Annals of Pediatric Cardiology
About us | Current Issue | Archives | Ahead of Print | Instructions | Submission | Subscribe | Advertise | Contact | Login 
     
     
 


 

 
     
    Advanced search
 

 
 
     
 
    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
    Email Alert *
    Add to My List *
* Registration required (free)  


    Abstract
   Clinical Summary
   Discussion
    References
    Article Figures

 Article Access Statistics
    Viewed61    
    Printed0    
    Emailed0    
    PDF Downloaded9    
    Comments [Add]    

Recommend this journal

 


 
Table of Contents   
IMAGES  
Year : 2020  |  Volume : 13  |  Issue : 4  |  Page : 368-370
Congenital right ventricular diverticulum: An atypical presentation


1 Department of Cardiac Surgery, Narayana Superspeciality Hospital, Howrah, West Bengal, India
2 Department of Cardiac Anaesthesia, Narayana Superspeciality Hospital, Howrah, West Bengal, India
3 Department of Pediatric Cardiology, Narayana Superspeciality Hospital, Howrah, West Bengal, India
4 Department of Pediatric Intensive Care, Narayana Superspeciality Hospital, Howrah, West Bengal, India

Click here for correspondence address and email

Date of Submission25-Mar-2020
Date of Decision08-May-2020
Date of Acceptance08-May-2020
Date of Web Publication24-Jul-2020
 

   Abstract 


Congenital ventricular diverticulum is a rare congenital malformation of the heart. It remains mostly asymptomatic unless associated with other congenital cardiac defects. We present a case of a 4-month-old child who had dextrocardia, large inlet ventricular septal defect, amounting to single ventricle, and severe pulmonary artery hypertension. There was a right ventricular diverticulum which passed through the foramina of Morgagni to give rise to a pulsating lump in the epigastrium. Right ventricular diverticulum presenting with a pulsating mass in the abdomen is a rare entity. The confirmation of diverticulum was made with histopathology.

Keywords: Cardiac anatomy, myocardial morphology, pathology, right ventricle

How to cite this article:
Das D, Dutta N, Das AK, Sharma MK, Koley R, Chattopadhyay A, Ghosh S, Das S. Congenital right ventricular diverticulum: An atypical presentation. Ann Pediatr Card 2020;13:368-70

How to cite this URL:
Das D, Dutta N, Das AK, Sharma MK, Koley R, Chattopadhyay A, Ghosh S, Das S. Congenital right ventricular diverticulum: An atypical presentation. Ann Pediatr Card [serial online] 2020 [cited 2020 Oct 28];13:368-70. Available from: https://www.annalspc.com/text.asp?2020/13/4/368/297234





   Clinical Summary Top


A 4-month-old child weighing 5 kg was admitted to our hospital with features of heart failure. Clinical examination of the child revealed a small pulsating lump in the epigastrium. Chest roentgenography suggested dextrocardia and cardiomegaly with pulmonary plethora.

Echocardiography revealed situs solitus, dextrocardia, bilateral superior vena cava, large inlet ventricular septal defect with outlet extension, amounting to single ventricle, and severe pulmonary artery (PA) hypertension. There was no evidence of ventricular diverticulum. Electrocardiogram revealed features of left heart volume overload without any arrhythmias.

In view of epigastric pulsation, contrast-enhanced computed tomography of the thorax was ordered. The scan gave an impression of divarication of recti in the midline in the lower chest. Although there was very faint opacification of the outpouching, it seemed to be arising from the right ventricle (RV) [Figure 1].
Figure 1: Computed tomography scan thorax revealing ventricular outpouching

Click here to view


We decided to do PA banding and excise any diverticulum, if found intraoperatively. Decision of banding was backed by very large ventricular septal defect amounting to single ventricle and low body weight.

Operation was done through midline sternotomy without cardiopulmonary bypass. There was a pulsating outgrowth from the anterior surface of the RV traversing through the foramina of Morgagni into abdomen [Figure 2]a and [Figure 2]b. The ventricular end of the diverticulum was clamped and divided, without cardiopulmonary bypass. The RV was repaired by continuous 4-0 prolene mattress sutures over Teflon felt [Figure 3]. The diaphragmatic opening was closed with 4-0 prolene by continuous technique. PA banding followed thereafter.
Figure 2: (a) Right ventricle diverticulum with clamp at the base. (b) Foramina of Morgagni showing the path through the diaphragm

Click here to view
Figure 3: Postventricular aneurysm repair and pulmonary artery band

