Left Atrial Enlargement as a Predictor of Atrial Fibrillation in Rheumatic Mitral Valve Disease: An Echocardiography-based Retrospective Cross-sectional Study.

BACKGROUND: Rheumatic heart disease is a major cause of cardiovascular morbidity and mortality in developing nations and is a leading cause of hospital admission due to cardiac problems in our country. This study will evaluate the association between left atrial size and the occurrence of atrial fibrillation and describe the clinical characteristics along with complications related to Rheumatic Mitral valve disease. METHODS: A retrospective cross-sectional study was conducted at a tertiary care center from January 2018 to December 2019. Reports of 207 patients admitted to medical and/or surgical wards with echocardiographic diagnosis of rheumatic mitral valve disease with or without atrial fibrillation were reviewed. Data were collected, entered, and analyzed using the Statistical Package for the Social Science version 25.0. RESULTS: Among 207 patients, atrial fibrillation was present in 90 (43.5%) patients. Atrial fibrillation was higher in patients with mixed mitral valvular lesions compared to isolated mitral stenosis or mitral regurgitation. Univariate and multivariate analysis revealed left atrial size [aOR=1.067, 95% CI: 1.023 - 1.113, P= 0.002] and age [aOR = 1.073, 95% CI: 1.042 - 1.105, P<0.001] as an independent predictor of atrial fibrillation. CONCLUSIONS: Larger left atrium was an independent predictor of atrial fibrillation. Besides this, atrial fibrillation was associated with increasing age, mixed mitral valvular lesion, and moderately reduced left ventricular ejection fraction, but not associated with gender and mitral stenosis severity. Left atrial clot was significantly higher in patients with atrial fibrillation than in sinus rhythm.

Camptodactyly-arthropathy-coxa vara-pericarditis syndrome and an unusual association with mitral stenosis.

BACKGROUND: Campotodactyly-artrhropathy-coxa vara-pericarditis (CACP) syndrome is a very rare autosomal recessive genetic disorder. It is characterized by flexion contracture of the fifth finger (camptodactyly); noninflammatory arthropathy; decreased angle between the shaft and the head of the femur (coxa vara) and pericarditis. Its association with mitral stenosis has not yet been reported. Hereby we report this unique association with CACP syndrome. CASE: An eleven-year-old girl presented with non-productive cough, dyspnea, and orthopnea. She was diagnosed CACP syndrome at the age of seven and a biallelic frameshift mutation in the PRG4 gene was determined. The physical examination revealed pectus excavatum, camptodactyly, genu valgum, tachypnea and orthopnea. The functional capacity was NYHA III-IV. She had 2/6 soft pansystolic murmur at 4th left intercostal space and a rumbling diastolic murmur at apex. Echocardiography revealed an enlarged left atrium, severe stenotic mitral valve with a mean diastolic transmitral gradient of 22.5 mmHg, mild mitral regurgitation and mild apical pericardial effusion. The patient had mitral comissurotomy and partial pericardiectomy operation. Her post-operative transmitral gradient decreased to 6.9 mmHg and the pulmonary pressure was 30 mmHg. Her functional capacity increased to NYHA I-II. CONCLUSIONS: The main defect is the proteoglycan 4 protein which acts like a lubricant in articular and visceral surfaces. Therefore, the leading clinical feature is arthropathy. Cardiac involvement other than clinically mild pericarditis is not usually expected. Three types of proteoglycans (decorin, biglycan, and versican) are present in the mitral valve. This could be the reason of mitral valve involvement in rare cases as like ours. It is important that these patients undergo echocardiographic examination regularly.

Differential Clubbing and a Triad of Patent Ductus Arteriosus, Ventricular Septal Defect and Supravalvular Ring Mitral Stenosis: A Case Report.

