An autopsy case of Parkes-Weber syndrome with high-output heart failure: Hemodynamic alterations following treatment for arteriovenous fistulas.
J Cardiol Cases
; 26(2): 118-121, 2022 Aug.
Article
en En
| MEDLINE
| ID: mdl-35949574
ABSTRACT
An autopsy case of Parkes-Weber syndrome presenting high-output heart failure in a patient who died at 52â¯years old, is reported. The patient had a tumor in the right buttock since childhood, that had grown up to a diameter of 40â¯cm diameter by the age of 43â¯years when he felt exertional dyspnea and was diagnosed as having high-output heart failure due to arteriovenous fistulas. Embolotherapy was attempted, which relieved the symptoms. After 4â¯years his heart failure deteriorated. We performed embolotherapy but his condition did not improve. He died 1.5â¯years later. The autopsy revealed the weight of the heart was 1040â¯g with abundant subendocardial and interstitial fibrosis. In this patient, the level of output had been over 16â¯L/min which lasted for nine years. The left ventricular ejection fraction (LVEF) decreased during the first five years. Each embolotherapy reduced the cardiac output (CO), which was achieved by a large decrease in heart rate (HR) and a small increase in stroke volume (SV) i.e. COâ¯=â¯HRâ¯×â¯SV, and was reflected in the increase in LVEF. Learning objectives:
â¢Parkes-Weber syndrome has occasionally extensive arteriovenous fistulas in the pelvis that show high-output heart failure.â¢Treatment of such cases is difficult, but embolotherapy has partial effects on improving hemodynamics.â¢A long-term high-output state induces interstitial myocardial fibrosis and collagenous subendocardial thickening.
Texto completo:
1
Colección:
01-internacional
Base de datos:
MEDLINE
Idioma:
En
Revista:
J Cardiol Cases
Año:
2022
Tipo del documento:
Article
País de afiliación:
Japón