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Patient (condition)
 
Aliases:
Truncal valve
Truncas arteriosus

Topic aliases are alternate phrasings for a particular topic.


A congenital anomaly caused by the failed development of TRUNCUS ARTERIOSUS into separate AORTA and PULMONARY ARTERY. It is characterized by a single arterial trunk that forms the outlet for both HEART VENTRICLES and gives rise to the systemic, pulmonary, and coronary arteries. It is always accompanied by a ventricular septal defect.
www.nlm.nih.gov/cgi/mesh/...

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Classification of truncus arteriosus is either by Collet and Edwards, or Van Praag.
Picture from Boston Children's Hospital www.childrenshospital.org...

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Deletion of chromosome 22q11 is present in approximately 11–35% of patients with truncus arteriosus, and this chromosomal abnormality is associated with DiGeorge syndrome and velocardio facial-syndrome.

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Most patients have a ventricular septal-defect, although an intact septum can occur.

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As the pulmonary vascular resistance falls, patients can develop pulmonary overcirculation manifesting as CHF with early development of pulmonary veno-occlusive-disease (PVOD).
Truncal valve regurgitation may further worsen heart failure, and coronary ischemia may be occur as a consequence of low diastolic pressures.
Alternatively, pulmonary artery stenosis may be present, limiting pulmonary blood flow.
Andropolous, Anaesthesia for Congenital Heart Disease. p440

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