MICHAEL GRAHAM
Chief Medical Examiner
St. Louis, MO
The decedent is a 23-year-old Caucasian woman who had no known serious disease. She did have a
history of chronic fatigue but had no significant limitation of activities. An examination by her
internist 1 week prior to death yielded no findings of apparent diagnostic significance. Laboratory
studies at that time demonstrated no anemia. Her sed rate was 35. She was "not feeling well" on the
day prior to her death but did attend a picnic and participate in activities. On the day of her death
she experienced "stomach pain" and nausea. Maalox did not offer relief from the discomfort. She was
talking with a relative and suddenly collapsed. Resuscitative efforts were unsuccessful. A
postmortem examination was performed.
The autopsy demonstrated her to be a normally developed,
nourished and hydrated woman who was 67 inches long and weighed 130 pounds. Her heart weighed 290
grams. There was gray-white relatively diffuse thickening of the intima of the proximal few
centimeters of the ascending aorta. A single coronary ostium was present. The coronary ostium arose
normally in proximity to the left sinus of Valsalva. The left main, left anterior descending and
circumflex arteries arose in the usual fashion from this ostium. Branches of the left coronary artery
supplied blood to the posterior aspect of the heart. The left main artery had a gray-white thickened
wall in association with adventitial fibrosis. There was fibrointimal proliferation of the left main
artery resulting in greater than 95% narrowing of the lumen. No right coronary ostium was identified.
The right coronary artery was not identified. There was mottling of the posterior left ventricular
myocardium. The heart was otherwise unremarkable. The remainder of the autopsy revealed no
additional lesions of significance. No ethanol or drugs were detected during routine toxicologic
screening of blood, urine and vitreous fluid.
H&E and Movat stained microscopic sections of the
ascending aorta, coronary ostium/initial coronary artery segment and left coronary artery are
available for your review.
BARBARA SAMPSON
Office of the Chief Medical Examiner
New York, NY
This previously healthy 30 year old obese woman presented to the emergency room with shortness of
breath and weakness. She also had nausea, vomiting, diarrhea, right subscalpular pain, and flank
pain. She was febrile and tachycardic. Her oxygen saturation on room air was 99%. Lab results
revealed a white blood cell count of 22,000, and urine analysis was positive for leucocyte esterase
and many white blood cells. She was diagnosed with pyelonephritis and discharged with PO antibiotics.
The next day she presented to the emergency room again with worsening shortness of breath. She was
hypotensive and tachypnic. Her oxygen saturation was 90% on room air. A chest x-ray was normal. EKG
showed ST elevations. Labs revealed a troponin of 119 and CPK of 960. She developed ventricular
tachycardia and ventricular fibrillation, and could not be resuscitated. The case was reported to and
accepted by the Office of Chief Medical Examiner of the City of New York based on the sudden and
unexpected nature of this woman=s death.
ALLEN P. BURKE
Armed Forces Institute of Pathology
Washington, DC
A 35-year old man with a history of palpitations collapsed while playing basketball. There was no
documented history of syncope, and the patient had had no prior cardiac evaluation. There was no
family history of sudden death.
At autopsy, the heart was 300 grams. There was mildly increased
epicardial fat. The foramen ovale was closed. The left ventricular chamber dimensions were normal:
the left ventricular cavity diameter was 32 mm, the left ventricular free wall was 11 mm thick, the
ventricular septum 10 mm thick. There was mild dilatation of right atrium and right ventricle, and
the right ventricle was 4 mm thick, without gross scars or abnormal fat infiltrates. The valves and
endocardium were grossly unremarkable. There was circumferential scarring in left ventricle,
subepicardial in the free wall, and in the right ventricular aspect of ventricular septum. A
histologic section is provided of the lateral wall of the left ventricle.
CRISTINA BASSO
University of Padua Medical School
Padua, Italy
A 35-year old trucker driver had been admitted to the hospital at age 30 years for evaluation of
recurrent syncopal episodes. He experienced an in-hospital sudden cardiac arrest due to recorded
ventricular fibrillation and was successfully resuscrequired direct current cardioversion at 300 J.
Results of a clinical examination were normal. Serial 12-lead ECGs showed sinus rhythm, first-degree
atrioventricular (AV) block (PR interval 220 ms), right bundle branch block with left axis deviation,
ST segment elevation and inverted T waves in the right precordial leads (Fig. 1). Chest roentgenogram
showed a normal cardiothoracic ratio; the results of baseline hematologic and biochemical studies were
within normal limits. Exercise stress testing and 24-h Holter ambulatory ECG monitoring showed no
arrhythmias. Cardiac catheterisation revealed normal cardiac pressure, as well as normal findings on
left ventricular and coronary angiography and ergonovine testing. Right ventricular angiography was
not performed. Intracardiac electrophysiologic recordings revealed a "borderline" HV interval (70
ms); programmed ventricular stimulation was not performed. The patient was given bet-adrenergic
blocking therapy and his clinical course was uneventful until he died suddenly at rest 5 years later.
Postmortem examination revealed a heart weight of 350 g. The coronary arteries were normal and
there was no evidence of recent or healed myocardial infarction. All cardiac valves were normal. The
right ventricle was moderately enlarged with dilatation of the pulmonary infundibulum. Despite
preserved right ventricular wall thickness (4 to 5 mm), there was significant myocardial fatty
infiltration in the right ventricular anterolateral wall.
Histopatologic examination of the right
ventricular myocardium showed remarkable transmural myocardial fibers dissociation by fatty
infiltration and slight interstitial fibrosis, in the absence of wall thinning, inflammatory
infiltrates and myocyte degenerative changes (Fig. 2). Study of the specialized conduction system
showed normal findings in the sinoatrial node, crista terminalis, atrionodal approaches, AV node and
penetrating AV bundle. Conversely, there was marked fibrosis of the bifurcating His and branch
bundles with fibrous interruption of the proximal right bundle branch (Fig. 3).
Pedigree analysis
revealed that 50% of the investigated family members exhibited some degree of right bundle branch
block or ST segment elevation, or both, in the right precordial leads, as to suggest a pattern of
inheritance compatible with an autosomal dominant pattern with variable expression.
Molecular
genetic investigation in the affected family members disclosed a sodium channel gene mutation (SCN5A)
probably accounting for both Brugada syndrome and Lenegre disease phenotypic expression (courtesy of
Prof. Priori and Prof. Danieli).
