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Although seen in ~5% of sarcoidosis patients, cardiac sarcoidosis (CS) accounts for nearly 25% of diseaserelated deaths. This study aimed to describe characteristics and outcomes among CS patients. Patients diagnosed with CS in 2016e2017 in the US National Inpatient Sample Database were evaluated to study patient characteristics, reasons ascribed to admission, in-hospital outcomes, and complications. A total of 2420 patients (median age 56 years) were included in the analysis. Most admissions occurred due to ventricular tachycardia (12.8%), followed by myocarditis (9.9%) with a mean length of stay of 7 ± 7 days. The overall incidence of in-hospital mortality was 2.5%
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RESUMEN Introducción: Estudios recientes sugieren combinar los hallazgos de la resonancia magnética cardíaca (RMC) y los de la tomografía por emisión de positrones (PET) para incrementar la sensibilidad del diagnóstico de la sarcoidosis cardíaca (SC). Objetivo: Evaluar el valor complementario de la RMC y la PET en el diagnóstico de la SC. Material y métodos: Entre diciembre 2018 y Julio 2020, 6 pacientes (4 hombres y 2 mujeres) fueron referidos a nuestro servicio con sospecha de SC para evaluación de inflamación del miocardio. Se efectuó un estudio de perfusión miocárdica en reposo (13N Amonio) y de 18F-Fluordesoxiglucosa (FDG)-PET para evaluar inflamación y/o fibrosis. A todos los pacientes se les realizó previamente una RMC con gadolinio. Resultados: La edad media fue de 60 ± 9 años. El 50% de los pacientes presentaban antecedente de sarcoidosis sistémica y el otro 50% sospecha de SC aislada. Ninguno de los pacientes presentó inflamación activa del miocardio por PET. Con la combinación de los patrones-PET y el realce por RMC se reclasificó a los pacientes: 50% tuvo menos del 10% de probabilidad de padecer SC y el otro 50% se clasificó como posible. Ninguno de los pacientes recibió tratamiento inmunosupresor. Conclusión: En nuestra población de pacientes con sospecha de SC e inflamación, realizamos un estudio PET luego de la RMC para calcular probabilidades de padecer SC. En ausencia de un patrón oro, se sugiere que el diagnóstico de SC se base en probabilidades de acuerdo a patrones de imágenes y cuadro clínico específicos.
ABSTRACT Background: Recent studies suggest combining the findings of cardiac magnetic resonance (CMR) and positron emission tomography (PET) to increase sensitivity in the diagnosis of cardiac sarcoidosis (CS). Objective: To evaluate the complementary value of CMR and PET in the diagnosis of CS. Methods: From December 2018 to July 2020, 6 patients (4 males and 2 females) with suspected CS were referred to our facility for evaluation of myocardial inflammation. A resting 13N Ammonia myocardial perfusion test and a 18F Fluorodeoxyglucose (FDG) PET were performed to evaluate myocardial inflammation and/or fibrosis. All patients had a previous gadoliniumenhanced CMR. Results: The average age was 60 ± 9 years. Fifty percent of the patients had a history of systemic sarcoidosis and the remaining 50% had suspected isolated CS. None of the patients had active myocardial inflammation based on the PET findings. With the combination of PET patterns and enhanced CMR, the patients were reclassified as follows: 50% had less than 10% chance of having CS and the other 50% was classified as possible cases of CS. None of the patients received immunosuppressants. Conclusion: In our patient population with suspected CS and inflammation, we conducted a PET study following a CMR to assess the potential for CS. In the absence of a gold standard, it is suggested that the diagnosis of CS should be based on probabilities according to specific imaging patterns and clinical features.
ABSTRACT
Sarcoid Heart Disease (SHD), often misdiagnosed as tuberculosis is a potentially life-threatening condition.Cardiac manifestation of sarcoidosis is a challenging clinical condition as it is associated with pericarditis, heartblock, ventricular arrhythmias, valve dysfunction, ventricular aneurysm, sudden cardiac death, and heart failure.This study presents a case of a syncopal attack due to complete heart block, which is the first indication of SHD.The study has also presented a literature review on the various management options employed for therapeuticand effective management of SHD. The study has proposed treatment approaches, such as Cardiac MagneticResonance (CMR), Magnetic Resonance Imaging (MRI), and Insertion of an Implantable CardioverterDefibrillators (ICD) in patients with complete heart block to improve the quality of life and increase overallsurvival rates.