It is not uncommon for congenital heart defects to occur in clusters. Those involving a right to left heart shunt commonly cause cyanosis and finger clubbing. Differential clubbing involving only the lower limb digits is a strong pointer to the presence of patent ductus arteriosus with reversal of shunt. We report a case of 25-year-old man with effort intolerance and differential clubbing. He was found to have the uncommon triad of patent ductus arteriosus, ventricular septal defect and supravalvular ring mitral stenosis. The presence of differential clubbing on a background of patent ductus arteriosus usually indicates a reversal of shunt and negates surgical intervention. This general rule may however not apply with co-existing mitral stenosis as the elevated pulmonary pressure may be predominantly post-capillary. The finding of mitral stenosis in a patient with patent ductus arteriosus and differential limb clubbing may signify a good prognostic surgical outcome.

Il n'est pas rare que des malformations cardiaques congénitales surviennent en clusters. Celles impliquant un shunt cardiaque droitegauche provoquent souvent une cyanose et un hippocratisme digital. L'hippocratisme digital différentiel touchant uniquement les orteils des membres inférieurs est un indicateur fort de la présence d'un canal artériel persistant avec inversion du shunt. Nous rapportons le cas d'un homme de 25 ans présentant une intolérance à l'effort et un hippocratisme digital différentiel. Il a été diagnostiqué avec la triade peu commune de canal artériel persistant, de communication interventriculaire et de sténose mitrale à anneau supravalvulaire. La présence d'un hippocratisme digital différentiel sur un fond de canal artériel persistant indique généralement une inversion du shunt et exclut une intervention chirurgicale. Cependant, cette règle générale peut ne pas s'appliquer en présence d'une sténose mitrale concomitante, car la pression pulmonaire élevée peut être principalement post-capillaire. La découverte d'une sténose mitrale chez un patient atteint de canal artériel persistant et d'un hippocratisme digital différentiel peut indiquer un bon pronostic pour l'intervention chirurgicale.

Mitral Annulus Calcium Score in Patients With Calcific Mitral Stenosis Undergoing Invasive Hemodynamic Assessment.

BACKGROUND: Aortic valve calcium score is associated with hemodynamic severity of aortic stenosis. Whether this association is present in calcific mitral stenosis remains unknown. METHODS AND RESULTS: This study was a retrospective analysis of consecutive patients with mitral stenosis secondary to mitral annular calcification (MAC) undergoing transseptal catheterization. All patients underwent invasive mitral valve assessment via direct left atrial and left ventricular pressure measurement. Computed tomography within 1 year of cardiac catheterization and with adequate visualization of the mitral annulus was included. MAC calcium score quantification by Agatston method was obtained offline using dedicated software (Aquarius, TeraRecon, V.4). Median patient age was 66.9±11.2 years, 47% of patients were women, 50% had coronary artery disease, 40% had atrial fibrillation, 47% had prior cardiac surgery, and 33% had prior chest radiation. Median diastolic mitral valve gradient was 9.4±3.4 mm Hg on echocardiography and 8.5±4 mm Hg invasively. Invasive median mitral valve area using the Gorlin formula was 1.87±0.9 cm2. Median MAC calcium score for the cohort was 7280±7937 Hounsfield units. MAC calcium score correlated with the presence of atrial fibrillation (P=0.02) but was not associated with other comorbidities. There was no correlation between MAC calcium score and mitral valve area (r=0.07; P=0.6) or mitral valve gradient (r=-0.03; P=0.8). CONCLUSIONS: MAC calcium score did not correlate with invasively measured mitral valve gradient and mitral valve area in patients with MAC-related mitral stenosis, suggesting that calcium score should not be used as a surrogate for invasive hemodynamic parameters.

Role of 3D transoesophageal echocardiography in correct diagnosis of congenital isolated double orifice mitral valve in a young adult misdiagnosed as rheumatic heart disease.

Double orifice mitral valve (DOMV) is an extremely rare congenital anomaly of the mitral valve (MV) wherein the MV orifice divides into two separate orifices by an accessory fibrous band.Isolated DOMV is a rarity and is often discovered incidentally. It may be associated with other congenital conditions wherein it is identified in early childhood. Its prevalence and prognostic relevance in adulthood remain unclear. DOMV patients may be asymptomatic or have symptoms due to mitral stenosis or regurgitation. We present a case of an asymptomatic young adult initially diagnosed with rheumatic mitral stenosis. However, after a thorough echocardiographic assessment, including three-dimensional transesophageal echocardiography, the accurate diagnosis of DOMV was made.

