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A case of pseudo-hypertrophic cardiomyopathy: a congenital heart disease.

Ieva R, Correale M, Di Biase M - Clinics (Sao Paulo) (2008)

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Echocardiography showed that the aorta extended from the morphological systemic ventricle (RV), which was identified by the three-leaflet tricuspid valve (Fig. 1) that was inserted more apically than the mitral valve (Fig. 2)... There was mild aortic regurgitation but no pulmonary valvular stenosis... A persistent patency of oval-shaped foramen was found... Holter monitoring demonstrated incessant tachycardia with only brief periods of sinus rhythm... The patient was submitted to electrophysiological evaluation, and the accessory pathway was successfully ablated using radiofrequency pulses... Thus, the LV supplies pulmonary circulation and the RV supports systemic circulation... CCTGA usually is associated with a severely reduced life expectancy due to ventricular septal defects (74%), pulmonary valvar stenosis (74%), systemic (tricuspid) valve abnormalities (38%), and complete heart block (5%)... Only 1–10% of individuals with CCTGA have no associated defects... Differential diagnosis includes hypertrophic cardiomyopathy, dilated cardiomyopathy, prominent normal myocardial trabeculations, cardiac tumors, and left ventricular apical thrombus... However, the position of the three-leaflet tricuspid valve, which was inserted more apically than the mitral valve, and particularly the atrioventricular and ventriculoarterial discordance indicated a diagnosis of congenitally corrected transposition of the great arteries (CCTGA)... Moreover, the mid-esophageal view showed the discontinuity between the aortic valve and the systemic atrioventricular valve because of the muscle interposition (Fig. 6); transthoracic view failed to reveal this discontinuity... As shown in the present case of echocardiographic visualization of severe ventricular hypertrophy and prominent trabeculations, examination of the ventriculoarterial and atrioventricular connections may be necessary... As a result of the impressive success of pediatric cardiac care, the number of adult patients with congenital heart disease is now greater than the number of pediatric cases.

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In prominent systemic ventricle hypertrophy, the aortic valve and the systemic atrioventricular valve are discontinuous because of muscle interposition
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f6-cln63_4p0557: In prominent systemic ventricle hypertrophy, the aortic valve and the systemic atrioventricular valve are discontinuous because of muscle interposition

Mentions: Prominent myocardial trabeculations can also be recognized in dilated cardiomyopathy, but to a lesser extent than in CCTGA. The transesophageal examination, which directly evaluated the morphological features of the appendage (Fig. 5), correctly determined atrial situs. Moreover, the mid-esophageal view showed the discontinuity between the aortic valve and the systemic atrioventricular valve because of the muscle interposition (Fig. 6); transthoracic view failed to reveal this discontinuity.


A case of pseudo-hypertrophic cardiomyopathy: a congenital heart disease.

Ieva R, Correale M, Di Biase M - Clinics (Sao Paulo) (2008)

In prominent systemic ventricle hypertrophy, the aortic valve and the systemic atrioventricular valve are discontinuous because of muscle interposition
© Copyright Policy
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC2664138&req=5

f6-cln63_4p0557: In prominent systemic ventricle hypertrophy, the aortic valve and the systemic atrioventricular valve are discontinuous because of muscle interposition
Mentions: Prominent myocardial trabeculations can also be recognized in dilated cardiomyopathy, but to a lesser extent than in CCTGA. The transesophageal examination, which directly evaluated the morphological features of the appendage (Fig. 5), correctly determined atrial situs. Moreover, the mid-esophageal view showed the discontinuity between the aortic valve and the systemic atrioventricular valve because of the muscle interposition (Fig. 6); transthoracic view failed to reveal this discontinuity.

View Article: PubMed Central - PubMed

AUTOMATICALLY GENERATED EXCERPT
Please rate it.

Echocardiography showed that the aorta extended from the morphological systemic ventricle (RV), which was identified by the three-leaflet tricuspid valve (Fig. 1) that was inserted more apically than the mitral valve (Fig. 2)... There was mild aortic regurgitation but no pulmonary valvular stenosis... A persistent patency of oval-shaped foramen was found... Holter monitoring demonstrated incessant tachycardia with only brief periods of sinus rhythm... The patient was submitted to electrophysiological evaluation, and the accessory pathway was successfully ablated using radiofrequency pulses... Thus, the LV supplies pulmonary circulation and the RV supports systemic circulation... CCTGA usually is associated with a severely reduced life expectancy due to ventricular septal defects (74%), pulmonary valvar stenosis (74%), systemic (tricuspid) valve abnormalities (38%), and complete heart block (5%)... Only 1–10% of individuals with CCTGA have no associated defects... Differential diagnosis includes hypertrophic cardiomyopathy, dilated cardiomyopathy, prominent normal myocardial trabeculations, cardiac tumors, and left ventricular apical thrombus... However, the position of the three-leaflet tricuspid valve, which was inserted more apically than the mitral valve, and particularly the atrioventricular and ventriculoarterial discordance indicated a diagnosis of congenitally corrected transposition of the great arteries (CCTGA)... Moreover, the mid-esophageal view showed the discontinuity between the aortic valve and the systemic atrioventricular valve because of the muscle interposition (Fig. 6); transthoracic view failed to reveal this discontinuity... As shown in the present case of echocardiographic visualization of severe ventricular hypertrophy and prominent trabeculations, examination of the ventriculoarterial and atrioventricular connections may be necessary... As a result of the impressive success of pediatric cardiac care, the number of adult patients with congenital heart disease is now greater than the number of pediatric cases.

Show MeSH
Related in: MedlinePlus