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University Journal of Medicine and Medical Sciences
ISSN 2455-2852
Volume 2 Issue 3 2016
Congenital Complete Heart Block A Case Report
GANESAPANDIAN PITCHAIMALLAIAN
Department of Paediatrics,
MADRAS MEDICAL COLLEGE AND GOVERNMENT GENERAL HOSPITAL
be completely asymptomatic in presence of
these autoimmune antibodies or may have
a diagnosis of a collagen vascular disease
Abstract :
(eg, systemic lupuserythematosus(SLE),
Congenital complete heart block or third- Sjogren syndrome). Isolated CAVB can
degree congenital atrioventricular block also occur due to myocarditis and rarely
(CAVB) is seen in a fetus or in a neonate hereditary conditions, such as storage disyounger than 28 days. CAVB can occur orders (eg, Hurler syndrome, Hunter synin a structurally normal heart (isolated drome). Often, no etiology is found for an
CAVB) or with congenital heart disease isolated CAVB. The prognosis in complete
(complex CAVB with congenital heart de- CAVB is relatively good but may be influfects). Congenital complete heartblock enced by the patient's age at presentation.
occurs in approximately 1 per 15,000- Patients presenting as fetuses or at birth
20,000 live births. Structural congenital have significantly higher morbidity and
heart block is rare, but with a higher pro- mortality rates than do patients presenting
portion of fetal loss. Patients with iso- later in childhood. Herewith we are prelated CAVB typically have a better over- senting a rare case of congenital complete
all outcome than do infants with complex heart block reported in a tertiary care hosstructural cardiac defects and or ven- pital in Chennai.
tricular dysfunction.
Keyword :Congenital complete heart Case report:
block, congenital atrioventricular block
A 10 days old full term newborn female
child weighing 3 kg was referred as having
Introduction:
low heart rate and admitted with respiratory
Isolated CAVB occurs in the absence of distress since birth. The child was delivother congenital heart defects. It is seen ered by emergency caeserian section and
in association with certain autoimmune cried immediately after birth. During the anantibodies in the mother that cross the tenatal ultrasonography, fetal bradycardia
placenta and damage the atrioventricular was madeout for
(AV) node of the fetus The mother can
An Initiative of The Tamil Nadu Dr. M.G.R. Medical University
University Journal of Medicine and Medical Sciences
which mother was investigated. She was
positive for anti SSA(Ro) and anti SS-B(La)
antibodies but was asymptomatic. There was
no history of consanguinity, maternal fever,
rashes, joint pain or drug intake. The patient
was first in order of birth and there was no
history of abortion or still birth.On examination, the child was pink and normothermic..
There was marked respiratory distress. Respiratory rate was 68/min with intercostal and
subcostal retractions..Heart rate was 42 per
minute.
The child was warm and all peripheral
pulses were palpable. Cardiovascular system examination revealed the apex beat on
left side in the fourth intercostal space. A
pansystolic murmur of grade III intensity was
audible all over precordium. Liver was 2 cm
below left costal margin and the liverspan
was 6.5cm. Bilateral crepitations were heard
in the chest. No other obvious congenital
anomaly was und. The oxygen saturation was
94%.
Chest X-ray showed levocardia with cardiomegaly. On electrocardiogram, atrial rate
was 150/min, and ventricular rate was 40/
min with complete A-V dissociation. 2D-Echo
revealed dilated cardiomyopathy, gross LA/
LV dilatation, mild MR, pulmonary hypertension- physiological and global hypokinesia.
The baby was negative for anti SS-A and
anti SS-B antibodies. The child was diagnosed as a case of complete congenital
heart block without congenital heart defect
(isolated CAVB). The baby was managed
with warmer care, oxygen inhalation, iv fluids
and Inj. Isoprenaline(0.05micrograms/kg/
min) as stop gap procedure. Baby underwent
pacemaker implantation. The baby is now on
followup without any specific new complaints.
Discussion:
The incidence of congenital complete
heart block has been estimated to be
about 1 out of 15,000 to 22,000 live
births(4,5). There is a tendency for female
preponderance in CAVB(6). Approximately 25% to 33% of all congenital
complete heart blocks are associated
with congenital heart disease like ventricular septal defect, endocardial cushion defect, patent ductus arteriosus, mitral incompetence, persistent foramen
ovale, transposition of great arteries
and Ebstein's anomaly. The most common among these associated lesions is
L-transposition of the great arteries. Our
case did not have any heart defects.
