ISSN 2249-4847
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REVIEW ARTICLE
Vitamin D and the Neonate: An Update
Journal of Clinical Neonatology • Volume 4 • Issue 1 • January-March 2015 • Pages 1-***
Hany Aly, Hesham Abdel-Hady
1
ORIGINAL ARTICLES
The Incidence of Birthmarks in Neonates Born in Zanjan, Iran
P. Khoshnevisasl, M. Sadeghzadeh, S. Mazloomzadeh, A. Azizi Zanjani
8
Does Intubation, Surfactant, and Extubation Play a Role in Late Preterm Neonates with Respiratory Distress
Syndrome: An Observational Cohort Study
Bonny Jasani, Ruchi Nanavati, Nandkishor Kabra
13
The accuracy of Transcutaneous Bilirubin Measurements in Preterm Infants
Tariq Rushdi Mohieldeen Alsafadi, Saad Abdullah Alsaedi
18
Griffiths Mental Development Scales as a Tool for the Screening of Motor Disability in Premature Infants:
Is it Worth it?
22
Augusto Biasini, Fiorella Monti, Isabella Gianstefani, Lucia Bertozzi, Francesca Agostini, Erica Neri
A Randomized Controlled Trial of Kangaroo Mother Care Versus Conventional Method on Vital Signs and Arterial
Oxygen Saturation Rate in Newborns Who were Hospitalized in Neonatal Intensive Care Unit
Khadijeh Dehghani, Zahra Pour Movahed, Hamideh Dehghani, Khadijeh Nasiriani
26
Can Utilizing Neurally Adjusted Ventilatory Assist in the Ventilation Support of Critically Ill Neonates Results in
Shorter Hospital Stay?
32
Aiman Yassin Rahmani, Ahmad Ali Imran, Unita Boats, Fares Chedid, Stephanie Woodworth, Junaid Khan
CASE REPORTS
Renal Dysplasia-Limb Reduction Defect Syndrome
Setu Rathod, Sunil Kumar Samal, Seetesh Ghose
38
Extending Thrombus within the PDA in an Infant with Tetralogy of Fallot and Pulmonary Atresia:
An Averted Disaster
42
Srinivasa Murthy Doreswamy, Jennifer Twiss, Dragos Predescu, Sandesh Kiran Puttappa Shivananda
Goldston Syndrome: A Rare Case Report
Setu Rathod, Sunil Kumar Samal
46
Sternal Cleft Associated with Congenital Aortic Aneurysm in a Neonate
Hmami Fouzia, Hbibi Mohamed, Jaffal Mohammed, Tizniti Siham, Atmani Samir, Bouharrou Abdelhak
Acrocallosal Syndrome with Additional Features in a Neonate
Deeparaj Ganapati Hegde, Jayashree Mondkar, Swati Manerkar
49
51
Monochorionic, Diamniotic Twins with Amniotic Band Syndrome at Exactly the Same Site for each:
A Rare Amniotic Band Syndrome Presentation
Anthony L. H. Moss, Tamsin A. Burgues
54
The Presence of Adrenomegaly and Transient Hyperinsulinemic Hypoglycemia in a
Newborn with Trisomy 13: Association or Coincidence?
Osman Bastug, Mehmet Adnan Ozturk, Mehmet Sait Dogan, Sabriye Korkut, Levent Korkmaz,
Hulya Halis, Tamer Gunes, Selim Doganay
57
IMAGES IN CLINICAL NEONATOLOGY
Osteogenesis Imperfecta Type IIA
Vol 4 / Issue 1 / January-March 2015
60
Affiliated to Saudi Neonatology Society
Aliza Mittal, Binit Sureka,
Neha Bansal, Harish Chellani
cAse RepORt ›››
Extending Thrombus within the PDA in an Infant with
Tetralogy of Fallot and Pulmonary Atresia: An Averted
Disaster
Srinivasa Murthy Doreswamy, Jennifer Twiss1, Dragos Predescu2, Sandesh Kiran Puttappa Shivananda1
Clinical Fellow Neonatal and Perinatal Medicine, McMaster Children’s Hospital, Hamilton, ON, Canada and JSS Medical College, Mysore, Karnataka, India,
1
Department of Pediatrics, Division of Neonatology, HSC 4F1D, 1280 Main Street West, Hamilton ON, L8S 4K1, 2Pediatric Cardiologist, McMaster Children’s
Hospital, 1200, Main Street West, L8N 3Z5, Canada
ABSTRACT
We report a case of a premature baby, who had an antenatal diagnosis of pulmonary atresia, with a double outlet right ventricle (DORV). This
baby was managed with prostaglandin infusion to keep the ductus patent. During the initial echo, the cardiologist noted a thrombus in the thin,
long, and tortuous duct, which rapidly progressed to partially occlude the left pulmonary artery. This was treated with unfractionated heparin
and the thrombus resolved within two hours of commencing the heparin infusion. This was followed by low molecular heparin for six weeks.
