Unusual cause of heart failure: Mitral stenosis and pulmonary venous obstructions caused by the direct invasion of primary cardiac sarcoma.
ABSTRACT: 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.
Project description:We describe a patient with symptoms of heart failure caused by severe mitral regurgitation. Echocardiography revealed an intracardiac mass embedding the posterior mitral valve leaflet, and cardiac magnetic resonance imaging showed two intracardiac thrombi and endomyocardial fibrosis. Eosinophil count kept rising and a mutation in the gene for platelet-derived growth factor receptor alpha was found. The combination of these findings led to the diagnosis of Loeffler's endocarditis. Treatment with prednisone and a tyrosine kinase inhibitor resulted in complete remission of the hypereosinophilia and mitral valve regurgitation was only mild at 9-month follow-up visit. <Learning objective: This case report presents a patient with severe mitral regurgitation and heart failure due to hypereosinophilic syndrome (HES). It leads to thrombus formation and endomyocardial thickening due to eosinophilic infiltration of the myocardium. Treatment with steroids and a tyrosine kinase inhibitor led to clinical improvement and only mild mitral regurgitaton after 6 months. Loeffler's endomyocarditis is a model disease for restrictive cardiomyopathy. It is important to recognize and treat this disease early and prevent morbidity and mortality. As far as we know there is no previous case report that describes the reversibility of severe mitral regurgitation after pharmacological treatment of HES, not needing mitral valve replacement.>.
Project description:Cardiac amyloidosis results in an infiltrative restrictive cardiomyopathy, with a number of characteristic features: biventricular hypertrophy, abnormal myocardial global longitudinal strain with relative apical sparing, biatrial dilation, and small pericardial effusion along with conduction abnormalities. Amyloid deposits leading to hemodynamically significant valvular heart disease are very rare. We describe a rare case of concomitant moderately severe tricuspid and mitral valve stenosis because of ongoing amyloid deposition in a patient with progressive multiple myeloma and fat pad biopsy-proven light chain amyloidosis. Worsening infiltrative cardiomyopathy and valvulopathy despite evidence-based chemotherapy and heart failure pharmacotherapy led to end-stage disease and death. Valvular involvement in cardiac amyloidosis requires early recognition of the underlying disease condition to guide directed medical therapy and prevent its progression. In this instance, valvuloplasty or valve replacement is not a viable option.
Project description:Background:Rheumatic mitral valve (MV) disease is the major cause of congestive cardiac failure in children and young adults, particularly in developing countries. Mitral valve repair with minimum prosthetic material is the gold standard treatment for this condition. However, MV repair for rheumatic MV disease is known to be technically demanding. Case summary:A 27-year-old woman without a history of cardiac disease presented with dyspnoea on exertion. Echocardiography revealed rheumatic severe mitral stenosis and regurgitation, with thickening of the bileaflets, doming of the anterior leaflet, shortening of the posterior leaflet, fusions of the lateral and particularly the medial commissure, and enlargement of the mitral annulus. We successfully performed robot-assisted MV repair with bicommissural release, patch augmentation of the two leaflets, and implantation of an originally sized partial band. Discussion:Robotic MV repair can contribute to precise valve inspection and operative procedures. This approach seems feasible for complex rheumatic MV disease particularly in young patients.
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.>.
Project description:Infective endocarditis (IE) is defined as infection of endocardial surface of the heart. It may include one or more heart valves, the mural endocardium or a septal defect. Its intracardiac effect includes severe valvular insufficiency which may lead to intractable congestive heart failure and myocardial abscess. Infective endocarditis especially complicated by an abscess is associated with high mortality, despite the medical and surgical therapeutic options available. Surgical intervention is indicated in cases of heart failure or uncontrolled infection and sometimes for the prevention of embolic phenomena. We report a case of 42 yrs/M with RVHD admitted in Dr D.Y.Patil hospital, Kolhapur. He had high grade, continuous fever, vomiting, cough with expectoration since 15 days prior to admission. He had prior embolic stroke 2 months back from which he recovered completely. The diagnosis of Infective endocarditis was confirmed clinically & echocardiographically by Duke's criteria. His ECHO showed severe MR, Moderate MS and large vegetations on AML oscillating through mitral orifice along with subvalval (mitral) abscess. Due to severe haematemesis following Mallory weiss tear surgical intervention was not possible. Patient succumbed as a result of refractory pulmonary oedema.
Project description:Background:Transcatheter mitral valve replacement (TMVR) may be a valuable treatment option for mitral annular calcification and severe mitral stenosis (MS) in patients at high operative risk. Pre-procedural virtual and printed simulations may aid in procedure planning, device sizing, and mitigate complications such as valve embolization or left ventricular outflow tract (LVOT) obstruction. Case summary:We describe a case of TMVR in which multi-detector computed tomography (MDCT) derived, three-dimensional virtual planning and a 3D-printed model of the patients' left heart provided enhanced understanding of an individual patient's unique anatomy to determine feasibility, device sizing, and risk stratification. This resulted in deployment of an adequately sized valve. Post-TMVR LVOT obstruction was treated with LVOT balloon dilatation and percutaneous transluminal septal myocardial ablation. Discussion:Advanced MDCT-derived planning techniques introduce consistent 3D modeling and printing to enhance understanding of intracardiac anatomical relationships and test device implantation. Still, static measurements do not feature haemodynamic factors, tissue, or device characteristics and do not predict device host interaction. Transcatheter mitral valve replacement is feasible in MS when adequately pre-procedurally planned. Multi-detector computed tomography-derived, 3D, virtual and printed models contribute to adequate planning in terms of determining patient eligibility, procedure feasibility, and device sizing. However, static 3D modeling cannot completely eliminate the risk of peri-procedural complications.
