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Congenital ventricular diverticulum presenting as sustained monomorphic ventricular tachycardia
International Journal of Cardiology 133 (2009) e70 – e72 www.elsevier.com/locate/ijcard
Letter to the Editor
Congenital ventricular diverticulum presenting as sustained monomorphic ventricular tachycardia Mark Sierra ⁎, Hieu Huynh, Christian Machado The Heart Institute at Providence Hospital and Medical Centers, Department of Internal, Medicine, Division of Cardiovascular Medicine, 16001 West Nine Mile Road, Southfield MI 48075, USA Received 29 August 2007; accepted 17 November 2007 Available online 18 January 2008
Abstract Congenital ventricular diverticulum is a rare congenital cardiac defect. Most patients with this abnormality will remain asymptomatic and without complications during long-term follow-up. However a subgroup of these patients may experience life threatening complications such as ventricular arrhythmias, systemic embolism, sudden death, spontaneous rupture, and valvular regurgitation. We present a case of a patient with a congenital ventricular diverticulum presenting with sustained monomorphic ventricular tachycardia and evaluated by 64-slice CT. This is an uncommon but possibly life threatening arrhythmia described in less than 10 cases in the medical literature. The natural history of such patients as well as the appropriate treatment is unclear. Most of the literature advocates early surgical intervention. We decided to provide the patient with an implantable cardioverter defibrillator (ICD) and not surgical intervention due to the close proximity of the defect to the mitral valve. © 2008 Elsevier Ireland Ltd. All rights reserved. Keywords: Diverticulum; Ventricular tachycardia; Computerized tomography
1. Case report A 38-year-old Caucasian female was admitted to the hospital with palpitations, chest discomfort, and near syncope. The patient was found to have sustained monomorphic ventricular tachycardia (SMVT) (Fig. 1) and successfully treated with lidocaine with a resulting sinus rhythm. The SMVT was associated with chest discomfort, tingling of the hands, throat tightness, and diaphoresis so she was referred for cardiac catheterization. Cardiac catheterization revealed no angiographic evidence of coronary disease and the ventriculogram showed a diverticulum of the left ventricle (Fig. 1). The diverticulum was further evaluated by 64-slice CT (Fig. 2). The patient
⁎ Corresponding author. Providence Hospital and Medical Centers, Heart Institute, 16001 West Nine Mile Road, Southfield MI 48075, USA. Tel.: +1 248 849 2000; fax: +1 248 849 2075. E-mail address: [email protected] (M. Sierra). 0167-5273/$ - see front matter © 2008 Elsevier Ireland Ltd. All rights reserved. doi:10.1016/j.ijcard.2007.11.019
was found to have a basal septal pouch like defect extending into the right ventricle which was contractile (as evidenced by CT; Fig. 2c), had a wide connection to the ventricle, closely associated with the anterior mitral valve leaflet/ annulus, and not associated with any other obvious cardiac or thoracoabdominal abnormality. The patient otherwise had normal echocardiographic findings to include a normal ejection fraction. The patient was treated with an intracardiac defibrillator for prevention of sudden death. Surgical intervention was not done due to the close proximity of the diverticulum to the mitral valve apparatus. 2. Discussion Congenital ventricular diverticulum is a rare congenital cardiac defect reported in 0.26% of patients undergoing cardiac catheterization and 0.4% of autopsies after cardiac death [1]. There are less than 100 cases reported worldwide and ventricular tachyarrythmias associated with congenital
M. Sierra et al. / International Journal of Cardiology 133 (2009) e70–e72
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Fig. 1. Presenting ECG with monomorphic sustained ventricular tachycardia; and ventriculogram demonstrating a basal diverticulum.
