Quadricuspid aortic valve, parossistyc supraventricular tachycardia and double right kidney: an uncommon association

Fourteen years after surgery for replacement of the aortic valve, an interesting case previously unreported was brought to our attention. The female patient came to our OP Dept for a routine follow-up: she had been found at surgery to have a quadricuspid aortic valve. Operation dated October 1985. At a careful appraisal of the world-wide literature, we noticed that such an association of pathologies had never been reported before: quadricuspid aortic valve, paroxystical supraventricular tachycardia and right double kidney with double renal pelvis and double proximal ureter. Other anomalies associated with the quadricuspid valve, available in the literature are: patent duct, subvalvular fixed aortic stenosis, ventricular septal defect, hypoplastic anterior mitral leaflet and pulmonary stenosis. The pathologic findings at autopsy of this congenital malformation vary between 0.008% and 0.033%; attention must be turned to the fact that the incidence can be underestimated if not expressly searched for. The first quadricuspid aortic valve was described in 1862 by Balington in an autoptic report and sixty other cases have been reported since. In rare cases this pathology has been diagnosed at angiography. At follow up our patient remains in SR with rare transitory episodes of supraventricular tachycardia.