Immunoglobulin G4-related constrictive pericarditis and the importance of a thorough workup: a case report

Abstract Background Constrictive pericarditis remains a problematic diagnosis and a thorough investigation is critical. Among possible aetiologies, immunoglobulin-G4 (IgG4)-related pericardial disease is an unusual cause of pericardial constriction. We report a challenging diagnostic case of pericar...

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Main Authors: Sérgio Maltês, Margarida Cabral, Pedro Freitas, Catarina Albuquerque, Carolina Fernandes, Diana Moura, Beatriz Santos, Miguel Mendes, José Neves
Format: Article
Language:English
Published: BMC 2022-02-01
Series:BMC Cardiovascular Disorders
Subjects:
Online Access:https://doi.org/10.1186/s12872-022-02468-1
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author Sérgio Maltês
Margarida Cabral
Pedro Freitas
Catarina Albuquerque
Carolina Fernandes
Diana Moura
Beatriz Santos
Miguel Mendes
José Neves
author_facet Sérgio Maltês
Margarida Cabral
Pedro Freitas
Catarina Albuquerque
Carolina Fernandes
Diana Moura
Beatriz Santos
Miguel Mendes
José Neves
author_sort Sérgio Maltês
collection DOAJ
description Abstract Background Constrictive pericarditis remains a problematic diagnosis and a thorough investigation is critical. Among possible aetiologies, immunoglobulin-G4 (IgG4)-related pericardial disease is an unusual cause of pericardial constriction. We report a challenging diagnostic case of pericardial constriction due to IgG4-related disease. Case presentation A 68-year old male with a history of inferior myocardial infarction with right ventricle (RV) involvement was thrice-hospitalized due to marked ascites and peripheral oedema. Systemic congestion was initially attributed to RV dysfunction due to previous infarction. Yet, at the final admission, a re-assessment echocardiogram followed by cardiac computed tomography, magnetic resonance and right heart catheterization raised a possible diagnosis of constrictive pericarditis with a finding of abnormal pulmonary venous return. Patient therefore underwent pericardiectomy and surgical correction of pulmonary venous return. Pericardium histology revealed an IgG4-related pericardial constriction. Patient was later discharged on corticosteroids with marked symptomatic improvement. Conclusion IgG4-related disease remains a rare cause of pericardium constriction while also presenting a challenging diagnosis in everyday clinical practice. This case exemplifies the difficulties faced by clinicians when reviewing a possible case of constrictive pericarditis, while highlighting the importance of a multimodality assessment.
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spelling doaj.art-b987452c051347fd9e408a466c997e852022-12-22T01:36:14ZengBMCBMC Cardiovascular Disorders1471-22612022-02-012211510.1186/s12872-022-02468-1Immunoglobulin G4-related constrictive pericarditis and the importance of a thorough workup: a case reportSérgio Maltês0Margarida Cabral1Pedro Freitas2Catarina Albuquerque3Carolina Fernandes4Diana Moura5Beatriz Santos6Miguel Mendes7José Neves8Cardiology Department, Hospital Santa Cruz, Centro Hospitalar Lisboa OcidentalCardiology Department, Hospital LeiriaCardiology Department, Hospital Santa Cruz, Centro Hospitalar Lisboa OcidentalAnatomical Pathology Department, Hospital Santa Cruz, Centro Hospitalar Lisboa OcidentalInternal Medicine Department, Hospital LeiriaInternal Medicine Department, Hospital LeiriaCardiology Department, Hospital LeiriaCardiology Department, Hospital Santa Cruz, Centro Hospitalar Lisboa OcidentalCardiothoracic Surgery Department, Hospital Santa Cruz, Centro Hospitalar Lisboa OcidentalAbstract Background Constrictive pericarditis remains a problematic diagnosis and a thorough investigation is critical. Among possible aetiologies, immunoglobulin-G4 (IgG4)-related pericardial disease is an unusual cause of pericardial constriction. We report a challenging diagnostic case of pericardial constriction due to IgG4-related disease. Case presentation A 68-year old male with a history of inferior myocardial infarction with right ventricle (RV) involvement was thrice-hospitalized due to marked ascites and peripheral oedema. Systemic congestion was initially attributed to RV dysfunction due to previous infarction. Yet, at the final admission, a re-assessment echocardiogram followed by cardiac computed tomography, magnetic resonance and right heart catheterization raised a possible diagnosis of constrictive pericarditis with a finding of abnormal pulmonary venous return. Patient therefore underwent pericardiectomy and surgical correction of pulmonary venous return. Pericardium histology revealed an IgG4-related pericardial constriction. Patient was later discharged on corticosteroids with marked symptomatic improvement. Conclusion IgG4-related disease remains a rare cause of pericardium constriction while also presenting a challenging diagnosis in everyday clinical practice. This case exemplifies the difficulties faced by clinicians when reviewing a possible case of constrictive pericarditis, while highlighting the importance of a multimodality assessment.https://doi.org/10.1186/s12872-022-02468-1Case reportConstrictive pericarditisImmunoglobulin G4-related diseaseRight heart failure
spellingShingle Sérgio Maltês
Margarida Cabral
Pedro Freitas
Catarina Albuquerque
Carolina Fernandes
Diana Moura
Beatriz Santos
Miguel Mendes
José Neves
Immunoglobulin G4-related constrictive pericarditis and the importance of a thorough workup: a case report
BMC Cardiovascular Disorders
Case report
Constrictive pericarditis
Immunoglobulin G4-related disease
Right heart failure
title Immunoglobulin G4-related constrictive pericarditis and the importance of a thorough workup: a case report
title_full Immunoglobulin G4-related constrictive pericarditis and the importance of a thorough workup: a case report
title_fullStr Immunoglobulin G4-related constrictive pericarditis and the importance of a thorough workup: a case report
title_full_unstemmed Immunoglobulin G4-related constrictive pericarditis and the importance of a thorough workup: a case report
title_short Immunoglobulin G4-related constrictive pericarditis and the importance of a thorough workup: a case report
title_sort immunoglobulin g4 related constrictive pericarditis and the importance of a thorough workup a case report
topic Case report
Constrictive pericarditis
Immunoglobulin G4-related disease
Right heart failure
url https://doi.org/10.1186/s12872-022-02468-1
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