CONGENITAL COMPLETE HEART BLOCK IN INFANT

WITH PATENT DUCTUS ARTERIOSUS AND SECUNDUM

ATRIAL SEPTAL DEFECT

Case Report

Oleh :
Audylia Hartono, dr.

Pembimbing :
Prof. Dr. Teddy Ontoseno, dr, SpA(K), SpJP, FIHA
Dr. Mahrus A. Rahman, dr, SpA(K)
Dr. Alit Utamayasa, dr., SpA(K)
Taufiq Hidayat, dr., SpA(K)

Departemen Ilmu Kesehatan Anak

Fakultas Kedokteran Universitas Airlangga
RSUD Dr. Soetomo
Surabaya
2021
 Lembar Pengesahan Makalah Ilmiah

CONGENITAL COMPLETE HEART BLOCK IN INFANT

WITH PATENT DUCTUS ARTERIOSUS AND SECUNDUM
ATRIAL SEPTAL DEFECT

CASE REPORT

Oleh
Audylia Hartono, dr.

..............................................................

Dilakukan dan dipresentasikan serta merupakan hak milik

Departemen Ilmu Kesehatan Anak
Fakultas Kedokteran Universitas Airlangga
RSUD Dr.Soetomo
Sebagai salah satu syarat untuk memperoleh gelar
Dokter Spesialis Anak

Telah disetujui untuk dipresentasikan oleh

PEMBIMBING
Surabaya, 7 Juli 2021

Nama Tanda Tangan

Prof. Dr. Teddy Ontoseno, dr, SpA(K), SpJP, FIHA ..............................

Dr. Mahrus A. Rahman, dr, SpA(K) .......... ....................

Dr. Alit Utamayasa, dr., SpA(K) ........... ...................

Taufiq Hidayat, dr., SpA(K) ...............................

i
INTRODUCTION

Complete Heart Block (CHB) is characterized by interference transfer of the

electrical nerve conduction that regulate the normal and rhythmic pumping action of
the heart muscle.1 Heart block is defined as congenital if diagnosed in utero, at birth,
or within the first month of life, whereas childhood heart block is diagnosed between
the first month and the 18th year of life. As a result of several different etiologies,
congenital and childhood heart block may occur in an entirely structurally normal heart
or in association with concomitant congenital heart disease.2 Individuals with complete
heart block may experience episodes of syncope, breathlessness, lethargy, hypotension.
In addition, complete heart block may be associated with congestive heart failure, chest
pain, palpitations, episodes of dizziness with or without loss of consciousness due to
heart fluttering (fibrillation) or cessation (asystole) of the heart (Stokes-Adams
attacks), and sudden death.1
Congenital heart block is a rare disorder that appears to affect males and
females in equal numbers. The estimated prevalence of congenital heart block is one
per 15,000–20,000 live births.2 The incidence of complete (third degree) congenital
heart block is one in approximately 20,000 to 25,000 live births. Over half the cases of
congenital heart block (60-90%) are associated with an autoimmune disorder in the
affected individual’s mother such as systemic lupus erythematosus or Sjogren’s
syndrome.1 Left untreated, congenital heart block is associated with a fetal and neonatal
mortality ranging between 14 and 34 %.2
The prognosis of patients with congenital heart block depends largely on the
following factors: presence or absence of underlying structural heart disease, rate of
ventricular activation and presence or absence of congestive heart failure. Specific risk
factors associated with in utero mortality include fetal hydrops, diagnosis of CHB at
<20 weeks, ventricular escape rate < 55 bpm, and impaired left ventricular function.
Fetal echocardiography is the standard tool for diagnosis and management of fetal
bradyarrhythmia. Diagnosing progression to CCHB is challenging due to its sudden
and unpredictable progression that serial fetal echocardiography has been shown to be

1
an imperfect tool even with weekly screening. Another promising approach to
diagnosing CCHB is institution of a home monitoring program in which the mother is
trained to measure fetal heart rate twice a day using a simple handheld Doppler system.
Corticosteroids are often initiated when a fetus develops first or second degree block
in attempts to prevent progression to CCHB. For newborns and children with CHB, the
main therapy is insertion of a pacemaker in order to prevent from sudden cardiac
death.2 Patients with CHB are usually unresponsive to atropine or other
pharmacological therapy. Therefore, early detection of congenital heart block and more
aggressive approach toward pacemaker treatment are the most important prognostic
factor in individual with congenital heart block.1
The objective of this case report is to present a case of infant with congenital
complete heart block focusing early detection and referral management of the patient.

