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 Table of Contents  
INTERESTING CASE REPORT
Year : 2021  |  Volume : 5  |  Issue : 3  |  Page : 236-238

Spontaneous Aortic Thrombosis in a Neonate: A Rare Differential Diagnosis for Aortic Coarctation


1 Department of Pediatric Cardiology, Madras Medical Mission, Chennai, Tamil Nadu, India
2 Department of Neonatology, Madras Medical Mission, Chennai, Tamil Nadu, India

Date of Submission10-May-2021
Date of Acceptance26-May-2021
Date of Web Publication06-Jul-2021

Correspondence Address:
Dr. Kothandam Sivakumar
Department of Pediatric Cardiology, Institute of Cardio Vascular Diseases, Madras Medical Mission, 4A Dr J J Nagar, Mogappair, Chennai - 600 037, Tamil Nadu
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jiae.jiae_12_21

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  Abstract 

Spontaneous aortic thrombosis is a serious clinical entity with clinical features that mimic neonatal coarctation of aorta. While both conditions manifest with weak femoral pulses, pallor and poikilothermia of lower limbs are unique to spontaneous aortic thrombosis. Proper imaging helps in precise diagnosis. A combination of thrombolysis and anticoagulation is used for resolution of thrombus. We present images of a neonate with spontaneous aortic thrombosis and its clinical management strategies.

Keywords: Alteplase, Doppler imaging, pulselessness, streptokinase, thrombolysis


How to cite this article:
Sivakumar K, Mohanty S, Karthikeyan S, Sankaranarayanan K. Spontaneous Aortic Thrombosis in a Neonate: A Rare Differential Diagnosis for Aortic Coarctation. J Indian Acad Echocardiogr Cardiovasc Imaging 2021;5:236-8

How to cite this URL:
Sivakumar K, Mohanty S, Karthikeyan S, Sankaranarayanan K. Spontaneous Aortic Thrombosis in a Neonate: A Rare Differential Diagnosis for Aortic Coarctation. J Indian Acad Echocardiogr Cardiovasc Imaging [serial online] 2021 [cited 2022 Jan 27];5:236-8. Available from: https://www.jiaecho.org/text.asp?2021/5/3/236/320781


  Introduction Top


Spontaneous aortic thrombosis is a rare disease in neonates, reported in 80 patients so far.[1] The median age of presentation is 2 days and outcome is fatal in 23%.[1] Even though some form of thromboembolic disease is diagnosed in 5.1/100,000 live births, they are often venous in location. Aortic thrombosis is very rare and is confined to 2%–3% of such patients.[2] Aortic thrombosis is immediately suspected when it is secondary to umbilical arterial cannulation. However, spontaneous aortic thrombosis masquerades clinically as aortic coarctation and is frequently misdiagnosed.[3],[4] When a neonate presents with clinical distress and absent femoral pulses, prostaglandin E1 infusion is initiated with a strong clinical suspicion of coarctation. This report describes an 8-h-old neonate with spontaneous aortic thrombosis, referred to our unit as critical coarctation of aorta.


  Case Report Top


A term neonate born to a primiparous mother with a birth weight of 2.7 kg had an APGAR score of 8, 9, and 9 at 1, 5, and 10 min, respectively. Antenatal ultrasound showed low-resistance flow in the middle cerebral artery and suggested a fetal head sparing effect from perinatal asphyxia. Labor was induced to facilitate vaginal delivery. After birth, the baby was noted to have absent femoral pulses. An echocardiogram after birth showed a left-to-right shunt through a patent oval foramen and patent arterial duct with severe aortic coarctation at another hospital, and he was referred to our unit after initiating prostaglandin E1 infusion along with fluid boluses for hydration.

Upon admission in our unit at 8 h of life, the baby was alert with no significant respiratory distress, lower limbs were cold and pale with no pulses, saturation was 99% in upper-limb pulse oximetry, and blood pressure in both upper limbs was 62/34 (49) mmHg. There were no significant abnormal precordial auscultatory findings. The prostaglandin E1 infusion did not result in any improvement in the lower-limb perfusion.

