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292
K. Özden et al.
Pulmonary atresia and ventricular septal defect
with MAPCAs associated with right sided
endocarditis and paradoxical embolic event
Kıvılcım Özden *, Bülent Mutlu, Gökhan Kahveci, Fatih Bayrak,
Levent Saltık, Salih Güran, Yelda Basaran
Kartal Kosuyolu Heart Education and Research Hospital, Sonomed Imaging Center,
Cerrahpasa Faculty of Istanbul University, Istanbul, Turkey
Received 16 September 2005; received in revised form 16 February 2006; accepted 2 March 2006
Available online 18 April 2006
KEYWORDS
Abstract Pulmonary atresia and ventricular septal defect (PA-VSD) with major aortopulmonary collaterals (MAPCAs) is a complex and extremely heterogeneous
anomaly. PA-VSD with both pulmonary arteries originating from systemic arterial
circulation without MAPCAs and patent ductus arteriosus (PDA) is a very rare disease
and according to our knowledge a case without cyanosis and symptoms of congestive
heart failure after the first decade of life has not been reported. The majority of untreated patients die in their first decade of life as a result of intractable congestive
heart failure or respiratory distress. This report informs about a 21-year-old PA-VSD
patient who presented without cyanosis with both pulmonary arteries arising from
aorta associated with right sided endocarditis and a paradoxical embolic event.
ª 2006 The European Society of Cardiology. Published by Elsevier Ltd. All rights
reserved.
Introduction
Pulmonary atresia and ventricular septal defect
(PA-VSD) with major aortopulmonary collaterals
(MAPCAs) is a complex and extremely heterogeneous anomaly. A case of PA-VSD with both
pulmonary arteries originating from systemic arterial circulation along with paradoxical embolia and
tricuspid valve endocarditis has not been reported.
This case seems to be the first to comprise all of
these pathologies.
Case report
A 21-year-old boy was admitted with symptoms of
fever, weakness and palpitation. He had symptoms
of weakness and dyspnea previously, it was his first
* Corresponding author. Bahar Sitesi B4 blok No: 9 Barbaros
Mah.Kosuyolu, Uskudar, Istanbul, Turkey. Tel.: þ905054549888.
E-mail address: drkivilcim@yahoo.com (K. Özden).
admission to a cardiology clinic. His physical
examination revealed a remarkable systolic murmur in precordium and a continuous murmur in
back. There were no clubbing and no cyanosis.
Laboratory examination demonstrated leucocytosis, increased acute phase reactants, positive
blood cultures of gram (þ), oxidase (), catalase
(þ) coccobacillus.
Chest X-ray showed increased right bronchovascularity, normal left bronchovascularity, right
arcus aorta and increased CTI. Transthoracic 2D
and Doppler echocardiography revealed wide VSD,
70% dextraposition of aorta, 0.8 1.3 cm sized,
mobile vegetation on anterior cuspis of tricuspid
valve, atresia of pulmonary valve and main
pulmonary truncus. Suprasternal echocardiography
revealed a large artery arising from left brachiocephalic truncus. Fig. 1A, B, and C shows suprasternal
echocardiographic view of LSA-RPA (left subclavian
artery-right pulmonary artery) collateral.
Pulmonary MRI angiography was performed to
demonstrate the pulmonary anatomy. It revealed
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Pulmonary atresia;
Ventricular septal
defect;
Tricuspid endocarditis;
Paradoxical embolia;
Abnormal origin of
both pulmonary
arteries
Pulmonary atresia and ventricular septal defect with MAPCAs
293
Discussion
Figure 1 (A) Suprasternal Doppler Echocardiographic
view of a large artery arising from left subclavian artery.
LSA, left subclavian artery; RPA, right pulmonary artery;
and AscAo, Ascending aorta. (B) Suprasternal Doppler
Echocardiographic view of right pulmonary artery arising
from the subclavian artery and branching of RPA to supply
pulmonary segments. LSA, left subclavian artery; RPA,
right pulmonary artery; and AscAo, Ascending aorta. (C)
Suprasternal Color Doppler Echocardiographic view of
a large artery (LSA-RPA colat) arising from left subclavian
artery. LSA-RPA colat, left subclavian artery-right pulmonary artery collateral; and LSA, left subclavian artery.
right arcus aorta, right descending aorta, pulmonary atresia, left brachiocephalic truncus, Right
Pulmonary Artery (RPA) arising from left brachiocephalic truncus, and Left Pulmonary Artery (LPA)
arising from descending aorta. The pulmonary
PA-VSD and MAPCAs is a complex and rare lesion in
which considerable morphologic variability exists
regarding the sources of pulmonary blood flow
leading to a wide spectrum of clinic presentation
and difficulty in surgical therapy.1 PA-VSD represents as the most severe form of tetralogy of
Fallot.
The Baltimore Washington Infant study reported
an incidence of 0.07 per 1000 live births for PAVSD. It accounts for 1.5% of all forms of congenital
heart disease and 20% of all forms of TOF.
