LETTER TO THE EDITOR

Imaging findings of an asymptomatic child with pulmonaryvein atresia

Taísa Davaus Gasparetto & Pedro Daltro &

Edson Marchiori

Received: 10 February 2010 /Accepted: 18 March 2010# Springer-Verlag 2010

Sir,We recently read with great interest the case reported byMataciunas et al. [1] about a patient diagnosed with isolatedunilateral pulmonary vein atresia. The report described a12-year-old boy who presented with recurrent pulmonaryinfections without haemoptysis and an intolerance ofphysical activity during childhood. The chest radiographyshowed a mild mediastinal shift to the right side andreduced vascularity of the right lung. The chest CT revealedan increased attenuation of the right lung parenchyma,interlobular septal thickening, and a small right hemithoraxwith an ipsilateral mediastinal shift. Angiography con-firmed the diagnosis of unilateral pulmonary vein atresia.The boy was treated conservatively because his clinicalcondition was favorable.

We would like to describe our case of a 13-year-old girldiagnosed with unilateral pulmonary vein atresia that wasconfirmed by MR angiography. In contrast to the casereported by Mataciunas and colleagues [1], our patient wascompletely asymptomatic until she had an abnormal chestradiograph during an evaluation of influenza symptoms.

The chest radiograph showed a small left hemithorax with amediastinal shift and interstitial thickening. The main CTscan findings were mediastinal shift, small hemithorax,increased attenuation of the lung parenchyma, and inter-lobular septal thickening. MR angiography demonstratesthe left pulmonary artery and the reduced left pulmonaryvascular bed. The left pulmonary veins are not visualized(Fig. 1).

The clinical presentation (an asymptomatic course) ofour patient was distinct from the case reported byMataciunas and colleagues [1] as well as most other casesdescribed in the literature that have presented with typicalclinical features such as recurrent pulmonary infections,haemoptysis, and exercise intolerance [1–5]. Mataciunasand colleagues [1] suggested that these clinical manifes-tations depend on anatomical variations of the pulmonaryvessels that supply and drain the affected lung parenchymaand have a subclinical course associated with well-developed venous collaterals [2–4]. In contrast to previousfindings, which describe other congenital heart defects andpulmonary artery hypertension as frequently related con-ditions, our case did not present with associated cardiovas-cular malformations, most likely leading to the benigncourse and late diagnosis [2–4].

Although surgery is the treatment of choice in mostcases reported [2, 3, 5], our patient received a conservativetreatment due to the lack of symptoms and associatedanomalies. A 5-year follow-up examination of our patientrevealed the same findings that were previously shown onthe chest radiograph at the time of the diagnosis. Clinically,the girl remained asymptomatic. Our case, as well as thecase reported by Mataciunas and colleagues, suggests that aconservative treatment may be a good option for patientswith pulmonary vein atresia who experience an asymptom-atic or mildly symptomatic clinical course.

A reply to this letter can be found at doi:10.1007/s00247-010-1664-0.

T. D. Gasparetto : P. Daltro : E. MarchioriDepartament of Radiology, Federal University of Rio de Janeiro,Rio de Janeiro, Brazil

T. D. Gasparetto : P. DaltroCDPI-Clínica de Diagnóstico Por Imagem,Rio de Janeiro, Brazil

T. D. Gasparetto (*)Rua Prefeito Dulcídio Cardoso, 1500, ap 1504, Barra da Tijuca,Rio de Janeiro, RJ, Brazil CEP 24220065e-mail: taisadavaus@gmail.com

Pediatr RadiolDOI 10.1007/s00247-010-1663-1

References

1. Mataciunas M, Gumbiene L, Cibiras S et al (2009) CT angiographyof mildly symptomatic, isolated, unilateral right pulmonary veinatresia. Pediatr Radiol 39:1087–1090

2. Heyneman LE, Nolan RL, Harrison JK et al (2001) Congenitalunilateral pulmonary vein atresia: radiologic findings in three adultpatients. AJR 177:681–685

3. Pourmoghadam KK, Moore JW, Khan M et al (2003) Congenitalunilateral pulmonary venous atresia: definitive diagnosis andtreatment. Pediatr Cardiol 24:73–79

4. Argueta-Morales RI, Garg R, DeCampli WM (2009) Diagnosis andmanagement of congenital right pulmonary venous atresia. CardiolYoung 19:648–651

5. Swischuk LE, L’Heureux P (1980) Unilateral pulmonary veinatresia. AJR 135:667–672

�Fig. 1 Unilateral pulmonary vein atresia in a 13-year-old girl. a Chestradiograph reveals a small left hemithorax with diffuse and increasedinterstitial markings in the left lung that are most prominentlydemonstrated in the lower lobe. b High-resolution CT scan (lungwindow) at the level of the upper lobes demonstrates ground-glassattenuation throughout the left lung with interlobular septal thicken-ing. c MR angiography of the chest shows the left pulmonary arteryand the reduced left pulmonary vascular bed. The left pulmonary veinsare not visualized. The MR angiography also demonstrates the normalarborization of the right pulmonary vascular bed and the presence ofthe right pulmonary veins

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