Role of echocardiography in sinus venosus atrial septal defect combined with systemic and pulmonary vascular disease.

Abstract

We present a case of persistent left superior vena cava, anomalous right pulmonary venous connection to the right-sided superior vena cava and sinus venosus atrial septal defect detected by computed tomography (CT) pulmonary angiography and echocardiography. These defects were surgically corrected using a double-patch technique. In fact, CT can provide anatomical information about a complex anomaly in the systemic and pulmonary vasculatures. Though modern imaging techniques are useful for detecting complex cardiovascular disease, careful echocardiographic examination should be performed to diagnose complex cardiac anomalies.

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Introduction
Persistent left superior vena cava (PLSVC) is one of the most
common venous anormaly of the thorax. However, the inci-
dence is in less than 0.5% in the general population but oc-
curs in approximately 4% of patients with congenital heart
disease.1) Sinus venosus atrial septal defect (SVD) accounts for
4-11% of all atrial septal defect (ASD) and partial anomalous
pulmonary venous connection (PAPVC) is present in about
90% of patients with SVD. Anomalies of systemic venous
connection are usually found incidentally in asymptomatic pa-
tients, but their identification is sometimes crucial especially
when cardiac or abdominal aorta surgery is planned. We pres-
ent a case of a complex anomaly of systemic and pulmonary
venous return associated with SVD which was detected by
computed tomography (CT) pulmonary angiography and
echocardiography. Modern imaging techniques are not only
useful for detecting congenital cardiac defects but also for de-
tecting systemic and pulmonary venous connection anomalies.
This case demonstrates the importance of knowledge of the
anatomical basis of venous connection and identification of
one lesion should prompt an appropriate investigation of oth-
er potential anomalies that may be less obvious.
Case
A 41-year-old man was admitted to the hospital because of
palpitation and exertional dyspnea. On auscultation, fixed
splitting of the second heart sound and a systolic murmur
over the pulmonary valve area were heard. Electrocardiogram
on admission showed paroxysmal supraventricular tachycar-
dia. A chest X-ray demonstrated mild cardiac enlargement.
Transthoracic echocardiography (TTE) showed enlargement of
the right ventricle and an extremely dilated coronary sinus
with normal function of both ventricles (Fig. 1A). On the sec-
ond day of admission, we performed a CT pulmonary angiog-
raphy. The study showed a PLSVC draining into the right
atrium (Fig. 2A). CT also revealed an ASD with anomalous
return of the right superior pulmonary vein to the right supe-
rior vena cava (SVC) (Fig. 2B and C). Transesophageal echo-
cardiography (TEE) also demonstrated a SVD (Fig. 1B). Si-
multaneously, contrast echocardiography was performed. A
pISSN 1975-4612/ eISSN 2005-9655
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http://dx.doi.org/10.4250/jcu.2012.20.1.49
CASE REPORT
J Cardiovasc Ultrasound 2012;20(1):49-51
Role of Echocardiography in Sinus
Venosus Atrial Septal Defect Combined
with Systemic and Pulmonary Vascular
Disease
Jihun Ahn, MD1, Sang-Ho Park, MD, PhD2, Dohoi Kim, MD, PhD1, Taehoon Kim, MD1,
Seongsik Jo, MD, PhD3, Hyeokgyu Lee, MD2 and Ara Cho, MD2
1Department of Internal Medicine, Soonchunhyang University Gumi Hospital, Gumi, Korea
2Departments of Internal Medicine, 3Radiology, Soonchunhyang University Cheonan Hospital, Cheonan, Korea
We present a case of persistent left superior vena cava, anomalous right pulmonary venous connection to the right-sided superior
vena cava and sinus venosus atrial septal defect detected by computed tomography (CT) pulmonary angiography and echocar-
diography. These defects were surgically corrected using a double-patch technique. In fact, CT can provide anatomical informa-
tion about a complex anomaly in the systemic and pulmonary vasculatures. Though modern imaging techniques are useful for
detecting complex cardiovascular disease, careful echocardiographic examination should be performed to diagnose complex cardi-
ac anomalies.
KEY WORDS: Atrial septal defect · Pulmonary veins/abnormalities · Computed tomography.
•?Received: September 23, 2011 •?Revised: November 23, 2011 •?Accepted: February 16, 2012
•?Address for Correspondence: Sang-Ho Park, Department of Internal Medicine, Soonchunhyang University Cheonan Hospital, 31 Suncheonhyang 6-gil,
Dongnam-gu, Cheonan 330-721, Korea Tel: +82-41-570-3670, Fax: +82-41-574-5762, E-mail: matsalong@schmc.ac.kr
•?This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0)
which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

