Beyond amyloidosis a case of cardiac light chain deposition disease
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A 48-year-old man presented with worsening dyspnea and was found to have reduced left ventricular ejection fraction and hypertrophy. Tests ruled out amyloidosis but showed monoclonal free light chains and cardiac light chain deposition. Biopsy demonstrated light chain immunoreactivity in fibrotic areas, confirming light chain deposition disease. Medical management was initiated but the patient deteriorated and died while being evaluated for heart transplantation.
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This document defines syncope as a transient loss of consciousness due to decreased blood flow to the brain. It accounts for 1-3% of emergency room visits. Causes include reflex syncope, orthostatic hypotension, cardiac issues like arrhythmias, and neurological or psychiatric conditions. Evaluation involves identifying life-threatening causes through history, physical exam, EKG and cardiac monitoring. Scoring systems can predict adverse outcomes at 1 year but have limited specificity.
Polyarteritis nodosa
Polyarteritis nodosa (PAN) is a systemic necrotizing vasculitis predominantly affecting medium arteries. It can be idiopathic or associated with hepatitis B virus (HBV) infection. The document discusses the epidemiology, pathogenesis, clinical presentation, diagnostic criteria and tests, imaging findings, and prognosis of PAN. It highlights that PAN affects medium-sized arteries and is differentiated from other vasculitides by the absence of glomerulonephritis, ANCA positivity, or involvement of arterioles/capillaries. HBV vaccination and certain drugs have been associated with PAN in some cases.
Pad1
1. Peripheral arterial disease (PAD) is a marker for increased risk of myocardial infarction and ischemic stroke, as it often co-exists with atherosclerosis in other arteries.
2. PAD is more common than diagnosed, so proper diagnosis using ankle-brachial pressure index is important. A low index reliably predicts increased risk of ischemic events.
3. Lifestyle changes and antiplatelet drugs like clopidogrel, which provides greater benefit than aspirin, are key for managing PAD patients and reducing cardiovascular risks.
Surgical and endovascular treatment of Paget-Schroetter
This document discusses Paget-Schroetter syndrome, also known as effort thrombosis, which is thrombosis (blood clot) of the axillary and subclavian veins caused by repetitive arm motions. It affects young, active individuals and causes swelling and pain in the arm. Treatment options discussed include anticoagulation alone versus thrombolysis or decompression of the thoracic outlet through surgery. Follow up of surgical patients found low complication rates, satisfactory long-term outcomes with patent veins, and good post-operative quality of life scores.
CTO and low ejection fraction
CTO and low ejection fraction
Alfredo R. Galassi, Italy
The Experts “Live” Workshop 2017
Friday, September 15th, 2017
Neurosarcoidosis
Neurosarcoidosis is a multisystem granulomatous disease of unknown etiology where noncaseating granulomas can affect multiple organs including the lungs, heart, skin and nervous system. It most commonly involves the lungs in 90% of cases. The central nervous system is involved in 5-16% of cases, with cranial neuropathies being the most frequent neurological manifestation. Diagnosis involves evidence of systemic sarcoidosis along with neurological symptoms, and can be supported by MRI, lumbar puncture and biopsy when possible. Corticosteroids are the main treatment to suppress inflammation and immune activity, and some severe or refractory cases may require additional immunosuppressants. Prognosis depends on location
Neurosarcoidosis
Neurosarcoidosis is a multisystem granulomatous disease of unknown etiology where noncaseating granulomas can affect multiple organs including the lungs, heart, skin and nervous system. It most commonly involves the lungs in 90% of cases. The central nervous system is involved in 5-16% of cases, with cranial neuropathies, encephalitis, meningitis and mass lesions being common neurological manifestations. Treatment involves corticosteroids as the mainstay, with immunosuppressants also used in refractory cases. Prognosis depends on location and type of involvement, with 72% of neurological cases deteriorating within 18 months if not treated.
Anaemia in heart failure
This document summarizes information on anemia in heart failure patients. Some key points:
1. The prevalence of anemia in heart failure patients ranges from 20-30% for outpatients to 30-40% for inpatients, depending on the definition and study.
2. Anemia is associated with worse prognosis and increased risk of hospitalization and mortality in heart failure patients.
3. Potential treatment options for anemia in heart failure include blood transfusions, erythropoietin-stimulating proteins (ESPs), and iron therapy. However, clinical trials of ESPs like darbepoetin alfa have not shown clear benefits.
4. The FAIR-HF trial found
Imaging: Multiple Pulmonary Cavitary Lesions
A 38-year-old male smoker presented with swelling all over the body for the past 4 months. Examination revealed pitting pedal edema. Investigations showed elevated ESR, CRP and P-ANCA positivity. CT scan showed multiple cavitary lung lesions. A diagnosis of Wegener's granulomatosis was made based on clinical features, lab investigations and radiological findings.