2
CASE PRESENTATION

A four-month-old baby girl was referred to Dr. Soetomo General Hospital from
Bhakti Dharma Husada General Hospital, with a chief complaint of lethargy. The
patient was diagnosed with complete heart block from the previous hospital and
referred for consideration pacemaker insertion.
The patient came to Bhakti Dharma Husada General Hospital with a chief
complaint of lethargy and slow heart beat. Three days before admitted the patient had
remittent fever with the highest temperature of 39oC. The fever was accompanied by
cough, but there was no dyspnea, chest pain, period of dizziness, or syncope. The
abnormal slow heart rate was noticed by the physician from the previous hospital. After
receiving initial treatment for infection, the heart rate remained slow so the patient was
referred for further pacemaker management at Dr. Soetomo General Hospital.
From further history taking, the patient is the fourth child from the parents.
During the pregnancy, from previous antenatal examination, the fetal heart beat was
found to be within normal limits. She was born by cesarean with indication of the
mother with a history of cesarean delivery in the previous pregnancy. The patient was
born at term, but soon after birth, the patient does not cry immediately. The initial
resuscitation steps are taken, after which the patient cried and was allowed to be
discharged three days after birth.
The patient had never been diagnosed with a heart disorder before. However,
the history taking shows that the patient was not strong enough to breastfeed for a long
duration, and often appears breathless during the feeding.
From further history taking of family history, the patient's mother was never
diagnosed with an autoimmune disorder. The patient's mother never experienced
symptoms such as joint pain, erythema rash on the face or other location, oral ulcer,
hair loss, or other autoimmune symptoms. From the history of pregnancy, it was found
that the patient's mother was healthy during pregnancy, had never suffered from illness,
routine antenatal care visit to the midwife, and it was said that the baby's heartbeat was
within normal limits. Both parents and three of the patient's siblings were never

3
diagnosed with a heart disorder. There were no abnormalities of the upper limb from
both of the parents and patient's siblings.
The patient received exclusive breastfeeding from birth until two months of
age, then continued with formula until this time. The patient drinks with sufficient
frequency but had not enough endurance to suck for a long duration of time and often
seems to stop and had some shortness of breath. The patient's birth weight was 3000g
and birth length was 52cm. At 1-month-old, the patient's body weight was 3900g, at
the age of 2-months-old, the patient's body weight was 4200g, at the age of 3-months-
old, the patient's body weight was 4400g, and now at the age of 4-months-old, during
the admission of the hospital the patient's body weight was 4500g with a body length
of 62 cm.
The patient is already capable to turn the face toward the voices, gives social
smile, put her hand in her mouth, roll over the body, but the patient is not capable to
raise his head and chest when lying on his stomach yet.
She got hepatitis B vaccine once after birth, BCG once with scar on upper right
arm, DPT and Hepatitis B one time, and oral polio one time.
The physical examination was done at Emergency Department Dr. Soetomo
General Hospital, which revealed an infant with bodyweight 4.5 kilograms, a length of
94 cm. The growth chart (z-score) showed that the body weight and height for a 4-
month-old baby girl was underweight and severely wasted, but the body length and
head circumference were within normal limit. (Figure-1,2,3).
She was fully alert with GCS 456, had round isochoric pupils 3mm/3mm,
positive light reflexes on both eyes. The blood pressure was 85/50 mmHg, the heart
rate was 52 beats per minute irregular, respiratory rate was 34 times per minute
adequately, axillary temperature 37oC, and the oxygen saturation was 99%. On
physical examination, the patient was not anemic, not cyanotic, no dyspnea. There was
no lymph node enlargement. There was no dysmorphism of the face.

4
Figure 1 WHO growth chart showed weight for age z-score was below -2 SD area.

Figure 2 WHO growth chart showed length for age z-score was median. 5
 Figure 3 WHO growth chart showed weight for length z-score was under -3SD area.

6
Figure 4 WHO growth chart showed head circumference for age z-score was below -1SD area. area.
 Chest physical examination showed symmetrical chest wall movement, no
retraction was observed. There were continuous murmurs with a punctum maximum
on the left subclavian region. There were no crackles and no wheezing from pulmonary
auscultation.
The abdomen was flat, bowel sound was normal, the liver was palpable (1x1x1
cm), along with a sharp edge, normal consistency, and no tenderness. There was no
enlargement of the spleen. Skin turgor was normal. The upper and lower extremities
had sufficient perfusion with a capillary refill time of fewer than two seconds and no
cyanosis nor clubbing finger were found.
The electrocardiography of the patient from Bhakti Dharma Husada Hospital
showed third degree/total heart block, which is characterized by the atria and ventricles
beat independently of one another and are not in sync, there were regular PP intervals,
with a rate comparable to the normal heart rate for the patient’s age (148 beats/minute).
The QRS complexes also are regular (regular R-R interval), with a rate much slower
than the P rate (53beats/minutes). In order to find the cause of heart block in the patient,
ECG examination was done on both parents of the patient in the previous hospital. The
results of the ECG of both the parents were within normal limits.

Figure 5 Patient’s ECG third degree/ total heart block.

