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 Table of Contents  
INTERSTITIAL LUNG DISEASES AND LUNG IN SYSTEMIC DISE
Year : 2020  |  Volume : 4  |  Issue : 2  |  Page : 200-202

Cardiac Diverticulum


Department of Cardiology, Hero DMC Heart Institute, Ludhiana, Punjab, India

Date of Submission21-May-2019
Date of Decision03-Nov-2019
Date of Acceptance24-Nov-2019
Date of Web Publication19-Aug-2020

Correspondence Address:
Dr. Rohit Tandon
Hero DMC Heart Institute, Ludhiana - 141 001, Punjab
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jiae.jiae_25_19

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  Abstract 

Left ventricular diverticulum (LVD) is a rare clinical entity which contains endocardium, myocardium, and pericardium with normal contractility. Contrary to LV aneurysm, which has thin and fibrotic LV wall with paradoxical movement compared to normal left ventricle contraction. LVD are two types, that is, congenital or acquired. Congenital is more common than acquired form. Classification according to the location is either apical or nonapical. Apical diverticula usually associated with midline thoracoabdominal defects and other cardiac malformations, but nonapical diverticula were isolated. Nonapical diverticula can arise from the anterior-free wall, the subaortic region, or, rarely, from both ventricles. A diagnosis can be usually done by echocardiography, whereas computed tomography angiography, magnetic resonance imaging, and invasive ventriculography also help. A major problem with LVD is thrombosis, embolism, rupture, ventricular arrhythmias, congestive heart failure, and valvular abnormalities. The management of LVD depends on the clinical condition and associated abnormalities. In most asymptomatic person can be managed conservatively. Treatment for high-risk cases includes surgery, anticoagulants, and treatment of arrhythmias.

Keywords: Diverticulum, echocardiography, left ventricle


How to cite this article:
Pandey N, Aseri R, Tandon R, Mohan B, Wander GS. Cardiac Diverticulum. J Indian Acad Echocardiogr Cardiovasc Imaging 2020;4:200-2

How to cite this URL:
Pandey N, Aseri R, Tandon R, Mohan B, Wander GS. Cardiac Diverticulum. J Indian Acad Echocardiogr Cardiovasc Imaging [serial online] 2020 [cited 2020 Sep 29];4:200-2. Available from: http://www.jiaecho.org/text.asp?2020/4/2/200/292623


  Introduction Top


Left ventricular diverticulum (LVD) is a rare clinical entity which contains endocardium, myocardium, and pericardium with normal contractility. Contrary to LV aneurysm, which has thin and fibrotic LV wall with paradoxical movement compared to normal left ventricle contraction. LVD are two types, that is, congenital or acquired. Congenital is more common than acquired form. Classification according to the location is either apical or nonapical. Apical diverticula usually associated with midline thoracoabdominal defects and other cardiac malformations, but nonapical diverticula were isolated. Nonapical diverticula can arise from the anterior-free wall, the subaortic region, or, rarely, from both ventricles. A diagnosis can be usually done by echocardiography, whereas computed tomography angiography, magnetic resonance imaging, and invasive ventriculography also help. A major problem with LVD is thrombosis, embolism, rupture, ventricular arrhythmias, congestive heart failure, and valvular abnormalities. The management of LVD depends on the clinical condition and associated abnormalities. In most asymptomatic person can be managed conservatively. Treatment for high-risk cases includes surgery, anticoagulants, and treatment of arrhythmias.


  Clinical Presentation Top


Case 1

A 24-year-old male presented with a history of two episodes of syncope. Electrocardiography (ECG) showed monomorphic right bundle branch block morphology ventricular tachycardia. Echocardiography revealed that normal left ventricular (LV) function with no regional wall abnormality, except an abnormal diastolic outpouching of the LV mid-septal wall [Videos 1-3].










Case 2

A 34-year-old female presented with progressive dyspnea. On evaluation, hemogram, renal function, and ECG were normal, except brain natriuretic peptide which was raised. Two-dimensional (2D) transthoracic echo was done which showed normal left and right ventricular systolic and diastolic function, but there was an LV apical diverticulum [Video 4].




Case 3

A 20-year-old male presented with a history of progressive dyspnea. A chest X-ray showing left hemidiaphragm elevation. His 2D echocardiography revealed basal septum diverticulum with characteristic diastolic expansion and systolic contraction [Figure 1] and Videos 5-7].
Figure 1: Left side elevated hemidiaphragm

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Case 4

A 30-year-old female presented with chest pain which was intermittent and not related to exertion. ECG was normal. 2D echocardiography at the apical four-chamber view revealed that patient had LV apical wall outpouching with systolic contraction and diastolic expansion [Video 8].




