Echocardiographic differentiation of atrioventricular septal defects from inlet VSD with or without mitral clefts

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Slide 1 :

Echocardiographic differentiation of atrioventricular septal defects from inlet VSD with or without mitral clefts Sulafa KM Ali, Tamimi OR, Godman MJ

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PM inlet ventricular septal defect (VSD) and “isolated” mitral valve clefts (MVC) are anatomically distinct from atrioventricular septal defect (AVSD). Introduction

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Echocardiographic differentiation is often difficult Introduction

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VSD and IMC often coexist in patients with Down’s syndrome who frequently have AVSD. In partial AVSD the size of the primum ASD as well as the inlet VSD can be small. AVSD can exist with intact interatrial and interventricular septa**. Smallhorn JF et al. Brit Heat J 1982 * Tamura et al J Am Coll Cardiol 2000

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This differentiation is of practical significance especially for the surgeon as the conduction system in AVSD is displaced posteriorly. Introduction

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1. Left ventricle inlet /outlet ratio (I/O ratio) 2. Percentage of the left atrioventricular valve guarded by the posterior leaflet. (PLPL) Patricia A., Robert H. Anderson et al.J Thora Cardiovasular Surg 1985 Morphological Differentiation

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Introduction We do not have quantitative echocardiographic data for the normal inlet / outlet ratio and the percentage of the left AVV guarded by the mural leaflet.

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ECHO measurement of inlet /outlet ratio and the percentage of the left AVV guarded by the mural leaflet has not been well established. Fraisser et al reported 5 patients (Cardiology in the Young 2002 )

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Gutgesell et al measured the contribution of atrial and ventricular septa to the total cardiac septal length and found that atrial component is normal in length. Gutgesell et al. J Am Coll Cardiol 1986 Dec

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Indirect measurement of the mural leaflet by measuring the arc between the bases of the two papillary muscles. Kohl et al. Am J Cardiol. 1996

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Position of papillary muscles and direction of the cleft. Kohl et al noted that cleft directional was not helpful in echo differentiation.* When there is a large VSD with the MVC the cleft can be pointing towards the interventricular septum.** *Kohl T et al. Am J Cardiol 1996. **S.Van Pragh et al, Ann Thorac Surg 2003

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Non - AVSD cleft AVSD *R Snieder. Echocardiography in Pediatric Heart Disease 1997

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Methodology

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January 2001- Dec 2003. Jan2001- June 2003: retrospective review. June – Dec 2003 : prospective using a pre-set protocol for measurements Methodology

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Methodology All echocardiographic studies were done using HP Sonos 5500 Enconcert system Images were stored digitally and measurements were done offline by two observers.

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Methodology Intra-observer variation : Random sample repeated by same observer. Inter-observer variation: Random sample repeated by a second observer.

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Inclusion criteria A. All patients with the diagnosis of: 1. AVSD (complete and partial). 2.Isolated perimembranous inlet VSD. 3.Mitral valve cleft ( with or without associated lesions). B. 100 controls with normal ECHO examination: infants 1-90 days of age.

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Exclusion criteria Patients with poor quality ECHO studies. Patients with associated complex cardiac abnormalities.

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Measurements

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Measurements

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Measurements

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Measurements

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Measurements

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Measurements A P P X 100 A+P

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Correlation of Measurements with TEE and Surgical Findings Operative reports of 19 patients with partial AVSD and inlet VSD +/- MVC. 17 had intra-operative TEE

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Results

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Results 152 patients. 3 groups: 1: Normal (n = 101) 2: AVSD (n = 36; 21 complete and 15 partial) 3: isolated inlet VSD with /without MVC (n = 15; 3 VSD + MVC).

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Results There was no difference between measurement of I/O ratio from PLA and that taken from the apical 4 chamber with aorta. No difference between I/O ratio in partial and complete AVSD. I/O ratio from 4 chamber/aorta view could not be done for complete AVSD. Left AVV measurement was not done for complete AVSD

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Results No intra observer variation No inter observer variation when measurements were done prospectively

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Mean+/-(SD)

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Results For AVSD group the I/O ratio was significantly lower than normal 0.82 +/- 0.062; p = 0.001 (95%CI: 0.180-0.237).

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Results For inlet VSD/ MVC group the I/O ratio was low compared to normal, 0.938 +/- 0.08, p =0 .001 (95%CI: 034-.116) but still higher than the AVSD group, p =0 .001 (95%CI -0.175--0.091).

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Results Percentage of the left atrioventricular valve guarded by the posterior leaflet For AVSD group it was 48.30 +/- 2.711, p=0 .001 (95% CI: 3.42-6.40). This percentage for inlet VSD/MVC group was NORMAL (53.92 +/- 1.96).

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Correlation of Measurements with TEE and Surgical Findings 12/19 (63%) were correctly diagnosed by conventional TT Echo 16/19 (83%) were correctly diagnosed by the new measurements. 15/17 (88%) diagnosed by TEE

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Three patients were missed by the measurement of PLPL Two/3 patients had AVSD with intact interatrial septum.

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This is the first study to set the normal and abnormal echo values for left ventricle inlet/outlet ratio and the percentage of the left AVV guarded by the posterior leaflet .

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I/O ratio allowed a clear distinction between normal patients and those with AVSD.

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Patients with inlet VSD with or without MVC had a ratio that is less than normal but this group was still distinct from AVSD

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Deficiency of the inlet septum may have led to a relatively elongated outflow tract.

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S.Van Praagh et al found that I/O ratio was low in patients with ‘isolated‘ MVC and normally related GA. Most of the patients (15/18) in her study had associated VSD (mainly inlet). S.Van Bragh et al, Ann Thorac Surg 2003

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The conclusion from that study was that: “ Isolated mitral cleft could be considered as a milder variation of AVSD”

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We disagree ……..

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We believe that a relatively low I/O ratio by itself is not enough evidence that this pathology is a “forme-fruste’ of AVSD.

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1. A normal mitral valve posterior leaflet dimension. 2. The I/O ratio is still distinctly higher than the AVSD range.

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These measurements improved diagnostic accuracy of TT Echo by 20% and approached TEE accuracy. Left AVV posterior leaflet measurement was more discriminative than I/O ratio.

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Limitations In retrospective cases the quality of echocardiograms would affect measurement. Small no of isolated clefts.

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Conclusion We established the normal inlet/outlet ratio and percentage of the left AVV guarded by mural leaflet echocardiographically. These two measurements are useful in differentiating inlet VSD (+/- MVC) from AVSD.

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Be thoughtful!!!

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