Giant Cell Myocarditis and Left Ventricular Apical Aneurysm in a Child With Severe Combined Immunodeficiency

2021 ◽  
Author(s):  
Jonathon A. Hagel ◽  
Kevin P. Daly ◽  
Chrystalle Katte Carreon ◽  
Antonio R. Perez-Atayde ◽  
Sunil J. Ghelani
Keyword(s):  
Giant Cell ◽  
Severe Combined Immunodeficiency ◽  
Left Ventricular ◽  
Combined Immunodeficiency ◽  
Giant Cell Myocarditis ◽  
Apical Aneurysm

scholarly journals Monomorphic Ventricular Tachycardia as a Presentation of Giant Cell Myocarditis

2019 ◽  
Vol 2019 ◽  
pp. 1-4
Author(s):  
Michael H. Chiu ◽  
Cvetan Trpkov ◽  
Saman Rezazedeh ◽  
Derek S. Chew
Keyword(s):  
Heart Failure ◽  
Ventricular Arrhythmia ◽  
Giant Cell ◽  
Complex Disease ◽  
Acute Myocarditis ◽  
Left Ventricular ◽  
Myocardial Inflammation ◽  
Icd Implantation ◽  
Severe Left Ventricular Dysfunction ◽  
Giant Cell Myocarditis

Background. Idiopathic giant cell myocarditis (GCM) has a fulminant course and typically presents in middle-aged adults with acute heart failure or ventricular arrhythmia. It is a rare disorder which involves T lymphocyte-mediated myocardial inflammation. Diagnosis is challenging and requires a high index of suspicion since therapy may improve an otherwise uniformly fatal prognosis. Case Summary. A previously healthy 54-year-old female presented with hemodynamically significant ventricular arrhythmia (VA) and was found to have severe left ventricular dysfunction. Cardiac MRI demonstrated acute myocarditis, and endomyocardial biopsy showed giant cell myocarditis. She was treated with combined immunosuppressive therapy as well as guideline-directed medical therapy. A secondary prevention implantable cardioverter defibrillator (ICD) was implanted. Discussion. GCM is a rare, lethal myocarditis subtype but is potentially treatable. Combined immunosuppression may achieve partial clinical remission in two-thirds of patients. VA is common, and patients should undergo ICD implantation. More research is needed to better understand this complex disease. Learning Objectives. Giant cell myocarditis is an incompletely understood, rare cause of myocarditis. Patients present predominately with heart failure and dysrhythmia. Diagnosis is confirmed by histopathology, and immunosuppression may improve outcomes. ICD implantation should be considered. In the absence of treatment, prognosis is poor with a median survival of three months.

scholarly journals Giant Cell Myocarditis: Not Always a Presentation of Cardiogenic Shock

2015 ◽  
Vol 2015 ◽  
pp. 1-4
Author(s):  
Rose Tompkins ◽  
William J. Cole ◽  
Barry P. Rosenzweig ◽  
Leon Axel ◽  
Sripal Bangalore ◽  
...  
Keyword(s):  
Cardiogenic Shock ◽  
Giant Cell ◽  
Systolic Function ◽  
Left Ventricular Systolic Function ◽  
Left Ventricular ◽  
Circulatory Support ◽  
Rapid Decline ◽  
Giant Cell Myocarditis ◽  
Hemodynamic Compromise ◽  
Hemodynamic Deterioration

Giant cell myocarditis is a rare and often fatal disease. The most obvious presentation often described in the literature is one of rapid hemodynamic deterioration due to cardiogenic shock necessitating urgent consideration of mechanical circulatory support and heart transplantation. We present the case of a 60-year-old man whose initial presentation was consistent with myopericarditis but who went on to develop a rapid decline in left ventricular systolic function without overt hemodynamic compromise or dramatic symptomatology. Giant cell myocarditis was confirmed via endomyocardial biopsy. Combined immunosuppression with corticosteroids and calcineurin inhibitor resulted in resolution of symptoms and sustained recovery of left ventricular function one year later. Our case highlights that giant cell myocarditis does not always present with cardiogenic shock and should be considered in the evaluation of new onset cardiomyopathy of uncertain etiology as a timely diagnosis has distinct clinical implications on management and prognosis.

