Multimodality Imaging of Intimal Sarcoma Causing Both Severe Mitral Stenosis and Mitral Regurgitation.

Intimal sarcomas (IS) are rare, malignant, rapidly progressive mesenchymal tumors that typically occur in the tunica intima of larger vessels, and they rarely involve the heart. IS are frequently misdiagnosed during the initial clinical presentation. This case report describes an uncommonly located IS, highlighting specific findings obtained through multimodality imaging.

Optimal patient selection for maze procedure in patients undergoing mitral valve disease.

OBJECTIVES: Although risk factors for unsuccessful Maze procedure have been demonstrated, an appropriate patient selection is still controversial. In our institute, Maze procedure is indicated for those whom normal sinus rhythm (NSR) was reestablished by intraoperative direct cardioversion (DC) after ventricular unloading by total cardiopulmonary bypass. The purpose of this study was to evaluate the effectiveness of our indication criteria for Maze procedure in patients with mitral valve disease. METHODS: Between October 2012 and October 2021, MAZE was indicated in 55 patients in whom normal sinus rhythm (NSR) was reestablished by intraoperative direct current cardioversion (DC). Three endpoints and predictors were examined: disappearance of atrial fibrillation (AF), NSR, and A-wave detection. RESULTS: Restoration of NSR by intraoperative DC was confirmed in 43 patients, and these patients underwent MAZE. AF disappeared in 39 patients (90.7%), and F-wave ≥ 0.1 mV was a significant predictive factor (odds ratio (OR) 20.99, 95% CI 1.22-1079.06). NSR was reestablished in 36 patients (83.7%), and F-wave ≥ 0.1 mV (odds ratio 15.62, 95% CI 1.62-359.86) + AF history ≤ 3 years (OR 8.30, 95% CI 1.09-177.04) were significant predictors. A-wave detection was confirmed in 26 patients (60.5%), and left atrial diameter ≤ 55 mm was a significant predictor (OR 5.22, 95% CI 1.28-24.79). CONCLUSIONS: Intraoperative DC after ventricular unloading resulted effective patient selection for concomitant Maze procedure. F-wave and AF history were predictive factor of electrical restoration of AF, and left atrial diameter was predictive factor of restoration of atrial function.

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.

Trends and Disparities in Valvular Heart Disease Mortality in the United States From 1999 to 2020.

BACKGROUND: Percutaneous heart valve procedures have been increasingly performed over the past decade, yet real-world mortality data on valvular heart disease (VHD) in the United States remain limited. METHODS AND RESULTS: We queried the Centers for Disease Control and Prevention's Wide-Ranging Online Data for Epidemiologic Research database among patients ≥15 years old from 1999 to 2020. VHD and its subtypes were listed as the underlying cause of death. We calculated age-adjusted mortality rate (AAMR) per 100 000 individuals and determined overall trends by estimating the average annual percent change using the Joinpoint regression program. Subgroup analyses were performed based on demographic and geographic factors. In the 22-year study, there were 446 096 VHD deaths, accounting for 0.80% of all-cause mortality (56 014 102 people) and 2.38% of the total cardiovascular mortality (18 759 451 people). Aortic stenosis recorded the highest mortality of VHD-related death in both male (109 529, 61.74%) and female (166 930, 62.13%) populations. The AAMR of VHD has declined from 8.4 (95% CI, 8.2-8.5) to 6.6 (95% CI, 6.5-6.7) per 100 000 population. Similar decreasing AAMR trends were also seen for the VHD subtypes. Men recorded higher AAMR for aortic stenosis and aortic regurgitation, whereas women had higher AAMR for mitral stenosis and mitral regurgitation. Mitral regurgitation had the highest change in average annual percent change in AAMR. CONCLUSIONS: The mortality rate of VHD among the US population has declined over the past 2 decades. This highlights the likely efficacy of increasing surveillance and advancement in the management of VHD, resulting in improved outcomes.

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.

Early outcomes of transapical mitral valve implantation versus surgical replacement in matched elderly patients at intermediate surgical risk.

