Pulmonary artery vegetation in a pediatric patient with ventricular septal defect: a case report

Pulmonary artery vegetation in a pediatric patient with ventricular septal defect: a case report

Haryo Aribowo1*

1Thoracic and Cardiovascular Surgery Division, Department of Surgery, Faculty of

Medicine, Univrsitas Gadjah Mada/Dr. Sardjito General Hospital, Yogyakarta, Indonesia

ABSTRACT

Infective endocarditis (IE) is one of the congenital heart disease complications which is

frequently seen in ventricular septal defects (VSD). The Duke criteria are the diagnostic

criteria for IE. One of the major criteria is evidence of vegetation. In VSD complicated with

IE, vegetation is frequently found on the opening of the defect, on the right ventricular

side of the opening, on the tricuspid valve, and less frequently it is found on the pulmonary

valve. Vegetation found in the lumen of pulmonary artery is rarely reported. In this article,

we reported a rare case of pulmonary artery vegetation in a boy with moderate VSD

and treated with combination of parenteral antibiotic followed by successful surgical

vegetation evacuation and VSD closure. A 6 years old boy was consulted with congenital

heart disease. His chief complaint was shortness of breath. He came with unspecific signs

and symptoms with a history of frequent hospitalization due to pneumonia and paleness.

Chest X-ray showed enlargement of heart chambers. Transthoracic echocardiography

(TTE) revealed moderate size VSD and multiple vegetation on right ventricle outflow

tract, pulmonary artery valve, and inside the lumen of main pulmonary artery and right

pulmonary artery. The blood culture showed a positive result for Streptococcus viridans.

He was treated with parenteral antibiotic and operated on later. We successfully performed

evacuation of the vegetation and VSD closure.

ABSTRAK
Endokarditis infektif merupakan salah satu komplikasi penyakit jantung bawaan yang
sering menyertai defek septum ventrikel (VSD). Kriteria Duke merupakan kriteria diagnosis
untuk endokarditis infektif. Salah satu kriteria mayor nya adalah bukti adanya vegetasi.
Pada VSD dengan komplikasi endokarditis infektif, vegetasi sering ditemukan pada
pembukaan dari defek, pada ventrikel kanan, pada katup trikuspid, dan yang paling jarang
pada katup pulmonalis. Vegetasi pada arteri pulmonalis jarang dilaporkan. Di artikel ini,
kami melaporkan kasus vegetasi arteri pulmonal pada anak laki-laki dengan VSD sedang
dan diterapi dengan kombinasi antibiotik parenteral diikuti dengan pembedahan evakuasi
vegetasi dan penutupan VSD. Anak laki-laki berusia 6 tahun dikonsultasikan dengan
penyakit jantung bawaan. Keluhan utamanya adalah sesak nafas. Pasien datang dengan
tanda dan gejala yang tidak khas dengan riwayat rawat inap berulang karena pneumonia dan
pucat. Hasil X-ray dada menunjukkan pembesaran ruangan-ruangan jantung. Transthoracic
echocardiography menunjukkan VSD berukuran sedang dan vegetasi multiple pada right
ventricle outflow track, katup pulmonalis, dan didalam lumen arteri pulmonalis komunis dan arteri pulmonalis kanan. Kultur darah positif untuk Streptococcus viridans. Pasien
diberi antibiotik parenteral selanjutnya dilakukan pembedahan. Kami berhasil melakukan
evakuasi vegetasi dan penutupan VSD.
Keywords: pulmonary artery – vegetation - ventricular septal defect - infective endocarditis
- surgery



INTRODUCTION
Infective endocarditis (IE) is one of
the congenital heart disease complications.
Incidence of IE is approximately 0.05-0.12
case/1000 patients/year.1 IE is found in 60%
of congenital heart disease cases, and 14% of
them are ventricular septal defects.2 The Duke
criteria are used to established IE diagnosis.
Vegetation is one of the major diagnostic
criteria for infective endocarditis.3,4 Vegetation
is defined as an intra-cardiac mass composed
of microorganisms (bacterial or fungal) and
surrounded by layers of platelet/fibrin which
are attached to the endothelium.5 In cases
of VSD complicated with IE, vegetation is
frequently found on the opening of the defect,
on the right ventricular side of the opening,
on the tricuspid valve and the less frequently
it is on the pulmonary valve. Vegetation
found in the lumen of pulmonary artery is
rarely reported.6 In this article, we reported
a rare case of pulmonary artery vegetation in
a pediatric patient with a ventricular septal
defect.

