CE: Swati; JCM-D-13-00421; Total nos of Pages: 3;

JCM-D-13-00421

Images in cardiovascular medicine

Cor triatriatum and hypertrophic cardiomyopathy Cyril Pellaton, Costas O’Mahony, Andrew J. Ludman, Neha Sekhri and Saidi Mohiddin Cor triatriatum is a rare congenital anomaly known to be associated with other inherited heart diseases. We present a nonrestrictive cor triatriatum sinistrum associated with hypertrophic cardiomyopathy to illustrate how different multimodality noninvasive imaging techniques complement each other and can help with the diagnosis. To the best of our knowledge, this coexistence has not been previously reported.

Keywords: congenital heart diseases, cor triatriatum, hypertrophic cardiomyopathy Department of Cardiology, London Chest Hospital, Barts Health NHS Trust, London, UK. Correspondence to Cyril Pellaton, MD, The London Chest Hospital, Bonner Road, London E2 9JX, UK E-mail: [email protected] Received 23 July 2013 Revised 03 December 2013 Accepted 19 January 2014

J Cardiovasc Med 2014, 15:000–000

A 56-year-old man with a 12-year history of shortness of breath, dizziness and palpitations was admitted for the first time to our institution after an episode of syncope. He had been diagnosed with atrial fibrillation 2 years

previously. The blood pressure was well controlled on a single antihypertensive agent. There was no history of previous syncope or myocarditis, and no family history of sudden death or hypertrophic cardiomyopathy (HCM).

Fig. 1

Transthoracic echocardiography and transoesophageal echocardiogram images showing cor triatriatum sinistrum. No turbulence or pressure gradient across the membrane in the LA is noted. TTE apical four-chamber view (a), zoom on LA (b), colour flow in the LA (c). TOE mid-oesophageal four-chamber view (d), with colour flow in the LA (e), mid-oesophageal 438 view (f).

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DOI:10.2459/JCM.0000000000000078

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2 Journal of Cardiovascular Medicine 2014, Vol 00 No 00

Transthoracic echocardiography (TTE) showed bi-atrial enlargement with a linear echogenic structure across the left atrium (LA), suggestive of cor triatriatum sinistrum. No turbulent flow or pressure gradient across the membrane in LA was detected. In addition, there was asymmetric left ventricular hypertrophy (LVH) with severe left ventricular systolic dysfunction (Fig. 1a–c). A transoesophageal echocardiogram (TOE) further confirmed the presence of cor triatriatum that did not impede flow between the LA chambers (Fig. 1d–f). A cardiac magnetic resonance (CMR) study also demonstrated the membrane in the LA. All the pulmonary veins

were identified draining into the proximal portion of the LA. The maximal basal left ventricular septal thickness was 14 mm (vs. 6 mm in the basal lateral wall) with no systolic anterior movement of the mitral valve or left ventricular outflow tract obstruction seen under scan conditions. The left ventricle (LV) was nondilated and globally hypokinetic with dyssynchronous movement of the septum (left ventricular ejection fraction of 26%). Tissue characterization showed extensive, diffuse, patchy, late gadolinium enhancement (LGE), more marked in the basal septal and anterior segments and also in the superior and inferior right ventricular insertion points (Fig. 2). A diagnosis of HCM with left ventricular systolic impairment was made.

Fig. 2

Cardiac magnetic resonance. Four-chamber view with accessory membrane in the LA (a), short axis view showing LVH (b), two-chamber view (c), four-chamber view with basal antero-lateral LGE (d), short axis view with diffuse patchy LGE, especially in the mid-anteroseptum and inferoseptum, including the insertion points of the right ventricle (e), two-chamber view with patchy mid anterior and mid inferior LGE (f).

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Cor triatriatum and hypertrophic cardiomyopathy Pellaton et al. 3

ECG monitoring detected rapidly conducted atrial fibrillation, left bundle branch block as well as pauses lasting over 3 s and frequent runs of nonsustained ventricular tachycardia. A cardiac resynchronization therapy defibrillator was implanted in addition to beta-blocker and warfarin therapy. No treatment specific to the nonrestrictive cor triatriatum was proposed. Cor triatriatum is a congenital anomaly in which the left (cor triatriatum sinistrum) or right atrium (cor triatriatum dextrum) is divided into two parts by a membrane or fibromuscular band. The former has been described in 0.1–0.4% of all congenital disease and the latter is extremely rare.1,2 The membrane can either be restrictive, impeding flow (through fenestrations of the membrane) or patent with no flow limitation.

Cor triatriatum is known to be associated with major congenital heart diseases1 and may require characterization by multimodality imaging techniques.3 To the best of our knowledge, this is the first case of cor triatriatum sinistrum coexisting with HCM, which is demonstrated elegantly by the capabilities of modern multimodality imaging.

References 1 2

3

Nassar PN, Hamdan RH. Cor triatriatum sinistrum: classification and imaging modalities. Eur J Cardiovasc Med 2011; 1:84–87. Choudhary D, Sivasankaran S, Venkateshwaran S, Sasidharan B. Cor triatiratum dexter: a rare cause of isolated right atrial enlargement. Pediatr Cardiol 2013; 34:198–199. Malik A, Fram D, Mohani A, et al. Cor triatriatum: a multimodality imaging approach. Can J Cardiol 2008; 24:e19–e20.

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Cor triatriatum and hypertrophic cardiomyopathy.

: Cor triatriatum is a rare congenital anomaly known to be associated with other inherited heart diseases. We present a nonrestrictive cor triatriatum...
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