- Email: [email protected]
Left ventricular muscular false tendon
Resuscitation (2008) 78, 3—4
available at www.sciencedirect.com
journal homepage: www.elsevier.com/locate/resuscitation
IMAGES IN RESUSCITATION
Left ventricular muscular false tendon Matthew Thomas Gwinnutt ∗ 185 Reservoir Road, Selly Oak, Birmingham B29 6SX, United Kingdom Received 4 December 2007; received in revised form 30 December 2007; accepted 16 January 2008
Fig. 1 ECG showing sinus rhythm, left axis deviation, abnormal R wave progression with a net positive QRS in lead V2, and ventricular repolarisation abnormalities (T wave inversion in leads l, aVL, V2, V3 and V4).
A 40-year-old, previously fit and healthy man collapsed whilst out running. Despite resuscitation attempts he died, and post-mortem revealed the cause of death to be hypertrophic obstructive cardiomyopathy (HOCM), the commonest cause of sudden cardiac death in young adults. In approximately 50% of cases, HOCM is inherited in an
∗
Tel.: +44 1214712447. E-mail address: [email protected].
autosomal dominant pattern with incomplete penetrance.1 Therefore familial screening is advised using electrocardiography (ECG) and transthoracic echocardiography (TTE) to allow early identification and treatment of those affected. The ECG of the deceased’s 16-year-old nephew was found to be abnormal (Figure 1). Subsequently, TTE appeared to show marked thickening of the inter-ventricular septum with normal left ventricular function. Exercise tolerance test (Bruce protocol) was normal. These findings were felt to confirm the diagnosis of HOCM. However, contrast echocar-
0300-9572/$ — see front matter © 2008 Elsevier Ireland Ltd. All rights reserved. doi:10.1016/j.resuscitation.2008.01.012
4
M.T. Gwinnutt tality but correctly identifying its presence is important as a misdiagnosis of HOCM has wider repercussions, e.g., the screening of relatives, avoiding exercise, obtaining insurance, and occupation. Because of the rarity and relative lack of knowledge of this condition, the patient is now being followed up with 6monthly 24-h ECGs, to detect paroxysmal arrhythmias, and a TTE every 2 years to identify any change in the muscular false tendon.
Conflict of interest statement None to declare.
Acknowledgements Fig. 2
Dr Simon Ray, Consultant Cardiologist, Wythenshawe Hospital, Manchester for kindly supplying the electrocardiograph and echocardiogram images. diography showed a large muscular false tendon running from the inter-ventricular septum to the apex of the left ventricle (A and B on Figure 2) that accounts for the ECG changes. This anomaly has been reported previously as benign, non-progressive, not affecting left ventricular working capacity, and distinct from HOCM.2 Three patients followed-up for between 8 and 18 years failed to develop any symptoms, exercise intolerance, or adverse cardiac events.2 This suggests that unlike HOCM, a muscular false tendon is not associated with any excess cardiac morbidity and mor-
References 1. Wigle ED, Rakowski H, Kimball BP, Williams WG. Hypertrophic cardiomyopathy clinical spectrum and treatment. Circulation 1995;92:1680—92. 2. St John Sutton MG, Dubrey S, Oldershaw PJ. Muscular false tendons, aberrant left ventricular papillary musculature, and severe electrocadiographic repolarisation abnormalities: a new syndrome. Br Heart J 1994;71:187—90.
available at www.sciencedirect.com
journal homepage: www.elsevier.com/locate/resuscitation
IMAGES IN RESUSCITATION
Left ventricular muscular false tendon Matthew Thomas Gwinnutt ∗ 185 Reservoir Road, Selly Oak, Birmingham B29 6SX, United Kingdom Received 4 December 2007; received in revised form 30 December 2007; accepted 16 January 2008
Fig. 1 ECG showing sinus rhythm, left axis deviation, abnormal R wave progression with a net positive QRS in lead V2, and ventricular repolarisation abnormalities (T wave inversion in leads l, aVL, V2, V3 and V4).
A 40-year-old, previously fit and healthy man collapsed whilst out running. Despite resuscitation attempts he died, and post-mortem revealed the cause of death to be hypertrophic obstructive cardiomyopathy (HOCM), the commonest cause of sudden cardiac death in young adults. In approximately 50% of cases, HOCM is inherited in an
∗
Tel.: +44 1214712447. E-mail address: [email protected].
autosomal dominant pattern with incomplete penetrance.1 Therefore familial screening is advised using electrocardiography (ECG) and transthoracic echocardiography (TTE) to allow early identification and treatment of those affected. The ECG of the deceased’s 16-year-old nephew was found to be abnormal (Figure 1). Subsequently, TTE appeared to show marked thickening of the inter-ventricular septum with normal left ventricular function. Exercise tolerance test (Bruce protocol) was normal. These findings were felt to confirm the diagnosis of HOCM. However, contrast echocar-
0300-9572/$ — see front matter © 2008 Elsevier Ireland Ltd. All rights reserved. doi:10.1016/j.resuscitation.2008.01.012
4
M.T. Gwinnutt tality but correctly identifying its presence is important as a misdiagnosis of HOCM has wider repercussions, e.g., the screening of relatives, avoiding exercise, obtaining insurance, and occupation. Because of the rarity and relative lack of knowledge of this condition, the patient is now being followed up with 6monthly 24-h ECGs, to detect paroxysmal arrhythmias, and a TTE every 2 years to identify any change in the muscular false tendon.
Conflict of interest statement None to declare.
Acknowledgements Fig. 2
Dr Simon Ray, Consultant Cardiologist, Wythenshawe Hospital, Manchester for kindly supplying the electrocardiograph and echocardiogram images. diography showed a large muscular false tendon running from the inter-ventricular septum to the apex of the left ventricle (A and B on Figure 2) that accounts for the ECG changes. This anomaly has been reported previously as benign, non-progressive, not affecting left ventricular working capacity, and distinct from HOCM.2 Three patients followed-up for between 8 and 18 years failed to develop any symptoms, exercise intolerance, or adverse cardiac events.2 This suggests that unlike HOCM, a muscular false tendon is not associated with any excess cardiac morbidity and mor-
References 1. Wigle ED, Rakowski H, Kimball BP, Williams WG. Hypertrophic cardiomyopathy clinical spectrum and treatment. Circulation 1995;92:1680—92. 2. St John Sutton MG, Dubrey S, Oldershaw PJ. Muscular false tendons, aberrant left ventricular papillary musculature, and severe electrocadiographic repolarisation abnormalities: a new syndrome. Br Heart J 1994;71:187—90.