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Congenital Left Ventricular Diverticulum Complicated by Ventricular Fibrillation
Congenital Left Ventricular Diverticulum Complicated by Ventricular Fibrillation Manabu Yamasaki, MD, Naoto Kawamatsu, MD, Kunihiko Yoshino, MD, Kohei Abe, MD, and Hiroyasu Misumi, MD Departments of Cardiovascular Surgery and Cardiovascular Medicine, St. Luke’s International Hospital, Tokyo, Japan
Congenital left ventricular diverticulum (CLVD) is a rare congenital anomaly and may be associated with fatal adverse events. A previously healthy 20-year-old man collapsed as a result of sudden ventricular fibrillation (VF). Despite intractable VF, he had return of spontaneous circulation with cardiopulmonary resuscitation and subsequent introduction of venoarterial extracorporeal membrane oxygenation (ECMO). After ECMO was discontinued, cardiac magnetic resonance imaging revealed CLVD at the posterolateral wall of the left ventricle. Given the risk of recurrent VF and left ventricular rupture, he underwent surgical repair for CLVD and implantation of a subcutaneous implantable cardioverter defibrillator. (Ann Thorac Surg 2017;104:e271–3) Ó 2017 by The Society of Thoracic Surgeons
C
ongenital left ventricular diverticulum (CVD) is a rare congenital anomaly with a prevalence of 0.4% from autopsy results in cardiac failure cases (1). Recent advances in cardiovascular imaging have enabled us to detect asymptomatic congenital ventricular diverticulum. Accepted for publication March 14, 2017. Address correspondence to Dr Yamasaki, Department of Cardiovascular Surgery, St. Luke’s International Hospital, 9-1 Akashicho, Chuoku, Tokyo 104-8560, Japan; email: [email protected].
However, fatal adverse events associated with CLVD, including ventricular arrhythmia, heart failure, and rupture, have been documented. Here, we present a rare case of CLVD complicated by ventricular fibrillation. A previously healthy 20-year-old man was admitted to our hospital because of ventricular fibrillation and subsequent cardiopulmonary arrest. In spite of intractable ventricular fibrillation, the introduction of intraaortic balloon pumping (IABP) and venoarterial extra corporeal membrane oxygenation (ECMO) enabled a return to spontaneous circulation. After the patient was weaned from IABP and ECMO, cardiac magnetic resonance imaging (MRI) revealed an outpouching structure of the posterolateral left ventricular wall (Fig 1a). An electrophysiologic study (EPS) showed a possibile origin of his ventricular fibrillation at this left ventricular outpouching. After confirmation of the relative positions of the coronary arteries and left ventricular outpouching by cardiac computed tomography (Fig 1b), the outpouching (Fig 2a) was surgically treated by closure of the orifice with an expanded polytetrafluoroethylene patch. Subsequent cryoablation of the left ventricular wall around the outpouching was also performed with the patient under cardiac arrest by the use of cardiopulmonary bypass (Fig 2b). Pathologic examination of the outpouching wall, which consisted of cardiac myocytes of different sizes with marked fibrosis (Fig. 3), confirmed the diagnosis of congenital left ventricular diverticulum (CLVD). After subsequent implantation of a subcutaneous implantable cardioverter defibrillator (SICD), he was discharged from the hospital without any neurologic deficits.
Comment The natural history of CLVD has not been studied in depth because it is a rare congenital disease [1]. Marijon Fig 1. (a) Cardiac magnetic resonance image showing the outpouching of the posterolateral left ventricular wall (2 2 2 cm) (arrow). (b) Cardiac computed tomographic view. The white arrow shows the relationship between the outpouching of left ventricular wall and oblique margin branch of corona artery.
Ó 2017 by The Society of Thoracic Surgeons Published by Elsevier Inc.
