Secondary Cardiac Involvement Research Articles

Targeted genetic therapies for inherited disorders that affect both cardiac and skeletal muscle.

Skeletal myopathies and ataxias with secondary cardiac involvement are complex, progressive and debilitating conditions. As life expectancy increases across these conditions, cardiac involvement often becomes more prominent. This highlights the need for targeted therapies that address these evolving cardiac pathologies. Musculopathies by and large lack cures that directly target the genetic basis of the diseases; however, as our understanding of the genetic causes of these conditions has evolved, it has become tractable to develop targeted therapies using biologics, to design precision approaches to target the primary genetic causes of these varied diseases. Using the examples of Duchenne muscular dystrophy, Friedreich ataxia and Pompe disease, we discuss how the genetic causes of such diseases derail diverse homeostatic, energetic and signalling pathways, which span multiple cellular systems in varied tissues across the body. We outline existing therapeutics and treatments in the context of emerging novel genetic approaches. We discuss the hurdles that the field must overcome to deliver targeted therapies across the many tissue types affected in primary myopathies.

COVID-19 AND NEGLECT OF CARDIOVASCULAR DISEASES TREATMENT

Coronavirus was first reported as an acute respiratory syndrome in December 2019 in Wuhan, China. This infectious disease spreads rapidly in many countries , and humans worldwide, shows different symptoms of the disease. High fever, cough, and fatigue were the most common symptoms that patients with covid-19 showed.1 The COVID-19 pandemic has created unprecedented challenges for health care in many countries worldwide. In addition to patients, COVID-19 is very dangerous for health care staff at the frontline of fighting this disease. The large number of COVID-19 virus patients who need to be hospitalized puts a heavy burden on the health system of countries so the treatment of other diseases is seriously disrupted.2 Cardiovascular disease is one of the most important diseases that has been neglected due to the focus of the Covid-19 pandemic and its special conditions, including completing the capacity of clinical departments and full-time participation of hospital staff.3 Among various diseases, cardiovascular diseases are the most common and the main cause of death in the world (Estimated 17.9 million death each year) and the most important factor of disability (Generally, about eleven percent of the total disease burden).4 Despite significant advances in the field of medicine, one-third of patients who suffer from a heart attack (myocardial infarction) still die, and two-thirds of those who never survive fully recover and are unable to lead normal lives.5 There is a close relationship between COVID-19 and cardiovascular diseases so cardiovascular diseases are more common in patients with COVID-19, and these patients are at higher risk of death.6 Cardiovascular disease is associated with more severe symptoms of COVID-19 and higher mortality rates. COVID-19 can have primary cardiac involvement (arrhythmia, myocardial infarction, and myocarditis) and secondary cardiac involvement (myocardial damage/elevated biomarkers and heart failure). COVID-19 can have several effects on the cardiovascular system of patients. Acute cardiovascular complications can cause many challenges. For example, one of these challenges can be the need for a multidisciplinary heart and lung team to assess the condition of patients with severe symptoms.7,8 Currently, the outbreak of COVID-19 disease has created unprecedented pressure on patients, physicians, and in general, the health care systems of the world and led to delays in the treatment of cardiovascular patients. Delay or neglect of treatment of cardiovascular patients is associated with inevitable complications and mortality. Because the living conditions of many of them are threatening and dangerous.9 Some early signs of cardiovascular disease (shortness of breath, chest pain) in patients may be confused with COVID-19 symptoms, and the patient may be misdiagnosed.10 The COVID-19 pandemic has faced the world in a difficult position. Hospitals and health centers have been severely affected by treating patients with COVID-19. Governments are attempting to prevent further outbreak of this disease effectively. Of course, the important point is that governments should not neglect other health priorities, such as non-contagious diseases and cardiovascular diseases.11 While many efforts of medical staff to fight COVID-19 are understandable, hospitals should not forget their primary role in caring for other patients and neglecting their essential needs. Neglecting cardiovascular diseases such as congenital heart disease (CHD) and acute coronary artery syndrome will lead to irreparable damage.12 Health care systems must establish the right balance between treating patients with COVID-19 and treating cardiovascular diseases.13 It is suggested that to better control COVID-19 and balance the management of other diseases, only a certain hospital capacity should be allocated to this disease. Due to the lack of definitive treatment for COVID-19, treatment for other diseases should not be delayed.