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Treatment Of Iatrogenic Artery Pseudoaneurysm By Ultrasound Guided Fibrin Glue Injection
Trattamento dello pseudoaneurisma iatrogeno mediante iniezione coguidata di colla di fibrina-
XXXIII° Congresso Nazionale della Società Italiana di Cardiologia Invasiva Porto Antico di Genova, Centro Congressi-3 ottobre 2012 Treatment of iatrogenic artery pseudoaneurysm by ultrasound guided fibrin glue injection: a single center experience Francesca Faresin; Francesca Franz; Marco Zennaro; Enrico Favaretto; Luigi Pedon; Salvatore Ronsivalle; Division of Vascular Surgery,Division of Cardiology, Cittadella Hospital, Padua, Italy-
(Angiologia-Chirurgia Vascolare-ULSS 15 Alta Padovana)
(Angiology- Vascular Surgery -ULSS 15 Alta Padovana)Polyarteritis nodasa and microscopic polyangitis
This document discusses Polyarteritis Nodosa (PAN) and Microscopic Polyangiitis (MPA). PAN is a necrotizing vasculitis predominantly affecting medium-sized arteries that spares small vessels. It is typically ANCA-negative. MPA is a pauci-immune necrotizing vasculitis involving small vessels and sometimes medium arteries, associated with ANCA positivity. Both diseases can affect multiple organ systems and have variable clinical manifestations. Differentiation is based on vessel size involvement and ANCA status according to the Chapel Hill consensus criteria.
Churg Strauss Syndrome
A 59-year-old Thai woman presented with weakness in her arms and legs over the past week. She had numbness that started in her right toes and progressed to involve her right leg up to the knee and left hand. She was diagnosed with inflamed nerves by a private hospital but showed no improvement with medication. Examination found weakness and impaired sensation in her extremities. Tests showed eosinophilia, positive P-ANCA, and nerve biopsy demonstrated vasculitis. She was diagnosed with Churg-Strauss syndrome.
Nuclear Imaging In Cardiology Cme
Nuclear imaging techniques have various applications in cardiology, including assessing coronary artery disease, left ventricular function, cardiomyopathy, valvular heart disease, cardiac shunts, pulmonary hypertension, and more. Myocardial perfusion imaging can accurately diagnose and assess the prognosis of coronary artery disease, viability after myocardial infarction, and effectiveness of revascularization procedures. Gated SPECT allows evaluation of both cardiac function and perfusion simultaneously. Other nuclear techniques help evaluate conditions like myocarditis, pulmonary embolism, and secondary causes of hypertension.
Hypoxia, platypnea orthodeoxia and transient ischemic attacks in a patient
This document summarizes the case of a 43-year-old man presenting with dyspnea, fatigue, flushing, and diarrhea for 4 months. Tests revealed carcinoid syndrome caused by a primary ileal tumor with liver metastases. He underwent tumor resection but later experienced a transient ischemic attack and worsening dyspnea due to a patent foramen ovale allowing a right-to-left shunt. Closure of the PFO with an Amplatzer septal occluder normalized gas exchange and improved symptoms. Follow-up showed no readmissions for dyspnea or further TIAs, with the patient maintaining good exercise tolerance.
Superior vena cava syndrome
This document discusses Superior Vena Cava Syndrome (SVCS), which occurs when the Superior Vena Cava (SVC) is compressed, reducing blood flow from the head and upper body. The document covers the case presentation of a patient with SVCS, including symptoms, imaging findings, and treatment. It then provides details on the anatomy, etiologies, clinical features, imaging and classification of SVCS. Treatment options for malignant causes of SVCS such as radiation therapy, chemotherapy and stenting are described. A grading system and treatment algorithm for SVCS are also presented.
