Cor Vasa 2005, 46(7-8):300-307

A fatal course of heart failure in a patient with Churg-Strauss syndrome

Hana Froňková^1,2,*, Petr Ošťádal¹, David Alan¹, Jiří Vejvoda¹, David Zemánek¹, Libor Fila³, Ludmila Hornofová⁴, Vladimír Vondra⁵

¹ Kardiologická klinika, Fakultní nemocnice Motol a 2. lékařská fakulta Univerzity Karlovy, Praha

² Interní oddělení, Nemocnice s poliklinikou Rakovník, Rakovník

³ Pneumologická klinika

⁴ Ústav patologie a molekulární medicíny, Fakultní nemocnice Motol a 2. lékařská fakulta Univerzity Karlovy

⁵ Plicní oddělení, Praha 5, Praha, Česká republika

Churg-Strauss syndrome (CSS) is a rare systemic disease, characterized by bronchial asthma, hypereosinophilia, and necrotizing vasculitis of small-and medium-size vessels with extravascular eosinophilic granulommas. While, in most cases, it is the lungs, peripheral nervous system, and skin, which become affected, involvement of the heart, gastrointestinal system, kidneys, and the central nervous system is associated with a very poor prognosis. The onset of vasculitis is usually preceded by bronchial asthma by several years.
This case report describes the course of hospitalization of a 37-year-old patient, admitted for symptoms of myocarditis rapidly developing into severe heart failure. Based on other symptoms and examinations, CSS was diagnosed four days after hospital admission; despite aggressive therapy, heart failure worsened progressively to cardiogenic shock. Multiorgan fail-ure developed, caused at least partly by specific-organ vasculitis. Despite aggressive management of systemic disease and its organ complication, the patient died on day 7 post-hospitalization from refractory shock and multiorgan failure.
This case report presents CSS as a rare but serious disease, which has to be considered in patients with a history of asthma, myocarditis, and hypereosinophilia.

Keywords: Churg-Strauss syndrome; Vasculitis; Myocarditis; Heart failure; Cardiogenic shock

Published: July 1, 2005  Show citation