Shifts in Clinical Characteristics, Treatment, and Outcome for Rheumatic Mitral Stenosis: Insights From a 20-Year Multicentre Registry Study in Korea.

BACKGROUND: The rapid economic development of South Korea provides a unique model to study changes in the clinical characteristics, treatment approaches, and clinical outcomes of patients with rheumatic mitral stenosis (MS) relative to socioeconomic growth. METHODS: From the Multicenter mitrAl STEnosis with Rheumatic etiology (MASTER) registry, 2,337 patients diagnosed with moderate or severe rheumatic MS between January 2001 and December 2020 were analyzed. Patients were grouped into consecutive 5-year intervals based on their year of diagnosis. Clinical characteristics, echocardiographic data, and clinical outcomes were assessed. RESULTS: Over 20 years, the severity of mitral stenosis increased from 79.1% to 90.2%; similarly, the average age at diagnosis increased from 54.3 to 63.0 years (all P < 0.001). Comorbidities such as hypertension and atrial fibrillation increased (6.3% to 29.5% and 41.4% to 46.9%, respectively; all P for trend < 0.05). The rate of mitral intervention within five years after diagnosis increased from 31.2% to 47.4% (P for trend < 0.001). However, clinical outcomes of rheumatic mitral stenosis deteriorated over time in the composite outcomes (log-rank test, P < 0.001). Conversely, the incidence of stroke remained stable (60.6-73.7%; P < 0.001), which might be attributed to the increased use of anticoagulation therapy. CONCLUSION: This study observed an increase in patient age, comorbidities, and valve disease severity as the country transitioned from a developing to developed status. Despite a rise in mitral valve interventions, clinical outcomes deteriorated over 20 years, highlighting the need for modified treatment approaches to improve patient outcomes.

The Spectrum, Severity and Outcomes of Rheumatic Mitral Valve Disease in Pregnant Women in Australia and New Zealand.

BACKGROUND: Rheumatic heart disease (RHD) poses significant perinatal risks. We aimed to describe the spectrum, severity and outcomes of rheumatic mitral valve disease in pregnancy in Australia and New Zealand. METHODS: A prospective, population-based cohort study of pregnant women with RHD recruited 2013-14 through the hospital-based Australasian Maternity Outcomes Surveillance System. Outcome measures included maternal and perinatal morbidity and mortality. Univariable and multivariable logistic regression analyses were undertaken to test for predictors of adverse maternal and perinatal outcomes. RESULTS: Of 274 pregnant women identified with RHD, 124 (45.3%) had mitral stenosis (MS) and 150 (54.7%) had isolated mitral regurgitation (MR). One woman with mild MS/moderate MR died. There were six (2.2%) stillbirths and two (0.7%) neonatal deaths. Babies born to women with MS were twice as likely to be small-for-gestational-age (22.7% vs 11.4%, p=0.013). In women with MS, use of cardiac medication (AOR 7.42) and having severe stenosis (AOR 16.35) were independently associated with adverse cardiac outcomes, while New York Heart Association (NYHA) class >1 (AOR 3.94) was an independent predictor of adverse perinatal events. In women with isolated MR, use of cardiac medications (AOR 7.03) and use of anticoagulants (AOR 6.05) were independently associated with adverse cardiac outcomes. CONCLUSIONS: Careful monitoring and specialist care for women with RHD in pregnancy is required, particularly for women with severe MS, those on cardiac medication, and those on anticoagulation, as these are associated with increased risk of adverse maternal cardiac outcomes. In the context of pregnancy, contraception and preconception planning are important for young women diagnosed with RHD.

An Incomplete Form of Shone's Complex With Double Orifice Mitral Valve.

We present the case of an adult male patient with an incomplete form of Shone's complex associated with bicuspid aortic valve and a double orifice mitral valve. Intraoperative inspection of the mitral valve showed double orifice configuration with a small, rudimentary left-sided mitral valve and a large, dominant, right-sided parachute mitral valve with Barlow-type of degeneration. The patient underwent reconstruction of both valves through a minimally invasive incision. At one year echocardiographic control both valves function normally.

Small Cavity of Left Ventricle Does Not Affect Short-term Outcome in Patients with Rheumatic Mitral Valve Stenosis Undergoing Mitral Valve Replacement.