Project description:<h4>Background</h4>Aortic stenosis is a common finding in cardiac amyloidosis (CA). Younger patients often remain asymptomatic. If unrecognized, this can lead to serious complications such as heart failure. Progression of aortic stenosis can be accelerated in patients with chronic kidney disease and need for dialysis. Perioperative risk in these patients is often high due to the underlying systemic disease.<h4>Case summary</h4>A 40-year-old Caucasian man with known AA amyloidosis, highly active Ankylosing Spondylitis and need for chronic dialysis due to end-stage chronic renal failure presented for echocardiographic routine exam without reporting any cardiac symptoms. At the last visit 4 years ago, a normal heart valve function was noted and no echocardiographic follow-up was performed in the following. Now, rapid progression with severe aortic valve and mitral valve stenosis was stated and the patient underwent combined aortic and mitral surgical valve replacement following discussion in the multidisciplinary cardiology meeting. Macroscopic examination of the valves revealed significant calcification and histological examination showed the high presence of amyloid by Congo-red staining and immunohistological staining for AA-Amyloid. Both valve prosthetic devices showed normal function as well as a normal left ventricular ejection fraction in initial post-operative transoesophageal echocardiography. After prolonged and complicated post-operative course in the intensive care unit the patient died 3 months after surgery due to intractable multiorgan failure in combined severe abdominal septic and cardiogenic shock.<h4>Discussion</h4>Concomitant CA and chronic dialysis can accelerate the onset of severe aortic valve stenosis. Young patients, as in this case, often stay asymptomatic, perioperative risk increases with duration of chronic dialysis and severity of valve stenosis. This increases the need for regular short-term echocardiographic examinations even in clinical stable patients.
Project description:Hammock valve, also known as anomalous mitral arcade is a rare mechanism for congenital mitral insufficiency. We report a case of a two-week-old neonate who presented with features of heart failure and an apical systolic murmur. Echocardiogram showed severe mitral regurgitation and abnormal mitral valve with direct attachment of mitral leaflets to papillary muscle without intervening chordae tendinae, typical of hammock valve. Heart failure was controlled with ionotrpes and diuretics. The literature on the hammock mitral valve is reviewed.
Project description:<h4>Aims</h4>Several approaches for transcatheter mitral valve repair for functional mitral valve regurgitation are established. Interventional direct annuloplasty is a novel trans-venous, trans-septal approach. While feasibility was proven recently, knowledge on its influence on cardiac dimensions, pressures, biomarkers, and clinical outcomes is sparse.<h4>Methods and results</h4>Patients consecutively treated with direct annuloplasty-only procedures between December 2015 and April 2018 were included in this monocentric analysis. Echocardiographic measurements, biomarker levels, clinical status [New York Heart Association (NYHA) class and 6 min walk test] were assessed at baseline, at discharge, and at a 30 day follow-up. Overall, 18 patients (in mean 77.0 ± 7.4 years, 44.4% women) with initially all high-grade mitral valve regurgitation (MR) were included in this study. Procedural success rate was high (94.4%) without severe complications. Direct annuloplasty resulted in MR-reduction (post-procedural-MR mild or no/trace: 72.2%) and the proportion of patients with severe dyspnoea (NYHA III/IV) was reduced (88.9% vs. 50%, P = 0.008). Clinical results were associated with a relevant diminution of left atrial volumes (-16.5%, P < 0.001) and cardiac pressures [left atrial pressure (-32.3%, P = 0.019) and systolic pulmonary arterial pressure (PAP, -15.8%, P = 0.025)]. Patients with lower baseline levels of PAP (P = 0.022) as well as elevated highly sensitive troponin (P = 0.034) were more likely to archive clinical benefit (improvement in NYHA class ?1 grade) after 1 month, which could not be correlated with the grade of MR-reduction.<h4>Conclusions</h4>Transcatheter mitral valve repair by direct annuloplasty results in a relevant reduction of intracardiac pressures, left atrial volumes, dyspnoea, and MR. Lower PAP and higher troponin values at baseline could be associated to dyspnoea reduction.
Project description:We report the case of a man in his 60s who had dilated cardiomyopathy with severe functional mitral regurgitation. Four years after a cardiac resynchronization therapy (CRT) device with an implantable cardioverter defibrillator was implanted, this device was replaced with an adaptive CRT device because of battery consumption. Seven months after replacement of this device, the left ventricular pacing to right ventricular activation and the atrioventricular delay from automatic adjustments contributed to less functional mitral regurgitation. The findings from our case suggest that optimal CRT, by measuring intracardiac conduction parameters, is effective for functional mitral regurgitation.