Congenital Mitral Valve Malformations Research Articles

Congenital mitral valve defects in pediatric population: a narrative review of surgical repair

Background and Objective: Congenital mitral valve malformations (CMVM) are rare and heterogeneous. The functional classification includes mitral regurgitation, stenosis, and combined lesions. Surgical strategies of CMVM remain challenging in the pediatric population due to various malformations, growth potential, the requirement of long-term anticoagulation, and the necessity of avoiding or delaying valve replacement. Surgical techniques for CMVM must be individually tailored for patients to achieve a proper valve function rather than “normal” anatomy. Compared with mitral repair, mitral valve replacement (MVR) is a less common procedure in children due to high mortality and morbidity rates. Options for cardiac valve replacement in children are limited to prostheses sizes that do not accommodate somatic growth, especially for neonates and infants. The need for lifelong anticoagulation may lead to adverse long-term pregnancy outcomes for female patients, limiting the application of MVR. This review aims to provide a comprehensive analysis of the current surgical techniques and developments for treating mitral valve (MV) regurgitation and stenosis and the outcome of various surgical strategies.

Mitral valve repair for mitral regurgitation in pediatrics and children: Initial experience

IntroductionMitral valve repair in pediatrics has been slow because of the great variety in congenital mitral valve malformations and the still unknown growing effect over the mitral valve. The aim of this study is to evaluate the early and mid-term results in surgical repair of mitral valve regurgitation in pediatrics and children.Patients and methodsA total of 16 patients had mitral valve repair for mitral regurgitation (MR) between November 2016 and December 2020 at the National Heart Institute. There were seven (43.75%) males and nine (56.25%) females. The mean age was 7.5 years (5 months and 14 years). The mean weight was 19.9 kg (5.5–40 kg). Concomitant procedures were done in 11 (68.75%) patients. The mean cardiopulmonary bypass time was 82 min (40–150 min), and the mean aortic cross-clamp time was 59 min (25–110 min).ResultsThere was only one (6.25%) early mortality after 3 months. The number of patients who had posterior patch annuloplasty was six (37.5%), rigid rings was four (25%), whereas suture annuloplasty was five (31.25%). The mean postoperative mitral valve gradient was 5 mmHg (3.5–8 mmHg). More than 50% of patients with suture annuloplasty had moderate MR, whereas posterior patch annuloplasty had trivial to mild MR and rigid ring annuloplasty had trivial MR. Aortic valve surgery was done in two (12.5%) cases. The mean hospital stay was 7.2 days (4–14 days) and ICU mean stay was 2.9 days (1–7 days), with a mean mechanical ventilation time of 6 h (2–15 h). Reoperation was done in four (26.6%) patients, with 81.25% freedom from reoperation for mitral valve prosthetic replacement.ConclusionMitral valve repair for mitral valve regurgitation in pediatrics and children improves symptoms, valve function, and myocardial performance and delays replacement. Posterior patch and ring annuloplasty have satisfactory early and mid-term results, low mortality, and a high freedom from reoperation.

Outcomes of Supra-annular Mechanical Atrioventricular Valve Replacement with Polytetrafluoroethylene Graft in Infants and Children.

Despite improvements in valve repair techniques, conditions in which infants and children need for mechanical valve replacement (MVR) are still present. We analyzed supra-annular MVR outcomes in infants and children with small annulus and compared them with conventional annular MVR outcomes. Data were collected retrospectively from medical records of infants and children (weighing < 20kg) who underwent atrioventricular valve replacement with mechanical valve in Seoul National University Children's Hospital between December 1984 and January 2019. We identified 8 patients (median age 20months, median weight 10.2kg) who underwent supra-annular MVR with polytetrafluoroethylene graft (supra-annular group). The patients were diagnosed with congenital mitral valve malformation (5 patients), complete atrioventricular septal defect (2 patients), and functional single ventricle (1 patient). The implanted mechanical valve size ranged from 16 to 23mm. Thirty-three patients (median age 40months, median weight 13kg) underwent conventional annular MVR (annular group). The survival rate was not significantly different between the supra-annular and annular groups (75.0 vs 78.8%, P = 0.816). In patients with biventricular repair (7 patients with supra-annular MVR and 28 patients with annular MVR), mechanical valve-to-mitral valve annulus size ratio was higher in the supra-annular group than in the annular group (1.24 ± 0.30 vs 0.96 ± 0.22, P = 0.035). No coronary complication or heart block were observed in the supra-annular group. Supra-annular MVR with polytetrafluoroethylene graft may be a feasible surgical option in children with a small annulus when valve repair is unsuccessful.

