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Drug reaction with eosinophilia and systemic symptoms (DRESS) is a severe drug reaction characterized by skin rash, organ involvement, lymph node swelling, eosinophilia, and atypical lymphocytosis, with myocarditis being a rare but potentially fatal complication. It has been reported that in patients with cardiac involvement due to DRESS, older age and shorter periods between offending drug exposure and symptom onset are associated with mortality. We report a case of fatal DRESS-associated myocarditis in a young woman, occurring one month after drug exposure, despite intensive immunosuppressive therapy. This case report highlights the risk of mortality from DRESS-associated myocarditis even in patients lacking known risk factors.
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A 27-year-old Japanese woman with a history of depression and an eating disorder presented to our emergency department with a chief complaint of generalized weakness. Electrocardiography showed prominent QT prolongation with multiple ventricular contractions. Chest X-ray plain computed tomography revealed pulmonary edema. Echocardiography showed decreased left ventricular systolic function. Suspecting acute myocarditis, we performed a myocardial biopsy from the right ventricular septum. The biopsy histology revealed extensive myocardial fibrosis and a very mild inflammatory cell infiltrate. In an additional detailed medical interview, the patient admitted that she had consumed three bottles of a first-aid liquid containing naphazoline approximately ~12â¯h before her presentation, in a suicide attempt. Her QTc and left ventricular ejection fraction improved during hospitalization. Learning objective: Acute drug intoxication can cause QT prolongation and ventricular arrhythmias, cardiomyopathy, and pulmonary edema. When acute QT prolongation, myocardial damage, and pulmonary edema are seen (suggesting acute myocarditis), naphazoline intoxication should be investigated in the differential diagnosis.
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Patients with damage of the mitral, aortic and tricuspid valves and systolic myocardial dysfunction associated with previous SARS-CoV-2 infection are described. The diagnosis of acquired defect was established in 4 patients based on medical history, electrocardiography, echocardiography, magnetic resonance imaging of the heart, endomyocardial or intraoperative myocardial biopsy, and in one case, autopsy. The study of the myocardium included H&E, Van Gieson staining, immunohistochemical (IHC) study with antibodies to CD3, CD20, CD45, CD68, to the nucleocapsid and Spike proteins of SARS-CoV-2. Previous valve diseases (prolapse, bicuspid aortic valve) served as a background for the development of the defect in 2 patients. In all cases, IHC studies revealed coronavirus proteins, lymphocytic endocarditis and myocarditis, moderate fibrosis, and signs of connective tissue disorganization. High titers of anticardiac antibodies indicated an autoimmune mechanism for carditis. No signs of infective endocarditis or thromboembolic complications were identified in any case. In patients with an unclear nature of valvular heart defects, a previous new coronavirus infection should be identified and taken into account as a possible etiological factor. The simultaneous development of lymphocytic myocarditis significantly increases the risk of surgical intervention on the valves and requires an integrated approach to treatment.
Subject(s)
COVID-19 , Endocarditis , Heart Defects, Congenital , Myocarditis , Humans , Myocarditis/complications , SARS-CoV-2 , COVID-19/complications , Endocarditis/diagnosis , Endocarditis/diagnostic imagingABSTRACT
Cardiac cysticercosis is rare in clinical practice and is usually accidentally identified during cardiac surgery or autopsies. Although mostly asymptomatic, cardiac cysticercosis could present with severe clinical conditions such as myocarditis, acute myocardial infarction, and arrhythmia. We present a 51-year-old female patient who accidentally discovered a solitary mass in the myocardium. The cardiac magnetic resonance imaging revealed a nonenhanced cyst in the interventricular septum protruding into the right ventricular chamber. Because of cardiac tamponade presenting during a right ventricular endomyocardial biopsy, an emergency open-heart surgery was performed to suture the ventricular wall perforation and remove the tumor. The histopathologic report demonstrated typical cysticercosis. Cardiac cysticercosis is an uncommon lesion and may present with atypical clinical and laboratory features. Therefore, this diagnosis should be considered single or multiple cardiac cystic lesions.
