Type:
Educational Exhibit
Keywords:
Cardiac, Cardiovascular system, Paediatric, CT-Angiography, Image manipulation / Reconstruction, Computer Applications-3D, Education, Congenital, Developmental disease, Education and training
Authors:
P. Narang1, S. Arya2, A. K. Verma3, N. Goyal 4, D. S. Agarawal4; 1DELHI, DELHI/IN, 2Delhi/IN, 3New Delhi, Delhi/IN, 4New Delhi/IN
DOI:
10.1594/ecr2018/C-0936
Background
- MAPCAS or Major Aorto-Pulmonary Collateral Arteries are the alternative pulmonary circulation in the congenital cyanotic heart disease,
when ever RVOT is compromised.
- They gain access to the lung through the hilum and connect with native pulmonary arteries in1) the mediastinum
2) at the lobar level
3) or at Subsegmental level.
- In some patients,
MAPCAs supplement the pulmonary blood supply creating a dual supply to the lungs,
while in some they may be the sole source of blood supply as in patients of pulmonary atresia with VSD.
- Precise visualization of primary and alternative pulmonary vasculature is mandatory for surgical planning and effective planning for unifocalization [1].
- CT angiography provides accurate depiction of complex cardio vascular anatomic features.
It also facilitates the assessment of extra cardiac systemic and pulmonary arterial and venous structure[4].
- Precise characterization of the condition of pulmonary arteries and MAPCAs is of paramount importance in managing patients with Pulmonary Artesia-Ventricular septal defect.
Using 3D imaging software,
a complex pulmonary blood supply can be noninvasively and accurately imaged with high-resolution MDCT[5].
-
In some patients,
MAPCAs supplement the pulmonary blood supply creating a dual supply to the lungs,
while in some they may be the sole source of blood supply as in patients of pulmonary atresia with VSD.
Fig. 1: Barbero-Marcial classification for the pulmonary vascular blood supply in pulmonary atresia with interventricular communication
- At Birth-All lung segments have a healthy blood supply,
from either PA or MAPCAS.
- In TOF/ PA,
the native pulmonary arteries do not grow after birth.
- MAPCAS expose the pulmonary parenchyma to high systemic pressures.
- With time,
pulmonary segments are lost due to abnormal arterial flow and pressure.
- Hence,
timely,
early management improves mortality and morbidity.