IMAGE
Heart, Lung and Circulation (2014) 23, e136–e138 1443-9506/04/$36.00 http://dx.doi.org/10.1016/j.hlc.2013.11.016
Giant Superior Vena-cava Aneurysm after Glenn Surgery: A New Complication of the Glenn Procedure Aashrayata Aryal Pandit, MDa, Jorge R. Alegria, MDa, Anil Pandit, MDa, Martina Mookadam, MDb, Farouk Mookadam, MDa* a
Division of Cardiovascular Diseases, Mayo Clinic, Arizona Department of Family Practice, Mayo Clinic, Arizona
b
Received 31 August 2013; received in revised form 11 November 2013; accepted 17 November 2013; online published-ahead-of-print 24 January 2014
Keywords
Superior vena cava dilatation Glenn surgery Congenital heart disease Shortness of breath Right sided aortic arch
Introduction Aneurysm of systemic veins is uncommon with only 30 cases reported in literature [1]. Aneurysms of the inferior vena cava have also been rarely reported [2]. The most common cause of SVC aneurysm is congenital or surgical weakness in the longitudinal muscles of SVC wall [3]. We present a case of a 27 year-old patient presenting with SVC aneurysm 13 years after a Glenn surgical procedure.
Case Report A 27 year-old male presented with chest tightness, shortness of breath on exertion, and palpitations. He had a history of double outlet right ventricle with transposition of the great vessels, sub pulmonic ventricular septal defect, membranous septal defect, superior vena cava to right pulmonic artery anastomosis (Bidirectional Glenn), atrial septostomy, right pulmonary arterioplasty 13 years earlier (Fig. 1). A pacemaker was placed for high-grade AV block. He had New York Heart Association (NYHA) class III heart failure and was on ACE-inhibitors and beta-blockers. Chest X ray showed an abnormal cardiac silhouette that was initially assumed to be right-sided aortic arch (Fig. 2, Arrow,
Panel A). A transthoracic echocardiography showed a left ventricular ejection fraction of 26%, mild left ventricular enlargement, severe decrease in right ventricular function, multiple ventricular septal defects, left to right shunt at atrial level, severe pulmonary valve stenosis with maximal instantaneous Doppler gradient of 71 mm Hg. The superior vena cava (SVC) was dilated measuring 6 cm. Computed tomographic (CT) angiogram confirmed the findings of the TTE demonstrating a severely dilated SVC at the site of the Glenn anastomosis (Fig. 2 Arrows, Panel B and C). Further, Fig. 3A demonstrates connection of superior vena cava (SVC) with right pulmonary artery (RPA) and Fig. 3B shows aorta (Ao) arising from single ventricle (V). The patient underwent both right and left heart catheterisation and findings are tabulated in Fig. 1. The patient is currently undergoing a cardiac transplant evaluation.
Discussion The Glenn procedure involves connection of superior vena cava to the pulmonary artery as a palliative procedure in many congenital heart diseases, including patients with a single ventricle as presented. The classical Glenn surgery involves a unidirectional connection of the right pulmonary
* Corresponding author. Mayo Clinic Arizona, 13400 E Shea Blvd, Scottsdale, AZ 85259. Tel.: +480 301 8000; fax: +480 301 8018, Email: [email protected] © 2014 Australian and New Zealand Society of Cardiac and Thoracic Surgeons (ANZSCTS) and the Cardiac Society of Australia and New Zealand (CSANZ). Published by Elsevier Inc. All rights reserved.
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artery to the superior vena cava and the blood from the SVC will flow only to right lung, because the connection of right pulmonary artery to the main pulmonary artery is severed and sewn. More commonly a bidirectional Glenn procedure is performed, also known as modified Glenn procedure as described in our case. The modified Glenn procedure involves connecting the SVC with right pulmonary artery and the venous blood freely flows through right and left pulmonary artery. The usual potential complications after this surgery are many and involve arrhythmias, protein losing enteropathy, cyanosis, pulmonary arteriovenous malformations, and hepatic dysfunction with hepatocellular carcinoma [4]. Moreover, aneurysm of SVC after bidirectional Glenn is extremely rare with only one case reported in the literature [5]. The natural history of superior vena cava aneurysms is unknown. Review of the literature suggests that SVC aneurysms can cause pulmonary embolism and thrombosis [6]. The exact pathogenesis of SVC aneurysm is not known and possibility of deficiency of longitudinal fibers has been reported [7]. The management of SVC aneurysms is not clearly defined in literature particularly in patients after a Glenn shunt. Figure 1 The diagram showing SVC aneurysm measuring about 6 cm with double out-let right ventricle, ventricular septal defect. The number represents pressure recordings during right and left heart catheterisation. The arrows indicate course of catheters.
