Endomyocardial biopsy in the clinical context: current indications and challenging scenarios

Introduction Primary cardiac lymphoma is an extremely rare malignancy involving the heart and pericardium. It is a disease that most commonly effects the right atrium and right ventricle. Left untreated it carries a very poor prognosis. Recent advancements in therapy including early recognition and initiation of chemotherapy has led to improved survival. Case Presentation A 78 year old female presented with weight loss, abdominal pain and distension. An ultrasound abdomen pre admission showed abdominal ascites. An echocardiogram performed during admission showed a large pericardial effusion with asymmetrical increase in left ventricular wall thickness and a new left atrial mass. Pericardial fluid analysis led to the diagnosis of diffuse large B cell non-Hodgkin’s lymphoma. Positron Emission Tomography CT (PET-CT) shows avid fluorodeoxyglucose (FDG) uptake in cardiac muscle. Prompt treatment was initiated with a chemotherapy regimen involving Rituximab, Cyclophosphamide, doxorubicin, vincristine, and prednisolone (R-CHOP). An echocardiogram 3 months post initiation of treatment showed resolution of the pericardial effusion and left atrial mass as well as an improvement in left ventricular wall thickness. A PET-CT following completion of treatment showed complete metabolic response. Discussion The diagnosis of primary cardiac lymphoma is uncommon and the presence of left rather than right heart involvement is rarer still. Multimodality imaging is key in diagnosis. Early recognition and treatment is vital in improving associated morbidity and mortality.

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“…With regards to cardiac masses, EMB is not indicated in tumours with a high embolic potential such as those that are left sided. 7 , 8 …”

Section: Discussionmentioning

confidence: 99%

Primary cardiac lymphoma: a case report

Saghie

Higgisson

2023

European Heart Journal - Case Reports

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“…In the contemporary cohort, ATTR-CA was associated with more favorable global and cardiovascular outcomes compared to AL-CA, reflecting the major advances in earlier diagnosis and treatment, especially for ATTR amyloidosis ( Figure 4 ). In 2016, a landmark study by Gillmore et al ( 17 ) paved the way for the clinical application of bone scintigraphy for the non-invasive diagnosis of ATTR-CA, demonstrating that the positive predictive value of a moderate-high myocardial uptake approaches 100% in the absence of a monoclonal protein in serum and urine, thus limiting the need for EMB to selected cases ( 31 , 32 ). Broadening the diagnostic horizon of CA, predominantly ATTR-CA ( 9 ), resulted in recognition of more patients in different stages of cardiac disease and in improved overall survival in the contemporary cohort compared to AL-CA patients ( Figure 3 ).…”

Section: Discussionmentioning

confidence: 99%

Evolving trends in epidemiology and natural history of cardiac amyloidosis: 30-year experience from a tertiary referral center for cardiomyopathies

Porcari

Allegro

Saro

et al. 2022

Front. Cardiovasc. Med.

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ObjectiveNatural history of cardiac amyloidosis (CA) is poorly understood. We aimed to examine the changing mortality of different types of CA over a 30-year period.Patients and methodsConsecutive patients included in the “Trieste CA Registry” from January 1, 1990 through December 31, 2021 were divided into a historical cohort (diagnosed before 2016) and a contemporary cohort (diagnosed after 2016). Light chain (AL), transthyretin (ATTR) and other forms of CA were defined according to international recommendations. The primary and secondary outcome measures were all-cause mortality and cardiac death, respectively.ResultsWe enrolled 182 patients: 47.3% AL-CA, 44.5% ATTR-CA, 8.2% other etiologies. The number of patients diagnosed with AL and ATTR-CA progressively increased over time, mostly ATTR-CA patients (from 21% before 2016 to 67% after 2016) diagnosed non-invasively. The more consistent increase in event-rate was observed in the long-term (after 50 months) in ATTR-CA compared to the early increase in mortality in AL-CA. In the contemporary cohort, during a median follow up of 16 [4–30] months, ATTR-CA was associated with improved overall and cardiac survival compared to AL-CA. At multivariable analysis, ATTR-CA (HR 0.42, p = 0.03), eGFR (HR 0.98, p = 0.033) and ACE-inhibitor therapy (HR 0.24, p < 0.001) predicted overall survival in the contemporary cohort.ConclusionIncidence and prevalence rates of ATTR-CA and, to a less extent, of AL-CA have been increasing over time, with significant improvements in 2-year survival of ATTR-CA patients from the contemporary cohort. Reaching an early diagnosis and starting disease-modifying treatments will improve long-term survival in CA.

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“…As such, in real-world clinical practice, CA is typically diagnosed using cMRI, scintigraphy, and positron emission tomography, while EMB is reserved for clinically ambiguous cases where patients present with mild or moderate uptake of 99m Tc-PYP. Alternatively, biopsy samples taken from less invasive regions can spare patients from EMB, where possible [ 65 , 66 ].…”

Section: Definitive Diagnosismentioning

confidence: 99%

Detection and Diagnosis of Cardiac Amyloidosis in Egypt

et al. 2023

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Cardiac amyloidosis is a life-threatening disease that occurs when amyloid proteins, most commonly immunoglobulin light chain or transthyretin, mutate or become unstable, misfold, deposit as amyloid fibrils, and accumulate in the myocardium. Early diagnosis of cardiac amyloidosis is hindered by insufficient awareness, specifically regarding clinical red flags and diagnostic pathways. Cardiac amyloidosis diagnosis comprises two important phases, clinical suspicion (phase one) followed by definitive diagnosis (phase two). Each phase is associated with specific clinical techniques. For example, clinical features, electrocardiography, echocardiography, and cardiac magnetic resonance imaging serve to raise suspicion of cardiac amyloidosis and facilitate early diagnosis, whereas laboratory tests (i.e., blood or urine electrophoresis with immunofixation), biopsy, scintigraphy-based nuclear imaging, and genetic testing provide a definitive diagnosis of cardiac amyloidosis. In Egypt, both the lack of cardiac amyloidosis awareness amongst healthcare providers and the unavailability of clinical expertise for the use of diagnostic techniques must be overcome to improve the prognosis of cardiac amyloidosis in the region. Previously published diagnostic algorithms for cardiac amyloidosis have amalgamated techniques that can raise clinical suspicions of cardiac amyloidosis with those that definitively diagnose cardiac amyloidosis. Though such algorithms have been successful in developed countries, diagnostic tools like echocardiography, scintigraphy, and cardiac magnetic resonance imaging are not ubiquitously available across Egyptian facilities. This review presents the current state of knowledge regarding cardiac amyloidosis in Egypt and outlines a new diagnostic algorithm which leverages regional nuclear imaging expertise. Importantly, the proposed diagnostic algorithm guides accurate amyloid-typing to mitigate misdiagnosis and erroneous treatment selection and improve the cardiac amyloidosis diagnostic accuracy in Egypt.

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Endomyocardial biopsy in the clinical context: current indications and challenging scenarios

Cited by 36 publications

References 52 publications

Primary cardiac lymphoma: a case report

Primary cardiac lymphoma: a case report

Evolving trends in epidemiology and natural history of cardiac amyloidosis: 30-year experience from a tertiary referral center for cardiomyopathies

Detection and Diagnosis of Cardiac Amyloidosis in Egypt

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