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Sarcoidosis is a multisystem disease characterized by noncaseating granulomas. Cardiac involvement is known to have poor prognosis because it can manifest as a serious condition such as the conduction abnormality, heart failure, ventricular arrhythmia, or sudden cardiac death. Although early diagnosis and early treatment is critical to improve patient prognosis, the diagnosis of CS is challenging in most cases. Diagnosis usually relies on endomyocardial biopsy (EMB), but its diagnostic yield is low due to the incidence of patchy myocardial involvement. Guidelines for the diagnosis of CS recommend a combination of clinical, electrocardiographic, and imaging findings from various modalities, if EMB cannot confirm the diagnosis. Especially, the role of advanced imaging such as cardiac magnetic resonance (CMR) imaging and positron emission tomography (PET), has shown to be important not only for the diagnosis, but also for monitoring treatment response and prognostication. CMR can evaluate cardiac function and fibrotic scar with good specificity. Late gadolinium enhancement (LGE) in CMR shows a distinctive enhancement pattern for each disease, which may be useful for differential diagnosis of CS from other similar diseases. Effectively, T1 or T2 mapping techniques can be also used for early recognition of CS. In the meantime, PET can detect and quantify metabolic activity and can be used to monitor treatment response. Recently, the use of a hybrid CMR-PET has introduced to allow identify patients with active CS with excellent co-localization and better diagnostic accuracy than CMR or PET alone. However, CS may show various findings with a wide spectrum, therefore, radiologists should consider the possible differential diagnosis of CS including myocarditis, dilated cardiomyopathy (DCM), hypertrophic cardiomyopathy, amyloidosis, and arrhythmogenic right ventricular cardiomyopathy. Radiologists should recognize the differences in various diseases that show the characteristics of mimicking CS, and try to get an accurate diagnosis of CS.
Subject(s)
Humans , Amyloidosis , Arrhythmias, Cardiac , Arrhythmogenic Right Ventricular Dysplasia , Biopsy , Cardiomyopathy, Dilated , Cardiomyopathy, Hypertrophic , Cicatrix , Death, Sudden, Cardiac , Diagnosis , Diagnosis, Differential , Early Diagnosis , Electrocardiography , Electrons , Gadolinium , Granuloma , Heart Defects, Congenital , Incidence , Magnetic Resonance Imaging , Myocarditis , Positron-Emission Tomography , Prognosis , Sarcoidosis , Sensitivity and SpecificityABSTRACT
A 44-year-old man presented with breathlessness and episodes of palpitations for the last one year. The imaging diagnosis of cardiac sarcoidosis was made based on chest radiography and cardiac magnetic resonance (MR) imaging findings, and was further confirmed by biopsy. Cardiac sarcoidosis is an uncommon entity, yet is potentially fatal with nonspecific clinical manifestations, including sudden cardiac death. Hence, it is important to diagnose and treat this entity at an early stage to improve morbidity and mortality. Cardiac MR imaging plays a pivotal role in facilitating diagnosis and monitoring therapeutic response. We describe the MR imaging features of cardiac sarcoidosis and discuss imaging features of other cardiomyopathies that may mimic cardiac sarcoidosis.
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Left ventricular overlapping operation was applied to a woman with cardiac sarcoidosis who presented with cardiopulmonary arrest. She did not have any particular history, with minimal risk factors for atherosclerosis. By-stander cardiopulmonary resuscitation delivered by her husband saved her life. Acute coronary syndrome was denied by emergency coronary arteriography. However, left vetriculography revealed broad dyskinetic area inconsistent with coronary distribution. Those findings raised a high suspicion of cardiac sarcoidosis, although this was unconfirmed. Broad ventricular aneurysm and a history of cardiac arrest and congestive heart failure prompted us to operate on her. Left ventricular overlapping operation was applied followed by ICD implantation and steroid induction. The pathologic examination of the ventricular wall showed noncaseous epithelioid granulomas and polynuclear giant cells, which are consistent with cardiac sarcoidosis. She has been leading a healthy daily life without signs of heart failure for one and a half years after discharge. Lesions of cardiac sarcoidosis have a broad spectrum from narrow localized to wide generalized aneurysm. Appropriate surgical strategy should be performed according to the location and the extent of the lesion. Left ventricular overlapping operation is among the alternatives.
