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Pathological Case of the Month

Pathological Case of the Month Abstract A 23-YEAR-OLD pregnant white woman, gravida 2, para 0, presented at 35 weeks' gestation with absence of fetal movements. The patient was admitted for fetal monitoring. The fetal heart rate had dropped to 70/min. Ultrasonographic evaluation showed breech presentation with evidence of ascites and pleural effusion suggesting hydrops fetalis. A live male infant weighing 3250 g was delivered by emergency cesarean section. The newborn had generalized edema, absent muscle tone, and bradycardia, with an Apgar score of 1 at 1 and 20 minutes. An echocardiogram detected a bright echo on the inferoposterior aspect of the interventricular groove with normal systolic function and ejection fraction (Figure 1). Despite resuscitation, the infant died 12 hours afterbirth. At autopsy, the heart weighed 42 g. There were numerous flame-shaped epicardial hemorrhages. Near the tip of the left ventricle on the lateral surface, a pale tan mass measuring 22 ×15×8mm was seen (Figure 2). References 1. Smicha A, Wells B, Tynan M, Waterston DJ. Primary cardiac tumors in childhood . Arch Dis Child . 1971;46:508-514.Crossref 2. Groves AMM, Fagg NLK, Cook AC, Allan LD. Cardiac tumors in intrauterine life . Arch Dis Child . 1992;67:1189-1192.Crossref 3. Smith HC, Watson GH, Patel RG, Super M. Cardiac rhabdomyomata in tuberous sclerosis: their course and diagnostic value . Arch Dis Child . 1989;64:196-200.Crossref 4. Harding CO, Pagon RA. Incidence of tuberous sclerosis in patients with cardiac rhabdomyoma . Am J Med Genet . 1990;37:443-446.Crossref 5. Smythe JF, Dyck JD, Smallhorn JF, Freedom RM. Natural history of cardiac rhabdomyoma in infancy and childhood . Am J Cardiol . 1990;66:1247-1249.Crossref 6. Soltan MH, Keohane C. Hydrops fetalis due to congenital cardiac rhabdomyoma . Br J Obstet Gynaecol . 1981;88:771-773.Crossref 7. Geva T, Santini F, Pear W, Driscoll S, Praagh RV. Cardiac rhabdomyoma: rare cause of death . Chest . 1991;99:139-143.Crossref 8. Mehta AV. Rhabdomyoma and ventricular preexcitation syndrome: a report of two cases and review of literature . AJDC . 1993;147:669-671. 9. Giacoia GP. Fetal rhabdomyoma: a prenatal echocardiographic marker of tuberous sclerosis . Am J Perinatol . 1992;9:111-114.Crossref 10. Farooki ZQ, Ross RD, Paridon SM, Humes RA, Karpawich PP, Pinsky WW. Spontaneous regression of cardiac rhabdomyoma . Am J Cardiol . 1991;67:897-899.Crossref 11. Fenoglio JJ, McAllister HA, Ferrans VJ. Cardiac rhabdomyoma: a clinicopathologic and electron microscopic study . Am J Cardiol . 1976;38:241-251.Crossref 12. Burke AP, Virmani R. Cardiac rhabdomyoma: a clinicopathologic study . Mod Pathol . 1991;4:70-74. 13. Saller DN, Lesser KB, Harrel U, Rogers BB, Oyer CE. The clinical utility of the perinatal autopsy . JAMA . 1995;273:663-665.Crossref http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Pediatrics & Adolescent Medicine American Medical Association

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Publisher
American Medical Association
Copyright
Copyright © 1996 American Medical Association. All Rights Reserved.
ISSN
1072-4710
eISSN
1538-3628
DOI
10.1001/archpedi.1996.02170330103019
Publisher site
See Article on Publisher Site

Abstract

Abstract A 23-YEAR-OLD pregnant white woman, gravida 2, para 0, presented at 35 weeks' gestation with absence of fetal movements. The patient was admitted for fetal monitoring. The fetal heart rate had dropped to 70/min. Ultrasonographic evaluation showed breech presentation with evidence of ascites and pleural effusion suggesting hydrops fetalis. A live male infant weighing 3250 g was delivered by emergency cesarean section. The newborn had generalized edema, absent muscle tone, and bradycardia, with an Apgar score of 1 at 1 and 20 minutes. An echocardiogram detected a bright echo on the inferoposterior aspect of the interventricular groove with normal systolic function and ejection fraction (Figure 1). Despite resuscitation, the infant died 12 hours afterbirth. At autopsy, the heart weighed 42 g. There were numerous flame-shaped epicardial hemorrhages. Near the tip of the left ventricle on the lateral surface, a pale tan mass measuring 22 ×15×8mm was seen (Figure 2). References 1. Smicha A, Wells B, Tynan M, Waterston DJ. Primary cardiac tumors in childhood . Arch Dis Child . 1971;46:508-514.Crossref 2. Groves AMM, Fagg NLK, Cook AC, Allan LD. Cardiac tumors in intrauterine life . Arch Dis Child . 1992;67:1189-1192.Crossref 3. Smith HC, Watson GH, Patel RG, Super M. Cardiac rhabdomyomata in tuberous sclerosis: their course and diagnostic value . Arch Dis Child . 1989;64:196-200.Crossref 4. Harding CO, Pagon RA. Incidence of tuberous sclerosis in patients with cardiac rhabdomyoma . Am J Med Genet . 1990;37:443-446.Crossref 5. Smythe JF, Dyck JD, Smallhorn JF, Freedom RM. Natural history of cardiac rhabdomyoma in infancy and childhood . Am J Cardiol . 1990;66:1247-1249.Crossref 6. Soltan MH, Keohane C. Hydrops fetalis due to congenital cardiac rhabdomyoma . Br J Obstet Gynaecol . 1981;88:771-773.Crossref 7. Geva T, Santini F, Pear W, Driscoll S, Praagh RV. Cardiac rhabdomyoma: rare cause of death . Chest . 1991;99:139-143.Crossref 8. Mehta AV. Rhabdomyoma and ventricular preexcitation syndrome: a report of two cases and review of literature . AJDC . 1993;147:669-671. 9. Giacoia GP. Fetal rhabdomyoma: a prenatal echocardiographic marker of tuberous sclerosis . Am J Perinatol . 1992;9:111-114.Crossref 10. Farooki ZQ, Ross RD, Paridon SM, Humes RA, Karpawich PP, Pinsky WW. Spontaneous regression of cardiac rhabdomyoma . Am J Cardiol . 1991;67:897-899.Crossref 11. Fenoglio JJ, McAllister HA, Ferrans VJ. Cardiac rhabdomyoma: a clinicopathologic and electron microscopic study . Am J Cardiol . 1976;38:241-251.Crossref 12. Burke AP, Virmani R. Cardiac rhabdomyoma: a clinicopathologic study . Mod Pathol . 1991;4:70-74. 13. Saller DN, Lesser KB, Harrel U, Rogers BB, Oyer CE. The clinical utility of the perinatal autopsy . JAMA . 1995;273:663-665.Crossref

Journal

Archives of Pediatrics & Adolescent MedicineAmerican Medical Association

Published: Aug 1, 1996

References