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Desminopathy is a cardiac and skeletal myopathy caused by disease-causing variants in the desmin (DES) gene and represents a subgroup of myofibrillar myopathies, where cytoplasmic desmin-postive immunoreactivity is the pathological hallmark. We herein report a 28-year-old Japanese man who was initially diagnosed with sporadic hypertrophic cardiomyopathy with atrioventricular block at 9 years old and developed weakness in the soft palate and extremities. The myocardial tissue dissected during implantation of the ventricular-assisted device showed a dilated phase of hypertrophic cardiomyopathy and intracellular accumulation of proteinase K-resistant desmin aggregates. Genetic testing confirmed a de novo mutation of DES, which has already been linked to desminopathy. As the molecular diagnosis of desminopathy is challenging, particularly if patients show predominantly cardiac signs and a routine skeletal muscle biopsy is unavailable, these characteristic pathological findings of endomyocardial proteinase K-resistant desmin aggregates might aid in clinical practice.
Subject(s)
Cardiomyopathies , Cardiomyopathy, Hypertrophic , Myopathies, Structural, Congenital , Male , Humans , Child , Adult , Desmin/genetics , Desmin/metabolism , Cardiomyopathies/diagnosis , Cardiomyopathies/genetics , Cardiomyopathies/pathology , Endopeptidase K/genetics , Mutation/geneticsABSTRACT
BACKGROUND: Immunoglobulin light chain (AL) amyloidosis is a rare disease characterized by deposition of ALs essentially in any organ or tissue, with cardiac involvement being very frequent (61%). Early diagnosis is of high importance because early initiation of treatment in AL amyloidosis may improve outcomes. Despite the administration of immunotherapeutic agents, in particular bortezomib and daratumumab, which have improved the outcomes of AL amyloidosis, anti-plasma cell therapy remains suboptimal for some patients. CASE SUMMARY: We report the case of a 55-year-old man presenting with heart failure who was diagnosed with cardiac AL amyloidosis by an endomyocardial biopsy. He experienced a short-term hematological remission with no organ response after being administered a bortezomib-daratumumab containing regimen. The treatment was switched to pomolidomide due to pulmonary involvement and progressive pleural effusion, in which flow cytometry analysis showed abnormal plasma cells. After two cycles of this regimen, the pleural effusion was controlled effectively with no recurrence. CONCLUSION: This case emphasizes the crucial role of endomyocardial biopsy in early diagnosis of cardiac amyloidosis and suggests that pomolidomide may be an effective treatment for patients with AL amyloidosis that is relapsed/refractory to both bortezomib and daratumumab.
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There are several causes of heart failure during pregnancy and the peripartum period, which include peripartum cardiomyopathy, Takotsubo cardiomyopathy or stress cardiomyopathy, exacerbation of a preexisting cardiomyopathy, and acute myocarditis. It is important to determine the cause of the heart failure as the medical treatment may be different based on the diagnosis. However, it has been sometimes challenging to diagnose the cause because of the limited diagnostic tools, especially in pregnant women. Cardiac MRI can characterize myocardial injury and can be used to track the changes in myocardial tissue. We herein report a 35-year-old woman diagnosed with peripartum mid-ventricular-type Takotsubo cardiomyopathy, who was referred to our hospital due to worsening dyspnea the day after cesarean delivery. On admission, electrocardiography showed sinus tachycardia and poor progression of R waves in the precordial leads. Bedside echocardiography revealed severe hypokinesis in the mid- and apical left ventricle (LV) with a LV ejection fraction of 20%. Cardiac catheterization showed normal coronary arteries, and myocardial biopsy revealed contraction band necrosis. On acute phase (Day 4), cardiac MRI showed prolonged native T1 and T2, and severe hypokinesis and decreased regional longitudinal peak strain in the mid-anterior LV wall. During the 1st week, precordial ST fluctuation was observed, and LV wall motion had gradually recovered. Repeat cardiac MRI revealed normalized LV wall motion and shortened values for global native T1 and T2. Thus, she was diagnosed with peripartum Takotsubo cardiomyopathy. Serial cardiac MRI may be able to differentiate Takotsubo cardiomyopathy during pregnancy and the peripartum period from other preexisting cardiomyopathies.
