Primary Cardiac Sarcoma Involving the Mitral Valve, an Insidious Form of Heart Failure.
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ABSTRACT:
SUBMITTER: Prol T
PROVIDER: S-EPMC7887519 | biostudies-literature | 2021 Feb
REPOSITORIES: biostudies-literature
Publications
Primary Cardiac Sarcoma Involving the Mitral Valve, an Insidious Form of Heart Failure.
Prol Todd T Petro Joseph J Jain Harsh H Raja Shashi S Rachofsky Edward E Koulogiannis Konstantinos P KP Horgan Stephen S
CASE (Philadelphia, Pa.) 20201124 1
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Project description:Secondary mitral regurgitation (MR) results from left ventricular dilatation and dysfunction. Quantification of secondary MR is challenging because of the underlying myocardial disease. Clinical and echocardiographic evaluation requires a multi-parametric approach. Severe secondary MR occurs in up to one-fourth of patients with heart failure with reduced ejection fraction, which is associated with a mortality rate of 40% to 50% in 3 years. Percutaneous edge-to-edge mitral valve repair (MitraClip) has emerged as an alternative to surgical valve repair to improve symptoms, functional capacity, heart failure hospitalizations, and cardiac haemodynamics. Further new transcatheter strategies addressing MR are evolving. The Carillion, Cardioband, and Mitralign devices were designed to reduce the annulus dilatation, which is a frequent and important determinant of secondary MR. Several transcatheter mitral valve replacement systems (Tendyne, CardiAQ-Edwards, Neovasc, Tiara, Intrepid, Caisson, HighLife, MValve System, and NCSI NaviGate Mitral) are emerging because valve replacement might be more durable compared with valve repair. In small studies, these interventional therapies demonstrated feasibility and efficiency to reduce MR and to improve heart failure symptoms. However, neither transcatheter nor surgical mitral valve repair or replacement has been proven to impact on the prognosis of heart failure patients with severe MR, which remains high with a mortality rate of 14-20% at 1 year. To date, the primary indication for treatment of secondary severe MR is the amelioration of symptoms, reinforcing the value of a Heart Team discussion. Randomized studies to investigate the treatment effect and long-term outcome for any transcatheter or surgical mitral valve intervention compared with optimized medical treatment are urgently needed and underway.
| S-EPMC6073015 | biostudies-literature
Project description:Primary cardiac hemangioendothelioma is a very rare tumor. Up to now, <20 cases have been reported worldwide; involvement of mitral valve by the tumor is extremely rare. In this report, a case of hemangioendothelioma arose from the mitral valve and was successfully resected, and after few months the tumor recurred and infiltrated the heart.
| S-EPMC6123280 | biostudies-other
Project description:BackgroundWhen feasible, guidelines recommend mitral valve repair (MVr) over mitral valve replacement (MVR) to treat primary mitral regurgitation (MR), based upon historic outcome studies and transthoracic echocardiography (TTE) reverse remodeling studies. Cardiovascular magnetic resonance (CMR) offers reference standard biventricular assessment with superior MR quantification compared to TTE. Using serial CMR in primary MR patients, we aimed to investigate cardiac reverse remodeling and residual MR post-MVr vs MVR with chordal preservation.Methods83 patients with ≥ moderate-severe MR on TTE were prospectively recruited. 6-min walk tests (6MWT) and CMR imaging including cine imaging, aortic/pulmonary through-plane phase contrast imaging, T1 maps and late-gadolinium-enhanced (LGE) imaging were performed at baseline and 6 months after mitral surgery or watchful waiting (control group).Results72 patients completed follow-up (Controls = 20, MVr = 30 and MVR = 22). Surgical groups demonstrated comparable baseline cardiac indices and co-morbidities. At 6-months, MVr and MVR groups demonstrated comparable improvements in 6MWT distances (+ 57 ± 54 m vs + 64 ± 76 m respectively, p = 1), reduced indexed left ventricular end-diastolic volumes (LVEDVi; - 29 ± 21 ml/m2 vs - 37 ± 22 ml/m2 respectively, p = 0.584) and left atrial volumes (- 23 ± 30 ml/m2 and - 39 ± 26 ml/m2 respectively, p = 0.545). At 6-months, compared with controls, right ventricular ejection fraction was poorer post-MVr (47 ± 6.1% vs 53 ± 8.0% respectively, p = 0.01) compared to post-MVR (50 ± 5.7% vs 53 ± 8.0% respectively, p = 0.698). MVR resulted in lower residual MR-regurgitant fraction (RF) than MVr (12 ± 8.0% vs 21 ± 11% respectively, p = 0.022). Baseline and follow-up indices of diffuse and focal myocardial fibrosis (Native T1 relaxation times, extra-cellular volume and quantified LGE respectively) were comparable between groups. Stepwise multiple linear regression of indexed variables in the surgical groups demonstrated baseline indexed mitral regurgitant volume as the sole multivariate predictor of left ventricular (LV) end-diastolic reverse remodelling, baseline LVEDVi as the most significant independent multivariate predictor of follow-up LVEDVi, baseline indexed LV end-systolic volume as the sole multivariate predictor of follow-up LV ejection fraction and undergoing MVR (vs MVr) as the most significant (p < 0.001) baseline multivariate predictor of lower residual MR.ConclusionIn primary MR, MVR with chordal preservation may offer comparable cardiac reverse remodeling and functional benefits at 6-months when compared to MVr. Larger, multicenter CMR studies are required, which if the findings are confirmed could impact future surgical practice.
