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Published on: April 21, 2010
Constrictive pericarditis in a patient with inherited factor VII deficiency
R V Ponomarev1, S V Model1, N I Zozulya1
1National Research Center for Hematology, Moscow, Russia.
Insights
Constrictive pericarditis, a heart failure cause, was diagnosed in a patient with factor VII deficiency. Surgical pericardectomy significantly improved the patient's heart function.
Area of Science:
- Cardiology
- Hematology
Background:
- Constrictive pericarditis (CP) is a severe cardiac condition resulting from pericardial inflammation, thickening, and calcification.
- It impedes diastolic filling, leading to reduced cardiac output and heart failure.
Observation:
- A clinical case of CP is presented in a patient with a rare inherited bleeding disorder, factor VII deficiency.
- Initial suspicion of heart failure due to CP was based on clinical presentation and non-invasive imaging (ultrasound, radiology).
Findings:
- Cardiac magnetic resonance imaging confirmed the diagnosis of constrictive pericarditis.
- The patient underwent pericardectomy, a surgical procedure to remove the pericardium.
Implications:
- The successful pericardectomy resulted in significant clinical improvement, highlighting the importance of accurate diagnosis and timely intervention.
- This case underscores the potential for CP to occur in patients with rare bleeding disorders and the effectiveness of surgical management.
Abstract:
Constrictive pericarditis (CP) is the final stage of a chronic inflammatory process characterized by fibrous thickening and calcification of the pericardium that impairs diastolic filling, reduces cardiac output, and ultimately leads to heart failure. We present a clinical case of CP in a patient with rare inherited bleeding disorder - factor VII deficiency. Heart failure due to CP was suspected based on clinical symptoms, results of ultrasonic and radiological investigations. The diagnosis was verified by the results of cardiac magnetic resonance imaging. Pericardectomy was performed resulting in significant improvement in the patient's condition.
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