Figure 2 A, chest X ray showing abnormal cardiac silhouette that was initially assumed to be right-sided aortic arch. Computed tomographic (CT) angiogram demonstrating severely dilated SVC on Glenn connection (Arrows, Panel B and C).
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Figure 3 A, computed tomographic (CT) angiogram demonstrating clear connection of superior venacava (SVC) with right pulmonary artery (RPA). B, saggital section of CT angiogram showing origin of aorta (Ao) from the single ventricle.
Conflict of Interest Authors declare that they have no conflict of interests.
References [1] Enright TR, Kanne JP. Saccular superior vena cava aneurysm - incidental diagnosis by MDCT. Clin Radiol 2010;65(5 May):421–2. [2] Mookadam F, Rowley VB, Emani UR, Al-Harthi MS, Baxter CM, Wilansky S, et al. Aneurysmal dilatation of the inferior vena cava. Echocardiography 2011;28(8 Sep):833–42. [3] Ream CR, Giardina A. Congenital superior vena cava aneurysm with complications caused by infectious mononucleosis. Chest 1972;62(6 Dec): 755–7.
[4] Asrani SK, Warnes CA, Kamath PS. Hepatocellular carcinoma after the Fontan procedure. N Engl J Med [Case Reports Letter] 2013;368(18 May 2): 1756–7. [5] Panduranga P, Thomas E, Al-Maskari S, Al-Farqani A. Giant superior vena caval aneurysm in a post-Glenn patient. Interact Cardiovasc Thorac Surg 2012;14(6 Jun):878–9. [6] Oh SG, Kim KH, Seon HJ, Yoon HJ, Ahn Y, Jeong MH, et al. Unusual cause of acute right ventricular dysfunction: rapid progression of superior vena cava aneurysm complicated by thrombosis and pulmonary thromboembolism. J Korean Med Sci 2011;26(5 May):690–3. [7] Varma PK, Dharan BS, Ramachandran P, Neelakandhan KS. Superior vena caval aneurysm. Interact Cardiovasc Thorac Surg 2003;2(3 Sep): 331–3.
Heart, Lung and Circulation (2014) 23, e136–e138 1443-9506/04/$36.00 http://dx.doi.org/10.1016/j.hlc.2013.11.016
Giant Superior Vena-cava Aneurysm after Glenn Surgery: A New Complication of the Glenn Procedure Aashrayata Aryal Pandit, MDa, Jorge R. Alegria, MDa, Anil Pandit, MDa, Martina Mookadam, MDb, Farouk Mookadam, MDa* a
Division of Cardiovascular Diseases, Mayo Clinic, Arizona Department of Family Practice, Mayo Clinic, Arizona
b
Received 31 August 2013; received in revised form 11 November 2013; accepted 17 November 2013; online published-ahead-of-print 24 January 2014
Keywords
Superior vena cava dilatation Glenn surgery Congenital heart disease Shortness of breath Right sided aortic arch
Introduction Aneurysm of systemic veins is uncommon with only 30 cases reported in literature [1]. Aneurysms of the inferior vena cava have also been rarely reported [2]. The most common cause of SVC aneurysm is congenital or surgical weakness in the longitudinal muscles of SVC wall [3]. We present a case of a 27 year-old patient presenting with SVC aneurysm 13 years after a Glenn surgical procedure.
Case Report A 27 year-old male presented with chest tightness, shortness of breath on exertion, and palpitations. He had a history of double outlet right ventricle with transposition of the great vessels, sub pulmonic ventricular septal defect, membranous septal defect, superior vena cava to right pulmonic artery anastomosis (Bidirectional Glenn), atrial septostomy, right pulmonary arterioplasty 13 years earlier (Fig. 1). A pacemaker was placed for high-grade AV block. He had New York Heart Association (NYHA) class III heart failure and was on ACE-inhibitors and beta-blockers. Chest X ray showed an abnormal cardiac silhouette that was initially assumed to be right-sided aortic arch (Fig. 2, Arrow,
Panel A). A transthoracic echocardiography showed a left ventricular ejection fraction of 26%, mild left ventricular enlargement, severe decrease in right ventricular function, multiple ventricular septal defects, left to right shunt at atrial level, severe pulmonary valve stenosis with maximal instantaneous Doppler gradient of 71 mm Hg. The superior vena cava (SVC) was dilated measuring 6 cm. Computed tomographic (CT) angiogram confirmed the findings of the TTE demonstrating a severely dilated SVC at the site of the Glenn anastomosis (Fig. 2 Arrows, Panel B and C). Further, Fig. 3A demonstrates connection of superior vena cava (SVC) with right pulmonary artery (RPA) and Fig. 3B shows aorta (Ao) arising from single ventricle (V). The patient underwent both right and left heart catheterisation and findings are tabulated in Fig. 1. The patient is currently undergoing a cardiac transplant evaluation.