Management of diffuse large B-cell lymphoma with cardiac and extra-cardiac locations.

A 71-year-old man presented with 6 kg weight loss since 4 months and asthenia. He also had mild cough for a few weeks, sudden stabbing thoracic pain recurring at rest without radiation, and no dyspnea. The patient had a history of chronic weaned alcoholism, active smoking, hypertension on triple therapy, and professional exposure to paint. The last transthoracic echocardiography (TTE) performed 6 months before his admission showed an isolated septal hypertrophy at 13 mm without supplementary cardiac abnormalities. At admission, hemoglobin was 138 g/L, platelet and leukocyte counts were 369 × 109/L and 10.1 × 109/L, respectively, C-reactive protein was 51 mg/L, lactate dehydrogenase (LDH) was 8.57 μkat/L, and troponin I was 86.6 μg/L. Neither clinical nor electrocardiogram additional abnormalities were identified. TTE exhibited a significant necrotic intracardiac mass. Computed tomography (CT) highlighted an 80 × 80 mm infiltrating heart mass centered on the right atrioventricular septum with extension to the tricuspid ring (Figure 1, left image), a mediastino-hilar lymphadenopathy, a 15 mm right posterobasal pulmonary nodule, and a thin layer of right pleural effusion. A biopsy of the mediastinal lesion by endoscopic ultrasound revealed large B cells, strongly positive for CD20 antigen as well as CD19, CD5 (low intensity), CD10, CD38, CD79b, and FMC7 marker proteins with a Ki-67 of 70%. Although biopsy was not large, the pathological diagnosis suggested a diffuse large B-cell lymphoma. During hospitalization, a repeated CT showed increased necrotic areas in mediastinal mass, layer of right pericardial effusion, right posterobasal pulmonary nodule, bilateral pleural effusions with passive atelectasis precluding another biopsy, and hepatosplenomegaly. After occurrence of second-degree atrioventricular (AV) block, a pacemaker was implanted in the right ventricular apex. Overall, these clinical, radiological, and histological observations were consistent with diagnosis of diffuse large B-cell lymphoma (DLBCL). Due to the accelerated progression of disease, patient started a first chemotherapy cycle without definitive histological diagnosis including intravenous cyclophosphamide, vincristine, and prednisone without anthracycline. Following six cycles of R-CHOP (Rituximab, Cyclophosphamide, Hydroxy-doxorubicin, Vincristine and Prednisone) combination regimen for treatment of DLBCL with extranodal involvement, CT scan showed highly significant regression of cardiac tumors masses (Figure 1, right image artifacted due to the pacemaker) as well as pleural effusions, lymphadenopathy, pulmonary mass, and hepatosplenomegaly. A complete metabolic response was highlighted by positron emission tomography 8.6 months after the first day of CVP. To date, both primary and secondary cardiac lymphomas (CL) remain aggressive diseases, reported in less than 1% and 10% of patients with DLBCL, respectively. We acknowledged that additional sampling procedures were precluded by the concurrence of rapid progression of DLBCL in a few weeks associated with the onset of high-grade AV block. Therefore, it remained difficult to definitively establish the primary origin of CL versus secondary cardiac involvement of lymphoma, although timeline of clinical and radiological events and the histopathological data argue for a primary CL in the first instance. Despite an improvement of cardiac lymphoma diagnosis through multimodal imaging, biopsy techniques, and chemotherapy regimen including rituximab, the prognosis of CL patients remains poor. The authors declare they have no conflicts of interest. Written informed consent was obtained from the patient. The data that support the findings of this study are available from the corresponding author upon reasonable request.

