Prolapse and Regurgitation of the Four Heart Valves in a Patient with Ehlers-Danlos Syndrome: a Case Report.

BACKGROUND:The Ehlers-Danlos Syndrome (EDS) is part of a group of connective tissue diseases that affect the synthesis and processing of collagen leading to alterations in the structure of many tissues and organs.CASE SUMMARY:Herein, we reported a case of a patient with prolapse of the four cardiac valves. Non-significant regurgitation of the mitral, aortic, and tricuspid valves was observed. The pulmonary regurgitation (PR) was considered significant. Dilatation of the right ventricle with preserved systolic function was also noted. A cardiac magnetic resonance confirmed the findings of the echocardiogram and determined a severe PR (regurgitant fraction of 41%). The physical examination revealed hyperlaxity of the joints, skin hyperelasticity, defects in wound healing, and abdominal hernias suggesting EDS. The stress test did not develop any symptoms or complex arrhythmias. In this patient, the heart team initially decided medical treatment and evolutionary control. At the moment, he remains asymptomatic.DISCUSSION:Valvular involvement in EDS is an infrequent event and the compromise of the four cardiac valves is exceptional. The existence of severe PR with a marked increase in ventricular volumes, even in the absence of symptoms, in most cases requires an intervention on the valve. However, in patients with EDS, there are a high rate of complications and interventions should be avoided as much as possible.