7
 The echocardiography examination was conducted in the previous hospital,
revealed moderate PDA (0.7cm) left to right shunt, moderate secundum ASD left to
right shunt (0.5cm), and mild TR. The ejection fraction was 72%.
Based on history taking, clinical manifestation, physical examination, and
radiologic findings, the patient was diagnosed as congenital complete heart block +
moderate PDA left to right shunt + moderate secundum ASD left to right shunt +
underweight + severely wasted and was planned to have urgent temporary pacemaker
insertion. The patient also received pharmacological therapy that consists of lisinopril,
furosemide, and dopamine syringe pump before the patient was placed in a temporary
pacemaker.

Figure 6 Patient’s echocardiography showed moderate secundum ASD left to right shunt (0.5cm) (a),
moderate PDA (0.7cm) left to right shunt (b).

8
 Figure 7 The insertion of transcatheter temporary pacemaker

Seven days after insertion of the temporary pacemaker, the patient underwent a
sternotomy for PDA ligation and permanent pacemaker insertion. The operation went
smoothly, with minimal bleeding.

Figure 8 PDA ligation process

Post-operation echocardiography evaluation showed no residual flow from

PDA, and no effusion in either the pericardium or pleural space. One day after surgery,
the chest drain was removed, the patient was admitted to the pediatric intensive care
unit for two days using nasal oxygen support. The electrocardiography showed regular

9
heart beat between 90 -110 beat per minute. The patient moved to low care unit on the
third-day post-operation.

a b

c
Figure 9 (a) post-operation chest X-ray. (b) post-operation ECG (c) patient clinical presentation

10
DISCUSSION

This case presented A four-month-old baby girl with a chief complaint of

lethargy and slow heartbeat, diagnosed with complete heart block, who was referred
for consideration pacemaker insertion. The presentation of congenital heart block is not
specific and varies with age of onset, underlying etiology, and type of heart block. In
newborns affected by CHB, the primary finding is a slow heart rate (bradycardia).
Some patients may also appear pale or diaphoretic, have intermittent gallops and
murmurs, and show signs of congestive heart failure (e.g. crackles in lungs, peripheral
edema, etc.). Individuals with complete heart block may experience episodes of
unconsciousness (syncope), breathlessness, lack of energy (lethargy), and/or low blood
pressure (hypotension). In addition, complete heart block may be associated with an
impaired ability of the heart to pump blood effectively (congestive heart failure); chest
pain and/or palpitations; episodes of dizziness with or without loss of consciousness
due to heart fluttering (fibrillation) or cessation (asystole) of the heart (Stokes-Adams
attacks); and/or enlargement of the heart (cardiomegaly). In rare cases, infants born
with complete heart block may have abnormal accumulation of fluid within tissues of
the body (hydrops fetalis). 1
The abnormal slow heart rate was noticed by the physician from the previous
hospital, accompanied with lethargy and shortness of breath that matched with the
clinical presentation of children with complete heart block. Based on clinical
presentation of the patient, an electrocardiography examination was performed in this
patient.
Atrioventricular block (AVB), is characterized by the interference of the transfer
of the electrical nerve impulses (conduction) that regulate the normal and rhythmic
pumping action of the heart muscle. Heart block is defined as congenital if diagnosed
in utero, at birth, or within the first month of life, whereas childhood heart block is
diagnosed between the first month and the 18th year of life. The severity of such
conduction abnormalities varies among affected individuals.2

11
 Heart blocks are categorized according to the degree of impairment of the patient
and the pattern of conduction abnormality. The categories are first, second, and third
degree heart block. Second degree heart block is further characterized into two types:
Mobitz type I and Mobitz type II. In first degree heart block, the PR interval is
prolonged beyond the upper limits of normal for the patient’s age and heart rate. Second
degree heart block is characterized by dropped or skipped beats. In Mobitz type I AV
block, the PR interval becomes progressively prolonged until one QRS complex is
dropped completely. Mobitz type II AV block is characterized by The AV conduction
is “all or none.” AV conduction is either normal or completely blocked.3
Third-degree heart block is widely known as complete heart block (CHB). The
diagnosis criteria include the presence of complete atrioventricular (AV) dissociation
with the atrial rate being higher than the ventricular rate. The atria and ventricles
depolarize independently of each other, and the ventricular rate is typically slower than
the atrial rate. The surface electrocardiograph (ECG) may reveal either narrow or wide
QRS configurations, depending on the location of the interruption and the foci of the
escape pacemaker tissue. For example, the QRS is narrow if the escape rhythm is a
supra-His bundle, or it is wide if the escape rhythm is intra-His or infra-His bundle.
Escape ventricular rhythms with narrow QRS may be more stable than those with wide
QRS morphologies.3,4

12
 Figure 10 The degree of Atrioventricular heart block

Sumber : Park MK, Park S. Park’s Pediatric Cardiology For Practitioners. seventh ed. Park MK, Salamat
M, editors. Philadelphia: Elsevier Inc; 2021. 335–337 p.

