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Anomalous unilateral pulmonary vein in a patient with aortic coarctation
Journal of Cardiovascular Computed Tomography (2008) 2, 274 –275
Images in Cardiovascular CT
Anomalous unilateral pulmonary vein in a patient with aortic coarctation Nicholaos Kakouros, BSc, MRCPa*, John Giles, FRCP, FRCRb, David Walker, MD, FRCPb a
St George’s Hospital, London, United Kingdom, and bConquest Hospital, East Sussex, United Kingdom KEYWORDS: Anomalous pulmonary vein; Aortic coarctation; Congenital heart disease
Abstract: A 34-year-old woman with previous aortic coarctation repair at the age of 5 years was referred for review. She was in good health. On clinical examination, there was no blood pressure difference between the arms, but pedal pulses were weak. A contrast-enhanced 64-slice thoracic CT scan was performed to assess the coarctation repair. © 2008 Society of Cardiovascular Computed Tomography. All rights reserved.
A healthy 34-year-old woman with previous aortic coarctation repair at the age of 5 years was referred for routine evaluation. On clinical examination, blood pressures were symmetric in the upper extremities, but the pedal pulses were weak. A transthoracic echocardiogram was normal. A contrast-enhanced 64-slice thoracic CT scan, performed to assess the coarctation repair, showed slight aortic dilatation proximal to the coarctation with some tortuosity but no restenosis. However, an aberrant left pulmonary vein was incidentally noted (Fig. 1). This aberrant pulmonary vein failed to enter the left atrium at the expected level (Fig. 1A) and traversed behind the right pulmonary artery and joined the right upper pulmonary venous system to drain into the left atrium (Fig. 1B–D, arrows; Supplementary Video 1). Anomalous unilateral pulmonary vein is an exceedingly rare, benign abnormality characterized by tortuous pulmonary venous drainage to the left atrium. The malformation is often discovered incidentally on a chest Supplementary material for this article may be found at http://www. CardiacCTjournal.com. Conflict of interest: The authors report no conflicts of interest. * Corresponding author. E-mail address: [email protected] Submitted March 30, 2008. Accepted for publication June 2, 2008.
radiograph, where it may be mistaken for a pulmonary arteriovenous malformation or Scimitar sign.1,2 Associated abnormalities include pulmonary arterial hypoplasia, lung bronchiectasis or hypoplasia, cor triatriatum, and diaphragmatic eventration.2,3 This is the first reported case of anomalous unilateral pulmonary circulation associated with aortic coarctation. Recognition of this rare entity and differentiation from Scimitar syndrome and pulmonary arteriovenous malformation are important because, despite the tortuous course of the pulmonary vein, the arterialized blood returns to the left atrium. Consequently, there is no hemodynamic shunt, and no therapeutic intervention is required.
References 1. Hanson JM, Wood AM, Seymour R, Petheram IS: Anomalous unilateral single pulmonary vein: two cases mimicking arteriovenous malformations and a review of the literature. Australas Radiol. 2005;49:246 –51. 2. Rey C, Vaksmann G, Francart CL: Anomalous unilateral single pulmonary vein mimicking partial anomalous pulmonary venous return. Cathet Cardiovasc Diagn. 1986;12:330 –3. 3. vanWassenaer AG, Lubbers LJ, Losekoo G: Partial abnormal pulmonary venous return in Turner syndrome. Eur J Pediatr. 1988;148: 101–3.
1934-5925/$ -see front matter © 2008 Society of Cardiovascular Computed Tomography. All rights reserved. doi:10.1016/j.jcct.2008.06.004
Kakouros et al
Anomalous pulmonary vein with aortic coarctation
275
Figure 1 (A) Curved multiplanar reconstruction along the length of the anomalous pulmonary vein. It arises as a pulmonary confluence in the left lung (arrow on right side of image), traversing behind the heart (arrowhead) and wrapping over the pulmonary artery (PA) to join the right pulmonary venous system. (B) Transverse section showing the posterior anomalous pulmonary vein course between the main pulmonary artery (PA) and the descending aorta (Ao). (C) Sagittal plane view with the anomalous vein marked with white arrow. The tortuosity at the site of the previous aortic coarctation repair can be also appreciated. (D) Three-dimensional volume-rendered image showing the drainage of the anomalous left pulmonary vein (arrow) into the right upper pulmonary vein (arrowhead) that subsequently drains into the left atrium.