Repeat echocardiography showed a left-to-right shunt through the patent arterial duct, and there was no evidence of coarctation [Figure 1] and [Video 1]. Abdominal aortic Doppler at diaphragmatic level showed normal pulsatile trace. However, there was a large occlusive echogenic thrombus identified in the lower abdominal aorta without any flow on color Doppler [Figure 2] and [Video 2]. After stopping prostaglandin E1 and confirming normal renal function, computed tomography confirmed infrarenal occlusive abdominal aortic thrombus extending to both common iliac arteries.
Figure 1: Suprasternal long-axis view of the arch (a) with spectral Doppler (b) showing normal-sized ascending aorta, transverse arch, isthmus, and descending aorta with no evidence of coarctation
AA: Ascending aorta, DA: Descending aorta, I: Isthmus, TA: Transverse arch


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Figure 2: Echocardiogram (a) showing infrarenal abdominal aortic thrombus extending to the aortoiliac bifurcation. Coronal reconstructed computed tomographic image (b) with a volume rendering (c) showing large thrombus (star) in the infrarenal abdominal aorta extending into aortoiliac bifurcation with reformation of right external iliac artery and left external iliac artery
AA: Ascending aorta, LEIA: Left external iliac artery, REIA: Right external iliac artery


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[Additional file 1]

Video 1: Suprasternal view of echocardiogram shows normal aortic arch with no evidence of coarctation.

[Additional file 2]

Video 2: Abdominal aortic imaging with echocardiographic probe shows a large thrombus in abdominal aorta below the superior mesenteric artery that arises from the anterior wall of the aorta.

As aortic occlusion caused signs of ischemia of legs, streptokinase infusion was initiated at a dose of 1000 U/kg/h for 24 h and escalated to 2000 U/kg/h till 72 h. Even though there was marginal improvement in pallor and hypothermia, femoral pulses failed to appear. Alteplase infusion was started at a dose of 0.6 mg/kg/h along with unfractionated heparin at a dose of 20 U/kg/h. After 24 h of alteplase, femoral pulses, color, and warmth of the legs improved along with normalization of lower-limb blood pressure and Doppler flows in aorta [Figure 3] and [Video 3]. Warfarin was added to maintain an international normalized ratio above 2.0. Even though an initial thrombophilia screen at neonatal age showed mild reduction in levels of antithrombin III, protein C, and protein S levels, a repeat study done after few months showed normal values. Warfarin was discontinued after 1 year, and a subsequent follow-up for another year showed no recurrences of thrombosis, normal lower-limb pulses, and perfusion.
Figure 3: Image of abdominal aorta with echocardiographic probe showing complete resolution of thrombus (a) and unobstructed flow on color Doppler (b)
AA: Ascending aorta


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[Additional file 3]

Video 3: After thrombolysis, abdominal aortic imaging with echocardiographic probe demonstrates resolution of thrombus and normalization of color Doppler flows.


  Discussion Top


Neonatal venous thrombosis is far more common than arterial thrombosis in clinical practice.[2] Symptomatic arterial thrombosis is rare clinically, but asymptomatic thrombus is seen in 10%–30% of neonates with indwelling catheters.[3] Umbilical arterial catheterization is the most common predisposition for aortic thrombosis. Suprarenal placement, calcium infusions, use of 3.5 F catheter, and longer duration are known risk factors.[5],[6] While neonates appear stable in early phases, thrombus progression leads to rapid deterioration causing hypertension, renal failure, necrotizing enteritis, and progressing to cardiogenic shock and death.[1],[4] Management strategies of postumbilical artery cannulation patients after classifying the aortic thrombosis as mild, moderate, and major, are not applicable in spontaneous aortic thrombosis.[6]

Spontaneous aortic thrombosis occurs in asphyxia, sepsis, meconium aspiration, extreme prematurity, hypernatremic dehydration, and thrombophilia.[1] Interpretation of thrombophilia is erroneous in the neonates due to developmentally low anticoagulant levels and their increased consumption within the thrombus.[7] The same occurred in our patient.[7] While asphyxia and dehydration were the possible causes in our patient, it is often contended that these two factors could have been the effect of the late fetal aortic thrombosis, rather than the cause.[1] Neonatal thrombosis due to deficiencies of antithrombin III, protein C, and protein S; mutations of factor V Leiden and prothrombin G20210A; methylenetetrahydrofolate reductase C677T and maternal antiphospholipid antibodies; and elevated fasting homocysteine and lipoprotein (a) are rare; they often cause thrombosis in adult life. Accordingly, most of these neonates do not receive lifelong anticoagulation.[8]

