Double chambered right ventricle with ventricular septal defect in adults: case series and review of the literature.

Double-chambered right ventricle (DCRV) is an uncommon congenital anomaly in which anomalous muscle bands divide the right ventricle into two chambers; a proximal high-pressure and distal low-pressure chamber. It may be associated with mid right ventricular obstruction. It is commonly associated with other congenital anomalies, most frequently perimembranous ventricular septal defect (PM-VSD). We herein present 5 adult patients with concomitant DCRV and PM-VSD who varied in their symptomatic presentations and the ways of management.

Right and Left Atrial Dissimilarities in Normal Subjects Explored by Speckle Tracking Echocardiography.

BACKGROUND: Atrial function is an important contributor of ventricular function and has a prognostic role in various cardiovascular diseases. We tested the hypothesis that right and left atrial (RA & LA) function may not be equal despite their accommodating identical cardiac output. METHODS: Two-dimensional (2D) speckle tracking echocardiography was acquired from the apical four-chamber view in 100 normal subjects. Both RA/LA subendocardial borders were traced to obtain atrial volumes, strain (ε) and strain rate (SR). Reservoir, conduit, and booster pump functions were evaluated. Consequently, εNeg (corresponding to pump function) and εPos (corresponding to conduit function) were gauged. The SR parameters (SRLateNeg, SRPos, and SREarlyNeg), corresponding respectively to atrial systole, inception of ventricular systole, and inception of ventricular diastole, were measured. RESULTS: Mean age was 39 ± 15 years with 50 men (50%). Volumetric indices revealed that reservoir (Filling Volume = 35.1 ± 10.4 mL for LA vs. 27.47 ± 11.93 mL for RA, expansion index = 52.18 ± 16.89% for LA vs. 45.03 ± 16.49% for RA and diastolic emptying index = 52.85 ± 16.85 for LA vs. 45.62 ± 16.5 for RA, P < 0.001) and conduit (passive emptying (%) of total emptying = 34.49 ± 10.4 for LA vs. 26.82 ± 11.98 for RA and passive emptying index = 52.63 ± 16.86 for LA vs. 45.39 ± 16.5 for RA, P < 0.001) functions were significantly higher in the LA compared to the RA. Nevertheless, deformation indices demonstrated an opposite pattern (SRpos = 1.88 ± 0.74 for RA vs. 1.56 ± 0.54 for LA, P = 0.03 and εPos = 59.56 ± 30.63 for RA vs. 45.94 ± 16.67 for LA, P < 0.001). Reservoir, conduit, and booster pump functions showed no statistical significance among both genders. CONCLUSIONS: Evaluation of global and regional RA/LA function by speckle tracking echocardiography is feasible. The current report provides insights regarding dissimilarities between both atria in healthy individuals. The significance of these findings and their potential application will warrant further work.

Patent ductus arteriosus with Eisenmenger syndrome.

Herein we report a 21 year-old woman with a previously documented patent ductus arteriosus and Eisenmenger physiology. She presented with increasing cyanosis and exercise intolerance which could be explained by a new finding of right to left shunting through an interatrial communication. She was started on Bosentan therapy aiming to reduce the pulmonary pressure with consideration for heart-lung transplantation should any further deterioration occur.

Unforeseen cardiac involvement in McArdle's disease.

McArdle's disease (glycogen storage disease type V) is a rare autosomal recessive metabolic myopathy due to myophosphorylase deficiency. It classically manifests by exercise intolerance, leg cramps, muscle pain and occasionally exercise induced myoglobinuria. The onset of exercise intolerance is typically in the second or third decades of life. It has a specific predilection to skeletal muscle involvement, yet cardiac muscle involvement is very rare. This report describes an unusual case of a 33 year-old man with known McArdle's disease who presented with an incidental finding of severe obstructive hypertrophic cardiomyopathy.

Reticent uneventful rupture of right coronary sinus of valsalva aneurysm into right ventricle.

Aneurysms of sinus of Valsalva are rare congenital or acquired cardiac anomalies. They most commonly involve the right or non-coronary sinuses. Herein we report a very rare case of a ruptured congenital right sinus of Valsalva aneurysm into the right ventricular outflow tract followed conservatively for at least 10 years without any events.