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Complete anomalous pulmonary venous return of the left lung—A variant of partial anomalous pulmonary venous return and unusual cause of pulmonary plethora presenting in later life
European Journal of Radiology Extra 76 (2010) e11–e13
Contents lists available at ScienceDirect
European Journal of Radiology Extra journal homepage: intl.elsevierhealth.com/journals/ejrex
Complete anomalous pulmonary venous return of the left lung—A variant of partial anomalous pulmonary venous return and unusual cause of pulmonary plethora presenting in later life Charles Ross Tapping, Gerard R. Avery ∗ Department of Radiology, Castle Hill Hospital, Castle Road, Cottingham, East Yorkshire HU16 5QJ, United Kingdom
a r t i c l e
i n f o
Article history: Received 4 May 2010 Received in revised form 29 July 2010 Accepted 2 August 2010 Keywords: Partial anomalous pulmonary venous return Thoracic vascular anomalies
a b s t r a c t We present a case of a 66-year-old woman with cough and palpitations who was found to have partial anomalous pulmonary venous return (PAPVR) of the left lung which drained into the left brachiocephalic vein. This is the first case of such an extensive asymptomatic PAPVR presenting in later life. This case demonstrates the importance of cross-sectional imaging in defining the spectrum of PAPVR and other vascular anomalies of the thorax. This case raises the possibility of a PAPVR as the location for a malpositioned central venous catheter tip lying in a left para-vertebral location. © 2010 Elsevier Ireland Ltd. All rights reserved.
Partial anomalous pulmonary venous return (PAPVR) is rare and is classically described as presenting in infancy [1] most often on the right side [2] and associated with a sinus venosus atrial septal defect [3]. There are limited cases of adults who present with the condition and most reports involve single lobe draining vessels, right sided lesions or Scimitar syndrome [1,4]. To our knowledge there has only been one case report describing complete drainage of a lung via a PAPRV into the systemic circulation and this focused on the surgical treatment of the vessel in a 29-year-old man [5]. This case is unusual, as the patient remained asymptomatic with such an extensive anomalous vessel until later life. We present the initial chest X-ray findings and CT findings of a PAPVR draining their entire left lung, inserting into the left brachiocephalic vein with no connection to the left atrium. We also emphasise the fact that clues to this unusual lesion being detected were present on the initial chest X-ray. 1. Case report A 66-year-old non-smoking woman under investigation for cough and palpitations presented to our department for a contrast enhanced CT (CECT) following the discovery of increased pulmonary vasculature on her chest X-ray. The referring team queried a respiratory cause for the findings and as such a non-cardiac gated CT was performed. Retrospective review of the initial chest X-ray
∗ Corresponding author. Tel.: +44 01482 875875. E-mail address: [email protected] (G.R. Avery). 1571-4675/$ – see front matter © 2010 Elsevier Ireland Ltd. All rights reserved. doi:10.1016/j.ejrex.2010.08.002
following the CT revealed that there was a linear opacity lateral to the descending aorta (Fig. 1). The CECT (Philips Brilliance 40 slice) revealed the left lung venous drainage was entirely to the left brachiocephalic vein. Transverse images (Fig. 2) and volume rendered image (Fig. 3a) demonstrated that the anomalous vessel passed adjacent to the left mediastinal border and close to, but was clearly separate from the left atrium. The left upper, lingula and lower lobe pulmonary veins joined this vessel which drained into the left brachiocephalic vein (Fig. 3b). The pulmonary arteries were grossly dilated, the heart was mildly enlarged but its position was normal. The remaining thoracic vasculature and upper abdominal viscera were normal. The cardiologist examining her subsequently noted a soft systolic murmur. Transthoracic echocardiography showed increased pulmonary artery pressure (PAP) and right heart dilatation and electrocardiogram (ECG) revealed partial right bundle branch block with no pre-excitation. 24-h blood pressure monitoring was normal (BP range 180/100–90/60 and pulse range 94–54, mean 65 beat per minute) and 24-h ECG monitoring diagnosed paroxysmal atrial fibrillation. She was treated with Bisoprolol 2.5 mg daily and Aspirin 75 mg daily. Routine blood tests were all within normal limits. Her symptoms were initially well controlled with the beta-blocker but further invasive tests were carried out to examine the extent of the shunt. Transoesophageal echocardiography (TOE) and diagnostic cardiac angiography revealed no atrial septal defect (ASD) was present. Invasive cardiac catheterisation was performed to assess the pulmonary pressures and shunt. The pulmonary artery pressure was 52 mm Hg systolic, 16 mm Hg diastolic with a mean of 32 mm Hg
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C.R. Tapping, G.R. Avery / European Journal of Radiology Extra 76 (2010) e11–e13
(right ventricular pressure was 48 mm Hg systolic and 5 mm Hg diastolic). The pulmonary artery wedge pressure was 21 mm Hg. The left-to-right shunt was 3.7 L/min. Due to the high pressures she was listed for surgical correction.
