Persistent Shunt After Closure of Ostium

Secundum Atrial Septal Defect

Frank F. Seghatol-Eslami, MD, Sang Gune K. Yoo, MD, Sarah L. Madira, BS,
Dilip S. Nath, MD, and Michael H. Lanier, MD, PhD, St. Louis, Missouri

A train can hide another one. tioning of the device against the rim of tissue surrounding the ASD.
–French adage However, although a small residual shunt may be present at the
completion of transcatheter ASD occluder device deployment, it usu-
ally becomes undetectable years after the procedure.1 Alternatively,
INTRODUCTION we considered the presence of other sources of left-to-right shunt at
the atrial level, such as sinus venosus ASD or coronary sinus defect
We describe a rare case of a patient previously diagnosed with that may have been missed at the time of previous transcatheter
ostium secundum atrial septal defect (ASD) and treated with closure device deployment.
transcatheter occluder device who presented 12 years later with Two-dimensional TEE with color flow Doppler showed a well-
dyspnea and persistent left-to-right shunt. Transesophageal echo- positioned ASD closure device. Injection of agitated saline into a
cardiography (TEE) and cardiac computed tomography (CCT) right arm vein showed an early appearance of large number of
showed new findings of superior sinus venosus ASD associated bubbles in the left atrium without clear evidence of bubbles
with partial anomalous pulmonary venous return (PAPVR) and crossing through or around the device (Figure 1A, Video 1).
persistent left superior vena cava (SVC). Given these findings This increased the likelihood of another shunt at the atrial level.
and the patient’s significant symptom burden, the consensus of Indeed, slight pull-back of the transesophageal probe medially re-
the heart team was to proceed with surgical intervention. vealed a defect in the superior and posterior aspect of the SVC
consistent with a superior sinus venosus ASD with an orifice
CASE PRESENTATION 1.35 cm in diameter (Figure 1B, Video 2). The next step was to
identify the pulmonary veins (PVs) and their connections, as
A 44-year-old patient was referred to the cardiology clinic of our insti- abnormal pulmonary venous connections are usually associated
tution because of dyspnea on exertion and poor exercise tolerance. with sinus venosus ASD. The left PVs were identified without dif-
Medical history was significant for transcatheter ASD closure device ficulty and showed normal drainage of left PVs into the left
12 years earlier. On physical examination, the patient was afebrile, atrium (Figure 2, Video 3). Visualization of the right PVs was
with blood pressure of 144/79 mm Hg, a heart rate of 89 beats/ more challenging and demonstrated the right upper PV draining
min, a respiratory rate of 14 breaths/min, and an O2 saturation of into the right atrium (Figure 3, Video 4). Upon further TEE, the
98% on room air. There was no cyanosis, finger clubbing, or signs presence of a persistent left SVC was also noted and confirmed
of heart failure. On cardiac auscultation, a soft 1/6 systolic murmur by injection of agitated saline into a left arm vein, with appear-
was heard at the left sternal border, but a split S2 was not heard. ance of bubbles in a dilated coronary sinus draining into a dilated
Transthoracic echocardiography with a bubble study performed at right ventricle (Figure 4, Video 5).
the referring institution showed a dilated right atrium and right Because of challenging anatomy, and to confirm the findings
ventricle associated with the presence of bubbles in the left atrium af- on TEE, the patient underwent CCT, which confirmed the supe-
ter intravenous injection of agitated saline consistent with persistent rior sinus venosus ASD as well as the anomalous right and middle
atrial shunt. pulmonary venous drainage into the right SVC just before its
Before proceeding with imaging investigations, our thoughts were junction with the right atrium, as well as a persistent left SVC
focused mainly on residual shunt after transcatheter ASD closure de- (Figure 5). Given the presence of left-to-right shunt, right heart
vice that could have occurred from dislodgment or improper posi- catheterization was performed to measure the pressures in the
right heart and the shunt ratio. This revealed a right atrial pressure
From the Division of Cardiology, Department of Medicine, Washington University
of 12 mm Hg, right ventricle 36/10 mm Hg, and PA 33/11 mm
School of Medicine and Barnes Jewish Hospital, St. Louis, Missouri (F.F.S.-E., Hg with a mean of 18 mm Hg. Oximetry showed step-up O2
S.G.K.Y.); Department of Cardiothoracic Surgery, Washington University School of saturation with inferior vena cava O2 saturation of 72%, SVC
Medicine and Children Hospital, St. Louis, Missouri (S.L.M., D.S.N.); and O2 saturation of 80%, and a calculated ratio of pulmonary to sys-
Mallinckrodt Institute of Radiology, Washington University School of Medicine and
temic flow of 2.1:1.0.
Barnes Jewish Hospital, St. Louis, Missouri (M.H.L.).
Subsequently, transesophageal echocardiography and cardiac
Keywords: Ostium secundum ASD, Sinus venosus ASD, Partial anomalous pul-
monary venous return, Multimodality imaging, Management options computed tomographic images were reviewed by our multidisci-
Correspondence: Frank F. Seghatol-Eslami, MD, Washington University School of
plinary team, including clinical and interventional cardiologists
Medicine, 660 South Euclid Avenue, Northwestern Tower, St. Louis, MO 63110. and a congenital heart surgeon, who considered both surgical
(E-mail: [email protected]). repair and transcatheter closure with a covered stent. Given the
Copyright 2024 by the American Society of Echocardiography. Published by patient’s symptoms, dilated right ventricular (RV) cavity (4.4 cm
Elsevier Inc. This is an open access article under the CC BY-NC-ND license (http:// at the RV base), as well as a shunt ratio of 2.1:1, a shared decision
creativecommons.org/licenses/by-nc-nd/4.0/). was made to proceed with surgical repair, which was done using
2468-6441 the Warden procedure via median sternotomy. A transcatheter
https://doi.org/10.1016/j.case.2024.08.002