In this case, the patient presented with symptoms of lethargy at the age of four
months old. The patient had symptoms of fever three days before the onset of
symptomatic bradycardia. On echocardiography examination, there was a moderate
PDA and a moderate secundum ASD. In the previous medical history, there were no
symptoms like syncope, dizziness, or dyspnea before. Therefore, the cause of heart
block, in this case, can still be caused by various causes. (Figure 11)
About 60-90 percent of cases of congenital heart block are associated with an
autoimmune disorder that affected an individual’s mother such as systemic lupus
erythematosus or Sjogren’s syndrome. The transplacental passage of maternal anti-
Ro/SSA and/or anti-La/SSB autoantibodies, entering the fetal circulation, can directly
bind L-type calcium channels on fetal cardiomyocytes and significantly, but reversibly,
inhibit the related currents. If the process is not stopped in this phase, the inflammatory
damage proceeds, thus eventually resulting in fibrosis and calcification of the cardiac
conduction system.5

13
 Immune-mediated
Congenital Heart Block

Inherited Progressive
Structurally normal
Cardiac Conduction
heart
Disease (PCCD)

Associated with
congenital heart Idiopathic
Atrioventricular disease
conduction disorders
Postoperative

Associatied with
acquired heart disease

Figure 11 Etiology of atrioventricular conduction disorders in children

Sumber : Baruteau AE, Pass RH, Thambo JB, Behaghel A, Le Pennec S, Perdreau E, et al. Congenital
and childhood atrioventricular blocks: pathophysiology and contemporary management. Eur J Pediatr.
2016;175:1235–48.

Autoimmune heart block is typical of the third degree and begins in utero, while
heart block due to other causes tends to present after birth and maybe first, second, or
third-degree. Fetal echocardiography is the gold standard for the diagnosis of
congenital heart block. Complete fetal heart block develops during gestational weeks
16 to 24, although a later onset of the phenomenon up to gestational week 34 has been
described.2 Diagnosing progression to CCHB in at-risk pregnancies is challenging due
to its sudden and unpredictable progression from normal sinus rhythm to first degree
heart block and then further to CCHB. It is due to this rapid conversion to CCHB that
serial fetal echocardiography has been shown to be an imperfect tool even with weekly
screening. Another promising approach to diagnosing CCHB is institution of a home
monitoring program in which the mother is trained to measure fetal heart rate twice a
day using a simple handheld Doppler system. These studies have been shown to be
feasible with up to 87% of patients completing the monitoring protocol.

14
 The mainstay of pharmacological management in CCHB is corticosteroids,
specifically fluorinated glucocorticoids such as dexamethasone or betamethasone,
which are anti-inflammatory drugs with the ability to cross the placenta.
Corticosteroids may reduce immune-mediated damage to the AV conduction tissue and
myocardial tissue that leads to myocarditis and cardiomyopathy in CCHB.
Corticosteroids are often initiated when a fetus develops first or second degree block
in attempts to prevent progression to CCHB, though there is limited evidence for
regression or resolution of heart block in these populations. Once a fetus has developed
CCHB, it is generally found to be irreversible. Thus, pharmacological therapy for
CCHB is aimed at limiting further immune mediated damage through the use of
corticosteroids, and at times augmenting cardiac output via beta stimulation of heart
rate. A recent meta-analysis comparing data from 8 studies for a total of 162 fetuses
found no difference in mortality or pacemaker implantation in steroid-treated versus
nontreated patients.6,7
In this case, from the family history, the patient's mother was never diagnosed
with an autoimmune disorder. The patient's mother never experienced symptoms such
as arthritis, malar rash, oral ulcer, hair loss, or other autoimmune or connective tissue
disorder symptoms. From the history of pregnancy, it was found that the patient's
mother was healthy during pregnancy, went to routine antenatal care visits to the
midwife, and the fetal heartbeat was within normal limits during the pregnancy.
Some isolated heart blocks diagnosed beyond the neonatal period could also are
immune-mediated, even with late detection of maternal anti-Ro/SSA autoantibodies.
This condition, emerging in childhood or even in adult age, represents a late
progressive congenital form of immune heart block with the late development of a
subclinical anti-Ro/SSA-induced congenital damage of the conduction system. In the
large majority of the cases, heart block occurs in fetuses of healthy, silently anti-
Ro/SSA antibodies-carrying mothers. The diagnosis of mothers’ seropositivity is thus
usually made only after congenital heart block detection.2
Although the clinical course of total heart block in this patient did not match the
characteristics of immune-mediated congenital heart block, it is still could not be ruled

