SN-858 OOOO, XXX 71-year-old-female

Date of Autopsy: 1999.11.22 (49 hours post mortem)

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Clinical Diagnoses by the Department of Cardiology:

1.Ventricular tachycardia
2. Hypertrophic cardiomyopathy
3. Hypertension
4. Chronic hepatitis (HCV)

1. Fresh and old myocardial infarction with apical aneurysm associated with hypertrophic myocardial change (If ignoring the history of long-lasting hypertension, C/W hypertrophic cardiomyopathy, middle type), [ventricular tachycardia]
2. Liver cirrhosis with portal hypertension

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GROSS:

External Examination: The body is that of a well-developed (well-nourished, obese female) weighing 57 kilograms, measuring 150 centimeters in length and appearing consistent with the recorded age of years. The skin is unremarkable.

The head is normally shaped. The sclerae are white and there is no bloody discharge from the ears, nose, or mouth. The hair is normally distributed. There is no external evidence of trauma. The thorax is normally shaped. The breasts are atrophic. The abdomen is flat. Inspection of the extremities reveals no edema of both legs. There is no clubbing.

Incision and Body Cavities: The usual y-shaped thoracoabdominal incision is made. The subcutaneous fat is increased and the exposed musculature is normal in appearance. The panniculus measures 3.0-3.5 cm in thickness. The peritoneal cavity contains no fluid. No adhesions are present.

The organs occupy their usual anatomic relationships. The liver edge is at 7 cm below the right costal margin. The left pleural cavity contains 400 cc of clear, straw-colored fluid, and the surface is smooth and glistening. The right one shows fibrous adhesion. The pericardial cavity contains a small amount of clear, straw-colored fluid.

Cardiovascular System: The heart weighs 530 grams. The epicardium is smooth and glistening. The origin and distribution of the coronary arteries is normal. Serial cross sections of the vessels reveal no remarkable atherosclerosis, without calcification. A complete occlusion is not present. Longitudinal four chamber section reveals remarkably thickened free wall and interventricular septum of the upper two thirds of the left ventricle, whereas the apex shows a 4 x 4 cm globular dilatation of the cavity with remarkably thinned myocardium with 1-2 mm thickness (apical aneurysm). The narrowing of the left ventricular outflow of the subaortic area is not present. The myocardium is reddish-brown and firm, but a focal hemorrhage is scattered in the thickened septum that measures 1.5 x 1.5 x 1.0 cm in size. The left ventricle measures 1.5 - 1.8 cm in thickness in the free wall and 2.0 cm in the septum, and the right ventricle measures 0.5 cm thick. The endocardium is smooth and glistening. The valves are normally shaped with thin, delicate leaflets, but the posterior leafleft of the mitral valve shows remarkable annular calcification at the base. The chordae-tendineae are normal. The valve ring circumferences are: tricuspid 9.0 cm: pulmonary 6.5 cm: mitral ND, and aortic ND. There is moderate atherosclerosis with calcification of the aorta with no aneurysms. The pulmonary arteries show mild dilatation and contain no emboli. The renal arteries are patent with no evidence of narrowing.

Respiratory System: The trachea and larynx are unremarkable. The bronchi are normally distributed and contain a small amount of mucous. The left lung weights 285 grams and the right lung weighs 520 grams. Right pleural surface is fibrously adhered. There is mild congestion of the parenchyma. The cut surface is soft and appears well aerated. There is no nodularity. No thrombi or infarcts are present. There is no fibrosis and no evidence of bronchiectasis, pneumonia and emphysema.

Gastrointestinal Tract: The oral cavity appears unremarkable. The esophagus shows the usual grayish-white, wrinkled mucosa and no evidence of varix. The stomach contains a small amount of gastric juice. Its wall is normal and no ulcerations are present. There is no obstruction of the pylorus. The duodenum is in its normal position and contains a small amount of bile-stained fluid. No ulcerations are present. The loops of small bowel appear unremarkable with no areas of induration or obstruction. The bowel is not dilated, and contains progressively formed feces. The colon is of normal size and is not dilated. There is no ulceration or obstruction and no polyps are identified.

Liver and Biliary Tract: The liver weighs 950 grams. Its capsule is finely granular and the edge is dull. A 1.0 cm solitary cyst is noticed on the upper surface. The cut surface shows thin-septal cirrhosis (Nagayo's type B). The extrahepatic biliary system is unremarkable.

Pancreas: The pancreas occupies its normal position. The peripancreatic fat is unremarkable. The cut surface of the pancreas reveals lobular tissue with remarkable post-mortem autolysis throughout, with no nodules or areas of induration. The pancreatic duct is not dilated.

Urinary Tract: The left kidney weighs 150 grams and the right kidney weighs 120 grams. The perinephric tissue and pelvic fat are unremarkable. The capsule is removed with ease revealing a fairly smooth, reddish-brown parenchymal surface. Corticomedullary demarcations are clear, the cortices measuring up to 6 mm in thickness. No tumors, infarcts, or areas of scarring are present. The calices are not dilated and the pelves are of normal size. The ureters are patent throughout with no dilatation. The urinary bladder contains a small amount of urine. No calculi are present in the urinary tract.

Genital System: The vagina, uterus, fallopian tubes are grossly normal, though atrophic which is consistent with the recorded age of years. Both ovaries are atrophic.

Lymph Nodes, Spleen, and Hematopoietic System: No abnormal lymph nodes are identified. The spleen weighs 340 grams and has a smooth, reddish-blue capsule. The cut surface is soft and reddish-brown with distinct malpighian bodies and mild congestion, the bone marrow from the lumbar vertebral body and the upper third of the femur is red and soft.

