Conducting defect with atrial septal aneurysm in a preterm neonate A case report from resource limited setting.html

Niger J Paed 2015; 42 (1): 64 – 67

CASE REPORT

Odetunde OI

Conducting defect with atrial

Ekwochi U

septal aneurysm in a preterm

Chinawa JM

Ejim EC

neonate: A case report from

Ezugwu FO

resource limited setting

DOI:http://dx.doi.org/10.4314/njp.v42i1,14

Accepted: 7th August 2014

Abstract:

Conducting

defect

at around the first year of life but

(CD) co-existing with interatrial

the associated heart electrical con-

Odetunde OI

(

)

septal anomaly (ISA) like Atrial

ducting system defects persisted.

Chinawa JM

Septal Aneurysm (ASA) is a rare

The report also emphasizes the

Department of Paediatrics,

congenital heart disease present-

challenges of managing a rare neo-

Ejim EC

ing with bradyarrhythmia in-utero

natal cardiac condition in a

Department of Medicine,

due to defects in the heart electri-

resource limited setting and the

University of Nigeria Teaching

cal conducting system. We report

importance of thorough foetal bio-

Hospital, Ituku Ozalla, Enugu,

a case of a preterm female

physical profile and early interven-

Enugu State,

‘CC’ (GA=35weeks) low birth

tional delivery when it is neces-

Nigeria.

weight (2.4kg) neonate with CD

sary.

Email: odetoyintola@yahoo.co.uk

and associated ASA. CC’s mother

presented with poor foetal move-

Key words: Conducting defect;

Ekwochi U

Department of Paediatrics,

ments and bradyarrhythmia and

neonatal; Atrial Septal Aneurysm.

had an emergency caesarian sec-

Ezugwu FO

tion at 35weeks with Apgar score

Department of Obstetrics and

of 3, 5. She was managed for peri-

Gynaecology,

natal Asphyxia. This report high-

Enugu State University Teaching

lights the fact that ASA resolved

Hospital, Enugu,

Nigeria.

Introduction

will entail surgical insertion of a permanent pacemak-

er . Access to this treatment option in our economy is

3,8

Conducting defect (CD) is a cardiac conduction disorder

strongly limited by cost and dearth of such service pro-

in which there is dissociation in the conduction of im-

viders.

pulse from the sino-atrial (SA) node to the atrio-

ventricular (AV) node . This may also result in dissocia-

However, in the absence of the above conditions, patient

tion between the atrial activity (P-wave) and the ven-

with CD could generally remain asymptomatic and may

tricular activity (QRS complex) .

need no active treatment.

This is a case report of CD in a female preterm, low

It is a disorder occurring in about 1in 22000 live births .

birth weight neonate with associated ASA that spontane-

It has a high morbidity and mortality rate especially

ously resolved at End of first year of life. Her heart rate

when associated with structural defects, common among

has persistently remained within the range of 60-70bpm.

which are; interatrial septal anomaly (ISA) like Atrial

She has remained generally asymptomatic and has

Septal Aneurysm (ASA), levo-transposition of great

gained weight progressively without insertion of a pace-

vessels, left atrial isomerism . The prevalence of ASA

maker.

has been reported high in the preterm neonates with high

We present this case because of its rarity and to high-

resolution rate at the end of first year of life . Although

4,4

light CD as a cause of foetal poor biophysical profile

ASA is considered to a benign and transient observation,

and peri natal asphyxia. A need for urgent intervention

reports have shown that it could be a cause of foetal

perinatally, further investigations and follow-up of such

atrial arrhythmia and impulse conduction disorders in

baby may be necessary.

adults

6,7.

Case report

In the presence of a co-existing structural cardiac defects

or very low heart rate in the tune of <55bpm, CD can be

We present a preterm female baby ‘’ CC’’ whose mother

complicated by low cardiac output, congestive cardiac

complained of poor fetal movements at a gestational age

failure and death. Treatment of choice in such condition

of 35weeks and obstetric ultrasonographic scan at same

period revealed a poor biophysical profile with a foetal

feed(3 houlry). The heart rate was 64bpm regular and

heart rate of 90bpm. Following the above findings, baby

she remained stable. Echocardiography was done to rule

was delivered via an emergency caesarean section. The

out presence of any congenital structural heart defect.