1
2 Seghatol-Eslami et al CASE: Cardiovascular Imaging Case Reports
- 2024

DISCUSSION
VIDEO HIGHLIGHTS
The association of sinus venosus ASD with PAPVR is well
Video 1: Two-dimensional TEE, midesophageal biplane image known.2,3 However, our case is unique in that it shows a rare
with agitated saline contrast study (from the right arm), dem- occurrence of ostium secundum and sinus venosus ASD with par-
onstrates the well-positioned ASD occluder device with the tial anomalous pulmonary venous connections in one patient. It
early appearance of bubbles in both atria. also illustrates the importance of a comprehensive diagnostic
approach in patients with congenital heart disease in general
Video 2: Two-dimensional TEE, upper esophageal long-axis
and ASD in particular, as the risk for missing a defect is real.
(110 ) view with agitated saline contrast (from the right arm), ASDs represent 25% to 30% of congenital heart disease in
demonstrates the sinus venosus ASD. adults.2 Ostium secundum ASDs represent 80% of all ASDs
Video 3: Two-dimensional TEE, midesophageal long-axis and are due to defects within the fossa ovalis.3,4 They are usually
(102 ) view with color flow Doppler, demonstrates the left single, although on occasion, several small defects or an atrial
normal pulmonary venous drainage into the left atrium. septum with multiple fenestrations is also observed. 5 Ostium pri-
Video 4: Rightward rotated two-dimensional TEE, mid- mum ASDs are part of atrioventricular septal defects and account
esophageal long-axis (102 ) view with color flow Doppler, for 15% of all ASDs.5 Sinus venosus defects represent 5% to 10%
demonstrates the anomalous right upper PV draining into the of all ASDs and are located outside the confines of the true
right atrium. septum, resulting from deficiency of the tissue that separates
the right upper PV from the SVC.6 The superior sinus venosus
Video 5: Two-dimensional TEE, deep esophageal view (20 )
defect allows the orifice of the SVC to override the septum and
with agitated saline contrast (from the left arm), demonstrates
drain into both atrial chambers. It is often associated with partial
the bubble-filled dilated coronary sinus and right ventricle. anomalous connection of the right upper PV to SVC.6 PAPVR is
View the video content online at www.cvcasejournal.com. present in 10% of patients with ostium secundum ASDs and in
80% of patients with sinus venosus ASDs.7 Therefore, it is impor-
tant to identify all PVs in both ostium secundum and sinus veno-
sus ASDs. Transthoracic echocardiography has limited use in the
assessment of anomalous pulmonary venous connections, but
covered stent was considered as an option; however, out of
TEE plays an important role in diagnosing the type of ASD and
concern of overlapping the covered stent with the previous de-
associated anomalies of pulmonary venous connections.6-8 For
vice, it was decided to proceed with surgery, which consisted of
patients in whom PV connections are not well visualized on
closing the sinus venosus ASD and redirecting the anomalous
TEE, cross-sectional imaging with cardiovascular magnetic reso-
PVs into the left atrium. The patient’s postoperative course was
nance and CCT provides excellent images of ASD type, location,
uneventful, and they were discharged in stable condition on the
and abnormal PV connection, particularly those associated with
fourth postoperative day. Follow-up echocardiography before
veins that may be difficult to image on TEE, such as the innomi-
the patient’s discharge showed a membrane in the right atrium
nate vein or vertical vein.9 At our institution, we perform CCT
consistent with a surgical patch. No residual shunt by color flow
with retrospective electrocardiographic gating.
Doppler was visualized.