Even though absent femoral pulses led to a diagnosis of coarctation, strong clinical clues such as pallor, pain, and poikilothermia along with pulselessness should favor spontaneous aortic thrombosis instead of coarctation that lacks the first three signs.[9] Echocardiographers can easily diagnose aortic thrombosis by a color Doppler interrogation of the abdominal aorta till its bifurcation.[10]

Combination of thrombolysis with heparin has shown the best outcomes compared to either of them alone, surgical thrombectomy, thrombus aspiration, and conservative medical management.[1] Suboptimal thrombolysis due to low plasminogen levels in neonates along with the need for higher doses of heparin to maintain adequate anti-Xa levels often results in delay in resolution of thrombus, as seen in our patient also.[1] Fresh frozen plasma or cryoprecipitate to substitute the deficient factors might improve the efficacy of thrombolytic agents.[4]


  Conclusions Top


Spontaneous aortic thrombosis is rare in neonates. Delay in recognition and treatment leads to significant morbidity. Clinical identification of pallor and poikilothermia along with absent femoral pulses differentiates them from coarctation of aorta. Echocardiographic screening of abdominal aorta with color Doppler clinches the diagnosis. A combination of thrombolysis with anticoagulation seems to be the best therapy though resolution of thrombus is delayed due to neonatal physiology. Diagnosis of thrombophilia is important to predict recurrences but challenging in neonatal age.

Consent to publish

Informed consent for the treatment and anonymous display of images in publications was obtained.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the guardian has given consent for images and other clinical information to be reported in the journal. The guardian understands that name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

K Sivakumar is an editorial board member of the Journal of The Indian Academy of Echocardiography & Cardiovascular Imaging. The article was subject to the journal's standard procedures, with peer review handled independently of this editor and their research groups.

There are no other conflicts of interest.

 
  References Top

1.
Mulcaire-Jones JP, Bailly DK, Frank DU, Verma AR, Barney BJ, Siefkes HM. Spontaneous aortic thrombosis in neonates: A case report and review of literature. Cardiol Young 2020;30:95-9.  Back to cited text no. 1
    
2.
Nowak-Göttl U, von Kries R, Göbel U. Neonatal symptomatic thromboembolism in Germany: Two year survey. Arch Dis Child Fetal Neonatal Ed 1997;76:F163-7.  Back to cited text no. 2
    
3.
Oppenheimer DA, Carroll BA, Garth KE. Ultrasonic detection of complications following umbilical arterial catheterization in the neonate. Radiology 1982;145:667-72.  Back to cited text no. 3
    
4.
Knadler JJ, Zobeck M, Masand P, Sartain S, Kyle WB. In utero aortic arch thrombosis masquerading as interrupted aortic arch: A case report and review of the literature. Pediatr Cardiol 2019;40:658-63.  Back to cited text no. 4
    
5.
Boo NY, Wong NC, Zulkifli SS, Lye MS. Risk factors associated with umbilical vascular catheter-associated thrombosis in newborn infants. J Paediatr Child Health 1999;35:460-5.  Back to cited text no. 5
    
6.
Nagel K, Tuckuviene R, Paes B, Chan AK. Neonatal aortic thrombosis: A comprehensive review. Klin Padiatr 2010;222:134-9.  Back to cited text no. 6
    
7.
Yang JY, Chan AK. Pediatric thrombophilia. Pediatr Clin North Am 2013;60:1443-62.  Back to cited text no. 7
    
8.
Wieland I, Jack T, Seidemann K, Boehne M, Schmidt F, Happel CM, et al. Neonatal aortic arch thrombosis: Analysis of thrombophilic risk factors and prognosis. Cardiol Young 2014;24:33-9.  Back to cited text no. 8
    
9.
Kohli U, Lodha R. Idiopathic neonatal aortic thrombosis. Indian J Pediatr 2006;73:1127-9.  Back to cited text no. 9
    
10.
Seibert JJ, Taylor BJ, Williamson SL, Williams BJ, Szabo JS, Corbitt SL. Sonographic detection of neonatal umbilical artery thrombosis: Clinical correlation. AJR Am J Roentgenol 1987;148:965-8.  Back to cited text no. 10
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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