APGAR score at birth was three in the first minute and

Mother was also screened for the presence antinuclear

five in the fifth minute. The liquor was clear. Relevant

antibody (ANA) against systemic lupus erythematosis

examination findings at birth include; heart rate (HR) of

(SLE). Mother’s serological screening tests were nega-

112bpm regular, core temperature of 35.6 c with acro-

tive for ANA,

cyanosis, grunting respiration (Respiratory Rate (RR)

=62cpm). The anterior fontanelle was patent and nor-

ANCA and anti ds Nase

motensive. There was poor motor activity and weak cry

On the 12 day of life, baby was weaned off incubator,

on stimulation. Moro reflex was incomplete and other

primitive reflexes depressed. Other systemic findings

HR was 68/min, tolerate 35mls of EBM per feed. Direct

were unremarkable. The birth weight was 2.4kg. The

suckling was commenced. 2-D-echo cardiogram re-

diagnosis at birth was low birth weight, prematurity with

vealed ASA protruding from the midline of the atrial

moderate perinatal asphyxia.

septum to the left atrium throughout the cardiorespira-

tory cycle (a type 2L ASA) (Figure 3). Two days later

Initial resuscitation was done by drying and provision of

she was discharged home and to be followed-up on

warmth, suctioning to clear the air way and intra nasal

regular appointment days visit in the clinic.

oxygen therapy commenced at 1L/min. she was then

Fig 3: Patient’s Echocardiography done on the 10 day of life

transferred to the incubator set at a real temperature of

36 c. Other treatments offered include; intravenous fluid

(Trans-thoracic Two-D-echocardiogram) revealed ASA pro-

10% dextrose in water, intravenous dexamethasone,

trudes from the midline of the atrial septum to the left atrium

intramuscular

vitamin

intravenous

ceftazidime

throughout the cardiorespiratory cycle (a type 2L ASA)

(antibiotic prophylaxis)

By the 3 day of life, the HR decreased to 70bpm, oxy-

gen saturation stabilizes at 94% without oxygen, baby

has passed meconium, grunting respiration resolved, and

suckling reflex was stronger. A diagnosis of bradyar-

rhythmia was made and she was to be investigated to

determine the cause. Myocardial dysfunction secondary

to perinatal asphyxia was considered as the cause and to

rule out presence of congenital heart block. Electrocar-

diogram (ECG) was done (with improvised available

adult leads) (Figure 1) and a diagnosis of conducting

Her appointments were kept regularly and by last follow

defect (2:1 degree block) with dissociation between the

-up visit at 1year old of age, patient has done well with a

atrial activity (P-wave) and the ventricular activity (QRS

weight of 10kg. Heart rate was 60bpm. At the end of

complex) (Figure 2).

first year of life, a follow-up Trans –thoracic echocardi-

ography (TTE) was done and revealed disappearance of

Fig 1: The ECG revealed complete dissociation between the

the ASA (Figure 4). Baby is still on follow-up and keep-

atrial activity (P-wave) and the ventricular activity (QRS com-

ing appointments and the need for pacemaker device has

plex)

been discussed and prompted.

Fig 4: Patient’s Echocardiography at the end of first year of

life showing resolution of the Atrial Septal Aneurysm.