Is Concomitant Mitral Stenosis Associated With Worse Outcomes in Patients Who Underwent TAVR? Insights from a National Database.

Concomitant mitral stenosis (MS) is present in 10% to 15% of all patients who underwent transcatheter aortic valve replacement (TAVR). Our aim is to assess outcomes of TAVR in patients with MS using a national database. The Nationwide Inpatient Sample database was used to identify patients who underwent TAVR from 2015 to 2020. We created 2 groups, patients with and those without MS. We then compared baseline characteristics, demographics, and in-hospital outcomes of the groups. Primary outcomes were in-hospital mortality, acute respiratory failure, and pacemaker placement. Secondary outcomes were length of stay and in-hospital costs. Our study indicates that patients with MS had greater incidence of acute respiratory failure (8.8% vs 4.89%, p = 0.001), complete heart block (13.54% vs 9.36%, p = 0.01), and permanent pacemaker placement (8.03% vs 6.03%, p = 0.05). In-hospital mortality was greater in the MS group; however, it was not statistically significant (1.32% vs 1.53%, p = 0.679).

Hemodynamic implications of mitral annular calcification in patients undergoing transcatheter aortic valve implantation for severe aortic stenosis.

PURPOSES: Predicting hemodynamic changes of stenotic mitral valve (MV) lesions with mitral annular calcification (MAC) following transcatheter aortic valve implantation (TAVI) may inform clinical decision-making. This study aimed to investigate the association between the MAC severity quantified by computed tomography (CT) and changes in mean transmitral gradient (mTMG), mitral valve area (MVA) and stroke volume index (SVi) following TAVI. METHODS AND RESULTS: A total of 708 patients (median age 81, 52% male) with severe aortic stenosis (AS) underwent pre-procedural CT and pre- and post-TAVI transthoracic echocardiography. According to the classification of MAC severity determined by CT, 299 (42.2%) patients had no MAC, 229 (32.3%) mild MAC, 102 (14.4%) moderate MAC, and 78 (11.0%) severe MAC. After adjusting for age and sex, there was no significant change in mTMG following TAVI (Δ mTMG = 0.07 mmHg, 95% CI -0.10 to 0.23, P = 0.92) for patients with no MAC. In contrast, patients with mild MAC (Δ mTMG = 0.21 mmHg, 95% CI 0.01 to 0.40, P = 0.018), moderate MAC (Δ mTMG = 0.31 mmHg, 95% CI 0.02 to 0.60, P = 0.019) and severe MAC (Δ mTMG = 0.43 mmHg, 95% CI 0.10 to 0.76, P = 0.0012) had significant increases in mTMG following TAVI, with greater changes associated with increasing MAC severity. In contrast, there was no significant change in MVA or SVi following TAVI. CONCLUSION: In patients with severe AS undergoing TAVI, MAC severity was associated with greater increases in post-procedural mTMG whereas MVA or SVi remained unchanged. MAC severity should be considered for potential subsequent MV interventions if TAVI does not improve symptoms.

Predicting stroke and mortality in mitral stenosis with atrial flutter: A machine learning approach.

BACKGROUND: Our study hypothesized that an intelligent gradient boosting machine (GBM) model can predict cerebrovascular events and all-cause mortality in mitral stenosis (MS) with atrial flutter (AFL) by recognizing comorbidities, electrocardiographic and echocardiographic parameters. METHODS: The machine learning model was used as a statistical analyzer in recognizing the key risk factors and high-risk features with either outcome of cerebrovascular events or mortality. RESULTS: A total of 2184 patients with their chart data and imaging studies were included and the GBM analysis demonstrated mitral valve area (MVA), right ventricular systolic pressure, pulmonary artery pressure (PAP), left ventricular ejection fraction (LVEF), New York Heart Association (NYHA) class, and surgery as the most significant predictors of transient ischemic attack (TIA/stroke). MVA, PAP, LVEF, creatinine, hemoglobin, and diastolic blood pressure were predictors for all-cause mortality. CONCLUSION: The GBM model assimilates clinical data from all diagnostic modalities and significantly improves risk prediction performance and identification of key variables for the outcome of MS with AFL.

