Relationship between the severity of functional mitral regurgitation at admission and one-year outcomes in patients hospitalized for acute heart failure with mildly reduced ejection fraction.

BACKGROUND: The epidemiological distribution of functional mitral regurgitation (FMR) in heart failure (HF) and mildly reduced ejection fraction (HFmrEF) patients and its impact on outcomes remains unclear. We attempt to investigate the prognosis of FMR in patients with HFmrEF. METHODS: The HF center registry study is a prospective, single, observational study conducted at the Second Affiliated Hospital of Shenzhen University, where 2330 patients with acute HF (AHF) were enrolled and 890 HFmrEF patients were included in the analysis. The patients were stratified into three categories based on the severity of FMR: none/mild, moderate, and moderate-to-severe/severe groups. Subsequently, a comparison of the clinical characteristics among these groups was conducted, along with an assessment of the incidence of the primary endpoint (comprising all-cause mortality and readmission for HF) during a one-year follow-up period. RESULTS: The one-year follow-up results indicated that the primary composite endpoint occurrence rates in the three groups were 23.5%, 32.9%, and 36.5%, respectively. The all-cause mortality rates in the three groups were 9.3%, 13.7%, and 16.4% respectively. Survival analysis demonstrated a statistically significant difference in the occurrence rates of the primary composite endpoint and all-cause mortality among the three groups (P < 0.05). Multifactor Cox regression revealed that moderate FMR and moderate-to-severe/severe FMR were independent risk factors for adverse clinical prognosis in HFmrEF patients, with hazard ratios and 95% confidence intervals of 1.382 (1.020-1.872, P = 0.037) and 1.546 (1.092-2.190, P = 0.014) respectively. CONCLUSIONS: Moderate FMR and moderate-to-severe/severe FMR independently predict an unfavorable prognosis in patients with HFmrEF.

Comparison of Mitral Valve Repair Versus Percutaneous Mitral Balloon Commissurotomy for Patients With Rheumatic Heart Disease: A Single-Centre Study.

BACKGROUND: Percutaneous mitral balloon commissurotomy (PMBC) is the standard treatment option for patients with rheumatic mitral stenosis (MS), according to current guidelines. This study aimed to compare the outcomes of rheumatic mitral valve repair (rMVR) and PMBC in this patient population. METHODS: Baseline, clinical, and follow-up data from 703 patients with rheumatic heart disease who underwent PMBC or rMVR at the current centre were collected and analysed. A 1:1 propensity score (PS) matching method was used to balance the differences in baseline characteristics between the two groups. The primary outcome was mitral valve reoperation, and the secondary outcome was all-cause mortality. RESULTS: Propensity score matching generated 101 patient pairs for comparison. In the matched population, there were no significant differences in the early clinical outcomes between the groups. The median follow-up time was 40.9 months. Overall, patients in the rMVR group had a statistically significantly lower risk of mitral valve reoperation than those in the PMBC group (HR 0.186; 95% CI 0.041-0.835; p=0.028). Regarding all-cause mortality, no statistically significant differences were observed between the rMVR and PMBC groups (HR 4.065; 95% CI 0.454-36.374; p=0.210). CONCLUSIONS: Compared with PMBC, rMVR has more advantages for the correction of valve lesions; therefore, it may offer a better prognosis than PMBC in select patients with rheumatic MS. However, this finding needs to be verified in future studies with larger sample sizes and longer follow-up periods.

Clinical outcome of patients with rheumatic tricuspid valve disease: matched cohort study.

