Limits...
Arrhythmogenic substrate at the interventricular septum as a target site for radiofrequency catheter ablation of recurrent ventricular tachycardia in left dominant arrhythmogenic cardiomyopathy.

Havranek S, Palecek T, Kovarnik T, Vitkova I, Psenicka M, Linhart A, Wichterle D - BMC Cardiovasc Disord (2015)

Bottom Line: No abnormalities were found at the RV free wall including the inferolateral peritricuspid annulus region.Histological examination confirmed the presence of abnormal fibrous and adipose tissue with myocyte reduction in endomyocardial samples taken from both the left and right aspects of the IVS.LDAC rarely manifests with sustained monomorphic ventricular tachycardia.

View Article: PubMed Central - PubMed

Affiliation: 2nd Department of Medicine - Department of Cardiovascular Medicine, First Faculty of Medicine, Charles University and General University Hospital in Prague, U Nemocnice 2, Prague, 128 08, Czech Republic. stepan.havranek@lf1.cuni.cz.

ABSTRACT

Background: Left dominant arrhythmogenic cardiomyopathy (LDAC) is a rare condition characterised by progressive fibrofatty replacement of the myocardium of the left ventricle (LV) in combination with ventricular arrhythmias of LV origin.

Case presentation: A thirty-five-year-old male was referred for evaluation of recurrent sustained monomorphic ventricular tachycardia (VT) of 200 bpm and right bundle branch block (RBBB) morphology. Cardiac magnetic resonance imaging showed late gadolinium enhancement distributed circumferentially in the epicardial layer of the LV free wall myocardium including the rightward portion of the interventricular septum (IVS). The clinical RBBB VT was reproduced during the EP study. Ablation at an LV septum site with absence of abnormal electrograms and a suboptimum pacemap rendered the VT of clinical morphology noninducible. Three other VTs, all of left bundle branch block (LBBB) pattern, were induced by programmed electrical stimulation. The regions corresponding to abnormal electrograms were identified and ablated at the mid-to-apical RV septum and the anteroseptal portion of the right ventricular outflow tract. No abnormalities were found at the RV free wall including the inferolateral peritricuspid annulus region. Histological examination confirmed the presence of abnormal fibrous and adipose tissue with myocyte reduction in endomyocardial samples taken from both the left and right aspects of the IVS.

Conclusion: LDAC rarely manifests with sustained monomorphic ventricular tachycardia. In this case, several VTs of both RBBB and LBBB morphology were amenable to endocardial radiofrequency catheter ablation.

No MeSH data available.


Related in: MedlinePlus

Distribution of late gadolinium enhancement (arrows) in short axis (left upper panel) and vertical long axis view (right upper panel) was distributed circumferentially in the subepicardial left ventricular free wall myocardium with discrete progression to the adjacent mid-anterior free wall of the right ventricle. Note the subendocardial involvement at the RV aspect of the interventricular septum. Histological assessment of endomyocardial samples: from the left ventricular site of abnormal electrograms indicated by the cross in Figure 3 (left bottom panel); from right ventricular aspect of the interventricular septum (right bottom panel). Arrows indicate abnormal fibrosis and adipose tissue. Staining: hematoxylin-eosin. Magnified 100 times.
© Copyright Policy - open-access
Related In: Results  -  Collection

License 1 - License 2
getmorefigures.php?uid=PMC4359501&req=5

Fig2: Distribution of late gadolinium enhancement (arrows) in short axis (left upper panel) and vertical long axis view (right upper panel) was distributed circumferentially in the subepicardial left ventricular free wall myocardium with discrete progression to the adjacent mid-anterior free wall of the right ventricle. Note the subendocardial involvement at the RV aspect of the interventricular septum. Histological assessment of endomyocardial samples: from the left ventricular site of abnormal electrograms indicated by the cross in Figure 3 (left bottom panel); from right ventricular aspect of the interventricular septum (right bottom panel). Arrows indicate abnormal fibrosis and adipose tissue. Staining: hematoxylin-eosin. Magnified 100 times.