BACKGROUND: Small cavity left ventricle (SCLV) may affect the clinical outcomes of patients undergoing mitral valve replacement (MVR). This study aims to investigate the incidence of SCLV in patients with rheumatic mitral valve stenosis undergoing MVR and analyze its effect on short-term patient outcomes. METHODS: We retrospectively examined all consecutive patients with isolated or concomitant MVR for rheumatic mitral valve stenosis in our center from 2013 to 2018. SCLV was defined as end-diastolic volume index ≤ 50 ml/m2. After inclusion and exclusion, a total of 1,437 patients were analyzed. The baseline information was collected and compared between SCLV and non-SCLV patients. Multivariate logistic regression analysis was conducted to determine the effect of SCLV on early mortality. RESULTS: A total of 1,437 patients were included in the study. SCLV was detected in 13.57% of the patients. Compared with the non-SCLV group, patients with SCLV were smaller-sized and primarily female. There were no significant differences between SCLV and non-SCLV patients regarding major postoperative complications, nor were there incidence of prosthesis-patient mismatch. Logistic regression analysis showed that SCLV was not a risk factor for short-term mortality (P = 0.998). CONCLUSIONS: Our results demonstrated that SCLV was not associated with poorer early outcomes after MVR surgery in patients with rheumatic mitral valve stenosis.

Mitral Stenosis After MitraClip: How to Avoid and How to Treat.

PURPOSE OF REVIEW: The goal of the paper is to highlight the importance of procedural planning and patient selection when using the MitraClip device in treating severe mitral regurgitation (MR). RECENT FINDINGS: Following the recent results of the COAPT trial and FDA approval for functional MR patients, the indications for mitral clip are continuing to expand. Because of this, mitral stenosis from mitral clip can become a problem if the appropriate patients are not selected. Proper valve imaging, utilizing 3D transesophageal echocardiography to identify the pathology, is important to prevent iatrogenic mitral stenosis. In the unfortunate event of severe mitral stenosis as a result of the MitraClip device, surgery is the only treatment.

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.

Massive hemoptysis in a pregnant woman: the first rare presentation of mitral valve stenosis.

Massive hemoptysis is one of the rare manifestations of mitral valve stenosis. Massive hemoptysis is considered as a potentially life threatening that frequently needs emergency procedures. A 43- year-old pregnant woman presented with coughing and massive hemoptysis. She was diagnosed severe rheumatic mitral stenosis. The patient underwent percutaneous transluminal mitral valvuloplasty (PTMC). Before ICU admission and hospitalization, she was healthy, without any past medical history and complaints.

First Experience of Sutureless Redo on Mitral Valve Using Valve-in-Valve Technique: Two-Stage Implantation on a Large Animal.

Sutureless implantation of the mitral valve bioprosthesis using the valve-in-valve method was performed on a large animal (sheep). According to the results of a two-stage implantation (primary implantation of a xenopericardial 26-mm framed bioprosthesis and reimplantation of the developed 23-mm bioprosthesis), minor changes in quantitative indicators were revealed: an increase in the transprosthetic gradient by 1.3 mm Hg and a decrease in the area of the mitral orifice by 21.6%. Considerable reduction in the intervention time by 18 min was achieved (by 40% in comparison with the primary prosthesis). The absence of adverse events in the animal and complications in the post-operative period, as well as physiological hemodynamic indicators indicate the safety of the developed medical device.

Chronic kidney disease and valvular heart disease: conclusions from a Kidney Disease: Improving Global Outcomes (KDIGO) Controversies Conference.

Chronic kidney disease (CKD) is a major risk factor for valvular heart disease (VHD). Mitral annular and aortic valve calcifications are highly prevalent in CKD patients and commonly lead to valvular stenosis and regurgitation, as well as complications including conduction system abnormalities and endocarditis. VHD, especially mitral regurgitation and aortic stenosis, is associated with significantly reduced survival among CKD patients. Knowledge related to VHD in the general population is not always applicable to CKD patients because the pathophysiology may be different, and CKD patients have a high prevalence of comorbid conditions and elevated risk for periprocedural complications and mortality. This Kidney Disease: Improving Global Outcomes (KDIGO) review of CKD and VHD seeks to improve understanding of the epidemiology, pathophysiology, diagnosis, and treatment of VHD in CKD by summarizing knowledge gaps, areas of controversy, and priorities for research.

Evaluation of the severity of mitral stenosis in patient with pulmonary hypertension: Role of exercise hemodynamics.

Dyspnea due to mitral stenosis (MS) and/or pulmonary hypertension (PHT) in patients who may have both conditions presents a challenging diagnostic dilemma. The hemodynamic response to exercise is probably the most helpful method to arrive at the correct diagnosis and treatment algorithm. In this hemodynamic rounds discussion, we evaluated a patient with MS and PHT prior to the decision for mitral valvuloplasty. KEY POINTS: Symptoms in patients with mitral stenosis and pulmonary hypertension cannot always be attributed to one pathology. Catheter-based hemodynamics are often needed to differentiate valve from lung disease. Exercise hemodynamics can clarify the predominant pathology in complex clinical scenarios.