Rheumatic heart disease (RHD) is still a major health problem, especially in low- to mid-income countries, leading premature deaths owing to valvular disease. Although left-sided valvular involvement is most commonly seen in RHD, the tricuspid valve can also be affected. However, there is a lack of information about the prognostic value of primary tricuspid valve (TV) disease in RHD. This study aimed to determine the impact of TV disease on clinical outcome in RHD. This prospective study enrolled patients with rheumatic mitral valve disease (MVD) referred to a tertiary center for management of heart valve disease. Primary rheumatic TV disease was defined by echocardiographic features including thickening of leaflets associated with some degree of restricted mobility. Patients with rheumatic TV disease were matched to patients with MVD using 1:1 genetic matching algorithm that maximized balance of baseline covariates prior to exploring outcome differences. The main outcome was either need for MV replacement or death. Among 694 patients eligible for the study, age of 47 ± 13 years, 84% female, 39 patients (5.6%) had rheumatic TV disease. After excluding patients with incomplete data, 33 patients with TV disease were matched to 33 controls based on age, right-sided heart failure, atrial fibrillation, and MV area. During a mean follow-up of 42 months (median 28, IQR 8 to 71 months), 32 patients (48.5%) experienced adverse events, including 6 cardiovascular deaths and 26 patients who underwent surgery for mitral valve replacement. The adjusted analysis demonstrated a significant association between TV disease and the outcome, with a hazard ratio (HR) of 3.386 (95% CI 1.559-7.353; P = 0.002) in the genetic matched cohort with balance on baseline covariates of interest. The model exhibited good discriminative ability, as indicated by a C-statistic of 0.837. In patients with rheumatic mitral valve disease, rheumatic TV disease significantly increased risk of adverse events compared with matched controls. The involvement of TV may express overall disease severity that adversely affects clinical outcome.

Dysplastic Mitral Valve in Costello Syndrome.

Costello syndrome is an autosomal dominant condition caused by variants in the HRAS gene. Cardiac presentation includes valvular disease (usually valvar pulmonary stenosis), arrhythmias, and hypertrophic cardiomyopathy. To our knowledge, this is the first such report of dysplastic mitral valve associated with Costello syndrome.

Outcomes of maze procedure and mitral valve surgery in atrial functional mitral regurgitation: a retrospective study.

BACKGROUND: Atrial functional mitral regurgitation (AFMR) is a newly discovered condition associated with longstanding atrial fibrillation. This retrospective study aimed to analyze the outcomes of the maze procedure and mitral regurgitation (MR) surgery in AFMR and atrial fibrillation in comparison with those in degenerative MR (DMR). METHODS: Patients who underwent mitral valve repair/replacement with a maze procedure at a hospital (July 2012-August 2021) were included. We excluded patients aged below 18 years undergoing concomitant coronary artery bypass grafting or atrial septal defect repair and those with MR etiology other than ARMR or DMR. RESULTS: We included 35 patients with AFMR and 50 patients with DMR. Patient characteristics and postoperative outcomes were not significantly different between the two groups. Long-term outcomes revealed no significant differences in the ratio of cardiac mortality, stroke, or hospital readmission. However, after the maze procedure, the sinus rhythm restoration rate was significantly lower (62% vs. 28.5%, p < 0.001), a junctional rhythm state (p < 0.001) and permanent pacemaker insertion for sick sinus syndrome (SSS) (p = 0.03) were significantly more common in AFMR than DMR. On postoperative transthoracic echocardiography (TTE), the pulmonary artery systolic pressure was significantly less decreased in the AFMR group than in the DMR group compared with that on preoperative TTE (p = 0.04). CONCLUSIONS: AFMR showed excellent mitral valve surgery outcomes, similar to DMR, but had a significantly higher risk of pacemaker insertion for SSS after the maze procedure.

Noncomplex ventricular arrhythmia associated with greater freedom from recurrent ectopy at 1 year after mitral repair surgery.

