A 71-year-old-woman with a history of diabetes mellitus on insulin, coronary artery disease (s/p percutaneous coronary intervention), chronic kidney disease (stage III, eGFR = 30 mL/min/1.73 m2), and longstanding cardiomyopathy (S/P CRT-D implantation) presents for evaluation of progressive dizziness and shortness of breath.
In the home setting, her blood pressure was 70/50 mmHg. She was admitted to the hospital for further testing. A transthoracic echocardiogram showed a dilated left ventricle (LV) with LV diastolic dimension of 6.8 cm and systolic dimension of 6.0 cm, LV ejection fraction of 26%, and significant mitral regurgitation (MR). Her laboratory testing revealed an NT-ProBNP of 3542.0 pg/mL (increased). A transesophageal echocardiogram was performed to assess the severity of MR and showed the following:
Markedly dilated and hypcontractile LV (ejection fraction ~18%) (Video 1A)
LV non-compaction with marked/deep trabeculations of the apex (Video 1B)
Markedly restricted leaflet motion with a slightly eccentric, posteriorly-directed jet along the entire coaptation line (Video 1C)
A proximal isovelocity surface area (PISA) regurgitant area of 46 mm2 with regurgitant volume of 59 cc
Quantitative Doppler regurgitant volume of 125 cc with regurgitant orifice area of 96 mm2 and regurgitant fraction of 76%, which was supported by the three-dimensional vena contracta area measurement (Video 1D)
Systolic reversal of pulmonary vein inflow.
A right and left heart catheterization revealed RA=2 mmHg, RV=58/4 mmHg, PA=55/25/m34 mmHg, PCWP=24 mmHg, PA sat=52%, Fick CO=2.82 L/min, Fick CI=1.7 L/min/m2 (BSA = 1.58 m2). Left heart catheterization showed non-obstructive coronary artery disease with patent circumflex stent.
The patient refused consideration of destination left ventricular assist device given her limited home support and was placed on home intravenous milrinone, as well as her standard medications of Torsemide, Spironolactone. She did well for 2 weeks but presented again with progressive shortness of breath and multiple ICD shocks for ventricular tachycardia.
Video:
Video 1
What is the best treatment option for the patient at this time?
Show Answer
The correct answer is: D. Transcatheter mitral valve repair is an appropriate treatment option for this patient with secondary MR and symptoms despite medical therapy.
Choice A is incorrect.
Although sacubitril/valsartan has been given a class IB indication for treatment of Stage C and D heart failure given the results of randomized, controlled trials, patients with low blood pressure and significant chronic renal disease were excluded from the trials. The utility of the drug is likely limited in this patient. However, some studies have shown that in appropriate patients, MR can be reduced with this medical therapy.1
Choice B is incorrect.
In the setting of a non-circular regurgitant orifice, the PISA calculation of regurgitant orifice area and volume may underestimate other direct measurements, such as quantitative, volumetric assessment, and three-dimensional planimetry of the vena contracta area. In this patient, the volumetric and three-dimensional measurements were very consistent and suggested very severe mitral regurgitation.2,3
Choice C is incorrect.
This patient has several high surgical risk clinical features: acute presentation, recent ventricular fibrillation, and need for intravenous inotropes. Her STS score would be >8 (and the actual calculation was 19%).
Choice D is correct.
The 2020 update of the ACC Expert Consensus Decision Pathway (ECDP) on the Management of Mitral Regurgitation4 acknowledges new data from the COAPT trial5 that supports the use of transcatheter edge-to-edge (ETE) repair in selected patients with HF and secondary MR. In this trial, 614 patients were randomized to either ETE device plus guideline-directed medical therapy (GDMT) or GDMT alone. Followed out to 2 years, the annualized rate of all hospitalizations for heart failure was 35.8% per patient-year in the device group as compared with 67.9% per patient-year in the control group (hazard ratio, 0.53; 95% confidence interval [CI], 0.40 to 0.70; P<0.001). In addition, death from any cause within 24 months occurred in 29.1% of the patients in the device group as compared with 46.1% in the control group (hazard ratio, 0.62; 95% CI, 0.46 to 0.82; P<0.001). Importantly, the EDCP states that consideration of ETE repair should follow documentation of persistent symptoms despite optimal GDMT for heart failure with reduced ejection fraction. This patient underwent ETE repair with reduction of MR to 2+ (3D vena contracta area = 26 mm2 and on follow-up, reported significant improvement in her dyspnea.
Choice E is incorrect.
LV non-compaction describes a ventricular wall anatomy characterized by prominent left ventricular trabeculae, a thin compacted layer, and deep intertrabecular recesses. Rarely, LVNC is intrinsically part of a cardiomyopathy with associated LV dilation and/or dysfunction, and arrhythmias. Although transplantation could be performed, the patient has a number of relative contraindications: age >70-years-old, significant renal dysfunction, and limited social support system1.
Educational grant support provided by Abbott Structural Heart
To visit the hub for the Understanding and Managing MR: Evolving Science and Policy Grant, click here!
References
Yancy CW, et al. 2017 ACC/AHA/HFSA Focused Update of the 2013 ACCF/AHA Guideline for the Management of Heart Failure: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines and the Heart Failure Society of America. Circulation 2017 Aug 8;136(6):e137-e161.
Nishimura RA, et al. 2017 AHA/ACC Focused Update of the 2014 AHA/ACC Guideline for the Management of Patients With Valvular Heart Disease: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines. Circulation 2017 Jun 20;135(25):e1159-e1195.
Zoghbi WA, et al. Recommendations for Noninvasive Evaluation of Native Valvular Regurgitation: A Report from the American Society of Echocardiography Developed in Collaboration with the Society for Cardiovascular Magnetic Resonance. J Am Soc Echocardiogr 2017 Apr;30(4):303-371.
Bonow RO, et al. 2020 Focused Update of the 2017 ACC Expert Consensus Decision Pathway on the Management of Mitral Regurgitation: A Report of the American College of Cardiology Solution Set Oversight Committee. J Am Coll Cardiol 2020 May 5;75(17):2236-2270.
Stone GW, et al. Transcatheter Mitral-Valve Repair in Patients with Heart Failure. N Engl J Med 2018 Dec 13;379(24):2307-2318.
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