One-year outcome of transcatheter aortic valve replacement for bicuspid aortic valve stenosis: a report from the Japanese Nationwide registry (J-TVT registry).

The outcome of transcatheter aortic valve replacement (TAVR) for patients with bicuspid aortic valve (BAV) remains unclear, particularly among Asian patients that are known to have different valvular morphology and lower body habitus. This study investigated patient characteristics, procedural and 1-year outcome of TAVR for BAV within national TAVR registry in Japan. The patient-level data were extracted from the J-TVT (Japanese Transcatheter Valvular Therapy) registry between August 2013 and December 2018; overall, there were 423 patients (2.5%) with BAV and 16,802 patients with tricuspid aortic valve (TAV). At baseline, patients with BAV were younger and had less arteriosclerotic comorbidities. There was no statistically significant difference between BAV and TAV groups in conversion to surgery (0.5% vs. 1.1%, p = 0.34) and 30-day mortality (0.5% vs. 1.3%, p = 0.18). Cumulative all-cause survival and survival from major adverse events were analyzed. Cox proportional hazard regression model was used to estimate the hazard ratio. All-cause mortality and major adverse event rate at 1 year were comparable between the two groups. Relative hazard for all-cause mortality for BAV compared to TAV was 1.01 (0.70-1.45; p = 0.96), and for major adverse event was 0.94 (0.69-1.27; p = 0.67). From the Japanese nationwide TAVR registry, procedural and 1-year outcome of TAVR in BAV was as favorable as TAVR in TAV.

Outcomes of Cerebral Embolic Protection for Bicuspid Aortic Valve Stenosis Undergoing Transcatheter Aortic Valve Replacement.

Background There was limited high-quality evidence that illuminated the efficiency of cerebral embolic protection (CEP) use during transcatheter aortic valve replacement (TAVR) for bicuspid aortic valve (BAV) stenosis. Methods and Results In this retrospective cohort study, patients with BAV stenosis undergoing TAVR with or without CEP were identified by querying the National Inpatient Sample database. The primary end point was any stroke during the hospitalization. The composite safety end point included any in-hospital death and stroke. We applied propensity score-matched analysis to minimize standardized mean differences of baseline variables and compare in-hospital outcomes. From July 2017 to December 2020, 4610 weighted hospitalizations with BAV stenosis undergoing TAVR were identified, of which 795 were treated with CEP. There was a significant increase in the CEP use rate for BAV stenosis (P-trend <0.001). A total of 795 discharges with CEP use were propensity score matched to 1590 comparable discharges but without CEP. CEP use was associated with a lower incidence of in-hospital stroke (1.3% versus 3.8%; P<0.001), which in multivariable regression was also independently associated with the primary outcome (adjusted odds ratio=0.38 [95% CI, 0.18-0.71]; P=0.005) and the safety end point (adjusted odds ratio=0.41 [95% CI, 0.22-0.68] P=0.001). Meanwhile, no significant difference was found in the cost of hospitalization ($46 629 versus $45 147; P=0.18) or the risk of vascular complications (1.9% versus 2.5%; P=0.41). Conclusions This observational study supported CEP use for BAV stenosis, which was independently associated with less in-hospital stroke without burdening the patients with a high hospitalization cost.

Left atrial strain predicts improvement in left atrial functions of severe rheumatic mitral stenoses undergoing successful percutaneous transmitral commissurotomy.

