Abstract
In 12 autopsy cases of myotonic dystrophy, the most frequently observed histopathologic lesions of the cardiac conduction system were fibrosis, fatty infiltration and atrophy. Fibrosis involved the sinus node in 6 cases, atrioventricular (AV) node in 7, AV bundle in 8, bundle branches in 10 and ventricular myocardium in 11. Fatty infiltration was observed in the sinus node in two cases, AV node in two, AV bundle in six, bundle branches in one and ventricular myocardium in nine. Atrophy was prominent in the AV bundle in five and bundle branches in eight. Lymphocytes infiltrated the conduction system in three cases and were associated with myotonic dystrophy in two and varicella myocarditis in one. Ventricular myocytes were hypertrophied in seven cases, vacuolated in three and exhibited disarray in two. The distribution and extent of conduction system lesions tended to correspond to antemortem electrocardiographic abnormalities, including prolonged PR interval in six cases, intraventricular conduction delay in six and bundle branch block in four. Cardiac involvement by myotonic dystrophy may have contributed to sudden death in four cases.
| Original language | English (US) |
|---|---|
| Pages (from-to) | 662-671 |
| Number of pages | 10 |
| Journal | Journal of the American College of Cardiology |
| Volume | 11 |
| Issue number | 3 |
| DOIs | |
| State | Published - Jan 1 1988 |
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ASJC Scopus subject areas
- Cardiology and Cardiovascular Medicine
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Pathology of the cardiac conduction system in myotonic dystrophy : A study of 12 cases. / Nguyen, Harold H.; Wolfe, James T.; Holmes, David; Edwards, William D.
In: Journal of the American College of Cardiology, Vol. 11, No. 3, 01.01.1988, p. 662-671.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Pathology of the cardiac conduction system in myotonic dystrophy
T2 - A study of 12 cases
AU - Nguyen, Harold H.
AU - Wolfe, James T.
AU - Holmes, David
AU - Edwards, William D.
PY - 1988/1/1
Y1 - 1988/1/1
N2 - In 12 autopsy cases of myotonic dystrophy, the most frequently observed histopathologic lesions of the cardiac conduction system were fibrosis, fatty infiltration and atrophy. Fibrosis involved the sinus node in 6 cases, atrioventricular (AV) node in 7, AV bundle in 8, bundle branches in 10 and ventricular myocardium in 11. Fatty infiltration was observed in the sinus node in two cases, AV node in two, AV bundle in six, bundle branches in one and ventricular myocardium in nine. Atrophy was prominent in the AV bundle in five and bundle branches in eight. Lymphocytes infiltrated the conduction system in three cases and were associated with myotonic dystrophy in two and varicella myocarditis in one. Ventricular myocytes were hypertrophied in seven cases, vacuolated in three and exhibited disarray in two. The distribution and extent of conduction system lesions tended to correspond to antemortem electrocardiographic abnormalities, including prolonged PR interval in six cases, intraventricular conduction delay in six and bundle branch block in four. Cardiac involvement by myotonic dystrophy may have contributed to sudden death in four cases.
AB - In 12 autopsy cases of myotonic dystrophy, the most frequently observed histopathologic lesions of the cardiac conduction system were fibrosis, fatty infiltration and atrophy. Fibrosis involved the sinus node in 6 cases, atrioventricular (AV) node in 7, AV bundle in 8, bundle branches in 10 and ventricular myocardium in 11. Fatty infiltration was observed in the sinus node in two cases, AV node in two, AV bundle in six, bundle branches in one and ventricular myocardium in nine. Atrophy was prominent in the AV bundle in five and bundle branches in eight. Lymphocytes infiltrated the conduction system in three cases and were associated with myotonic dystrophy in two and varicella myocarditis in one. Ventricular myocytes were hypertrophied in seven cases, vacuolated in three and exhibited disarray in two. The distribution and extent of conduction system lesions tended to correspond to antemortem electrocardiographic abnormalities, including prolonged PR interval in six cases, intraventricular conduction delay in six and bundle branch block in four. Cardiac involvement by myotonic dystrophy may have contributed to sudden death in four cases.
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U2 - 10.1016/0735-1097(88)91547-1
DO - 10.1016/0735-1097(88)91547-1
M3 - Article
C2 - 3278037
AN - SCOPUS:0023853927
VL - 11
SP - 662
EP - 671
JO - Journal of the American College of Cardiology
JF - Journal of the American College of Cardiology
SN - 0735-1097
IS - 3
ER -