0003-4975/$36.00 http://dx.doi.org/10.1016/j.athoracsur.2017.03.029
e272
CASE REPORT YAMASAKI ET AL CONGENITAL LEFT VENTRICULAR DIVERTICULUM
Ann Thorac Surg 2017;104:e271–3
Fig 2. Operative photo showing (a) a contractile but thin outpouching of posterolateral left ventricular wall and (b) an expanded polytetrafluoroethylene patch, excluding the orifice of the outpouching (around 3 cm).
and colleagues [2] described that CLVD on a contractile wall consists of three layers of outpouched wall, as opposed to congenital left ventricular aneurysm (CLVA), which lacks wall contractility and the three layers of outpouching wall. Patients with CLDA also have a poorer prognosis than do those with CLVD. Furthermore, there are two subtypes of CLVD: apical type cases are always associated with other cardiac anomalies or midline thoracoabdominal defects, whereas nonapical type cases occur in isolation [2]. However, the defining difference between CLVD and CLVA has not yet been clearly done. In regard to our case, cardiac MRI revealed contractile motion of the left ventricular wall outpouching, which was located on the posterolateral wall of the left ventricle. Pathologic examination showed that this outpouching wall had contained a cardiac muscle layer, although the size of cardiac myocytes varied and the layer demonstrated marked fibrosis. Inasmuch as the patient had no other congenital anomaly, we defined this case as nonapical type CLVD. Regarding the treatment of CLVD, it has been reported that surgical treatment is a reasonable option,
considering the risk of left ventricular rupture [3]. However, an appropriate consideration of the operative risks and benefits is warranted because a previous study reported a lack of changes in CLVD size over a period of long-term medical follow-up [4]. Nonetheless, Yalonetsky and colleagues [5] reported that a subset of CLVD may be associated with several potentially fatal adverse events, including systemic ventricular tachyarrythmias (VT), such as the ventricular fibrillation (VF) seen in our case, in addition to systemic embolism, sudden death, spontaneous rupture, and severe valvular regurgitation. In our case, EPS exploration triggered VF, which we identified as originating from the outpouching left ventricle. Although a previous case report describes the placement of an intravenous implantable cardioverter defibrillator (IVICD) for a patient with sustained monomorphic VT and CLVD in close proximity to the mitral valve [6], we decided to perform a surgical intervention for our patient, followed by left ventricular wall cryoablation to prevent both recurrent VF and left ventricular rupture. Before discharging the patient, we opted to implant an SICD rather than an IVICD in view of the lower rates of lead-related adverse events, like infective endocarditis [7], in addition to the fact that the patient did not require pacing and the possibility of later removal. Here, we present a case of a patient with a CLVD complicated by VF, which needed aggressive intervention including cardiopulmonary resuscitation and ECMO, who successfully underwent subsequent surgical treatment for CLVD followed by SICD placement. We think that the combination of the operation with the exclusion of CLVD and cryoablation and the implantation of an SICD was an appropriate treatment because the outpouched wall was very thin, the patient was young and at a low risk for surgical repair, and he also had the potential of recurrent VT in the future.
References Fig 3. Photomicrograph of tissue sample from outpouching showing myocardial tissue with different sizes of cardiac myocytes and marked fibrosis (hematoxylin and eosin stain, 4).
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.
Ann Thorac Surg 2017;104:e271–3
2. Marijon E, Ou P, Fermont L, et al. Diagnosis and outcome in congenital ventricular diverticulum and aneurysm. J Thorac Cardiovasc Surg 2006;131:433–7. 3. Tanaka M, Kawahito K, Kaneda H, Saito S. Congenital left ventricular diverticulum in an adult. Eur Heart J 2007;28:1537. 4. Archbold RA, Robinson NM, Mills PG. Long-term follow up of a true contractile left ventricular diverticulum. Am J Cardiol 1999;83:810–3; A11.
CASE REPORT YAMASAKI ET AL CONGENITAL LEFT VENTRICULAR DIVERTICULUM
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5. Yalonetsky S, Agmon Y, Lessick J. Contrast echocardiographic imaging of left ventricular diverticulum in adult patients. J Am Soc Echocardiogr 2006;20:e1–3. 6. Sierra M, Huynh H, Machado C. Congenital ventricular diverticulum presenting as sustained monomorphic ventricular tachycardia. Int J Cardiol 2009;133:e70–2. 7. Brouwer TF, Yilmaz D, Lindeboom R, et al. Long-term clinical outcomes of subcutaneous versus transvenous implantable defibrillator therapy. J Am Coll Cardiol 2016;68:2047–55.