ventricular diverticulum are even rarer with less than 10 cases reported previously. We present a case of left ventricular diverticulum with contractile function presenting as SMVT. The case was evaluated by 64-slice CT and ventriculography. Congenital ventricular diverticula is defined as a protrusion of one of the ventricles including myocardium, endocardium and occasionally pericardium. The diverticulum may arise from either the right or left ventricle but are more common from the left ventricle [2]. The etiology of congenital ventricular diverticula has not been elucidated but, it is likely due to disrupted embryogenesis [3]. Ventricular diverticula may be differentiated into fibrous and muscular types. The fibrous type occurs more often in blacks or Africans. Is usually found near the atrioventricular valve apparatus. Is a contractile pouch with a wide connection to the ventricle and is never associated with other congenital cardiac malformations or midline defects The muscular diverticula is usually associated with the apex of the ventricle, is a contractile finger like structure with a
narrow point of connection to the left ventricle and is frequently associated with midline thoracoabdominal defects and other various congenital cardiac abnormalities [4,5]. Most patients with congenital diverticula will remain asymptomatic, with no complication during long-term follow-up. However, a subgroup of these patients will develop complications which may be fatal. Complications such as systemic ventricular tachyarrythmias, systemic embolism, sudden death, spontaneous rupture, and severe valvular regurgitation have been reported [6]. This patient likely has a fibrous ventricular diverticulum. It is likely that the monomorphic ventricular tachycardia seen in this patient was caused by this congenital abnormality either by acting as an ectopic focus of activity or a site for reentry. The natural history of these patients is unknown and most people advocate early surgical intervention even in asymptomatic patients. In our opinion the proximity of the diverticula to the mitral apparatus provided an unacceptable surgical risk for this patient. Therefore, surgical intervention
Fig. 2. (A) CT hollow image and (B) full volume image showing ventricular diverticulum. (C) CT long axis cut of the left ventricle in diastole and systole showing a contractile diverticulum.
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was not done at this time and the patient received an ICD for protection against sudden death. References [1] Walton-Shirley M, Smith SM, Talley JD. Left ventricular diverticulum: case report and review of the literature. Catheter Cardiovasc Diagn 1992;26:31–3. [2] Gueron M, Hirsch M, Opschitzer I, Mogel P. Left ventricular diverticulum and mitral incompetence in asymptomatic children. Circulation 1976;53:181–6. [3] McAulife FM, Hornberger LK, Johnson J, Chitayat D, Ryan G. Cardiac diverticulum with pericardial effusion: report of two new cases treated bi
in utero pericardiocentesis and a review of the literature. Ultrasound Obstet Gynecol 2005;25:401–4. [4] Parthenakis FI, Kochiadakis GE, Patrianakos AP, et al. Peripheral arterial embolization due to a left ventricular diverticulum in a young adult. Chest 2005;127:1452–4. [5] Cay S, Tufekcioglu O, Ozturk S, et al. Left ventricular diverticulum with contractile function in an unusual site. J Am Soc Echocardiogr 2006;1293: e3–6. [6] Yalonetsky S, Agmon Y, Lessick J. Contrast echocardiographic imaging of left ventricular diverticulum in adult patients. J Am Soc Echocardiogr 2006;20(198):e1–3.
Letter to the Editor
Congenital ventricular diverticulum presenting as sustained monomorphic ventricular tachycardia Mark Sierra ⁎, Hieu Huynh, Christian Machado The Heart Institute at Providence Hospital and Medical Centers, Department of Internal, Medicine, Division of Cardiovascular Medicine, 16001 West Nine Mile Road, Southfield MI 48075, USA Received 29 August 2007; accepted 17 November 2007 Available online 18 January 2008
Abstract Congenital ventricular diverticulum is a rare congenital cardiac defect. Most patients with this abnormality will remain asymptomatic and without complications during long-term follow-up. However a subgroup of these patients may experience life threatening complications such as ventricular arrhythmias, systemic embolism, sudden death, spontaneous rupture, and valvular regurgitation. We present a case of a patient with a congenital ventricular diverticulum presenting with sustained monomorphic ventricular tachycardia and evaluated by 64-slice CT. This is an uncommon but possibly life threatening arrhythmia described in less than 10 cases in the medical literature. The natural history of such patients as well as the appropriate treatment is unclear. Most of the literature advocates early surgical intervention. We decided to provide the patient with an implantable cardioverter defibrillator (ICD) and not surgical intervention due to the close proximity of the defect to the mitral valve. © 2008 Elsevier Ireland Ltd. All rights reserved. Keywords: Diverticulum; Ventricular tachycardia; Computerized tomography
1. Case report A 38-year-old Caucasian female was admitted to the hospital with palpitations, chest discomfort, and near syncope. The patient was found to have sustained monomorphic ventricular tachycardia (SMVT) (Fig. 1) and successfully treated with lidocaine with a resulting sinus rhythm. The SMVT was associated with chest discomfort, tingling of the hands, throat tightness, and diaphoresis so she was referred for cardiac catheterization. Cardiac catheterization revealed no angiographic evidence of coronary disease and the ventriculogram showed a diverticulum of the left ventricle (Fig. 1). The diverticulum was further evaluated by 64-slice CT (Fig. 2). The patient
⁎ Corresponding author. Providence Hospital and Medical Centers, Heart Institute, 16001 West Nine Mile Road, Southfield MI 48075, USA. Tel.: +1 248 849 2000; fax: +1 248 849 2075. E-mail address: [email protected] (M. Sierra). 0167-5273/$ - see front matter © 2008 Elsevier Ireland Ltd. All rights reserved. doi:10.1016/j.ijcard.2007.11.019
was found to have a basal septal pouch like defect extending into the right ventricle which was contractile (as evidenced by CT; Fig. 2c), had a wide connection to the ventricle, closely associated with the anterior mitral valve leaflet/ annulus, and not associated with any other obvious cardiac or thoracoabdominal abnormality. The patient otherwise had normal echocardiographic findings to include a normal ejection fraction. The patient was treated with an intracardiac defibrillator for prevention of sudden death. Surgical intervention was not done due to the close proximity of the diverticulum to the mitral valve apparatus. 2. Discussion Congenital ventricular diverticulum is a rare congenital cardiac defect reported in 0.26% of patients undergoing cardiac catheterization and 0.4% of autopsies after cardiac death [1]. There are less than 100 cases reported worldwide and ventricular tachyarrythmias associated with congenital
M. Sierra et al. / International Journal of Cardiology 133 (2009) e70–e72
e71
Fig. 1. Presenting ECG with monomorphic sustained ventricular tachycardia; and ventriculogram demonstrating a basal diverticulum.