2. Discussion
Fig. 1. Initial PA chest X-ray performed demonstrating pulmonary plethora and a linear opacity lateral to the descending aorta (arrows).
PAPVR consists of a variety of vascular developmental malformations in which blood is returned to the right atrium or its tributaries instead of the left atrium, creating a left-to-right shunt [6]. The most common sites for insertion of the anomalous vein are the superior vena cava (SVC), right atrium, inferior vena cave (IVC) and coronary sinus [7]. The most recent citation reporting prevalence of the condition suggests it affects approximately 0.2% of the population [1]. Authors disagree whether or not it occurs most commonly on the left side (79%) [1] or on the right side (90%) [3]. It is twice as common in males than in females [4], and the vast majority (80–90%) are associated with atrial septal defects of either the sinus venosus or secundum type [3,8]. The largest series in adult patients found that 79% had a left upper lobe, 17% right upper lobe
Fig. 2. CT transverse images demonstrating the anomalous vessel, dilated right side of heart and dilated pulmonary arteries (window width 400, level 40): (a) left upper lobe vessels draining into anomalous vessel and (b) extent of right heart enlargement.
Fig. 3. Volume rendered images following removal of the heart and enlarged pulmonary arteries demonstrating the extent of the anomalous vessel: (a) connection of anomalous vessel to left brachiocephalic vein (superiorly to the brachiocephalic vein) and (b) the left pulmonary drainage following removal of all thoracic structures.
C.R. Tapping, G.R. Avery / European Journal of Radiology Extra 76 (2010) e11–e13
and 3% right lower lobe PAPVR [1]. There were no occurrences of multiple lobe shunts as in this case. The clinical signs and symptoms of PAPVR are related to the degree of left-to-right shunt, the presence and size of an ASD which makes the shunt more complex and potentially greater and, associated cardiorespiratory abnormalities. These factors also predict the age at presentation and the need for further intervention. Single lobe shunts usually results in asymptomatic shunting, whereas larger anomalous vessels can result in significant shunting and symptoms. Associated comorbidities especially if they involve the cardiorespiratory system, such as an ASD, may cause patients with previously asymptomatic shunts to become symptomatic. Thus, whilst in this case there was a large anomalous vessel the lack of ASD and other cardiovascular comorbidities meant that this patient remained asymptomatic until 66 years of age. Features that distinguish a left sided SVC versus PAPVR have been well described [9] but in this case there was no connection of the extensive anomalous vein to any cardiac structure. Authors have also emphasized that the paucity of CT reports describing PAPVR mean it may often go undetected, misdiagnosed or be overlooked [9,10]. In this case the initial chest X-ray reporter had missed the linear opacity lateral to the descending aorta and the clinicians had therefore concentrated on searching for a respiratory cause for her symptoms. This case reinforces the attention to detail required when interpreting chest X-rays and although this patient did have the condition diagnosed on her CT a more appropriate CT investigation could have been performed initially. Whilst there are more
