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
   Introduction
   Case Report
   Discussion
    References
    Article Figures

 Article Access Statistics
    Viewed1973    
    Printed46    
    Emailed0    
    PDF Downloaded173    
    Comments [Add]    

Recommend this journal

 


 
Table of Contents   
CASE REPORT  
Year : 2013  |  Volume : 6  |  Issue : 2  |  Page : 200-201
VACTERL association with double-chambered left ventricle: A rare occurrence


1 Department of Pediatric Cardiology, Royal Hospital, Muscat, Sultanate of Oman
2 Department of Adult Congenital Heart Disease, Royal Hospital, Muscat, Sultanate of Oman

Click here for correspondence address and email

Date of Web Publication20-Jul-2013
 

   Abstract 

VACTERL association is a non-random association of birth defects of unknown etiology derived from structures of embryonic mesoderm. The common cardiac defects seen with VACTERL association are ventricular septal defects, atrial septal defects, and tetralogy of Fallot. We present a 2-year-old child with VACTERL association in whom we detected double-chambered left ventricle on transthoracic echocardiography.

Keywords: Double-chambered left ventricle, VACTERL association, birth defects

How to cite this article:
Al-Farqani A, Panduranga P, Al-Maskari S, Thomas E. VACTERL association with double-chambered left ventricle: A rare occurrence. Ann Pediatr Card 2013;6:200-1

How to cite this URL:
Al-Farqani A, Panduranga P, Al-Maskari S, Thomas E. VACTERL association with double-chambered left ventricle: A rare occurrence. Ann Pediatr Card [serial online] 2013 [cited 2019 Apr 18];6:200-1. Available from: http://www.annalspc.com/text.asp?2013/6/2/200/115283



   Introduction Top


VACTERL association is defined by the presence of at least three of the following congenital anomalies: Vertebral defects, anal atresia, cardiac defects, tracheo-esophageal fistula, renal anomalies, and limb abnormalities. [1] The incidence is 1 in 10,000 to 1 in 40,000 live-born infants. [1] In 40-80% of patients with VACTERL association, cardiac malformations are reported. [1] The common cardiac defects seen with VACTERL association are ventricular septal defects, atrial septal defects, hypoplastic left heart syndrome, patent ductus arteriosus, transposition of the great arteries, truncus arteriosus, and tetralogy of Fallot. [2],[3] We present a 2-year-old child with VACTERL association in whom we detected double-chambered left ventricle on transthoracic echocardiography, which has not been reported previously in this syndrome.


   Case Report Top


A 2-year-old male asymptomatic child came for echocardiogram referred from an orthopedic center. His past history was significant for various limb and urachal anomalies of VACTERL association including bilateral radial aplasia, club hand, absent thumbs, sacral agenesis, and urachal sinus. At 9 months of age, he was diagnosed to have apical muscular ventricular septal defects and secundum atrial septal defect by echocardiography done elsewhere. On examination, he had normal development and normal intelligence for his age. There were dysmorphic facial features with bilateral radial club hand, absent thumbs, and short upper and lower limbs [Figure 1]a. Chest examination was normal with cardiac auscultation revealing regular heart sounds with no murmur. His ECG and Chest X-ray were unremarkable. Sacral X-ray showed partial sacral agenesis [Figure 1]b.
Figure 1: X-ray of right upper limb demonstrating radial aplasia, ulnar bowing, absent thumb, and four metacarpals (a) and partial agenesis of sacrum (b) in a patient with VACTERL association and double-chambered left ventricle

Click here to view


Transthoracic echocardiography demonstrated situs solitus with normal atrioventricular and ventriculoarterial concordance. There were no ventricular septal defects. The left ventricle (LV) was abnormally configured with a muscle band extending from the interventricular septum toward the apex dividing the LV into two separate contracting LV chambers. A typical "figure-of-eight" appearance was seen in the parasternal short-axis view in diastole indicating double-chambered left ventricle (DCLV) [Figure 2]a arrowheads, [Video 1]. During systole, the LV was seen leading to aorta [Figure 2]b. There was laminar flow inside the LV with no intraventricular gradient. Rest of the cardiac examination was normal with good biventricular function.
Figure 2: Transthoracic echocardiography in short-axis inverted view showing a transverse muscle band traversing the left ventricle dividing the left ventricle into two halves producing a typical "figure-of-eight" appearance indicating a double-chambered left ventricle in a patient with VACTERL association (a), (b) Demonstrates the left ventricle in systole leading to aorta confirming double-chambered left ventricle. RV = right ventricle; LV = left ventricle chamber; LV1 = left ventricle chamber 1; LV2 = left ventricle chamber 2; AO = aorta

