Isolated severe mitral stenosis as an uncommon manifestation of cardiac amyloidosis.
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ABSTRACT:
SUBMITTER: Kwak S
PROVIDER: S-EPMC11295530 | biostudies-literature | 2024 Aug
REPOSITORIES: biostudies-literature
Publications
Isolated severe mitral stenosis as an uncommon manifestation of cardiac amyloidosis.
Kwak Soongu S Lim Sojung S Kim Yong-Jin YJ Kim Hyung-Kwan HK
Journal of cardiovascular imaging 20240802 1
Similar Datasets
Project description:Left atrium and left atrial appendage thrombus is common in patients with mitral stenosis, causing significant morbidity and mortality. We described a case of rheumatic mitral stenosis, a 48-year-old female patient, who had undergone percutaneous transvenous mitral commissurotomy 26 years back. She presented with an episode of palpitations and breathlessness on mild exertion. She found to have rapid atrial fibrillation and heart failure. Her echocardiography showed severe mitral stenosis and large left atrial thrombus intermittently obstructing the mitral valve. While she was prepared for urgent valve replacement she had cardiac arrest and died. The echocardiogram showed the thrombus was stuck and closed the mitral valve orifice. From this events we did a review in our institution about the mitral stenosis patients who found to have left atrium thrombus by routine transthoracic echocardiography in five years period. Looking for factors contributing to thrombus formation and outcome predictor, as to outline management plan. <Learning objective: Our aim is to give more attention to the factors contributing for left atrium thrombus formation and thrombus character in mitral stenosis patients and its clinical impact to prevent miserable events in such high risk patients.>.
| S-EPMC6718824 | biostudies-literature
Project description:BackgroundTranscatheter therapy has become an alternative for functional mitral regurgitation (FMR) in patients at high surgical risk. However, the intervention of FMR in cardiac amyloidosis (CA) with transcatheter edge-to-edge repair (TEER) is controversial due to the potential risk of left atrial pressure (LAP) elevation.Case summaryAn 83-year-old woman with repeated heart failure (HF) and severe mitral regurgitation (MR) was referred to our centre for TEER. Pre-procedural transthoracic echocardiography (TTE) and transoesophageal echocardiography (TOE) confirmed the degree of MR and a functional aetiology. A peculiar LAP increase in this patient occurred immediately after successful TEER clip implantation and her n-terminal prohormone of brain natriuretic peptide significantly increased post-operatively. The diagnosis of CA was suspected and was subsequently established through endomyocardial biopsy. Aggressive anti-HF therapy was initiated and the patient was discharged after her HF symptoms were relieved. At 6-month follow-up, the patient was still alive and no episode of acute HF was experienced.DiscussionSevere functional MR in CA treated with TEER has the potential risk of increasing LAP. During the short-term follow-up, TEER appears beneficial for left heart function (reduction of MR) but harmful for right heart function (increase of LAP). CA patients with severe FMR should be carefully evaluated about the benefits and potential harm of TEER intervention.
| S-EPMC9792274 | biostudies-literature
Project description:To know the regression of right ventricular pressure after successful percutaneous transluminalmitral commissurotomy (PTMC) in patients with severe isolated mitral stenosis.This descriptive study was performed in inpatient and outpatient department of National Institute of Cardiovascular Disease from 1st February 2016 to 31st August 2016. Echocardiography of all patients with successful PTMC were recorded 24 hours and 06 months following PTMC to see for Regression of right ventricular pressure along with other baseline echocardiographic parameters.A total of 99 patients with severe isolated mitral stenosis who had undergone successful PTMC were studied. Females were 65(65.7%) and males 34(34.3%). Mean age was 27.44±6.26 years. TTE performed before and after PTMC showed significant difference in mean mitral valve area (0.89cm ±0.089cm2 vs. 1.68±0.128 cm2, p valve <0.001) and mean left atrial diameter (4.66± .82cm vs. 4.46± 0.65cm). Mean mitral valve gradient pre PTMC was significantly higher (16.38±2.51 mm of Hg) than that of post PTMC 24 hours (4.75±1.31 mm of Hg) and Post PTMC 06 months (5.22±1.21 mm of Hg), p valve <0.001. Mean right ventricular systolic pressure (RVSP) pre PTMC was significantly higher 62.3±10.91 mm of Hg than that of post PTMC 24 hour's 57.51±9.67 mm of Hg and post PTMC 06 moths 46.49±7.8mm of Hg, p value 0.001. Mean LVEF 50.14± 5.82.There was a significant regression of right ventricular pressure following successful PTMC in mid-term (06 months) follow up of severe isolated mitral stenosis patients.