e13
common causes for a central venous catheter tip lying in a left paravertebral location, such as a left sided SVS or superior intercostal vein for example, this case raises the possibility of a PAPVR as an alternative site of inadvertent catheterisation. References [1] Haramati LB, Moche IE, Rivera VT, et al. Computed tomography of partial anomalous pulmonary venous connection in adults. J Comput Assist Tomogr 2003;27:743–9. [2] Brody H. Drainage of the pulmonary veins into the right side of the heart. Arch Pathol 1942;33:221–40. [3] Senocak F, Ozme S, Bilgic¸ A, Ozkutlu S, Ozer S, Sarac¸lar M. Partial anomalous pulmonary venous return. Evaluation of 51 cases. Jpn Heart J 1994;35: 43–50. [4] Snellen HA, van Ingen HC, Hoefsmit EC. Patterns of anomalous pulmonary venous drainage. Circulation 1968;38:45–63. [5] Cooley DA, Mahaffey DE. Anomalous pulmonary venous drainage of entire left lung: report of case with surgical correction. Ann Surg 1955;142:986–91. [6] Ferguson EC, Krishnamurthy R, Oldham SA. Classic imaging signs of congenital cardiovascular abnormalities. Radiographics 2007;27:1323–34. [7] Burney K, Young H, Barnard SA, McCoubrie P, Darby M. CT appearances of congential and acquired abnormalities of the superior vena cava. Clin Radiol 2007;62:837–42. [8] Toyoshima M, Sato A, Fukumoto Y, et al. Partial anomalous pulmonary venous return showing anomalous venous return to the azygos vein. Intern Med 1992;31:1112–6. [9] Dillon EH, Camputaro C. Partial anomalous pulmonary venous drainage of the left upper lobe vs duplication of the superior vena cava: distinction based on CT findings. AJR Am J Roentgenol 1993;160:375–9. [10] Pennes DR, Ellis JH. Anomalous pulmonary venous drainage of the left upper lobe shown by CT scans. Radiology 1986;159:23–4.
Contents lists available at ScienceDirect
European Journal of Radiology Extra journal homepage: intl.elsevierhealth.com/journals/ejrex
Complete anomalous pulmonary venous return of the left lung—A variant of partial anomalous pulmonary venous return and unusual cause of pulmonary plethora presenting in later life Charles Ross Tapping, Gerard R. Avery ∗ Department of Radiology, Castle Hill Hospital, Castle Road, Cottingham, East Yorkshire HU16 5QJ, United Kingdom
a r t i c l e
i n f o
Article history: Received 4 May 2010 Received in revised form 29 July 2010 Accepted 2 August 2010 Keywords: Partial anomalous pulmonary venous return Thoracic vascular anomalies
a b s t r a c t We present a case of a 66-year-old woman with cough and palpitations who was found to have partial anomalous pulmonary venous return (PAPVR) of the left lung which drained into the left brachiocephalic vein. This is the first case of such an extensive asymptomatic PAPVR presenting in later life. This case demonstrates the importance of cross-sectional imaging in defining the spectrum of PAPVR and other vascular anomalies of the thorax. This case raises the possibility of a PAPVR as the location for a malpositioned central venous catheter tip lying in a left para-vertebral location. © 2010 Elsevier Ireland Ltd. All rights reserved.