Case 5

A 70-year-old female with permanent atrial fibrillation presented complaints of shortness of breath. On evaluation, echo was done which showed pulmonary artery anterior wall abnormal outpouching which has systolic contraction and diastolic expansion [Videos 9 and 10].








  Discussion Top


Cardiac diverticulum is a rare clinical entity that involves left ventricle more commonly. LV diverticulum (LVD) is an outpouching of the left ventricle which contains endocardium, myocardium, and pericardium with normal contractility. Contrary to LV aneurysm which has thin and fibrotic LV wall with paradoxical movement compared to normal left ventricle contraction.

LVD first described in the 1800s.[1],[2] It is a rare clinical entity The incidence of LVD was approximately 0.04%–0.4% in the previous studies. Nowadays with high-quality imaging techniques, the incidence of LVD is increased to 2.2%.[3]

It develops in the 4th embryonic week that forms along both the right and left ventrolateral borders of the cardiac tube. Partial cessation in the development of the embryonic ventricle may be responsible for the development of a congenital LVD.[4] Possible etiologies are either intrinsic abnormalities of embryogenesis or acquired malformations (such as viral infections, arrhythmia-related vascular accidents, or cardiomyopathies) during intrauterine life.[5]

LVD are two types, that is, congenital or acquired. Congenital is more common than acquired form. Classification according to the location is either apical (70%) or nonapical (30%).[6] Nonapical diverticula can arise from the anterior free wall, the subaortic region,[7] or, rarely, from both ventricles.[8] Most of the apical diverticulum is found with midline thoracoabdominal defects [9] and complex cardiac abnormalities.

A syndrome associated with LVD is Cantrell's syndrome was described by Cantrell et al. in 1958,[10],[11] which includes abdominal wall defects, sternal defects, pulmonary atresia, atrial septal defect, patent ductus arteriosus, Tetralogy of Fallot, ectopia cordis, and diverticulum.[10],[12] Besides these features, associated abnormality include craniofacial abnormalities; hypoplasia of lung,[13] liver,[14] kidneys,[15] and adrenal; limb defects; and malrotation of the gut, there are defects in the development of mesoderm of the anterior transverse septum of the diaphragm is a probable cause.[13]

Differential diagnosis of LV myocardial diseases includes aneurysm, pseudoaneurysm, diverticulum, and LV noncompaction.

Differentiation between a ventricular aneurysm from a diverticulum is critically important. Diverticulum has a narrow mouth and synchronous contractility. Whereas aneurysms show akinesia or paradoxical movement of the outpouching, which is asynchronous with the rest of the heart.[16]

Diagnosis can be done by echocardiography, computed tomography angiography, magnetic resonance imaging, and invasive ventriculography. Functional hemodynamic evaluation can be done and any associated intracardiac abnormalities can be detected with echocardiography.


  Conclusion Top


As LVD is a rare anomaly, the natural history of LVD is not completely understood. Major concerns are thrombosis, embolism, rupture, ventricular arrhythmias, congestive heart failure, and valvular abnormalities. A review of 22 patients with congenital LVD revealed that apical diverticula were linked with midline thoracoabdominal defects and other cardiac malformations, but nonapical diverticula were isolated. Ten out of 22 patients with LV diverticulum were not surgically treated. In them, two had spontaneous regression, and eight remained asymptomatic for a mean follow-up period of 8.4 years. In one report on LVD, it was found that LVD did not increase in size over 13 years, favoring its benign nature.[17] Even so, patients with LVD associated sustained monomorphic ventricular tachycardia require either an implantable cardioverter defibrillator or cryoablation surgery.[18],[19],[20] Another school of thought suggests immediate surgical resection to prevent complications.[21] In view of the inadequate data for universal guidance, decisions on the management of LVD should be tailored to the clinical characteristics of each patient, taking into consideration associated abnormalities and potential complications.