CASE REPORT
A 6 years old boy was consulted to
thoracic and cardiovascular surgery division
of Dr. Sardjito General Hospital, Yogyakarta
with congenital heart disease. The patient’s
chief complaint was shortness of breath. There
was a history of paleness, frequent cough and
cold, weight loss, and fatigue/intolerance
to exercise. Those symptoms were reported
since he was 2 years old and worsened in the
past week. There were also several histories
of prior hospitalization due to pneumonia
and anemia. On physical examination, blood
pressure was 83/46 mmHg, heart rate was 116
bpm, respiratory rate was 32x/minutes, and
there was no febrile. On auscultation, there
was inconstant S2 split and grade 4/6 pan
systolic murmur in the left parasternal line.
Other physical exams showed no abnormality.
Chest x-rays showed cardiomegaly with
enlargement of the left atrium and ventricle
(FIGURE 1). Transthoracic echocardiography
revealed a perimembranous VSD with the
size of 1.4 cm with multiple vegetation
and moderate tricuspid regurgitation. The
vegetation was located on the right ventricle
outflow tract (RVOT), pulmonary artery valve,
and inside the lumen of the main pulmonary
artery (MPA) and the right pulmonary artery
(RPA) (FIGURE 2 and 3). Vegetation on the
pulmonary artery valve was moving into the
main pulmonary artery at the systolic phase.
Blood culture was done following the TTE
finding which suggested the diagnosis of IE.
The blood culture result showed a growth of S.
viridans and was tested sensitive to ampicillin
and ceftriaxone.

FIGURE 1. Chest x-ray showed enlargement

of the heart with cardiac-thoracic

ratio >0.5

FIGURE 2. Transthoracic echocardiography showed moderate size of VSD.

RV: right ventricle; LV: left ventricle; RA: right atrium; LA: left

atrium; VSD: ventricular septal defect.

