Midterm Outcomes After Surgical Management for Mitral Valve Regurgitation in Infancy.

BACKGROUND: Mitral valve repair is preferred for pediatric mitral valve disease. However, it is technically difficult because of complex lesions, poor surgical exposure, and tissue fragility, especially in infants. We investigated the midterm outcomes of mitral valve surgery for mitral regurgitation in infancy. METHODS: We retrospectively reviewed 18 patients (aged <12 months old) undergoing mitral valve surgery for mitral regurgitation at our institution between October 2005 and March 2019. The patients had 10 acquired and 8 congenital valve lesions as follows: torn chordae (n = 6), leaflet prolapse (n = 4), posterior leaflet hypoplasia (n = 3), anterior leaflet cleft (n = 2), infective endocarditis (n = 1), papillary muscle rupture (n = 1), and hammock valve (n = 1). RESULTS: All patients initially underwent mitral valve repair. There was no operative mortality, and 1 case of late death. The median follow-up period was 7 years and 9 months. Reoperation was performed in 3 patients, re-repair (twice) in 1 patient with a hammock valve, and mitral valve replacement in 2 patients. Fifteen patients had at most mild mitral regurgitation at the last follow-up. A transmitral mean pressure gradient of over 5 mm Hg was observed in 3 cases, including the patient with a hammock valve. Postoperative mitral annular diameter increased within the normal range in all patients. Survival and reoperation-free rates at 5 and 10 years were 94.4% and 83.0%, respectively. CONCLUSIONS: Mitral valve repair for mitral regurgitation in infancy is safe and feasible with satisfactory midterm outcomes, even under serious preoperative conditions.

A teenager with CHD and coronavirus disease 2019.

A 16-year-old girl with history of treated congenital mitral valve disease and signs of respiratory infection was admitted to our paediatric cardiology department. She was tested positive for severe acute respiratory syndrome coronavirus 2. Despite her severe pre-existing cardiac conditions with pulmonary hypertension, atrial arrhythmias and mitral valve stenosis, the infection did not lead to any cardiac or pulmonary deterioration. In adults, cardiac co-morbidities are known risk factors for a severe course of coronavirus disease 2019 infections. This case illustrates that in children even severe cardiac disease is not necessarily associated with a severe course of coronavirus disease 2019.

Interannular Bridge: A Novel Approach to Address Congenital Mitral Regurgitation.

A male infant with Kabuki syndrome had trivial congenital mitral regurgitation diagnosed at birth. At the age of 2 years and 9 months, the regurgitation worsened from mild to severe; thus, expedited surgical treatment was pursued. The primary operative finding was severe dysplastic two-leaflet disease. After completing chordal replacement as a conventional repair procedure, more-than-moderate central regurgitation caused by establishing a shallow coaptation between the anterior and posterior leaflets persisted. We report a successful case of mitral valve repair involving the novel option of interannular bridge for valvuloplasty to address congenital mitral regurgitation.

Echocardiographic examination of mitral valve abnormalities in the paediatric population: current practices.

We reviewed the recent literature for echocardiographic assessment of mitral valve abnormalities in children. A literature search was performed within the National Library of Medicine using the keywords "mitral regurgitation and/or stenosis, children." The search was refined by adding the keywords "echocardiographic definition, classification, and evaluation." Thirty-one studies were finally included. Significant advances in echocardiographic imaging of mitral valve defects, mainly due to the implementation of three-dimensional technology, contribute to a better understanding of the underlying anatomy. However, heterogeneity between classification systems of mitral valve disease severity is a serious problem. For regurgitant lesions, there is only very limited evidence from small studies that support the adoption of quantitative/semi-quantitative indexes commonly employed in adults. Despite the lack of evidence base, qualitative evaluation of regurgitation severity is often employed. For stenotic lesions, no clear categorisation based on trans-valvular echocardiography-derived "gradients" has been consistently applied to define mild, moderate, or severe obstruction across different paediatric age ranges. Quantitative parameters such as valve area have also been poorly validated in children. Adult recommendations are frequently applied without validation for the paediatric age. In conclusion, significant advances in the anatomical evaluation of mitral valve diseases have been made, thanks to three-dimensional echocardiography; however, limitations remain in the quantitative/semi-quantitative estimation of disease severity, both with respect to valvular regurgitation and stenosis. Because adult echocardiographic recommendations should not be simply translated to the paediatric age, more specific paediatric guidelines and standards for the assessment of mitral valve diseases are needed.