BACKGROUND: Data comparing transcatheter mitral valve implantation (TMVI) with surgical mitral valve replacement (SMVR) are lacking. AIMS: This study sought to compare the 30-day Valve Academic Research Consortium (VARC)-3 device success of TMVI with that of SMVR. METHODS: Matching protocol combined exact matching (sex, atrial fibrillation, previous surgical aortic valve replacement [SAVR] or coronary artery bypass grafting [CABG]), coarsened exact matching (age) and propensity score matching (body mass index, mitral valve pathology and concomitant tricuspid regurgitation). RESULTS: A total of 40 Tendyne TMVI and 80 SMVR patients with similar baseline characteristics were analysed (TMVI vs SMVR): age (78 years [interquartile range [{IQR} 75; 80] vs 78 years [IQR 73; 80]; p=0.8), female (60% vs 60%; p=1.0), atrial fibrillation (67.5% vs 63.7%; p=0.8), previous SAVR (12.5% vs 10.0%; p=0.8), previous CABG (20.0% vs 16.2%; p=0.8), body mass index (25.54 kg/m² vs 25.24 kg/m²; p=0.7) and valve pathology (mitral regurgitation: 70.0% vs 73.8%, mitral stenosis: 7.5% vs 3.8%, and mixed disease: 22.5% vs 22.5%; p=0.6). Most baseline characteristics not included in the matching model were balanced among the TMVI/SMVR cohorts: European System for Cardiac Operative Risk Evaluation (EuroSCORE) II (5.8% [IQR 2.9; 7.5] vs 4.2% [IQR 2.4; 6.8]; p=0.3) and Society of Thoracic Surgeons Predicted Risk of Mortality (STS-PROM) score (5.2% [IQR 3.2; 8.6] vs 4.1% [IQR 3.3; 6.1]; p=0.076). Coronary artery disease (67.5% vs 32.5%; p<0.001) and previous percutaneous coronary intervention (47.5% vs 25.0%; p=0.023) differed among groups. Mitral VARC (MVARC) device success at 30 days was achieved in 82.5% of patients after TMVI and 57.5% of patients after SMVR (p=0.04). MVARC procedural success at 30 days was 75.0% after TMVI versus 52.5% after SMVR (p=0.07). Thirty-day mortality (2.5% vs 3.8%; p=0.47), technical success (97.5% vs 97.5%; p=1.0), major bleeding (17.5% vs 18.7%; p=0.087), stroke (5.0% vs 4.9%; p=1.0) and postoperative haemodialysis (7.5% vs 5.2%; p=0.4) were similar in both groups. CONCLUSIONS: Patients with intermediate surgical risk, according to STS-PROM and EuroSCORE II, demonstrated higher rates of MVARC device at 30 days after TMVI compared to 30 days after SMVR. Rates of survival and procedural success, neurological, renal and bleeding complications were similar. Transfusion count and length of stay were lower after TMVI. For elderly patients at intermediate risk, a TMVI eligibility assessment may be considered.

Imaging of the Mitral Valve.

Echocardiographic imaging is the foundation for the evaluation of mitral valve dysfunction. Both transthoracic and transesophageal echocardiography provide insight into the anatomy, pathology, and classification mitral valve dysfunction. Echocardiography also provides a multi-parametric approach with semi-quantitative and quantitative parameters to assess the severity of mitral regurgitation and mitral stenosis. Transesophageal imaging is essential in the assessment of patients considered for surgical or transcatheter interventional strategies to treat mitral valve dysfunction. Cardiac computed tomography (CT) and cardiac MRI are useful adjunctive imaging techniques in mitral valve disease with CT providing detailed procedural specificity and MRI providing detailed ventricular and regurgitant flow analysis.

Transcatheter Therapy for Mitral Valve Stenosis.

Mitral valve stenosis remains highly prevalent among the US population although with dramatically shifting demographics. The significance of rheumatic mitral disease in developing nations persists, despite improvements in preventative measures and early detection, and its presence in developed countries is still evident as observed through international migration. In addition, the substantial growth in the aging population with a heightened occurrence of concurrent cardiovascular risk factors is leading to an increased prevalence of chronic calcific degeneration and degeneration of previously repaired or replaced valves. This article aims to review various transcatheter therapies in the treatment of mitral valve stenosis.

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 leaflet separation index correlation with mitral stenosis severity, a reliable easy 2-d echocardiography technique.

