Rare murmur in a patient with constrictive pericarditis.
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ABSTRACT:
SUBMITTER: Abe Y
PROVIDER: S-EPMC8169184 | biostudies-literature | 2021
REPOSITORIES: biostudies-literature
Publications
Rare murmur in a patient with constrictive pericarditis.
Abe Yukio Y Saito Toshio T Naruko Takahiko T
Cardiology journal 20210101 3
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Project description:IntroductionConstrictive pericarditis is characterized by constriction of the heart secondary to pericardial inflammation. Cardiovascular magnetic resonance (CMR) imaging is useful imaging modality for addressing the challenges of confirming this diagnosis. It can be used to exclude other causes of right heart failure, such as pulmonary hypertension or myocardial infarction, determine whether the pericardium is causing constriction and differentiate it from restrictive cardiomyopathy, which also causes impaired cardiac filling.Case presentationA 77-year-old man from a country with high incidence of tuberculosis presented with severe dyspnea. Echocardiography revealed a small left ventricle with normal systolic and mildly impaired diastolic function. Left heart catheterization revealed non-obstructive coronary disease, not felt contributory to the dyspnea. Anatomy imaging with cardiovascular magnetic resonance imaging (CMR) showed global, severely thickened pericardium. Short tau inversion recovery (STIR) sequences for detection of oedema/ inflammation showed increased signal intensity and free breathing sequences confirmed septal flattening on inspiration. Late gadolinium imaging confirmed enhancement in the pericardium, with all findings suggestive of pericardial inflammation and constriction.ConclusionsCMR with STIR sequences, free breathing sequences and late gadolinium imaging can prove extremely useful for diagnosing constrictive pericarditis.
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Project description:BackgroundConstrictive pericarditis represents a chronic condition and systemic inflammatory diseases are a known, yet uncommon, cause. Pericardial involvement is seldom reported in primary Sjögren's syndrome, usually occurring in association with pericardial effusion or pericarditis. We report a case of constrictive pericarditis with an insidious course and unusual evolution associated with primary Sjögren's syndrome. Due to the challenging nature of the diagnosis, clinical suspicion and multimodality imaging are essential for early identification and prompt initiation of treatment. Long-term outcomes remain uncertain. To the best of our knowledge, no other cases linking this autoimmune disease to constrictive pericarditis have been reported.Case presentationWe present the case of a 48-year-old male patient with moderate alcohol habits and a history of two prior hospitalizations. On the first, the patient was diagnosed with primary Sjögren's syndrome after presenting with pleural effusion and ascites, and empirical corticosteroid regiment was initiated. On the second, two-years later, he was readmitted with complaints of dyspnea and abdominal distension. Thoracic computed tomography revealed a localized pericardial thickening and a thin pericardial effusion, both of which were attributed to his rheumatic disease. A liver biopsy showed hepatic peliosis, which was considered to be a consequence of glucocorticoid therapy. Diuretic therapy was adjusted to symptom-relief, and a tapering corticosteroid regimen was adopted. Four years after the initial diagnosis, the patient was admitted again with recurrent dyspnea, orthopnea and ascites. At this time, constrictive pericarditis was diagnosed and a partial pericardiectomy was performed. Although not completely asymptomatic, the patient reported clinical improvement since the surgery, but still with a need for baseline diuretic therapy.ConclusionAlbeit uncommon, connective tissue disorders, such as primary Sjögren's syndrome, should be considered as a potential cause of constrictive pericarditis, especially in young patients with no other classical risk factors for constriction. In this case, after excluding possible infectious, neoplastic and autoimmune conditions, a primary Sjögren´s syndrome in association with constrictive pericarditis was assumed. This case presents an interesting and challenging clinical scenario, highlighting the importance of clinical awareness and the use of multimodal cardiac imaging for early recognition and treatment.
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