ABSTRACTS
THE SPECTRUM
OF CARDIAC SARCOIDOSIS:A
LOGIC
STUDY
OF 83 PATIENTS.
Grover
M.
Hutchins,
F.A.C.C.
M.D.,
Kenneth
Bernadine
The Johns Hopkins Medical
Although
sarcoid may involve
CLINICOPATHO-
J.
Silverman,
M.D.,
H. Bulkley,
M.D.,
Institutions,
Baltimore,Md.
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
patientsranged 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. other I8 patients (82%) had trivial myocardial involvement lesions evident
only microscopically.
ventricular arrhythmias, sudden death.
7 (39%)
Of these,
The with
only 2 (II%)
hod
conduction block and 4 (22%)
Comparing the 22 patients with and the 61 without cardiac sarcoid, 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 tive heart failure diac sarcoid, The results
was as frequent
in patients
lesions.
Congs-
with and without
car-
and correlated more with systemic hypertension. show that although myocardial involvement occurs in
over 25% of patients
with sarcoid,
trivial
silent.
and clinically
it is most often morphologically Arrhythmias and heart block are more
common inthe cardiac sarcaid group, only that small subset of patients cardial
evident
but the findings
suggest that
with severe grossly evident
myo-
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 angiography was assessed in patients (pts) with 1) constrictive pericarditis (CP), 2) cardiomyopathy (CM), and 3) normals (NM). CP was established in 7 pts by characteristic 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 fractions (EF), >55%. The 10 CM pts had abnormal EF (mean 25 +3 SEM) and normal CA. The 10 NM pts had normal hemodynamics, 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 calcification. Thus, absent SF of the CA with normal LVgram is suggestive 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 6.1.9mnths after surgery. 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 unclassified visceral lymphoma; one (19 yrs) died of diffuse histocytic 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 longterm 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 warrant 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 defect (ASD), few data are available to support this contention. Therefore aortic and left heart chamber dimensions in 24 oatients with catheter documented ASD were compared to 20 normals (N) by echocardiography, cineangiography 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?rvs 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 respectively (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 reduced and aortic and left atria1 size are diminished in patients with atria1 septal defect.
February 1978
The American Journal of CARDIOLOGY
Volume 41
445