AIMS: Chronic rheumatic heart disease (RHD) is prevalent in India. The mitral valve in isolation or combination with the aortic or tricuspid valve is involved in 31.6% and 52.8% of chronic RHD patients, respectively. The left atrium (LA) functions as a reservoir during the cardiac cycle. Therefore, the LA enlargement leads to longitudinal lengthening, measured as a positive strain, permitting the measurement of the longitudinal strain of LA. This study aimed to assess the LA functions using peak atrial longitudinal strain (PALS) in patients with severe rheumatic mitral stenoses (MS) in sinus rhythm who underwent successful percutaneous transvenous mitral commissurotomy (PTMC). MATERIAL AND METHODS: We recruited 56 patients with severe rheumatic MS for the study, of which 06 PTMC procedures were considered unsuccessful. So, 50 patients of chronic severe rheumatic MS in sinus rhythm undergoing PTMC were enrolled in a tertiary care centre of the Armed Forces from August 2017 to May 2019. Patients included in the study were not consecutive, and patients with atrial fibrillation (AF) were excluded. RESULTS: PALS improved post-PTMC (P < .001) in this study, effectively concluding that PALS is impaired in patients with severe symptomatic MS and is acutely enhanced after treatment. CONCLUSIONS: PALS is a good indicator of LA function and may predict the success of PTMC on the rheumatic mitral valve.

What Is the Optimal Mitral Valve Repair for Isolated Posterior Leaflet Prolapse to Achieve Long-Term Durability?

Background This study assessed risk factors for mitral regurgitation (MR) recurrence or functional mitral stenosis during long-term follow-up in patients undergoing mitral valve repair for isolated posterior mitral leaflet prolapse. Methods and Results We assessed a consecutive series of 511 patients who underwent primary mitral valve repair for isolated posterior leaflet prolapse between 2001 and 2021. Annuloplasty using a partial band was selected in 86.3%. The leaflet resection technique was used in 83.0%, whereas the chordal replacement without resection was used in 14.5%. Risk factors were analyzed for MR recurrence ≥grade 2 or functional mitral stenosis with mean transmitral pressure gradient ≥5 mm Hg using a multivariable Fine-Gray regression model. The 1-, 5-, and 10-year cumulative incidence of MR ≥grade 2 was 7.8%, 22.7%, and 30.1%, respectively, whereas that of mean transmitral pressure gradient ≥5 mm Hg was 8.1%, 20.6%, and 29.3%, respectively. Risk factors for MR ≥grade 2 included chordal replacement without resection (hazard ratio [HR], 2.50, P<0.001) and larger prosthesis size (HR, 1.13, P=0.023), whereas factors for functional mitral stenosis were use of a full ring (partial band versus full ring, HR, 0.53, P=0.013), smaller prosthesis size (HR, 0.74, P<0.001), and larger body surface area (HR, 3.03, P=0.045). Both MR ≥grade 2 and mean transmitral pressure gradient ≥5 mm Hg at 1 year post surgery were significantly associated with the long-term incidence of reoperation. Conclusions Leaflet resection with a large partial band may be an optimal strategy for isolated posterior mitral valve prolapse.

A rare coexistence: Hammock mitral valve and aortopulmonary window.

Congenital mitral stenosis is a broad-spectrum pathology in which blood flow to the left ventricle is obstructed both functionally and anatomically. Hammock mitral valve, also known as anomalous mitral arcade, is a rare congenital anomaly particularly in infants and children. Hammock mitral valve may not be suitable for repair regarding the advanced dysplastic mitral valve structure. Aortopulmonary window is an unusual cardiac anomaly which is defined as a communication between the main pulmonary artery and the ascending aorta. As a result of the excessive left-to-right shunt, early intervention and surgical closure deemed mandatory to avoid development of severe pulmonary hypertension and its consequences. All patients with an aortopulmonary window necessitates prompt repair immediately. In this brief report, mitral valve replacement with a mechanical valve and repair of aortopulmonary window with a Dacron patch were performed simultaneously in a 5-month-old patient with a hammock mitral valve and accompanying aortopulmonary window.

Surgical treatment of caseous calcification of the mitral annulus causing mitral stenosis and mimicking the clinical picture of infective endocarditis: A case report.