Figure 1 Two-dimensional TEE (A), midesophageal biplane image with agitated saline contrast study (from the right arm) and (B) up-
per esophageal long-axis (110 ) view of the atrial septum, demonstrates the well-positioned ASD occluder device with the early
appearance of bubbles in both atria and the sinus venosus ASD. LA, Left atrium; SV, sinus venosus.
CASE: Cardiovascular Imaging Case Reports Seghatol-Eslami et al 3
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A B

Figure 2 Two-dimensional TEE, midesophageal long-axis (102 ) view with (A) color flow Doppler and (B) pulsed-wave Doppler, dem-
onstrates the normal left pulmonic venous drainage into the left atrium. D, Diastolic flow; LLPV, left lower PV; LUPV, left upper PV; S,
systolic flow.

Electrocardiographic gating minimizes motion artifacts for tolic pulmonary artery pressure <50% of systolic systemic pres-
detailed anatomic evaluation and allows the assessment of car- sure, and pulmonary vascular resistance less than one-third of
diac function. For adequate opacification of cardiac chambers, the systemic vascular resistance (class 1 recommendation).10
iodinated contrast is power-injected at a rate of 5 mL/sec, with Transcatheter closure of ASDs has become the treatment of
a region of interest placed on the ascending aorta. Image acquisi- choice for most patients with suitable anatomy and adequate sur-
tion is triggered once attenuation of ascending aorta reaches 100 rounding rim of tissue.11 Three-dimensional TEE provides an en
Hounsfield units. Images are reconstructed in thin isotropic slices face view of ostium secundum ASDs with clear visualization of
($1.0  1.0 mm) during diastole and systole and at 10% intervals the defect shape and the surrounding tissue and allows measure-
along the cardiac cycle to improve quality of multiplanar refor- ment of the defect size with better correlation against balloon
matted images and to allow functional analysis. Because of its sizing.12 In recent years, advanced imaging with three-dimen-
high spatial and temporal resolution and wide field of view, sional printing and multimodal fusion imaging process have pro-
CCT is an excellent tool for the detection and characterization vided simulation on printed model to ensure preprocedural
of septal defects and associated anomalies of pulmonary and sys- guidance for transcatheter covered stent deployment for sinus ve-
temic connections and is critical for accurate reporting and plan- nosus ASD, with excellent results in select patients.13
ning of surgical or transcatheter therapy.9 Surgery is an option for patients with sinus venosus ASDs who
The decision to close an ASD in an adult is related to shunts are not good candidate for transcatheter covered stents. The basic
large enough to cause functional impairment, evidence of right concept behind the surgical repair of a sinus venosus ASD is to
atrial and RV enlargement, atrial arrhythmias, net left-to-right close the interatrial defect and redirect the anomalous pulmonary
shunt ratio > 1.5:1 without cyanosis at rest or during exercise, sys- venous drainage into the left atrium. Several procedures have

