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Cardiovascular Pathology
Tuesday, March 4, 2008 , 7:30 PM
Convention Center 103
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Moderator:
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ALLEN BURKE
University of Maryland School of Medicine
Baltimore, MD
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Disclosure:
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The speakers have indicated they have nothing to disclose.
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Clinical histories are displayed below.
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for Text and References
Submitted by: Cristina Basso
Z.A., Male 15 Year Old
Circumstances of death.
Athlete, sudden death while sleeping
Previous medical history.
Asymptomatic. Annual screening for competitive sport activity:
- Normal physical examination
- No symptoms (palpitations, syncope)
- Normal 12-lead ECG
The last 12-lead resting ECG, performed eight months before death for sports eligibility, was available and showed (fig.1):
- Incomplete right bundle branch block
- Mild ST segment elevation in V1-V2
- Negative T wave in V1
- Q wave in D2, D3 and AVF
Medico-legal autopsy ruled out unnatural and extracardiac causes of death.
Gross examination of the heart: Heart weight of 300 gr, transverse diameter 9,5 cm, longitudinal diameter 9,5 cm. LV wall thickness 13 mm, septal thickness 14 mm, RV free wall 3 mm.
Origin and course of coronary arteries are normal, patent. Semilunar and atrioventricular valves are normal.
No evidence myocardial hypertrophy, cavity enlargement, fatty infiltration and aneurysm formation (fig.2a)
Presence of an "infarct-like" withish band in the outer mid subepicardial layer of the postero-septal and postero-lateral walls of the left ventricle (fig.2b).
Multiple samples of the myocardium are taken for histology as well as for molecular pathology investigation, including spleen and blood (frozen -80°).
Histology of the myocardium. (fig.3a,b,c,d)
Acute-subacute myocyte necrosis associated with inflammation. The inflammatory infiltrate was polymorphous and was associated with contraction band necrosis, myocardlysis, granulation tissue and loose fibrous and fatty tissue repair.
Few months later the index case of the same family, a guy who had an aborted sudden death at the age of 18, was found...
The underlying disease at risk of sudden death and differential diagnosis with other conditions will be discussed.
Molecular pathology findings as well as results of family screening will be presented.
Case 1 - Slide 1
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Case 1 - Figure 1
12-lead ECG performed eight months before death shows: incomplete right bundle branch block, mild ST segment elevation in V1-V2, negative T wave in V1, Q wave in D2, D3 and AVF.
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Case 1 - Figure 2
Gross examination of the heart. A) anterior, external view showing a apparently normal heart, in the absence of cardiomegaly, fatty infiltration and right and/or left ventricular aneurysm formation; B) cross sections at mid and apical level showing an "infarct-like" whitish band in the outer mid subepicardial layer of the postero-septal and postero-lateral walls of the left ventricle.
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Case 1 - Figure 3A & B
Histology of the myocardium. A) Panoramic full thickness section of the postero-lateral wall of the left ventricle, confirming the fibrous band in the subepicardial layer; B) Same features in the postero-septal region.
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Case 1 - Figure 3C & D
Histology of the myocardium. C) At higher magnification, acute-subacute myocyte necrosis associated with inflammatory infiltrates is evident. D) The inflammatory infiltrate appears polymorphous and is associated with myocytolysis, granulation tissue and loose fibrous and fatty tissue repair.
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for Text and References
Submitted by: Gaetano Thiene
P.E., Male 16 Year Old
Circumstances of death
While watching a soccer game on television, he exulted at the goal and soon after he had sudden loss of consciousness (10:30 PM).
Prompt cardiopulmonary resuscitation (CPR) manoeuvers were unsuccessful and death was ascertained 1 hour later (11:30 PM).
Previous medical history.
- At the age of 3, tonsillectomy
- At the age of 9, surgical repair of left inguinal hernia.
- Regular sport activity (karate) since the age of 8.
- At the age of 12, first syncopal episode on emotion (watching television): cardiological check-up (physical examination and 12 lead ECG) normal (fig.1).
- No family history of cardiac diseases or juvenile sudden death.
Medico-legal autopsy ruled out unnatural and extracardiac causes of death.
Gross examination of the heart: Heart weight 380 gr, transverse diameter 9,5 cm, longitudinal diameter 9,5 cm. LV wall thickness 13 mm, septal thickness 14 mm, RV free wall 3 mm (fig.2).
Normal origin and course of the coronary arteries, patent. Semilunar and atrioventricular valve are normal, except for mild myxoid degeration of mitral valve leaflets (Fig.3a,b). Grossly normal heart (no hypertrophy, scar, acute myocardial infarction, fatty infiltration, aneurysms).
Multiple samples of the myocardium are taken for histology as well as for molecular pathology investigation, including spleen and blood (frozen -80°).
Histology of the myocardium. Diffuse interstitial oedema with scarce interstitial inflammatory cells, in the absence of myocyte damage (fig.4a,b,c,d).
No replacement-type fibrosis, fatty infiltration, small vessel disease, myocardial disarray.
Differential diagnosis among heart diseases at risk of sudden death on emotion will be discussed
Molecular pathology findings as well as results of family screening will be presented.
Case 2 - Slide 1
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Case 2 - Figure 1
Basal 12 lead ECG performed at the time of first syncopal episode (12 years): sinus rhythm (69 bpm), normal PQ and QT intervals and ST segment
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Case 2 - Figure 2
Gross examination of the heart: transverse section of the heart at the apical third showing a normal myocardium.
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Case 2 - Figure 3
Gross examination of the heart, view of the left atrial cavity from above. Note the normal mitral valve leaflets with mild myxoid degeneration (b, close-up of a)
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Case 2 - Figure 4A & B
Histology of the ventricular myocardium. a, b ) mild interstitial edema in the absence of myocyte necrosis
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Case 2 - Figure 4 C & D
Histology of the ventricular myocardium. c ) mild interstitial edema in the absence of myocyte necrosis; d) immunohistochemistry investigation reveals focal T lymphocytes (CD45RO positive)
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for Text and References
Submitted by: Bill Edwards - Mayo Clinic, Rochester, MN
A 59-year-old woman was staying in Rochester, Minnesota, with her husband while he was recuperating following surgery at the Mayo Clinic. On the night of her death, she was last seen alive at 9:30 p.m. and was found dead in bed by her daughter at 12:30 a.m. There was no evidence of suicide or foul play. The county coroner ordered an autopsy.
According to the husband and daughter, she had been in good general health but had recently complained of nausea, exhaustion, and swollen feet. She was obese. There was also a long-standing history of polycythemia vera and a remote history of poliomyelitis.
Case 3 - Slide 1
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for Text and References
Submitted by: Allen Burke - CVPath Institute, Gaithersburg, MD
A 33-year-old Asian woman complained of dizziness for several months. She did not seek medical care, and had no prior medical history other than for obstetric care, which was uncomplicated. There was no family history of cardiovascular disease or sudden death. She died suddenly and unexpectedly after a witnessed collapse.
A forensic autopsy was performed.
Case 4 - Slide 1
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