Objective: The effect of mitral valve (MV) surgery on the natural history of ventricular arrhythmia (VA) in patients with arrhythmic MV prolapse remains unknown. We sought to evaluate the cumulative incidence of VA at 1 year after surgical mitral repair. Methods: A retrospective review of progressively captured data identified 204 consecutive patients who underwent elective MV repair for significant degenerative mitral regurgitation as a first-time cardiovascular intervention in a quaternary reference center between January 2018 and December 2020. A subset of 62 consecutive patients with diagnosed arrhythmic MV prolapse was further evaluated for recurrent VA after MV repair. Results: The median age was 62 years (range, 27-77 years) and 26 of 62 (41.9%) were female. The median time from initial mitral regurgitation/MV prolaspe diagnosis-to-referral was 13.8 years (interquartile range [IQR], 5.4-25) and from VA diagnosis-to-referral was 8 years (IQR, 3-10.6). Using the Lown-Wolf classification, complex VA (Lown grade ≥3) was identified in 36 of 62 patients (58%) at baseline, whereas 8 of 62 (13%) had a cardioverter/defibrillator implanted for primary (4/8) or secondary (4/8) prevention. Left ventricular myocardial scar was confirmed in 23 of 34 (68%) of patients scanned at baseline. The prevailing valve phenotype was bileaflet Barlow (59/62; 95.2%). All patients underwent surgical MV repair by the same team. Surgical repair was stabilized with an annuloplasty prosthesis (median size 36 mm [IQR, 34-38]). Concomitant procedures included tricuspid valve repair (51/62; 82.3%), cryo-maze ± left atrial appendage exclusion (14/62, 23%), and endocardial cryoablation of VA ectopy (4/62; 6.5%). The 30-day and 1-year freedom from recurrent VA were 98.4% and 75.9%, respectively. Absent VA after mitral repair was uniformly observed in patients with minor VA at baseline. Absent VA after mitral repair was uniformly observed in patients with minor VA preoperatively. Complex baseline VA was the strongest predictor of recurrent VA (hazard ratio, 10.8; 95% confidence interval, 1.4-84.2; P = .024), irrespective of myocardial fibrosis. Conclusions: In a series of 62 consecutive patients operated electively for arrhythmic mitral prolapse, VA remained undetected in 75.9% of patients at 1 year. Freedom from recurrent VA was greater among patients without complex VA preoperatively, whereas baseline Lown grade ≥3 was the strongest independent risk factor for recurrent VA at 1 year. These findings attest to the importance of early recognition and prompt referral of patients with mitral prolapse and progressive VA to specialty interdisciplinary care.

Cardiac myxoma as a rare acute heart failure etiology in paediatrics: A case report.

Cardiac myxoma is extremely rare in children. However, if not treated immediately, it may cause varying symptoms until sudden death. A-9-years old male Javanese child was brought to the emergency department of Prof. Soekandar General Hospital, Mojokerto with progressive dyspnoea since one month which got worse in the left decubitus position. There was no significant past medical history. Physical examination revealed hypotension, mitral stenosis, tricuspid regurgitation, and pulmonary congestion. Transthoracic echocardiography revealed a round pedunculated 3x3.3 cm mass in the Left Atrium that swingingly moved to the Left Ventricle during diastole. This was diagnosed provisionally as Myxoma with a differential of thrombus. After stabilization, he was referred to a tertiary hospital for emergency excision. Histopathology confirmed the myxoma. There were no symptoms and activity limitations during the 6 months follow-up. To the best of our knowledge, this is the first paediatric cardiac myxoma with Acute Heart Failure symptoms reported in Indonesia. Echocardiography is imperative for diagnosing myxoma. Appropriate and timely management results in an excellent outcome.

LEOPARD syndrome with accelerated idioventricular rhythm and systolic anterior motion of the posterior mitral leaflet: a case report.

Background: PTPN11 is ubiquitously expressed and has a variety of phenotypes even in a single heart. We examined LEOPARD syndrome (LS) in a patient with PTPN11 variants through pathological, electrophysiological, and anatomical studies. Case summary: A 49-year-old man with no previous medical history was brought to our emergency department because of syncope. An electrocardiogram (ECG) revealed alternating bundle branch block, and echocardiography revealed hypertrophic cardiomyopathy-like morphology with systolic anterior motion of the posterior mitral valve. Atrioventricular block, left ventricular outflow tract (LVOT) obstruction, and ventricular tachycardia were considered the differential diagnoses; however, the treatment plan was difficult to determine. An electrophysiological study revealed the cause of the ECG abnormality to be accelerated idioventricular rhythm, and the programmed ventricular stimulation was negative. Genetic testing revealed LS with PTPN11 variant, which was speculated to be the cause of these various unique cardiac features. The cause of syncope was considered to be exacerbation of LVOT obstruction due to dehydration, and the patient was treated with oral beta-blockers. Implantable loop recorder observation for 1 year revealed no arrhythmia causing syncope, and an implantable cardioverter-defibrillator and pacemaker were deemed unnecessary for primary prevention of syncope. During 2.5 years of follow-up, the LVOT peak velocity fluctuated between 2.5 and 3.5 m/s, but the patient remained stable with no recurrent syncope. Conclusion: We confirmed that LS is distinct from other cardiomyopathies using characterization, physiological, electrophysiological, and pathological examinations. Evidence supporting a specific treatment strategy for LS is limited, and understanding the pathogenesis may help establish effective treatment strategies.

