The Impact of Aortic Valve Replacement on Left Ventricular Remodeling in Children

There are scant data in pediatrics on the optimal timing for aortic valve repair (AVR). This study assesses the midterm response to AVR and possible predictors of poor outcome. From 2001 to 2006, 41 patients had greater than 3-month follow-up after AVR for aortic insufficiency, aortic stenosis, or both. Pre-, peri-, and post-operative data were collected, including demographics and clinical symptoms. Two reviewers measured echocardiographic parameters from the pre-operative and latest follow-up echocardiograms. Ventricular dimensions were indexed to body surface area (z-score). Median age at AVR was 13 years with 83 % having a Ross operation. The average left ventricular end-diastolic dimension pre-op, z-score of +1.3, significantly decreased at last follow-up to a mean z-score of ?0.1 (p < 0.001). Similarly the indexed LV mass decreased from +3.9 to +0.5 (p < 0.001). There was no significant correlation between the presence of pre-op symptoms and the presence of post-op LV dilatation, hypertrophy, or dysfunction. In the subset of patients (7/41) with persistent LV dysfunction at last follow-up, there was a significant correlation with pre-op LV dilatation as assessed by both LVEDD (p = 0.02) and LVESD (p = 0.05). Children demonstrate significant reverse remodeling after AVR. Pre-op LV dilatation may predict patients with persistent LV dysfunction post-AVR. Symptoms are less useful in children, suggesting the need for more objective data for functional assessment.     

Congenital Malformations of the Tricuspid Valve in Siblings

The occurrence of familial heart disease in association with tricuspid atresia is rare. The first reported instance of tricuspid atresia and Ebstein's anomaly in siblings is presented. The presence of these two distinct pathologic variants of tricuspid valve malformations in siblings suggests that these malformations result from a common abnormality occurring during the development of the inlet portion of the right ventricle.     

Mitral Valve Replacement with the Pulmonary Autograft in Children: A Word of Caution

A 4½-month-old patient who underwent mitral valve replacement for congenital mitral stenosis using a pulmonary autograft is reported. Failure of the autograft resulted in pulmonary hypertension, leading to pulmonary regurgitation in the reconstructed right ventricular outflow tract, then tricuspid regurgitation, refractory right heart failure, and death. Caution should be exercised in applying this procedure with children, particularly those at risk for pulmonary hypertension.     

前尿道瓣膜并憩室6例

目的探讨前尿道瓣膜并憩室早期发现和治疗的最佳方法。方法对6例前尿道瓣膜并憩室患儿采用排泄性膀胱尿道造影和静脉尿路造影进行诊断。确诊患儿行前尿道憩室切除术及尿漏修补术。肾功能不全或电解质紊乱者需先置尿管引流,改善全身情况。结果6例患儿均经手术治愈,排尿通畅,但均留有不同程度的后遗症状。结论前尿道憩室临床较少见,与后尿道瓣膜一样后遗症状严重,早期诊断与治疗疗效较好。     

Predictors of Left Ventricular Performance After Valve Replacement in Children and Adolescents with Chronic Aortic Regurgitation

Aortic valve replacement has been recommended in patients who have severe symptoms, in patients with extreme left ventricle (LV) dilatation (end diastolic dimension >4 SD above normal) or LV ejection fraction <50%. However, the occurrence of advanced symptoms or severe LV dilatation raises concern about irreversible LV dysfunction. This study sought to determine the influence of preoperative symptoms, LV size and function on mortality, and postoperative LV performance in children and adolescence after valve replacement for aortic regurgitation (AR). A total of 49 patients 18 years old or younger (mean, 13.9 ± 3) who underwent valve replacement for chronic AR between 1991 and 2001 were followed up for 1–10 years (mean, 3.3 ± 2.1). Baseline and postoperative characteristics were compared between 13 patients (group 1) with extreme LV dilatation and 34 patients (group 2) with a lesser degree of LV enlargement. Preoperative low ejection fraction (p < 0.008), extreme LV dilatation (p < 0.05), and LV end systolic dimension >4 SD above normal (p < 0.05) were predictors of persistent LV dysfunction. Extreme LV dilatation (p < 0.0003), LV end systolic dimension (p < 0.0007), and reduced LV ejection fraction (p < 0.01) predicted persistent LV dilatation. In the setting of chronic AR, preoperative symptoms, LV systolic function, and LV internal dimensions are the main predictors for persistent LV dysfunction and dilatation. Surgical correction should be performed before LV systolic dysfunction and/or extreme LV enlargement occurs.     

Successful Management of Severe Tricuspid Regurgitation Associated with Hydrops Fetalis in a Case of Dysplastic Tricuspid Valve

We report a case with hydrops fetalis and severe tricuspid regurgitation due to dysplastic tricuspid valve, diagnosed in utero and followed after birth. The patient was successfully managed on the basis of useful echocardiographic informations.     

Morphology of Tricuspid Valve in Pulmonary Atresia with Intact Ventricular Septum

Pulmonary atresia with intact ventricular septum (PAIVS) is a rare congenital cardiac anomaly that has been classified into two types: one is a more frequent type having dysplasia of tricuspid valve (TV) with a small annulus, underdeveloped right ventricle (RV) with a hypoplastic cavity and a hypertrophic wall; the other type has severe dysplasia of TV and dilatation of RV, right atrium (RA), and right atrioventricular junction with thinning of the RV wall. We performed a morphologic study on 11 autopsied hearts with PAIVS, giving particular emphasis to the variation of morphology of the TV. We could classify these hearts into 3 groups according to the degree of right ventricular development. In the first group of 7 cases (type I), the RVs were underdeveloped. Thick leaflets, restricted valve apparatus with short chordae, and small annuli were characteristics of the TV. In the second group of 3 cases (type II), the RVs showed marked enlargement of the cavity and thinning of the wall. The TV showed redundant, dysplastic, sail-like anterior leaflets, and the downward displacement of septal leaflet and/or posterior leaflet, which are the findings frequently observed in Ebstein's malformation. The RVs were dilated and with partially unguarded tricuspid orifice. The septal leaflet of the TV was dysplastic and, in two cases, the septal leaflet showed chordal structure at the upper surface facing the RA, which is a peculiar finding that has not been described in the literature. The remaining case was a heart with a moderately developed RV (type III). The TV showed mildly dysplastic appearance and we classify this as a separate type, because we could expect the best surgical results in this type. This type had optimal size of RV and the mildest degree of dysplasia of TV. In PAIVS, the morphology of TV correlates well with the type of the right ventricular development.