# Severe mitral valve papillary muscle rupture of isolated Whipple's endocarditis: a case report and review of the literature

## TL;DR

A young woman with lupus developed severe heart failure from a rare form of endocarditis caused by Tropheryma whipplei, diagnosed through advanced genetic testing.

## Contribution

First reported case of isolated Whipple's endocarditis in a young Asian female with atypical presentation and rapid progression.

## Key findings

- Mitral valve papillary muscle rupture was the sole symptom of isolated TWE in a 35-year-old female.
- Metagenomic next-generation sequencing of bronchoalveolar lavage fluid detected T. whipplei, enabling diagnosis.
- Targeted antibiotic therapy improved septic shock and allowed discontinuation of ECMO support.

## Abstract

Tropheryma whipplei endocarditis (TWE) is rarely reported. Diagnosis is particularly challenging when it occurs as isolated TWE without classical manifestations of Whipple's disease.

A 35-year-old Asian female with systemic lupus erythematosus presented with acute heart failure secondary to mitral valve papillary muscle rupture as her sole symptom, requiring emergent veno-arterial extracorporeal membrane oxygenation support and urgent valve replacement. Intraoperative absence of vegetations and negative conventional microbiological examination preliminarily ruled out infective endocarditis. However, on postoperative day (POD) 3, her condition rapidly deteriorated into septic shock. Follow-up chest CT revealed bilateral asymmetric pulmonary infiltrates inconsistent with cardiogenic pulmonary edema alone. Metagenomic next-generation sequencing (mNGS) of bronchoalveolar lavage fluid detected T. whipplei, providing a crucial diagnostic breakthrough. Subsequent periodic acid-Schiff staining of the resected valve confirmed the definitive diagnosis of isolated TWE. Targeted meropenem therapy for 5 days resulted in significant improvement in both pneumonia and septic shock, permitting ECMO discontinuation. The patient was successfully extubated by POD 12 and discharged on POD 22 with oral co-trimoxazole and doxycycline in a stable condition.

We present the first case of isolated TWE in a young Asian female, notable for its atypical clinical presentation, fulminant progression, and profound diagnostic challenges. Clinicians should maintain a high vigilance for blood culture–negative endocarditis. Timely diagnosis and appropriate treatment are crucial for improving prognosis. mNGS analysis of samples from suspected disseminated sites may yield crucial diagnostic breakthrough.