| S-EPMC10373289 | biostudies-literature
Project description:This case presentation discusses a rare cardiac malignancy initially thought to be a benign tumor. A 36-year-old presented with syncope, dyspnea, Computed Tomography Pulmonary angiography study obtained was negative for pulmonary emboli but revealed a left atrial mass. A transesophageal echocardiogram (TEE) confirmed a mass with multiple lobes and a broad base attached to the septum, encroaching into the right atrium, aortic root wall, base of the anterior mitral leaflet flowing to the mitral orifice in diastole also obstructing the right pulmonary vein. Despite a quick diagnosis and plan to begin treatment, the patient rapidly declined owing to the extent and aggressive nature of this cardiac malignancy. This case reports the insidious nature of these tumors as well as how challenging and life threatening they are at the time of their clinical manifestation.
| S-EPMC6476112 | biostudies-literature
Project description:Cardiac tumor can produce a variety of symptoms and clinical findings depending on the location, size, and histologic type. It may cause heart failure usually by interfering with intracardiac blood flows associated with the mass effects. Here, we report an extremely rare case of heart failure caused by primary cardiac sarcoma with a review of the literature. The cause of heart failure was moderate to severe mitral steno-insufficiency due to the direct tumoral invasion of mitral valve apparatus combined with obstruction of the pulmonary veins.
| S-EPMC6269419 | 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:ObjectiveTo assess the association between cardiac morphology and function assessed with cardiac MRI (CMRI) and haemodynamics at rest and during exercise in patients with primary mitral regurgitation (MR).MethodsIn an observational study, subjects with significant primary MR (N = 46) with effective regurgitant orifice ≥ 0.30 cm2 and left ventricular (LV) ejection fraction > 60% were examined with right heart catheterisation during rest and exercise and CMRI at rest. End-diastolic pressure volume relationship (EDPVR) was assessed using a single beat method using pulmonary capillary wedge pressure (PCWP) and end-diastolic volume. Patients were divided according to normal PCWP at rest (> 12 mm Hg) and with exercise (> 28 mm Hg). Results: Resting regurgitant volume correlated positively with resting PCWP, (r = 0.42, p = 0.002). However, with exercise no association between PCWP and regurgitant volume was seen (r = 0.09, p = 0.55). At rest left atrial (LA) maximal, minimal and volume index at atrial contraction correlated positively with PCWP (r = 0.60; r = 0.55; r = 0.58, all p < 0.001); in contrast none of these correlated with exercise PCWP (all p > 0.2). EDPVR in patients with high PCWP at rest was shifted towards higher volumes for the same pressures. The opposite was seen for patients with high PCWP during exercise where estimated volumes were smaller for the same pressure than patients with normal exercise PCWP.ConclusionIn patients with significant MR the degree of regurgitation and LA dilatation is associated with resting PCWP. However, with exercise this association disappears. Estimation of EDPVR suggests lower LV compliance in patients where PCWP is increased with exercise.Clinical trial registrationURL: https://clinicaltrials.gov/ct2/show/NCT02961647?term=HEMI&rank=1. ID: NCT02961647.
