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Cytomegalovirus Infection

PEDIATRICS Vol. 108 No. 6 December 2001, pp. e114

ELECTRONIC ARTICLE

Neonatal Aortic Arch Thrombosis as a Result of Congenital Cytomegalovirus Infection

Marcello Lanari, MD, PhD*, Tiziana Lazzarotto, MB{ddagger}, Irene Papa, MD*, Valentina Venturi, MD*, Gabriele Bronzetti, MD§, Brunella Guerra, MD||, Giacomo Faldella, MD*, Luigi Corvaglia, MD*, Fernando Maria Picchio, MD, FESC§, Maria Paola Landini, MD{ddagger} and Gian Paolo Salvioli, MD*

* Department of Preventive Pediatrics and Neonatology, University of Bologna, Bologna, Italy
{ddagger} Department of Clinical and Experimental Medicine (Section of Microbiology), University of Bologna, Bologna, Italy
§ Department of Pediatric Cardiology, University of Bologna, Bologna, Italy
|| Department of Obstetrics and Gynecology, University of Bologna, Bologna, Italy

Thrombotic disease is rare in neonates. The main risk factors at this age are perinatal asphyxia, maternal diabetes, sepsis, polycythemia, dehydration, a low cardiac output, and in primis the catheterization of central lines. Another important risk factor is inherited thrombophilia. Arterial thrombosis is even more rare than venous thrombosis and less related to most of the risk factors listed above; it occurs more frequently in the iliac, femoral, and cerebral arteries but very rarely in the aorta. Most of the described cases of aortic thrombosis are associated with the catheterization of an umbilical artery and involve the descending tract and the renal arteries; very few relate to the ascending tract and the aortic arch. The possible role of virus-induced primary vascular endothelium damage in the etiopathogenesis of neonatal arterial thrombosis has been previously hypothesized. Herpesviruses, particularly human cytomegalovirus (HCMV), can infect endothelial cells and directly damage intact vascular endothelium, altering its thromboresistant surface as a result of procoagulant activity mediated by specific viral surface phospholipids, necessary for the coagulation enzyme complex assembly that leads to thrombin generation. We describe a case of congenital aortic arch thrombosis. The clinical, laboratory, and virologic pictures; the anatomopathologic findings (fully compatible with viral infection); the detection of HCMV in various tissues (including the aorta); and the absence of other causes of aortic thrombosis make it possible to attribute the case to a severe congenital HCMV infection with multiple organ involvement, after the primary infection of the mother. The hemostatic system disorders and hemodynamic disturbances related to viral cardiac damage explain the clinical features of the case and indicate that congenital HCMV infection should be included among the causes of neonatal aortic thrombosis.

Key Words: human cytomegalovirus • congenital infection • aortic thrombosis • myocarditis • polymerase chain reaction

Abbreviations: HCMV, human cytomegalovirus • IUGR, intrauterine growth retardation • NICU, neonatal intensive care unit • qPCR, quantitative polymerase chain reaction

Received for publication May 14, 2001; Accepted Jul 30, 2001.