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From clinic to echocardiography to microscope – the multimodal journey of a rare disease Cover

From clinic to echocardiography to microscope – the multimodal journey of a rare disease

Romanian Journal of Cardiology
Volume 33 (2023): Issue 2 (June 2023)
By: Mihnea Casian,  Ciprian Jurcuţ,  Camelia Dobrea,  Bogdan Radulescu and  Ruxandra Jurcuţ  
Open Access
|Jun 2023

Figures & Tables

Figure 1

Echocardiographic red flags. A, B: “Annulus paradoxus” with medial annular E’ (12.9 cm/s) higher than lateral annular E’ (9.9 cm/s); C: Hepatic vein PW Doppler profile with diastolic flow reversal; D: Dilated inferior vena cava (30 mm) with no respiratory variations

Figure 2

CT findings. Notice the thickened pericardium and lymph node

Figure 3

Cardiac catheterization. Notice the square root sign (oval) and ventricular interdependence (arrow)

Figure 4

Removed pericardium and lymph node microscopy. Notice the frequent IgG producing plasma cells

Previous investigations and medical history_ GI – gastrointestinal; TTE – transthoracic echocardiography; LVEF – left ventricular ejection fraction; ESR – erythrocyte sedimentation rate; hs-CRP – high-sensitivity C-reactive protein; UNL – upper normal limit; ANA – antinuclear antibodies; dsDNA – double strand DNA antibody; CCP – cyclic citrullinated peptide antibodies; cANCA – diffuse staining antineutrophil cytoplasmic antibodies; pANCA – perinuclear staining antineutrophil antibodies; ADA- adenosine deaminase; LDH – lactate dehydrogenase; ACR – albumin creatinine ratio

March 2020AscitesParacentesis with fluid analysis: transudateUpper and lower GI endoscopy: no abnormal findingsWhole-body CT: mild pericardial effusion, small mediastinal nodules, non-specific small pulmonary nodulesECG: low voltage, otherwise normalTTE: LVEF 60%, grade I diastolic dysfunction, mild pericardial effusion, no significant valve disease
October 2020Right pleural effusion AscitesESR: 83 mm/h; hs-CRP: 52 ng/mlIgG: 2402 mg/dl (UNL <1600 mg/dl); serum immunofixation (negative)
April 2021Bilateral pleural effusion AscitesArthralgiaESR: 88 mm/h: hs-CRP: 45 ng/mlIgG: 2650 mg/dl (UNL <1600 mg/dl); serum immunofixation (negative)Rheumatoid factor, ANA, dsDNA, CCP, cANCA, pANCA antibodies (negative)Bone marrow biopsy: 15% lymphocites, 3% plasmocytes, otherwise normal
May 2021Bilateral pleural effusionQuantiFERON test: negativePleural tap: exudate, ADA <39 U/L, glucose 111 mg/dl, LDH 81 U/LPleural biopsy: no particular aspect
March 2022Lower limb edema24 h proteinuria: 355 mgACR 90 mg/g
November 2022NYHA class IIIReferral to our clinic

TTE diastolic function assessment_ Notice that the E/E’ is abnormally low (annulus paradoxus) and that septal TDI velocities have higher values than the ones sampled from the lateral wall (“annulus reversus”)

EAE/ASeptal E’Lateral E’E/E’ (avg)Septal S’Lateral S’
70551.312.99.96.1486
DOI: https://doi.org/10.2478/rjc-2023-0010 | Journal eISSN: 2734-6382 | Journal ISSN: 1220-658X
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Language: English
Page range: 72 - 77
Published on: Jun 30, 2023
Published by: Romanian Society of Cardiology
In partnership with: Paradigm Publishing Services
Publication frequency: 4 issues per year
Keywords:
constrictive pericarditis,
IgG4 disease,
rare disease,
anasarca
Related subjects:
Medicine,
Clinical medicine,
Internal medicine,
Cardiology,
Pediatrics and juvenile medicine,
Paediatric cardiology,
Surgery,
Cardiac surgery

© 2023 Mihnea Casian, Ciprian Jurcuţ, Camelia Dobrea, Bogdan Radulescu, Ruxandra Jurcuţ, published by Romanian Society of Cardiology
This work is licensed under the Creative Commons Attribution 4.0 License.

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