Intrapericardial Aortic Pseudoaneurysm: A Rare Masquerade of Constrictive Pericarditis.
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ABSTRACT:
SUBMITTER: Lee KT
PROVIDER: S-EPMC6710824 | biostudies-literature | 2019 Aug
REPOSITORIES: biostudies-literature
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Intrapericardial Aortic Pseudoaneurysm: A Rare Masquerade of Constrictive Pericarditis.
Lee Kuo Ting KT Murugesu Kalaivani K Liew Houng Bang HB
CASE (Philadelphia, Pa.) 20190613 4
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Project description:Effusive-constrictive pericarditis (ECP) is an uncommon diagnosis, frequently missed due to its heterogeneous presentation, but a potentially reversible cause of heart failure. A 62-year-old Caucasian male presented with remittent right heart failure and mild-moderate pericardial effusion. Following an initial diagnosis of idiopathic pericarditis, indomethacin was started, but the patient shortly relapsed, presenting with severe pericardial effusion and signs of cardiac tamponade, requiring pericardiocentesis. ECP was diagnosed on cardiac catheterization. Cardiac computed tomography showed non-calcified, mildly thickened and inflamed parietal pericardium. Pericardiectomy was performed with symptoms remission. On histological examination of pericardium, chronic non-necrotizing granulomatous inflammation was noted. Polymerase chain reaction assay was positive for non-tuberculous mycobacteria. This case represents a rare finding of ECP with unusual presentation due to atypical mycobacteriosis in a non-immunocompromised patient and in a non-endemic area. Pericardiectomy can be an effective option in cases unresponsive to anti-inflammatory treatment, even in the absence of significant pericardial thickening or calcification.
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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:BackgroundChylothorax is a rare clinical condition that results from thoracic duct damage with leakage of chyle from the lymphatic system to the pleural space. Rarely, constrictive pericarditis has been associated with chylothorax, but to our knowledge only in relation to secondary causes such as tuberculosis, HIV, or malignancy.Case summaryA previously healthy 63-year-old man presented with effusive-constrictive pericarditis, recurrent right-sided pleural effusion, and chylothorax. There was no history of co-morbidities, surgical illness, or cardiac procedures. No single aetiologic factor was identified despite comprehensive assessment. Substantial immunosuppressive therapy was given without a sufficient clinical response. Pericardiectomy resulted in resolution of the constrictive haemodynamics and terminated chylous effusion.DiscussionThe hypothesized mechanisms for development of chylothorax in association with constrictive pericarditis are the increased effective capillary infiltration secondary to central venous hypertension and reduced lymphatic drainage due to high pressure in the left subclavian vein. Increased capillary filtration may result in excessive lymph formation. However, the mechanism is not completely understood.
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Project description:Familial constrictive pericarditis is extremely rare. We report a case of two brothers both suffering constrictive pericarditis along with having multiple painless joint deformities. Genetic workup confirmed the clinical diagnosis of camptodactyly-arthropathy-coxa vara-pericarditis (CACP) syndrome CACP syndrome and also revealed a rare mutation in the causative gene.
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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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Project description:Constrictive pericarditis (CP) is an uncommon post inflammatory disorder. It is described as pericardial thickening, myocardial constriction, and impaired diastolic filling. The most common etiologies are idiopathy, mediastinal radiotherapy, and prior cardiac surgery. Less common etiologies include viral infections, collagen vascular disorders, renal failure, sarcoidosis, tuberculosis, and blunt chest trauma. CP can less commonly be caused by malignancy. We report a very rare case of non-Hodgkin's lymphoma (NHL) presenting twice with attacks of decompensated heart failure. Echocardiography revealed that CP was responsible for the patient's symptoms as the first manifestation of NHL. Chest computed tomography scan and biopsy findings were compatible with the diagnosis of NHL. The patient received R-CHOP (cyclophosphamide, hydroxydaunorubicin, Oncovin®, and prednisone or prednisolone, combined with the monoclonal antibody rituximab) chemotherapy. Three months later, there was significant improvement in the patient's symptoms and considerable decrease in pericardial thickness.
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