Measurement of mitral valve area in patients with mitral stenosis by 3D echocardiography: A comparison between direct planimetry on 3D zoom and 3D quantification.

BACKGROUND: Measurement of the mitral valve area (MVA) in patients with mitral stenosis (MS) by 3D echocardiography (3DE) is usually done via 3D quantification (3DQ). The present study on patients with severe MS sought to evaluate the agreement regarding the MVA measurement between 3DQ and direct planimetry on 3D zoom and also between 3DE and 2DE. METHODS: Twenty-six patients (22 female, mean age:34.5 ± 14.0 years) with severe MS diagnosed by 2D transthoracic echocardiography(2DTTE) underwent 3D transesophageal echocardiography (3DTEE). Direct planimetry, the pressure half-time (PTH), and the continuity equation(CE) constituted 3 conventional 2DTTE methods, and 3DQ and direct planimetry on 3D zoom comprised two 3DTEE methods applied for the MVA measurement. Agreement between the 2D and 3D methods was assessed using the Bland-Altman plot and measuring the intra-class correlation coefficient (ICC). RESULTS: The mean MVA measured by 3DQ was significantly larger than that derived by direct planimetry on 3D zoom (0.935 ± 0.23 cm2 vs 0.846 ± 0.22 cm2 , respectively; P = 0.026). The agreement between 3DQ and 3D zoom for the MVA measurement was moderate to good by the Bland-Altman plot (ICC = 0.67). The mean MVA measured by 2DE (all 3 methods of direct planimetry, the PTH, and the CE) was significantly larger than that derived by 3DE (both methods of 3DQ and direct planimetry on 3D zoom) (all Ps < 0.05). A moderate agreement between 3DQ and 2D planimetry (ICC = 0.43) was found by the Bland-Altman plot. CONCLUSIONS: The MVA measurement by direct planimetry on 3D zoom showed a moderate-to-good agreement with 3DQ; it may, thus, be used in clinical practice as a simple method for the measurement of the MVA in patients with MS.

The strange case of congenital mitral stenosis in an adult man with cor triatriatum.

We report the case of a 74-year-old male, with a medical history of cor triatriatum, admitted with a 10-day history of intermittent fever. Three sets of blood cultures were positive for Providencia rettgeri. Transthoracic and transesophageal echocardiogram excluded infective endocarditis, but revealed a congenital accessory tissue adhering to the mitral valve, causing supravalvular mitral stenosis. Cor triatriatum sinistrum is a rare congenital cardiac anomaly, even more uncommon in adults, and quite exceptional when associated with mitral valve disease. Because the patient had no symptoms related to the heart valve disease, no surgical indication was given and he was managed conservatively.

Level of agreement in three-dimensional planimetric measurement of mitral valve area between transthoracic and transesophageal echocardiography.

BACKGROUND: Echocardiographic measurement of mitral valve area (MVA) is critical prior to percutaneous transmitral commissurotomy (PTMC). This study aimed to compare the agreement between transthoracic (TTE) and transesophageal echocardiography (TEE) in three-dimensional (3D) planimetric measurement of the MVA among patients with severe mitral stenosis. METHODS: MVA was measured with planimetry in 105 patients before undergoing PTMC. 3D reconstruction was applied to both TTE and TEE examinations. The MVA values from four different methods of 3D reconstruction were compared to the average values of 3D methods in TEE as the gold standard measurement method for the MVA in this study. The agreement levels between the two examinations were evaluated and analyzed for various reconstruction methods. RESULTS: The mean age was 49 ± 12 years for 28 men and 77 women who were enrolled. The image quality was graded as "excellent" in 57% of 3D images obtained by TTE, while it was graded as "excellent" in 81% of 3D images obtained by TEE. The ventricular zooming method in TTE with a bias of -0.006 ± 0.065 cm2 (P < 0.0001) had the highest agreement with the 3D-MVA in TEE. While 2D-TTE and 3D-TEE measurements of the MVA (R = 0.91; P < 0.0001) were significantly correlated, 2D-TTE overestimated the MVA by 0.19 cm2 . CONCLUSION: Although the quality of 3D images was significantly better in TEE than those in TTE, a good agreement existed between the measured 3D-TTE and 3D-TEE studies. We also demonstrated that 2D-TTE overestimated the MVA compare to 3D-TEE.