Mentions: A thirty-five-year-old male was referred for evaluation of recurrent hemodynamically tolerated sustained monomorphic ventricular tachycardia of 200 bpm, which had right bundle branch block (RBBB) morphology with leftward axis deviation (Figure 1). He suffered from non-syncopal palpitations in the past 3 months. He was in functional class NYHA I and had no symptoms suggestive of ischemic heart disease. His medical history was unremarkable. There was no family history of cardiomyopathies or sudden unexplained death. His 12-lead ECG in sinus rhythm was clearly abnormal with borderline Q-waves in the inferior leads, mid-QRS notching and slurring of narrow QRS complexes (QRSd of 98 ms) in limb and right precordial leads, respectively, and flattened biphasic or negative T waves in the inferolateral leads (Figure 1). Echocardiography detected slight LV dilatation (end-diastolic diameter of 63 mm) with mild global hypokinesia (ejection fraction of 42%). CT coronary angiography excluded coronary artery disease. Cardiac magnetic resonance imaging (CMR) showed late gadolinium enhancement (LGE), which was distributed circumferentially in the epicardial layer of the LV free wall myocardium (approximately one-third of the LV wall thickness) including the rightward portion of the interventricular septum (IVS) (Figure 2). The LGE spread also to a small adjacent region of the mid-anterior free wall of right ventricle (RV). Moreover, T1-weighted and SPIR magnetic resonance sequences visualised adipose infiltration of myocardium in the anterior right IVS and an adjacent portion of the anterior RV free wall in the zone of positive LGE. The LV was slightly dilated (end-diastolic diameter of 62 mm, end-diastolic volume of 287 mL) with mild global hypokinesia (ejection fraction of 50%). There were no wall motion abnormalities of the RV. The typical scar distribution together with ECG abnormalities and VT of RBBB morphology suggested the diagnosis of LDAC. Treatment with bisoprolol 2.5 mg and trandolapril 4 mg daily was initiated.Figure 1


Arrhythmogenic substrate at the interventricular septum as a target site for radiofrequency catheter ablation of recurrent ventricular tachycardia in left dominant arrhythmogenic cardiomyopathy.

Havranek S, Palecek T, Kovarnik T, Vitkova I, Psenicka M, Linhart A, Wichterle D - BMC Cardiovasc Disord (2015)

Distribution of late gadolinium enhancement (arrows) in short axis (left upper panel) and vertical long axis view (right upper panel) was distributed circumferentially in the subepicardial left ventricular free wall myocardium with discrete progression to the adjacent mid-anterior free wall of the right ventricle. Note the subendocardial involvement at the RV aspect of the interventricular septum. Histological assessment of endomyocardial samples: from the left ventricular site of abnormal electrograms indicated by the cross in Figure 3 (left bottom panel); from right ventricular aspect of the interventricular septum (right bottom panel). Arrows indicate abnormal fibrosis and adipose tissue. Staining: hematoxylin-eosin. Magnified 100 times.
© Copyright Policy - open-access
Related In: Results  -  Collection