Subject(s)
Cardiomyopathies , Heart Failure , Takotsubo Cardiomyopathy , Adult , Cardiomyopathies/complications , Cardiomyopathies/etiology , Female , Heart Failure/complications , Heart Ventricles , Humans , Peripartum Period , Pregnancy , Takotsubo Cardiomyopathy/diagnosis , Takotsubo Cardiomyopathy/etiologyABSTRACT
BACKGROUND: Despite marked progress in endovascular treatment (EVT) techniques and devices, calcified lesions remain one of the toughest obstacles to EVT success. Moreover, because the common femoral artery (CFA) is known as a "non-stenting zone," endovascular strategies for this area are controversial. CASE PRESENTATION: Here we describe the technical tips for a novel, less invasive, and effective debulking strategy for severe nodular calcification using an endovascular maneuver. This technique was demonstrated in a 73-year-old man with severe calcified stenosis of the CFA. To complete a stent-less strategy for CFA, we conducted aggressive debulking of the nodular calcification, established a bidirectional approach from the radial artery and the superficial femoral artery (SFA), and inserted a balloon-guiding catheter in the SFA. Under distal protection provided by this catheter, we crushed the nodular calcification 43 times using myocardial biopsy forceps. After achieving a volume reduction of nodular calcification through this maneuver, we completed the procedure by inflating a 6-mm drug-coated balloon catheter. Final angiography demonstrated a reduced filling defect of the contrast medium in the CFA and favorable blood flow as far as the ankle. The puncture site on the SFA was closed with a vascular suture assisted by balloon inflation inside the vessel, which allowed the patient to be ambulatory immediately after the procedure without requiring bed rest. CONCLUSIONS: Severely calcified lesions in the CFA are usually difficult to treat using an endovascular strategy, but our novel and less invasive method may become a promising technique for managing these lesions.
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Objectives: Fulminant myocarditis (FM) is a rapidly progressive and frequently fatal form of myocarditis that has been difficult to classify. This study aims to compare the clinical characteristics, treatments and outcomes in patients with fulminant giant cell myocarditis (FGCM) and fulminant lymphocytic myocarditis (FLM). Methods and Results: In our retrospective study, nine patients with FGCM (mean age 47.9 ± 7.5 years, six female) and 7 FLM (mean age 42.1 ± 12.3 years, four female) patients confirmed by histology in the last 11 years were included. Most patients with FGCM and FLM were NYHA functional class IV (56 vs. 100%, p = 0.132). Patients with FGCM had significantly lower levels of high-sensitivity C-reactive protein [hs-CRP, 4.4 (2.0-10.2) mg/L vs. 13.6 (12.6-14.6) mg/L, P = 0.004, data shown as the median with IQR], creatine kinase-myoglobin [CK-MB, 1.4 (1.0-3.2) ng/ml vs. 14.6 (3.0-64.9) ng/ml, P = 0.025, median with IQR], and alanine aminotransferase [ALT, 38.0 (25.0-61.5) IU/L vs. 997.0 (50.0-3,080.0) IU/L, P = 0.030, median with IQR] and greater right ventricular end-diastolic diameter (RVEDD) [2.9 ± 0.3 cm vs. 2.4 ± 0.6 cm, P = 0.034, mean ± SD] than those with FLM. No differences were observed in the use of intra-aortic balloon pump (44 vs. 43%, p = 1.000) and extracorporeal membrane oxygenation (11 vs. 43%, p = 0.262) between the two groups. The long-term survival rate was significantly lower in FGCM group compared with FLM group (0 vs. 71.4%, p = 0.022). A multivariate cox regression analysis showed the level of hs-CRP (hazard ratio = 0.871, 95% confidence interval: 0.761-0.996, P = 0.043) was an independent prognostic factor for FM patients. Furthermore, the level of hs-CRP had a good ability to discriminate between patients with FGCM and FLM (AUC = 0.94, 95% confidence interval: 0.4213-0.9964). Conclusions: The inflammatory response and myocardial damage in the patients with FGCM were milder than those with FLM. Patients with FGCM had distinctly poorer prognoses compared with those with FLM. Our results suggest that hs-CRP could be a promising prognostic biomarker and a hs-CRP level of 11.71 mg/L is an appropriate cutoff point for the differentiating diagnosis between patients with FGCM and FLM.