scholarly journals Fulminant cardiac sarcoidosis resembling giant cell myocarditis: a case report

2021 ◽  
Vol 5 (3) ◽  
Author(s):  
Ginger Y Jiang ◽  
Qizhe Cai ◽  
E Wilson Grandin ◽  
Marwa A Sabe
Keyword(s):  
Giant Cell ◽  
Endomyocardial Biopsy ◽  
Cardiac Sarcoidosis ◽  
Obstructive Coronary Artery Disease ◽  
Left Ventricular ◽  
Left Ventricular Ejection ◽  
High Dose ◽  
Rare Presentation ◽  
Ventricular Ejection Fraction ◽  
Giant Cell Myocarditis

Abstract Background Severe cardiac sarcoidosis (CS) can share clinical and histopathologic features with giant cell myocarditis (GCM). Case summary A 56-year-old female presented with 1 week of exertional chest pressure and dyspnoea. Echocardiogram demonstrated extensive regional dysfunction with left ventricular ejection fraction (LVEF) 38%. Cardiac catheterization revealed no obstructive coronary artery disease and cardiac index 1.5 L/min/m2. Cardiac magnetic resonance imaging (MRI) demonstrated diffuse late gadolinium enhancement. Positron emission tomography with fluorodeoxyglucose (FDG) (FDG-PET) computed tomography showed FDG uptake in the anteroseptal and anterior wall and no extracardiac activity. Endomyocardial biopsy (EMB) demonstrated fragments of endocardial fibrosis with mixed inflammatory infiltrate including histiocytic giant cells, which could be due to CS or GCM. She was initially treated for GCM with high dose steroids, tacrolimus, and mycophenolate mofetil. Repeat EMB was pursued and demonstrated multiple granulomas with sharp demarcation from adjacent uninvolved myocardium consistent with CS. A dual-chamber implantable cardioverter-defibrillator was placed, and immunosuppression was changed to prednisone alone with plan for infliximab. Discussion This case illustrates a rare presentation of fulminant isolated CS. Endomyocardial biopsy with sufficient tissue was critical to establish a diagnosis and initiate appropriate immunosuppression.

Abstract 15886: When the Giant is Hidden but Not Dormant

Circulation ◽  
2020 ◽  
Vol 142 (Suppl_3) ◽  
Author(s):  
Yevgeniy Brailovsky ◽  
Amirali Masoumi ◽  
Rachel Bijou ◽  
Estefania Oliveros ◽  
Gabriel T Sayer ◽  
...  
Keyword(s):  
Ventricular Arrhythmia ◽  
Cardiogenic Shock ◽  
Heart Transplantation ◽  
Giant Cell ◽  
Endomyocardial Biopsy ◽  
Left Ventricular ◽  
Left Ventricular Ejection ◽  
Pathological Examination ◽  
Giant Cell Myocarditis ◽  
Hemodynamic Deterioration