Fig 2: Patient with improvised adult ECG Leads

Discussion

There are many evidences associating Atrial Septal An-

eurysm (ASA) and cardiac impulse conduction disorder

Baby continued to improve on admission and by the 9

6,7

. Several reports have shown possible association of

Right Bundle Branch Block (RBBB) and ASA

6,7

day of life; tolerates 15mls of expressed breast milk per

but few

or no data in medical literature have reported association

Significant pauses on 24-hour ambulatory electrographic

between ASA and Congenital Conducting defect (CD).

monitoring may also be an indication for putting in a

pacemaker. Some studies have suggested that a pro-

CD is estimated to occur in one of every 22000 live

longed QTC interval or a wide QRS escape rhythm with

births with 25% to 33% of cases associated with other

complex ventricular ectopic may warrant urgent inter-

vention . Our patient did not have any of these findings.

congenital heart diseases . This rarity is also confirmed

in this case, since this is the first time it is been recorded

in our setting. The prevalence of ASA is said to be high

The use of permanent pacemakers as initial pacing mo-

among preterm neonate with high resolution rate at the

dality or use of temporary epicardial pacing wires in the

end of first year of life without any complication . The

high-risk neonate with CD is a definitive surgical treat-

index patient is premature baby and the Trans –thoracic

ment. Due to lack of facility, our patient had no pacing,

echocardiography (TTE) done shortly after birth re-

more so she survived without much intervention, she is

vealed type 2L ASA which resolved at end of one year

one year old now and had no complication. Medical

management involves the use of steroid and atropine .

of patient’s age with persistent of the CD. Historically,

CD was thought to be a relatively benign condition, with

Atropine therapy enhances conduction through the AVN

the only significant morbidity being the need for pace-

by reducing vagal tone via receptor blockade. It also

maker implantation to guard against the risk of Stokes-

improves the ventricular rate if the site of block is in the

AVN . In some cases patient may be asymptomatic and

Adams attacks and sudden cardiac death . This is also

in tandem with our case where the patient was relatively

needs no intervention. This is akin to our patient. We

stable with a low heart rate.

use dexamethasone in our patient as a prophylaxis for

chronic lung disease owing to the gestational age at de-

Identified risk factors in CD include the presence of

livery. It is noted that early postnatal dexamethasone

hydrops , low ventricular rate (usually defined as <55

therapy has been used recently for the possible preven-

beats/min), and prematurity . Our patient although pre-

tion of chronic lung disease (CLD) in preterm infants

with a very good outcome . More so, the use of dexa-

term, never had a recorded heart rate of < 55beats /min.

methasone has been noted to be beneficial to children

A study of American Lupus Registry showed 113 chil-

with Congenital heart block. For instance, it is docu-

dren with CD in which 85 of them were diagnosed in

mented that steroid given as 0.1 mg per kilogram to

utero . The mortality was 19%, with most deaths occur-

newborns with congenital heart block reduces mortality

and morbidity .

ring in the neonatal period or in-utero. The only risk

factor for death was delivery at <34 weeks gestation age.

Our patient was 35 weeks gestation age and this may

explain why she survived. It is important to note that

some maternal autoimmune disease such as SLE

Conclusion

(systemic lupus erythromatosis) may predispose foetus

to CD due to auto antibodies (anti-Ro antibodies).

Conducting defect especially with ASA is rare, and

Women with serum titers of anti-Ro antibody carry a

when coexisting, the ASA could still runs the natural

3% risk of having a child with neonatal lupus syndrome.

course of resolution at the end of first year of life and

If she has a prior experience with affected foetuses, her

the fact remains that in the absence of other coexisting

risk rises to about 18%

. The mechanism of causation

cardiac anomaly or very low heart rate, patients with CD

of neonatal lupus is not completely understood but evi-

could thrive without a pacemaker device in the neonatal

dence points to the foetus beginning life with a normal

and early infant age.

cardiac structure and conducting system. Providentially,

the mother of this patient was screened for lupus anti-

Conflict of Interests: None

bodies and she was negative.

Funding: None

Eronen et al reported the results of 91 infants with CD

(83 diagnosed in utero) . The mortality was 16%, with

most deaths occurring in infancy. The risk factors for the

Acknowledgement

mortality are low birth weight, male sex, and complica-

tions from prematurity or neonatal lupus . Our patient is

We wish to acknowledge the medical and nursing staff

a female child with borderline low birth weight. Again

Neonatal Special Care Unit of St Patrick’s Hospital,

this might also explain the factor why our index patient

Owerri Road, Enugu, Nigeria where the baby was

survived beyond the neonatal period .

delivered and primarily managed.

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