15
out as the etiology of heart block because the maternal anti-Ro/SSA and/or anti-La/SSB
autoantibodies examination still not available yet.
The second differential diagnosis is inherited progressive cardiac conduction
disease (PCCD) is diagnosed in patients less than 50 years of age with an unexplained
progressive conduction abnormality but with an otherwise structurally normal heart,
especially if there is a family history of PCCD. Inherited PCCD in structurally normal
hearts presents as a primary electrical disease and has been linked to genetic variants
in the ion channel genes SCN5A, SCN1B, SCN10A, TRPM4, and KCNK17 as well as
in genes coding for cardiac connexin proteins.4
In this case, there was no history of heart disease in both of the parents, no history
of sudden death, syncope, or congestive heart failure in her family. Then the inherited
heart block diagnosis can be ruled out.
The third differential diagnosis was congenital heart disease associated heart
block. Recent genetic findings suggest that approximately 10 % of sporadic congenital
heart disease may have de novo mutations that significantly contribute to the disease
process. Mutations in genes encoding for transcription factors critical for cardiac
chamber formation, endocardial cushion remodeling, and conduction system
development, like NKX2.5 and Tbx5, may lead to heart block associated with CHD.
Numerous mutations in NKX2.5 have been reported with various CHD phenotypes,
such as secundum atrial septal defect, tetralogy of Fallot, truncus arteriosus, double-
outlet right ventricle, L-transposition of great arteries, interrupted aortic arch,
ventricular noncompaction, and hypoplastic left heart, with or without conduction
disorders.2
The fourth differential diagnosis was acquired heart block. Before a congenital
heart block diagnosis is established, the physician must first rule out the possible
underlying conditions that can cause an acquired heart block.8 Acquired heart block in
the young can also be derived from a wide variety of causes such as surgical or
catheterization-induced trauma, coronary artery disease, acute or chronic infectious
processes (diphtheria, rheumatic fever, chorea, and congenital syphilis), myocarditis,
hypersensitivity cardiomyopathy, metabolic abnormalities, hypothyroidism, or

16
infiltrative processes. The incidence of AV node dysfunction is higher in patients with
Kawasaki disease, possibly caused by myocarditis or an abnormal microcirculation in
the AV node artery. In unstable patients with Lyme carditis, a complete heart block is
usually reversible with appropriate antibiotics. Acute rheumatic carditis must also be
kept in mind in the diagnostic work-up of patients with AV conduction disorder. In
association with acquired heart disease, particularly when it occurs in pediatric
patients; Most cases are reversible first or second-degree heart blocks.4 In this case, the
patient came with chief complain three days of remittent fever with the highest
temperature of 39oC, but after receiving initial treatment for infection, the heart rate
remained slow so the patient was referred for further pacemaker management. So
infection could be excluded from the cause of heart block in this patient.
This patient was 4 months old when diagnosed with congenital complete heart
block. The early diagnosis of congenital heart block must be made from 16-24 weeks
of gestational age. Specific risk factors associated with in utero mortality include fetal
hydrops, diagnosis of CHB at <20 weeks, ventricular escape rate < 55 bpm, and
impaired left ventricular function. The US Research Registry for Neonatal Lupus found
that cases of CCHB had a mortality rate > 50% when the fetus had either dilated
cardiomyopathy or endocardial elastofibriosis (EFE) and a mortality of 100% when the
fetus had both risk factors. Postnatally these patients require close monitoring as their
mortality risks continue after birth. Almost two thirds of these patients will require a
pacemaker, the majority within the first 10 days of life. However, with pacemaker
implantation, most of these children have an overall good prognosis and can be
expected to have a near normal life expectancy if ventricular function is preserved.6
In this case, during the pregnancy, from previous antenatal examination, the fetal
heart beat was found to be within normal limits. The diagnosis of congenital heart block
might be delayed in this patient because of a relatively high ventricular rate at birth (up
to 100 beats/min). Persistent ductus arteriosus and atrial septal defects of secundum
type with left-to-right shunts involve an extra volume load on the ventricles have
already compensated for the low heart rate by increasing the stroke volume up to 100%.

17
It makes problems in establishing the diagnosis of a block associated with cardiac
defects because it can appear, disappear, and reappear after birth.8
In this case, the results of the patient's echocardiography showed moderate
secundum ASD and moderate PDA. Left-to-right shunt from ASD and PDA in this
patient can cause an increase in blood volume to the right ventricle which can increase
the ventricular rate which may cause a delay in the diagnosis of heart block in this
patient.
From the further history taking about patient recent clinical symptoms, history of
pregnancy and birth, family history, physical findings, electrocardiography, and
echocardiography, this patient showed a suspicion of heart block associated with
congenital heart disease even though immune-mediated heart block cannot be excluded
because they still require anti-Ro/SSA and/or anti-La/SSB maternal autoantibodies.
The patient’s nutritional status was underweight and severely wasted. The patient
had normal developmental milestones according to her age. Newborns with congenital
heart block II-III had a significantly lower weight, shorter length, and smaller head
circumference. Malnutrition in this patient was attributed to inadequate protein and
calorie intake due to feeding difficulties and the increased energy expenditure.
Malnutrition can affect growth and development of the muscles, bones, brain, and
nerve cells. Dyspnea and shortness of breath in this patient can also affect the motoric
developmental milestones. 9
Pharmacological therapy has a distinct role in the acute management of severe
bradycardia and should typically be carried out alongside parallel efforts to arrange for
transcutaneous pacing and temporary cardiac pacing in order to prevent from sudden
cardiac death.2 The patient received pharmacological therapy that consists of lisinopril,
furosemide, and dopamine syringe pump before the patient was placed in a temporary
pacemaker. Beta-adrenergic agonists such as isoproterenol, dopamine, dobutamine,
and epinephrine exert direct effects to enhance atrioventricular nodal and, to a lesser
degree, His-Purkinje conduction. These drugs may also enhance automaticity of
subsidiary atrioventricular junctional and ventricular pacemakers in the setting of
complete atrioventricular block. In this case, after the initial pharmacological therapy,