Hammock Mitral Valve Repair in Infancy: Operative Steps Toward a Customized Reconstruction After Preoperative Planning.

Hammock mitral valve (MV) repair is historically technically difficult with a guarded prognosis. Surgical experience is extremely limited and variable outcomes are reported. The perioperative strategy and technical details of hammock MV repair in an infant who presented with severe mitral stenosis are described and review of the existing literature was undertaken.

Supra-Annular Mitral Valve Replacement in Infants with Congenital Mitral Valve Disease.

Mitral valve replacement in infants is rare and causes a relatively high mortality, especially for patients under the age of 1. Supra-annular valve replacement is a viable technique for infants with a small valve annulus. Here, we report two infants who underwent mitral valve replacement via the supra-annular technique. The age and body weight of these babies were 2 months and 3 months and 4.1 kg and 4.7 kg, respectively. Aortic mechanical valves were reversely implanted with a short segment of PTFE graft. The purpose of this strategy was to insert a larger mechanical valve and delay resternotomy. A two-year follow-up exam showed normal ventricular function without mechanical valve-related complications. This method is useful in treating neonates and infants. Although the technique of mitral valve repair has improved over several decades, mitral valve replacement still is necessary at times. In neonates and infants with a small annulus, implantation of commercially available prosthetic valves in the annular position can be a challenge, and an age less than 1 year is a risk factor for early death [Selamet 2008]. Supra-annular mitral valve replacement (SMVR) is an alternative when a traditional annular implantation is not feasible [Sung 2008]. Herein, we report the cases of two patients, who underwent SMVR with a follow-up after two years.

Are probiotics safe? Bifidobacterium bacteremia in a child with severe heart failure.

Although few cases of bacteremia or sepsis caused by probiotics have been reported, it is important to consider their pathogenic potential, especially in some categories of patients. We report a case of Bifidobacterium spp bacteremia in a child with heart disease, undergoing probiotic supplementation to prevent antibiotic-associated diarrhea.

Mitral Valve Replacement in Infants Using a 15-mm Mechanical Valve.

BACKGROUND: The 15-mm mechanical valve was approved by the US Food and Drug Administration in March 2018. We review our experience in infants with this valve in the mitral position (MV), focusing on outcomes and timing to repeat MV replacement (MVR). METHODS: Between 2006 and 2017 7 patients underwent eight MVRs (one repeat) with a 15-mm mechanical valve. Retrospective chart review was performed to examine short- and long-term outcomes. RESULTS: There were no operative deaths. Mean follow-up was 5.8 ± 4.8 years (range, 0.72 to 11.1). Six patients underwent an MV operation 53 ± 39 days (range, 9 to 118) before MVR with the 15-mm valve. All patients were on mechanical ventilatory support at the time of operation. Mean age, body weight, and body surface area at time of 15-mm MVR were 0.5 ± 0.3 years (range, 0.2 to 0.9), 5.6 ± 0.8 kg (range, 4.8 to 6.6), and 0.29 ± 0.03 m2 (range, 0.27 to 0.32), respectively. Two patients required pacemaker implantation for atrioventricular block, both after their second MVR. Two patients are well at 16 and 24 months. Four patients underwent repeat MVR because of somatic growth and patient-prosthesis mismatch. Mean time to repeat MVR was 23 months (range, 6 to 40). There were two late deaths, one at 10 months unrelated to the valve in a child with a chromosomal abnormality. The other child had a congenital diaphragmatic hernia, early valve thrombosis, and died of multiple complications after a fourth MVR. CONCLUSIONS: The 15-mm mechanical valve was useful in treating MV disease in infants 2 to 12 months of age. This newly approved smallest available mechanical valve has a predicted mean time to replacement of 23 months in the mitral position.