The management of LVD depends on the clinical condition and associated abnormalities. In most asymptomatic person can be managed conservatively, a similar approach which we had adopted in all our cases. Treatment for high-risk cases includes surgery, anticoagulants, and treatment of arrhythmias.[22]

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
Mardini MK. Congenital diverticulum of the left ventricle. Report of two unusual cases. Br Heart J 1984;51:321-6.  Back to cited text no. 1
    
2.
Suilen C, Friedli B, Rutishauser W. Congenital intrathoracic left ventricular diverticulum in an adult. Chest 1990;98:750-1.  Back to cited text no. 2
    
3.
Srichai MB, Hecht EM, Kim DC, Jacobs JE. Ventricular diverticula on cardiac CT: More common than previously thought. AJR Am J Roentgenol 2007;189:204-8.  Back to cited text no. 3
    
4.
Coker WG, Beale JD, Gross FB. Pseudo trilocular biatrial heart without pulmonary stenosis; report of a case and discussion of the related embryology. AMA Am J Dis Child 1951;81:671-4.  Back to cited text no. 4
    
5.
Makkuni P, Kotler MN, Figueredo VM. Diverticular and aneurysmal structures of the left ventricle in adults: Report of a case within the context of a literature review. Tex Heart Inst J 2010;37:699-705.  Back to cited text no. 5
    
6.
Gowitt GT, Zaki SA. Rupture of a cardiac diverticulum resulting in sudden death. Am J Forensic Med Pathol 1988;9:155-8.  Back to cited text no. 6
    
7.
Deng Y, Sun Z, Dong N, Du X. Congenital cardiac diverticulum in the subaortic valve area. J Thorac Cardiovasc Surg 2006;132:1087-91.  Back to cited text no. 7
    
8.
Maroto C, Maroto E, García EJ, Vallés P, Delcán JL, Arcas R. Congenital cardiac diverticulum originating from both ventricles. Rev Esp Cardiol 1991;44:351-4.  Back to cited text no. 8
    
9.
Klein G. Casuistics of congenital cardiac diverticulum. Zentralbl Allg Pathol 1953;90:14-7.  Back to cited text no. 9
    
10.
Knight L, Neal WA, Williams HJ, Huseby TL, Edwards JE. Congenital left ventricular diverticulum. Part of a syndrome of cardiac anomalies and midline defects. Minn Med 1976;59:372-5.  Back to cited text no. 10
    
11.
El Kettani NE, Dafiri R. Isolated congenital left ventricular diverticulum: Report of a paediatric case. J Radiol 2006;87:56-8.  Back to cited text no. 11
    
12.
Edgett JW Jr., Nelson WP, Hall RJ, Fishback ME, Jahnke EJ. Diverticulum of the heart. Part of the syndrome of congenital cardiac and midline thoracic and abdominal defects. Am J Cardiol 1969;24:580-3.  Back to cited text no. 12
    
13.
Halbertsma FJ, van Oort A, van der Staak F. Cardiac diverticulum and omphalocele: Cantrell's pentalogy or syndrome. Cardiol Young 2002;12:71-4.  Back to cited text no. 13
    
14.
Toyama WM. Combined congenital defects of the anterior abdominal wall, sternum, diaphragm, pericardium, and heart: A case report and review of the syndrome. Pediatrics 1972;50:778-92.  Back to cited text no. 14
    
15.
Yuan SM, Shinfeld A, Mishaly D. An incomplete pentalogy of Cantrell. Chang Gung Med J 2008;31:309-13.  Back to cited text no. 15
    
16.
Fernández MS, López A, Vila JJ, Lluna J, Miranda J. Cantrell's pentalogy. Report of four cases and their management. Pediatr Surg Int 1997;12:428-31.  Back to cited text no. 16
    
17.
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.  Back to cited text no. 17
    
18.
Sierra M, Huynh H, Machado C. Congenital ventricular diverticulum presenting as sustained monomorphic ventricular tachycardia. Int J Cardiol 2009;133:e70-2.  Back to cited text no. 18
    
19.
Deepak BV, Alsous F, Mathur R, Zarich S. Congenital left ventricular diverticulum presenting as ventricular tachycardia in an elderly woman. Am J Geriatr Cardiol 2007;16:262-5.  Back to cited text no. 19
    
20.
Shen EN, Fukuyama O, Herre JM, Yee E, Scheinman MM. Ventricular tachycardia with congenital ventricular diverticulum. Chest 1991;100:283-5.  Back to cited text no. 20
    
21.
Tanaka M, Kawahito K, Kaneda H, Saito S. Congenital left ventricular diverticulum in an adult. Eur Heart J 2007;28:1537.  Back to cited text no. 21
    
22.
Perlitz Y, Mukary M, Lorber A, Ben-Ami M. Prenatal diagnosis of fetal cardiac right ventricular diverticulum disappearing at three months of age. A case report and literature review. Fetal Diagn Ther 2009;25:44-6.  Back to cited text no. 22
    


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