Partial anomalous pulmonary venous return (PAPVR) is rare and is classically described as presenting in infancy [1] most often on the right side [2] and associated with a sinus venosus atrial septal defect [3]. There are limited cases of adults who present with the condition and most reports involve single lobe draining vessels, right sided lesions or Scimitar syndrome [1,4]. To our knowledge there has only been one case report describing complete drainage of a lung via a PAPRV into the systemic circulation and this focused on the surgical treatment of the vessel in a 29-year-old man [5]. This case is unusual, as the patient remained asymptomatic with such an extensive anomalous vessel until later life. We present the initial chest X-ray findings and CT findings of a PAPVR draining their entire left lung, inserting into the left brachiocephalic vein with no connection to the left atrium. We also emphasise the fact that clues to this unusual lesion being detected were present on the initial chest X-ray. 1. Case report A 66-year-old non-smoking woman under investigation for cough and palpitations presented to our department for a contrast enhanced CT (CECT) following the discovery of increased pulmonary vasculature on her chest X-ray. The referring team queried a respiratory cause for the findings and as such a non-cardiac gated CT was performed. Retrospective review of the initial chest X-ray
∗ Corresponding author. Tel.: +44 01482 875875. E-mail address: [email protected] (G.R. Avery). 1571-4675/$ – see front matter © 2010 Elsevier Ireland Ltd. All rights reserved. doi:10.1016/j.ejrex.2010.08.002
following the CT revealed that there was a linear opacity lateral to the descending aorta (Fig. 1). The CECT (Philips Brilliance 40 slice) revealed the left lung venous drainage was entirely to the left brachiocephalic vein. Transverse images (Fig. 2) and volume rendered image (Fig. 3a) demonstrated that the anomalous vessel passed adjacent to the left mediastinal border and close to, but was clearly separate from the left atrium. The left upper, lingula and lower lobe pulmonary veins joined this vessel which drained into the left brachiocephalic vein (Fig. 3b). The pulmonary arteries were grossly dilated, the heart was mildly enlarged but its position was normal. The remaining thoracic vasculature and upper abdominal viscera were normal. The cardiologist examining her subsequently noted a soft systolic murmur. Transthoracic echocardiography showed increased pulmonary artery pressure (PAP) and right heart dilatation and electrocardiogram (ECG) revealed partial right bundle branch block with no pre-excitation. 24-h blood pressure monitoring was normal (BP range 180/100–90/60 and pulse range 94–54, mean 65 beat per minute) and 24-h ECG monitoring diagnosed paroxysmal atrial fibrillation. She was treated with Bisoprolol 2.5 mg daily and Aspirin 75 mg daily. Routine blood tests were all within normal limits. Her symptoms were initially well controlled with the beta-blocker but further invasive tests were carried out to examine the extent of the shunt. Transoesophageal echocardiography (TOE) and diagnostic cardiac angiography revealed no atrial septal defect (ASD) was present. Invasive cardiac catheterisation was performed to assess the pulmonary pressures and shunt. The pulmonary artery pressure was 52 mm Hg systolic, 16 mm Hg diastolic with a mean of 32 mm Hg
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C.R. Tapping, G.R. Avery / European Journal of Radiology Extra 76 (2010) e11–e13
(right ventricular pressure was 48 mm Hg systolic and 5 mm Hg diastolic). The pulmonary artery wedge pressure was 21 mm Hg. The left-to-right shunt was 3.7 L/min. Due to the high pressures she was listed for surgical correction.
2. Discussion
Fig. 1. Initial PA chest X-ray performed demonstrating pulmonary plethora and a linear opacity lateral to the descending aorta (arrows).
PAPVR consists of a variety of vascular developmental malformations in which blood is returned to the right atrium or its tributaries instead of the left atrium, creating a left-to-right shunt [6]. The most common sites for insertion of the anomalous vein are the superior vena cava (SVC), right atrium, inferior vena cave (IVC) and coronary sinus [7]. The most recent citation reporting prevalence of the condition suggests it affects approximately 0.2% of the population [1]. Authors disagree whether or not it occurs most commonly on the left side (79%) [1] or on the right side (90%) [3]. It is twice as common in males than in females [4], and the vast majority (80–90%) are associated with atrial septal defects of either the sinus venosus or secundum type [3,8]. The largest series in adult patients found that 79% had a left upper lobe, 17% right upper lobe
Fig. 2. CT transverse images demonstrating the anomalous vessel, dilated right side of heart and dilated pulmonary arteries (window width 400, level 40): (a) left upper lobe vessels draining into anomalous vessel and (b) extent of right heart enlargement.
