U Kocabas and others

Co-existence of PLSVC, HCM and ASD

ID: 16-0017; September 2016 DOI: 10.1530/ERP-16-0017

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A complicated trilogy: persistent left superior vena cava with hypertrophic cardiomyopathy and atrial septal defect Umut Kocabas MD, Esra Kaya MD and Cahide Soydas Cinar MD Department of Cardiology, Ege University School of Medicine, Izmir, Turkey

Correspondence should be addressed to U Kocabas Email [email protected]

Summary A 28-year-old asymptomatic woman was referred to our clinic for evaluation of a heart murmur. Transthoracic echocardiography (TTE) demonstrated ventricular hypertrophy with septal thickness of 26 mm, consistent with hypertrophic cardiomyopathy (HCM) (Fig.  1A).

The diagnosis of HCM was confirmed by contrast-enhanced magnetic resonance imaging, and late gadolinium enhancement was observed in the anterior wall (Fig.  1B and C). The patient’s coronary sinus (CS) was dilated and, therefore, underwent contrast echocardiography

Figure 1 (A) TTE demonstrating ventricular hypertrophy with a septal thickness of 26 mm, consistent with HCM. (B and C) HCM diagnosis confirmed via contrastenhanced MRI, and late gadolinium enhancement was observed in the anterior wall. (D) The patient’s CS was dilated therefore underwent contrast echocardiography with a prediagnosis of PLSVC. (E) Saline was administered through a left brachial vein, and the CS opacified first followed by the right atrium. (F) TTE revealed a secundum ASD, and Doppler echocardiography confirmed a left-to-right shunt. This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivs 4.0 International License.

© 2016 The authors 

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U Kocabas and others

with a prediagnosis of persistent left superior vena cava (PLSVC) (Fig. 1A and D). Saline was administered through a left brachial vein, and the CS opacified first followed by the right atrium (Fig.  1E and Video  1). After saline was administered through the patient’s right arm, her right atrium opacified before the CS. She was diagnosed with PLSVC. TTE revealed a secundum atrial septal defect (ASD), and Doppler echocardiography confirmed a leftto-right shunt (Fig. 1F and Video 2). Although the Qp:Qs ratio was

A complicated trilogy: persistent left superior vena cava with hypertrophic cardiomyopathy and atrial septal defect.

Extract: A 28-year-old asymptomatic woman was referred to ourclinic for evaluation of a heart murmur. Transthoracic echocardiography (TTE) demonstrate...
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