Authors
J W Chen, H L Zhang, K Wang
Published in
Zhonghua jie he he hu xi za zhi = Zhonghua jiehe he huxi zazhi = Chinese journal of tuberculosis and respiratory diseases. Volume 49. Issue 8. Pages 849-856. Aug 12, 2026.
Abstract
Objective: To summarize the clinical features, imaging findings, and diagnostic and therapeutic experience of adult patients with congenital pulmonary vein atresia (CPVA), in order to improve clinicians' awareness of this rare disease. Methods: The clinical data of 2 adult patients initially diagnosed with CPVA in our hospital were retrospectively analyzed. In combination with literature retrieved from PubMed and Web of Science up to June 2025, we performed a pooled analysis of CPVA cases diagnosed at age ≥18 years. A total of 30 cases from 27 articles were included; together with 2 cases, a total of 32 cases were analyzed. Their clinical manifestations, imaging characteristics, treatment, and prognosis were summarized. Results: Both of our 2 patients were female, aged 24 and 68 years. Case 1 presented with cough and blood-streaked sputum during pregnancy, with symptom relief after delivery; imaging revealed absence of the right pulmonary veins and a right aortic arch. Case 2 presented with chronic cough and was found to have right upper pulmonary vein atresia with collateral circulation. Among the 32 pooled patients, the median age at diagnosis was 25 years. The main symptoms were hemoptysis (13 cases), dyspnea (11 cases), and cough with sputum (6 cases); 5 cases were asymptomatic. Unilateral involvement occurred in 24 cases (right 16, left 8). Conservative treatment was given in 16 cases, and pneumonectomy in 8 cases. Most patients improved after conservative or surgical treatment, with only 1 death due to postoperative complications. On imaging, CPVA often manifested as non-visualization of the affected pulmonary veins, interstitial lung changes, and systemic-to-pulmonary collaterals, and was easily misdiagnosed as interstitial lung disease or infectious lesions. Conclusion: Although CPVA is rare in adults, some cases can be well compensated and remain undiagnosed until adulthood or even old age. The clinical manifestations are nonspecific, and imaging examinations often provide the first clue to diagnosis; therefore, vascular malformations should be considered in the differential diagnosis of unilateral pulmonary lesions. Treatment should be individualized: conservative follow-up is suitable for asymptomatic or mild cases, whereas surgery or interventional therapy may be considered for those with massive hemoptysis or severe symptoms.
PMID:
42557071
Bibliographic data and abstract were imported from PubMed on 06 Aug 2026.
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