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Improvement of dilated cardiomyopathy with methylprednisolone in a patient with multiple fibrosclerosis.

Altun I, Guz G, Akin F, Kose N, Altun I, Gunaydin ZY, Sahin C - Int Cardiovasc Res J (2014)

Bottom Line: There are few reports of multiple fibrosclerosis with heart failure.Here, we reported a case of retroperitoneal fibrosis with massive mediastinal involvement extending to pleura and pericardium causing pleuro- pericardial effusion with dilated cardiomyopathy.Systolic dysfunction was improved and pericardial effusion disappeared with methylprednisolone treatment.

View Article: PubMed Central - PubMed

Affiliation: Mugla Sitki Kocman University, Faculty of Medicine, Department of Cardiology, Mugla, Turkey.

ABSTRACT
Multifocal fibrosclerosis is a rare syndrome of unknown cause that is characterized by fibrosis involving multiple organ systems. Definitive diagnosis can only be made based on biopsy findings. In this case, the biopsy specimen of the patient demonstrates pulmonary hyalinated granuloma or sclerosing mediastinitis. There are few reports of multiple fibrosclerosis with heart failure. Here, we reported a case of retroperitoneal fibrosis with massive mediastinal involvement extending to pleura and pericardium causing pleuro- pericardial effusion with dilated cardiomyopathy. Systolic dysfunction was improved and pericardial effusion disappeared with methylprednisolone treatment.

No MeSH data available.


Related in: MedlinePlus

Transthoracic Echocardiogram Showing the Left Ventricle. Ejection Fraction Was Calculated as 45%
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fig13734: Transthoracic Echocardiogram Showing the Left Ventricle. Ejection Fraction Was Calculated as 45%

Mentions: Ureterolysis was planned after 3 months. We were concerned about the malignancy and performed thoracoscopic biopsy from the most involved site on Positron Emission Tomography (PET) (anterolateral mediastinum on the level of left 6 - 7 the intercostal space). Pathological evaluations revealed cross-sections of thick walled vessels, hyalinated connective tissue with lenphoid cells on some areas, pulmonary hyalinated granuloma, and sclerosing mediastinitis. According to cardiac MRI, we considered myocarditis as a cause of dilated cardiomyopathy (Figure 1), but did not perform biopsy. Initially, the patient received methylprednisolone 32 mg per day, tapering 4 mg every 2 weeks. After initiation of corticosteroid treatment, the patient felt better and denied heart failure symptoms. Control echocardiogram was performed two weeks later. Accordingly, ejection fraction was increased to 35% and pericardial effusion was minimal. The patient was discharged with desirable clinical conditions. During outpatient follow-up 1.5 months later, the control echocardiogram showed ejection fraction to be 45% (Figure 2).


Improvement of dilated cardiomyopathy with methylprednisolone in a patient with multiple fibrosclerosis.

Altun I, Guz G, Akin F, Kose N, Altun I, Gunaydin ZY, Sahin C - Int Cardiovasc Res J (2014)

Transthoracic Echocardiogram Showing the Left Ventricle. Ejection Fraction Was Calculated as 45%
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4302507&req=5

fig13734: Transthoracic Echocardiogram Showing the Left Ventricle. Ejection Fraction Was Calculated as 45%
Mentions: Ureterolysis was planned after 3 months. We were concerned about the malignancy and performed thoracoscopic biopsy from the most involved site on Positron Emission Tomography (PET) (anterolateral mediastinum on the level of left 6 - 7 the intercostal space). Pathological evaluations revealed cross-sections of thick walled vessels, hyalinated connective tissue with lenphoid cells on some areas, pulmonary hyalinated granuloma, and sclerosing mediastinitis. According to cardiac MRI, we considered myocarditis as a cause of dilated cardiomyopathy (Figure 1), but did not perform biopsy. Initially, the patient received methylprednisolone 32 mg per day, tapering 4 mg every 2 weeks. After initiation of corticosteroid treatment, the patient felt better and denied heart failure symptoms. Control echocardiogram was performed two weeks later. Accordingly, ejection fraction was increased to 35% and pericardial effusion was minimal. The patient was discharged with desirable clinical conditions. During outpatient follow-up 1.5 months later, the control echocardiogram showed ejection fraction to be 45% (Figure 2).

Bottom Line: There are few reports of multiple fibrosclerosis with heart failure.Here, we reported a case of retroperitoneal fibrosis with massive mediastinal involvement extending to pleura and pericardium causing pleuro- pericardial effusion with dilated cardiomyopathy.Systolic dysfunction was improved and pericardial effusion disappeared with methylprednisolone treatment.

View Article: PubMed Central - PubMed

Affiliation: Mugla Sitki Kocman University, Faculty of Medicine, Department of Cardiology, Mugla, Turkey.

ABSTRACT
Multifocal fibrosclerosis is a rare syndrome of unknown cause that is characterized by fibrosis involving multiple organ systems. Definitive diagnosis can only be made based on biopsy findings. In this case, the biopsy specimen of the patient demonstrates pulmonary hyalinated granuloma or sclerosing mediastinitis. There are few reports of multiple fibrosclerosis with heart failure. Here, we reported a case of retroperitoneal fibrosis with massive mediastinal involvement extending to pleura and pericardium causing pleuro- pericardial effusion with dilated cardiomyopathy. Systolic dysfunction was improved and pericardial effusion disappeared with methylprednisolone treatment.

No MeSH data available.


Related in: MedlinePlus