Background: Giant cell myocarditis is a rare etiology of cardiogenic shock, which requires high index of suspicion, rapid immunosuppressive therapy, and sometimes mechanical circulatory supportCase presentation69 year old woman with prior medical history of non-ischemic dilated cardiomyopathy and recovered left ventricular ejection fraction (LVEF) presented with cardiogenic shock complicated by acute renal and liver injury requiring support with Impella 5.5. Suspicion for myocarditis was high and patient underwent endomyocardial biopsy and started on high dose steroids. Biopsy was negative for myocarditis. Despite support, patient had progressive shock and incessant ventricular arrhythmia necessitating IV antiarrhytmics and escalation to VA ECMO in addition to Impella and eventually to central Centrimag BiVAD with drainage cannulas in right atrium and LV apex, and reinfusion catheters in pulmonary artery and ascending aorta. (Figure 1) She was urgently listed for heart transplantation as a status 1 and one day later underwent successful orthotopic heart transplantation. Pathological examination of the explanted heart was consistent with Giant cell Myocarditis (Figure 2) Conclusion: Giant cell myocarditis is a rare etiology of cardiogenic shock. Suggestive features include rapid hemodynamic deterioration and incessant ventricular arrhythmia. Negative initial endomyocardial biopsy is not enough to rule out the disease in high clinical suspicion. Rapid escalation of support is warranted in fulminant cases to preserve end organ function and improve survival.(Figure 1 Created with BioRender.com)

scholarly journals Transient Myocarditis Associated with Fulminant Colitis

ISRN Surgery ◽  
2011 ◽  
Vol 2011 ◽  
pp. 1-3 ◽  
Author(s):  
J. M. L. Williamson ◽  
R. S. J. Dalton
Keyword(s):  
Inflammatory Response ◽  
Giant Cell ◽  
Complete Recovery ◽  
Left Ventricular ◽  
Left Ventricular Failure ◽  
Fistula Formation ◽  
Fulminant Colitis ◽  
Ventricular Failure ◽  
Giant Cell Myocarditis ◽  
History Of

Case Summary. An 18-year old man presented with a three-week history of abdominal pain, weight loss and bloody diarrhoea. He was profoundly septic, with generalised abdominal tenderness. CT and flexible sigmoidosopy confirmed colitis of the colon with rectal sparing. Laparotomy was performed when conservative management failed to improve his condition. Subtotal colectomy, with end ileostomy and mucus fistula formation, was performed in light of active colitis. Despite successful operative intervention the patient acute left ventricular failure, raising the possibility of giant cell myocarditis, which fully resolved before a definitive diagnosis could be reached. Discussion. It is possible that the transient cardiac failure in this case may represent an overwhelming inflammatory response or myocarditis. Inflammatory bowel disease is rarely associated with giant cell myocarditis (GCM). GCM usually affects a young population and its prognosis is variable, ranging from complete recovery, remission with recurrence and fatality. The management of this group of patients is still relatively experimental. Conclusion. Fulminant colitis can be associated with a rapid deterioration in cardiac function. Causes include sepsis, systemic inflammatory response syndrome or myocarditis. GCM should be considered in patients with new onset of left ventricular failure that decline rapidly.

scholarly journals Manifestations and Outcome of Cardiac Sarcoidosis and Idiopathic Giant Cell Myocarditis by 25‐Year Nationwide Cohorts

2021 ◽  
Author(s):  
Hanna‐Kaisa Nordenswan ◽  
Jukka Lehtonen ◽  
Kaj Ekström ◽  
Anne Räisänen‐Sokolowski ◽  
Mikko I. Mäyränpää ◽  
...  
Keyword(s):  
Giant Cell ◽  
Myocardial Injury ◽  
Cardiac Sarcoidosis ◽  
Troponin T ◽  
Left Ventricular ◽  
Left Ventricular Ejection ◽  
Ventricular Ejection Fraction ◽  
Long Term Outcome ◽  
Cox Regression Analysis ◽  
Giant Cell Myocarditis