Congenital left ventricular diverticulum (CLVD) is a rare congenital anomaly and may be associated with fatal adverse events. A previously healthy 20-year-old man collapsed as a result of sudden ventricular fibrillation (VF). Despite intractable VF, he had return of spontaneous circulation with cardiopulmonary resuscitation and subsequent introduction of venoarterial extracorporeal membrane oxygenation (ECMO). After ECMO was discontinued, cardiac magnetic resonance imaging revealed CLVD at the posterolateral wall of the left ventricle. Given the risk of recurrent VF and left ventricular rupture, he underwent surgical repair for CLVD and implantation of a subcutaneous implantable cardioverter defibrillator. (Ann Thorac Surg 2017;104:e271–3) Ó 2017 by The Society of Thoracic Surgeons
C
ongenital left ventricular diverticulum (CVD) is a rare congenital anomaly with a prevalence of 0.4% from autopsy results in cardiac failure cases (1). Recent advances in cardiovascular imaging have enabled us to detect asymptomatic congenital ventricular diverticulum. Accepted for publication March 14, 2017. Address correspondence to Dr Yamasaki, Department of Cardiovascular Surgery, St. Luke’s International Hospital, 9-1 Akashicho, Chuoku, Tokyo 104-8560, Japan; email: [email protected].
However, fatal adverse events associated with CLVD, including ventricular arrhythmia, heart failure, and rupture, have been documented. Here, we present a rare case of CLVD complicated by ventricular fibrillation. A previously healthy 20-year-old man was admitted to our hospital because of ventricular fibrillation and subsequent cardiopulmonary arrest. In spite of intractable ventricular fibrillation, the introduction of intraaortic balloon pumping (IABP) and venoarterial extra corporeal membrane oxygenation (ECMO) enabled a return to spontaneous circulation. After the patient was weaned from IABP and ECMO, cardiac magnetic resonance imaging (MRI) revealed an outpouching structure of the posterolateral left ventricular wall (Fig 1a). An electrophysiologic study (EPS) showed a possibile origin of his ventricular fibrillation at this left ventricular outpouching. After confirmation of the relative positions of the coronary arteries and left ventricular outpouching by cardiac computed tomography (Fig 1b), the outpouching (Fig 2a) was surgically treated by closure of the orifice with an expanded polytetrafluoroethylene patch. Subsequent cryoablation of the left ventricular wall around the outpouching was also performed with the patient under cardiac arrest by the use of cardiopulmonary bypass (Fig 2b). Pathologic examination of the outpouching wall, which consisted of cardiac myocytes of different sizes with marked fibrosis (Fig. 3), confirmed the diagnosis of congenital left ventricular diverticulum (CLVD). After subsequent implantation of a subcutaneous implantable cardioverter defibrillator (SICD), he was discharged from the hospital without any neurologic deficits.
Comment The natural history of CLVD has not been studied in depth because it is a rare congenital disease [1]. Marijon Fig 1. (a) Cardiac magnetic resonance image showing the outpouching of the posterolateral left ventricular wall (2 2 2 cm) (arrow). (b) Cardiac computed tomographic view. The white arrow shows the relationship between the outpouching of left ventricular wall and oblique margin branch of corona artery.
Ó 2017 by The Society of Thoracic Surgeons Published by Elsevier Inc.