Images in Cardiovascular CT
Anomalous unilateral pulmonary vein in a patient with aortic coarctation Nicholaos Kakouros, BSc, MRCPa*, John Giles, FRCP, FRCRb, David Walker, MD, FRCPb a
St George’s Hospital, London, United Kingdom, and bConquest Hospital, East Sussex, United Kingdom KEYWORDS: Anomalous pulmonary vein; Aortic coarctation; Congenital heart disease
Abstract: A 34-year-old woman with previous aortic coarctation repair at the age of 5 years was referred for review. She was in good health. On clinical examination, there was no blood pressure difference between the arms, but pedal pulses were weak. A contrast-enhanced 64-slice thoracic CT scan was performed to assess the coarctation repair. © 2008 Society of Cardiovascular Computed Tomography. All rights reserved.
A healthy 34-year-old woman with previous aortic coarctation repair at the age of 5 years was referred for routine evaluation. On clinical examination, blood pressures were symmetric in the upper extremities, but the pedal pulses were weak. A transthoracic echocardiogram was normal. A contrast-enhanced 64-slice thoracic CT scan, performed to assess the coarctation repair, showed slight aortic dilatation proximal to the coarctation with some tortuosity but no restenosis. However, an aberrant left pulmonary vein was incidentally noted (Fig. 1). This aberrant pulmonary vein failed to enter the left atrium at the expected level (Fig. 1A) and traversed behind the right pulmonary artery and joined the right upper pulmonary venous system to drain into the left atrium (Fig. 1B–D, arrows; Supplementary Video 1). Anomalous unilateral pulmonary vein is an exceedingly rare, benign abnormality characterized by tortuous pulmonary venous drainage to the left atrium. The malformation is often discovered incidentally on a chest Supplementary material for this article may be found at http://www. CardiacCTjournal.com. Conflict of interest: The authors report no conflicts of interest. * Corresponding author. E-mail address: [email protected] Submitted March 30, 2008. Accepted for publication June 2, 2008.
radiograph, where it may be mistaken for a pulmonary arteriovenous malformation or Scimitar sign.1,2 Associated abnormalities include pulmonary arterial hypoplasia, lung bronchiectasis or hypoplasia, cor triatriatum, and diaphragmatic eventration.2,3 This is the first reported case of anomalous unilateral pulmonary circulation associated with aortic coarctation. Recognition of this rare entity and differentiation from Scimitar syndrome and pulmonary arteriovenous malformation are important because, despite the tortuous course of the pulmonary vein, the arterialized blood returns to the left atrium. Consequently, there is no hemodynamic shunt, and no therapeutic intervention is required.
References 1. Hanson JM, Wood AM, Seymour R, Petheram IS: Anomalous unilateral single pulmonary vein: two cases mimicking arteriovenous malformations and a review of the literature. Australas Radiol. 2005;49:246 –51. 2. Rey C, Vaksmann G, Francart CL: Anomalous unilateral single pulmonary vein mimicking partial anomalous pulmonary venous return. Cathet Cardiovasc Diagn. 1986;12:330 –3. 3. vanWassenaer AG, Lubbers LJ, Losekoo G: Partial abnormal pulmonary venous return in Turner syndrome. Eur J Pediatr. 1988;148: 101–3.
1934-5925/$ -see front matter © 2008 Society of Cardiovascular Computed Tomography. All rights reserved. doi:10.1016/j.jcct.2008.06.004
Kakouros et al
Anomalous pulmonary vein with aortic coarctation
275
Figure 1 (A) Curved multiplanar reconstruction along the length of the anomalous pulmonary vein. It arises as a pulmonary confluence in the left lung (arrow on right side of image), traversing behind the heart (arrowhead) and wrapping over the pulmonary artery (PA) to join the right pulmonary venous system. (B) Transverse section showing the posterior anomalous pulmonary vein course between the main pulmonary artery (PA) and the descending aorta (Ao). (C) Sagittal plane view with the anomalous vein marked with white arrow. The tortuosity at the site of the previous aortic coarctation repair can be also appreciated. (D) Three-dimensional volume-rendered image showing the drainage of the anomalous left pulmonary vein (arrow) into the right upper pulmonary vein (arrowhead) that subsequently drains into the left atrium.