Even Mended Hearts Can Break: Takotsubo After Transcatheter Mitral Valve Replacement.

Transcatheter mitral valve replacement represents an innovative interventional technique for implanting a new mitral prosthesis without surgery. Although technical success is high, post-procedural issues and complications may occur. This report emphasizes the importance of considering alternative cardiac pathologies that may be seen post valvular interventions. (Level of Difficulty: Beginner.).

Transcatheter mitral valve replacement (TMVR) is a novel method of implanting an inverted, balloon-expandable, transcatheter aortic valve in the mitral position for degenerated mitral bioprostheses in high risk patients. The procedure represents a percutaneous option that reduces invasiveness and facilitates early recovery and improved outcomes. Despite being minimalistic, the post-procedural course may be complicated by unexpected issues. This case highlights the occurrence of Takotsubo cardiomyopathy (TCM) after TMVR and helps readers expand the differential diagnosis and management options after complex valvular interventions.

Learning Objectives

To recognize and evaluate the common causes of cardiac decompensation after TMVR.

To acknowledge that TCM may be a cause of cardiac failure after TMVR and requires urgent coronary angiography to clarify the diagnosis.

History of Presentation

A 79-year-old Caucasian woman presented with increasing dyspnea on exertion and worsening lower extremity swelling. Physical examination was remarkable for large V waves of the jugular vein, 3/6 diastolic murmur at the apex without opening snap, 3/6 holosystolic murmur at the left lower sternal border, mild right ventricular heave, and 2+ bilateral lower extremity pitting edema.

Past medical history

Her medical history included atrial fibrillation s/p dual chamber pacemaker, type 2 diabetes mellitus, cerebrovascular accident, systolic cardiomyopathy (ejection fraction 35% to 40%), history of 2 bioprosthetic mitral valve replacements (2003 and 2011 [29-mm Carpentier-Edwards PERIMOUNT (Edwards Lifesciences, Irvine, California]), and tricuspid ring repair (32 mm).

Investigations

Transthoracic echocardiography (TTE) and transesophageal echocardiography (TEE) demonstrated ejection fraction of 35% to 40% (previously 45% to 50%), right ventricular systolic pressure of 80 mm Hg (previously 42 mm Hg), severe tricuspid regurgitation (previously mild to moderate), and severely degenerated mitral valve leaflets with thickening, calcification, and restricted mobility associated with severe mitral regurgitation. The mean mitral gradient 15 mm Hg, peak mitral E-wave velocity 3.0 m/s, and a mitral valve velocity-time integral to left ventricular outflow tract velocity-time integral ratio of 3.75 were suggestive of severe bioprosthetic mitral stenosis (Figures 1A and 1B). Coronary angiogram demonstrated no significant obstructive coronary disease and right heart catheterization demonstrated pulmonary capillary wedge to left ventricular end-diastolic mean pressure gradient of 20 mm Hg, wedge pressure V waves to 40 to 50 mm Hg, and preserved cardiac output (Figure 2).

Figure 1

3D Transesophageal Echocardiogram and Mitral Valve Gradients

(A) Pre-implant 3-dimensional (3D) diastolic frame of the mitral valve from the left atrial perspective demonstrates restricted opening with thickening and calcification of the leaflets. Note the linear density crossing the central orifice is the guidewire used for the transcatheter mitral valve replacement. (B) Transesophageal echocardiogram continuous-wave doppler evaluation demonstrates a mean mitral gradient of 15 mm Hg. (C) Post-implant 3D diastolic frame of the mitral valve from the left atrial perspective demonstrates a completely open valve that is normal functioning. (D) Transthoracic echocardiogram continuous-wave doppler evaluation demonstrates a mean mitral gradient of 7 mm Hg.

Figure 2

Pre-Procedure Hemodynamic Tracing

Simultaneous pulmonary capillary wedge pressure and left ventricular end-diastolic pressure (EDP) tracings demonstrating a mean pressure gradient of approximately 20 mm Hg. Note the large V waves up to 40 to 50 mm Hg suggestive of severe mitral regurgitation. LV = left ventricular; PCW = pulmonary capillary wedge.

Clinical course

After a comprehensive heart team discussion, the patient was deemed prohibitive surgical risk and appropriate for TMVR with a 29-mm SAPIEN 3 valve (Edwards Lifesciences) based on the true internal diameter of the surgical bioprosthetic mitral valve and estimation of the neo–left ventricular outflow tract (LVOT) by embedding a virtual valve on multidetector computed tomography (Figure 3). The patient underwent transfemoral venous, transseptal access with TEE. After 14-mm balloon septostomy, during rapid ventricular pacing, a nominally prepped, inverted 29-mm SAPIEN 3 was deployed without complication (Figure 4) and TEE demonstrated a normal functioning mitral prosthesis (mean gradient 7 mm Hg) and a small, residual left-to-right shunt (5 to 6 mm) (Figures 1C and 1D). The entire procedure was approximately 60 min and the patient was extubated per protocol. After 2 to 3 h, the patient became hypotensive and dyspneic with accessory muscle use. She was immediately reintubated and intravenous pressor support was initiated.

Figure 3

Multidetector Computed Tomography With Virtual Mitral Valve

Estimation of the neo–left ventricular outflow tract area demonstrating an area of 3.95 cm2 (acceptable >1.7 cm2).