Features of Inflammatory Markers in Patients With Coronary Heart Disease
Features of Inflammatory Markers in Patients With Coronary Heart DiseaseHealthcare and Medical Sciences
Aim: of the study was to conduct a comparative analysis of inflammatory markers in patients with coronary heart disease of stable and unstable flow. Methods: 78 patients aged 36 to 75 years were enrolled in this study (mean age 58.2±12.6 years). Laboratory and instrumental data were obtained and assessed. IL-6, TNF-α in blood plasma was carried out by the method of enzyme immunoassay on a solid-phase analyzer «Humareader Single». Statistical processing of the obtained results was carried out using vibrational statistics methods recommended for biomedical research on the IBM PC AT Pentium IV. Results: In patients with unstable angina (UA), the frequency of elevated levels of CRP, TNF-α, and leukocytes was statistically significantly higher than in the group with stable ischemic heart disease (P<0.05). The mean levels of these markers were statistically significantly higher in patients with UA compared with patients with stable form of coronary heart disease (CHD, P<0.05): CRP (4.3 ± 2.4 and 2.9 ± 2.3 mg / L, p <0.05, respectively), TNF-α (10.5 ± 2.5 and 7.7 ± 3.4 pg / ml, p <0.05) and leukocytes (9.2 ± 2.5 6.9 ± 2.3x109 / l, p <0.05). The level of interleukin-6 in patients with UA was higher in comparison with patients with stable angina (SA, 3.4 ± 1.7 and 2.9 ± 0.5 pg/ml), but the difference was statistically not significant (p> 0.05 ). There were no significant differences in the level of fibrinogen and ESR between patients with UA and SA. Conclusion: It was noted that the signs of inflammation are detected both in patients with unstable forms and in patients with stable form of CHD, but the degree of inflammation in patients with UA (level of TNF-α, CRP and leukocytes) is higher than in patients with stable ischemic heart disease.Update on Pulmonary Arterial Hypertension in Scleroderma
Dr. Roberto Machado from the University of Illinois at Chicago presented an update on PAH at a Patient Education Conference on March 15, 2014 hosted by the Scleroderma Foundation, Greater Chicago Chapter.
Carotid stenosis (cqc)
This document discusses evidence for performing carotid endarterectomy (CEA) on asymptomatic patients with severe carotid artery stenosis. Three major trials from 1993-2004 found CEA reduced future stroke risk in asymptomatic patients with 60-99% stenosis by 38-53% compared to medical management alone. However, the benefits were smaller than for symptomatic patients and varied between patient subgroups. Current guidelines state CEA may be considered for asymptomatic high-grade stenosis if the surgeon has a perioperative stroke/death rate below 3%. Individual patient factors must be weighed.
Similar to Beyond amyloidosis a case of cardiac light chain deposition disease (20)
Stroke Epilepsy after Thrombolysis RTPA Cortical Involvement
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Should functional mr be fixed in heart failure
This document discusses functional mitral regurgitation (FMR) in heart failure patients. It presents evidence that even mild FMR results in poor survival outcomes, and that FMR is not just a late marker but also a cause of worse prognosis. Surgical mitral repair using a small, complete, rigid ring to reduce the mitral annulus has been shown to improve survival, ventricular remodeling, and functional status compared to no repair or incomplete repairs that do not fully correct FMR. Ongoing studies are exploring newer percutaneous approaches to treating FMR, but surgical repair remains the standard treatment when anatomically feasible to fully correct FMR.
Aortic Valve Stenosis with low EF : TAVR versus Replacement
1) Patients with low ejection fraction (EF < 50%) and severe aortic stenosis who undergo transcatheter aortic valve replacement (TAVI) have similar mortality at 1 year compared to those with higher EF, despite being higher risk.
2) TAVI is associated with significant improvements in EF, symptoms, and quality of life over 1 year in patients with very low EF (≤30%). However, mortality remains higher compared to those with EF >30%.
3) Both TAVI and surgical aortic valve replacement (SAVR) are associated with improvements in EF at 3 months in propensity matched populations with low EF. Short term outcomes are similar, but TAVI is associated with more pacemakers
When is less more minimally invasive surgery in low ef
The document discusses treatment options for patients with reduced ejection fraction and secondary mitral regurgitation, including the use of minimally invasive mitral valve surgery which can be considered for elderly patients or those with comorbidities. It presents a case study of a 69-year-old male with severe secondary mitral regurgitation who underwent a minimally invasive mitral valve repair which eliminated his mitral regurgitation and improved his symptoms and ejection fraction. Long-term data on isolated mitral valve surgery in patients with reduced ejection fraction shows improvement in mitral regurgitation and functional status with no difference in survival between repair and replacement
When to consider tricuspid valve repair
The document discusses when to consider tricuspid valve repair. Tricuspid regurgitation is associated with poor prognosis, especially when secondary to left-sided heart lesions, cardiomyopathy, pulmonary hypertension, or in the setting of LVAD placement or heart transplantation. Tricuspid valve repair is indicated for severe, symptomatic primary tricuspid regurgitation, and may also be considered for significant functional regurgitation concurrent with mitral valve surgery, after isolated mitral valve surgery if regurgitation is severe, or when placing a continuous-flow LVAD. Prophylactic tricuspid annuloplasty during heart transplantation reduces the severity of post-operative regurgitation and is associated with improved long-term survival
Cad and low ef does viability assessment matter
This document discusses the value of viability studies in patients with coronary artery disease (CAD) and low ejection fraction (EF). It summarizes several studies on the topic and discusses their limitations. A key trial was the STICH trial, which found no significant difference in outcomes between revascularization and medical therapy alone, challenging the belief that revascularization benefits those with viable myocardium. The document concludes that while viability concepts are biologically plausible, recent trials create confusion and there is no consensus on how to apply viability testing in practice.