Objectives: To evaluate accuracy of mitral leaflet separation index for the determination of mitral stenosis severity in patients with rheumatic mitral stenosis. METHODS: The prospective, cross-sectional study was conducted at the National Institute of Cardiovascular Diseases, Karachi, from March 2021 to February 2022, and comprised patients with rheumatic mitral stenosis detected on echocardiography. The best end-diastole parasternal long axis and apical four-chamber views were acquired and Mitral leaflet separation was measured as the distance between the inner edges of the tip of mitral leaflets. Pearson correlation coefficient was computed for mitral leaflet separation index and mitral valve area. Receiver operating characteristic curve was used to determine the cut-off value of the mitral leaflet separation indexto categorise mitral stenosis. Data was analysed using SPSS 19. RESULTS: Of the 277 patients, 205(74%) were females and 72(26%) were males. The overall mean age was 39.93±11.22 years. The mean mitral leaflet separation index value was 7.65±2.23. The correlation was significant and strong between mitral leaflet separation index and mitral valve area on planimetry (p<0.001), and was significant and moderate when measured by pressure half-time (p< 0.001). Mitral leaflet separation index cut-off value <8.625mm and <8.25mmcould predict severe mitral stenosis with 84% and 86.3% sensitivity and 84.6% and 78.3% specificity on planimetry and pressure half-time, respectively. CONCLUSIONS: The mitral leaflet separation index was found to be an independent, reliable and simple measure for assessing mitral stenosis severity.

[Lipoprotein(a): relationships with atherosclerosis and valvular heart disease, and emerging therapies]. / Lipoproteina(a): associazione con la malattia aterosclerotica e valvolare e terapie emergenti.

Lipoprotein(a) [Lp(a)] is a well-established cardiovascular risk factor, whose relationship with atherosclerotic disease has been confirmed by epidemiological, genome-wide association, Mendelian randomization, and meta-analysis studies. This association is determined by its pro-atherogenic, pro-thrombotic and pro-inflammatory properties. Lp(a) is the most common monogenic risk factor for atherosclerosis, with a prevalence of about 1 in 5 people. Recently, its etiopathogenetic relationship with calcific and degenerative valvular heart diseases, particularly with aortic and mitral stenosis, has been suspected. It has not yet been demonstrated whether its reduction translates into a lower risk of cardiovascular events. Up to now, Lp(a) has been considered a non-modifiable risk factor, as current lipid-lowering drugs have limited effects on its levels. New specific lipid-lowering therapies with high efficacy in reducing circulating Lp(a) levels are being investigated in randomized trials; however, the effects of this reduction on cardiovascular outcomes are still being studied.

[Association of a myxoma of the left atrium and a mitral stenosis : About a case]. / Association d'un myxome de l'oreillette gauche et d'un rétrécissement mitral : à propos d'un cas.

Atrial myxoma is a rare and benign clinical entity. It remains common in women and mainly affects the left atrium. Its clinical picture is polymorphic. We report the case of a 53-year-old woman who consulted for dyspnea with cardiac failure's picture. The diagnosis of myxoma of the left atrium was made on echocardiography which also objectified the existence of mitral stenosis. The patient underwent surgical excision and mitral valve replacement with good postoperative outcomes.

An Undiagnosed Shone Complex in a 52-Year-Old Female: A Case Report.

Shone complex (SC) is a rare congenital heart disease characterized by four obstructive anomalies, including parachute mitral valve (PMV), left atrial supra-valvular ring, subaortic stenosis, and coarctation of the aorta. Typically, SC manifests early in life. However, we encountered a 52-year-old female with a history of hypertension diagnosed at 26 years and left-sided weakness poststroke. She presented with worsening dyspnea and palpitations, prompting a thorough investigation. Echocardiography revealed a heavily calcified bicuspid aortic valve with severe aortic stenosis and parachute mitral valve with severe mitral stenosis and preserved ejection fraction, raising suspicions regarding the presence of SC. Cardiac catheterization, aortic-angiography, and noncontrast chest computed tomography (CT) revealed abrupt occlusion of the postductal aorta, giving a picture of aortic coarctation with well-established collateral vessels including prominent right and left internal mammary arteries. So, she was diagnosed with an incomplete SC at the age of 52. Shone complex is a rare congenital heart disease that typically presents in early childhood, but late presentations due to misdiagnosis or incomplete work up are possible. This case emphasizes the rarity of late presentations of SC and highlights the importance of early diagnosis and intervention to improve outcomes. An incomplete SC should be considered in adult patients presenting with left-sided obstructive lesions.

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.