Discussion The Glenn procedure involves connection of superior vena cava to the pulmonary artery as a palliative procedure in many congenital heart diseases, including patients with a single ventricle as presented. The classical Glenn surgery involves a unidirectional connection of the right pulmonary
* Corresponding author. Mayo Clinic Arizona, 13400 E Shea Blvd, Scottsdale, AZ 85259. Tel.: +480 301 8000; fax: +480 301 8018, Email: [email protected] © 2014 Australian and New Zealand Society of Cardiac and Thoracic Surgeons (ANZSCTS) and the Cardiac Society of Australia and New Zealand (CSANZ). Published by Elsevier Inc. All rights reserved.
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artery to the superior vena cava and the blood from the SVC will flow only to right lung, because the connection of right pulmonary artery to the main pulmonary artery is severed and sewn. More commonly a bidirectional Glenn procedure is performed, also known as modified Glenn procedure as described in our case. The modified Glenn procedure involves connecting the SVC with right pulmonary artery and the venous blood freely flows through right and left pulmonary artery. The usual potential complications after this surgery are many and involve arrhythmias, protein losing enteropathy, cyanosis, pulmonary arteriovenous malformations, and hepatic dysfunction with hepatocellular carcinoma [4]. Moreover, aneurysm of SVC after bidirectional Glenn is extremely rare with only one case reported in the literature [5]. The natural history of superior vena cava aneurysms is unknown. Review of the literature suggests that SVC aneurysms can cause pulmonary embolism and thrombosis [6]. The exact pathogenesis of SVC aneurysm is not known and possibility of deficiency of longitudinal fibers has been reported [7]. The management of SVC aneurysms is not clearly defined in literature particularly in patients after a Glenn shunt. Figure 1 The diagram showing SVC aneurysm measuring about 6 cm with double out-let right ventricle, ventricular septal defect. The number represents pressure recordings during right and left heart catheterisation. The arrows indicate course of catheters.
Figure 2 A, chest X ray showing abnormal cardiac silhouette that was initially assumed to be right-sided aortic arch. Computed tomographic (CT) angiogram demonstrating severely dilated SVC on Glenn connection (Arrows, Panel B and C).
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Figure 3 A, computed tomographic (CT) angiogram demonstrating clear connection of superior venacava (SVC) with right pulmonary artery (RPA). B, saggital section of CT angiogram showing origin of aorta (Ao) from the single ventricle.
Conflict of Interest Authors declare that they have no conflict of interests.
References [1] Enright TR, Kanne JP. Saccular superior vena cava aneurysm - incidental diagnosis by MDCT. Clin Radiol 2010;65(5 May):421–2. [2] Mookadam F, Rowley VB, Emani UR, Al-Harthi MS, Baxter CM, Wilansky S, et al. Aneurysmal dilatation of the inferior vena cava. Echocardiography 2011;28(8 Sep):833–42. [3] Ream CR, Giardina A. Congenital superior vena cava aneurysm with complications caused by infectious mononucleosis. Chest 1972;62(6 Dec): 755–7.
[4] Asrani SK, Warnes CA, Kamath PS. Hepatocellular carcinoma after the Fontan procedure. N Engl J Med [Case Reports Letter] 2013;368(18 May 2): 1756–7. [5] Panduranga P, Thomas E, Al-Maskari S, Al-Farqani A. Giant superior vena caval aneurysm in a post-Glenn patient. Interact Cardiovasc Thorac Surg 2012;14(6 Jun):878–9. [6] Oh SG, Kim KH, Seon HJ, Yoon HJ, Ahn Y, Jeong MH, et al. Unusual cause of acute right ventricular dysfunction: rapid progression of superior vena cava aneurysm complicated by thrombosis and pulmonary thromboembolism. J Korean Med Sci 2011;26(5 May):690–3. [7] Varma PK, Dharan BS, Ramachandran P, Neelakandhan KS. Superior vena caval aneurysm. Interact Cardiovasc Thorac Surg 2003;2(3 Sep): 331–3.