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Among non-ischemic cardiomyopathies, cardiac amyloidosis is one of the most common, being caused by extracellular depositions of amyloid fibrils in the myocardium. Two main forms of cardiac amyloidosis are known so far, including 1) light-chain (AL) amyloidosis caused by monoclonal production of light-chains, and 2) transthyretin (ATTR) amyloidosis, caused by dissociation of the transthyretin tetramer into monomers. Both AL and ATTR amyloidosis are progressive diseases with median survival from diagnosis of less than 6 months and 3 to 5 years, respectively, if untreated. In this regard, death occurs in most patients due to cardiac causes, mainly congestive heart failure, which can be prevented due to the presence of effective, life-saving treatment regimens. Therefore, early diagnosis of cardiac amyloidosis is crucial more than ever. However, diagnosis of cardiac amyloidosis may be challenging due to variable clinical manifestations and the perceived rarity of the disease. In this regard, clinical and laboratory reg flags are available, which may help clinicians to raise suspicion of cardiac amyloidosis. In addition, advances in cardiovascular imaging have already revealed a higher prevalence of cardiac amyloidosis in specific populations, so that the diagnosis especially of ATTR amyloidosis has experienced a >30-fold increase during the past ten years. The goal of our review article is to summarize these findings and provide a practical approach for clinicians on how to use cardiovascular imaging techniques, such as echocardiography, cardiac magnetic resonance, bone scintigraphy and, if required, organ biopsy within predefined diagnostic algorithms for the diagnostic work-up of patients with suspected cardiac amyloidosis. In addition, two clinical cases and practical tips are provided in this context.
Subject(s)
Amyloid Neuropathies, Familial , Amyloidosis , Cardiomyopathies , Algorithms , Amyloid Neuropathies, Familial/diagnostic imaging , Amyloidosis/diagnostic imaging , Cardiomyopathies/diagnostic imaging , Echocardiography , Humans , PrealbuminABSTRACT
Multisystem Inflammatory Syndrome (MIS) is a novel hyperinflammatory syndrome associated with SARS-CoV-2 infection. It predominantly affects children (MIS-C) a few weeks after a usually asymptomatic SARS-CoV-2 infection and is only rarely seen in adults above 21 years (MIS-A). Only scarce data on histological findings in both pediatric and adult patients has been published so far. An 18-year-old male patient was admitted to hospital in a febrile state, which progressed to severe cardiogenic shock and multi-organ failure requiring extracorporeal life support. Myocardial biopsy revealed small vessel-associated immune cell infiltrates. Diagnosis of MIS-C was made after ruling out all potential differential diagnosis. Use of immunosuppressive treatment with steroids, interleukin-1 blockade and high-dose intravenous immunoglobulins resulted in the patient's full recovery. Multisystem Inflammatory Syndrome (MIS) is a new differential diagnosis of cardiac dysfunction in pediatric and adult patients. The lack of myocardial necrosis differentiates the disease from other viral myocarditis and offers an explanation for the fast response to immunomodulatory therapy and the favorable prognosis. The preceding SARS-CoV-2 infection might only have been mildly symptomatic or even asymptomatic.