Cardiovascular manifestations secondary to COVID-19: A narrative review

The coronavirus disease 2019 (COVID-19) pandemic has spread rapidly, becoming a major threat to global health. In addition to having required the adaptation of healthcare workers for almost 2 years, it has been much talked about, both in the media and among the scientific community. Beyond lung damage and respiratory symptoms, the involvement of the cardiovascular system largely explains COVID-19 morbimortality. In this review, we emphasize that cardiovascular involvement is common and is associated with a worse prognosis, and that earlier detection by physicians should lead to better management. First, direct cardiac involvement will be discussed, in the form of COVID-19 myocarditis, then secondary cardiac involvement, such as myocardial injury, myocardial infarction and arrhythmias, will be considered. Finally, worsening of previous cardiovascular disease as a result of COVID-19 will be examined, as well as long-term COVID-19 effects and cardiovascular complications of COVID-19 vaccines.

C15. Biventricular Heart Failure and Pulmonary Hypertension in Systemic Sclerosis: a rare and unusual case report

Abstract Backgrounds Systemic sclerosis (SSc), chronic multisystem autoimmune disease, characterized by excessive fibrosis and vascular endothelial dysfunction. Cardiovascular involvement may be induced by various pathways, either primary or secondary to pulmonary hypertension or renal involvement. Case Summary A 46 years old woman presented to emergency department with cold, dark purplish ulcers in fingertips with progressive worsening pain in upper extremities, claudication intermitten, and dyspneu since 4 months ago. She had a mask-like facial, shiny skin, microstomia, restricted mouth opening, calcinosis cutis, Raynauds phenomenon, and sclerodactyly. There were pansystolic murmur grade III/6 in tricuspid area, and rales in basal lungs. ECG showed sinus rhythm with fragmented QRS, poor R-wave progression. Chest X-Ray revealed pancardiomegaly with pleural effusion and HRCT thorax revealed interstitial lungs disease. The laboratory revealed leucocytosis, increase D-dimer (870 ug/L), negative ANA and ds-DNA test, positive anti SCl test. Echocardiography showed biventricular dysfunction with decreased LVEF 37% (Teichz) and TAPSE 10 mm, RA RV dilatation, severe TR with high probability for PH. Extremities Doppler showed normal arteries and veins system. These findings indicates that primary and secondary cardiac involvement in SSc. Heart failure, pulmonary hypertension, and cyclophosphamide medication were administered Discussion Primary cardiac involvement, a direct consequence of SSc, may manifest as fibrosis of the myocardial, conduction system, pericardial and, valvular disease. Furthermore, a secondary phenomenon due to pulmonary arterial hypertension (PAH), and kidney pathology. Though the burden remains low, it may worsen the outcome, consequently, all patients with SSc should undergo screening.

C15. Biventricular Heart Failure and Pulmonary Hypertension in Systemic Sclerosis: a rare and unusual case report

Abstract Backgrounds Systemic sclerosis (SSc), chronic multisystem autoimmune disease, characterized by excessive fibrosis and vascular endothelial dysfunction. Cardiovascular involvement may be induced by various pathways, either primary or secondary to pulmonary hypertension or renal involvement. Case Summary A 46 years old woman presented to emergency department with cold, dark purplish ulcers in fingertips with progressive worsening pain in upper extremities, claudication intermitten, and dyspneu since 4 months ago. She had a mask-like facial, shiny skin, microstomia, restricted mouth opening, calcinosis cutis, Raynauds phenomenon, and sclerodactyly. There were pansystolic murmur grade III/6 in tricuspid area, and rales in basal lungs. ECG showed sinus rhythm with fragmented QRS, poor R-wave progression. Chest X-Ray revealed pancardiomegaly with pleural effusion and HRCT thorax revealed interstitial lungs disease. The laboratory revealed leucocytosis, increase D-dimer (870 ug/L), negative ANA and ds-DNA test, positive anti SCl test. Echocardiography showed biventricular dysfunction with decreased LVEF 37% (Teichz) and TAPSE 10 mm, RA RV dilatation, severe TR with high probability for PH. Extremities Doppler showed normal arteries and veins system. These findings indicates that primary and secondary cardiac involvement in SSc. Heart failure, pulmonary hypertension, and cyclophosphamide medication were administered Discussion Primary cardiac involvement, a direct consequence of SSc, may manifest as fibrosis of the myocardial, conduction system, pericardial and, valvular disease. Furthermore, a secondary phenomenon due to pulmonary arterial hypertension (PAH), and kidney pathology. Though the burden remains low, it may worsen the outcome, consequently, all patients with SSc should undergo screening.

