[Table/Fig-16]:
Subtypes: Collett and Edwards classification [18] |
Type I = pulmonary trunk arises from proximal truncus Type II and III= no pulmonary trunk; branch PA arise from posterior and lateral mid-segments of truncus Type IV=pulmonary circulation dependent on MAPCAs. |
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Incidence | 1% of CHD. 1 in 11,000 live births(10–5) | ||||
Association [13] | Right aortic arch, interrupted aortic arch, coarctation, PDA Coronary artery anomalies(stenotic coronary ostia, abnormal branching and course) Unilateral absence of pulmonary artery | ||||
Syndromes | DiGeorge syndrome Pierre Robin syndrome | ||||
Imaging features Plain | Moderate cardiomegaly with narrow base | High cephalic origin of PA | Right aortic arch | Depressed diaphragm | Thymic atrophy |
Imaging features specific | Echo large truncus, large VSD and a large echogenic truncal valve with fibrous continuity with anterior mitral leaflet MRI -similar findings | ||||
Management | Primary repair: done in first week of life; consists of VSD closure and placement of valve conduit between RV and PA (Rastelli procedure) |