When annuloplasty is not enough: a case report of ventricular arrhythmias stepwise abolition after mitral valve re-repair.

Background: Some patients affected by mitral valve (MV) prolapse (MVP) are at higher risk of ventricular arrhythmias (VAs), but the underlying pathogenesis, as well as the effects of surgery on VA, remain not fully understood. Mitral valve repair, however, represents a privileged point of view to deepen the understanding of arrhythmogenesis in this context. Hence, we report an interesting case of MV re-repair. Case summary: A 52-year-old man was referred to our institution for severe mitral regurgitation (MR) due to P2 prolapse in the context of myxomatous MV degeneration. Pre-operative imaging showed systolic mitral annular disjunction, left ventricular (LV) wall curling, Pickelhaube's sign, and a prolapsing tricuspid valve (TV) with only mild regurgitation. Twenty-four-hour electrocardiogram (ECG) Holter revealed a significant burden of premature ventricular contractions (PVCs), most of them originating from anterior papillary muscle (APM), posterior papillary muscle (PPM), and mitral annulus (MA). Quadrangular resection of P2 and mitral annuloplasty were performed. One year later, relapse of severe MR due to a residual P2M1 prolapse occurred. Twenty-four-hour ECG Holter showed no PVCs from PPM and MA, while those from APM persisted. A central edge-to-edge repair was effectively used to fix the residual prolapse. After 1 year from REDO surgery, a third ECG Holter confirmed the absence of any remaining LV PVCs, but still few ectopic beats originating from TV were recorded. Discussion: Here, we report a case of VA resolution after specific, anatomical triggers addressing surgical gestures. Our experience confirms that MV surgery may have a role in MVP patients' arrhythmias correction.

A Hole in the Heart, a Hole in the Defenses: A Case of Pseudomonas Endocarditis.

Infective endocarditis (IE) is a rare but serious infection of the cardiac endothelium. This case report presents a rare instance of left-sided Pseudomonas aeruginosa endocarditis in an immunocompetent patient without traditional risk factors for IE. Pseudomonas endocarditis is uncommon and usually associated with specific factors. The patient in this case was a 30-year-old male with end-stage renal disease, receiving hemodialysis through a tunneled dialysis catheter, who developed a fever. Blood cultures confirmed P. aeruginosa as the causative agent, which prompted the administration of appropriate antibiotics and the removal of the catheter. However, subsequent imaging revealed significant damage to the mitral valve. Despite timely mitral valve replacement and aggressive medical treatment, the patient's condition worsened, and he ultimately succumbed to the infection. This case also emphasizes the necessity of timely diagnosis and intervention. In this patient, by the time it was diagnosed and managed, significant mitral valve damage had already occurred. Therefore, it should be considered a differential diagnosis even in patients with no risk factors and should be managed vigorously. Pseudomonas endocarditis is associated with high mortality, and successful treatment often requires a combination of antipseudomonal antibiotics due to the organism's ability to develop resistance. Surgical intervention, such as valve replacement, is frequently necessary. This case underscores the importance of considering P. aeruginosa infection, even in patients without traditional risk factors for IE. Early diagnosis, appropriate antibiotic therapy, and timely surgical intervention are critical for improving outcomes in Pseudomonas endocarditis cases.

Type III fetal redundancy of the foramen ovale flap mimics hemodynamic changes of mitral stenosis.

Type III redundancy of the foramen ovale flap (RFOF) mimics hemodynamic changes of mitral stenosis(MS), which has not been particularly highlighted in previous literature but carries a favorable prognosis.