| S-EPMC5510097 | biostudies-other
Project description: Not available
| S-EPMC11551943 | biostudies-literature
Project description:AimsConcomitant cardiac amyloidosis (CA) in severe aortic stenosis (AS) is difficult to recognize, since both conditions are associated with concentric left ventricular thickening. We aimed to assess type, frequency, screening parameters, and prognostic implications of CA in AS.Methods and resultsA total of 191 consecutive AS patients (81.2 ± 7.4 years; 50.3% female) scheduled for transcatheter aortic valve replacement (TAVR) were prospectively enrolled. Overall, 81.7% underwent complete assessment including echocardiography with strain analysis, electrocardiography (ECG), cardiac magnetic resonance imaging (CMR), 99m Tc-DPD scintigraphy, serum and urine free light chain measurement, and myocardial biopsy in immunoglobulin light chain (AL)-CA. Voltage/mass ratio (VMR; Sokolow-Lyon index on ECG/left ventricular mass index) and stroke volume index (SVi) were tested as screening parameters. Receiver operating characteristic curve, binary logistic regression, and Kaplan-Meier curve analyses were performed. CA was found in 8.4% of patients (n = 16); 15 had transthyretin (TTR)-CA and one AL-CA. While global longitudinal strain by echo did not reliably differentiate AS from CA-AS [area under the curve (AUC) 0.643], VMR as well as SVi showed good discriminative power (AUC 0.770 and 0.773, respectively), which was comparable to extracellular volume by CMR (AUC 0.756). Also, VMR and SVi were independently associated with CA by multivariate logistic regression analysis (P = 0.016 and P = 0.027, respectively). CA did not significantly affect survival 15.3 ± 7.9 months after TAVR (P = 0.972).ConclusionBoth TTR- and AL-CA can accompany severe AS. Parameters solely based on ECG and echocardiography allow for the identification of the majority of CA-AS. In the present cohort, CA did not significantly worsen prognosis 15.3 months after TAVR.
| S-EPMC7687139 | biostudies-literature
Project description:Reported cases of uni-leaflet mitral valve (MV) were related to the absence or dysplasia of the posterior mitral leaflet with ample anterior mitral leaflet. We present here a new entity of uni-leaflet MV where the MV appears as a membrane-like structure with a single slit-like orifice at its lateral part with no commissures.Case reportContinuous Doppler flow revealed a mean pressure gradient of 19 mmHg across the mitral valve indicating severe mitral stenosis. In 3D images from the left atrial view, the MV appeared like a membrane with a single orifice in its lateral part toward the left atrial appendage, the area of this orifice by 3D was 0.52 cm2, there were no commissures or even any residual lines at the site where commissures should be. The diagnosis of congenital severe mitral stenosis due to acommissural MV was confirmed. During surgery, the surgical appearance of the MV confirmed our diagnosis by 3D.ConclusionIsolated congenital severe mitral stenosis presenting in adulthood is rare, uni-leaflet MV as a cause is only reported in a few cases. MV replacement is usually indicated due to the abnormal anatomy of MV leaflets and the subvalvular apparatus.<Learning objectives: How to diagnose uni-leaflet congenital mitral stenosis and how to differentiate it from other causes of congenital mitral stenosis.>.
| S-EPMC6546642 | biostudies-literature
Project description:Infective endocarditis (IE) rarely results in mitral stenosis (MS), but MS in patients with IE can be life-threatening. We present a case of prosthetic MS secondary to IE. A 69-year-old Japanese man underwent mitral valve replacement with a bioprosthetic valve 2 years previously. The patient presented with a 1-month history of illness, and we diagnosed prosthetic valve IE with severe MS and planned for time-sensitive surgery. However, the patient developed cardiogenic shock in response to prosthetic mitral valve obstruction while awaiting surgery. The patient then had to undergo emergency surgery. There are no management guidelines for IE-induced valve stenosis, whose treatment differs from that of valve regurgitation. Our literature review reveals that achieving survival in patients with MS secondary to IE is difficult without surgical intervention. Patients with MS caused by IE may require surgery, and specific criteria should be outlined in future guidelines.
