MASSIVE MYOCARDIAL CALCIFICATION IN THE PERINATAL-PERIOD

Massive myocardial calcification (MMC) in the perinatal period is an u nusual finding considered to be a unique tissue reaction. This report summarizes the clinical and pathologic findings of seven cases of peri natal MMC. All patients presented clinical evidence of myocardial dama ge. In two cases arrhythmia was detected in utero. Four cases presente d with hydrops, one of which was associated with major heart malformat ion. One case was a trisomy 13. Three cases had polyhydramnios. Our re sults demonstrate that calcification follows progressive stages from p atches of calcified myocardial cells (stage I), to coagulative and col liquative myocytolysis with clusters of interstitial mononucleated cel ls (stage II), to collapsing fibrosis with granulation tissue and mult inucleated regenerative myocardial cells (stage III), and finally to f ibrous scars containing entrapped remaining myocardial cells (stage IV ). Literature review and our findings suggest that perinatal MMC resul ts from different conditions inducing hypoxic-ischemic damage that lat er is followed by progressive scarring if the patient survives the acu te stage. The lesion may represent the human counterpart of the so-cal led dystrophic cardiac calcinosis in mice. This disease is related to an abnormality at the Dyscalc locus of proximal chromosome 7 (syntenic with human chromosome 19q13 and 11p15).