Background Cardiac sarcoidosis (CS) and giant cell myocarditis (GCM) share many histopathologic and clinical features. Whether they are parts of a one‐disease continuum has been discussed. Methods and Results We compared medical record data of 351 CS and 28 GCM cases diagnosed in Finland since the late 1980s and followed until February 2018 for a composite end point of cardiac death, aborted sudden death, and heart transplantation. Heart failure was the presenting manifestation in 50% versus 15% ( P <0.001), and high‐grade atrioventricular block in 21% versus 43% ( P =0.044), of GCM and CS, respectively. At presentation, left ventricular ejection fraction was ≤50% in 81% of cases of GCM versus in 48% of CS ( P =0.004). The median (interquartile range) of plasma NT‐proBNP (N‐terminal pro‐B‐type natriuretic peptide) was 5273 (2782–11309) ng/L on admission in GCM versus 859 (290–1950) ng/L in CS ( P <0.001), and cardiac troponin T exceeded 50 ng/L in 17 of 19 cases of GCM versus in 48 of 239 cases of CS ( P <0.001). The 5‐year estimate of event‐free survival was 77% (95% CI, 72%–82%) in CS versus 27% (95% CI, 10%–45%) in GCM ( P <0.001). By Cox regression analysis, GCM predicted cardiac events with a hazard ratio of 5.16 (95% CI, 2.82–9.45), which, however, decreased to 1.58 (95% CI, 0.71–3.52) after inclusion of markers of myocardial injury and dysfunction in the model. Conclusions GCM differs from CS in presenting with more extensive myocardial injury and having worse long‐term outcome. Yet the key determinant of prognosis appears to be the extent of myocardial injury rather than the histopathologic diagnosis.

scholarly journals Different reverse remodelling between left ventricle and right ventricle in fulminant heart failure due to giant cell myocarditis: a case report

2021 ◽  
Vol 5 (8) ◽  
Author(s):  
Hiroaki Yokoyama ◽  
Masashi Yamaguchi ◽  
Kazuki Tobita ◽  
Shigeru Saito
Keyword(s):  
Heart Failure ◽  
Left Ventricle ◽  
Right Ventricle ◽  
Giant Cell ◽  
Functional Recovery ◽  
Left Ventricular ◽  
Giant Cell Myocarditis ◽  
Reverse Remodelling ◽  
The Right ◽  
Immunosuppression Therapy

Abstract Background Giant cell myocarditis (GCM) is a rare cause of fulminant heart failure (HF). The most common presentation is progressive hemodynamic deterioration, and a few cases present with idiopathic complete atrioventricular block (cAVB). The prognosis of GCM is poor, and GCM patients usually die of HF and ventricular arrhythmia unless cardiac transplantation is performed. Few reports have described the effects of treatments such as immunosuppression and detailed reverse remodelling in GCM patients. Case summary A 69-year-old female presented with cAVB. Transvenous pacemaker was implanted via the left subclavian vein. One and a half months later, she exhibited left ventricular dyssynchrony and lower left ventricular ejection fraction (LVEF), resulting in hospitalization for HF. She received cardiac resynchronization therapy; however, this had no apparently positive effects on her cardiac function. To investigate the cause of the lower LVEF, an endomyocardial biopsy was taken from the right ventricular septum. She was diagnosed with GCM and immediately received immunosuppression therapy with prednisolone and ciclosporin. This resulted in the functional recovery of the right ventricle; on the other hand, the left ventricle had still not recovered based on transthoracic echocardiography. Fortunately, she successfully recovered from severe HF without recurrence. Discussion This is a case of fulminant HF due to GCM which initially presented as cAVB. Moreover, this case demonstrates the quite difference of the functional recovery between the left ventricle and the right ventricle with immunosuppression therapy.

scholarly journals Clinical Identification and Characteristic Analysis of Giant Cell Myocarditis in 12 Cases

2021 ◽  
Vol 8 ◽  
Author(s):  
Shangyu Liu ◽  
Lihui Zheng ◽  
Lishui Shen ◽  
Lingmin Wu ◽  
Yan Yao
Keyword(s):  
Giant Cell ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Viral Myocarditis ◽  
Clinical Manifestations ◽  
Left Ventricular ◽  
Left Ventricular Ejection ◽  
Pathological Examination ◽  
Characteristic Analysis ◽  
Ventricular Ejection Fraction ◽  
Giant Cell Myocarditis

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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