Neonatal aortic arch thrombosis

Abstract

The infant was commenced on a prostaglandin E2 infusion and transferred to the regional cardiothoracic unit for further investigation.An echocardiogram demonstrated normal intracardiac structures and connections with a structurally normal aortic valve and no evidence of coarctation. There was a small left to right shunt across a patent foramen ovale, right ventricular hypertrophy and moderate tricuspid reflux, indicating raised right ventricular pressure. The most impressive finding was that of a large mass in the aortic arch, extending from just proximal to the innominate artery to beyond the left subclavian artery (fig 1). The mass was irregular in shape with an echodense envelope. It projected into the innominate and left carotid arteries, partially obstructing flow up both carotids, left subclavian artery, and down the aorta (gradient 35 mm Hg). There was no evidence of an intracardiac mass.Magnetic resonance imaging (MRI) of chest and head was performed at 3 days of age. In addition to confirming the intra-aortic mass, it also showed areas of subacute infarction in the right frontal and left parietal cerebral cortex (fig 2A). Haemoglobin, platelet count, a clotting screen, and thrombophilia screen (including antithrombin III, protein C and S) were all within the normal range for age. Both parents had normal protein C activity. A diagnosis was made of an aortic arch thrombus with cerebral embolisation.Cardiopulmonary bypass or aortic cross clamping and surgical removal of the thrombus was not possible because it extended towards Figure 1 Two dimensional echocardiographic view in high right parasternal long axis plane showing the thrombus, partially lysed centrally, almost occluding the origin of the innominate artery. T= thrombus, A = innominate artery, AA =aortic arch, and PA =pulmonary artery.

Neonatal arterial thrombus mimicking congenital heart disease

“…N eonatal arterial thrombosis presenting as suspected congenital heart disease, particularly coarctation of the aorta, has been reported previously. [1][2][3][4] The incidence is not known; however, the incidence of symptomatic neonatal arterial thromboembolic disease is reported to be approximately 0.25/10 000 live births, with 90% of these associated with arterial access devices. 5 Mortality is high, irrespective of treatment, and has been quoted as high as 33% in infants with aortic thrombosis.…”

A case of neonatal arterial thrombosis mimicking interrupted aortic arch

“…When we screened the literature, we found 10 cases where AC or IA was considered initially, but a diagnosis of aortic thrombosis was made subsequently (Table 1) (3)(4)(5)(6)(7)(8)(9)(10)(11). In 9 of these 10 patients, the symptoms started in the first 48 hours of life as in our patient.…”

Aortic Arch Thrombosis in a Neonate With Heterozygous Carrier Status of Factor V Leiden Mutation

“…Medline search of English literature over the last 20 years detected only 12 cases, similar to the one we have described (see the table). [7][8][9][10][11][12][13][14][15][16][17] This clinical entity usually manifests within the first hours to days of life with severe heart failure and symptoms mimicking coarctation or interruption of the aorta. Other more frequently suggested diagnoses include cyanotic congenital heart disease and hypoplastic left heart.…”

“…Prognosis of spontaneous aortic arch thrombosis depends on the location of the thrombus, involvement of other large vessels, and the presence of neurological complications. 11,16 Both surgery and thrombolysis have been advocated as possible primary treatment options. 9,[13][14][15]28 Surgery offers the advantage of immediate relief of the life-threatening obstruction and has been successfully used in the treatment of organized, partially calcified thrombi in the aortic arch, without the involvement of other major vessels.…”

“…9 The feasibility of surgical treatment in thrombosis, starting proximal to the origin of the innominate artery, has been questioned. 16 Some authors have advocated a primary trial of thrombolytic therapy in a clinically stable child; successful resolution of abdominal aortal as well as aortic arch thrombi has been achieved by thrombolysis alone. 17,28 Conti and colleagues considered thrombolysis as a primary treatment option in case of early thrombosis of the descending aorta with clinical signs developing over the first 10-16 days of life.…”