License 1 - License 2
Show All Figures
getmorefigures.php?uid=PMC4359501&req=5

Fig2: Distribution of late gadolinium enhancement (arrows) in short axis (left upper panel) and vertical long axis view (right upper panel) was distributed circumferentially in the subepicardial left ventricular free wall myocardium with discrete progression to the adjacent mid-anterior free wall of the right ventricle. Note the subendocardial involvement at the RV aspect of the interventricular septum. Histological assessment of endomyocardial samples: from the left ventricular site of abnormal electrograms indicated by the cross in Figure 3 (left bottom panel); from right ventricular aspect of the interventricular septum (right bottom panel). Arrows indicate abnormal fibrosis and adipose tissue. Staining: hematoxylin-eosin. Magnified 100 times.
Mentions: A thirty-five-year-old male was referred for evaluation of recurrent hemodynamically tolerated sustained monomorphic ventricular tachycardia of 200 bpm, which had right bundle branch block (RBBB) morphology with leftward axis deviation (Figure 1). He suffered from non-syncopal palpitations in the past 3 months. He was in functional class NYHA I and had no symptoms suggestive of ischemic heart disease. His medical history was unremarkable. There was no family history of cardiomyopathies or sudden unexplained death. His 12-lead ECG in sinus rhythm was clearly abnormal with borderline Q-waves in the inferior leads, mid-QRS notching and slurring of narrow QRS complexes (QRSd of 98 ms) in limb and right precordial leads, respectively, and flattened biphasic or negative T waves in the inferolateral leads (Figure 1). Echocardiography detected slight LV dilatation (end-diastolic diameter of 63 mm) with mild global hypokinesia (ejection fraction of 42%). CT coronary angiography excluded coronary artery disease. Cardiac magnetic resonance imaging (CMR) showed late gadolinium enhancement (LGE), which was distributed circumferentially in the epicardial layer of the LV free wall myocardium (approximately one-third of the LV wall thickness) including the rightward portion of the interventricular septum (IVS) (Figure 2). The LGE spread also to a small adjacent region of the mid-anterior free wall of right ventricle (RV). Moreover, T1-weighted and SPIR magnetic resonance sequences visualised adipose infiltration of myocardium in the anterior right IVS and an adjacent portion of the anterior RV free wall in the zone of positive LGE. The LV was slightly dilated (end-diastolic diameter of 62 mm, end-diastolic volume of 287 mL) with mild global hypokinesia (ejection fraction of 50%). There were no wall motion abnormalities of the RV. The typical scar distribution together with ECG abnormalities and VT of RBBB morphology suggested the diagnosis of LDAC. Treatment with bisoprolol 2.5 mg and trandolapril 4 mg daily was initiated.Figure 1

Bottom Line: No abnormalities were found at the RV free wall including the inferolateral peritricuspid annulus region.Histological examination confirmed the presence of abnormal fibrous and adipose tissue with myocyte reduction in endomyocardial samples taken from both the left and right aspects of the IVS.LDAC rarely manifests with sustained monomorphic ventricular tachycardia.

View Article: PubMed Central - PubMed

Affiliation: 2nd Department of Medicine - Department of Cardiovascular Medicine, First Faculty of Medicine, Charles University and General University Hospital in Prague, U Nemocnice 2, Prague, 128 08, Czech Republic. stepan.havranek@lf1.cuni.cz.

ABSTRACT

Background: Left dominant arrhythmogenic cardiomyopathy (LDAC) is a rare condition characterised by progressive fibrofatty replacement of the myocardium of the left ventricle (LV) in combination with ventricular arrhythmias of LV origin.

Case presentation: A thirty-five-year-old male was referred for evaluation of recurrent sustained monomorphic ventricular tachycardia (VT) of 200 bpm and right bundle branch block (RBBB) morphology. Cardiac magnetic resonance imaging showed late gadolinium enhancement distributed circumferentially in the epicardial layer of the LV free wall myocardium including the rightward portion of the interventricular septum (IVS). The clinical RBBB VT was reproduced during the EP study. Ablation at an LV septum site with absence of abnormal electrograms and a suboptimum pacemap rendered the VT of clinical morphology noninducible. Three other VTs, all of left bundle branch block (LBBB) pattern, were induced by programmed electrical stimulation. The regions corresponding to abnormal electrograms were identified and ablated at the mid-to-apical RV septum and the anteroseptal portion of the right ventricular outflow tract. No abnormalities were found at the RV free wall including the inferolateral peritricuspid annulus region. Histological examination confirmed the presence of abnormal fibrous and adipose tissue with myocyte reduction in endomyocardial samples taken from both the left and right aspects of the IVS.

Conclusion: LDAC rarely manifests with sustained monomorphic ventricular tachycardia. In this case, several VTs of both RBBB and LBBB morphology were amenable to endocardial radiofrequency catheter ablation.

No MeSH data available.


Related in: MedlinePlus