ventricular diverticulum are even rarer with less than 10 cases reported previously. We present a case of left ventricular diverticulum with contractile function presenting as SMVT. The case was evaluated by 64-slice CT and ventriculography. Congenital ventricular diverticula is defined as a protrusion of one of the ventricles including myocardium, endocardium and occasionally pericardium. The diverticulum may arise from either the right or left ventricle but are more common from the left ventricle [2]. The etiology of congenital ventricular diverticula has not been elucidated but, it is likely due to disrupted embryogenesis [3]. Ventricular diverticula may be differentiated into fibrous and muscular types. The fibrous type occurs more often in blacks or Africans. Is usually found near the atrioventricular valve apparatus. Is a contractile pouch with a wide connection to the ventricle and is never associated with other congenital cardiac malformations or midline defects The muscular diverticula is usually associated with the apex of the ventricle, is a contractile finger like structure with a
narrow point of connection to the left ventricle and is frequently associated with midline thoracoabdominal defects and other various congenital cardiac abnormalities [4,5]. Most patients with congenital diverticula will remain asymptomatic, with no complication during long-term follow-up. However, a subgroup of these patients will develop complications which may be fatal. Complications such as systemic ventricular tachyarrythmias, systemic embolism, sudden death, spontaneous rupture, and severe valvular regurgitation have been reported [6]. This patient likely has a fibrous ventricular diverticulum. It is likely that the monomorphic ventricular tachycardia seen in this patient was caused by this congenital abnormality either by acting as an ectopic focus of activity or a site for reentry. The natural history of these patients is unknown and most people advocate early surgical intervention even in asymptomatic patients. In our opinion the proximity of the diverticula to the mitral apparatus provided an unacceptable surgical risk for this patient. Therefore, surgical intervention
Fig. 2. (A) CT hollow image and (B) full volume image showing ventricular diverticulum. (C) CT long axis cut of the left ventricle in diastole and systole showing a contractile diverticulum.
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M. Sierra et al. / International Journal of Cardiology 133 (2009) e70–e72
was not done at this time and the patient received an ICD for protection against sudden death. References [1] Walton-Shirley M, Smith SM, Talley JD. Left ventricular diverticulum: case report and review of the literature. Catheter Cardiovasc Diagn 1992;26:31–3. [2] Gueron M, Hirsch M, Opschitzer I, Mogel P. Left ventricular diverticulum and mitral incompetence in asymptomatic children. Circulation 1976;53:181–6. [3] McAulife FM, Hornberger LK, Johnson J, Chitayat D, Ryan G. Cardiac diverticulum with pericardial effusion: report of two new cases treated bi
in utero pericardiocentesis and a review of the literature. Ultrasound Obstet Gynecol 2005;25:401–4. [4] Parthenakis FI, Kochiadakis GE, Patrianakos AP, et al. Peripheral arterial embolization due to a left ventricular diverticulum in a young adult. Chest 2005;127:1452–4. [5] Cay S, Tufekcioglu O, Ozturk S, et al. Left ventricular diverticulum with contractile function in an unusual site. J Am Soc Echocardiogr 2006;1293: e3–6. [6] Yalonetsky S, Agmon Y, Lessick J. Contrast echocardiographic imaging of left ventricular diverticulum in adult patients. J Am Soc Echocardiogr 2006;20(198):e1–3.