Download Atrial Baffle Stenosis: A Late Complication after Mustard Repair for d

APRIL 2013
ISSUE 55
Atrial Baffle Stenosis: A Late Complication after Mustard Repair for d-TGA
Ali D. Karaosmanoglu,MD; Suhny Abbara, MD; Sanjeev Francis, MD; Doreen DeFaria Yeh, MD; and Godtfred Holmvang, MD
Clinical History
A 37 year old male with a history of d-transposition of the great arteries
and Mustard atrial switch operation as an infant presented with abdominal
distension and facial swelling.
Cardiac MRI and CT were performed for evaluation of the atrial baffle and
biventricular function.
Findings
The Cardiac MRI demonstrated significant stenosis at the superior and
inferior limbs of the baffle (figure 1). The atrial baffle path for pulmonary
vein flow was widely patent. The systemic right ventricle was hypertrophied
with low normal systolic function (RVEF=45%).
Cardiac CT demonstrated an anomalous left circumflex artery arising from
a common trunk with the right coronary artery from the posterior sinus
of Valsalva and anteriorly located ascending aorta relative to the main
pulmonary artery (figure 2). Ascites and liver cirrhosis due to chronic right
atrial hypertension were also visualized.
The patient underwent cardiac catheterization followed by successful
angioplasty and stenting of the stenotic segments in the atrial baffle. Post
procedure, his neck fullness resolved and ascites markedly improved.
Discussion
D-transposition of the great arteries (d-TGA) refers to the dextroposition
of the bulboventricular loop and ventriculoarterial discordance (1). Due
to failure of spiral septation of the truncus arteriosus, the great arteries
course parallel to each other rather than crossing. The aorta arises from
the morphologic right ventricle and the main pulmonary artery from the
morphologic left ventricle. Communication between the pulmonary and
systemic circuits is necessary for infant survival either via an atrial septal
defect, patent ductus arteriosus or ventricular septal defect. Ventricular
septal defects are the most common associated anomaly, observed
in almost half of the cases. Pulmonary outflow tract obstruction and
coarctation of the aorta may also be seen (2).
Atrial septostomy was the original surgical procedure first performed
in the 1960s. The atrial switch procedure (Mustard or Senning types)
then became the most common surgical treatment. Right ventricular
dysfunction, tricuspid regurgitation, atrial arrhythmias and heart block are
frequently encountered in the follow-up of these patients. The arterial switch
procedure has become the preferred technique to repair d-TGA.It involves
the transection and switching of the great arteries with reimplantation of the
coronary arteries, thus restoring normal ventriculo-arterial concordance.
Baffle leaks and obstruction are common late complications of atrial switch
surgery.
Baffle leaks should be considered in patients with stroke or paradoxical
embolism and baffle obstruction should be considered in patients with
signs and symptoms of systemic venous hypertension. Percutaneous
angioplasty or surgery is the main treatment strategy in baffle obstruction.
Editors:
Suhny Abbara, MD, MGH Department of Radiology
Figure 1A
Figure 1B
Figure 2A
Figure 2B
Figure 1(A,B,C): MR images of the atrial baffle. (A) SSFP image of
the heart demonstrating superior (curved arrow) and inferior (solid
arrow) vena cavae and the baffle obstruction point (arrowhead)
(B) Baffle stenosis with a minimal luminal diameter of 4mm (arrow)
leading to obstruction of systemic venous return. (C) Volume
rendered image showing unobstructed drainage of all five pulmonary
veins into the tricuspid inflow region(RU: Right upper pulmonary
vein, RM: Right middle pulmonary vein, RL: Right lower pulmonary
vein, LU: Left upper pulmonary vein, LL: Left lower pulmonary vein,
TV Inflow: Right atrium leading to tricuspid valve).
Figure 2: Axial image showing abnormal anterior and rightward
location of the ascending aorta relative to the main pulmonary
artery. The posterior sinus of Valsalva gives rise to a right coronary
artery and left circumflex artery (arrowhead) via a common trunk.
Left pulmonary vein drains into the right atrium (arrow). AO:
Ascending aorta, MPA: Main pulmonary artery, RA: Right atrium.
REFERENCES
1. Warnes CA. Transposition of the great arteries. Circulation.
2006;114:2699-2709.
2. Hornung TS, Derrick GP, Deanfield JE, Redington AN.
Transposition complexes in the adult: a changing perspective
Cardiol Clin. 2002;20:405-420.
3. Hornung TS, Benson LN, McLaughlin PR. Catheter interventions
in adult patients with congenital heart disease. Curr Cardiol Rep.
2002 Jan;4(1):54-62.
Sanjeev A. Francis, MD, MGH Division of Cardiology