Functional Classification of Mitral Valve Diseases Carpentier's functional classification of mitral valve anomalies82 describes leaflet motion, regardless of the anatomy and the cause It is an essential tool for mitral valve repair and should be studied on echocardiography preoperatively It can also give significant clues to the lesions that will eventually be found during surgery Type I is normal leaflet motion The anomaly can be either a perforation, a defect of one or two leaflets, or an annular dilation Annular dilation associated with a type II anomaly of the anterior leaflet is most likely functional Type II is enhanced leaflet motion Most pediatric mitral valve regurgitations with a predominant type II anomaly started as functional and progressively elongated the suspension apparatus of the anterior leaflet Type III is restricted leaflet motion The valve may be stenotic, regurgitant, or both Most patients with congenital anomalies of the mitral valve belong to this type Medical Management In terms of diagnosis and treatment, our discipline still suffers from the lack of guidelines For patients with congenital stenosis, medical management is frequently dictated by the nature and severity of the associated lesions In patients with an isolated valvar lesion, treatment with diuretics may buy some time Although surgical repair is possible, the long-term results may be disappointing Unfortunately, pulmonary hypertension is frequently encountered in these patients, thus forcing the hand of the cardiologist and surgeon There are little data for balloon valvuloplasty performed in the setting of congenital mitral valvar stenosis, although encouraging results have now been reported from a single center with many years of experience, the results being comparable to those provided by surgical intervention.83 In this series, patients with a supramitral ring, prior mitral valvar regurgitation, and of younger age at the time of intervention all suffered a poorer outcome after dilation For those who did not have these adverse risk factors, there was a good initial reduction in gradient albeit a high risk of recurrent stenosis, with more than 25% developing significant valvar regurgitation A new role for percutaneous dilation of the mitral valve is emerging, following the development of new techniques of mitral valve replacement with bioprostheses (see later) Regurgitation is generally managed medically until there is evidence of clinical symptoms that are not improved by a standard therapeutic regimen combined with a trial of afterload reduction With the introduction of inhibitors of angiotensin converting enzyme, which reduce the afterload on the left ventricle, morbidity and mortality have both been shown to improve in adults with left ventricular dysfunction.84,85 Thus far, however, no studies have demonstrated that such treatment in isolation prolongs the time from diagnosis to eventual repair or replacement in cases where regurgitation is the primary lesion and is not secondary to left ventricular dysfunction Indeed, in one investigation using an animal model of chronic mitral regurgitation, inhibition of angiotensin converting enzyme decreased the end-diastolic pressure in the left ventricle along with the pulmonary capillary wedge pressure, but it did not increase the forward stroke volume or the contractility.86 The addition of β-blockade to the inhibition of the angiotensin converting enzyme had similar effects, although β-blockade also increased forward stroke volume and contractility