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Abstract
BackgroundAbsent pulmonary valve is a rare cardiovascular anomaly that can result in profound tracheobronchial compression.ObjectiveTo demonstrate the advantage of multi-slice CT in diagnosing tracheobronchial compression, its severity as related to the adjacent dilated pulmonary arteries, and associated lung and cardiac lesions.Materials and methodsWe included children with absent pulmonary valve who were reviewed by multi-slice CT during a 17-year period. The number and locations of stenoses and lung lesions were noted and the severity of stenosis was categorized. The diameter of the pulmonary artery was measured and associated cardiac defects were demonstrated.ResultsThirty-one children (14 girls and 17 boys) were included. Of these, 29 had ventricular septal defect and 2 had an intact ventricular septum. Twenty-nine children (94%) had tracheobronchial compression, judged to be mild in nine children (31%), moderate in 10 (34%) and severe in 10 (34%). The different locations of the stenosis (carina, main bronchi, lobar and segmental bronchi) were observed. And the number and location of lung lesions demonstrated that the right middle and left upper and lower lobes were often affected. The diameter of the pulmonary artery in these children was well above normal published values, and Spearman rank correlation analysis showed a correlation between the size of the pulmonary artery and the severity of the tracheobronchial stenosis. Nineteen children (61%) underwent surgery and 4 of these children had a multi-slice CT post-operative follow-up study.ConclusionAbsent pulmonary valve can cause significant morbidity and mortality in children. Multi-slice CT can accurately depict areas of tracheobronchial compression, associated lung lesions and cardiac defects, helping to direct the surgeon.
Highlights
Absent pulmonary valve is a rare cardiovascular anomaly that occurs with ventricular septal defect or much less commonly with an intact septum
Absent pulmonary valve with ventricular septal defect is often defined as absent pulmonary valve syndrome or tetralogy of Fallot variant
Absent pulmonary valve with an intact ventricular septum can present with patent ductus
Summary
Absent pulmonary valve is a rare cardiovascular anomaly that occurs with ventricular septal defect (absent pulmonary valve with tetralogy of Fallot or with double-outlet right ventricle) or much less commonly with an intact septum (isolated absent pulmonary valve). Characteristic features of absent pulmonary valve with ventricular septal defect include varying degrees of right ventricular outflow tract obstruction, profoundly dilated branch pulmonary arteries, absent ductus arteriosus and association with DiGeorge syndrome and 22q11.2 deletion syndrome. Absent pulmonary valve with ventricular septal defect is often defined as absent pulmonary valve syndrome or tetralogy of Fallot variant. Especially absent pulmonary valve syndrome, can lead to profound bronchial and carina compression caused by the adjacent dilated pulmonary arteries. Absent pulmonary valve is a rare cardiovascular anomaly that can result in profound tracheobronchial compression
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