FIGURE 3. Vegetation inside the lumen

of main pulmonary artery and

RVOT. AO: aorta; MPA: main

pulmonary artery; RVOT: right

ventricular outflow

The diagnosis of IE was established
regarding the Duke criteria. We found two
major criteria of IE: positive blood culture
and evidence of vegetation. A final diagnosis
of VSD, IE of RVOT and the pulmonary
artery valve with the involvement of MPA and
RPA, and moderate tricuspid insufficiency
was considered. Due to the IE, intravenous
antibiotic treatment was given. He was treated
with intravenous ampicillin (50 mg/kg/day)
and gentamycin (3mg/kg/day) for 4 weeks.
We evaluated the patient after the antibiotic
therapy, and dyspnea was less than before,
blood culture was negative for any pathogens,
however, some remaining vegetation was
still to be found on pulmonary artery valve
and main pulmonary artery. We switched the
antibiotics to ceftriaxone (50 mg/kg/day) for
2 weeks and gentamycin (3 mg/kg/day) for 3
days. On the TTE evaluation, the vegetation
was not significantly reduced and tended to be
permanently attached. Later on, we decided to
do surgical evacuation due to no significant
reduction in size and the mobility of the
vegetation.
The VSD was closed through the median
sternotomy approach. Superior vena cava,
inferior vena cava, and aorta were cannulated
and connected to a cardiopulmonary bypass
machine. Cardioplegic agent was administered
and the heart became asystole. First incision
was made on the pulmonary artery, where we
found multiple vegetation with size varied
between 2-5 mm. The remaining vegetation
in the pulmonary valve and pulmonary
artery was manually evacuated. We found
perimembranous VSD with diameter of 14
mm. We performed VSD closure using a
GoreTex patch sewn to the rightward aspect
of the defect. We closed the heart and placed a
pericardium drain.
DISCUSSION
We found IE with pulmonary artery
involvement, which is rarely to be found. Once
it exists, it is most likely to be associated with
pulmonary artery valve endocarditis. Isolated
pulmonary valve endocarditis and pulmonary
artery endarteritis is a very rare condition.6 IE
pathogenesis involves pathogen access to the
systemic blood flow, endothelial damage, and
formation of vegetation which may become an
emboli and flow to other site.7 In this study, the
patient has VSD as a risk factor for developing
IE. Children with congenital heart disease
(CHD) have a greater risk for developing IE,
with 42% of pediatric IE cases reported with
CHD as an underlying disease.8 VSD is the
third most common defect among patients
with IE, after cyanotic CHD and Atrium septal
defect.9 The abnormal hemodynamic from the
heart defect shunting may cause injury to the
endothelial layer and provide suitable medium
for bacterial colonization resulting in IE.
We found nonspecific complaints related
to IE in this patient. One study shows various
clinical presentations of pediatric IE, where
it is classified into two categories, subacute
and acute. Subacute IE shows low grade fever
and nonspecific symptoms such as fatigue,
arthralgia, myalgia, weight loss, fatigue/
exercise intolerance, and diaphoresis, while
Acute IE shows rapidly progressive disease
with high fever and severely ill appearance.10
However, low-grade fever is only present in
3-15% of patients.11
The murmurs on specific locations that
we found in this case were suggestive to be
caused by the VSD and tricuspid regurgitation.
Murmurs were also found in 80% to 85% of IE
patients as a sign of valvular regurgitation.12
Other signs of IE such as Osler’s nodes,
Janeway lesions, and Roth spots are rarely to
be found.13 We used TTE in order to observe the vegetation in the heart and pulmonary artery.
TTE is a diagnostic modality used in detecting
the presence of vegetation in pediatric patients
with IE. TTE had a mean sensitivity of 97%
for the detection of vegetation in pediatric
patients.14 Vegetation is further defined as
an echogenic mass adhering to the wall or
valve leaflet with different characteristics
from the remaining original heart tissue.15 In
this case, we found the vegetation extended
from the right ventricular outflow tract, to
the pulmonary artery. The characteristic of
the vegetation is best described as tending to
extend from the heart defect into the upstream
chamber after the defect.16
We found no complications related to
the presence of vegetation in the pulmonary
artery. However, prior research shows
it may cause pulmonary hypertension,
lung embolization, and pulmonary artery
dissection which increases the patient’s
morbidity and mortality rate.10,17 In this case,
we found positive blood culture result for S.
viridans. Blood culture is one of the major
Duke criteria for IE. The causative pathogen
is found in 86% of IE cases, and the most
frequently found pathogens are gram-positive
bacteria. The remaining are fungi and gramnegative
bacteria.2 In community-acquired
IE, Streptococcus viridans is the most
isolated bacteria, followed by S. aureus.18
Other causative pathogens are classified
into the HACEK group which consists of
Haemophillus spp, Aggregatibacter spp, C.
hominis, E. corrodens, and Kingella spp.19 Our
finding is concordant with a prior study which
states that S. viridans infection manifests as
subacute IE.10
We administered a combination of
antibiotics followed by surgical therapy in
this patient. Intravenous antibiotic therapy
should be started as soon as IE diagnosis is
established, and it should be administered
for at least 4 weeks and may be continued
to 6-8 weeks. Continuing the antimicrobial
treatment despite the negative blood culture
is reasonable as a prophylactic against
reinfection.19 Surgical interventions are a
secondary therapy in IE. The right timing to
perform surgical intervention in IE is still
controversial, however, earlier intervention
should be considered in order to reduce the
risk of severe complications and improves
patient prognosis.17 Surgical interventions in
IE include: removal of vegetation, repair of
damaged heart tissue, and correction of the
abnormal structure.20
CONCLUSION
We reported a rare case of pulmonary
artery vegetation in a boy with moderate
VSD that we treated with a combination of
parenteral antibiotics followed by successful
surgical vegetation evacuation and VSD
closure.
ACKNOWLEDGEMENT
We would like to thank the patient who
has participated in this study.
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