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Published on: April 8, 2018
Surgical treatment of chronic aortic dissection with liver dysfunction due to constrictive pericarditis caused by
Akie Shimada1, Taira Yamamoto1, Shizuyuki Dohi1
1Department of Cardiovascular Surgery, Juntendo Nerima Hospital, Takanodai 3-1-10, Nerima-Ku, Tokyo 177-8521, Japan.
Insights
Severe liver failure and ascites in a 72-year-old man were caused by constrictive pericarditis and aortic dissection, identified as immunoglobulin G4-related disease (IgG4-RD). Successful aortic surgery was performed, highlighting the importance of collaborative care.
Area of Science:
- Cardiology
- Gastroenterology
- Immunology
Background:
- Ascites and liver failure can be manifestations of cardiac conditions like constrictive pericarditis and aortic dissection.
- Immunoglobulin G4-related disease (IgG4-RD) can present with diverse systemic manifestations, including cardiovascular and hepatic involvement.
Observation:
- A 72-year-old male presented with ascites and scrotal edema, initially managed for liver issues.
- Computed tomography revealed DeBakey type II aortic dissection, pericardial thickening, and right ventricular dysfunction.
- Histopathological analysis showed IgG4-related disease (IgG4-RD) as the underlying cause, confirmed by response to steroid treatment.
Findings:
- The patient underwent successful ascending aortic replacement.
- IgG4 staining of the aortic wall indicated a ratio of 35% IgG4/IgG positive cells.
- Despite initial suspicion of IgG4-related aortitis, the diagnosis of IgG4-RD was established based on clinical and laboratory findings.
Implications:
- This case underscores the critical role of cardiac evaluation in patients presenting with ascites and liver dysfunction.
- Early diagnosis and management of IgG4-RD are crucial for preventing severe systemic complications.
- Multidisciplinary collaboration is essential for managing complex cases involving multiple organ systems.
Background:
Severe liver failure with ascites may be associated with cardiac disease and may be the primary manifestation of constrictive pericarditis or aortic dissection. We report a case of a patient with a chief complaint of ascites for whom close examination revealed that the liver injury was attributed to constrictive pericarditis and chronic aortic dissection, with immunoglobulin G4 (IgG4)-related disease (IgG4-RD) as the primary cause.
Case Summary:
A 72-year-old man presented to the emergency department with scrotal oedema and ascites. Initially, the patient was hospitalized in the Department of Hepatology. However, computed tomography (CT) revealed aortic dissection (DeBakey type II), pericardial thickening, and impaired right ventricular dilatation. Therefore, we performed an ascending aortic replacement. IgG4 staining of the aortic wall revealed an IgG4/IgG-positive cell ratio of 35%. Pathological examination did not confirm the diagnosis of IgG4-related aortitis; however, the patient was diagnosed with IgG4-RD because of decreased blood IgG4 levels in response to steroid medication and the presence of heterogeneous thickened lesions in the pericardium. The patient took prednisolone 5 mg/day for 1 month post-operatively. His IgG4 level decreased but re-elevated above the baseline value after discontinuation of oral medication.
Discussion:
Liver cirrhosis was suspected given the ascites, although a CT scan on admission confirmed insufficiency of systemic circulation due to cardiac constrictive pericarditis with aortic dissection. Despite the complexity of various pathologies in this patient, collaborative efforts and effective communication within the medical team enabled successful aortic surgery, averting life-threatening complications.
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