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Journal of Cardiovascular Ultrasound 20 | March 2012
50
right arm peripheral vein was injected with agitated saline,
and normal opacification was noted in the right atrium, fol-
lowed by opacification of the right ventricle (Fig. 1C). Next,
the left arm peripheral vein was injected with agitated saline
and abnormal opacification of the coronary sinus was first not-
ed, followed by opacification of the right atrium and then
right ventricle (Fig. 1D). On the third day of admission, coro-
nary CT angiography was performed. Coronary CT angiogra-
phy with 3-dimensional reconstruction demonstrated a more
detailed structure of PLSVC drainage into the right atrium via
a dilated coronary sinus (Fig. 2D). The patient underwent sur-
gical correction with the double-patch technique. Five
Fig. 1. Echocardiographic findings in the patient. A: Modified apical 4-chamber view showing markedly dilated right-sided heart chambers and
coronary sinus (arrow). B: Transesophageal echocardiography demonstrating sinus venosus atrial septal defect. C: Normal opacification of the right
side of the heart following an agitated saline injection through a right brachial vein. D: Early opacification of the abnormally large coronary sinus
(arrow) following an agitated saline injection through a left brachial vein. RA: right atrium, SVC: superior vena cava.
D
AB
C
ABCD
Fig. 2. Computed tomography (CT) appearance of the patient. A: Coronal reconstruction of the CT pulmonary angiogram (CTA-PA) showing the
course of the left superior vena cava (SVC) (arrow). B: Cross-sectional CTA-PA displaying the right superior pulmonary vein draining into the right SVC
(arrow). C: CTA-PA showing atrial septal defect (arrow). D: Three-dimensional reconstruction of the coronary CT angiogram demonstrating more
detailed structural information of the drainage of persistent left superior vena cava (arrow) into the right atrium via a dilated coronary sinus. CS:
coronary sinus, RA: right atrium, RV: right ventricle.

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Imaging Modalities in Complex Heart Disease | Jihun Ahn, et al.
51
months later, follow-up transthoracic echocardiography
showed near-normalization of the right ventricular and coro-
nary sinus size.
Discussion
PLSVC occurs in approximately 0.3-0.5% of the general
population and characteristically drains into the coronary si-
nus. During and after embryonic development of SVC, SVC
develops on the right side from a portion of the right anterior
cardinal vein. On the left side, part of the left anterior cardinal
vein undergo normal regression to form the ligament of the
left vena cava.1) PLSVC results from the persistence of the left
anterior cardinal vein. Usually, PLSVC is asymptomatic and
discovered incidentally during imaging study and pacemaker
implantation or central catheterization but sometimes their
elucidation is crucial especially during cardiovascular surgery.2)
PLSVC should be considered whenever a dilated coronary si-
nus is identified at echocardiography and the diagnosis could
be confirmed by saline contrast echocardiography.3) Other
modern imaging modalities such as CT or magnetic resonance
imaging (MRI) can be used to confirm the diagnosis. In our
case, we could not consider the presence of PLSVC before per-
forming CT pulmonary angiography just because of the focus
on volume overload of right-sided heart chambers. We per-
formed a contrast echocardiography based on the information
obtained from CT pulmonary angiography.
In our case, PAPVC associated with SVD which is a rarer
anomaly than PLSVC was also found. PAPVC is frequently
associated with congenital heart disease such as an ASD. It is
estimated that 10-15% of patients have an ASD and approxi-
mately 85% of PAPVC are associated with SVD.4)5) Usually,
the diagnosis of PAPVC can be made by echocardiography,
and cardiac catheterization along with angiography is often per-
formed for confirmation of the diagnosis. Nowadays, CT, MRI
and TEE with contrast examination are considered as sensitive
methods for the detection of PAPVC.6) In our case, the diag-
nosis of PAPVC was missed on routine TTE, and a definitive
diagnosis of PAPVC could be made on CT pulmonary angi-
ography.
Some authors have previously reported a combined anomaly
of systemic and pulmonary venous return associated with
SVD.7)8) These authors especially emphasized the importance
of new imaging modalities in diagnosing complex anomaly of
systemic and pulmonary venous return associated with SVD.
Also in our case, CT made a definitive diagnosis of the anomaly
and provided more detailed structural information. However,
this case report also illustrated that careful echocardiographic
examination should be performed using several windows and
even contrast for diagnosing the anomalies of systemic and
pulmonary venous return combined with congenital heart dis-
ease. And consideration is required when the case has accom-
panying cardiac abnormalities besides PLSVC such as an
ASD, when coronary sinus is extremely dilated and it is coex-
isting with enlargement of right ventricle.
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