Four-Tiered Echocardiographic Analysis Approach for Congenital Mitral Valve Malformations

Four-Tiered Echocardiographic Analysis Approach for Congenital Mitral Valve Malformations

Four-tiered echocardiographic analysis approach for congenital mitral valve malformations: Four years of experience

BackgroundTraditional 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. MethodsData 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. ResultsFrom 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). ConclusionsDetailed 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.

Rare congenital mitral valve malformations assessed by real-time three-dimensional echocardiography

Rare congenital mitral valve malformations assessed by real-time three-dimensional echocardiography

Early and Mid-Term Outcome of Pediatric Congenital Mitral Valve Surgery.

Background:Congenital lesions of the mitral valve are relatively rare and are associated with a wide spectrum of cardiac malformations. The surgical management of congenital mitral valve malformations has been a great challenge.Objectives:The aim of this study was to evaluate the early and intermediate-term outcome of congenital mitral valve (MV) surgery in children and to identify the predictors for poor postoperative outcomes and death.Patients and Methods:In this retrospective study, 100 consecutive patients with congenital MV disease undergoing mitral valve surgery were reviewed in 60-month follow-up (mean, 42.4 ± 16.4 months) during 2008 - 2013. Twenty-six patients (26%) were under one-year old. The mean age and weight of the patients were 41.63 ± 38.18 months and 11.92 ± 6.12 kg, respectively. The predominant lesion of the mitral valve was MV stenosis (MS group) seen in 21% and MR (MR group) seen in 79% of the patients. All patients underwent preoperative two-dimensional echocardiography and then every six months after surgeryResults:Significant improvement in degree of MR was noted in all patients with MR during postoperative and follow-up period in both patients with or without atrioventricular septal defect (AVSD) (P = 0.045 in patients with AVSD and P = 0.008 in patients without AVSD). Decreasing trend of mean gradient (MG) in MS group was statistically significant (P = 0.005). In patients with MR, the mean pulmonary artery pressure (PAP) had improved postoperatively (P < 0.001). Although PAP in patients with MV stenosis was reduced, this reduction was not statistically significant (P = 0.17). In-hospital mortality was 7%. Multivariate analysis demonstrated that age (P < 0.001), weight (P < 0.001), and pulmonary stenosis (P = 0.03) are strong predictors for mortality. Based on the echocardiography report at the day of discharge from hospital, surgical results were optimal (up to moderate degree for MR group and up to mild degree for MS group) in 85.7% of patients with MS and in 76.6% of patients with MR. Age (P = 0.002) and weight (P = 0.003) of patients are strong predictors for surgical success in multivariate analysis.Conclusions:Surgical repair of the congenital MV disease yields acceptable early and intermediate-term satisfactory valve function and good survival at intermediate-term follow-up. Strong predictors for poor surgical outcome and death were age smaller than 1 year, weight smaller or equal than 6 kg, and associated cardiac anomalies such as pulmonary stenosis.

Effectiveness of mitral valve repair in congenital mitral valve malformation in infancy

Objective: In infants every effort should be made to preserve the native mitral valve (MV). The aim of the study was to analyze effectiveness of MV repair regarding to preservation of native valve in infancy.

Echocardiographic diagnosis of congenital mitral valve malformation

Objective To investigate the clinical value of the echocardiography in the diagnosis of congenital mitral valve malformation and summarize its occurrence and type associated with other cardiac malformations.Methods The echocardiographic image data of 101 patients with congenital mitral valve malformation were retrospectively analyzed,the characteristics of different types were summarized and compared with operation results,CT,cardiac catheterization,transesophageal echocardiography,as well as the data of echocardiographic follow-up.Results Among 101 patients with echocardiographic examination showed mitral valve congenital malformation,63 cases were isolated valve set malformation (62.4％),and multiple valve set malformation were found in 38 cases (32.7％).In another side,39 cases were of single mitral valve malformation (38.6％),while 62 patients combined with other type cardiac malformations (61.4％).No significant difference of dynamic change was shown between patients with solitary and multiple malformation of mitral valve (P ＞0.05).Conclusions Echocardiography can display congenital mitral valve malformation in a fast,robust,real-time way.It has an important clinical value in the diagnosis of congenital mitral valve malformation. Key words: Echocardiography; Heart defects, congenital ; Mitral valve malformation

