Indonesia Journal of Biomedical Science, Volume 10, Number 1: 6-11

ISSN: 2085-4773, 2302-2906

TRUNCUS ARTERIOSUS TYPE I: A CASE REPORT

Putu Fristia Indarini1, Eka Gunawijaya2

1
Department of Child Health Udayana University, Sanglah Hospital
2
Medical School of Udayana University, Sanglah Hospital
Corresponding: Fristia, e-mail: fristiaindarini@me.com

ABSTRACT
Truncus Arteriosus (TA) is a rare congenital cyanotic heart disease which is characterized by single
arterial trunk arising from heart which in turn gives rise to pulmonary trunk in various ways. TA
constitutes 1.2% of all congenital heart malformations. According to Collett-Edwards classification there
are 4 types of the disease. The most common variety is Type-1 which is characterized by short main
pulmonary artery that originate from the trunk and give rise to right and left pulmonary arteries. Early
surgical intervention is advised in all cases within first 2 month of life, in case of severe pulmonary
hypertension, surgery is contraindicated. A full-term male newborn suspected with congenital cyanotic
heart disease is referred to Sanglah Hospital. The patient had respiratory distress and looked cyanotic
after breastfed. Physical examination revealed murmur on the chest auscultation. The chest X-ray
showed prominent pulmonary vascular markings and unusually high hilar areas. The echocardiography
showed truncus arteriosus type I, with moderate regurgitation, large pulmonary atresia, large
ventricular septal defect, functionally single atrium, moderate mitral regurgitation, mild tricuspid
regurgitaton. Patient was also diagnosed with heart failure ROSS II. The patient was given milk through
orogastric tube, oxygen, and oral therapy for heart failure such as furosemide, spironolactone and
digoxin. Another echocardiography was planned in the next 3 month. Pulmonary hypertension, heart
failure, infective endocarditis and desaturation are recommended to be closely monitored.

Keywords: truncus arteriosus, heart failure

INTRODUCTION arteries.4,5 According to Collett‐Edwards

Truncus arteriosus (TA) constitutes 1.2% of classification, type 1 disease is characterized by a
all congenital heart malformations.1 In TA, systemic single pulmonary arterial trunk that originates from
pulmonary and coronary circulation originate from a the common trunk and gives off right and left
single vessel that is emerging from the right and left pulmonary arteries. While in Type 2 TA, main
ventricles by a truncal valve usually tricuspid, but pulmonary arterial trunk is absence and the right
quadricuspid and bicuspid valves have also been and left pulmonary arteries originate together. In
reported.2 The valve leaflets may be up to six in type 3 TA, there is no pulmonary truncus present,
number.3 This condition may lead to valvular however, the right and left pulmonary arteries
regurgitation, stenosis or both. Patients with TA branched from the truncus separately. Type 4 is
typically have a large subarterial ventricular septal identified by the absence of pulmonary arteries; the
defect (VSD) located beneath the truncal valve. lungs are supplied by major aorto‐pulmonary
TA classification is made by two systems collateral arteries, which form an anatomic picture
developed by Collett‐Edwards and Van Praagh, that may also be named as pulmonary atresia with
which are based on the origins of the pulmonary VSD.

