Heart Is Stone
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After 2 months in the neonatal ward the weight had increased to 2,110 grams and surgery was performed. Arterial cannulation was performed using a Gore-Tex tube sewn to the right carotid artery and venous bicaval cannulation. Ten seconds after initiation of cardiopulmonary bypass the heart went into a fixed state of hypercontractility and stone heart was evident.
Antegrade cardioplegia with a solution set to 4 C was attempted to relieve the heart. However, this only increased the contractility of the myocardium. It was decided to continue the operation. The corrections of the cardiac abnormalities were performed despite the edematous and contracted state of the myocardium. With each dose of cardioplegia the contraction increased, but slowly decreased afterwards.
When introducing cardiopulmonary bypass ventricular fibrillation occurred immediately. After unsuccessful defibrillation the heart went into a hypercontractile state. In an attempt to resolve the hypercontractile state, blood cardioplegia was initiated without any effect on the contracted myocardium.
Approximately 15 minutes after initiation of cardio pulmonary bypass the abdomen became enlarged. The liver was displaced into the mediastinum and made further access to the heart difficult. In order to enable surgery the abdomen was opened to allow for pressure relief. No signs of intra- or retroperitoneal bleeding were seen. No abnormalities in neither the descending aorta, portal vein, nor inferior vena cava were present to explain the enlarged abdomen. The inside of the right ventricle could now be inspected. The myocardium was extensively hemorrhagic. At this point the heart had gone into a more flaccid state. Blood cardioplegia was repeated, this time in a retrograde fashion. Immediately the myocardium became severely contracted. The VSD was closed using a Gore-Tex patch. After declamping the aorta, reperfusion was commenced, but due to acidosis, hyperkalemia, no signs of cardiac recovery and an obvious non-existing peripheral circulation, surgery was stopped.
The autopsy revealed that the digestive tract was dark, swollen and ischemic from the ventricle to the rectum. The lumen contained fluid and a little blood. The psoas muscles were dark with signs of fresh bleeding. Inspection of the heart showed that the surgical corrections had gone as planned. Both the right and left side of the myocardium was a dark grey-red color, worst on the left side and in the septum. Microscopic examination of the myocardium revealed ischemic changes and fresh diffuse bleeding. The septum and posterior wall of the myocardium showed more signs of more extensive fresh bleeding with the cells being separated by focal bleeding. Microscopy of the lungs showed small alveoli and fibrosis of the parenchyma, but no signs of inflammation.
Patients with congenital heart disease often have pressure or volume overloaded cardiac chambers and therefore may be more prone to myocardial ischemia (6). The use of propranolol and hypothermia has been proven to reduce the risk of stone heart in adults and in animal studies of neonates (1,6). Intermittent stretching of the myocardium has been shown to decrease ischemic contraction during surgery on rabbits (9). However, no studies have examined the effect of these measures in neonates.
O to love thee with a love like this! My heart is stone, melt it with thy love, My heart is locked, let thy love be the master key to open it; O Father, I adore thee for thy great love in the gift of Jesus, O Jesus, I bless thee for resigning thy life for me, O Holy Spirit, I thank thee for revealing to me this mystery; Great God, let thy Son see in me the travail of his soul! Bring me away from my false trusts to rest in him, and him only. Let me not be so callous to his merit as not to love him, so indifferent to his blood as not to desire cleansing.Lord Jesus, Master, Redeemer, Savior, come and take entire possession of me; this is thy right by purchase. In the arms