A Trileaflet “Mitral Valve” with Three Papillary Muscles: Brand New Echocardiographic Finding

Congenital mitral valve malformations are rare, but are well known and described entities. Mitral valve malformations involve mitral valve apparatuses (leaflets and annulus) and subvalvar apparatuses (chordae and papillary muscle). Case reports of accessory mitral leaflets were already described, but were usually an appendix of the normal valve. We describe here a case report and present the images of a trileaflet mitral valve sustained by three papillary muscles in a young girl with subaortic stenosis.

Surgical repair of congenital mitral valve malformations in infancy and childhood: A single-center 36-year experience

We sought to evaluate the results of surgical repair and determine predictors for the late outcome of congenital mitral valve dysplasia. Preoperative, operative and postoperative data were obtained from an institutional database; follow-up data came from regular clinical evaluation at our institution or elsewhere. Patients were divided into isolated and complex cases according to the complexity of associated lesions. Between 1972 and 2008, 93 patients (43 male and 50 female patients) underwent mitral repair (median, 4.5 years; range, 0.16-19.8 years). Predominant mitral regurgitation was present in 52%. Associated cardiac anomalies were present in 72%. Sixty-one patients were in the complex group. All patients underwent successful mitral repair. Surgical repair was tailored to the patient's valve anatomy. Early death was 7.5%. The postoperative course was uneventful in 86% of patients. At a mean follow-up of 10.3 years (median, 8.4 years; completeness, 94%), late mortality is 8% (7 patients). Twelve patients underwent mitral reintervention (11 replacements and 1 repair). Among the 80 survivors, 82.5% were in New York Heart Association class I or II, and 61.2% had some degree of persistent mitral regurgitation or stenosis, despite stable hemodynamics. Stenosis is a statistically significant risk factor for surgical intervention at less than 1 year of age and is related to higher overall mortality and incidence of late cardiac failure and mitral dysfunction; parachute mitral valve is related to higher mortality and morbidity. Mitral valve repair shows acceptable early mortality and reoperation rates. Mitral malformations in the complex group are related to a significantly higher risk of reoperation on the mitral valve. Parachute mitral valve is associated with a higher rate of early mortality.

Mitral Valve Repair in Children Using Carpentier's Techniques

Controversy remains regarding whether valve repair is preferable to valve replacement in children suffering from rheumatic mitral valve disease. To answer this question, 130 children aged 3 to 15 years (mean age, 11.8 +/- 2.8 years) undergoing surgery between January 1992 and December 1997 using Carpentier's techniques were reviewed. There were 111 cases of rheumatic valve diseases (85%), 17 cases of congenital mitral valve malformations (13%), one case of Barlow's syndrome (1%), and one case of bacterial endocarditis (1%). Valve dysfunction was classified into three types according to Carpentier's classification. There were 14 type II (leaflet prolapse), 78 type III (restricted leaflet motion), and 38 associated type II (anterior leaflet) and III (posterior leaflet) cases. There was one early (in-hospital) death (0.7%); the remaining 129 children were examined every 6 months. At the last examination, 99.2% of the patients were still alive, 96.8% were free of reoperation, and 89% showed no sign of significant residual stenosis or insufficiency on echocardiography. There have been no thromboembolisms. We conclude that mitral valve repair using Carpentier's techniques is the preferred procedure in the surgical treatment of mitral valve incompetence in children, even in those with rheumatic valvular disease. Copyright 1999 by W.B. Saunders Company

Mitral valve repair for mitral regurgitation in paediatric patients at the Ho Chi Minh City heart institute: Five-year retrospective study

