Int J Cardiovasc Imaging (2014) 30:783–784 DOI 10.1007/s10554-014-0396-x
IMAGES IN CARDIOLOGY
Congenital muscular diverticulum of the left ventricular apex Anto´nio Miguel Ferreira • Hugo Cafe´ • Katya Reis Santos • Ju´lia Toste • Hugo Marques
Received: 21 February 2014 / Accepted: 26 February 2014 / Published online: 7 March 2014 Ó Springer Science+Business Media Dordrecht 2014
Abstract We report the incidental finding of a muscular congenital diverticulum of the left ventricular apex in a young adult with AV node reentry tachycardia. The role of cardiovascular magnetic resonance in the differential diagnosis of this rare cardiac malformation is briefly discussed. Keywords Diverticulum Left ventricle Congenital heart disease Cardiovascular magnetic resonance A 34 year-old man presented with sporadic episodes of sudden tachycardia which were typically interrupted by Valsalva manoeuvres. He had a past history of transitory losses of consciousness during childhood and was temporarily treated with antiepileptic drugs. Physical examination was unremarkable and his resting ECG and transthoracic echocardiogram were considered normal, but ambulatory Holter monitoring revealed a short run of accelerated idioventricular
Electronic supplementary material The online version of this article (doi:10.1007/s10554-014-0396-x) contains supplementary material, which is available to authorized users. A. M. Ferreira (&) H. Cafe´ K. R. Santos J. Toste H. Marques Cardiology and Cardiovascular Imaging Departments, Hospital da Luz, Avenida Lusı´ada, 100, 1500-650 Lisbon, Portugal e-mail: [email protected]; [email protected]
rhythm at 93 beats per minute. Cardiac magnetic resonance imaging (CMR) was performed to exclude structural heart disease. CMR revealed a small apical outpouching of the left ventricle with normal contractile function and no delayed enhancement, consistent with a congenital muscular diverticulum of the left ventricular apex (Fig. 1 and Movie 1). He underwent an electrophysiological study, in which ventricular arrhythmias could not be induced. Yet, an AV node reentry tachycardia was disclosed and successfully treated with radiofrequency ablation. Since ventricular arrhythmias could not be elicited, a strategy of watchful waiting was favored for the apical diverticulum. The patient has since been asymptomatic. A left ventricular diverticulum is a rare cardiac malformation, with an incidence of 0.4 % in autopsy studies of adults with cardiac death [1]. Two types have been described: muscular and fibrous. Muscular diverticula are usually diagnosed in early childhood because of their frequent association with other cardiac malformations and midline thoraco-abdominal anomalies. In adults, they are usually asymptomatic and typically discovered incidentally, but systemic embolization, heart failure, ventricular wall rupture, and ventricular arrhythmias have been described [1,2]. The differential diagnosis includes left ventricular aneurisms and pseudoaneurisms, myocardial crypts, and left ventricular non-compaction [3]. A muscular diverticulum of the left ventricle usually takes the form of a small apical outpouching with a narrow mouth. Unlike aneurysmal formations, muscular diverticula show normal contractility and no fibrotic tissue (no delayed enhancement). Diverticula can be distinguished from myocardial crypts (discrete clefts in otherwise normal myocardium) and left ventricular noncompaction since these do not
123
784
Int J Cardiovasc Imaging (2014) 30:783–784
Fig. 1 Cardiac magnetic resonance: long-axis cine images in diastole and systole; Delayed enhancement (DE) images showing the absence of fibrosis in the left ventricular apical diverticulum
extend beyond the confines of the left ventricular cavity and myocardial margins. CMR seems particularly suited for the differential diagnosis of these situations due to its high spatial
123
resolution and the ability to differentiate normal myocardium from fibrotic tissue. Conflict of interest
None.
IMAGES IN CARDIOLOGY
Congenital muscular diverticulum of the left ventricular apex Anto´nio Miguel Ferreira • Hugo Cafe´ • Katya Reis Santos • Ju´lia Toste • Hugo Marques
Received: 21 February 2014 / Accepted: 26 February 2014 / Published online: 7 March 2014 Ó Springer Science+Business Media Dordrecht 2014
Abstract We report the incidental finding of a muscular congenital diverticulum of the left ventricular apex in a young adult with AV node reentry tachycardia. The role of cardiovascular magnetic resonance in the differential diagnosis of this rare cardiac malformation is briefly discussed. Keywords Diverticulum Left ventricle Congenital heart disease Cardiovascular magnetic resonance A 34 year-old man presented with sporadic episodes of sudden tachycardia which were typically interrupted by Valsalva manoeuvres. He had a past history of transitory losses of consciousness during childhood and was temporarily treated with antiepileptic drugs. Physical examination was unremarkable and his resting ECG and transthoracic echocardiogram were considered normal, but ambulatory Holter monitoring revealed a short run of accelerated idioventricular
Electronic supplementary material The online version of this article (doi:10.1007/s10554-014-0396-x) contains supplementary material, which is available to authorized users. A. M. Ferreira (&) H. Cafe´ K. R. Santos J. Toste H. Marques Cardiology and Cardiovascular Imaging Departments, Hospital da Luz, Avenida Lusı´ada, 100, 1500-650 Lisbon, Portugal e-mail: [email protected]; [email protected]
rhythm at 93 beats per minute. Cardiac magnetic resonance imaging (CMR) was performed to exclude structural heart disease. CMR revealed a small apical outpouching of the left ventricle with normal contractile function and no delayed enhancement, consistent with a congenital muscular diverticulum of the left ventricular apex (Fig. 1 and Movie 1). He underwent an electrophysiological study, in which ventricular arrhythmias could not be induced. Yet, an AV node reentry tachycardia was disclosed and successfully treated with radiofrequency ablation. Since ventricular arrhythmias could not be elicited, a strategy of watchful waiting was favored for the apical diverticulum. The patient has since been asymptomatic. A left ventricular diverticulum is a rare cardiac malformation, with an incidence of 0.4 % in autopsy studies of adults with cardiac death [1]. Two types have been described: muscular and fibrous. Muscular diverticula are usually diagnosed in early childhood because of their frequent association with other cardiac malformations and midline thoraco-abdominal anomalies. In adults, they are usually asymptomatic and typically discovered incidentally, but systemic embolization, heart failure, ventricular wall rupture, and ventricular arrhythmias have been described [1,2]. The differential diagnosis includes left ventricular aneurisms and pseudoaneurisms, myocardial crypts, and left ventricular non-compaction [3]. A muscular diverticulum of the left ventricle usually takes the form of a small apical outpouching with a narrow mouth. Unlike aneurysmal formations, muscular diverticula show normal contractility and no fibrotic tissue (no delayed enhancement). Diverticula can be distinguished from myocardial crypts (discrete clefts in otherwise normal myocardium) and left ventricular noncompaction since these do not
123
784
Int J Cardiovasc Imaging (2014) 30:783–784
Fig. 1 Cardiac magnetic resonance: long-axis cine images in diastole and systole; Delayed enhancement (DE) images showing the absence of fibrosis in the left ventricular apical diverticulum
extend beyond the confines of the left ventricular cavity and myocardial margins. CMR seems particularly suited for the differential diagnosis of these situations due to its high spatial
123
resolution and the ability to differentiate normal myocardium from fibrotic tissue. Conflict of interest
None.
