Donato, Paulo

Silva, Nuno Pereira

English

Acta Radiológica Portuguesa

ACTA RADIOLÓGICA PORTUGUESA
January-April 2019   Vol 31 nº 3   13-14
ARP Case Report Nº 15: Pulmonary Atresia with Ventricular Septal 
Defect and Major Aortopulmonary Collateral Arteries
Caso Clínico ARP Nº15: Atrésia da Válvula Pulmonar com Defeito do Septo 
Interventricular e Macroartérias Colaterais Aortopulmonares
Nuno Pereira da Silva, Paulo Donato
Imaging Department, University Centre Hospitals of  Coimbra. Faculty of  
Medicine, University of  Coimbra, Portugal
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Presentation of  the case
A 21-year-old male is admitted to our hospital’s emergency 
ward with cyanosis. Laboratory routines revealed severe 
hypoxia. The patient had an history of  congenital heart 
defect. Computer tomography angiography with ECG gating 
was performed.
The exam revealed right aortic arch, an interventricular 
communication associated with an “overriding” aorta and 
increased trabeculation of  the right ventricular cavity.
Three major aortopulmonary collateral arteries (MAPCA) 
can be seen branching from the descending aorta. The 
pulmonary arteries are hypoplastic and have no visible 
connection to the right ventricle, arising from the largest 
MAPCA which completely supplies them.
The findings are consistent with Pulmonary Atresia with 
Ventricular Septal Defect and Major Aortopulmonary 
Collateral Arteries (PA-VSD-MAPCAs). 
Discussion
PA-VSD-MAPCAs is a rare complex congenital heart defect, 
the most severe form of  tetralogy of  Fallot. In this condition, 
the normal connection between the right ventricle and the 
pulmonary arteries is absent and a ventricular sept defect 
is apparent. Central pulmonary arteries may be hypoplastic 
or even non-existing and, if  present, tend to be confluent. 
Consequently, patients develop aortopulmonary collaterals 
in utero to compensate for a lack normal pulmonary blood 
flow. The anatomical layout and dimension of  the collateral 
vessels is extremely diverse and is the reason for the variety in 
clinical progressions and post-surgical outcomes. Aneurysmal 
dilatation of  MAPCAs may be visible, especially in cases of  
long duration of  the disease.
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Cardiac catheterization remains the diagnostic gold 
standard thanks to the vast and accurate hemodynamic 
data provided in the procedure. However, MR and CT 
angiography can accurately identify the pulmonary blood 
flow with similar results. CT is especially elucidative in the 
characterization of  MAPCA’s, of  paramount importance 
as the surgical goal is often to eliminate the extracardiac 
sources of  pulmonary arterial blood flow by combining the 
MAPCAs into a confluent pulmonary artery, thus creating a 
complete pulmonary arterial system. This process is called 
“unifocalization”.
Mortality rates are high without surgical intervention, up to 
60% in the first year, less than 50% survival in 10 years, and 
less than 20% in 20 years.
Bibliography
Presnell LB, et al. 2015. An overview of  pulmonary atresia and major 
aortopulmonary collateral arteries. World Journal for Pediatric and 
Congenital Heart Surgery. 2015;6:630-9.
Rajeshkannan R, et al. Role of  64-MDCT in evaluation of  pulmonary 
atresia with ventricular septal defect. American Journal of  Roentgenology. 
2010;194:110-8. 


Caso Clínico ARP Nº15: Atrésia da Válvula Pulmonar com Defeito do Septo Interventricular e Macroartérias Colaterais Aortopulmonares

https://revistas.rcaap.pt/actaradiologica/article/download/17765/14114

Caso Clínico ARP Nº15: Atrésia da Válvula Pulmonar com Defeito do Septo Interventricular e Macroartérias Colaterais Aortopulmonares

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