Multimodality imaging.
This document discusses the conundrum of managing mitral regurgitation (MR) in patients with heart failure. It highlights the importance of using multimodality imaging to:
1) Assess the severity of MR at rest and with exercise to determine risk and need for intervention.
2) Evaluate left ventricular function, dyssynchrony, viability and ischemia to determine indications for cardiac resynchronization therapy or revascularization.
3) Assess left ventricular remodeling and mitral valve deformation to predict risk of recurrent MR after repair and determine the best repair/replacement option.
Imaging provides essential information to optimize treatment strategies for MR in heart failure.
The complex patient vad transplant exchange or hospice
This document discusses the case of a 76-year-old man presenting with heart failure symptoms and recurrent cough syncope. Testing revealed cardiac amyloidosis due to a TTR gene mutation. For patients with TTR amyloidosis, treatment options include organ transplantation, TTR stabilizers in clinical trials, or enrollment in hospice. Given his age and comorbidities, the man's options included a left ventricular assist device, extended criteria transplant, or a clinical trial for TTR amyloidosis treatment. He was ultimately listed and transplanted, and has since recovered well.
The complex patient vad transplant exchange or hospice
This document discusses the case of a 76-year-old man presenting with heart failure symptoms and recurrent cough syncope. Testing revealed cardiac amyloidosis due to a TTR gene mutation. For patients with TTR amyloidosis, treatment options include organ transplantation, TTR stabilizers in clinical trials, or enrollment in hospice. Given his age and comorbidities, the man's options included a left ventricular assist device, extended criteria transplant, or a clinical trial for TTR amyloidosis treatment. He was ultimately listed and transplanted within a month, and has since recovered well.
Surgical director heart transplant and mechanical assist device program
The document discusses a 55-year-old female patient with a history of rheumatic valve disease and multiple prior heart surgeries who was admitted with recurrent heart failure and an ejection fraction below 10%, outlining her medical history and current status, treatment options, and clinical course including optimization, a redo aortic valve replacement and implantation of a HeartMate II left ventricular assist device.
The complex patient vad ransplant vad exchange or hospice
L.B. is a 62-year-old man with a long history of coronary artery disease and heart failure who has undergone multiple coronary bypass surgeries. He now has an ejection fraction of 25% and intractable ventricular tachycardia despite medical management. Due to the risks of ablation, the team is considering options like hospice care, cardiac transplantation, VAD implantation, or VAD exchange to treat his advanced heart failure. A cardiac catheterization revealed multiple occluded arteries and stenoses. Given his medical history and surgical history, the team must determine the best treatment approach for his condition.
The road ahead.
This document discusses the management of mitral regurgitation (MR) in heart failure patients. It explores the differences between primary and functional (secondary) MR, and notes that correcting primary MR may improve outcomes but the benefits are less clear for functional MR which is primarily a ventricular problem. The document reviews potential management options for MR in heart failure including optimal medical therapy, cardiac resynchronization therapy, surgery, and percutaneous techniques such as the MitraClip system. It presents evidence from studies on the acute effects of CRT and the impact of CRT on functional MR severity. It also discusses guidelines on indications for mitral valve surgery in chronic secondary MR and barriers to surgery.
Whom to refer for mitral valve repair and whom not
This document discusses the treatment of mitral regurgitation in patients with heart failure. It describes the mechanisms of functional and ischemic mitral regurgitation. While medical therapy can improve symptoms and survival, cardiac resynchronization therapy may also help reduce mitral regurgitation severity and improve outcomes. Surgery to repair the mitral valve is an option but the risk of recurrence of mitral regurgitation is high, especially with more advanced left ventricular remodeling. Randomized trials are still needed to determine whether surgical correction provides clear benefits over medical therapy alone in high-risk patients. Percutaneous mitral valve repair may be a lower risk option for inoperable patients to reduce symptoms.
Devices and intervention in heart failure.
- The document discusses the speaker's receipt of honoraria and research support from numerous pharmaceutical and device companies.
- It summarizes several journal articles and studies related to left ventricular remodeling post-myocardial infarction, baroreflex activation therapy for heart failure, and the effects of bi-ventricular pacing on left ventricular ejection fraction and end-systolic volume.
