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The purpose of this study is to determine the site of attachment of the posterior mitral annulus to the left ventricle in patients with mitral annular disjunction (MAD) and mitral valve prolapse (MVP). The posterior annulus normally attaches to the inlet of the left ventricle. Some histological findings suggest that the disjunctive annulus may instead attach anomalously to the left ventricular (LV) crest in patients with MVP. We used cardiac magnetic resonance imaging to determine the site of attachment of the posterior mitral annulus (crest vs inlet) in 25 patients with MVP with MAD (MAD+ group) and 24 patients with MVP without MAD (MAD- group). The site of annular attachment was determined in the 3-chamber view during diastole. Our data demonstrate complete separation in mitral annular attachment site between MAD+ and MAD- groups. All patients in the MAD+ group demonstrated annular attachment to the LV crest, whereas all those in the MAD- group demonstrated annular attachment to the LV inlet (p <0.001). The presence of anomalous annular attachment in MAD+, but not MAD- patients, suggests this anatomic abnormality represents a feature of the MAD phenotype rather than the myxomatous phenotype. Anomalous annular attachment may potentially influence the arrhythmic potential of MAD.

Two mutations in the gene DCHS1 are shown to cause non-syndromic mitral valve prolapse (MVP), a common cardiac valve disease; understanding the role of DCHS1 in mitral valve development and MVP pathogenesis holds therapeutic potential. A genetic cause of mitral valve abnormality The genetic cause of non-syndromic mitral valve prolapse (MVP) — a common cardiac valve disease — has been elusive. Here, Susan Slaugenhaupt and colleagues report two mutations in the DCHS1 gene that segregate with MVP in three families. Knockdown of the zebrafish homologue dachsous1b caused a cardiac atrioventricular canal defect that could be rescued by wild-type human DCHS1 , but not by mutant DCHS1 mRNA. Dchs1 +/− mice had prolapse of thickened mitral leaflets due to developmental errors in valve morphogenesis. DCHS1 deficiency in human and mouse mitral valve interstitial cells resulted in altered migration and cellular patterning. Understanding the role of DCHS1 in mitral valve development and MVP pathogenesis holds potential for therapeutic insights for this very common disease. Mitral valve prolapse (MVP) is a common cardiac valve disease that affects nearly 1 in 40 individuals 1 , 2 , 3 . It can manifest as mitral regurgitation and is the leading indication for mitral valve surgery 4 , 5 . Despite a clear heritable component, the genetic aetiology leading to non-syndromic MVP has remained elusive. Four affected individuals from a large multigenerational family segregating non-syndromic MVP underwent capture sequencing of the linked interval on chromosome 11. We report a missense mutation in the DCHS1 gene, the human homologue of the Drosophila cell polarity gene dachsous ( ds ), that segregates with MVP in the family. Morpholino knockdown of the zebrafish homologue dachsous1b resulted in a cardiac atrioventricular canal defect that could be rescued by wild-type human DCHS1 , but not by DCHS1 messenger RNA with the familial mutation. Further genetic studies identified two additional families in which a second deleterious DCHS1 mutation segregates with MVP. Both DCHS1 mutations reduce protein stability as demonstrated in zebrafish, cultured cells and, notably, in mitral valve interstitial cells (MVICs) obtained during mitral valve repair surgery of a proband. Dchs1 +/− mice had prolapse of thickened mitral leaflets, which could be traced back to developmental errors in valve morphogenesis. DCHS1 deficiency in MVP patient MVICs, as well as in Dchs1 +/− mouse MVICs, result in altered migration and cellular patterning, supporting these processes as aetiological underpinnings for the disease. Understanding the role of DCHS1 in mitral valve development and MVP pathogenesis holds potential for therapeutic insights for this very common disease.