Long term prognosis and cardiovascular complications of patients with systemic sclerosis-related cardiomiopathy

Abstract Background Both primary (SSc related cardiomyopathy) and secondary cardiac involvement in systemic sclerosis (SSc) is frequent albeit mostly asymptomatic. It represents a negative prognostic factor as almost 25% of SSc patients die from either heart failure or arrhythmia complications. Speckle tracking global longitudinal strain has been proven to be an effective tool both to identify and detect the progress of subclinical heart disease in SSc. The aim of our study was to assess the association between SSc diagnosis and the development of heart failure, pulmonary hypertension, death and need for hospitalization. Materials and methods We conducted an observational prospective study enrolling all patients with a diagnosis of SSc and no overt cardiac disease. We excluded all patients with a known diagnosis of pulmonary hypertension and atrial fibrillation. For each patient standard echocardiogram and GLS variables were collected. Results We enrolled 70 patients (61 females, age 56.2±15.4 years) who were followed for a median of 3 years. 68% of the patients had a limited variant of the disease. All-cause mortality was 10% in a 5-year follow-up. During the same period, PH was reached in 13% of all patients, HF in 7% and 18% required at least one hospital admission for cardiovascular causes. A diagnosis of PH was associated with an increased risk of death (ARR 34%; p<0.001) and hospitalization for CV causes (ARR 73%; p<0.001). Moreover, a diagnosis of HF was associated with an increased risk of death (ARR 50%; p<0.001) and hospitalization for CV causes (ARR 61%; p<0.001). A 1% worsening of GLS was associated with an increased risk to develop PH in the following 5 years, both for the left ventricle (OR 1.2; 95% CI: 1.1–1.4; p=0.043) and for the right ventricle (OR 1.1; 95% CI: 1.1–1.3; p=0.045). Conclusions Pulmonary hypertension and heart failure often occur in patients with cardiac involvement. Both proved themselves to be associated with an increased risk of death and hospitalization for CV causes. Moreover, GLS worsening of both the left and right ventricles may allow us to predict the diagnosis of PH and therefore preemptively start appropriate management. Funding Acknowledgement Type of funding sources: None.

Heart failure with preserved ejection fraction (HFpEF) pathophysiology study (IDENTIFY-HF): rise in arterial stiffness associates with HFpEF with increase in comorbidities

Abstract Background There has been a shift in paradigm proposing that comorbidities play a significant role towards the pathophysiology of the heart failure with preserved ejection fraction (HFpEF) syndrome. Further, HFpEF patients have abnormal macrovascular function, potentially contributing significantly in altered ventricular-vascular coupling in these patients. However, our full understanding of the role of comorbidities, arterial stiffness and it relationship with HFpEF remains incomplete. Purpose The IDENTIFY-HF study aims to shed light on the HFpEF pathophysiology and investigates whether gradually increase in arterial stiffness (in addition to ageing) due to increasing common comorbidities, such as hypertension and diabetes, is associated with HFpEF. Methods Arterial compliance was assessed in five groups (Groups A to E) matched for age, (≥70 years), sex and renal function: Group A; normal healthy volunteers without major comorbidities (control). Group B; patients with hypertension only. Group C; patients with hypertension and diabetes mellitus only. Group D; patients with HFpEF. Group E; patients with heart failure and reduced ejection fraction (HFrEF); the parallel group. Arterial compliance was assessed using pulse wave velocity (PWV), as the primary outcome measure and was compared between Group A to D. A separate comparison was made between Groups D and E. To avoid confounding factors, participants were asked to omit their morning blood pressure medication and abstain from caffeine for 12 hours prior to the study. Results From the 95 volunteers recruited, PWV was obtained in 94 subjects. The mean PWV in group A, B, C, D and E was 10.2-, 12.2-, 13.0-, 13.7- and 10.0 m/s respectively. After adjusting for covariance (age, sex, BMI and renal function), the mean difference between Group A (healthy volunteers) and D (HFpEF) was 2.14 m/s (p=0.023). Whilst the mean difference between the HFpEF and HFrEF group D and E respectively was 2.68 m/s (p=0.003). Conclusion Rise in comorbidities increases arterial stiffness, as measured by pulse wave velocity, which in turn significantly associates with HFpEF (p=0.023). It is therefore possible that the HFpEF syndrome may not be due to a primary cardiac pathology but rather an end-result of non-cardiac comorbidities affecting vascular resistance with perhaps some secondary cardiac involvement. Funding Acknowledgement Type of funding sources: Public Institution(s). Main funding source(s): 1)West Midlands Clinical Research Network, National Institute of Health Research, UK2)Research, Development & Innovation department of the University Hospitals Coventry & Warwickshire NHS Trust (RDI, UHCW), UK.