Robotic Repair of a Congenital Isolated Cleft of Anterior Tricuspid Valve Leaflet.

Congenital isolated tricuspid valve (TV) cleft in the anterior leaflet is a rare occurrence, while clefts of the mitral valve leaflets are more common and are usually associated with other congenital heart diseases. In this article, we report a case of TV regurgitation in a young adult female due to an isolated congenital cleft in the anterior TV leaflet, which was surgically repaired using a minimally invasive robotic approach.

Mid-term result of atrioventricular valve replacement in patients with a single ventricle.

OBJECTIVES: Atrioventricular valve replacement is the last option to treat the atrioventricular valve regurgitation in single ventricle. This study investigates the mid-term outcomes of the atrioventricular valve replacement based on the Japan Cardiovascular Surgery Database registry. METHODS: From 2008 to 2014, 56 patients [34 males (61%) and 22 females (39%)] with a single ventricular circulation, underwent atrioventricular valve replacement. Questionnaires were collected to review operative data, mid-term mortality, morbidity and redo replacement. Risk factor analysis was performed by the Cox regression model for death and redo replacement. RESULTS: Heterotaxy, a right systemic ventricle and a common atrioventricular valve was present in 46% (26/56), 64% and 57% of patients, respectively. The most common timings for atrioventricular valve replacement were the interstage between the second and third palliations (34%) and after the Fontan operation (34%). Twenty died during the 3.7 ± 2.6-year follow-up. Eleven received redo atrioventricular replacement. The cumulative incidences of redo atrioventricular valve replacement and survival at 3 years were 20% [95% confidence interval (CI) 9-30] and 66% (95% CI 55-80), respectively. Univariable Cox regression analysis revealed that a tricuspid valve was a risk factor for redo valve replacement [hazard ratio (HR) 6.76, 95% CI 1.79-25.6; P = 0.005] and that young age was a risk factor for death (HR 0.77, 95% CI 0.62-0.96; P = 0.019). Fourteen patients required a pacemaker implantation. CONCLUSIONS: Valve replacement for uncontrollable atrioventricular valve regurgitation in single ventricular circulation was associated with a moderately high risk of death, redo replacement and pacemaker implantation, whereas valve replacement at a later period and with a larger prosthetic valve size was associated with low mortality.

Cardiac Calcified Amorphous Tumor in a Newborn.

Calcified amorphous tumors (CATs) of the heart are rare, nonneoplastic, intracavitary lesions, previously thought of as pseudotumors, hamartomas, or calcified thrombi, only reported in few adults in the available literature. This report describes a case of a pedunculated oscillating CAT arising from the left atrial appendage that prolapses through the mitral valve and causes severe mitral regurgitation in a newborn. This is the only case of cardiac CAT described in a neonate.

Congenital mitral regurgitation with Down syndrome.

A 19-year-old man with Down syndrome had congenital mitral regurgitation. He had slight mental retardation and difficulty in performing independent oral drug administration. We planned mitral valve plasty to avoid postoperative anticoagulant therapy. The mitral valve lesions were complicated. A prolapsed anterior mitral leaflet, shortened posterior leaflet, abnormal cleft, abnormal papillary muscle, and enlarged annulus were observed. Successful mitral valve plasty was performed using several techniques. The postoperative course was uneventful, and no significant mitral regurgitation was found.

The Challenge of Hammock Mitral Valve During Infancy: Precise Preoperative Advanced Imaging and Three-Dimensional Modeling Augments Customized Operative Valve Reconstruction.

Hammock mitral valve (MV), also known as anomalous mitral arcade, is a rare congenital anomaly. We report a case of a 10-month-old child who presented with congestive heart failure and was found to have severe mitral stenosis (MS) secondary to a hammock MV anomaly. Detailed advanced imaging with cardiac MRI and three-dimensional (3D) echocardiography favorably navigated a customized valve-sparing surgical reconstruction of the congenitally abnormal MV. Repair of a hammock MV is technically difficult with a guarded prognosis. Surgical experience is extremely limited and variable outcomes are reported. A rare case of severe MS secondary to hammock MV deformity with successful surgical repair and review of worldwide literature are presented.