Inverted Aortic Prosthesis in the Mitral Position: Is Upside Down Always the Right Side Up?

Evaluation of prosthetic valve function is a challenging task. The clinician has to employ multiple parameters to quantify dysfunction (if present), the results of which can be mutually discrepant. This results from heterogeneity in the design of the valves themselves, implantation techniques, and both intra- and interpatient hemodynamic variability. Specifically, the location and angle of valve implantation can have a profound impact on its flow characteristics that can lead to symptoms despite satisfactory mechanical function. The authors present the case of inverted implantation of a prosthesis designed for the aortic position in the mitral annulus and resultant mitral stenosis. What follows is an examination of how the flow characteristics, such as pressure recovery, energy loss, and vortex formation, create a gradient that could not be explained by valve size alone.

Rheumatic Mitral Valve Stenosis: Diagnosis and Treatment Options.

PURPOSE OF REVIEW: This review provides an update on rheumatic mitral stenosis. Acute rheumatic fever (RF), the sequela of group A ß-hemolytic streptococcal infection, is the major etiology for mitral stenosis (MS). RECENT FINDINGS: While the incidence of acute RF in the Western world had substantially declined over the past five decades, this trend is reversing due to immigration from non-industrialized countries where rheumatic heart disease (RHD) is higher. Pre-procedural evaluation for treatment of MS using a multimodality approach with 2D and 3D transthoracic and transesophageal echo, stress echo, cardiac CT scanning, and cardiac MRI as well as hemodynamic assessment by cardiac catheterization is discussed. The current methods of percutaneous mitral balloon commissurotomy (PMBC) and surgery are also discussed. New data on long-term follow-up after PMBC is also presented. For severe rheumatic MS, medical therapy is ineffective and definitive therapy entails PMBC in patients with suitable morphological mitral valve (MV) characteristics, or surgery. As procedural outcomes depend heavily on appropriate case selection, definitive imaging and interpretation are crucial. It is also important to understand the indications as well as morphological MV characteristics to identify the appropriate treatment with PMBC or surgery.

Rapidly Growing Intra-Cardiac Mass Mimicking a Local Recurrence of Lung Cancer or a Thrombus in the Left Atrium.

Cardiac myxoma typically is thought to be a slow-growing, benign primary. Atrial myxomas can lead to many complications and can also mimic mitral stenosis, infective endocarditis, and other vascular diseases associated with systemic embolization. A 75-year-old woman with a history of lung cancer (pT1cN1, adenocarcinoma), atrial fibrillation, and a cerebral infarction presented with dysarthria and visual disturbances. In our case, we had to consider some questionable issues with the left atrial mass, and whether the recurrence of cerebral events was due to the thrombotic material in the left atrium or from locally recurrent lung cancer from the stump margin of the previously resected left superior pulmonary vein. We present a case with a rapidly-growing left atrial myxoma with a growth rate of 12.60 mm/month, rather than a thrombus or local recurrence of tumor under a medication of non-VKA oral antagonists.

Maternal and Neonatal Complications of Pregnant Women with Mitral Stenosis.

BACKGROUND: Rheumatic mitral stenosis (MS) is a relatively rare diagnosis in the developed countries and its treatment during pregnancy is challenging due to hemodynamic changes. With the demographic changes due to recent waves of immigration an increase in the prevalence of rheumatic heart disease is expected. OBJECTIVES: To evaluate maternal and neonatal complications in patients with mitral stenosis. METHODS: During the years 2006-2017, 22 women who underwent 31 pregnancies were followed at the Sheba Medical Center in Israel. We collected on regarding hemodynamic changes and their clinical course. MS was classified as mild, moderate, or severe according to mitral valve area by echocardiography. Maternal and fetal adverse events were evaluated according to severity of MS and compared by Poisson regression modeling. RESULTS: MS was severe in 7 pregnancies (22.6%), moderate in 9 (29%), and mild in 15 (48.4%). Twenty patients were managed conservatively and 2 underwent a successful percutaneous mitral balloon valvuloplasty (PBMVP) during pregnancy. All pregnancies ended with a liveborn neonate and with no maternal mortality. Peak and mean mitral pressure gradients increased during pregnancy from 13.3 ± 5.3 to 18.6 ± 5.1 mmHg and from 5.9 ± 2.3 to 9.6 ± 3.4 mmHg respectively (P < 0.05). Eight pregnancies (25.8%) were complicated by pulmonary congestion, 2/15 (13.3%) with mild MS, 2/9 (22.2%) with moderate, and 4/7 (57.1%) with severe MS. The adverse event rate was higher among patients with severe MS compared with moderate and mild MS [hazard ratio (HR) 3.15, 95% confidence interval (95%CA) 1.04-9.52 and HR 4.06, 95%CI 1.4-11.19 respectively, P < 0.05]. Nine of 31 deliveries were vaginal; 6 of 22 cesarean sections (27.3%) were performed for cardiac indications. CONCLUSIONS: The number of total adverse events were higher among patients with severe MS. Patients with moderate and mild MS should be treated attentively, but good obstetric and maternal outcome can be expected.