Combining 2D Planimetry and Yeo's Index Can Help Accurately Identify Patients with Severe Rheumatic Mitral Stenosis-A Perspective from a 3D Assessment Using Transoesophageal Echocardiography.

BACKGROUND: Yeo's index is a novel measure of the severity of rheumatic mitral valve stenosis (MS). It is derived from the product of the mitral leaflet separation index and dimensionless index. This study aims to validate Yeo's index using a transesophageal echocardiogram (TEE) three-dimensional (3D) mitral valve area (MVA) as a comparator and to compare the concordance of existing echocardiographic measures of the MVA with TEE 3DMVA. METHODS AND RESULTS: We studied 111 patients with rheumatic MS who underwent both transthoracic echocardiography (TTE) and a TEE assessment of MS severity. Yeo's index, the MVA determined by 2D planimetry, pressure half-time (PHT) and continuity equation (CE) measured on TTE were compared with the TEE 3DMVA. With a linear correlation, Yeo's index showed the best correlation with TEE 3DMVA (r2 = 0.775), followed by 2D planimetry (r2 = 0.687), CE (r2 = 0.598) and PHT (r2 = 0.363). Using TEE 3DMVA as comparator, Yeo's index (ρc = 0.739) demonstrated the best concordance, followed by 2D planimetry (ρc = 0.632), CE (ρc = 0.464) and PHT (ρc = 0.366). When both Yeo's index and 2D planimetry suggested significant MS, the positive predictive value was high (an AUC of 0.966 and a PPV of 100.00% for severe MS, and an AUC of 0.864 and a PPV of 85.71% for very severe MS). When both measures suggested the absence of significant MS, the negative predictive value was also high (an AUC of 0.940 and an NPV of 88.90% for severe MS, and an AUC of 0.831 and an NPV of 88.71% for very severe MS). CONCLUSIONS: Yeo's index performed well in identifying severe MS when compared with TEE 3DMVA and may be a useful adjunct to existing methods of measuring MS severity. Combining it with 2D planimetry could further enhance its accuracy.

20 Years of triple-valve surgery in the UK: demographic and outcome trends.

OBJECTIVES: To describe evolving demographic trends and early outcomes in patients undergoing triple-valve surgery in the UK between 2000 and 2019. METHODS: We planned a retrospective analysis of national registry data including patients undergoing triple-valve surgery for all aetiologies of disease. We excluded patients in a critical preoperative state and those with missing admission dates. The study cohort was split into 5 consecutive 4-year cohorts (groups A, B, C, D and E). The primary outcome was in-hospital mortality, and secondary outcomes included prolonged admission, re-exploration for bleeding, postoperative stroke and postoperative dialysis. Binary logistic regression models were used to establish independent predictors of mortality, stroke, postoperative dialysis and re-exploration for bleeding in this high-risk cohort. RESULTS: We identified 1750 patients undergoing triple-valve surgery in the UK between 2000 and 2019. Triple valve surgery represents 3.1% of all patients in the dataset. Overall mean age of patients was 68.5 ± 12 years, having increased from 63 ±12 years in group A to 69 ± 12 years in group E (P < 0.001). Overall in-hospital mortality rate was 9%, dropping from 21% in group A to 7% in group E (P < 0.001). Overall rates of re-exploration for bleeding (11%, P = 0.308) and postoperative dialysis (11%, P = 0.066) remained high across the observed time period. Triple valve replacement, redo sternotomy and poor preoperative left ventricular ejection fraction emerged as strong independent predictors of mortality. CONCLUSIONS: Triple-valve surgery remains rare in the UK. Early postoperative outcomes for triple valve surgery have improved over time. Redo sternotomy is a significant predictor of mortality. Attempts should be made to repair the mitral and/or tricuspid valves where technically possible.

Doing a double take: when transthoracic echocardiography fails for mitral valve annuloplasty ring endocarditis with Streptococcus pyogenes: a case report.

