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.

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.

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.

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.

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.

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.

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.

Successful robot-assisted repair of congenital mitral valve regurgitation.

Congenital mitral valve regurgitation (CMR) is very uncommon in adults and is usually associated with other congenital malformations. Repair of the mitral valve remains the standard of care. Very limited reports are available on minimally invasive surgical approaches in treating CMR. This report represents the first case series of the successful application of robotics in correcting CMR and associated anomalies, including a partial atrioventricular canal defect.

A standardized repair-oriented strategy for mitral insufficiency in infants and children: midterm functional outcomes and predictors of adverse events.

OBJECTIVE: Surgical management of mitral regurgitation (MR) in children remains a challenge because of the heterogeneity of the anatomy, growth potential, and necessity to avoid valve replacement. Our objective was to assess the functional outcomes and prognostic factors of a standardized strategy of mitral valve (MV) repair for children with MR. METHODS: Consecutive patients aged <18 years who had undergone surgery for severe MR from 2001 to 2012 were studied retrospectively. The standardized repair strategy mainly included leaflet debridement, annuloplasty, and leaflet augmentation. Multivariable risk analyses for recurrent MR (grade>II), transmitral mean echocardiographic gradient>5 mm Hg, MV reoperation, replacement, and mortality were performed. RESULTS: A total of 106 patients were included (median age, 5.1 years; range, 11 days to 18 years). The mean follow-up period was 3.9±3.2 years (range, 2 months to 11 years). The proportion of congenital and left heart obstruction-related (left ventricular outflow tract obstruction) etiology was 49% (n=52) and 11% (n=12), respectively. MV repair was performed primarily in 97% of the patients. The mortality, reoperation, replacement, and MR rate at the last follow-up visit was 4.5% (n=5), 23% (n=24), 5.5% (n=6), and 17% (n=18), respectively. Actuarial survival was 93%±2% at 10 years. Freedom from MV replacement was 95%±2% and 86%±7% at 5 and 15 years, respectively. Native valve preservation was obtained in 85% of the infants and 94% beyond infancy. Independent predictors of recurrent MR, MV reoperation, and replacement included left ventricular outflow tract obstruction etiology (hazard ratio, 45; P=.004), associated preoperative mitral stenosis (hazard ratio, 21; P=.03), and young age (hazard ratio, 1.2; P=.04). CONCLUSIONS: A standardized and reproducible MV repair strategy can achieve satisfactory functional results in infants and children with severe MR, allowing native valve preservation. The left ventricular outflow tract obstruction-related etiology was the main independent predictor of recurrent MR, MV reoperation, and MV replacement.

Agenesis of the mitral posterior leaflet in elderly.

Congenital mitral valve regurgitation is a rare disease that is found in infancy and childhood, and sometimes in elderly people. In the case presented, mitral regurgitation that was tolerated well until the sixth decade of life is reported. A 62-year-old male suffering from dyspnea was referred to our hospital. Transthoracic echocardiographic examination demonstrated severe mitral regurgitation with suspicion of agenesis of the posterior leaflet with a long, mobile anterior leaflet. A transesophageal echocardiogram and surgical evaluation verified agenesis of the posterior mitral valve. The patient was successfully treated using mitral valve replacement, and no complications occurred.

Stented bovine jugular vein graft (Melody valve) for surgical mitral valve replacement in infants and children.

OBJECTIVE: The options for mitral valve replacement in children with irreparable mitral valve disease have been limited to fixed-diameter prostheses that do not accommodate for somatic growth. We have modified an externally stented bovine jugular vein graft (Melody valve) for implantation in this cohort. Because it is not a fixed-diameter prosthesis, we hypothesized that the valve can be expanded in the catheterization laboratory as the child grows. METHODS: The medical records of patients who had undergone Melody valve implantation in the mitral or left atrioventricular valve position from 2010 to 2013 were reviewed. RESULTS: Eleven patients had undergone Melody valve implantation at a median age of 7 months (range, 2-28). The techniques of valve modification and implantation included stent shortening, adding a pericardial sewing cuff, intraoperative balloon expansion, and fixation of the distal stent to the inferior left ventricle wall. The valve was competent, with a low gradient acutely postoperatively in all patients. One patient died, and one required permanent pacemaker implantation. One patient developed valve dysfunction and required explantation. Two patients without a pericardial sewing cuff developed paravalvular leaks. One patient who had not undergone distal stent fixation developed left ventricular outflow tract obstruction. Three patients who had undergone subsequent catheter-based balloon expansion of the valve have continued to demonstrate acceptable valvular function. CONCLUSIONS: The Melody valve has demonstrated acceptable short-term function. Implantation techniques to prevent left ventricular outflow tract obstruction (suture fixation of the distal stent) and paravalvular leaks (the addition of a pericardial cuff) should be considered. The Melody valve can be percutaneously expanded as the child grows.