Had this been missed, the pulmonary circulation would have been severely compromised and would have led to refractory cardiac failure. This
article highlights one of the possible causes of failure of prostaglandin infusion in maintaining ductal patency in duct‑dependent cardiac lesions
Key words:
Congenital heart disease, heparin, patent ductus arteriosus, prostaglandin, thrombus
INTRODUCTION
Ductus arteriosus is an essential conduit in fetal life. After
birth the duct will close, which results in connecting the
pulmonary and systemic circulation in the series. In healthy
term newborn babies, the functional closure of the duct
commences within a few hours of birth and is followed by
an anatomical closure over the next few weeks. A similar,
but delayed pattern is seen in late preterm babies. Postnatal
closure of the duct is important in an anatomically normal
heart, however, its patency is essential in duct-dependent
congenital heart lesions. This is achieved by prostaglandin
infusion.
Thrombus in Patent Ductus Arteriosus (PDA) or
pulmonary arteries is extremely rare and can lead to
pulmonary hypertension, shock, and refractory cardiac
arrest in duct-dependent congenital heart disease.[1] We
report here, a case of a preterm infant, with antenatal
diagnosis of Tetralogy of Fallot (TOF) and pulmonary
atresia, who developed a rapidly progressive thrombus in
the left pulmonary artery and ductus arteriosus. Timely
intervention with anticoagulation resulted in the quick
resolution of the thrombus and averted life-threatening
complications.
and atrial septal defect, repaired at five years of age. Her
antenatal serology was protective. Her blood sugars
were normal. She was treated for pregnancy-induced
hypertension with labetalol from 30 weeks of pregnancy.
There was no family history of thrombophilia. The fetal
echocardiogram at 19 weeks of gestation confirmed
TOF with double outlet right ventricle (DORV)-type of
ventriculoarterial connection and pulmonary atresia.
The infant was delivered at 34 + 3/7 weeks gestation by an
emergency Cesarean section on account of fetal distress.
She was vigorous at birth and had APGAR scores of 6 and 8,
at one and five minutes, respectively. She did not need any
resuscitation. Her birth weight was 1770 grams. The cord
arterial blood gas was normal. She was cyanotic with
pre- and postductal saturations of 73 and 76%, respectively.
Address for correspondence:
Dr. Srinivasa Murthy Doreswamy,
1001, Main Street West, Apt. 1004,
Hamilton, ON, Canada, L8S 1A9.
E‑mail: drdsrinivasa@gmail.com
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CASE REPORT
DOI:
Our patient was a female infant, born to a 28-year-old
primigravida, with a past medical history of hypothyroidism
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10.4103/2249‑4847.151167
Journal of Clinical Neonatology | Vol. 4 | Issue 1 | January-March 2015
Doreswamy, et al.: PDA thrombus in tetralogy of fallot
Her cardiovascular examination revealed normal S1, single
S2, and a grade three out of six continuous murmur at the left
upper sternal border. The rest of the physical examination
was unremarkable.
At one hour of age she received low flow oxygen at 200
ml/minute due to decreasing oxygen saturation (SpO2).
Infusion of prostaglandin E1 (PGE1) was started at
a dose of 0.05 mcg/kg/minute and later increased to
0.1 mcg/kg/minute, to attain an SpO2 between 80 and
85%. Umbilical arterial and venous catheters were placed
and the tip position was confirmed on x-ray. She had to be
intubated due to apnea following prostaglandin infusion.
An echocardiogram (ECHO) was performed by the
attending cardiologist at two hours of age, which confirmed
the antenatal diagnosis. Ductus arteriosus was tortuous,
thin, and long. During the course of the ECHO, a thrombus
was noted in the left pulmonary artery partially occluding
its lumen, which rapidly enlarged and extended into the
ductus arteriosus [Figures 1 and 2].
pulmonary artery and ductus arteriosus thrombosis in
a neonate with duct-dependent congenital heart lesion.
Occlusion of the ductus arteriosus by the thrombus would
have been catastrophic in this setting.[1-3] Thrombus in the
duct or pulmonary artery can easily be missed on clinical
examination or by ECHO unless there is a high index of
suspicion.[2,3]
After confirming the absence of an intracranial bleed and
a normal coagulation profile, a bolus of unfractionated
Heparin 75 units/kg was given followed by 28 units/kg/hour.
as an infusion. Hematocrit was 55%. Thrombophilia workup
was not done as she was already receiving heparin through
the umbilical arterial line. A repeat ECHO done at four hours
of life and, one hour after initiation of heparin infusion,
demonstrated complete resolution of the thrombus from
both the duct and left pulmonary artery [Figure 3]. Heparin
infusion was discontinued and low molecular weight
heparin (enoxaparin) was commenced to maintain the
anti-Xa levels between 0.2 and 0.4 (prophylactic regimen).
Enoxaparin was continued for six weeks, without any
complication. No recurrence of thrombus was noted in the
ECHO after two weeks of discontinuation of enoxaparin.