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BACKGROUND: We aim to evaluate the value of Cardiac magnetic resonance (CMR) feature tracking (CMR-FT) in addition to Task Force Criteria(TFC) in patients with (arrhythmogenic cardiomyopathy) AC biopsy-proved. METHODS: Thirty-five patients with AC histologically proven who performed CMR with late gadolinium enhancement (LGE) acquisition were enrolled. The study population was divided in Group1 (negative CMR TFC and LV ejection fraction≥55%) and Group2 (positive CMR TFC and/or LVEF<55%) and compared to an age and gender-matched control group. CMR datasets of all patients were analyzed to calculate LV indexed end-diastolic (LVEDi) and end-systolic (LVESi) volumes and RV indexed end-diastolic (RVEDi) and end-systolic (RVESi) volumes, both LV ejection fraction (LVEF) and RV ejection fraction (RVEF). Moreover, LV and RV global longitudinal (GLS), circumferential (GCS) and radial (GRS) strain were measured. RESULTS: The AC patients showed both higher LVEDi (p:0.002) and RVEDi (p:0.017) and lower LVEF (p: 0.016) as compared to control patients. Moreover, AC patients showed impaired LV-GLS (p < 0.001), LV-GRS (p < 0.001), LV-GCS (p < 0.001) and RV-GRS (p:0.026) as compared to control subjects. Group1 patients showed a significant reduction of LV-GRS (p < 0.05) and LV-GCS p < 0.01) as compared to control subjects. At univariate analysis LV-GCS was the most discriminatory parameter between Group1 vs heathy subjects with an optimal cut-off of -15.8 (Sensitivity: 74%; Specificity: 10%). CONCLUSIONS: In patients with AC biopsy-proven, CMR-FT could improve the diagnostic yield in the subset of patients who results negative for imaging TFC criteria resulting as useful gatekeeper for indication of myocardial biopsy in case of equivocal clinical and imaging presentation.
Subject(s)
Cardiomyopathies , Contrast Media , Biopsy , Gadolinium , Humans , Magnetic Resonance Imaging, Cine , Magnetic Resonance Spectroscopy , Predictive Value of Tests , Stroke Volume , Ventricular Function, LeftABSTRACT
Aims: Giant cell myocarditis (GCM) is a rare, rapidly progressing cardiomyopathy with high mortality, if not diagnosed and treated in time. We analyzed the progression and clinical manifestations of patients with definitive diagnosis of GCM. Methods and Result: We enrolled 12 patients diagnosed with GCM in the explanted heart during heart transplantation (HTx) or by endomyocardial biopsy (EMB) and collected information on demographic data, cardiac structure and function, arrhythmias, preliminary diagnosis, and delay of the diagnosis. Seven cases were diagnosed from biopsy samples during HTx, and five cases were diagnosed through EMB. Before the diagnosis of GCM based on pathological analysis, these patients had been incorrectly diagnosed with arrhythmogenic right ventricular cardiomyopathy (n = 5), dilated cardiomyopathy (n = 2), ventricular tachycardia (n = 2), viral myocarditis (n = 1), cardiac amyloidosis (n = 1), and ischemic cardiomyopathy (n = 1) based on clues such as symptoms, arrhythmia, and cardiac imaging. Patients diagnosed with GCM through EMB had a shorter symptom-onset-to-diagnosis time (6.6 ± 2.7 months) and milder heart damage (left ventricular ejection fraction, 47.2 ± 8.8%) than those diagnosed during HTx (11.0 ± 3.3 months, P = 0.034; 31.4 ± 10.9%, P = 0.024). Conclusion: GCM is easily misdiagnosed as other types of myocarditis and cardiomyopathy. Pathological examination of the myocardium is the most reliable diagnostic method for GCM. Endocardial biopsy can identify patients with GCM at an earlier stage.
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Scleredema is a rare cutaneous mucinosis characterized by diffuse swelling and non-pitting induration. A 63-year-old man reported a 5-year history of skin thickening of the trunk and a 3-week history of dyspnea. Echocardiography revealed diffuse hypokinesis. Skin biopsies obtained from the waist showed thickened dermis with mucin. Myocardial biopsies showed alcian blue-stained tissue between the muscle fibers. The patient was referred to a dermatologist for phototherapy. Cardiomyopathy should be considered in patients with scleredema. Scleredema usually has a good prognosis; however, the mortality risk could be high when accompanied by cardiomyopathy.
