MD; Edward B. Stinson, MD, FACC, Divisions of Cardiology and Oncology, and Cardiovascular Surgery Dept., Stanford Medical Center, Stanford CA.

Experience with de "ova malignancy complicating organ -- transplantation has previously accumulated for series of renal but not cardiac transplant recipients. From January, 1968, to April, 1977, 124 patients had undergone cardiac transplantation at Stanford. All were followed to August, 1977, or until death (mean and median follow-up of 18.3 and 9.7 months, respectively), and "one were lost to follow-up. Seven malignancies occurred, 4.5 to 6.1.9 mnths after sur-

gery. Malignancy was responsible for 4 (11%) of the 35 deaths occurring later than 3 months Eollowing surgery. Lymphoproliferative disease occurred in the 3 youngest of the 7 patients: one (26 yrs) died of #extensive unclassi- fied visceral lymphoma; one (19 yrs) died of diffuse histo- cytic lymphoma (DHL) involving lung and cerebellum; and one (14 yrs) has entered prolonged remission following radiotherapy for primary DHL of brain. A 45 yr old patient receiving cyclophosphamide developed fatal acute myeloge- "ous leukemia. Adenocarcinoma of colon, metastatic to liver, led to the death of a 54 yr old patient. Two long- term survivors developed squamous cell carcinomas of skin. A high frequency of HLA antigens Al and B8 occurred in the malignancy group. Mean age in the 7 "atients was less, but not significantly so. Number of rejection episodes, type of immunosuppressive agent, blood type, underlying disease, and sex did not differentiate those developing malignancy. Cardiac and renal transplantation appear to involve a similar risk of de nova nmlignancy consequent to -~ long-term immunosuppression. The risks of malignancy war- rant consideration, especially in younger patients being evaluated for cardiac transplantation. An aggressive approach to primary DHL of brain is indicated.

THE MYTH OF REDUCED AORTIC AND LEFT ATRIAL SIZE IN ATRIAL SEPTAL DEFECT Leslie E. Oliver, MD; Dean T. Mason, MD, FACC; Hugo Bogren, MD, FACC; Lily George, MD; and Antiiony N. DeMaria, MD, FACC, University of California, Davis, California.

Although it is widely held that aortic and left atria1 size are diminished in patients with atria1 septal de- fect (ASD), few data are available to support this con- tention. Therefore aortic and left heart chamber dimen- sions in 24 oatients with catheter documented ASD were compared to 20 normals (N) by echocardiography, cine- angiography and qualitative estimation of aortic size by chest x-ray. Stroke index was similar in ASD and N: (mean) 37.5 vs 42.8 ml/M2 by dye-dilution and 35.1 vs 36.3 ml/M2 by angiography (both NS); as was shortening fraction by echo: .34 vs .36 (NS). Echographic left ventricular diastolic dimension index was less in ASD than N: 2.5?r vs 3.0 cm/M' (pc.02); however diastolic volume index by angio was similar in these groups: 50.1 vs 52.9 (NS). Importantly, the size of the aorta was similar in ASD and N 1.7 vs 1.7 cm/M2 by echogram and 1.6 vs 1.5 cm/M* by angiogram (both NS), while both left atria1 and left atria1 to aortic ratio were greater in ASD than N: 2.2 "s 1.9 (NS) and 1.3 vs 1.1 cm/M2 re- spectively (pc.02). Although aortic size was estimated to be small in 12 of 24 (50%) ASD patients by chest x-ray, no differences existed in aortic measurements by echocardiography or angiography from patients judged to have normal sized aortas. Thus, these data do not support the long held belief that stroke volume is re- duced and aortic and left atria1 size are diminished in patients with atria1 septal defect.

February 1978 The American Journal of CARDIOLOGY Volume 41 445