The infant underwent a surgical repair at eight weeks and
was discharged home at 10 weeks of life.
There are case reports of thrombus in the ductus arteriosus and
main pulmonary artery or its branches by echocardiography
in term newborns presented with hypoxemia, respiratory
distress, cyanosis, and systolic murmur. Three of these babies
needed surgical removal of the thrombus, as anticoagulant
management failed. One of them was successfully managed
with low molecular weight heparin and one on long-term
aspirin.[4-7] None of these babies had congenital heart disease,
thrombotic diseases, or central lines. Thrombus in ductus and
pulmonary arteries in term infants, presenting as congenital
heart disease needing surgical thrombectomy has been
reported.[4,6,7] None of them had any identifiable risk factors
for thrombosis. Gen Niwayana[8] has reported an autopsy
series of six babies who had thrombus in the ductus and
pulmonary artery, who failed to respond to both medical and
surgical treatments. Monica et al., have argued that premature
closure of the duct in the face of an immature coagulation
system in newborns might tilt the balance toward formation
of the thrombus in the closing duct and its extension into
the pulmonary artery.[5] The ductus arteriosus in babies
with pulmonary atresia is thin and tortuous. This causes a
turbulent blood flow. Experimental evidences suggest that
turbulence in blood flow can predispose to thrombosis.[9] The
histological tissue characteristics are also altered in infants
receiving PGE1.[10] Premature closure of the duct, immature
coagulation system in newborns, tortuous ductus arteriosus
with turbulent flow in the setting of pulmonary atresia,
and the presence of vascular catheters, might have all been
involved in the mechanism of thrombus formation and rapid
progression in our case.
Discussion: The exact incidence of neonatal pulmonary
artery thrombosis is not known and to the best of our
knowledge, this is the first case report of a rapidly progressive
Therapeutic options: Anticoagulation, thrombolytics, and
surgical thrombectomy are the therapeutic options available
in term and preterm neonates.[11,12] Surgical thrombectomy
Figure 1: Echocardiogram showing growing thrombus in the duct and LPA
Journal of Clinical Neonatology | Vol. 4 | Issue 1 | January-March 2015
43
Doreswamy, et al.: PDA thrombus in tetralogy of fallot
Figure 2: Echocardiogram showing turbulence in blood flow due to
the thrombus
is indicated if medical therapy fails[13,14] and carries a higher
risk of complications.
Unfractionated Heparin and Low molecular weight
Heparin (Enoxaparin) alter the prothrombotic status
in the infant and prevent progression of the thrombus.
Unfractionated heparin molecules have higher binding sites
and initiate anticoagulation more rapidly than low molecular
weight heparin. In acute life-threatening situations,
unfractionated heparin may have an advantage.[15] In our
case we have used unfractionated Heparin in the initial
phase of treatment, due to its rapidity of action and good
safety profile. With Enoxaparin therapeutic levels may be
difficult to achieve in a limited time frame.[16] However, it
has a predictable and sustained action in the long term and it
needs less frequent monitoring of the coagulation status.[17]
We initiated it as soon as resolution of the thrombus was
demonstrated. We did not the use the recombinant tissue
plasminogen activator (r-TPA) as first-line intervention,
because of the lower safety profile in the premature infant.
Left pulmonary artery (LPA) coarctation due to closing
ductus arteriosus is a dangerous complication and a cause
of LPA occlusion in babies with TOF with pulmonary
atresia.[18,19] This case suggests that thrombosis of the
LPA may be an additional mechanism for LPA occlusion
that needs to be considered in patients deteriorating on
prostaglandin infusion.
In our case, if an ECHO had not been conducted in time, the
clinical condition would have only deteriorated and ended
up in an increased dose of prostaglandins and respiratory
support, without much benefit.
Figure 3: An echocardiogram done two hours after commencing
Heparin shows dissolution of the thrombus
•
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CONCLUSIONS
•
44
Thrombus formation in the branch pulmonary arteries
or ductus arteriosus may be a cause of persistent or
acute desaturation in babies with duct-dependent
cardiac lesions on prostaglandin infusion. Rapid access
to echocardiography is of immense benefit in this
setting
Initial therapy with unfractionated Heparin may be
beneficial while getting organized for other treatments
like recombinant tissue plasminogen activator and
surgery.
Journal of Clinical Neonatology | Vol. 4 | Issue 1 | January-March 2015
Doreswamy, et al.: PDA thrombus in tetralogy of fallot
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Hirsh J, Anand SS, Jonathan L. Halperin JL, Fuster V. Mechanism
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How to cite this article: Doreswamy SM, Twiss J, Predescu D,
Shivananda SP. Extending thrombus within the PDA in an infant
with tetralogy of Fallot and pulmonary atresia: An averted disaster.
J Clin Neonatol 2015;4:42‑5.
Source of Support: Nil, Conflict of Interest: None declared.
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