Clinical features of isolated unilateral absence of pulmonary artery
Wang Xinxia
Zhu Guangfa
Gao Yuanming
Zhu Chenxi
Abstract：Objective To analyze clinical features of isolated unilateral absence of pulmonary artery (UAPA).Methods Six patients diagnosed of UAPA from April 2001 to September 2016 in Beijing Anzhen Hospital,Capital Medical University were retrospectively analyzed;clinical data included general information,clinical manifestation,laboratory examination,chest imaging examination,diagnosis,treatment and prognosis.Results There were 2 males and 4 females,with a mean age of (53 ± 9) years.There was 1 case of left pulmonary artery absence and 5 cases of right pulmonary artery absence.Unilateral absence of pulmonary artery had no early clinical manifestation and the symptoms appeared at age of (46 ± 10)years.The main manifestations were chest tightness,shortness of breath,palpitation,cough and expectoration,hemoptysis,intermittent syncope and edam of lower extremities;all patients had hyoxemia;some had pulmonary artery hypertension,type Ⅱ respiratory failure and pulmonary heart disease.All patients were diagnosed by selective pulmonary angiography and multi-slice spiral CT pulmonary angiography.Patients with pulmonary artery absence were usually misdiagnosed of bronchiectasis,pulmonary embolism and coronary heart disease;the mean misdiagnosis time was 4.5 (2.5,12.5)years.One case had proximal bronchial artery coil embolization;1 case had no special treatment;4 cases had medical symptomatic treatments and discharged after recovery.Conclusion UAPA is a rare congenital pulmonary vascular malformation with no specific clinical manifestations.
Keywords：Unilateral absence of pulmonary arteryPulmonary artery hypoplasiaPulmonary artery agenesisPulmonary vascular malformation
Publication Date：2017-07-30
Online Publishing Date：2026-09-14(First online date of this platform, not the publication date of the document)
Pages：4( 1001-1004 )