The association of collagen vascular
disease in mothers of infants with congenital complete heart block is significant(7) . About 60% of mothers who deliver children with CAVB have anti SSA
and anti SSB antibodies. Mothers with
SLE who have had one child with congenital heart block are at risk of having
subsequent offspring with heart block
(6). Maternal Lupus may not become
manifest for years even after the birth of
an infant with CAVB. In our case also
the mother was asymptomatic. Maternal
lupus is known to influence fetal and
neonatal outcomes and is associated
with increased incidence of obstetric
complications such as stillbirth, abortion, prematurity, intrauterine growth restriction (IUGR), and neonatal complications such as neonatal lupus erythematosus syndrome, which is characterized
by transient lupus dermatitis, hepatic
and haematological abnormalities like
hemolytic anemia, leucopenia, thrombocytopenia and/or isolated CAVB,CCF,
endocardial fibroelastosis (8). Skin rash,
hepatitis and thrombocytopenia generally resolve without sequel. By contrast,
the heart block is permanent and
An Initiative of The Tamil Nadu Dr. M.G.R. Medical University
University Journal of Medicine and Medical Sciences
requires a pacemaker in about 66% of cases
(8). The presence of anti SS-A or SS-B antibodies in mothers and neonates with complete
heart block has been clearly documented(9).
These antibodies are IgG against SSA and
SSB ribonucleoproteins, can cross the placenta and appear in the fetus at around the
16th week of gestation and affect the conduction system, cause sclerosis of the AV node.
All fetuses are not affected. Other factors that
could be responsible for the development of
congenital complete heart block are HLA type,
timing of antibody transfer, and inutero environment. The presence of certain HLA types
(HLA-DR3, B8, DRW52, and DQW2) in the
mother and fetus increase the possibility of
complete heart block. The clinical features of
the neonate depend on the effect of heart rate
on cardiac output. At birth, the infant may present with congestive heart failure, anasarca,
hepatomegaly and metabolic acidosis requiring emergency pacing. Isolated CAVB is only
rarely accompanied by CCF(6) which was observed in our case. It is becoming increasingly
recognised that dilated cardiomyopathy(DCM)
either fetal or postnatal is a rare but serious
outcome of autoimmune congenital complete
heart block. Our case had dilated cardiomyopathy. The cause of the cardiomyopathy
is unclear but may be related to an autoimmune myocarditis occurring inutero rather
than primarily caused by bradycardia.(6) The
SSA/Ro and SSB/La antibodies have been
suggested to be a major determinant (10,11) in
the patients with CAVB with DCM. Evidence
for a potential immunopathologic role of the
SSA/Ro and SSB/La antibodies in CAVB is
described in several studies (12,13). Immunouorescent studies have shown IgG and IgM
deposition throughout the myocardium on
postmortem examination(14,15).However, affected newborns often appear asymptomatic
and may have accelerated ventricular rates
approaching those of healthy newborns. An
associated finding in isolated
CAVB may be the presence of discoid skin lesions. Our case did not
have any skin lesions. Children with
structural heart defects may present
with cyanosis, failure to thrive or recurrent pneumonias or may be completely asymptomatic in childhood
(such as children with L-transposition
of the great arteries and intact ventricular septum). Prolonged QTc is not
a constant finding in congenital block
but is common with associated malformations and symptomatic patients. In
asymptomatic patients, prolonged
QTc may herald the onset of symptoms(16). There is an approximately
95% twenty year survival of patients
without anatomic heart defect(17). After
birth, electrocardiography (ECG) is
recommended to assess for CAVB
and to assess the QT interval that can
be prolonged. Neonatal assessment
should include a measurement of antiRo and anti-La antibody levels. Echocardiography should be performed initially and in periodic follow-up care in
affected fetuses, infants and children
to assess ventricular function and size
and to rule out congenital or acquired
cardiac malformations or valve dysfunction(18). In utero management of
fetuses with transuterine fetal cardiac
pacing have been tried without success(19).Administration of steroids, immunoglobulins andplasmapheresis in
the mother are useful in first and second degree heartblocks. The use of
intravenous dopamine and isoproterenol in babies has shown some success. Pacemaker therapy is needed in
symptomatic babies. Temporary pacing can be done by transcutaneous,
transesophageal and transvenous
routes. However permanent pacemaker is eventually needed for most
infants. It is of two types namely
An Initiative of The Tamil Nadu Dr. M.G.R. Medical University
University Journal of Medicine and Medical Sciences
epicardial and endocardial pacing. The indications for pacing are HR < 50 per minute,
atrial rate>140 per minute, atrio ventricular
block pauses lasting > 3 seconds, features
of CCF, low cardiac output, ventricular dysfunction, post surgical CAVB..
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University Journal of Medicine and Medical Sciences