- Key findings from the PACE trial are highlighted showing improvements in left ventricular ejection fraction and end-systolic volume up to 2 years with bi-ventricular pacing compared to right ventricular pacing alone.
European Journal of Heart Failure's year in Cardiology
This document contains information about Prof. Fausto J. Pinto who is the Head of Cardiology at University Hospital Sta Maria-HPV and University of Lisbon in Portugal. It discloses that he has received consultancy fees and lecture fees from various pharmaceutical companies. It also contains several figures and images from various medical studies and publications related to cardiology.
The EHJ's and EJHF's Year in Cardiology
This document lists the collaborations and conflicts of interest for speakers Thomas F. Lüscher and Marco Metra. It notes that they have received research grants, educational grants, and honoraria from numerous pharmaceutical companies. The rest of the document discusses the European Heart Journal, including new associate editors, submission rates and acceptance rates, impact factors, and plans to launch new open access and supplement journals.
Acute and advanced heart failure.
This document summarizes a presentation on cardiology topics including acute and advanced heart failure. It discusses trends in heart failure hospitalizations and mortality. It describes different hemodynamic profiles in acute heart failure patients and their corresponding treatments. It also discusses topics like iron deficiency in heart failure, sleep disordered breathing, and a study showing sleep disordered breathing is common in acute heart failure and predicts mortality.
Prevention is the best treatment
This document discusses the importance of prevention in treating cardiovascular disease. It outlines stages of heart failure progression from asymptomatic left ventricular dysfunction to refractory heart failure. Clinical trials show benefits of treating hypertension and post-MI left ventricular dysfunction to prevent heart failure. Treatment with ACE inhibitors reduces mortality and morbidity from heart failure. Prevention of risk factors is emphasized as the best strategy to avoid full-blown heart disease.
Can we afford heart failure management in the future
Heart failure is a major global health problem, affecting 26 million people worldwide. It accounts for 1-3% of hospital admissions in Europe and North America. Hospitalization is the main driver of the high economic costs of heart failure management, which is estimated to rise significantly in the coming decade. To better manage heart failure costs in the future, new models of coordinated and integrated care will need to be implemented, with a focus on preventing hospitalizations and readmissions through improved education, care transitions, and treatment of comorbidities.
The deadly statistics of heart failure.
This document discusses statistics related to heart failure. It summarizes data on outcomes for hospitalized heart failure patients compared to chronic heart failure patients. Hospitalized patients generally have worse outcomes, with high 1-year mortality rates around 25-27%. Chronic heart failure patients have lower but still significant 1-year mortality rates of around 5-6%. The document also reviews real-world data showing high readmission rates after hospitalization for heart failure. It concludes that while treatments for chronic heart failure with reduced ejection fraction have improved outcomes over decades, more efforts are still needed to improve care and outcomes for hospitalized patients and those with preserved ejection fraction.
The heart failure association global awareness programme.
The Global Heart Failure Awareness Programme aims to make the prevention and management of heart failure a global health priority. It is led by the Heart Failure Association of the European Society of Cardiology and supported by educational grants. The programme's objectives are to build a common global approach to raising awareness of heart failure among targeted audiences and to call for heart failure to be a health priority in every country. It plans to achieve this through developing educational content, building advocacy coalitions, and implementing initiatives at the national and local levels between 2015 and 2017.
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- 2. 48 y.o. Man, no CV risk factors Worsening dyspnoea (NYHA III) LVEF 26% Hypertrophy Pleural effusion T2-TRIPLE Oedema LGE Midwall/subepic. fibrosis
- 3. Blood analyses CRP 0.41 mg/dL eGFR 71 mL/min/1.73m2 HS-TnT 9.42 ng/L NT-proBNP 2,348 ng/L Negative rheumatological and microbiological analyses Negative coronary angiography ECG Chest X-ray Echocardiogram
- 4. Serum protein: 7.84 g/dL Gamma band: 20.9% Immunofixation: IgG lambda monoclonal component Lambda free light chain: 282 mg/dl (r.v. 90-210) Cardiac amyloidosis? Negative Congo Red staining - heart - bone marrow - periumbilical fat Toor AA et al, 2006 Immunohistochemistry Light chain immunoreactivity (λ/k 2:1), ++ in the fibrotic areas Light chain deposition disease
- 5. bisoprolol – ramipril – spironolactone – furosemide levosimendan – ICD implantation Clinical worsening, hypotention, atrial fibrillation NT-proBNP 16,587 ng/L eGFR 34 mL/min/1.73m2 Pre-HTC evaluation ECMO death Worsening of sistolic and diastolic LV function