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 Indonesia Journal of Biomedical Science, Volume 10, Number 1: 6-11
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In Van Praagh classification system, TA Type Antenatal care was performed by an obstetrician
A1 and A2 are similar to Collet‐Edwards TA Type 1 regularly and the antenatal ultrasound was normal.
and Type 2, respectively. Type A3 is characterized by Vitamin K and hepatitis B immunization was
a branch of pulmonary artery (usually right) performed on the first hour of life.
originating from the common trunk while other lung On the 10th day of life, the patient looked
is perfused by either the pulmonary artery cyanotic and unchanged by a positional change. The
originating from the aortic arch or a vessel with an parents also complaint about the poor feeding.
origin from ductus arteriosus or major aorto‐ Then, he was then brought to a private hospital in
pulmonary collaterals. There is an associated which a chest x-ray (Figure 1) and an
interrupted aortic arch in addition to Type 1 and 2 in echocardiography were performed. The chest X-ray
Type A4. showed a prominent pulmonary vascular markings
The coronaries usually originate and an unusually high hilar areas. The
independently and have a normal course. However, echocardiography, performed by a general
rare anomalous coronary take offs or courses may pediatrician, showed a suspected TGA, a large peri-
also be observed. 6% of cases are characterized by a membrane VSD and a single functional atrium. The
persistent left superior vena cava (PLSVC) draining doctor and family decided to refer this patient to
into coronary sinus.3 The disorder is accompanied by Sanglah hospital for further examination.
an interrupted aortic arch or an aortic coarctation in
14% of cases. A patent foramen ovale and an atrial
septal defect are common. However, a coarctation
of the aorta, an anomalous pulmonary venous
drainage, a hypoplasia of the left lung, an anomalous
origin of subclavian arteries, a single ventricle, an
absent right arteries‐veins connection, a hypoplastic
right ventricle, a tubular hypoplasia of the aortic
arch, and a double aortic anomaly may rarely
present.3

CASE REPORT
An eighteen-days-old male baby was referred
from a private hospital in West Nusa Tenggara, to
our Sanglah Hospital’s emergency room, Bali, with
main complain respiratory distress and cyanosis.
Based on the echocardiography, he was suspected Figure 1. The chest X-ray
with a transposition of the great artery (TGA), a large
peri-membrane ventricular septal defect (VSD) and a He was admitted to our hospital on August
single functional atrium. 31, 2014. A physical examination revealed an alert
The patient was the fourth child, with no baby, a heart rate of 140 times per minute, regular,
history of congenital heart defect in both parents and the respiration rate was 60 times per minute,
nor his second grade family. He was born as a regular. The peripheral oxygen saturation was 86%.
vigorous baby, with a body weight of 4100 grams, a The body temperature was 36.7oC. Body weight on
body length of 52 cm. There was no illness during admission was 4300 gram. The head was normal-
the pregnancy period noted, nor a history of a shape and the hair was thick, black, and the fontanel
traditional medicine consumption. The patient was was flat. There was no jaundice on sclera, neither
delivered at full term gestation with a cesarean conjunctiva injection, anemia, nor sunken. The pupil
section due to an early membrane rupture.

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 Indonesia Journal of Biomedical Science, Volume 10, Number 1: 6-11
ISSN: 2085-4773, 2302-2906

light reflex was normal. The ear, nose, and throat mild tricuspid regurgitation (Figure 2, 3). Based on
examination were within normal limit. The sucking the clinical manifestation, the chest x-ray, and the
reflex was normal. There was no lymph node echocardiography, the patient was diagnosed with a
enlargement on the neck. The chest was heart failure ROSS II, truncus arteriosus type I, with
symmetrical both on rest and during respiration. On moderate regurgitation, large PA, large VSD,
auscultation, the breath sound was bronchovesicular functionally single atrium, moderate MR, mild TR.
without rales or wheezing. The first and second Digoxin was added as an oral therapy. The
heart sound were normal, regular and murmur is echocardiography will be repeated in 3 months. A
audible on the fifth left intercostal space (ICS), grade close monitoring of the early signs of a pulmonary
III/6. There was no lymph node enlargement on both hypertension, a heart failure, an infective
of the axillas endocarditis and a desaturation, was performed. A
The skin elasticity was normal, no epigastric surgical intervention was not performed because
distention, the bowel sound was normal, the liver such procedure cannot be done in our medical
was palpable about 2 cm below costal arc and 2 cm center.
below xiphoid process and the spleen were not
palpable. There was no inguinal lymph node
enlargement found on both sides. The extremities
were normal. Moreover, the power, tonus, and the
reflexes of the superior and inferior extremities were
normal.
The patient was diagnosed with a congenital
heart defect: a suspected TGA, a large peri-
membrane VSD, a single functional atrium and a
heart failure ROSS II. He was given a breastmilk on
demand via orogastric tube, oxygen, and an oral
therapy consists of furosemide and spironolactone. Figure 3. The echocardiography showed truncus
Echocardiography was planned for this patient. arteriosus type I
On September 9, 2014, this patient was
transferred to the Mataram Hospital in his home
province, West Nusa Tenggara, to continue his
treatment.