BACKGROUND: This case highlights several complications of a late and rare presentation of culture-negative Streptococcus pyogenes endocarditis of a previously repaired mitral valve with an annuloplasty ring including recurrent cardioembolic strokes, which was initially missed on transthoracic echocardiography. CASE PRESENTATION: A 66-year-old Caucasian female with prior mitral valve prolapse status post mitral valve annuloplasty and left atrial appendage occlusion, followed by two strokes, presented with supraventricular tachycardia that resolved spontaneously. During an inpatient admission, she developed symptoms of another stroke, and imaging studies were suggestive of recurrent cardioembolic phenomenon. Additional workup revealed two small intra-atrial masses adherent to the mitral annuloplasty ring missed on prior evaluation for recurrent stroke. She underwent surgical repair in the setting of a chronic culture-negative infectious endocarditis with Streptococcus pyogenes and recovered well with no further cardioembolic phenomenon. CONCLUSION: This case serves to highlight the importance of having a higher index of suspicion in any cardiac prosthesis patient for endocarditis when presenting with symptoms such as recurrent stroke, arrhythmias, and abnormal cardiac lab work. It also demonstrates the need for appropriate imaging with transthoracic echocardiography followed by transesophageal echocardiography and reviews surgical indications to diagnose and treat culture-negative endocarditis.

Impact on Outcome of Different Mechanisms, Baseline Degree and Changes of Mitral Regurgitation in Patients With Aortic Stenosis Who Underwent Transcatheter Aortic Valve Replacement.

BACKGROUND: Mitral regurgitation (MR) is frequent in patients with aortic stenosis (AS). Although primary MR is an established negative prognostic factor, whether different mechanisms of MR have different effects on outcome is currently unknown. The aim of this study was to evaluate the impact of the MR mechanism in patients undergoing transcatheter aortic valve replacement (TAVR). METHODS AND RESULTS: This is a retrospective observational study of patients who underwent TAVR for severe aortic stenosis in a high-volume tertiary care center. Echocardiographic comprehensive MR assessment was performed at baseline and within 3 months post TAVR. The study population was divided into 4 groups according to MR mechanism: Group I: fibro-calcific leaflet degeneration; Group II: prolapse/flail; Group III: ventricular secondary MR (functional MR); and Group IV: atrial functional MR. The study end point was a combination of death from cardiovascular cause and heart failure-related hospitalization. The study population included 427 patients (mean age 81.7±6.5 years; 71% primary MR; 62% ≥moderate MR). At 3-year follow-up, survival free from the composite end point significantly differs according to MR mechanism: it was higher in group IV (atrial functional MR, 96.6%) compared with group I (80.4%, P=0.002) and group II patients (60.7%, P=0.001), and group III (84.8%, P=0.037); patients with MR due to leaflet prolapse showed poorer prognosis compared with patients with functional MR (group III, P=0.023 and group IV, P=0.001) and with group I (P=0.040). Overall, severe MR after TAVR identified patients with poorer prognosis and was significantly more frequent in group II (46.4%, P=0.001). CONCLUSIONS: In patients undergoing TAVR, preprocedural identification of MR mechanism and mechanism provides prognostic insights.

Mitral valve replacement in young patients: review and current challenges.

Mitral valve repair is the ideal intervention for mitral valve disease with excellent long-term survival comparable to the age-matched general population. When the mitral valve is not repairable, mechanical prostheses may be associated with improved survival as compared with biological prostheses. Newer mechanical and biological valve prostheses have the potential to improve outcomes following mitral valve replacement in young patients. Patients presenting for mitral valve surgery after failed transcatheter mitral valve-in-valve have high rates of postoperative mortality and morbidity, exceeding those seen with reoperative mitral valve surgery, which poses issues in young patients who have a higher cumulative incidence of reintervention.

Patients presenting with mitral valve disease, the most common type of heart valve disease, have a survival advantage when they undergo mitral valve repair as opposed to replacement, and this is particularly true for young patients. When the mitral valve is not repairable, mechanical prostheses (prosthetic implants) may be associated with improved survival as compared with biological prostheses, and this difference is mostly observed until the age of 70 years. Newer techniques of treating mitral valve disease without requiring open heart surgery have not yet been shown to be superior or even equivalent to traditional open heart surgery in the general population. Patients presenting for mitral valve surgery after failure of these newer techniques have high rates of death, exceeding those seen with mitral valve reoperation, which has important implications for young patients with mitral valve disease.