Use of Transcatheter Valves for Open Surgical Mitral Valve Replacement. Australian Experience.

BACKGROUND: Mitral valve procedures remain a surgical challenge in the presence of extensive annular calcification, which presents a formidable technical challenge. Aggressive debridement is limited by risk of serious complications and the technical complexity of pericardial patch reconstruction of the debrided area. METHODS: An open surgical approach with a transcatheter valve allows the valve to be placed under direct visualisation to facilitate positioning and to evaluate the likelihood of both perivalvular leakage and atrioventricular disruption. The open approach has the additional advantage of performing concomitant surgeries like other valve procedures, arrhythmias surgeries and coronary bypass. RESULTS: We present our experience with open surgical mitral valve replacement (MVR) using transcatheter valve in different patients requiring varied procedures. These patients were not suitable for MVR using standard prosthetic valve and techniques. They were also not suitable for percutaneous MVR because of heavily calcified anterior mitral leaflet and the other concomitant procedures required. CONCLUSIONS: Open MVR with a transcatheter balloon-expandable valve can avoid the need for technically challenging and high-risk decalcification of mitral annulus. These novel techniques using transcatheter valves can be successful in complex cases where standard prosthetic valves are impossible to implant in a heavily calcified mitral annulus.

Novel Technique of Transfemoral Venous Pacing in a Rare Case of Inferior Vena Cava Stenosis.

Temporary transvenous pacing is a simple and routinely performed invasive procedure for treatment of life threatening bradyarrhythmias. We present a novel technique for transfemoral venous pacing in a patient with rare co-occurrence of inferior vena cava stenosis, rheumatic mitral stenosis, left ventricular dysfunction and digitoxicity.

The impact of mitral stenosis on outcomes of aortic valve stenosis patient undergoing surgical aortic valve replacement or transcatheter aortic valve replacement.

BACKGROUND: The concomitant presence of mitral stenosis (MS) in the setting of symptomatic aortic stenosis represent a clinical challenge. Little is known regarding the outcome of mitral stenosis (MS) patients undergoing transcatheter aortic valve replacement (TAVR) or surgical aortic valve replacement (SAVR). Therefore, we sought to study the outcome of MS patients undergoing aortic valve replacement (AVR). METHOD: Using weighted data from the National Inpatient Sample (NIS) database between 2011 and 2014, we identified patients who were diagnosed with MS. Patients who had undergone TAVR as a primary procedure were identified and compared to patients who had SAVR. Univariate and multivariate logistic regression analysis were performed for the outcomes of in-hospital mortality, length of stay (LOS), blood transfusion, postprocedural hemorrhage, vascular, cardiac and respiratory complications, permanent pacemaker placement (PPM), postprocedural stroke, acute kidney injury (AKI), and discharge to an outside facility. RESULTS: A total of 4524 patients were diagnosed with MS, of which 552 (12.2%) had TAVR and 3972 (87.8%) had SAVR. TAVR patients were older (79.9 vs 70.0) with more females (67.4% vs 60.0%) and African American patients (7.7% vs 7.1%) (P < 0.001). In addition, the TAVR group had more comorbidities compared to SAVR in term of coronary artery disease (CAD), congestive heart failure (CHF), chronic lung disease, hypertension (HTN), chronic kidney disease (CKD), and peripheral vascular disease (PVD) (P < 0.001 for all). Using Multivariate logistic regression, and after adjusting for potential risk factors, TAVR patients had lower in-hospital mortality (7.9% vs 8.1% adjusted Odds Ratio [aOR], 0.615; 95% confidence interval [CI], 0.392-0.964, P = 0.034), shorter LOS. Also, TAVR patients had lower rates of cardiac and respiratory complications, PPM, AKI, and discharge to an outside facility compared with the SAVR group. CONCLUSION: In patients with severe aortic stenosis and concomitant mitral stenosis, TAVR is a safe and attractive option for patients undergoing AVR with less complications compared with SAVR.