The objective of this study was to determine whether the known myocard
ial degeneration in TTP is due to apoptosis. In TTP the heart is often
involved, including the cardiac conduction system. Despite many plate
let occlusions of small coronary arteries, there is little myocardial
necrosis. Why the intermittent clinical episodes begin or end is unkno
wn. Six hearts of patients dying with TTP were examined with routine a
nd immuno-histochemical stains. In addition to ventricular and atrial
myocardium we examined the cardiac conduction system and coronary chem
oreceptor. Numerous small coronary arteries were occluded with platele
t thrombi in all these sites, including especially the sinus node, AV
node and His bundle. The myocardial degeneration we found was conspicu
ously devoid of inflammation and the myocytes were relatively intact.
These characteristics combined with TUNEL-positivity in the degenerati
ng cells are typical of apoptosis. The focal degeneration in TTP is pr
imarily apoptotic. Because circulating serotonin is carried by platele
ts and is released during aggregation, and because serotonin can cause
a powerful cardiogenic hypertensive chemoreflex, we suggest that such
a response may dislodge early platelet aggregations. Lessons from TTP
may have special relevance for better understanding of myocardial rep
erfusion problems associated with angioplasty, thrombolysis and ischem
ic preconditioning.