Case of Osseous Lymphoma Involving Bilateral Femur with Secondary Cardiac Involvement

We present a case of a 38-year-old male with osseous Diffuse large B-cell lymphoma (DLBCL) involving bilateral femur, with secondary cardiac dissemination involving right atrium and pericardium extending into SVC. The article discusses the osseous involvement of lymphoma and emphasizes the presence of cardiac involvement in the extra nodal lymphoma which may cause secondary symptoms like SVC obstruction. Such cases carry poor prognosis and require treatment modification and careful monitoring to avoid cardiac complications after initiation of chemotherapy. Only few such cases have been described in literature so far.

Secondary cardiac involvement in anti-SRP-antibody-positive myopathy: an 87-year-old woman with heart failure symptoms as the first clinical presentation

BackgroundNecrotizing myopathy (NM) is defined by the dominant pathological feature of necrosis of muscle fibers without substantial lymphocytic inflammatory infiltration. Anti-signal recognition particle (SRP)-antibody-positive myopathy is related to NM. Anti-SRP-antibody-positive myopathy can comorbid with other disorders in some patients, however, comorbidity with malignant tumor and myopericarditis has still not been reported.Case presentationAn 87-year-old woman with dyspnea on exertion and leg edema was referred to our hospital because of suspected heart failure and elevated serum creatine kinase level. Upon hospitalization, she developed muscle weakness predominantly in the proximal muscles. Muscle biopsy and immunological blood test led to the diagnosis of anti-SRP-antibody-positive myopathy. A colon carcinoma was also found and surgically removed. The muscle weakness remained despite the tumor resection and treatment with methylprednisolone. Cardiac screening revealed arrhythmia and diastolic dysfunction with pericardial effusion, which recovered with intravenous immunoglobulin (IVIg) treatment.ConclusionsWe reported the first case of anti-SRP-positive myopathy comorbid with colon carcinoma and myopericarditis. This case is rare in the point that heart failure symptoms were the first clinical presentation. The underlying mechanism is still not clear, however, physicians should be carefully aware of the neoplasm and cardiac involvement in anti-SRP-antibody positive-myopathy patients and should consider farther evaluation and management.

Electrocardiographic markers for the prediction of ventricular arrhythmias in patients with systemic sclerosis.

SSc is an autoimmune disease characterized by microvascular damage, endothelial dysfunction and fibrosis of the skin and the internal organs. Cardiac manifestation in patients with SSc is one of the major organ involvements. Approximately 20% of SSc patients suffer from primary cardiovascular disease and another 20% may have secondary cardiac involvement. Although cardiac arrhythmias are mostly linked to myocardial fibrosis, atrioventricular conduction abnormalities are secondary to the fibrosis of the pulse conduction system. Despite the severe consequences of ventricular rhythm disturbances in patients with SSc, the exact role of electrocardiographic markers in the prediction of these arrhythmias has not yet been clearly elucidated. Therefore, the question is whether certain ECG parameters reflecting ventricular repolarization may help to recognize scleroderma patients with increased risk for ventricular arrhythmias and sudden cardiac death.

