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Journal compilation © 2007, Blackwell Publishing, Inc. Congenit Heart Dis. 2007;2:265–269
Blackwell Publishing IncMalden, USACHDCongenital Heart Disease1747-079X© 2007, the Authors; Journal compilation © 2007 Blackwell Publishing, Inc.? 200724265269CASE REPORT Interventional Rerouting of Scimitar Vein to Left AtriumSingh et al.
CASE REPORTS
Interventional Rerouting of Scimitar Vein to Left Atrium Using an
Amplatzer Vascular Plug
Harminder Singh, MD, FACC, Manoj Luthra, MCh, Prashant Bharadwaj, MD, and Ravi Kumar, MD
Military Hospital, Cardio-thoracic Center, Pune, Maharashtra, India
ABSTRACT
Scimitar syndrome is a rare congenital anomaly accounting for about 0.5% of all congenital heart diseases. In its
most common form, it is characterized by anomalous drainage of one or all right side pulmonary veins into inferior
vena cava or rarely into hepatic, portal, or azygous veins. Other associated anomalies can be hypoplasia or agenesis
of right lung, hypoplasia of right pulmonary artery, bronchial anomalies, aberrant blood supply and (or) sequestra-
tion of right lung, and variety of intracardiac defects which commonly includes atrial or ventricular septal defects
and tetrology of Fallot. The usual treatment of these patients is surgical rerouting of Scimitar vein into left atrium
(LA) by baffle formation, lobectomy, embolization or ligation of aberrant blood supply and various combinations
of these. Nonsurgical and interventional treatments are rarely possible. We report a rare case where complete
rerouting of anomalous venous drainage to LA could be achieved by interventional treatment by a simple deploy-
ment of a vascular plug in combination with embolization of aberrant vascular supply to the same area, thus avoiding
surgical intervention.
Key Words. Scimitar Vein; Amplatzer Vascular Plug; Left Atrium; Pulmonary Veins; Left to Right Shunt
Case Report
he patient is a 3-year-old boy born out of
nonconsanguineous marriage, who had re-
current chest infections since infancy. Clinically he
had cardiomegaly and signs of right ventricular
volume overload and mid-systolic murmur in left
upper sternal edge for which he was referred to
our hospital. Echocardiography showed situs
solitus, levocardia, with no intracardiac defects.
There was dilatation of right atrium (RA), right
ventricle, and pulmonary arteries (PA). A contin-
uous flow was seen in inferior vena cava (IVC)
near its entry into RA. Interatrial and interventric-
ular septa were intact. Only left-sided pulmonary
veins were seen entering into left atrium (LA).
Overall picture was diagnostic of Scimitar syn-
drome; however, the Scimitar vein (SV) could not
be well profiled on echocardiography. Magnetic
resonance imaging, however, readily confirmed
the diagnosis. Magnetic resonance imaging also
revealed stenosis of the SV near its entry point
T
into IVC. The child was taken up for diagnostic
catheterization. Catheterization revealed pulmo-
nary to systemic flow ratio of 1.7:1 with a PA
pressure of 42/18 (mean 29 mm Hg) and pulmo-
nary vascular resistance of 1.9 woods/m
2
body sur-
face area. Diagnostic right PA angiogram showed
normal sized PA and during the levo-phase
showed both right upper and lower pulmonary
veins forming SV which drained into the IVC
(Figure 1A). Stenosis of the SV could be well dem-
onstrated on selective injections in SV (Figure 1B
and C). Selective angiogram also revealed a
tortuous collateral connecting SV and the LA
(Figure 1B and C). Abdominal aortic angiogram
showed an aberrant vessel supplying the basal seg-
ments of the right lower lobe. These segments had
dual supply from the pulmonary artery and
abdominal aorta.
Occlusion of the SV at the stenotic site just
before the entry point into the IVC would reroute
the pulmonary venous drainage into the LA
through the collateral communication as reported