Long-term outcomes following mitral valve replacement in children at heart center Leipzig: a 20-year analysis.

BACKGROUND: Although mitral valve repair is the preferred surgical strategy in children with mitral valve disease, there are cases of irreparable severe dysplastic valves that require mitral valve replacement. The aim of this study is to analyze long-term outcomes following mitral valve replacement in children in a tertiary referral center. METHODS: A total of 41 consecutive patients underwent mitral valve replacement between February 2001 and February 2021. The study data was prospectively collected and retrospectively analyzed. Primary outcomes were in-hospital mortality, long-term survival, and long-term freedom from reoperation. RESULTS: Median age at operation was 23 months (IQR 5-93), median weight was 11.3 kg (IQR 4.8-19.4 kg). One (2.4%) patient died within the first 30 postoperative days. In-hospital mortality was 4.9%. Four (9.8%) patients required re-exploration for bleeding, and 2 (4.9%) patients needed extracorporeal life support. Median follow-up was 11 years (IQR 11 months - 16 years). Long-term freedom from re-operation after 1, 5, 10 and 15 years was 97.1%, 93.7%, 61.8% and 42.5%, respectively. Long-term survival after 1, 5, 10 and 15 years was 89.9%, 87%, 87% and 80.8%, respectively. CONCLUSION: If MV repair is not feasible, MV replacement offers a good surgical alternative for pediatric patients with MV disease. It provides good early- and long-term outcomes.

How to fix residual mitral regurgitation due to ring detachment after valve-in-ring procedure.

Mitral valve-in-ring procedure can be an alternative to surgical valve replacement. Malposition or residual mitral regurgitation and need for a second transcatheter valve is more common in mitral valve-in-ring than in valve-in-valve procedure. This case reveals how the implantation of a second prosthesis was able to fix a significant residual mitral regurgitation due to mitral ring detachment, "closing" the free flow cage of the first prosthesis and avoiding mitral regurgitation through the ring detachment.

An Unusual Presentation of Acute Severe Mitral Regurgitation.

Mitral regurgitation (MR) results from retrograde blood flow from the left ventricle to the left atrium. Common etiologies of acute severe MR include papillary muscle rupture from myocardial infarction, leaflet perforation in infective endocarditis, chordal rupture (pop) in myxomatous valve disease, acute rheumatic fever with carditis, or functional MR due to cardiomyopathies, myocarditis or Takotsubo cardiomyopathy. Here, we present an unusual case of acute severe MR due to ruptured chordae tendineae likely secondary to degenerative valve disease. A 59-year-old male with a past medical history of hypertension and renal calculi was evaluated in the outpatient office for a urologic procedure. He was sent to the emergency room with left-sided chest pain, 6/10 in intensity, burning in nature, and non-radiating with no aggravating and relieving factors. He had nausea and vomiting for the past three days. He reported similar chest pain at rest and on exertion multiple times over the past year. He also had a chronic cough with no recent changes. His examination was unremarkable. Chest X-ray showed interstitial lung markings. Electrocardiography revealed an old right bundle branch block, but no ST/T-wave changes. He was admitted and treated for atypical pneumonia with ceftriaxone and azithromycin. The following morning, he complained of persistent chest pain 9/10 in intensity which improved with nitroglycerin. His examination revealed a new onset holosystolic murmur heard over the precordium. A two-dimensional echocardiogram showed a preserved ejection fraction of 55-60%, severe MR with eccentric jet, concerning for partially flail leaflet of the mitral valve. He was transferred to the university hospital for mitral valve replacement. Patients with acute rupture of chordae tendineae usually progress to severe mitral valve regurgitation. These patients usually present with pulmonary edema, signs of heart failure, and cardiogenic shock. Papillary muscle dysfunction, as well as partial or complete rupture of the mitral chordae can be detected as a new-onset holosystolic murmur and can be a crucial sign for early recognition. In our case, the patient developed a new holosystolic murmur on day two of admission which was recognized early, and prompt surgical intervention was performed.