Challenges in Remote Cardiac Care; a Rare Cardiac Tumour

Introduction: Synovial sarcoma is a rare soft tissue malignancy that may present with secondary cardiac involvement. Multi-modal imaging is necessary for diagnosis and management requires specialist medical and surgical input. Many Indigenous Australians reside in remote locations with limited services. Accessing necessary healthcare can involve travelling great distances away from family and familiar surroundings. This case report describes a rare case of cardiac tumour with the added challenges faced by an Indigenous patient living in a remote location. Case report: A 52 year old indigenous male presented to an outreach cardiology clinic in a remote community with increasing dyspnoea. A history of synovial sarcoma excision seven years prior was noted. Transthoracic echocardiography revealed a large mass within the left ventricle, extending from the lateral wall, filling a portion of the chamber. The patient subsequently travelled to the closest tertiary hospital, several hundred kilometres away, for further investigation. He was then required to travel interstate for cardiac magnetic resonance imaging (CMR) and consideration of treatment options. The CMR demonstrated findings of malignant tumour invasion of the myocardium, most consistent with metastatic synovial sarcoma. The mass was considered inoperable and the patient was referred back to his closest hospital for palliative treatment closer to his family and community. Conclusion: This report demonstrates a rare case of synovial sarcoma with cardiac metastasis and the imaging modalities involved, as well as highlighting the difficulties faced by those living in remote locations to obtain necessary healthcare.

ISOLATED MYOCARDIAL RECURRENCE OF ACUTE MYELOGENOUS LEUKEMIA DIAGNOSED BY PERCUTANEOUS ENDOMYOCARDIAL BIOPSY

While primary malignancies of the heart are rare, secondary cardiac involvement in patients with cancer is relatively common and should be considered when these patients develop cardiovascular symptoms. A 63-year-old man presented with dyspnea on exertion. He had a history of myelodysplastic

A rare case of primary cardiac B cell lymphoma

Primary cardiac lymphomas represent an extremely rare entity of extranodal lymphomas and should be distinguished from secondary cardiac involvement of disseminated lymphomas belonging to the non-Hodgkin’s classification of blood cancers. Only 90 cases have been reported in literature. Presentation of cardiac lymphomas on imaging studies may not be unambiguous since they potentially mimic other cardiac neoplasms including myxomas, angiosarcoma or rhadomyomas and therefore require multimodality cardiac imaging, endomyocardial biopsy, excisional intraoperative biopsy and pericardial fluid cytological evaluation to establish final diagnosis.Herein we report the case of a 70 y/o immunocompetent Caucasian female with a rapidly progressing superior vena cava syndrome secondary to a large primary cardiac diffuse large B cell lymphoma (NHL lymphoma) almost completely obstructing the right atrium, right ventricle and affecting both mitral and tricuspid valve. The patient had no clinical evidence of disseminated disease and was successfully treated with extensive debulking during open-heart surgery on cardiopulmonary bypass and 6 cycles of rituximab, cyclophosphamide, doxorubicin, vincristine and prednisone chemotherapy (R-CHOP).

Indicaciones de la biopsia cardiaca

Endomyocardial biopsy is a common procedure for the evaluation of cardiac tissue for transplant monitoring, myocarditis, drug toxicity, CMP, arrhythmia, secondary cardiac involvement by systemic diseases, and cardiac masses. It is the gold standard for the diagnosis of cardiac rejection, myocarditis, and infiltrative/storage disorders and can be crucial for differential diagnosis in heart failure and ventricular arrhythmias. Currently, in skilled hands, has a very low rate of serious complications. In the last years, several Scientific Societies have published consensus documents about its indications.