| S-EPMC11646871 | biostudies-literature
Project description:Intimal sarcomas are a very rare medical entity and usually arise from the pulmonary artery or the thoracic aorta. Sarcomas should be considered in the differential diagnosis in patients with suspected mitral valve disease. Echocardiography should be performed as early as possible to establish a prompt diagnosis and management.
| S-EPMC5538049 | biostudies-other
Project description:BackgroundAmyloidosis is a multi-organ disease of emerging significance in the field of cardiology. Chylothorax, a specific form of pleural effusion characterized by lymphatic fluid accumulation in the pleural cavity, is an extremely rare manifestation of amyloidosis. Notably, only five cases of chylothorax related to cardiac amyloidosis have been reported worldwide, all in amyloid light chain (AL) amyloidosis. No cases have been documented in amyloid transthyretin (ATTR) amyloidosis. Furthermore, elevated levels of serum carbohydrate antigen (CA) 125 have been associated with a poor prognosis in patients with AL cardiac amyloidosis.Case summaryWe report the case of an 85-year-old Austrian man with pronounced left ventricular hypertrophy, monoclonal gammopathy, and a history of atrial fibrillation. Further examinations, including myocardial biopsy, confirmed the diagnosis of ATTR cardiac amyloidosis. A significant right-sided pleural effusion was also observed. Thoracocentesis diagnosed chylothorax, confirmed by lymphangiography. Elevated CA 125 levels were found in both serum and pleural fluid, with no other findings suspicious for malignancy. The patient underwent a short break in oral anticoagulation and received prophylactic low-molecular-weight heparin for myocardial biopsy, thoracocentesis, and lymphangiography. However, they died a few days later due to an embolic stroke.DiscussionAt this time, we can only speculate about the pathomechanism of chylothorax development in the context of amyloidosis. We recommend further investigation of similar cases to deepen understanding of the underlying causes and identify potential treatment strategies. Additionally, the utility of CA 125 as a prognostic marker in ATTR amyloidosis needs further investigation.
| S-EPMC11694664 | biostudies-literature
Project description:BackgroundOlder patients with severe aortic stenosis (AS) are increasingly identified as having cardiac amyloidosis (CA). It is unknown whether concomitant AS-CA has worse outcomes or results in futility of transcatheter aortic valve replacement (TAVR).ObjectivesThis study identified clinical characteristics and outcomes of AS-CA compared with lone AS.MethodsPatients who were referred for TAVR at 3 international sites underwent blinded research core laboratory 99mtechnetium-3,3-diphosphono-1,2-propanodicarboxylic acid (DPD) bone scintigraphy (Perugini grade 0: negative; grades 1 to 3: increasingly positive) before intervention. Transthyretin-CA (ATTR) was diagnosed by DPD and absence of a clonal immunoglobulin, and light-chain CA (AL) was diagnosed via tissue biopsy. National registries captured all-cause mortality.ResultsA total of 407 patients (age 83.4 ± 6.5 years; 49.8% men) were recruited. DPD was positive in 48 patients (11.8%; grade 1: 3.9% [n = 16]; grade 2/3: 7.9% [n = 32]). AL was diagnosed in 1 patient with grade 1. Patients with grade 2/3 had worse functional capacity, biomarkers (N-terminal pro-brain natriuretic peptide and/or high-sensitivity troponin T), and biventricular remodeling. A clinical score (RAISE) that used left ventricular remodeling (hypertrophy/diastolic dysfunction), age, injury (high-sensitivity troponin T), systemic involvement, and electrical abnormalities (right bundle branch block/low voltages) was developed to predict the presence of AS-CA (area under the curve: 0.86; 95% confidence interval: 0.78 to 0.94; p < 0.001). Decisions by the heart team (DPD-blinded) resulted in TAVR (333 [81.6%]), surgical AVR (10 [2.5%]), or medical management (65 [15.9%]). After a median of 1.7 years, 23% of patients died. One-year mortality was worse in all patients with AS-CA (grade: 1 to 3) than those with lone AS (24.5% vs. 13.9%; p = 0.05). TAVR improved survival versus medical management; AS-CA survival post-TAVR did not differ from lone AS (p = 0.36).ConclusionsConcomitant pathology of AS-CA is common in older patients with AS and can be predicted clinically. AS-CA has worse clinical presentation and a trend toward worse prognosis, unless treated. Therefore, TAVR should not be withheld in AS-CA.
| S-EPMC7805267 | biostudies-literature