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Commentary: Every picture tells a story

  • Lawrence M. Wei
    Correspondence
    Address for reprints: Lawrence M. Wei, MD, Department of Cardiovascular and Thoracic Surgery, West Virginia University Heart and Vascular Institute, One Medical Center Dr, PO Box 8003, Morgantown, WV 26506.
    Affiliations
    Department of Cardiovascular and Thoracic Surgery, West Virginia University, Morgantown, WVa
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Open AccessPublished:June 11, 2021DOI:https://doi.org/10.1016/j.xjtc.2021.06.016
      Images illustrate the efficacy of transapical myectomy in management of apical hypertrophic cardiomyopathy.
      See Article page 71.
      Sun and colleagues
      • Sun D.
      • Schaff H.V.
      • Nishimura R.A.
      Reoperation for nonobstructive hypertrophic cardiomyopathy in a patient with extreme septal thickness.
      present a case of hypertrophic cardiomyopathy (HCM) managed by transapical myectomy, a technique their group has described to treat midventricular obstruction in the apical variant of HCM.
      • Schaff H.V.
      • Brown M.L.
      • Dearani J.A.
      • Abel M.D.
      • Ommen S.R.
      • Sorajja P.
      • et al.
      Apical myectomy: a new surgical technique for management of severely symptomatic patients with apical hypertrophic cardiomyopathy.
      This patient had undergone transaortic septal myectomy (SM) at another institution without relief of heart failure symptoms. He had residual diffuse hypertrophy with a septal thickness of 4.8 cm and a small left ventricular cavity. Pictures and a video clip of the transthoracic echocardiogram illustrate the patient's heart before and after apical myectomy.
      HCM is a genetic disease of the myocardium characterized by hypertrophic ventricular muscle and heart failure symptoms caused by left ventricular outflow tract (LVOT) obstruction and/or diastolic dysfunction. Multiple phenotypic patterns of hypertrophy can affect the basal, midventricular, and apical septum causing LVOT obstruction. In some cases, the free wall is involved, resulting in concentric hypertrophy. Extended septal myectomy, commonly performed via a transaortic approach, is the surgical treatment of choice for HCM patients with LVOT obstruction and symptoms refractory to medical therapy. This technique effectively relieves heart failure symptoms and restores life expectancy to that of the general population.
      • Ommen S.R.
      • Maron B.J.
      • Olivotto I.
      • Maron M.S.
      • Cecchi F.
      • Betocchi S.
      • et al.
      Long-term effects of surgical septal myectomy on survival in patients with obstructive hypertrophic cardiomyopathy.
      Myectomy must be tailored to each patient's anatomy. This case shows that transaortic SM alone may be insufficient and ineffective in a patient with diffuse septal hypertrophy and midventricular obstruction. Additionally, some patients have papillary muscle anomalies or intrinsic pathology of the mitral valve that require intervention. Excellent preoperative imaging is essential to identify such anatomical features and to develop a surgical plan. Transthoracic and transesophageal echocardiography and cardiac magnetic resonance imaging provide detailed pathoanatomic data and are the most useful modalities for planning septal resection. These studies, if completed, may have provided this patient's original surgeon with valuable assistance to perform a more effective operation or to determine that referral to a more experienced HCM center was advisable.
      The images display the extent of myectomy achievable via a transapical approach. This case shows that symptoms of heart failure may be caused not only by LVOT obstruction but also by diastolic dysfunction and a small LV cavity. The pre- and post-resection echocardiographic images provide striking visual confirmation of the degree of resection and improvement in LV cavity dimensions.
      Providing optimal care to patients with HCM requires a multidisciplinary team, including cardiologists and cardiac surgeons with experience in contemporary management of HCM. An HCM center offers comprehensive diagnostic and treatment options, including genetic testing, echocardiography and cardiac magnetic resonance imaging, surgical SM, and alcohol septal ablation.
      • Gersh B.J.
      • Maron B.J.
      • Bonow R.O.
      • Dearani J.A.
      • Fifer M.A.
      • Link M.S.
      • et al.
      2011 ACCF/AHA guideline for the diagnosis and treatment of hypertrophic cardiomyopathy: a report of the American College of Cardiology Foundation/American Heart Association task force on practice guidelines.
      Proficiency in management of atrial fibrillation and implantation of cardioverter/defibrillators is mandatory, along with staff to counsel and support HCM patients. Centers performing higher volumes of SM achieve superior outcomes compared with lower volume centers.
      • Wei L.M.
      • Thibault D.P.
      • Rankin J.S.
      • Alkhouli M.
      • Roberts H.G.
      • Vemulapalli S.
      • et al.
      Contemporary surgical management of hypertrophic cardiomyopathy in the United States.
      This case is a beautiful example of the efficacy of transapical myectomy in treating apical HCM. More broadly, it exemplifies the importance of imaging in the management of structural heart disease: from diagnosis, to treatment planning, to evaluation of therapeutic results.

      References

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        • Schaff H.V.
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        Reoperation for nonobstructive hypertrophic cardiomyopathy in a patient with extreme septal thickness.
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        • Brown M.L.
        • Dearani J.A.
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        • Sorajja P.
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        Apical myectomy: a new surgical technique for management of severely symptomatic patients with apical hypertrophic cardiomyopathy.
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        Long-term effects of surgical septal myectomy on survival in patients with obstructive hypertrophic cardiomyopathy.
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        • Fifer M.A.
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        2011 ACCF/AHA guideline for the diagnosis and treatment of hypertrophic cardiomyopathy: a report of the American College of Cardiology Foundation/American Heart Association task force on practice guidelines.
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        • Wei L.M.
        • Thibault D.P.
        • Rankin J.S.
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        • et al.
        Contemporary surgical management of hypertrophic cardiomyopathy in the United States.
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      Linked Article

      • Reoperation for nonobstructive hypertrophic cardiomyopathy in a patient with extreme septal thickness
        JTCVS TechniquesVol. 8
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          A 24-year-old male patient with familial hypertrophic cardiomyopathy presented with New York Heart Association class III heart failure. Two years earlier, he had undergone transaortic septal myectomy at another institution to relieve left ventricular outflow tract obstruction. However, his heart failure symptoms did not improve. On evaluation at our Clinic, transthoracic echocardiography confirmed nonobstructive hypertrophic cardiomyopathy. There was no left ventricular outflow tract gradient, but the left ventricular cavity size was small and the interventricular septum measured 48 mm (Figure 1, A).
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