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

Casian, Mihnea; Jurcuţ, Ciprian; Dobrea, Camelia; Radulescu, Bogdan; Jurcuţ, Ruxandra

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

CT findings. Notice the thickened pericardium and lymph node

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

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

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 times per year

Keywords:

constrictive pericarditis,

Related subjects:

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

Figures & Tables

Figure 1

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Figure 4

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”)

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