Figure 4

Still Frame of Transcatheter Mitral Valve Replacement Implantation

Differential diagnosis

The differential diagnoses at the time were pericardial tamponade, coronary embolization, access site–related bleeding, LVOT obstruction (owing to small neo-LVOT area), worsening left-to-right shunt, reversal of shunt (right to left), anesthetic drug effects, or undiagnosed transcatheter valve dysfunction.

Management

Urgent TTE demonstrated ejection fraction of 20% to 25%, new apical akinesis, preserved basal function, well-functioning mitral prosthesis (mean gradient 6 to 8 mm Hg), no significant aortic gradient, no pericardial effusion, and a small left-to-right atrial shunt.

She underwent right radial coronary angiography with optical coherence tomography of the left anterior descending artery, which showed no concerns for coronary obstruction (Figure 5). Right heart catheterization showed a transmitral gradient of 7 mm Hg, with an expected oxygen saturation step up of 7% to 9% at the atrial level (pulmonary artery oxygen saturation 79%). Left ventriculography demonstrated findings suggestive of TCM (Figure 6, Video 1) and no significant pullback systolic gradient across the aortic valve.

Figure 5

Post–Transcatheter Mitral Valve Replacement Coronary Angiogram

Right anterior oblique-cranial projection of the left coronary system shows no obvious obstruction.

Figure 6

Post–Transcatheter Mitral Valve Replacement Left Ventriculography

Diastolic and systolic still frames of the left ventricle show preserved basal systolic function and an akinetic apex (Takotsubo cardiomyopathy). Transcatheter mitral valve replacement is seen and no significant opacification of the left atrium is seen during systole (no mitral regurgitation). See Video 1.

Online Video 1

Left Ventriculogram

Post–transcatheter mitral valve replacement left ventriculography reveals mid to distal anterior, apical, and inferoapical akinesis with preserved basal segment contractility suggestive of Takotsubo cardiomyopathy. The fluoroscopic image of the transcatheter mitral valve replacement is seen and no significant mitral regurgitation is seen.

The patient was managed with diuresis and minimal vasopressor or inotropic support, and ultimately extubated the next day. She convalesced appropriately and was ultimately discharged to home on aggressive medical therapy for congestive heart failure.

Discussion

TCM, in its classic form, is characterized as an acute, transient worsening of left ventricular systolic function characterized by apical akinesis without the presence of obstructive coronary disease in the setting of a severe emotional or physical stressor (1). Central to the pathophysiology is excessive sympathetic stimulation due to an acute catecholamine surge, resulting in epicardial vasospasm, microvascular dysfunction, and cellular toxicity (2,3). The mainstay of therapy includes diuretics and noncatecholamine vasopressor support, and the addition of guideline-directed heart failure therapy after stabilization (1).

With the advent of transcatheter valvular interventions, TMVR has become an option in the management of failing surgical mitral valve bioprostheses. Before TMVR, the standard of care was surgical management. Multiple instances of TCM have been reported after cardiac surgery including mitral valve replacement (4,5). However, to our knowledge, this is the first report of post-TMVR TCM. Similarly, TCM after transcatheter aortic valve replacement has only rarely been described (6). Despite being a minimally invasive percutaneous procedure as compared with open cardiac surgery, TMVR still represents a significant physical stressor that may invoke the onset of TCM in the appropriate clinical circumstance. The anesthesia, large-bore access, TEE, transseptal puncture, balloon septostomy, and rapid ventricular pacing all represent potential procedural elements that may result in significant cardiac stress. Further, rapid ventricular pacing may be prolonged or repeated in various instances during the procedure and thus may in itself induce transient left ventricular dysfunction in addition to that which may be potentially seen with TCM. Although no one epidemiologic factor has been linked to the development of TCM, elderly, post-menopausal women with pre-existing psychiatric or physiologic stressors tend to be more likely to manifest the condition (1), all of which were seen in the present case.

Typically, post-TMVR concerns, as noted previously, should be evaluated by immediate TTE. TCM is acutely indistinguishable from coronary embolization to the left anterior descending artery; thus, immediate coronary angiography (± intravascular imaging) is necessary to definitively exclude this life-threatening complication. We also suggest right heart catheterization to exclude a very large interatrial shunt that may need to be percutaneously closed in certain instances, as well as to assess cardiac output, and to guide diuresis and vasopressor or inotropic therapies.

Follow-up

One month later, the patient was markedly improved and demonstrated an ejection fraction of 45%. The apical wall motion abnormality had resolved and the new transcatheter mitral valve was functioning appropriately (Figure 7, Video 2).

Figure 7

30-Day Transthoracic Echocardiogram

Diastolic and systolic 2-chamber still frames demonstrating improvement in the apical wall motion abnormality and the overall left ventricular systolic function. See Video 2.

Online Video 2

30-Day 2-Chamber Transthoracic Echocardiogram

Resolution of the previously seen wall motion abnormalities is no longer seen, which provides corroborative evidence regarding the diagnosis of Takotsubo cardiomyopathy, given the transient nature of the cardiomyopathy.

Conclusions

TMVR represents an advanced interventional option for those who are at high surgical risk for standard mitral reoperation. Post-procedural complications are infrequent with meticulous pre-procedural planning and careful stepwise intraprocedural manipulation. Structural heart interventionalists should be able to not only complete the procedure successfully, but also handle post-procedural issues. This case demonstrates the need to keep TCM in the differential of post-TMVR cardiac failure.