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BACKGROUND: In patients with severe mitral regurgitation (MR), additional echocardiographic indices could be helpful to optimize surgical timing before irreversible left heart myocardial dysfunction has occurred. We investigated the correlation of left atrial (LA) strain by speckle tracking echocardiography with prognosis after mitral surgery for severe MR, and its association with LA fibrosis. METHOD: 71 patients with primary severe MR undergoing pre-operative echocardiographic assessment were initially enrolled. Exclusion criteria were: other valvular disease>moderate, history of coronary artery disease, heart failure (HF), hypertrophic cardiomyopathy, left bundle branch block, previous pacemaker implantation, heart transplantation, poor acoustic window. The primary endpoint was the occurrence of composite events (HF and mortality); the secondary endpoint was post-operative functional capacity (NYHA and Borg CR10 class). LA fibrosis was assessed by atrial biopsy specimens in a subset of patients. RESULTS: Of 65 eligible patients, the primary endpoint occurred in 30 patients (medium follow-up: 3.7 ± 1 years for event-group, 6.8 ± 1 years for non-event group). After Kaplan-Meier analysis, peak atrial longitudinal strain (PALS) provided good risk stratification (5-year event-free survival:90 ± 5% for PALS≥21% vs 30 ± 9% for PALS<21%, p < 0.0001); it was an independent and incremental predictor of outcome in four multivariate Cox adjusted models. There was also an association between PALS and the secondary endpoint (NYHA: r2 = 0.11, p = 0.04; Borg CR10: r2 = 0.10, p = 0.02) and an inverse correlation between PALS<21% and LA fibrosis (r2 0.80, fibrosis: 76.6 ± 20.7% vs 31.9 ± 20.8%;p < 0.0001). CONCLUSIONS: Global PALS emerged as a reliable predictor of outcome and functional capacity for severe primary MR, and as a marker of LA fibrosis.
Subject(s)
Mitral Valve Insufficiency , Atrial Function, Left , Echocardiography , Heart Atria/diagnostic imaging , Heart Atria/surgery , Humans , Mitral Valve Insufficiency/diagnostic imaging , Mitral Valve Insufficiency/surgery , PrognosisABSTRACT
A 73-year-old woman with a history of diarrhea for one year and other various symptoms was admitted to our hospital. Gastrointestinal endoscopy that included enteroscopy with multiple biopsies was performed. However, no significant findings were observed. Electrocardiography showed low voltage in all limb leads, and an echocardiogram showed thickened cardiac walls with granular sparkling pattern. A myocardial biopsy revealed amyloidosis, and a bone marrow biopsy showed multiple myeloma. This case suggests that we should suspect the possibility of amyloidosis in a patient with diarrhea and various symptoms involving multiple organ systems. Additionally, electrocardiograms and echocardiograms should be performed even when gastrointestinal biopsies reveal negative results.
Subject(s)
Amyloidosis , Multiple Myeloma , Aged , Amyloidosis/complications , Amyloidosis/diagnosis , Biopsy , Diarrhea/etiology , Echocardiography , Female , Humans , Multiple Myeloma/complications , Multiple Myeloma/diagnosisABSTRACT
We report a case of lethal myocarditis and myositis after pembrolizumab treatment for advanced upper urinary tract urothelial carcinoma. A 69-year-old man underwent pembrolizumab therapy as a second-line treatment. He had myalgia and a slightly elevated creatinine kinase (CK) on the day of the second administration of pembrolizumab. Five days later, the patient was admitted with severe fatigue and an abnormal gait. Physical examination revealed reduced muscle reflexes and proximal muscle weakness. An electrocardiogram (ECG) demonstrated a wide QRS complex ventricular rhythm. A marked elevation of cardiac enzymes, including CK, myoglobin, and cardiac troponin I, was detected. Myocardial biopsy revealed inflammatory cell infiltration and the partial impairment of myocardial tissue. The electromyogram was normal, but inflammation in myofibers was noted in a muscle biopsy. Myocarditis and myositis as immune-related adverse events (irAEs) were suspected, and the patient began intravenous steroid therapy and plasma exchange. However, the patient underwent cardiac arrest three days after admission and began extracorporeal membrane oxygenation and intra-aortic balloon pumping therapy. Despite steroid pulse therapy, the patient demonstrated no sign of improvement and subsequently died 17 days after admission. Immune-mediated myocarditis is a rare but fatal irAE of an immune checkpoint inhibitor (ICI). The present case suggests that myositis precedes myocarditis. Therefore, if myositis is suspected, subsequent myocarditis may need attention. In conclusion, we found that myositis and myocarditis developed in a patient with advanced urothelial carcinoma after pembrolizumab treatment. A routine follow-up of CK and cardiac troponin I, as well as an ECG, should be performed to identify any possible ICI-induced myocarditis and myositis quickly.