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Resumen La miocarditis de células gigantes es una enfermedad rara, con gran impacto en cuanto a la mortalidad de pacientes usualmente jóvenes y sanos, que provoca diversas presentaciones clínicas como: la falla cardiaca, las arritmias ventriculares refractarias y los bloqueos. Su diagnóstico se establece con un alto índice de sospecha y con la confirmación por medio de la biopsia endomiocárdica por presencia de células gigantes multinucleadas sin presencia de un patrón granulomatoso. Su tratamiento inmunomodulador ha cambiado de manera importante en los últimos años, con una evidente disminución en los índices de mortalidad, aunque aún se requieren estrategias complementarias de manejo como el uso de dispositivos intracardiacos y/o el trasplante cardiaco para lograr mejores desenlaces a largo plazo.
Abstract Giant cell myocarditis is a rare condition with a great impact with regards to mortality of otherwise young and healthy patients, causing multiple clinical presentations, such as heart failure, refractory ventricular arrhythmias and blocks. Its diagnosis is reached with a high degree of suspicion and with the confirmation by means of a endomyocardial biopsy with the presence of multinucleated giant cells without a granulomatous pattern. Its immunomodulatory treatment has considerably changed during the last years, with a clear reduction in mortality rates, though complementary management strategies are still required, such as the use of intracardiac devices and/or heart transplantation in order to achieve better longterm results.
Subject(s)
Humans , Male , Adult , Giant Cells , Heart Failure , Myocarditis , Arrhythmias, Cardiac , Sarcoidosis , MortalityABSTRACT
Sarcoidosis is a systemic disease of young adults. Cardiac involvement is rarely diagnosed clinically. In most cases it presents with arrhythmias and conduction disorders. We report a case of sudden death of a young female, wherein sarcoidosis with prominent cardiac involvement was diagnosed at autopsy. The other organs involved were lung and liver. Cardiac sarcoidosis should be considered in young patients with unexplained conduction disorders.
ABSTRACT
Sarcoidosis, in general, has a low mortality rate. But cardiac sarcoidosis (CS) is a serious condition which may lead to death. Here, we report a rare case of CS that was treated by heart transplantation. A 47-year-old male had occasional syncopes and atypical chest pain. Ventricular tachycardia with right bundle branch block was noted by electrocardiogram. Multiple fixed myocardial perfusion defects in the interventricular septum and both the inferior-posterior ventricular walls were observed by thallium scan. Coronary angiography was unremarkable. Neither perihilar nor mediastinal lymphadenopathy was noted. The patient also suffered three times from tonic-clonic generalized seizures in 3 years, but no neurologic abnormalities were detected. The explanted heart displayed multiple white patches on the endomyocardial surface, measuring up to 8x7 cm. On microscopic examination, the lesion consisted of multiple well-formed and confluent granulomas with numerous scattered multinucleated giant cells, CD68-positive epithelioid histiocytes, and T-lymphocytes. Neither microorganisms nor foreign material was identified on special stain and culture study. It has been six months since the heart transplant, and the patient has been doing well.
Subject(s)
Humans , Male , Middle Aged , Bundle-Branch Block , Chest Pain , Coronary Angiography , Electrocardiography , Giant Cells , Granuloma , Heart , Heart Transplantation , Histiocytes , Lymphatic Diseases , Mortality , Perfusion , Sarcoidosis , Seizures , Syncope , T-Lymphocytes , Tachycardia, Ventricular , ThalliumABSTRACT
Patients with significant cardiac sarcoidosis are at increased risk of sudden death from ventricular dysrhythmias or conduction disturbances. We report a patient in whom there was radiographic and histologic evidence of systemic sarcoidosis; though histologic confirmation of involvement of heart by sarcoidosis is lacking, the clinical manifestations, radionuclide image findings, rhythm disturbances, and the response to steroid therapy are strong evidence in favor of myocardial involvement by the granulomatous process.