Takotsubo cardiomyopathy complicating transcatheter mitral valve-in-valve replacement.

A 73-year-old female presented with symptomatic heart failure (NYHA class III) due to a failed 29-mm Sorin (St. Jude Medical, St. Paul, MN) bioprosthetic heart valve surgically implanted 10-year before for severe primary mitral valve regurgitation (flail of the anterior mitral leaflet at A2 scallop). Preprocedural echocardiography showed preserved left ventricular function with severe bioprosthetic valve stenosis and concomitant moderate regurgitation (Video 1 in Supplement). Because of prohibitive surgical risk, a transeptal mitral valve-in-valve (ViV) replacement was planned.[

Pre procedural CT showed an unfavorable aortomitral angle of 62° and a predicted neo-LVOT area of > 200 mm. For this patient, the novel BE transcatheter heart valve MyVal (Meril Life Science, Vapi, India) was selected, as it is the only balloon-expandable valve which can be retracted in case the device cannot be crossed through the interatrial septum. [ Based on preprocedural screening, a 27.5-mm MyVal THV was selected for this patient. Approval for investigational use of this device was obtained from the Ethics Committee of our institution.

The procedure was performed under general anesthesia with transesophageal echocardiogram (TEE) guidance. The valve was adjusted to cover the frame of the bioprosthesis, positioning the ventricular edge of the new valve at the ventricular edge of the preexisting bioprosthesis (Video 2 in Supplement). After transcatheter valve deployment the patient became unstable in need of inotropic and vasopressor therapy. TEE showed dysfunction of the newly deposed bioprosthesis with severe transvalvular and paravalvular regurgitation due to malcoaptation of the leaflets while a part of the Sorin bioprosthesis was protruding in the valve (Figure 1) (Video 3 in Supplement). Post dilatation resulted to no improvement in hemodynamics and imaging (Video 4 in Supplement). Decision was made then to proceed to a valve in valve in valve implantation (Video 5 in Supplement) with consequent good hemodynamics, no evidence of regurgitation, stenosis or left ventricle outflow tract obstruction (Video 6 in Supplement). The patient was transferred to the intensive care unit at a stable clinical state. Few hours later, the patient developed cardiogenic shock. TTE showed a well-implanted and functioning valve but identified severe left ventricular systolic dysfunction, with apical akinesia and compensatory hyperkinesia of all basal myocardial segments (Figure 2; Video 7 in Supplement). An intra-aortic balloon pump (IABP) was placed for hemodynamic support. Post-procedural electrocardiogram (ECG) demonstrated atrial fibrillation. Blood testing after the procedure showed elevation of high sensitive troponin at 312pg/mL (normal < 15 pg/mL) (versus 11 pg/mL at admission) that progressively decreased. On the basis of a typical echocardiographic appearance and a prior coronary angiogram showing no obstructive coronary artery disease (CAD), stress-induced cardiomyopathy (Takotsubo syndrome) was suspected. Gradual recovery was achieved the following days with eventual weaning from IABP and inotropes. Patient was eventually transferred to a cardiac rehabilitation unit 15 days after the procedure, preceding her returning home. Latest follow-up was obtained 2 months after the procedure: the patient was asymptomatic, transthoracic echocardiography showed well-functioning left ventricle with preserved ejection fraction and no wall-motion abnormalities. Valve was in situ without any regurgitation and the mean prosthetic gradient was stable (about 3 mmHg) (Video 8 in Supplement).

Figure 1

Severe dysfunction of the first implanted transcatheter valve due to the surgical valve leaflets interfering with its coaptation mechanism.

Figure 2

TTE post intervention at the ICU showing apical akinesia and compensatory hyperkinesia of all basal myocardial segments.

Takotsubo cardiomyopathy was diagnosed due to the presence of each of the following proposed Mayo Clinic criteria: (1) transient left ventricular wall motion abnormalities beyond a single epicardial vascular distribution; (2) absence of obstructive coronary artery disease; (3) modest elevation of high-sensitive troponin; and (4) absence of pheochromocytoma and myocarditis.[

Reports of Takotsubo syndrome after surgical mitral valve replacement are few.[ To the best of our knowledge, this is the first case describing the onset of a Takotsubo cardiomyopathy after transcatheter ViV implantation in a degenerated mitral bioprosthesis. Takotsubo is often described as being associated with a catecholamine surge, usually caused by stressful events or organic triggers. The procedural events that may have contributed to an adrenergic surge in this patient could have been the fast ventricular pacing for valve deposition and the need for administration of vasoactive and inotropic drugs. Still, the aforementioned factors may not be the sole cause for Takotsubo cardiomyopathy in this patient. One could speculate that the acute left ventricular volume loading after valve deposition may be an additional factor in a patient with prior severely stenotic mitral bioprosthesis. Indeed, acute volume loading may have led to an increase in left ventricular end-diastolic volume and pressure with concomitant decrease in coronary perfusion pressure which together with an increased myocardial oxygen demand, might have caused myocardial ischemia, left ventricular dysfunction and Takotsubo syndrome.

Takotsubo syndrome should be considered in the differential diagnosis of patients presenting with cardiogenic shock after transcatheter valve implantation in degenerated mitral bioprostheses and in most cases has a favorable outcome. In the subgroup of patients with severely stenotic mitral bioprostheses, acute left ventricular volume overload after valve deposition may presumably trigger Takotsubo syndrome.

CONFLICT OF INTEREST

None

TTE: transesophageal echocardiogram.

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