PubMed · 15028087
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Hanno L Tan, Fred H M Wittkampf, Hiroshi Nakagawa, Richard Derksen. 2004. Atriofascicular accessory pathway.. https://doi.org/10.1046/j.1540-8167.2004.03181.x
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Complete atrioventricular (AV) canal defects usually have right and left valve components of approximately equal size, although unbalanced forms do occur. Optimal management of unbalanced AV canal defects is controversial, with no proven guidelines for choosing between biventricular repair and univentricular palliation. We describe successful biventricular repair of a right-dominant unbalanced AV canal with severe left AV valve stenosis. This case illustrates that severely unbalanced AV canal defects can be repaired successfully by tailoring the AV valve to distribute it equally between the ventricles, The increase in left ventricular volume after surgery exceeded prediction based on conformational change alone.
We observed a case of a 47-year-old male patient with hypertrophic cardiomyopathy and family history of sudden death. During cardiac catheterization, the patient presented spontaneous intermittent atrioventricular junctional rhythm without significant changes related to sinus heart rate. The gradient was absent with sinus beats, but the junctional rhythm induced the appearance of a left ventricular-aorta gradient, with parallel reduction of aortic pressure from 156 to 120 mm Hg. Subsequently, a couple of pre-mature ventricular beats induced an important post-extrasystolic potentiation of the gradient, followed in the last beats by sinus rhythm with disappearance of the gradient. Our case suggests that the decrease of left ventricular volume, due to the absence of an effective atrial systole, leads to left ventricular increased gradient, not imputable to an increased inotropic effect or heart rate modifications but to the decrease of pre-load.
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