A B

Figure 3 Rightward rotated two-dimensional TEE, midesophageal long-axis (102 ) view with (A) color flow Doppler and (B) pulsed-
wave Doppler, demonstrates the anomalous right upper PV draining into the right atrium (RA). LA, Left atrium; RUPV, right upper PV.
4 Seghatol-Eslami et al CASE: Cardiovascular Imaging Case Reports
- 2024

CONCLUSION

Our case demonstrates the challenges in the diagnosis of sinus venosus

ASD associated with PAPVR, its rare coexistence with ostium secun-
dum ASD, as in our case, and the importance of using multimodality
imaging (TEE, CCT, cardiovascular magnetic resonance) to delineate
the anatomy and guide the appropriate intervention.

ETHICS STATEMENT

The authors declare that the work described has been carried out in
accordance with The Code of Ethics of the World Medical
Association (Declaration of Helsinki) for experiments involving humans.

Figure 4 Two-dimensional TEE, deep transesophageal view

(0 ), demonstrates the dilated coronary sinus (CS) and right CONSENT STATEMENT
ventricle (RV).
The authors declare that since this was a non-interventional, retro-
spective, observational study utilizing de-identified data, informed
been described. Warden repair, also called double patch repair, consent was not required from the patient under an IRB exemption
consists of transecting the right SVC above the drainage of the status.
abnormal PV. This portion of SVC along with anomalous PV con-
nections are rerouted into the left atrium. Then the SVC is anas- FUNDING STATEMENT
tomosed to the right atrial appendage.14 Long-term results are
excellent, with survival comparable with that among a normal The authors declare that this report did not receive any specific grant from
age-matched population.15 funding agencies in the public, commercial, or not-for-profit sectors.

A B

C D

Figure 5 CCT demonstrates the anomalous right and middle PV connections to the right SVC and the sinus venosus ASD with (A and
D) whole-heart, volume-rendered reconstruction displays and (B) oblique coronal and (C) axial multiplanar reformatted views.
CASE: Cardiovascular Imaging Case Reports Seghatol-Eslami et al 5
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DISCLOSURE STATEMENT 7. Feigenbaum H. Textbook of echocardiography 7th edition. In:

Armstrong WF, Ryan T, editors. Chapter 19: Congenital Heart Diseases.
The authors report no conflict of interest. Philadelphia, PA: Lippincott Williams & Wilkins; 2024:544-610.
8. Pascoe RD, Oh JK, Warnes CA, Danielson GK, Tajik JS. Diagnosis of sinus
venosus atrial septal defect with transesophageal echocardiography. Circu-
lation 1996;94:1049-55.
SUPPLEMENTARY DATA 9. Raptis DA, Bhalla S. Current Status of cardiac CT in adult congenital heart
disease. Semin Roentgenol 2020;55:230-40.
Supplementary data related to this article can be found at https://doi. 10. Stout KK, Daniels CJ, Aboulhosn JA, Bozkurt B, Broberg CS, Colman JM,
org/10.1016/j.case.2024.08.002. et al. 2018 AHA/ACC guidelines for the management of adults with
congenital heart disease: a report of the American College of Cardiol-
ogy/American Heart Association task force on clinical practice guidelines.
J Am Coll Cardiol 2019;73:e81-192.
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