Nonobstructive Hypertrophic Cardiomyopathy in a Patient With Mitral Prosthesis

Transapical septal myectomy can be performed for patients with nonobstructive hypertrophic cardiomyopathy and diastolic dysfunction to enlarge the LV cavity. The procedure may also include limited shaving of severely hypertrophied papillary muscles. This report illustrates usefulness muscle resection left ventricle in a patient preexisting mitral valve prosthesis. Videos viewed online version this article [10.1016/j.athoracsur.2020.09.059] on http://www.annalsthoracicsurgery.org. Approximately one-third symptomatic (HCM) have form. Some these present reduction volume ventricular (LV) cavity, reduced end-diastolic contributes stroke as well cardiac output exercise. Surgical enlargement cavity through transapical approach improve function.1Schaff H.V. Brown M.L. Dearani J.A. et al.Apical myectomy: new surgical technique management apical cardiomyopathy.J Thorac Cardiovasc Surg. 2010; 139: 634-640Abstract Full Text PDF PubMed Scopus (66) Google Scholar In addition, muscles prevent midventricular obstruction.2Nguyen A. Schaff Nishimura R.A. advanced heart failure.J 2020; 159: 145-152Abstract (32) Scholar,3Kunkala M.R. al.Transapical obstruction cardiomyopathy.Ann 2013; 96: 564-570Abstract (65) report, we describe HCM who had failure (HF) prosthesis; was treated by A 48-year-old woman history presented severe exertional dyspnea. She found at 26 years age, 38 age another institution underwent transaortic replacement 27-mm St Jude prosthesis (St Medical, Paul, MN) outflow tract regurgitation. did subsequent 8 years, but more than 1 year before presentation, she developed what thought respiratory infection. long-standing persistent atrial fibrillation experienced chest pain shortness breath causing marked functional limitation (New York Heart Association class III). Her ejection fraction 67% no dynamic gradient. felt referred our Clinic consideration further including transplantation. At time evaluation, transthoracic echocardiography demonstrated normal function only mild transprosthetic There subaortic or gradient either rest provocation, maximum interventricular thickness 24 mm midcavity level (Figure 1). On magnetic resonance imaging, index 41 mL/m2 (normal range, 47-88 mL/m2) (Video invasive hemodynamic exercise testing able achieve workload 30 W. pulmonary capillary wedge pressure mildly elevated prominent V-wave, which increased 2). very low (1.5 L•min•m2) minimally (1.9 due blunted response (42 mL 43 peak exercise), suggesting an inability augment preload Frank-Starling reserve.Figure 2Pulmonary (blue) central venous (green) (left) during (right) measured testing.View Large Image Figure ViewerDownload Hi-res image Download (PPT) To chamber size her LV, incision. During normothermic cardiopulmonary bypass cardioplegic arrest, ventriculotomy made carefully expose anterolateral posteromedial were greatly enlarged excised along chordae tendineae residual posterior leaflet. We then from thickened septum LV. vein isolation appendage ligation. aorta cross-clamped minutes total. Postoperatively, pump both improved; 27% following 52 mL/m2, 25 ml/m2 32 26-60 ml/m2) evidence defect regional wall motion abnormality. is alive symptoms intolerance 7 after myectomy, most recent echocardiogram demonstrates 55%. Nonobstructive benign form general, 10% phenotype progress develop HF.4Hebl V.B. Miranda W.R. Ong K.C. al.The natural cardiomyopathy.Mayo Clin Proc. 2016; 91: 279-287Abstract (28) fact, nearly half progressive HF are identified having subtype, preserved fraction.5Melacini P. Basso C. Angelini al.Clinicopathological profiles cardiomyopathy.Eur J. 31: 2111-2123Crossref (151) For small alternative treatment, when other option transplantation.1Schaff Scholar,2Nguyen volume, compromises reserve leads activity. V waves observed absence significant regurgitation 2) indicate that atrium distended noncompliant portion its pressure-volume relationship, increasing hydrostatic pressures contribute congestion.6Reddy Y.N.V. Obokata M. Wiley B. haemodynamic basis lung congestion fraction.Eur 2019; 40: 3721-3730Crossref (88) Excision addition volume. previous study, Kochi colleagues7Kochi K. Okada Watari Orihashi Sueda T. Papillary treatment obstruction.Ann 2002; 8: 109-111PubMed reported relieve cardiomyopathy. However, intrinsic disease, concomitant intervention rarely necessary even obstruction. Similarly, should undergoing via myectomy. Indeed, early identification preservation important first step whose myocardial hypertrophy confined mid distal portions chamber. HCM, often displaced apically. case unique excision increase possible because concern removal chordal structures interruption leaflet-chordae-papillary complex. Previous studies suggest preserving continuity annulus associated higher better recovery regurgitation.8Komeda David T.E. Rao V. Sun Z. Weisel R.D. Burns R.J. Late effects replacement.Circulation. 1994; 90: II190-II194PubMed extensively patient, benefit outweighed potential disadvantage disrupting tethering effect attachment summary, use aid standard augmenting dimension maintains near-normal systolic surgery has enjoyed subjective improvement tolerance. it emphasized utilized selected subgroup patients. decision remove guided etiology HF, adequacy function, need intervention. work supported Paul Ruby Tsai Family. https://www.annalsthoracicsurgery.org/cms/asset/0364fe33-0889-4284-9a29-019766fe872f/mmc1.mp4Loading ... .mp4 (0.23 MB) Help files Supplemental Video 1https://www.annalsthoracicsurgery.org/cms/asset/12d90022-f408-4545-9902-32bff88e9a6e/mmc2.mp4Loading (0.25 2