The source of pulmonary blood flow in PA-VSD is
the systemic arterial circulation. PA-VSD is classified into three types according to the source of
pulmonary blood flow.2 Surgical options of each
type are based on the presence or absence of
NPA (Native Pulmonary Arteries) and MAPCAs. In
the most frequent type of PA-VSD, type A,
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arteries were arising from aorta as well developed
large branches. There were no extra collaterals.
LPA and RPA were supplying all pulmonary segments. Pulmonary MRI angiographic images are
shown in Fig. 2A and B.
Right and left heart catheterization showed
pulmonary atresia and the anomalous origin of
pulmonary arteries. Fig. 2C and D is the catheterization image. There was a stenosis in distal portion of left pulmonary artery and the systolic
pressure of LPA (55 mmHg) was lower than the systolic pressure of RPA (100 mmHg). Systemic arterial and pulmonary arterial blood O2 saturation
was 96%. Fig. 2E demonstrates the whole abnormal
anatomy of pulmonary arteries.
After evaluation of symptoms, echocardiography and blood culture analysis, patient was diagnosed as infective endocarditis. Vancomycin and
gentamycin combination therapy was applied for 6
weeks. The clinical symptoms of endocarditis and
echocardiographic sign of vegetation diminished
during the therapy. On the 24th day of therapy, the
patient underwent femoral embolectomy due to
the right femoral embolia. Pathological examination of embolectomy material was consistent with
healed vegetation. Macroscopy of the vegetation is
shown in Fig. 3.
After the medical therapy of infective endocarditis, the patient recovered completely. Surgery
for the complex congenital anomaly to reduce the
pulmonary blood flow was planned. However, the
patient and his family refused the operation due
to its high risk. The patient is now followed up
medically.
294
K. Özden et al.
pulmonary circulation is through the PDA into the
confluent pulmonary arteries supplying all of the
bronchopulmonary segments. PA-VSD and MAPCAs
comprises only about 25% of PA-VSD. In type B,
NPA (native confluent pulmonary arteries) are
present with MAPCAs. In type C, there are only
MAPCAs, without NPA.
The nonconfluent pulmonary arteries exist in
20e30% of the PA-VSD patients. MAPCAs are large
and distinct arteries, highly variable in number
that usually arise from the descending aorta, but
uncommonly may originate from the aortic arch,
the subclavian, the carotid or even the coronary
arteries.3 There is at least one MAPCA in the
whole cases with nonconfluent pulmonary
arteries.4,5
Aotsuka et al. reported a case of anomalous
origin of confluent pulmonary arteries arising from
ascending aorta without an intracardiac defect.6 In
previously defined PA-VSD congenital anomalies,
nonconfluent well developed native pulmonary
arteries without MAPCAs or PDA were not reported. In our case, the pulmonary arteries arise
from the subclavian artery and the descending
aorta like MAPCAs but they are well developed
and branch to supply all of the pulmonary
segments.
A complex congenital anomaly with right sided
endocarditis that is complicated with peripheral
embolia has not been reported previously. Infective endocarditis (IE) occurs less commonly in
children than in adults (1/1280 per year).7 Because of the increased survival rate of children
with congenital heart disease (CHD) and the overall decrease in rheumatic valvular heart disease
in developed countries, CHD now constitutes the
predominant underlying condition for IE in children.8 CHD with left to right shunt is a risk factor
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Figure 2 (A) Pulmonary MRI image demonstrating the large branch arising from the junction of left brachiocephalic
truncus and the subclavian artery that forms the right pulmonary artery. (B) Pulmonary MRI image shows the large
branch arising from the descending aorta that forms the left pulmonary artery. (C) Catheterization image. The right
pulmonary artery arises from the left subclavian artery as a large branch. (D) The left pulmonary artery arises from
descending aorta as a large branch. (E) Schematic explanation of anatomy of pulmonary arteries. LSA-RPA colat; left
subclavian artery-right pulmonary artery collateral, Desc.Ao-LPA colat; descending aorta-left pulmonary artery collateral. LScA, left subclavian artery; RPA, right pulmonary artery; Desc.Ao, DA: descending aorta; LPA, left pulmonary
artery; and obs; obstruction-stenosis in the distal portion of the branch.
Pulmonary atresia and ventricular septal defect with MAPCAs
295
unifocalization surgery. In our opinion, the stenosis
of the distal portion of LPA has protected the left
pulmonary segments from advanced pulmonary
disease. But due to the large systemic pulmonary
arteries, pulmonary vascular disease development
is inevitable in following years. A stepwise surgical
approach to reduce the pulmonary blood flow was
planned for our case.
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The option of surgery of PA-VSD and MAPCAs
without central pulmonary artery is the
1525-2167/$32 ª 2006 The European Society of Cardiology. Published by Elsevier Ltd. All rights reserved.
10.1016/j.euje.2006.03.004
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Figure 3
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