Subject(s)
Antibodies, Monoclonal, Humanized/adverse effects , Antineoplastic Agents, Immunological/adverse effects , Carcinoma, Transitional Cell/drug therapy , Kidney Neoplasms/pathology , Liver Neoplasms/drug therapy , Lung Neoplasms/drug therapy , Myocarditis/chemically induced , Myositis/chemically induced , Aged , Carcinoma, Transitional Cell/secondary , Creatine Kinase/blood , Creatine Kinase, MB Form/blood , Echocardiography , Electromyography , Extracorporeal Membrane Oxygenation , Fatal Outcome , Glucocorticoids/therapeutic use , Heart Arrest , Humans , Intra-Aortic Balloon Pumping , Kidney Pelvis , Liver Neoplasms/secondary , Lung Neoplasms/secondary , Male , Muscle, Skeletal/pathology , Myocarditis/blood , Myocarditis/diagnostic imaging , Myocarditis/pathology , Myocardium/pathology , Myoglobin/blood , Myositis/blood , Myositis/pathology , Myositis/physiopathology , Plasma Exchange , Troponin I/bloodABSTRACT
BACKGROUND: Eosinophilic myocarditis (EM) is rare but accounts for 12-22% of histologically proven acute myocarditis cases. Acute necrotizing EM is considered an aggressive, life-threatening disease which is usually treated by high-dose corticosteroid therapy. CASE SUMMARY: We report the case of a 27-year-old man with acute severe pericarditic chest pain, moderately reduced left ventricular (LV) ejection fraction, and a small pericardial effusion. Troponin I level was highly elevated in the absence of coronary artery disease, leading to the diagnosis of acute myopericarditis. In the absence of blood eosinophilia and despite a negative cardiac magnetic resonance study, LV endomyocardial biopsy revealed an acute necrotizing EM. With conventional antiphlogistic and heart failure therapy, the patient became symptom-free and inflammatory and cardiac necrosis markers as well as LV ejection fraction normalized within days. Thus, in the absence of a systemic hypereosinophilic disorder, there was no need for steroid therapy. Long-term follow-up over 12 months showed sustained normalization of cardiac structure and function. DISCUSSION: Acute necrotizing eosinophilic myopericarditis is not always a dreadful cardiac disease. There are idiopathic cases which may quickly resolve without immunosuppression. There seems to be a publication bias towards critical cases.
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A 69-year-old woman with previous pancreaticoduodenectomy was admitted for evaluation of chest discomfort on effort and leg edema for a few months. Oral flosemide before admission for 1 week failed to relieve her symptoms. Her blood pressure was 105/51 mmHg and heart rate was 76 beats/min. Chest X-ray revealed an enlarged heart and mild pulmonary congestion. Echocardiography demonstrated normal left ventricular ejection fraction and diastolic dysfunction with no left ventricular hypertrophy. Cardiac catheterization showed normal coronary arteries, high cardiac index, and elevated intracardial pressures. Myocardial biopsy from the right ventricular septum revealed nearly normal findings. Cardiac magnetic resonance imaging (CMRI) showed both ventricles enlarged and increased global extracellular volume fraction (ECV) of 37%, but normal native T1 and T2 values. As she had pancreaticoduodenectomy, beriberi was suspected. Vitamin B1 significantly increased urine output and lowered intracardiac pressures and cardiac index. After 3 months of vitamin B1, CMRI exhibited that the right ventricle had decreased in size and the global ECV value had been lowered. Our case highlights that chronic beriberi may be associated with little myocardial damage. The increased ECV suggests that the diffuse expansion of extracellular space unrelated to myocardial edema might have been reversed by vitamin B1treatment. Morphological changes in the ventricles and myocardial damage by wet beriberi can be demonstrated by CMRI.