18
the patient didn’t s show any improvement of the heartbeat. Patients with CHB are
usually unresponsive to atropine or other pharmacological therapy. Therefore, early
detection of congenital heart block and more aggressive approach toward pacemaker
treatment are the most important prognostic factor in individual with congenital heart
block.10
In this case, the patient received temporary pacemaker in order to prepare
permanent pacemaker insertion device. Indications for pacemaker implantation in
infant are : complete atrioventricular block following cardiac surgery, symptomatic
congenital complete heart block, asymptomatic congenital complete heart block in
association with: low mean heart rate <50-55 bpm in neonates, nocturnal pauses,
bradycardia related arrhythmias, acquired heart block in myopathies, symptomatic
bradycardias in: sick sinus syndromes, long QT syndromes, reflex anoxic seizures with
secondary anoxic, epileptic seizures. 10 (Tabel 1)
In essence, every symptomatic, non-reversible AV node disease requires
permanent pacemaker implantation. Pacing must also be considered in asymptomatic
high degree AV blocks with specific risk conditions. Although historical series of
isolated congenital AV block reported a high incidence of unpredictable Stokes-Adams
attacks and a high mortality associated with the first attack, latest studies of pediatric
cardiac pacing showed that prophylactic pacing, as currently recommended, is
associated with strong reduction in the morbidity and mortality due to Stokes-Adams
attacks.
Recent indications for cardiac pacing in children and CHD patients are
summarized in Table 1. Levels of evidence are classified in “level A” if data are derived
from multiple randomized clinical trials or meta-analyses, “level B” if data are derived
from a single randomized clinical trial or large non-randomized studies, and “level C”
if there is a consensus of opinion of the experts and/or if data are derived from small
studies, retrospective studies, or registries. Recommendations are listed according to
the commonly used class I, IIa, IIb, and III classification and the corresponding
language: “is recommended” for a class I recommendation; “can be useful” for a class

19
IIa recommendation; “may be considered” to signify a class IIb recommendation; and
“should not” or “is not recommended” for a class III recommendation.
In this case, the patient had a symptomatic congenital complete heart block,
with a low mean heart rate, which was 52 beats per minute so the patient fulfilled the
pacemaker implantation indication. The patient received temporary pacemaker in order
to prepare permanent pacemaker (epicardial pacemaker) insertion device.

Table 1 Pacing indications in children and patients with congenital heart disease11,12

ACCF/AHA/HRS
ESC guidelines
guidelines
Congenital heart block
Symptomatic advanced second- or third-degree heart block
Class I, level C Class I, level C
Asymptomatic high degree heart block with ventricular dysfunction Class I, level C Class I, level B
Asymptomatic high degree heart block with prolonged QTc interval Class I, level C –
Asymptomatic high degree heart block with complex ventricular ectopy Class I, level C Class I, level B
Asymptomatic high degree heart block with wide QRS escape rhythm Class I, level C Class I, level B
Asymptomatic high degree heart block with abrupt ventricular Class I, level C Class IIa, level B
pauses >threefold the basic cycle length
Asymptomatic third-degree heart block in the infant with a – Class I, level C
ventricular rate <55 bpm or with CHD and a ventricular rate
<70 bpm
Third-degree heart block beyond the first year of life with an – Class IIa, level B
average heart rate <50 bpm
Asymptomatic high degree heart block with a ventricular rate <50 bpm Class I, level C –
Third-degree heart block beyond the first year of life with – Class IIa, level B
symptoms due to chronotropic incompetence
High degree heart block in asymptomatic Class IIb, level C Class IIb, level B
children/adolescents in absence of the above risk
conditions
Asymptomatic type I second-degree heart block – Class III, level C
Postoperative heart block
Postoperative advanced second- or third-degree heart Class I, level B Class I, level B
block that persists >7 days after cardiac surgery (10 days
in ESC guidelines)
Transient postoperative third-degree heart block that reverts to Class IIa, level C Class IIb, level C
sinus rhythm with residual bifascicular block
Unexplained syncope in the patient with prior CHD surgery – Class IIa, level B
complicated by transient complete heart block with
residual fascicular block
Transient postoperative heart block with return of normal – Class III, level B
AV conduction in the otherwise asymptomatic patient
Asymptomatic postoperative bifascicular block with/without – Class III, level C
first-degree heart block in the absence of prior transient
complete heart block
Brignole M, Auricchio A, Baron-Esquivias G, Bordachar P, Boriani G, Breithardt OA, et al. 213 ESC Guidelines on cardiac pacing
and cardiac resynchronization therapy. Eur Heart J. 2013;34:2281–329.
Epstein AE, Dimarco JP, Ellenbogen KA, Estes NAM, Freedman RA, Gettes LS, et al. 2012 ACCF/AHA/HRS focused update
incorporated into the ACCF/AHA/HRS 2008 guidelines for device-based therapy of cardiac rhythm abnormalities:A report of the
American college of cardiology foundation/american heart association task force on practice guide.Circ. 2013;127:283–352.

