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

Mihnea Casian
Mihnea Casian
, 
Ciprian Jurcuţ
Ciprian Jurcuţ
, 
Camelia Dobrea
Camelia Dobrea
, 
Bogdan Radulescu
Bogdan Radulescu
 et 
Ruxandra Jurcuţ
Ruxandra Jurcuţ
   | 30 juin 2023
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Article Category: Case Raport
Publié en ligne: 30 juin 2023
Pages: 72 - 77
DOI: https://doi.org/10.2478/rjc-2023-0010
Mots clés
© 2023 Mihnea Casian et al., published by Sciendo
This work is licensed under the Creative Commons Attribution 4.0 International License.

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
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
CT findings. Notice the thickened pericardium and lymph node

Figure 3

Cardiac catheterization. Notice the square root sign (oval) and ventricular interdependence (arrow)
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
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 2020 Ascites Paracentesis 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 2020 Right pleural effusion Ascites ESR: 83 mm/h; hs-CRP: 52 ng/mlIgG: 2402 mg/dl (UNL <1600 mg/dl); serum immunofixation (negative)
April 2021 Bilateral pleural effusion AscitesArthralgia ESR: 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 2021 Bilateral pleural effusion QuantiFERON test: negativePleural tap: exudate, ADA <39 U/L, glucose 111 mg/dl, LDH 81 U/LPleural biopsy: no particular aspect
March 2022 Lower limb edema 24 h proteinuria: 355 mgACR 90 mg/g
November 2022 NYHA class III Referral 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”)

E A E/A Septal E’ Lateral E’ E/E’ (avg) Septal S’ Lateral S’
70 55 1.3 12.9 9.9 6.14 8 6
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Medicine, Clinical Medicine, Internal Medicine, Cardiology, Pediatrics and Juvenile Medicine, Paediatric Cardiology, Surgery, Cardiac Surgery
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