Background: Double-orifice mitral valve (DOMV) is a rare congenital anomaly. Repair strategies for mitral valve prolapse in the setting of DOMV remain technically challenging. Case summary: We report a case of DOMV presenting with severe mitral regurgitation (MR) due to chordal rupture and annular dilatation, successfully treated with totally endoscopic mitral valve repair. Regarding the surgical approach, preserving the fibrous bridge, we repaired a DOMV via an endoscopic approach with neochords and an edge-to-edge stitch for leaflet prolapse, and a flexible band for annular dilatation, achieving trivial MR without stenosis. Conclusion: This case demonstrates minimally invasive mitral valve repair for DOMV, in which the regurgitation was successfully controlled while preserving the fibrous bridge and avoiding mitral stenosis.

The singular leaflet mitral valve is a rare and complex entity that has been described in only a few case reports. It is commonly observed in infancy and combined with symptomatic mitral regurgitation (MR). In most asymptomatic individuals, this complication is attributed mainly to a hypoplastic posterior mitral leaflet and is usually revealed in adulthood. We present the case of a 30-year-old man who presented with chest pain and dyspnea for two days. The electrocardiogram showed complete atrioventricular block with a beat rate of 30 bpm. Echocardiography revealed an elongated anterior mitral valve leaflet and an almost completely absent posterior mitral valve leaflet with no MR. Our case is unusual presentation of a unileaflet mitral valve that should be investigated even in adulthood as these patients are likely to be at risk of developing or worsening MR later in life; hence, performing periodic echocardiograms is important for detecting the progression of MR early.

Congenital mitral stenosis is a rare anomaly. We report successful surgical management of congenital mitral stenosis in a patient with Kabuki syndrome. A 15-month-old male with Kabuki syndrome was referred and echocardiography showed isolated congenital mitral stenosis. The valve was not repairable as it was showing severe stenotic features in multilevel fashion. Valve was replaced with mechanical valve. Postoperative course was uneventful without any rhythm problem and he has been doing well clinically during the 25 months follow-up with regular international normalized ratio checks.

Congenital anomalies of the mitral valve apparatus are rare. Of such cases, congenital mitral stenosis, atresia, accessory valvular tissue, and cleft mitral valve are more common. Descriptions of unileaflet mitral valves (either partial or complete leaflet agenesis/hypoplasia) are extremely rare and largely limited to a few case reports. We report herein a 45-year-old nulliparous woman presenting to our outpatient clinic with chest discomfort and dyspnea on minimal exertion. Mitral valve with a single leaflet leading to a significant obstruction in left ventricular outflow was seen on transthoracic echocardiography and confirmed with transesophageal two- (2D) and three-dimensional (3D) echocardiography. Metoprolol was prescribed to relieve obstruction and symptoms.

Backgrounds We aimed to study the outcomes of mitral valve (MV) reoperations in children with congenital MV diseases and to summarize our treatment experience. Methods In this single-center retrospective study, we enrolled 24 patients aged < 18 years who underwent MV reoperation from among 265 patients who underwent MV repair between January 2013 and July 2023. MV reoperations were based on the types of MV disease. Cox regressions were used to analyze the risk factors for death and second MV reoperation. Results A total of 5 patients underwent second MV reoperations. 3 patients experienced early death, and 1 experienced late death. The 3- and 5-year survival rates of the entire cohort were 86.6% ± 7.3% and 72.1% ± 14.5%, respectively. Patients who had the double-orifice MV technique applied during MV reoperation were significantly more prone to receive mechanical MV replacement ( P  < 0.0001). The use of double-orifice MV technique during MV reoperation was identified as an independent risk factor for second MV reoperation ( HR  = 8.136, 95% CI  = 1.099–60.240; P  = 0.040). Conclusions The reoperation of the MV in children with congenital MV diseases poses a formidable challenge, manifested by a high postoperative mortality rate and re-intervention rate. Patiently and meticulously repair based on the types of MV disease has demonstrated the capacity to enhance and sustain stable valve function and cardiac function in the vast majority of children. The use of the double-orifice MV technique did not achieve ideal therapeutic results in children with complex valve lesions.