20
 There is a general consensus that the smallest infants are best served with
epicardial pacing systems, with a cutoff weight around 15–20 kg.10 Epicardial leads are
more likely to fracture and are prone to exit block, and implantation requires a major
operation that is accompanied by the inherent risks and need for perioperative support.
However, many groups standardly use epicardial pacing in infants and young children
and argue that endocardial systems may carry a significant risk of venous thrombosis
in infants, which can result in loss of venous access in the future, leading to a more
complicated lead revision later in the patient’s life.12 Up to a 19 % transvenous lead-
related failure rate has been reported by others who choose the endocardial approach.
Extraction of abandoned transvenous leads in the pediatric population is also
problematic, and optimal lead management still remains to be defined. On the other
hand, there is a global trend towards using endocardial leads in younger patients and
some institutions actively implant transvenous leads in children weighing less than 15
kg. It has been shown that an 80-mm right atrial lead loop will allow 6 to 12 years
(mean, 8 years) of growth in infants and children without the need for reoperation to
adjust lead length. Long-term follow-up demonstrates that the longevity of an
endocardial system exceeds that of its epicardial counterpart, also an important
consideration in patients who will be exposed to the cumulative burden of repeated lead
and device reimplantation. Despite growing experience, endocardial implantation is
not universally accepted most publications reporting results of transvenous.13
In this case, the patient had got temporary pacemaker within 24 hours after she
was admitted to Dr. Soetomo General Hospital. Although historical series of congenital
heart block reported a high incidence of unpredictable Stokes-Adams attacks and a high
mortality associated with the first attack, latest studies performed in our era of pediatric
cardiac pacing showed that prophylactic pacing, as currently recommended, is
associated with strong reduction in the morbidity and mortality due to Stokes-Adams
attacks.4
Long-term potential complications in all patients include development of
ventricular dilatation and dysfunction. Children with autoimmune CCHB may also
have frequent ectopy and primary or secondary long QT syndrome. Patients without a

21
pacemaker may develop AV valve regurgitation, atrial rhythm disorders,
thromboembolism, congestive failure, or sudden death. Patients with a pacemaker may
develop pacing system–related complications, including lead fracture, malsensing, and
pacing system infections. Replacement of pacemakers is required at intervals and can
lead to complications from the procedure.14
Congenital heart block carries a significant risk of morbidity and mortality (15-
30%), especially in utero or in the first few months of life. Around 63% of all
recognized cases are reported to require pacemaker implantation before reaching
adulthood. In another study, out of 65% of cases who required lifelong pacing, 20%
resulted in mortality. In several studies, it has been demonstrated that mortality due to
CHB mostly occurs in fetal, neonatal or infant periods. In the study of Buyon, et al.,
including 113 patients with CHB, the neonatal and fetal mortality rate was found to be
19%.15 Eronen, et al., reported that the mortality was 16% among 91 infants with CHB,
with deaths mostly occurring in infancy.16 In another study of 36 fetuses with CHB and
structurally normal heart, the mortality occurred in 9%, occurring in utero or neonatal
period. In the study of Jaeggi, et al. 45% of CHB cases diagnosed in utero died. In the
presence of hydrops, reported mortality rates for infants born with CHB exceeded
80%.17
Identified factors for poor prognosis include: hydrops fetalis, low heart rate (<50-
55 beats per minute) or sudden rapid decrease of the heart rate, endocardial
fibroelastosis (EFE) dilated cardiomyopathy, valvular dysfunction, low birth weight,
male sex, delivery at <34 weeks of gestation, and complications from prematurity or
neonatal lupus. The significant echocardiographic predictors of mortality are only
hydrops and EFE.5

