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Ann Thorac Surg 2004;77:2260-2261
© 2004 The Society of Thoracic Surgeons
Department of Cardiovascular Surgery, Istanbul University, Millet Caddesi, Capa, Istanbul 34390, Turkey
e-mail: dr_murat_basaran{at}hotmail.com
To the Editor:
We read with great interest the case report by Dr Simsic and colleagues [1] concerning a male newborn who experienced two episodes of systemic–pulmonary artery shunt thrombosis. On the basis of this experience, the authors emphasized the importance of hematologic evaluation of pediatric patients with unexpected thrombotic complications.
A male neonate weighing 3.2 kg with the diagnosis of intact ventricular septum and pulmonary atresia was referred to our clinic. Preoperative ductal patency was maintained with prostaglandin E1 infusion. Transthoracic echocardiography confirmed the initial diagnosis and revealed a tricuspid Z value of less than –4. On day 4 of life, a modified right Blalock-Taussig shunt was made through a median sternotomy. Systemic arterial oxygen saturation was 92%, and postoperative anticoagulation was achieved with intravenous administration of heparin sodium (50 U · kg–1 · day–1).
Five hours after operation, acute hemodynamic collapse accompanied by systemic acidosis and decreased arterial oxygen saturation occurred. With a presumptive diagnosis of early shunt thrombosis, the patient was taken urgently to the operating room. The previous shunt was found to be occluded with thrombus, and after systemic heparinization (100 U/kg), a new 5-mm Gore-Tex graft was placed between the innominate and main pulmonary arteries. However, the new graft also became occluded with fresh thrombus while the patient was still in the operating room. We decided to perform a central shunt operation with a 5-mm graft between the ascending aorta and the main pulmonary artery. Systemic arterial oxygen saturation again increased to 97%, and with moderate inotropic support, the patient was transferred to the intensive care unit. Postoperatively, a heparin infusion and fresh frozen plasma were given. After the first postoperative day, the patient again experienced an acute decrease in arterial oxygen saturation and hemodynamic collapse, and he died before he could be returned to the operating theater. Hematologic studies performed during the early stay in the intensive care unit revealed that the patient was homozygous for hereditary protein C deficiency. The functional protein C level was less than 2% of the normal value.
Patients with hereditary protein C deficiency are fairly rare. Therefore, it is difficult to make firm guidelines about when or how to search for deficiency states and how to treat or counsel affected patients. We agree with Simsic and coauthors that the combination of these abnormalities and a low antithrombin III level during the neonatal period can increase the risk of thrombosis. In patients without other systemic diseases, the hereditary deficiency syndromes may not pose serious problems; however, we believe that they are important, especially in neonates who require shunt operations very early in life.
In conclusion, we emphasize that early thrombosis is a dreadful event after a modified Blalock-Taussig shunt operation and that hereditary deficiency syndromes should be considered in neonates who experience unexpected thrombotic complications.
References
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