Cardiac involvement of malignant lymphoma and its improvement after chemotherapy

A 63-year-old female was admitted to the emergency room in our hospital due to chest oppression at rest. Physical examination showed no significant abnormal findings. Her heart rate was 92 beats/min and blood pressure was 158/92 mmHg. Laboratory findings showed no abnormal data. ECG on admission showed normal sinus rhythm with first-degree atrio-ventricular block and negative T wave in II, III, aVF, and low voltage in limb leads. Echocardiography revealed wall thickening in the infero-posterior segments of the left ventricle (LV) with wall motion abnormality, and wall thickening in intra-atrial septum and right atrium (Fig. 1). In echocardiograms, end-diastolic and end-systolic LV diameter, septal and posterior wall thickness, and ejection fraction were 40, 23, 9, 9 mm, and 50 %, respectively. Pulsed Doppler echocardiography showed pseudonormal pattern in transmitral flow [early diastolic and atrial wave velocity ratio (E/A) = 1.75, deceleration time = 113 ms]. Tissue Doppler echocardiography showed a ratio of early diastolic transmitral flow and mitral annulus velocity (E/E0) of 15.5. Coronary angiogram revealed no significant coronary stenosis. Computed tomography (CT) scan revealed (1) multiple masses in left iliopsoas muscle, (2) lymph node swelling in the paraaortic and right inguinal portions, and (3) wall thickening in the LV, right atrium, and intra-atrial septum. She was diagnosed as having diffuse large B cell lymphoma by biopsy from the lymph node in the right inguinal portion. Chemotherapy was performed (R-CHOP, 7 courses). Follow-up echocardiograms showed a decrease in cardiac mass after the second course of R-CHOP. ECG showed normal findings, and no apparent mass was found by CT scan. After the completion of the chemotherapy, echocardiography revealed no wall thickening and improvement in the inferior wall motion (Fig. 2). E/A was decreased from 1.75 to 0.87. E/E0 was decreased from 15.5 to 11.5. Secondary cardiac involvement of lymphoma has been reported as between 16 and 28 % of extracardiac lymphomas [1–3]. These results were obtained from autopsy studies. However, cardiac involvement is not commonly diagnosed before an autopsy [3–5] for the following reasons. It is usually asymptomatic or a nonspecific symptom in cardiac involvement in patients with lymphoma because most of the cardiac involvement of lymphoma is microscopic. ECG usually shows nonspecific findings in cardiac involvement. Symptoms and signs due to coexistent involvement of other organs may induce misdiagnosis of cardiac involvement. Myocardial or atrial wall thickening as shown in the present echocardiograms has been reported in previous reports of cardiac involvement of lymphoma [3, 4]. Infiltration of tumor cells in these lesions has been shown in the autopsy in the previous reports. Echocardiography revealed a decrease in myocardial and atrial wall thickening after 4 courses of chemotherapy, and no wall thickening after the completion of the chemotherapy, which was confirmed by CT scan. In comparison with CT, echocardiography is a completely noninvasive and feasible method. Thus, echocardiography is more useful than CT for the serial assessment of the involved mass in the heart after chemotherapy. This case shows the usefulness of echocardiography H. Ochi (&) M. Yamamoto M. Yamasaki Echocardiography Laboratory, Osaka Saiseikai Noe Hospital, 1-3-25 Furuich, Joutou-ku, Osaka 536-0001, Japan e-mail: hiroyuki18ochi@yahoo.co.jp

The management of malignant cardiac tumors: clinical considerations

Primary sarcomas of the heart are extremely rare, whereas secondary cardiac involvement with malignancy is relatively common, especially in the setting of widespread metastatic disease. Patients may present acutely with catastrophic hemodynamic instability, but presentation is more commonly nonspecific. The symptoms of primary cardiac sarcomas are often misattributed to more common conditions, and secondary cardiac involvement may be overlooked in the constellation of symptoms associated with widespread metastases. Initial diagnosis of cardiac malignancy is best made with echocardiography, but a more complete assessment of local and regional disease extent with CT and MRI is required when curative management is contemplated. Early diagnosis and radical excision are essential to long-term survival for primary cardiac sarcoma and can lead to long-term survival, although this is rarely accomplished and overall results are very disappointing. Neoadjuvant chemotherapy and radiotherapy have a limited role in primary management, but can be beneficial and should be considered for selected cases. Orthotopic cardiac transplantation is a controversial treatment for primary cardiac sarcoma, but has been associated with prolonged survival in selected cases. Secondary cardiac involvement with malignancy usually occurs at the terminal phase of a prolonged disease course, and palliative chemotherapy, radiotherapy, and surgery can provide temporary symptomatic relief. In the specific instance where secondary cardiac involvement leads to the initial diagnosis of malignancy, or is the first evidence of small-volume metastatic recurrence, aggressive surgical management may result in long-term survival, provided that all gross tumor can be excised. Management is always complex, and patients with malignant cardiac involvement should be referred to centers of specialized expertise for multidisciplinary assessment and treatment whenever possible.