Fig. 3. Volume rendered images following removal of the heart and enlarged pulmonary arteries demonstrating the extent of the anomalous vessel: (a) connection of anomalous vessel to left brachiocephalic vein (superiorly to the brachiocephalic vein) and (b) the left pulmonary drainage following removal of all thoracic structures.
C.R. Tapping, G.R. Avery / European Journal of Radiology Extra 76 (2010) e11–e13
and 3% right lower lobe PAPVR [1]. There were no occurrences of multiple lobe shunts as in this case. The clinical signs and symptoms of PAPVR are related to the degree of left-to-right shunt, the presence and size of an ASD which makes the shunt more complex and potentially greater and, associated cardiorespiratory abnormalities. These factors also predict the age at presentation and the need for further intervention. Single lobe shunts usually results in asymptomatic shunting, whereas larger anomalous vessels can result in significant shunting and symptoms. Associated comorbidities especially if they involve the cardiorespiratory system, such as an ASD, may cause patients with previously asymptomatic shunts to become symptomatic. Thus, whilst in this case there was a large anomalous vessel the lack of ASD and other cardiovascular comorbidities meant that this patient remained asymptomatic until 66 years of age. Features that distinguish a left sided SVC versus PAPVR have been well described [9] but in this case there was no connection of the extensive anomalous vein to any cardiac structure. Authors have also emphasized that the paucity of CT reports describing PAPVR mean it may often go undetected, misdiagnosed or be overlooked [9,10]. In this case the initial chest X-ray reporter had missed the linear opacity lateral to the descending aorta and the clinicians had therefore concentrated on searching for a respiratory cause for her symptoms. This case reinforces the attention to detail required when interpreting chest X-rays and although this patient did have the condition diagnosed on her CT a more appropriate CT investigation could have been performed initially. Whilst there are more
e13
common causes for a central venous catheter tip lying in a left paravertebral location, such as a left sided SVS or superior intercostal vein for example, this case raises the possibility of a PAPVR as an alternative site of inadvertent catheterisation. References [1] Haramati LB, Moche IE, Rivera VT, et al. Computed tomography of partial anomalous pulmonary venous connection in adults. J Comput Assist Tomogr 2003;27:743–9. [2] Brody H. Drainage of the pulmonary veins into the right side of the heart. Arch Pathol 1942;33:221–40. [3] Senocak F, Ozme S, Bilgic¸ A, Ozkutlu S, Ozer S, Sarac¸lar M. Partial anomalous pulmonary venous return. Evaluation of 51 cases. Jpn Heart J 1994;35: 43–50. [4] Snellen HA, van Ingen HC, Hoefsmit EC. Patterns of anomalous pulmonary venous drainage. Circulation 1968;38:45–63. [5] Cooley DA, Mahaffey DE. Anomalous pulmonary venous drainage of entire left lung: report of case with surgical correction. Ann Surg 1955;142:986–91. [6] Ferguson EC, Krishnamurthy R, Oldham SA. Classic imaging signs of congenital cardiovascular abnormalities. Radiographics 2007;27:1323–34. [7] Burney K, Young H, Barnard SA, McCoubrie P, Darby M. CT appearances of congential and acquired abnormalities of the superior vena cava. Clin Radiol 2007;62:837–42. [8] Toyoshima M, Sato A, Fukumoto Y, et al. Partial anomalous pulmonary venous return showing anomalous venous return to the azygos vein. Intern Med 1992;31:1112–6. [9] Dillon EH, Camputaro C. Partial anomalous pulmonary venous drainage of the left upper lobe vs duplication of the superior vena cava: distinction based on CT findings. AJR Am J Roentgenol 1993;160:375–9. [10] Pennes DR, Ellis JH. Anomalous pulmonary venous drainage of the left upper lobe shown by CT scans. Radiology 1986;159:23–4.