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Weekly Chest CasesArchive of Old Cases

Case No : 1130 Date 2019-06-27

  • Courtesy of Jae-Kwang Lim / Kyungpook National University Hospital
  • Age/Sex 14 / F
  • Chief ComplaintIncidental abnormality on routine examination
  • Figure 1
  • Figure 2
  • Figure 3
  • Figure 4
  • Figure 5
  • Figure 6
  • Figure 7
  • Figure 8

Diagnosis With Brief Discussion

Diagnosis
Unilateral pulmonary vein atresia
Radiologic Findings
Figs 1. Chest PA shows a small left hemithorax, with reticular opacities in left lung.
Fig 2-6. Mediastinal setting of axial CT images show absent left pulmonary vein with ipsilateral pulmonary artery hypoplasia. .
Fig 7. Mediastinal setting of coronal reformatted CT image shows absent left pulmonary vein and increased attenuation of mediastinal fat adjacent to venous atresia.
Figs 8. Lung setting of coronal reformatted CT shows a hypoplastic left lung with interlobular septal thickening.
3D-volume rendering image shows absent left pulmonary vein with ipsilateral pulmonary artery hypoplasia
Brief Review
Unilateral pulmonary vein atresia is a rare congenital abnormality. It results from failure of incorporation of the common pulmonary vein into the left atrium during the embryological development. It may occur in either lung, with no right- or left-sided predominance, and it usually presents in infants. The most frequent presenting complaints in infancy include recurrent infections in the hypoplastic lung and hemoptysis due to the systemic collateral supply to the affected lung. Other associated congenital heart defects are found in approximately 50% of patients.
Chest radiographs show small hemithorax, ipsilateral mediastinal shift, and reticular opacities. The CT scan shows characteristic findings of a hypoplastic lung, smooth margins of left atrium without evidence of rudimentary pulmonary veins, and smooth thickening of interlobular septa likely due to the dilation of pulmonary lymphatics and bronchial veins. There is associated small ipsilateral pulmonary artery, attributed to preferential perfusion to contralateral side and confluent low attenuation soft tissue in the mediastinum adjacent to the left atrium that contains pulmonary to systemic collaterals. Treatment of unilateral congenital pulmonary venous atresia may be conservative in relatively asymptomatic patients, and pneumonectomy is done in patients with progressive dyspnea, significant pneumonia, or recurrent pneumonia.
References
1. Narayanan R. et al. Isolated unilateral pulmonary vein atresia. Lung India 2016 Sep-Oct;33(5):571-572
2. Laura E. et al. Congenital unilateral pulmonary vein atresia radiologic findings in three adult patients. AJR Am J Roentgenol. 2001 Sep;177(3):681-685
Keywords
pulmonary vein, ipsilateral pulmonary venous atresia,

No. of Applicants : 68

▶ Correct Answer : 26/68,  38.2%
  • - Showa University Fujigaoka Hospital , Japan KYOKO NAGAI
  • - Chonbuk National University Medical School , Korea (South) HYEJIN YANG
  • - Chonbuk National University Hospital , Korea (South) MINGI SHIN
  • - NIMS, HYDERABAD , India BHASKAR K
  • - Ajou University Hospital , Korea (South) KYUNGMIN LEE
  • - Other , Korea (South) SEONGSU KANG
  • - Seoul National University Hospital , Korea (South) Ju Gang Nam
  • - university of montreal , Canada Andrei Bogdan Gorgos I
  • - The University of Tokyo Hospital , Japan TOSHIHIRO FURUTA
  • - Narayana Multispeciality Hospital Jaipur Rajasthan , India JAINENDRA JAIN
  • - Chonbuk National University Hospital , Korea (South) YOUNGKWANG LEE
  • - Ajou University Hospital , Korea (South) DAYOUNG KIM
  • - radiology bellvitge , Spain patricio luburich
  • - Diskapi Yildirim Beyazit Hospital, Ankara , Turkey MERIC TUZUN
  • - Centre Hospitalier Regional de Lanaudiere , Canada MATTHIEU STORME
  • - Ajou University Hospital , Korea (South) HYERIN KIM
  • - Azienda Ospedaliera di Cremona , Italy Pietro Sergio
  • - Chonbuk National University Hospital , Korea (South) KUM JU CHAE
  • - Chonbuk National University Hospital , Korea (South) SOL KI KIM
  • - Ajou University Hospital , Korea (South) SUBIN HEO
  • - Ichinomiya Nishi Hospital , Japan Takao Kiguchi
  • - Hamamatsu University Hospital , Japan YUKI HAYASHI
  • - the first affiliatited hospital of nanjing medical univercity , China HAI XU
  • - Ajou University Hospital , Korea (South) YONG HO JANG
  • - Ewha Womans University Hospital , Korea (South) YOOKYUNG KIM
  • - Ajou University Hospital , Korea (South) YOO YOUNGJIN
▶ Correct Answer as Differential Diagnosis : 7/68,  10.3%
  • - Kinki University Faculty of Medicine, , Japan MITSURU MATSUKI
  • - McGill University Health Center , Canada Alexandre Semionov
  • - Oita University, Faculty of Medicine , Japan Fumito Okada
  • - TB centre kasaragod. , India rikhy krishnan
  • - Korea University Anam Hospital , Korea (South) KYU-CHONG LEE
  • - Kizawa Memorial Hospital , Japan Shoji Okuda
  • - Toranomon Hospital , Japan KAORU SUMIDA
▶ Semi-Correct Answer : 3/68,  4.4%
  • - The University of Kyoto Hospital , Japan TOMOAKI OTANI
  • - Gifu University Hospital , Japan Yo Kaneko
  • - Seoul National University Hospital , Korea (South) JI HEE KANG
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