| S-EPMC6307562 | biostudies-literature
Project description:AimsTherapeutic options for patients with heart failure with preserved ejection fraction (HFpEF) are sparse. Mitral regurgitation (MR) is a common feature of HFpEF and worsens heart failure symptoms and prognosis. Our study examines the outcome of patients with preserved left ventricular ejection fraction (LVEF) and elevated left atrial (LAP) or left ventricular filling pressures (LVEDP), indicative of HFpEF, after undergoing percutaneous edge-to-edge mitral valve repair (pMVR) for moderate-severe MR.Methods and resultsTwo hundred eleven patients with preserved LVEF (>50%), who underwent pMVR, were dichotomized by LAP (< / ≥15 mmHg) and LVEDP (< / ≥16 mmHg). Forty-nine per cent of patients showed elevated LAP, and LVEDP was elevated in 55%, both indicating HFpEF. Patients with elevated filling pressures featured typical clinical characteristics of HFpEF, higher N-terminal pro-brain natriuretic peptide levels (5544.9 pg/mL in high LAP group vs. 3071.7 pg/mL in normal LAP group, P = 0.06; 5061.0 pg/mL in high LVEDP group vs. 3230.3 pg/mL in normal LVEDP group, P = 0.08), and higher prevalence of pulmonary hypertension (mean pulmonary artery pressure 36.4 mmHg in high LAP group vs. 26.3 mmHg in normal LAP group, P < 0.001; 35.2 mmHg in high LVEDP group vs. 29.7 mmHg in normal LVEDP group, P = 0.004) and atrial fibrillation (78.8% in normal LAP group vs. 61.0% in high LAP group, P = 0.04; 75.3% in high LVEDP group vs. 67.5% in normal LVEDP group, P = 0.25). Pre-treatment MR grade and New York Heart Association (NYHA) class were similar in both normal filling pressure and HFpEF groups. pMVR in HFpEF patients achieved effective heart failure symptom relief comparable with patients with normal filling pressures: significant decrease of MR grade and NYHA class, as well as significant reduction of heart failure hospitalizations 12 months after compared with 12 months before MitraClip.ConclusionPercutaneous edge-to-edge mitral valve repair for moderate-severe MR is an effective treatment option for symptom relief in HFpEF patients.
| S-EPMC8712801 | biostudies-literature
Project description:The role of percutaneous mitral valve repair (PMVr) in management of high-risk patients with severe mitral regurgitation (MR) and acute decompensated heart failure (ADHF) is undetermined. We screened all patients who underwent PMVr between October 2015 and March 2020. We evaluated immediate, 30-day, and 1-year outcomes in patients who underwent PMVr during hospitalization due to ADHF as compared to elective patients. From a cohort of 237 patients, we identified 46 patients (19.4%) with severe MR of either functional or degenerative etiology who underwent PMVr during index hospitalization due to ADHF, including 17 (37%) critically ill patients. Patients' mean age was 75.2 ± 9.8 years, 56% were males. There were no differences in background history between ADHF and elective patients. Patients with ADHF were at higher risk for surgery, reflected in higher mean EuroSCORE II, compared with elective patients. After PMVr, we observed higher 30-day mortality rate in ADHF patients as compared to the elective group (10.9% vs. 3.1%, respectively, p = 0.042). One-year mortality rate was similar between the groups (21.7% vs. 17.9%, p = 0.493). Clinical and echocardiographic follow-up showed improvement of NYHA functional class and sPAP reduction in both groups ((54 ± 15 mmHg to 50 ±15 in the elective group (p = 0.02), 58 ± 13 mmHg to 52 ± 12 in the ADHF group (p = 0.02)). PMVr could be an alternative option for treatment of patients with severe MR and ADHF.
| S-EPMC8704045 | biostudies-literature
Project description:We here report the results of a mitral valve transcriptome study designed to identify genes and molecular pathways involved in development of congestive heart failure (CHF) following myxomatous mitral valve disease (MMVD) in dogs. The study is focused on a cohort of elderly age-matched dogs (n=34, age ~10 years) from a single breed – Cavalier King Charles Spaniels (CKCS) – with a high incidence of MMVD. The cohort comprises 19 dogs (10♀, 9♂) without MMVD-associated CHF, and 15 dogs (6♀, 9♂) with CHF caused by MMVD. I.e. we compare gene expression in breed and age matched groups of dogs, which only differ with respect to CHF status. We identify 56 genes, which are differentially expressed between the two groups. In this list of genes, we confirm an enrichment of genes related to the TNFβ signaling pathway, extracellular matrix organization, vascular development, and endothelium damage, which also have been identified in previous studies. However, the genes with the greatest difference in expression between the two groups are CNTN3 and MYH1. Both genes encode proteins, which are predicted to have an effect on the contractile activity of myocardial cells, which in turn may have an effect on valvular performance and hemodynamics across the mitral valve. This may result in shear forces with impact on MMVD progression.
2023-11-10 | GSE217750 | GEO