Stone heart syndrome

Stone heart syndrome

Abstract: Global ischemic contracture of the heart resulting in a firm myocardium and loss of intracavitary volume is termed stone heart syndrome. It was described in the early years of open heart surgery with cardiopulmonary bypass.

Global ischemic contracture of the heart resulting in a firm myocardium and loss of intracavitary volume is termed stone heart syndrome.¹ Physiologists have long described cardiac arrest in systole with calcium which is also considered as the danger of intravenous administration of calcium in the presence of digoxin toxicity (digitoxicity). Stone heart syndrome was described as a complication of cardiopulmonary bypass in the early years of cardiac surgery as an inability to successful resuscitation.² This complication of cardiopulmonary bypass has largely been prevented in later years with good cardioplegia.

Prevention of stone heart syndrome

In experimental animals, beta blockade has been shown to delay myocardial ischemic contracture.³ Moderate hypothermia was also shown to be effective in preventing ischemic contracture in the same study. A recent cardiac magnetic resonance imaging based study in porcine model has shown that even mild hypothermia is useful in prevention of the dreaded stone heart syndrome of ischemic cardiac contracture.⁴ In this context, hypothermia can be classified into mild (32°C – 34°C), moderate (28°C-32°C), deep  (28°C-15°C), and profound (< 15°C). Successful resuscitation after ice water drowning gives a good clinical example of hypothermia protecting against stone heart. A two and a half year old girl had good neurological recovery even after 66 minutes of submersion in cold water with core temperature dropping to 19°C.⁵ In this remarkable event, cardiopulmonary resuscitation was maintained for over two hours before extracorporeal rewarming.

Genesis of ischemic contracture in stone heart syndrome

31P based nuclear magnetic resonance spectroscopy imaging has documented that ischemic contracture coincides with the stopping of anaerobic glycolysis in a murine model.⁶ It is known that ischemic contracture is the result of formation of rigor bond when the adenosine triphosphate (ATP) levels are low in the myofibrils. Stoppage of glycolysis naturally leads to depletion of ATP levels and ischemic contracture, causing the stone heart syndrome.

References

1. Cooley DA, Reul GJ, Wukasch DC. Ischemic contracture of the heart: “stone heart”. Am J Cardiol. 1972 Apr;29(4):575-7.
2. Wukasch DC, Reul GJ, Milam JD, Hallman GL, Cooley DA. The “stone heart” syndrome. Surgery. 1972 Dec;72(6):1071-80.
3. MacGregor DC, Wilson GJ, Tanaka S, Holness DE, Lixfeld W, Silver MD, Rubis LJ, Goldstein W, Gunstensen J, Bigelow WG. Ischemic contracture of the left ventricle. Production and prevention. J Thorac Cardiovasc Surg. 1975 Dec;70(6):945-54.
4. Sorrell VL, Paleru V, Altbach MI, Hilwig RW, Kern KB, Gaballa M, Ewy GA, Berg RA. Mild hypothermia delays the development of stone heart from untreated sustained ventricular fibrillation–a cardiovascular magnetic resonance study. J Cardiovasc Magn Reson. 2011 Mar 6;13:17.
5. Bolte RG, Black PG, Bowers RS, Thorne JK, Corneli HM. The use of extracorporeal rewarming in a child submerged for 66 minutes. JAMA. 1988 Jul 15;260(3):377-9.
6. Kingsley PB, Sako EY, Yang MQ, Zimmer SD, Ugurbil K, Foker JE, From AH. Ischemic contracture begins when anaerobic glycolysis stops: a 31P-NMR study of isolated rat hearts. Am J Physiol. 1991 Aug;261(2 Pt 2):H469-78.