Background: Traditional methods of describing and classifying congenital mitral valve malformations (CMVMs) often lack specificity and scientificity. Thus, documentation is incomplete, especially in terms of ultrasound findings., Methods: Data were collected from 436 patients (mean age, 36.6±26.8years; male 47.9%), each subjected to echocardiographic evaluation of CMVM. Valvar characteristics were studied and analyzed via a four-tiered echocardiographic analysis (FTEA) approach: (1) supravalvular region and annulus, (2) valvar leaflets and commissures, (3) chordae tendineae, and (4) papillary muscles. A clinical random ultrasonic reading controlled trial was designed to the compare conventional diagnostic method and FTEA in patients with CMVMs., Results: From a total of 246,507 echocardiograms, CMVMs were methodically investigated in 436 (0.18%) patients. Of these, 16 (3.7%) had multi-level malformations; and in 133 (30.5%), CVCMs were associated with other cardiac defects. Using a FTEA approach, involvement was distributed as follows: (1) supravalvular region and annulus (n=7 [1.6%]; excessive supravalvular tissue, 3; abnormal annulus, 4 [overriding, 1; shifted, 2; bridging/cord-like accessory tissue, 1]); (2) valvar leaflets and commissures (n=421 [96.3%]; lengthy or excessive, 210; underdeveloped, 35; contracture,12; atretic, 3; anomalously connected, 1; loose or billowy, 63; clefts, 57; dual orifice, 5; localized bulging, 6; accessory tissue element, 4; fibrotic, 18; fused leaflet cusps, 3; abnormal commissures, 4 [fused, 1; clefts, 3]); (3) chordae tendineae (n=14 [3.2%]; confined to single papillary muscle, 4; excessive, 2; thickened and fused, 2; shortened, 2; fibrotic, 2; accessory tissue element, 1; straddling, 1); and (4) papillary muscles (n=13 [3.0%]; absent, 2; single, 5; asymmetric, 2; abnormally located, 3; fibrotic, 1). According to the report comparing one by one each section among the inexperienced (groups A and B) and experienced (group C) groups out of 100 patients with CMVMs, group A of the inexperienced group using the conventional diagnostic method had a score of 76±12, while group B of the inexperienced group using the FTEA method had a score of 85±11; group B scored significantly higher than group A (all P<0.05)., Conclusions: Detailed incremental FTEA method in a systematic manner clearly defines malformations of the MV apparatus, ensuring more accurate diagnostic imaging. The results of clinical randomized controlled trials confirm that FTEA diagnostic accuracy was higher than conventional methods in CMVMs., (Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.)