Prótesis de yugular bovina con stent (Melody) en posición mitral. ¿Posible alternativa a la prótesis mecánica en población pediátrica? / Stented bovine jugular vein graft (Melody Valve) in mitral position. could be an alternative for mechanical valve replacement in the pediatric population?

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Challenging mitral valve repair for double-orifice mitral valve with noncompaction of left ventricular myocardium.

Double-orifice mitral valve (DOMV) is a relatively rare cardiac anomaly. Although usually associated with various cardiac anomalies, co-presence of DOMV and noncompaction of left ventricular myocardium (NCLVM) is extremely rare. Here, we present a 24-year-old male who underwent mitral valve repair using artificial chordae and annuloplasty at the posterior commissure for severe mitral regurgitation (MR), resulting from flail anterior leaflet of the larger postero-medial orifice and dilatation of left ventricle with NCLVM. One year later, he underwent second mitral valve repair for recurrence of MR. Further endoscopic evaluation of the left ventricle, and reinforcement via artificial ring, enabled us to achieve repair.

Asymptomatic and isolated accessory mitral valve tissue in an adult.

Accessory mitral valve (AMV) tissue is a congenital anomaly that occurs in association with other congenital anomalies, and is an uncommon cause of left ventricular outflow tract obstruction. It is usually detected in early childhood when accompanied by symptoms of obstruction of the left ventricular outflow tract, and is rarely diagnosed in adults. We present a case of a 53-year-old man who was referred to our institution for evaluation of a systolic heart murmur. Echocardiography disclosed a diagnosis of AMV tissue. This case was uncommon because of the lack of severe obstruction of left ventricular outflow, cardiac symptoms, or other cardiac anomalies. We were able to carry out surgical resection of AMV tissue to avert possible progression of aortic insufficiency and the risk of a cerebrovascular embolization. The patient's postoperative course was uneventful, and postoperative echocardiography showed no residual accessory mitral tissue.

Contemporary long-term outcomes of an aggressive approach to mitral valve repair in children: is it effective and durable for both congenital and acquired mitral valve lesions?

OBJECTIVES: We analysed the long-term outcomes of mitral valve (MV) repair in children and compared the repairs for both congenital and acquired lesions. METHODS: A review of 634 children (≤18 years) who underwent MV repair from 1992 to 2011 was conducted [excluding patients with complete atrioventricular septal defect (AVSD), single ventricle and atrioventricular (AV) discordance]. Associated cardiac anomalies were present in 473 patients (75%). Congenital mitral lesions were found in 270 (43%) patients compared with an acquired aetiology in 364 (57%) [mainly rheumatic: 329 patients (90%)]. Mitral regurgitation (MR) was predominant in 606 (96%) patients, and 544 (86%) of these showed ≥3+ MR. Modified techniques of MV reconstructions were used. RESULTS: The early mortality rate was 2% (14 patients). The mean follow-up was 55 months (1-240 months; 85% complete). The late mortality rate was 4% (23 patients) and survival rates at 10 and 15 years were 91 and 86%, respectively. There was no significant difference in 10-year survival between repairing the congenital (98%) and acquired lesions (87%) (P = 0.17). The rate of freedom from reoperation after MV repair for the entire population was 79% at 10 years, with no significant difference between congenital (80%) and acquired lesions (79%) (P = 0.20). Fifty-six patients (9%) required reoperation. Mixed MV lesions, commissural fusions and residual MR (≥2+) were the predictors of valve failure and reoperation. All survivors remain in New York Heart Association class I and none had thromboembolism or pacemaker insertion. CONCLUSIONS: MV repair can be successfully applied to both congenital and acquired MV disease in children. Aggressive repair techniques and avoidance of residual MR have improved durability and survival.