Among masses involving the mitral valve and annulus, caseous calcification of the mitral annulus (CCMA) is a rare disease. CCMA accounts for .63% of all mitral annular calcification (MAC) cases. The pathophysiology is still unknown. The correct diagnosis and treatment of this disease is very important to prevent complications. We present a case of giant CCMA with advanced mitral stenosis and hypertrophic cardiomyopathy, presenting with symptoms of infection and therefore a preliminary diagnosis of infective endocarditis. Because of these features, we wanted to share our case as it is the 1st case in the literature.

Predictors of mitral valve haemodynamics after mitral valve repair for degenerative mitral regurgitation.

OBJECTIVE: Intraoperative predictors of functional mitral valve (MV) stenosis after surgical repair of mitral regurgitation (MR) caused by prolapse remain poorly characterised. This study evaluated the effect of annuloplasty size on postoperative MV haemodynamics during exercise and evaluated predictors of MV hemodynamics. METHODS: 104 patients were randomly assigned to leaflet resection or preservation for surgical repair of MR in the Canadian Mitral Research Alliance CardioLink-2 study. In this post hoc analysis, we compared MV haemodynamics between the two surgical groups and examined the relationship between annuloplasty size and MV haemodynamics 1 year after repair in the combined groups. Echocardiograms were performed at baseline and intraoperatively. Exercise transthoracic echocardiography was performed 1 year postoperatively. Multivariable linear regression analysis was used to identify predictors of exercise MV gradients at follow-up. RESULTS: Mean age of participants was 65±10 years, and 83% were male. Median annuloplasty size was 34 (IQR 32-36). Dividing by the median, 48 (46%) had annuloplasty size of <34 mm and 56 (54%) had ≥34 mm. Mean and peak exercise gradients at 1 year were 11±5 mm Hg and 22±9 mm Hg in <34, and 6±3 mm Hg and 14±5 mm Hg in ≥34 (p<0.001). Rate of residual MR was similar in both groups. In multivariable analyses, annuloplasty size of ≥34 mm was associated with lower mean and peak exercise gradients at 12 months, after adjustment for repair type, age, sex, heart rate and body surface area (ß -4.1, 95% CI -6 to -3, p<0.001, and ß -7 95% CI -10 to -4, p<0.001, respectively). Intraoperative mean and peak MV gradients by transesophageal echocardiography independently predicted mean and peak resting and exercise gradients at follow-up (p<0.001). Similar results were obtained in both leaflet resection and preservation. CONCLUSION: Annuloplasty size of ≥34 mm is associated with a 4 and 7 mm Hg reduction in mean and peak exercise MV gradients, respectively, 1 year post MV repair regardless of the repair strategy used. Intraoperative TEE MV gradients predict exercise MV gradients 1 year post repair. TRIAL REGISTRATION NUMBER: NCT02552771.

Concomitant transcatheter transfemoral double native valve replacement for severe aortic stenosis and severe mitral stenosis with mitral annular calcification.

Concomitant mitral and aortic valve stenosis in a patient with mitral annular calcification and porcelain aorta poses a unique problem to the surgical team. Transcatheter aortic and mitral valve replacements in native valves offer a viable option for such selected group of patients. We present the case of a 54-year-old male who presented with severe aortic stenosis (AS) and severe mitral stenosis (MS) but was deemed high risk for surgery owing to intense calcification of the aorta and mitral annular calcification, and successfully underwent transcatheter double native valve replacement.

Mitral Valve Replacement for Mitral Stenosis Secondary to Massive Mitral Annular Calcium.

Mitral annular calcium (MAC) may produce mitral stenosis (MS) if its quantity is massive. We define massive MAC as the presence of a huge quantity of calcium underlying the posterior mitral leaflet and extending across all or nearly all of the ventricular aspect of the anterior mitral leaflet. This report was prompted to emphasize the hazards of performing mitral valve replacement in patients with MS secondary to massive MAC. The clinical data and morphology of the operatively excised mitral valves from the 11 patients who had mitral valve replacement for MS secondary to massive MAC are described. Of the 11 patients, 6 died postoperatively, 5 of whom had 4+/4+ MAC. The high mortality in these patients suggests that the decision to perform mitral valve replacement needs to be carefully considered if the quantity of MAC is massive.