Heyde syndrome: Experiences with the use of semiautomatic vWF multimer analysis for diagnosis and TAVI for treatment - case report

Abstract

Rationale: Aortic stenosis (AS) is currently the most frequent valve disorder. In addition, the angiodysplasias are the most common vascular malformations of the gastrointestinal tract. Heyde syndrome (HS) is a rare disease which links these 2 pathological conditions.

Patient concerns: Currently, there are no clearly defined guidelines for the confirmation of HS diagnosis and for timing of aortic valve replacement in patients with confirmed HS, despite the fact that HS is connected with increased mortality and the need for numerous rehospitalizations and multiple blood transfusions. Therefore, the aim of this study was to report (our) first experiences with novel diagnostic method for acquired von Willebrand (vW) syndrome and transcatheter aortic valve replacement (TAVR) for treatment of HS.

Diagnoses: We report a case of a 68-year-old man who was diagnosed with HS with the use of semiautomatic vW factor multimer analysis.

Interventions: A successful TAVR was used for treatment of AS.

Outcomes: After the interventional treatment of AS, the patient did not have a recurrence of the anemic syndrome.

Lessons: This is a unique case of a patient with HS in whom semiautomatic vW factor multimer analysis was used for diagnosis of acquired vW syndrome together with TAVR procedure for HS treatment.

Keywords: Heyde syndrome; acquired von Willebrand syndrome; aortic stenosis; semiautomatic von Willebrand factor multimer analysis.

Figure 1.

Severe aortic stenosis on transesophageal echocardiography (A), pulsed Doppler echocardiography during dobutamine loading focusing on blood flow in the left ventricular outflow tract (B), continuous Doppler echocardiography during dobutamine loading focused on the aortic valve (C).

Figure 2.

Analysis of vWF multimers with the Hydrasys 2 scan densitometric device: example of device (A), example of a study protocol (B), study protocol of our patient confirming acquired von Willebrand syndrome/blue curve – patient sample, yellow curve – control (C). vWF = von Willebrand factor.

Figure 3.

Transfemoral TAVI – positioning of the bioprosthesis in the stenotic native valve (A), Expanding the bioprosthesis with a balloon (B), Suppression of the native valve by a new bioprosthesis that immediately assumes a role in the cardiac cycle (C). TAVI = transcatheter aortic valve implantation.

Figure 4.

The dynamics of hemoglobin levels during treatment of various etiologies and iron/red blood cell concentrates supplementation and after TAVI procedure. TAVI = transcatheter aortic valve implantation.