Congenital quadricuspid aortic valve associated with aortic insufficiency and mitral regurgitation.

Congenital quadricuspid aortic valve is a rare cardiac anomaly. More than half of the patients with this abnormality will develop aortic insufficiency in adulthood. It is vital that patients with quadricuspid aortic valve who present with progressive aortic regurgitation undergo valve replacement or repair at appropriate time. Here, we present two cases of quadricuspid aortic valve. We first describe a 58-year-old man who had mitral regurgitation and ascending aorta dilatation with quadricuspid aortic valve. He underwent aortic valve replacement and mitral valve plasty and recovered well. The second patient is a 20-year-old asymptomatic boy who has been closely followed up and has not received any surgical treatment.

Atrial septal hematoma after mitral valve and left coronary artery surgery in a child.

Atrial septal hematoma (ASH) has been reported mostly in adult patients to occur after spontaneous ascending aortic dissection (Circulation 46(3):537-545, 1972) and after surgery for coronary artery (Anesthesiology 83(3):620-621, 1995), mitral valve, and aortic valve (Rev Esp Cardiol 55(8):867-871, 2002). ASH in the pediatric age group is rare, and to our knowledge only two cases have been published (Am J Perinatol 27(6):481-483, 2010; J Am Soc Echocardiogr 1087-1089, 1998). We report a case of ASH diagnosed by intraoperative transesophageal echocardiography in a 28-month-old child who underwent mitral valvuloplasty and left main coronary arterioplasty for anomalous origin of the left coronary artery from pulmonary artery. ASH subsided on its own within 24 h.

[Clinical and morphological parallels in mitral valve disorders in infants with atrioventricular defect].

Mitral valves tissue samplings from children with complete (13 patients) and partial (6 patients) atrioventricular defects at the age of from I month to 3 years old were examined. The biopsy material was received during the repeat surgical operation on mitral valve, performed due to residual mitral valve regurgitation grade 3-4 at the period of time from 2 days to 1 year after radical defect correction. On histological examination the areas of myxomatous tissue degeneration occupying more than 50% of mitral valves surface were found in 6 (32%) of the 19 patients. There were dispersed star-shaped cells, architectonics disturbances, deposition of acid mucopolysaccharides and increased content of matrix metalloproteinase 13 in such areas of myxomatous degeneration. The sizes of these areas correlated with mitral valve regurgitation grade. After the radical correction of atrioventricular defect the sutures on the folds and fibrous ring of the mitral valve "cut through" reliably more often in patients with wider areas of myxomatous degeneration, which indicates poor prognosis. According to the ultrastructural classification the majority of mitral valve cells regarded as fibroblasts; there also were found cells with the signs of myogenic differentiation--myofibroblasts and isolated hystiocytes. According to the immunohistochemistry assay the cells phenotype regarded as fibroblastic and endothelial differentiation; in some patients there were found cells of smooth muscle origin.

Mitral valve dysplasia characterized by isolated cleft of the anterior leaflet resulting in fixed left ventricular outflow tract obstruction.

A 7-month-old, sexually intact male English toy spaniel weighing 4 kg was referred for evaluation of a subclinical cardiac murmur. Echocardiography disclosed fixed left ventricular outflow tract obstruction that was caused by attachment of a cleft anterior mitral valve leaflet to the interventricular septum. Neither atrial nor ventricular septal defects were detected. Fixed obstruction of the left ventricular outflow tract by a malformed mitral valve is rare in human beings and has not been previously reported in the dog.