Midterm Outcome of Valve Repair for Rheumatic Mitral Stenosis: 6-year Experience in a Single Mid-Volume Cardiac Center.

OBJECTIVES: Previous studies have reported satisfactory long-term results of mitral valve (MV) repair for rheumatic mitral disease. However, the effects of this procedure in isolated rheumatic mitral stenosis remain unclear. In addition, protective effects of MV repair on cardiac function have not been verified in rheumatic MV disease. This study retrospectively evaluated early mortality and mid-term results of MV repair for isolated rheumatic mitral stenosis in a mid-volume cardiac centre, and explored the effects of this procedure on cardiac function. METHODS: Between January 2015 and May 2021, 360 patients with isolated rheumatic mitral stenosis and combined (concomitant) atrial fibrillation (AF) underwent MV repair (100 patients) or MV replacement (260 patients). Perioperative characteristics were compared between the two groups and a regression analysis for early mortality and mid-term left ventricular ejection fraction was conducted. In addition, mid-term survival was compared between the two groups. RESULTS: Baseline characteristics of the two groups were balanced after matching. Compared with patients in the replacement group, patients with MV repair had a lower occurrence of postoperative hypotension and AF. There was no difference in early mortality or mid-term survival between the two groups. However, MV repair was associated with a higher mid-term left ventricular ejection fraction. During follow-up, four thromboembolic events and four haemorrhagic events occurred in the replacement group. No blood coagulation-related complications occurred in the repair group. CONCLUSION: Mitral valve repair for isolated rheumatic mitral stenosis and concomitant AF was feasible in a mid-volume cardiac centre, with satisfactory perioperative results and mid-term outcomes. Furthermore, this procedure preserved mid-term left ventricular systolic function.

Primary cardiac rhabdomyosarcoma in a mitral valve involved with rheumatic disease.

A 51-year-old female underwent emergency mitral valve replacement for mitral stenosis with an undetermined mass which was attached to the anterior mitral leaflet. Histopathological testing of the excised specimen confirmed the diagnosis of rheumatic mitral disease in combination with a primary rhabdomyosarcoma. Postoperative adjuvant chemotherapy with pazopanib hydrochloride was given. At 10 months of follow-up, repeated computed tomographic screening has not shown any signs of local recurrence or secondary metastases. The potential for the existence of primary rhabdomyosarcomas should be borne in mind when faced with undetermined masses on mitral leaflets, even in the presence of rheumatic disease.

Outcomes of Severe Mitral Stenosis With the Revised Severity Criteria: Mitral Valve Replacement vs Percutaneous Mitral Valvuloplasty.

BACKGROUND: This study aimed to compare the outcomes, according to percutaneous mitral valvuloplasty (PMV) vs mitral valve replacement (MVR), of severe mitral stenosis (MS) with the updated criteria (MVA ≤ 1.5 cm2). METHODS: From the Multicenter Mitral Stenosis With Rheumatic Etiology (MASTER) registry of 3140 patients, we included patients with severe MS who underwent PMV or MVR between January 2000 and December 2021 except for previous valvular surgery/intervention, at least moderate other valvular dysfunction, and thrombus at the left atrium/appendage. Moderately severe MS (MS-MS) and very severe MS (VS-MS) were defined as 1.0 cm2 < MVA ≤ 1.5 cm2 and MVA ≤ 1.0 cm2, respectively. Primary outcomes were a composite of cardiovascular (CV) death and heart failure (HF) hospitalization. Secondary outcomes were a composite of primary outcomes and redo intervention. RESULTS: Among 442 patients (mean 56.5 ±11.9 years, women 77.1%), the MVR group (n = 260) was older, had more comorbidities, higher echoscore, larger left chambers, and higher right ventricular systolic pressure than the PMV group (n = 182). During a mean follow-up of 6.9 ± 5.2 years with inverse probability-weighted matching, primary outcomes did not differ, but the MVR group experienced fewer secondary outcomes (P = 0.010). In subgroup analysis of patients with MS-MS and VS-MS, primary outcomes did not differ. However, the MVR group in patients with VS-MS showed better secondary outcomes (P = 0.012). CONCLUSIONS: PMV or MVR did not influence CV mortality or HF hospitalization in both MS-MS and VS-MS. However, because of increased early redo intervention in the PMV group in VS-MS, MVR would be the preferable option without clear evidence of suitable morphology for PMV.