TRUNCUS ARTERIOSUS

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Last Updated : 5/8/2016
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Mossab Saeed
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Definition
This condition defined as the presence of a single semilunar valve and annulus with a single great vessel, usually termed the common trunk, arising from the ventricles.Also it known as persistent truncus arteriosus, truncus arteriosus communis, common aorticopulmonary trunk

Background
the first case with truncus arteriosus was reported by Wilson in 1798, and existence of the entity was confirmed by accurate clinical and autopsy reports of a 6-month-old infant by Buchanan in 1864.

Morphogenises and genetics
The actual cause of the embryological disturbance that results in the development of truncus arteriosus lesions is not known.

Most of the early embryological studies suggesting the association between the number of migrated neural crest to the aortopulmonary outflow.

Monoallelic microdeletion of chromosome 22q11 (DiGeorge syndrome) is identified in up to 40% of patients with truncus arteriosus

Anatomy
- large common outflow trunk for right and left sides of the heart with single valve.

-Truncal Valve: can vary from bicuspid leaflet valve up to 7 leaflet in the valve. the communist anatomy is the trileaflet valve.

- Pulmonary arteries vary in position (more details in classification)

- The ventricular septal defect; almost always present at sub-arterial position.

- Coronary ostium position anomalies in not unusual especially the left ostium near by the pulmonary arteries takeoff or with right ostium.

Classification
The first classification was published on 1949 by Collett and Edwards, they classified the anomaly to IV types depend on the origin of the pulmonary arteries from the truncus artery. (see figure I)

on 1965, Vann Praaghs published another classification adding the absence of the ventricular septal defect as group B (see figure I)

Recently on 2011, Russell et al published a simplified method to classify this anomaly after revising 28 autopsies (Figure II)

Figure 1
Figure 1


Figure 2
Figure 2


Associated anomalies
- Right aortic arch 25%
- Aberrent subclavian artery in 10%
- Interrupted aortic arch 10 %
- TAPVR

Pathophysiology
- In early neonatal period usually the babies are asymptomatic due to high pulmonary vascular resistance (PVR) with semi-balanced circulation and arterial saturation 75-80% and episodic cyanosis. When the PVR start to fall down after first week of life the saturation will start to increase with starting of pulmonary overflow and heart failure symptoms if there is no stenosis in the pulmonary arteries.

Usually if the patient can tolerate the heart failure in the first two month the PVR will start to increase and the symptoms may reduce, but they will be prone to irreversible pulmonary hypertension by 6 month of age.

- Additional feature may appear depend on any associated anomaly (like truncal valve stenosis or regurgitation, etc.)

Natural History
The lesion is rapidly lethal in most patients, with median
survival times being about 5 weeks to 5 months, less than 20% survive more than a year, and less than 5% more than 5 years except in the series reported by Collett and Edward and by Fontana and Edwards

References

Contributor Information and Disclosures Mossab Saeed
MBBS MRCSI
Boston Childrens Hospital


First Created : 1/8/2001
Last Updated : 5/8/2016
References
Contributor Information and Disclosures

Last Updated : 5/8/2016
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