Primary cardiac plasmablastic (diffuse large B-cell) lymphoma mimicking left ventricular aneurysm with mural thrombus

Primary cardiac lymphoma is rare and is usually of the non-Hodgkin type. By definition, it involves only the heart and the pericardium, with no evidence of extracardiac disease. Primary cardiac lymphoma accounts for 1% of primary cardiac tumors and 0.5% of extranodal lymphomas [Gowda RM, Kahn RA. Clinical perspectives of primary cardiac lymphoma. Angiology 2003;54(5):599-604]. On the other hand, secondary cardiac involvement can occur in approximately 20% of patients with disseminated extracardiac lymphoma [Gowda RM, Kahn RA. Clinical perspectives of primary cardiac lymphoma. Angiology 2003;54(5):599-604]. The majority of primary cardiac lymphomas are diffuse large B-cell lymphomas with centroblastic or immunoblastic cytologic features. Herein, we describe an exceptional case of a primary cardiac plasmablastic variant of diffuse large B-cell lymphoma (Epstein-Barr virus-positive) presenting in an immunocompetent host with chest pain that mimicked a left ventricular apical thrombus.

Afección cardíaca en la esclerosis sistémica

Systemic sclerosis (SS) can involve the pericardium, myocardium, conduction system, and cardiac valves. The presence of overt clinical signs of cardiac disease is a poor prognostic sign. Clinical manifestations include dyspnea, palpitations, chest pain, syncope, and symptoms of right heart failure. Prevalence of clinically symptomatic pericardial disease is 5-16%. However, ecocardiographic prevalence is 5.4- 41% and at autopsy is 33-77.5%. Patchy fibrosis is the characteristic myocardial finding in SS. Contraction band necrosis is the typical pathological finding. Important complications of fibrosis include left ventricular hypertrophy, as well as systolic and diastolic dysfunction of both ventricles. Early detection of these abnormalities is very important, mainly of the diastolic dysfunction, since it occurs before the systolic dysfunction and can predict important cardiac damage. Association of skeletal myositis with myocardial disease has been described. Patients with skeletal myositis are more likely to develop congestive heart failure, sustained symptomatic arrythmias, and cardiac sudden death. Coronary arteries are normal in systemic sclerosis, but there is no endomyocardial vessel involvement. There is an increased prevalence of arrhytmias, mainly premature atrial and ventricular contractions, as well as conduction system disease. Cardiac valvular involvement is minor in systemic sclerosis; mitral valve is the most frequently affected. Other abnormalities described in this disease include peripheral large vessels stiffness and secondary cardiac involvement due to pulmonary and systemic arterial hypertension. Cardiac involvement confers a high morbi-mortality risk in systemic sclerosis.

Coronary artery encasement by metastatic cardiac lymphoma: Multidetector CT and MR imaging findings

Secondary cardiac involvement by malignant lymphoma is not uncommon. However, it is frequently undetected before death, because the clinical manifestations of cardiac lymphoma are usually nonspecific. We describe the multidetector computed tomography and magnetic resonance imaging findings of a secondary cardiac lymphoma case in a patient with B-cell non-Hodgkin lymphoma. It manifested as a large mass filling the right atrium and ventricle without causing tricuspid stenosis. The mass showed heterogeneous contrast enhancement on gadolinium-enhanced magnetic resonance images. The right coronary artery was encased by the mass, but no findings suggesting intraluminal involvement were detected.