Echocardiography. 2018;1–4. wileyonlinelibrary.com/journal/echo
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1 © 2018 Wiley Periodicals, Inc.
1 | CASE PRESENTATION
A 10-month-old male presented with persistent hypoxemia and oxy-
gen requirement of 1.5 L throughout the day. He had a fetal diagnosis
of large secundum atrial septal defect (ASD), pulmonary stenosis (PS),
and mildly hypoplastic right ventricle (RV). Additionally, he had multi-
ple comorbidities including tethered cord, bilateral chronic subdural
hematomas status post ventriculo-peritoneal shunt placement, and
laryngeal web status post multiple tracheal dilations. He had under-
gone balloon pulmonary valvuloplasty at 4 months of age with re-
portedly good result, despite which he continued to have persistent
oxygen requirement. On clinical examination, he was cyanosed with
oxygen saturations in the 80s, despite supplemental oxygen ther-
apy. He had a normal cardiac examination without the presence of
Received: 29 July 2018
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Revised: 10 September 2018
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Accepted: 27 September 2018
DOI: 10.1111/echo.14175
CASE REPORTS-PEDIATRIC
Cor triatriatum dexter with right ventricular hypoplasia: Role of
multimodality imaging in decision making
Sruti Rao MD | Patcharapong Suntharos MD | Hani Najm MD | Rukmini Komarlu MD
Cleveland Clinic Children’s Hospital,
Cleveland, Ohio
Correspondence
Sruti Rao, Cleveland Clinic Children’s
Hospital, Cleveland, OH.
Email: raos@ccf.org
Cor Triatriatum Dexter (CTD) is a rare congenital anomaly involving the systemic
venous valves. Failure of regression of the right-sided sinus venosus valve leads to
abnormal septation of the right atrium and a variety of right atrial and tricuspid valve
obstructive lesions. The presentation can be varied ranging from asymptomatic to
persistent neonatal cyanosis. We describe a late diagnosis of CTD in a 10-month-old
male with persistent hypoxia despite balloon valvuloplasty for mild pulmonic valve
stenosis with a large secundum atrial septal defect and a mildly hypoplastic right
ventricle.
KEYWORDS
atrial septal defect, cardiac imaging, Cor triatriatum, pulmonary valve stenosis, right
ventricular volume
FIGURE 1 Transthoracic
echocardiogram (TTE) subcostal long-
axis view (2D and color compare)
demonstrating a membrane in the
supratricuspid region resulting in right-to-
left shunting across the secundum ASD.
ASD = atrial septal defect