0003-4975/$36.00 http://dx.doi.org/10.1016/j.athoracsur.2017.03.029
e272
CASE REPORT YAMASAKI ET AL CONGENITAL LEFT VENTRICULAR DIVERTICULUM
Ann Thorac Surg 2017;104:e271–3
Fig 2. Operative photo showing (a) a contractile but thin outpouching of posterolateral left ventricular wall and (b) an expanded polytetrafluoroethylene patch, excluding the orifice of the outpouching (around 3 cm).
and colleagues [2] described that CLVD on a contractile wall consists of three layers of outpouched wall, as opposed to congenital left ventricular aneurysm (CLVA), which lacks wall contractility and the three layers of outpouching wall. Patients with CLDA also have a poorer prognosis than do those with CLVD. Furthermore, there are two subtypes of CLVD: apical type cases are always associated with other cardiac anomalies or midline thoracoabdominal defects, whereas nonapical type cases occur in isolation [2]. However, the defining difference between CLVD and CLVA has not yet been clearly done. In regard to our case, cardiac MRI revealed contractile motion of the left ventricular wall outpouching, which was located on the posterolateral wall of the left ventricle. Pathologic examination showed that this outpouching wall had contained a cardiac muscle layer, although the size of cardiac myocytes varied and the layer demonstrated marked fibrosis. Inasmuch as the patient had no other congenital anomaly, we defined this case as nonapical type CLVD. Regarding the treatment of CLVD, it has been reported that surgical treatment is a reasonable option,
considering the risk of left ventricular rupture [3]. However, an appropriate consideration of the operative risks and benefits is warranted because a previous study reported a lack of changes in CLVD size over a period of long-term medical follow-up [4]. Nonetheless, Yalonetsky and colleagues [5] reported that a subset of CLVD may be associated with several potentially fatal adverse events, including systemic ventricular tachyarrythmias (VT), such as the ventricular fibrillation (VF) seen in our case, in addition to systemic embolism, sudden death, spontaneous rupture, and severe valvular regurgitation. In our case, EPS exploration triggered VF, which we identified as originating from the outpouching left ventricle. Although a previous case report describes the placement of an intravenous implantable cardioverter defibrillator (IVICD) for a patient with sustained monomorphic VT and CLVD in close proximity to the mitral valve [6], we decided to perform a surgical intervention for our patient, followed by left ventricular wall cryoablation to prevent both recurrent VF and left ventricular rupture. Before discharging the patient, we opted to implant an SICD rather than an IVICD in view of the lower rates of lead-related adverse events, like infective endocarditis [7], in addition to the fact that the patient did not require pacing and the possibility of later removal. Here, we present a case of a patient with a CLVD complicated by VF, which needed aggressive intervention including cardiopulmonary resuscitation and ECMO, who successfully underwent subsequent surgical treatment for CLVD followed by SICD placement. We think that the combination of the operation with the exclusion of CLVD and cryoablation and the implantation of an SICD was an appropriate treatment because the outpouched wall was very thin, the patient was young and at a low risk for surgical repair, and he also had the potential of recurrent VT in the future.
References Fig 3. Photomicrograph of tissue sample from outpouching showing myocardial tissue with different sizes of cardiac myocytes and marked fibrosis (hematoxylin and eosin stain, 4).
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.
Ann Thorac Surg 2017;104:e271–3
2. Marijon E, Ou P, Fermont L, et al. Diagnosis and outcome in congenital ventricular diverticulum and aneurysm. J Thorac Cardiovasc Surg 2006;131:433–7. 3. Tanaka M, Kawahito K, Kaneda H, Saito S. Congenital left ventricular diverticulum in an adult. Eur Heart J 2007;28:1537. 4. Archbold RA, Robinson NM, Mills PG. Long-term follow up of a true contractile left ventricular diverticulum. Am J Cardiol 1999;83:810–3; A11.
CASE REPORT YAMASAKI ET AL CONGENITAL LEFT VENTRICULAR DIVERTICULUM
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5. Yalonetsky S, Agmon Y, Lessick J. Contrast echocardiographic imaging of left ventricular diverticulum in adult patients. J Am Soc Echocardiogr 2006;20:e1–3. 6. Sierra M, Huynh H, Machado C. Congenital ventricular diverticulum presenting as sustained monomorphic ventricular tachycardia. Int J Cardiol 2009;133:e70–2. 7. Brouwer TF, Yilmaz D, Lindeboom R, et al. Long-term clinical outcomes of subcutaneous versus transvenous implantable defibrillator therapy. J Am Coll Cardiol 2016;68:2047–55.