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The CT scans show dilated pulmonary arteries (PAs) (48 mm) in a 59-year-old woman. Absent pulmonary valve syndrome (APVS) was considered at tetralogy of Fallot (ToF) repair in 1961, when no pulmonary leaflets were seen. Moderate dilatation of PAs (34 mm) was noted when a pulmonary homograft replacement for pulmonary regurgitation was inserted in 1997. Recurrent chest infections, breathlessness with good LV and RV function (PA pressure 28/14) and hoarseness secondary to a recurrent laryngeal nerve palsy led to CT scanning, confirming large PAs (panel A) compressing the right middle lobe bronchus (panel B).
APVS is a rare congenital malformation of unknown frequency. It may occur in 3–6% of patients with ToF, but can be associated with ventricular septal defect, atrial septal defect, coarctation of the aorta and tricuspid atresia. Characteristic features include aneurysmal dilatation of PAs, which often leads to major bronchial and intrapulmonary compression, obligate pulmonary regurgitation and degrees of pulmonary branch stenoses.
Long-term follow-up of APVS without early plication of PAs is limited. Management in this case is difficult but may include right middle lobe resection or a Lecompte procedure (translocation of the PA anterior to the aorta away from the airways to eliminate bronchial compression), with PA plication.
Acknowledgments
This article has been adapted from Howard S, Wan S, Freeman L J. Congenital absence of the pulmonary valve Heart 2007;93:779