Click here to view


Histopathological examination of the specimen showed a central lumen lined by all three layers of cardiac tissue, i.e., endocardium, myocardium, and epicardium [Figure 4]a, ×20, H and E, stain]. In addition, there were some feeding vessels in myocardium with intraluminal thrombus [Figure 4]b, ×4, H and E, stain] and lined by the endothelium. Immunohistochemistry revealed CD34 cells lining the feeding vessels [Figure 4]c IHC]. The presence of intramural thrombus and CD34 cells is pathognomonic of hemangiomatous changes in the wall of diverticulum.
Figure 4: (a) Photomicrograph showing all layers, H and E, ×20, (b) feeding vessels in myocardium with intraluminal thrombus H and E, ×4, (c) immunohistochemistry showing CD34 cells

Click here to view


The child made an uneventful recovery after the surgery and is doing well on follow-up.


   Discussion Top


Congenital ventricular diverticulum is a very rare anomaly. They are characterized by outgrowth of the full-thickness ventricular wall and corresponding finger-like projection from the internal cavity. The true incidence remains unknown partly because of rarity and asymptomatic nature of the pathology. A prevalence of 0.05/10,000 live births has been reported in very old papers.[1]

In Ohlow's series,[2] 60% of patients were without symptoms. The most common presentations were syncope and rhythm disturbances, followed by chest pain and embolic events. A vast majority of patients are incidentally diagnosed when subjected to cardiac imaging for associated heart defects. Magrassi et al. found an interesting association between right ventricular diverticulum and tetralogy of Fallot though any heart defect is possible.[3] Sherman et al.[4] also found conotruncal anomalies to be the most common associated lesions.

Ventricular diverticulum is importantly differentiated from ventricular aneurysm by having all three layers of myocardium. In fact, it shows a contractile function synchronous with the ventricle. Contrastingly, aneurysms are always akinetic or dyskinetic.[5]

Ventricular diverticula are either apical or nonapical in origin. Apical diverticula are contractile projections extending into the abdomen through a defect in the diaphragm. There may be midline thoracoabdominal defects, as described by Cantrell et al.[6] These apical projections are very commonly associated with other congenital heart defects and malpositions of the heart. Nonapical diverticula are generally isolated without any midline defects. Our experience was completely opposite in this case. The child had nonapical diverticulum (arising from the anterior surface of the RV) but with all the associated features of apical ventricular diverticula, namely dextrocardia, heart defects, and pulsating epigastric hernia.

Histopathological examination of the diverticular tissue was also uncharacteristic in having typical hemangiomatous changes in the myocardium. The intramural thrombus inside the feeding vessels and abundance of CD34 cells were very typical of hemangiomas.

Natural course of these diverticula is decided by associated heart defects. Isolated diverticula can be put on close follow-up as complications are very rare. As the world literature of this entity is scanty, it is not surprising to encounter atypical presentations.

Consent

Consent for publication was granted by the patient's parents.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the parents have given their consent for images and other clinical information to be reported in the journal. The parents understand that names and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Buyse ML. Birth defects Encyclopedia. Cambridge, Massachusetts: Blackwell Scientific Publications; 1990.  Back to cited text no. 1
    
2.
Ohlow MA. Congenital left ventricular aneurysms and diverticula: Definition, pathophysiology, clinical relevance and treatment. Cardiology 2006;106:63-72.  Back to cited text no. 2
    
3.
Magrassi P, Chartrand C, Guerin R, Kratz C, Stanley P. True diverticulum of the right ventricle: Two cases associated with tetralogy of Fallot. Ann Thorac Surg 1980;29:357-63.  Back to cited text no. 3
    
4.
Sherman SJ, Leenhouts KH, Utter GO, Litaker M, Lawson P. Prenatal diagnosis of left ventricular aneurysm in the last second trimester: A case report. Ultrasound Obstet Gynecol 1996;7:456-457.  Back to cited text no. 4
    
5.
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. 5
    
6.
Cantrell JR, Haller JA, Ravitch MM. A syndrome of congenital defects involving the abdominal wall, sternum, diaphragm, pericardium and heart. Surg Gynecol Obstet 1958;107:602-14.  Back to cited text no. 6
    

Top
Correspondence Address:
Dr. Debasis Das
Department of Cardiac Surgery, Narayana Superspeciality Hospital, 120/1 Andul Road, Howrah - 711 103, West Bengal
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/apc.APC_49_20

Rights and Permissions


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

Top