Controversy remains regarding whether valve repair is preferable to valve replacement in children suffering from rheumatic mitral valve disease. To answer this question, 130 children aged 3 to 15 years (mean age, 11.8 ± 2.8 years) undergoing surgery between January 1992 and December 1997 using Carpentier's techniques were reviewed. There were 111 cases of rheumatic valve diseases (85%), 17 cases of congenital mitral valve malformations (13%), one case of Barlow's syndrome 11%), and one case of bacterial endocarditis (1%). Valve dysfunction was classified into three types according to Carpentier's classification. There were 14 type II (leaflet prolapse), 78 type III (restricted leaflet motion), and 38 associated type II (anterior leaflet) and III (posterior leaflet) cases. There was one early (in-hospital) death (0.7%); the remaining 129 children were examined every 6 months. At the last examination, 99.2% of the patients were still alive, 96.8% were free of reoperation, and 89% showed no sign of significant residual stenosis or insufficiency on echocardiography. There have been no thromboembolisms. We conclude that mitral valve repair using Carpentier's techniques is the preferred procedure in the surgical treatment of mitral valve incompetence in children, even in those with rheumatic valvular disease.

Congenital malformations of the mitral valve in children: Pathology and surgical treatment

A systematic study of congenital mitral valve malformations was undertaken on a surgical basis in an attempt to develop techniques of valvular reconstruction adapted to the various lesions. Forty-seven children between the ages of 4 months and 12 years (average 6 years, 4 months) have been operated upon between January, 1970, and March, 1976. Valvular lesions were classified into four group: Group I, mitral insufficiency owing to valvular lesions: Group II mitral insufficiency with subvalvular lesions; Group III, mitral insufficiency and stenosis; Group IV, stenosis. Associated lesions (ventricular or atrial septal defects, coarctation, or aortic valve stenosis) were present in 31 patients (65 per cent) and were corrected during the same operation. Valve reconsruction was possible in 38 patients whereas valve replacement was necessary in 9 patients. In the valve repair group there were three operative deaths (8 per cent), no late deaths, one reoperation for residual ventricular septal defect, and one myocardial infarction. In the valve replacement group of 9 patients, there were three operative deaths, three late deaths, and one case of repeated embolization. Thirty-one of 38 patients in the valve repair group were classified into Functional Class I after the operation (86 per cent), 2 were in Class II, and one in Class III. Minimal or moderate regurgitation and cardiomegaly persisted in the majority of the patients. Pulmonary artery pressure significantly decreased, however, as demonstrated by postoperative catheterization in 17 patients.

The pathological anatomy of surgically reconstructable or prosthetically correctable congenital valvular malformation of the mitral region.

The special pathology of reconstructable or only prosthetically correctable congenital malformations of the mitral valve is described on the basis of the following examples taken from our own operative and autopsy material of the last 5 years: 1. Congenital isolated mitral stenosis in female twins (7 month old infant and 33 month old child). 2. Congenital isolated mitral insufficiency in a 7 1/2 year old boy. 3. Combined mixed mitral valve malformations with a parachute valve-like mitral valve anomaly, combined with hypoplasia of the ascending and descending aortas, in a 6 1/2 year old girl. 4. Congenital mitral insufficiency with a parachute mitral valve, combined with supravalvular aortic stenosis and multiple peripheral stenoses of the pulmonary arteries in a 13 1/4 year old boy. 5. Insufficiency of the mitrally inverted tricuspid valve with so-called corrected transposition of the great vessels in a 6 year old boy and with Ebstein's anomaly in a 2 1/2 year old boy. 6. A second mitral ostium in the aortic mitral leaflet with a partial atrioventricular canal in a 6 3/4 year old girl with Ellis-van Creveld syndrome. 7. Bland-White-Garland syndrome with relative mitral insufficiency in a 5 month old and a 4 month old boy. Despite the recurrence of similar and comparable findings, each of our cases of congenital or early acquired noninfectious mitral valve malformation was formally different. n his was also true for the cases of congenital isolated mitral stenosis in twins. Therefore, surgical correction requires a unique procedure for each case. It is possible to reliably infer the degree of malfunction of the atrioventricular valve in a mitral position from the special pathology only by considering the clinical data. On the other hand, a detailed evaluation of congenital mitral valve malformations is possible only through direct inspection--either by the surgeon or through an autopsy--despite modern cardiodiagnostic methods. Typical secondary findings are also discussed--for instance, endocardial fibrosis of the left atrium and the configuration of the heart. The anatomical prerequisites for surgical reconstruction or replacement of the valve with a prosthesis are mentioned.