DISCUSSION
Truncus arteriosus is a rare congenital
cyanotic heart disease characterized by a single
arterial trunk arising from the heart, which gives rise
to pulmonary trunk. There are four types of
pulmonary trunk recognized, the most common
Figure 2. The echocardiograpgy showed a VSD variety is type 1, characterized by short main
pulmonary artery that originate from truncus and
The second echocardiography, performed by give rise to right and left Pulmonary arteries. In type
a pediatric cardiologist, showed truncus arteriosus 2, the pulmonary artery arises from separate ostia
type I, with moderate regurgitation, large pulmonal from the side of truncus. In type-3, the pulmonary
artery (PA), large ventricular septal defect, single arise from a separate ostium from the back of the
functional atrium, moderate mitral regurgitation and truncus. In type 4, the pulmonary artery is absent or

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 Indonesia Journal of Biomedical Science, Volume 10, Number 1: 6-11
ISSN: 2085-4773, 2302-2906

atretic. The truncal valve could be quadricuspid (40- and a poor feeding. His birthweight was 4100 grams,
50%), bicuspid, tricuspid or hexacuspid. The left and only gain 200 grams after 18 days of life.
coronary artery arise from the left posterior aspect On examination, there are evidence of a
of the truncus, the right coronary artery arise from cardiomegaly, a collapsing pulse, a loud single
the right anterior aspect of the truncus. A VSD is second heart sound, a systolic murmur (harsh,
always nonrestrictive, and roofed by a truncal valve, blowing, 3/6 – 4/6, best heard on the 3/4 left
setting a stage for truncal valve regurgitation.1,2 In intercostal space), preceded by an ejection click.
our patient, the echocardiography showed truncus Initially, the pulmonary vascular resistance was low.
arteriosus type I, with moderate regurgitation, a As the pulmonary ventricular resistance (PVR) rises
large pulmonal artery, a large ventricular septal after birth, the pulmonary vascular resistance
defect, a single functional atrium, moderate mitral becomes equal to systemic and causes Eisenmenger
regurgitation, mild tricuspid regurgitation. syndrome, cyanosis, and bidirectional shunting. 4,5
A case of truncus arteriosus usually shows a The patient was cyanotic and a grade III/6 murmur
tachypnea, a poor feeding, and a sign of failure to was audible on the fifth left intercostal space. The
thrive within the first few weeks of life. The patients liver was palpable about 2 cm below the costal arc
seldom reach their first birthday.3 Our patient was and 2 cm below xiphoid process, as signs of a heart
brought to the hospital on the 10th day of life with a failure.
main complaints of a respiratory distress, a cyanosis,

Figure 4. The surgical method used in the Rastelli cohort

(A) Right ventricle incision is placed away from epicardial vessels and retraction sutures are placed to provide
exposure to the VSD and tricuspid valve apparatus. (B) The VSD is routinely enlarged by excision of a wedge of the
septal tissue anterosuperiorly using an 11 blade. In addition, excision of a large portion of infundibular septum
beneath the aortic valve is usually done. (C) A cylindrical patch is used to construct the baffle with running sutures
to the right ventricular tissue. (D) The patch should be redundant to provide an unobstructed baffle between the
left ventricle and aorta. (E) Right ventricle outflow reconstruction is completed with a homograft valved conduit.

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 Indonesia Journal of Biomedical Science, Volume 10, Number 1: 6-11
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