Click here to view



   Discussion Top


In this patient with VACTERL association, we detected DCLV, which is an uncommon association. DCLV was first conclusively described by Kay et al., in 1983. [4] DCLV represents division of the LV into two chambers by abnormal muscular tissue similar to double-chambered right ventricle. However, the chambers of a DCLV are in parallel with no significant pressure gradient, as both chambers contract synchronously. [5] The pathogenesis of DCLV is less understood and it is thought to be congenital and non-progressive. [5] It was initially thought to be a cardiomyopathy, [4] but other authors describe it as a congenital anomaly due to abnormal septum or a muscle bundle across the LV between septum and left ventricular wall. [5],[6],[7],[8] Congenital LV out-pouching like LV aneurysms or diverticula need to be differentiated from DCLV, and if in doubt, computed tomography scan, magnetic resonance imaging, or invasive angiography will aid in the diagnosis. [5],[6],[7] However, in this patient, there was clear separation of two chambers within the LV cavity which contract during systole. DCLV, by itself, may not require any treatment. If DCLV causes obstruction or tachyarrhythmias, then the muscle bundles need to be surgically excised. [5],[6],[8] B-blocker use or surgical resection has been reported to reduce the gradient if present. [8],[9] In conclusion, this case report documents that patients with VACTERL association may rarely have a DCLV as the cardiac anomaly.

Viseo 1:


 
   References Top

1.Solomon BD. VACTERL/VATER Association. Orphanet J Rare Dis 2011;6:56.2.  Back to cited text no. 1
    
2.Botto LD, Khoury MJ, Mastroiacovo P, Castilla EE, Moore CA, Skjaerven R, et al. The spectrum of congenital anomalies of the VATER association: An international study. Am J Med Genet 1997;71:8-15.  Back to cited text no. 2
    
3.Kallen K, Mastroiacovo P, Castilla EE, Robert E, Kallen B. VATER non-random association of congenital malformations: Study based on data from four malformation registers. Am J Med Genet 2001;101:26-32.  Back to cited text no. 3
    
4.Kay PH, Rigby M, Mulholland HC. Congenital double chambered left ventricle treated by exclusion of accessory chamber. Br Heart J 1983;49:195-8.  Back to cited text no. 4
    
5.Nacif MS, Mello RA, Lacerda Junior OO, Sibley CT, Machado RA, Marchiori E. Double-chambered left ventricle in an adult: Diagnosis by CMRI. Clinics (Sao Paulo) 2010;65:1393-5.  Back to cited text no. 5
    
6.Awad SM, Patel AS, Polimenakos A, Braun R, Abdulla RI. Left ventricular accessory chamber: A case report and review of the literature. Pediatr Cardiol 2009;30:1022-5.  Back to cited text no. 6
    
7.Breithardt OA, Ropers D, Seeliger T, Schmid A, von Erffa J, Garlichs C, et al. A heart within the heart: Double-chambered left ventricle. Eur J Echocardiogr 2008;9:739-41.  Back to cited text no. 7
    
8.Köz C, Yokuþoðlu M, Uzun M, Baysan O, Bulakbaºi N. Double-chambered left ventricle with nonsustained ventricular tachycardia. Anadolu Kardiyol Derg 2009;9:E5.  Back to cited text no. 8
    
9.Dogan OF, Alehan D, Duman U. Successful surgical management of a double-chambered left ventricle in a 13-year-old girl: A report of a rare case. Heart Surg Forum 2004;7:E198-200.  Back to cited text no. 9
    

Top
Correspondence Address:
Prashanth Panduranga
Department of Adult Congenital Heart Disease, Royal Hospital, Post Box 1331, Muscat-111
Sultanate of Oman
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0974-2069.115283

Rights and Permissions


    Figures

  [Figure 1], [Figure 2]



 

Top