Endocrine System: The thyroid weighs 22 grams and is composed of reddish-brown, waxy-appearing tissue throughout with no nodularity. The adrenal glands weigh 5.5 grams and 4.5 grams, respectively, and occupy their normal positions. The cortices are bright yellow and the medullae are gray with no nodules. No thymus is identified.

Musculoskeletal System: The musculature appears normally developed, with no gross degeneration or atrophy. Compression fracture of moderate grade is present in the 4^th lumber vertebra, otherwise there are no gross deformities of the skeletal system.

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MICROSCOPIC:

• Fresh myocardial infarction with hemorrhage and granulocytic infiltrate of the antero-septal portion of the left ventricle, in association with fresh subendocardial coagulative necrosis and old patchy fibrosis of the whole circumference of the left ventricle. Moderate arteriosclerosis, without total occlusion, of the coronary arteries.
• Apical aneurysm of the left ventricle of the heart.
• Disarray of myocardial fibers with interstitial fibrosis, compatible with hypertrophic cardiomyopathy on a purely morphological ground. The penetrating bundle and left branch of His, showing degeneration and atrophy, embedded in the fibrosing process.
• Post-hepatitic, thin-septal liver cirrhosis, with mild esophageal varix and moderate splenomegaly.
• Bilateral residual ovaries identified, showing atrophy.

Direct Cause of Death: Fresh myocardial infarct clinically associated with ventricular tachycardia

Manner of Death: Natural

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Clinical Summary: A 71-year-old house wife was transported to the hospital on an ambulance car because of palpitation on November 18 1999. She had a past history of hypertension since the age thirties and an operation of ovary 40 years ago, otherwise her familial and past medical history is unremarkable.

She noticed edema of the lower extremities at the age of 48 and prescribed with diuretics. During an admission to the orthopedics department of this hospital in April 1 1996, a remarkable cardiac hypertrophy was revealed by cardiac echogram with 3^rd degree of mitral regurgitation, for which a diagnosis of hypertrophic cardiomyopathy with a middle portion thickening was made. She was admitted to this hospital on February 23 1998 because of pneumonia, after that event her daily activity had been lowered. Chronic hepatitis in transition to liver cirrhosis was pointed out with abdominal echogram in February 1999.

Admission examination revealed VT on ECG with a wide QRS complex and tachycardia (160) with clear consciousness. After counter shock recovered her rhythm to the sinus one, she was transferred to the CCU. She died in cardiac arrest on November 20 1999.

Laboratory Data: (November 19 1999) WBC 11,300, RBC 414 x 10^4, Ht 41.5, Hb 13.0, Plt 10.2, T.P. 6.6, T.Bil 1.5, GOT 857, GPT 131, Al-P 325, LDH 1,585, CPK 4,425, Glucose 181, Urea-N 22.8, HCV-Ab positive, HBs-Ag negative.

Comments: This patient died from fresh myocardial infarct of the whole circumference of the subendocardial portion of the left ventricle and of the interventricular septum, associated with a preceding clinical picture of arrhythmia (VT). Considering that there is no remarkable obstruction of the coronary arteries, neither from atheroma nor from emboli, and that disarray of myocardial fibers with plexiform fibrosis is microscopically identified, it is fairly likely from a purely morphological point of view that hypertrophic cardiomyopathy (HCM) was a preexistent pathology in this patient. It is currently argued that myocardial bridging, i.e., intramural coronary artery covered by myocardial fibers, is an important cause of myocardial infarction in children with HCM (Ref.1). However, HCM is explicitly defined in a standard textbook that

The hypertrophy is thus not secondary to a cardiovascular or systemic disease, such as hypertension or aortic stenosis, that places a hemodynamic burden on the left ventricle(Ref.2)

This disease is characterized by left ventricular hypertrophy, typically of a nondilated chamber, without obvious cause such as hypertension or aortic stenosis. [Clinical Features] In symptomatic patients, the most common complains is dyspnea largely due to increased stiffness. Other symptoms include angina pectoris, fatigue, syncope, and near-syncope(Ref.3),

and in this context the attending pathologist (A.H.) still refrains from rendering a diagnosis of hypertrophic cardiomyopathy on this case.

It is intriguing, however, that some workers at Kyoto University lately argue that HCV is an important causal agent in the pathogenesis of not only dilated but also hypertrophic cardiomyopathy (Ref. 4,5,6).

Liver cirrhosis is also confirmed, which might have caused by blood transfusion at the time of alleged operation of the ovary 40 years back there are no clues for the pathology of both the ovaries from the tissue obtained at the autopsy.

References:

1. Gori F, Basso C and Thiene G. Myocardial infarction in a patient with hypertrophic cardiomyopathy. N Eng J Med 2000;342:593-594.
2. Wilson J, Braunwald E et al, eds. Harrison's principles of internal medicine, 12^th ed. New York: McGrawhill, 1991:978-980.
3. Fauci, Braunwald E et al, eds. Harrison's principles of internal medicine, 14^th ed. New York: McGrawhill,1998.
4. Matsumori A, et al. Apical hypertrophic cardiomyopathy and hepatitis C virus infection. Jpn Circ J. 1999;63:433-8.
5. Matsumori A et al. Detection of hepatitis C virus RNA from the heart of paatients with hypertrophic cardiomyopathy. Biochem Biophys Res Commun. 1996;222:678-82.
6. Matsumori A. Molecular and immune mechanisms in the pathogenesis of cardiomyopathy. Role of viruses, cytokines, and nitric oxide. Jpn Circ J. 1997;61:275-91.

March 13 2000

Akio Hasegawa, MD, PhD

Certified Pathologist by the Japanese Society of Pathology (#832)
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Copyright (c) by A. Hasegawa. 2000

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