Libman-Sacks Endocarditis in a Congenital Valve Defect: A Case Report

TL;DR

A 20-year-old woman with lupus and a heart valve defect developed a rare heart condition that led to severe complications and death despite treatment.

Contribution

This case report highlights the fatal outcome of Libman-Sacks endocarditis in a patient with congenital valve malformation.

Findings

LSE occurred in a patient with congenital mitral valve agenesis and SLE.

Despite treatment, severe valvulopathy led to fatal right heart failure.

Congenital valve defects may worsen outcomes in LSE patients.

Abstract

Libman-Sacks endocarditis (LSE) is a rare complication of systemic lupus erythematosus (SLE), characterized by noninfectious vegetation on normal heart valves. We present the case of a 20-year-old woman with SLE and a congenital valve malformation. Despite a year of effective SLE treatment, she later developed stage IV dyspnea, chest pain, and signs of right heart failure. Investigations revealed active lupus, mitral valve vegetation, agenesis of the posterior mitral leaflet, and severe mitral insufficiency. The patient was treated with corticosteroids, antibiotics, anticoagulants, and symptomatic heart failure management. Despite initial improvement, her condition deteriorated, and she did not respond to resuscitation. While LSE often responds well to treatment, severe valvulopathy, particularly with congenital valve defects, can result in fatal outcomes.

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