Abstract OBJECTIVES Mitral annular disjunction is a recognized risk factor for malignant ventricular arrhythmias in patients with mitral valve prolapse. This study aimed to evaluate the impact of mitral valve surgery on the burden of supraventricular and ventricular ectopic activity in patients with mitral annular disjunction. METHODS Data from 32 patients who underwent mitral valve surgery between 2017 and 2024 were retrospectively analysed. The primary end-point was the change in the burden of supraventricular and ventricular ectopy following surgery. The secondary end-point was to assess whether this change differed according to the extent of the mitral annular disjunction distance. RESULTS The mean age of the patients was 38.8 ± 14 years, and 53.1% were female. The vast majority (96.9%) underwent successful mitral valve repair. The mean cardiopulmonary bypass and aortic cross-clamp times were 133.3 ± 38 min and 95.8 ± 32 min, respectively. Postoperatively, the mean burden of supraventricular ectopy decreased from 13.8% to 3.7%, and ventricular ectopy burden decreased from 7.1% to 2.1%. Among patients with a mitral annular disjunction distance ≥8.5 mm, both supraventricular and ventricular ectopic activity significantly declined. In contrast, for those with a mitral annular disjunction distance <8.5 mm, a significant reduction was observed only in ventricular ectopy, with no statistically significant change in supraventricular ectopy. CONCLUSIONS Mitral valve surgery is associated with a substantial reduction in both supraventricular and ventricular ectopic activity in patients with mitral annular disjunction. Furthermore, a mitral annular disjunction distance greater than 8.5 mm appears to be a predictor of a more pronounced reduction in arrhythmic burden following surgery. Mitral annular disjunction (MAD) is defined as the separation of the mitral valve annulus from the tip of the left ventricle, along with an abnormal systolic excursion of the leaflet hinge point into the left atrium [1]. Graphical Abstract

We assessed the effect of anomalous aortic origin of a coronary artery on the risk of early and late postoperative events after aortic or mitral valve replacement in adults. Between 2005 and 2022, 29,579 adults underwent surgical aortic or mitral valve replacement at Cleveland Clinic. Among these, 29 had an unrepaired coronary artery rising anomalously from the aorta that was not intervened upon during valve surgery, 19 (65%) an anomalous circumflex, and 9 (31%) an anomalous right. Operative outcomes were compared between the 29 patients with anomalous coronary arteries and 87 balancing score (1:3) matched patients with normal coronary origin. Median follow-up was 6.5 years. Among matched groups, major morbidity and mortality 24% ( = 7) in patients with anomalous coronaries and 20% ( = 17) among patients with normal coronary origin ( = .7). Ten-year freedom from coronary reintervention was 83% versus 100% ( [log-rank] = .005), and 10-year survival was 59% versus 53% ( [log-rank] = .8). One patient experienced a coronary injury from valve surgery, in which the incidentally found anomalous retroaortic circumflex was immediately repaired without further complication. There was no coronary reintervention after discharge in the normal coronary origin group and three in the anomalous coronary group; however, only one of these patients required intervention on the anomalous coronary. Anomalous coronaries were uncommon in surgical valve replacement patients at a high-volume centre. The origin and course of each coronary should be assessed before valve replacement. With careful planning, valve replacement does not result in a significantly higher prevalence of postoperative ischaemia, mortality, or reintervention.

Accessory mitral valve tissue is a rare congenital cardiac anomaly that can lead to left ventricular outflow tract obstruction. We present the case of an older female patient with accessory mitral valve tissue, a subaortic membrane, an unruptured aneurysm of the aortic sinus, and left ventricular outflow tract obstruction. Successful excision of the accessory mitral valve tissue and repair of the aortic sinus were performed. Postoperative echocardiography showed complete removal of the subaortic membrane, with a residual accessory mitral valve tissue (5 mm). Color Doppler imaging revealed a significant reduction in mosaic flow signals in the left ventricular outflow tract, with the peak blood flow velocity decreasing to 1.6 m/s. The postoperative course was uneventful, and the patient was followed up with echocardiography at 3 days, 5 days, 1 month, 3 months, and 6 months after the surgery. Accessory mitral valve tissue is a rare congenital defect frequently associated with other cardiovascular congenital malformations. This report also provides a comprehensive clinical review of accessory mitral valve tissue, covering anatomical classification, associated cardiac anomalies, pathophysiology, diagnostic approaches, and treatment strategies to offer an improved clinical understanding of the condition.