22
SUMMARY

A case report of congenital heart block in a four-month-old baby girl has been
reported. The patient was referred with the chief complaint of abnormally slow heart
rate, with ECG examination revealed third-degree heart block, and considered to have
pacemaker implantation. The diagnosis of congenital heart block might be delayed
because of a relatively high ventricular rate at birth. Persistent ductus arteriosus and
atrial septal defects of secundum type with left-to-right shunts involve an extra volume
load on the ventricles have already compensated for the low heart rate by increasing
the stroke volume up to 100%. From the further history taking about patient recent
clinical symptoms, history of pregnancy and birth, family history, physical findings,
electrocardiography, and echocardiography, this patient showed a suspicion of
congenital heart block associated with congenital heart disease even though immune-
mediated congenital heart block cannot be excluded because they still require anti-
Ro/SSA and/or anti-La/SSB maternal autoantibodies
The patient received pharmacological therapy that consists of lisinopril,
furosemide, and a dopamine syringe pump before the patient was placed in a temporary
pacemaker. Pharmacological therapy has a distinct role in the acute management of
severe bradycardia and should typically be carried out alongside parallel efforts to
arrange for transcutaneous pacing and temporary cardiac pacing to prevent sudden
cardiac death. In this case, the patient had an asymptomatic congenital complete heart
block, with a low mean heart rate, which was 52 beats per minute so the patient fulfilled
the pacemaker implantation criteria.

23
REFERENCES

1. Smithells RW. Congenital Heart Block. Br Med J. 1960;2:599.

2. Baruteau AE, Pass RH, Thambo JB, Behaghel A, Le Pennec S, Perdreau E, et
al. Congenital and childhood atrioventricular blocks: pathophysiology and
contemporary management. Eur J Pediatr. 2016;175:1235–48.
3. Park MK, Park S. Park’s pediatric cardiology for practitioners. seventh ed. Park
MK, Salamat M, editors. Philadelphia: Elsevier Inc; 2021. 335–337 p.
4. Berul CI. Pediatric Third-Degree Acquired Atrioventricular Block. 2018;1–7.
5. Hastanesi A, Cevizli DC. 2013 Neonatal congenital heart block, Ind. Ped.,
Yildirim, Tunaoolu, Karaaoac. 2013;
6. Pruetz JD, Miller JC, Loeb GE, Silka MJ, Bar-Cohen Y, Chmait RH. Prenatal
diagnosis and management of congenital complete heart block. Birth Defects
Res. 2019;111:380–8.
7. Ciardulli A, D’Antonio F, Magro-Malosso ER, Manzoli L, Anisman P, Saccone
G, et al. Maternal steroid therapy for fetuses with second-degree immune-
mediated congenital atrioventricular block: a systematic review and meta-
analysis. Acta Obstet Gynecol Scand. 2018;97:787–94.
8. Michaelsson M, Riesenfeld T, Jonzon A. Natural history of congenital complete
atrioventricular block. PACE - Pacing Clin Electrophysiol. 1997;20:2098–101.
9. Skog A, Wahren-Herlenius M, Sundström B, Bremme K, Sonesson SE.
Outcome and growth of infants fetally exposed to heart block-associated
maternal Anti-Ro52/ssa autoantibodies. Pediatrics. 2008;121:803–9.
10. Kusumoto FM, Schoenfeld MH, Barrett C, Edgerton JR, Ellenbogen KA, Gold
MR, et al. 2018 ACC/AHA/HRS Guideline on the Evaluation and Management
of Patients With Bradycardia and Cardiac Conduction Delay: A Report of the
American College of Cardiology/American Heart Association Task Force on
Clinical Practice Guidelines and the Heart Rhyth. Vol. 140, Circulation. 2019.
382–482 p.
11. Brignole M, Auricchio A, Baron-Esquivias G, Bordachar P, Boriani G,
Breithardt OA, et al. 213 ESC Guidelines on cardiac pacing and cardiac
resynchronization therapy. Eur Heart J. 2013;34:2281–329.
12. Epstein AE, Dimarco JP, Ellenbogen KA, Estes NAM, Freedman RA, Gettes
LS, et al. 2012 ACCF/AHA/HRS focused update incorporated into the
ACCF/AHA/HRS 2008 guidelines for device-based therapy of cardiac rhythm
abnormalities: A report of the American college of cardiology
foundation/american heart association task force on practice guide. Circulation.
2013;127:283–352.

iii
13. McLeod KA. Congenital heart disease: Cardiac pacing in infants and children.
Heart. 2010;96:1502–8.
14. Gupta M. Pediatric Congenital Atrioventricular Block. Medscape Ref [Internet].
2018;1–10. Available from: https://emedicine.medscape.com/article/894703-
overview#a5%0D
15. JP Buyon, J Waltuck, C Kleinman and J Copel. In Utero identification and
therapy of congenital heart block. Lupus. 1995;4: 116-121
16. Eronen M, Sirèn MK, Ekblad H, Tikanoja T, Julkunen H, Paavilainen T. Short-
and long-term outcome of children with congenital complete heart block
diagnosed in utero or as a newborn. Pediatrics. 2000;106:86–91.
17. Jaeggi ET, Hamilton RM, Silverman ED, Zamora SA, Hornberger LK. Outcome
of children with fetal, neonatal or childhood diagnosis of isolated congenital
atrioventricular block: A single institution’s experience of 30 years. J Am Coll
Cardiol. 2002;39:130–7.

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