Heart, Lung and Circulation (2014) 23, e136–e138

1443-9506/04/$36.00

http://dx.doi.org/10.1016/j.hlc.2013.11.016

IMAGE

Giant Superior Vena-ca

va Aneurysm after Glenn Surgery: A New Complication ofthe Glenn Procedure

Aashrayata Aryal Pandit, MDa, Jorge R. Alegria, MDa, Anil Pandit, MDa,Martina Mookadam, MDb, Farouk Mookadam, MDa*

aDivision of Cardiovascular Diseases, Mayo Clinic, ArizonabDepartment of Family Practice, Mayo Clinic, Arizona

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

IntroductionAneurysm 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 ReportA 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,

© 2014 Australian and New Zealand Society of Cardiac and Thoracic Surgeons (ANZSCTS) a

Inc. All rights reserved.

* Corresponding author. Mayo Clinic Arizona, 13400 E Shea Blvd, Scottsdale, AZ 85

Email: mookadam.farouk@mayo.edu

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 instan-

taneous Doppler gradient of 71 mm Hg. The superior vena

cava (SVC) was dilated measuring 6 cm. Computed tomo-

graphic (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 trans-

plant evaluation.

DiscussionThe 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

nd the Cardiac Society of Australia and New Zealand (CSANZ). Published by Elsevier

259. Tel.: +480 301 8000; fax: +480 301 8018,

Figure 1 The diagram showing SVC aneurysm measur-ing about 6 cm with double out-let right ventricle, ven-tricular septal defect. The number represents pressurerecordings during right and left heart catheterisation.The arrows indicate course of catheters.

Figure 2 A, chest X ray showing abnormal cardiac silhouette

Computed tomographic (CT) angiogram demonstrating severely

Giant Superior Vena-cava Aneurysm e137

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 mal-

formations, and hepatic dysfunction with hepatocellular car-

cinoma [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 aneur-

ysms 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.

that was initially assumed to be right-sided aortic arch. dilated SVC on Glenn connection (Arrows, Panel B and C).

Figure 3 A, computed tomographic (CT) angiogram demonstrating clear connection of superior venacava (SVC) with rightpulmonary artery (RPA). B, saggital section of CT angiogram showing origin of aorta (Ao) from the single ventricle.

e138 A.A. Pandit et al.

Conflict of InterestAuthors declare that they have no conflict of interests.

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