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Provokable left ventricular outflow tract obstruction in a patient without hypertrophy

Nature Reviews Cardiology volume 6, pages 313–316 (2009)Cite this article

Abstract

Background. A 61-year-old man presented with shortness of breath and chest pain on exertion. He had been diagnosed as having hiatus hernia 2 years previously and was taking proton-pump inhibitors as necessary. He had a family history of ischemic heart disease and subarachnoid hemorrhage.

Investigations. Physical examination, electrocardiography, echocardiography, cardiopulmonary exercise testing, coronary angiography, transoesophageal echocardiography, stress echocardiography.

Diagnosis. Provokable left ventricular outflow tract obstruction without electrocardiographic abnormalities or left ventricular hypertrophy on echocardiography.

Management. Pharmacological therapy (atenolol 50 mg daily, disopyramide 250 mg twice daily), dual-chamber pacemaker implantation.

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Figure 1: Resting electrocardiography on presentation.
Figure 2: Echocardiography performed during an upright bicycle stress test with patient off medication showing flow acceleration in the left ventricular outflow tract and the gradient on continuous wave Doppler monitoring.

References

  1. Maron, B. J. et al. American College of Cardiology/European Society of Cardiology clinical expert consensus document on hypertrophic cardiomyopathy. A report of the American College of Cardiology Foundation Task Force on Clinical Expert Consensus Documents and the European Society of Cardiology Committee for Practice Guidelines. J. Am. Coll. Cardiol. 42, 1687–1713 (2003).

    Article  Google Scholar 

  2. Maron, M. S. et al. Effect of left ventricular outflow tract obstruction on clinical outcome in hypertrophic cardiomyopathy. N. Engl. J. Med. 348, 295–303 (2003).

    Article  Google Scholar 

  3. Shah, J. S. et al. Prevalence of exercise-induced left ventricular outflow tract obstruction in symptomatic patients with non-obstructive hypertrophic cardiomyopathy. Heart 94, 1288–1294 (2008).

    Article  CAS  Google Scholar 

  4. Maron, M. S. et al. Hypertrophic cardiomyopathy is predominantly a disease of left ventricular outflow tract obstruction. Circulation 114, 2232–2239 (2006).

    Article  Google Scholar 

  5. Page, S. P., Pnatazis, A. & Elliott, P. M. Acute myocardial ischemia associated with latent left ventricular outflow tract obstruction in the absence of left ventricular hypertrophy. J. Am. Soc. Echocardiogr. 20, 772.e1–772.e4 (2007).

    Article  Google Scholar 

  6. Bos, J. M. et al. Relationship between sex, shape, and substrate in hypertrophic cardiomyopathy. Am. Heart J. 155, 1128–1134 (2008).

    Article  Google Scholar 

  7. Fananapazir, L. et al. Long-term results of dual-chamber (DDD) pacing in obstructive hypertrophic cardiomyopathy. Evidence for progressive symptomatic and hemodynamic improvement and reduction of left ventricular hypertrophy. Circulation 90, 2731–2742 (1994).

    Article  CAS  Google Scholar 

  8. Nishimura, R. A. et al. Dual-chamber pacing in hypertrophic cardiomyopathy: a randomized, double-blind, crossover trial. J. Am. Coll. Cardiol. 29, 435–441 (1997).

    Article  CAS  Google Scholar 

  9. Maron, B. J. et al. Assessment of permanent dual-chamber pacing as a treatment for drug-refractory symptomatic patients with obstructive hypertrophic cardiomyopathy. A randomized, double-blind, crossover study (M-PATHY). Circulation 99, 2927–2933 (1999).

    Article  CAS  Google Scholar 

  10. Linde, C., Gadler, F., Kappenberger, L. & Rydén L. Placebo effect of pacemaker implantation in obstructive hypertrophic cardiomyopathy. PIC Study Group. Pacing In Cardiomyopathy. Am. J. Cardiol. 83, 903–907 (1999).

    Article  CAS  Google Scholar 

  11. Binder, J., Ommen, S. R., Sorajja, P., Nishimura, R. A. & Tajik, A. J. Clinical and echocardiographic variables fail to predict response to dual-chamber pacing for hypertrophic cardiomyopathy. J. Am. Soc. Echocardiogr. 21, 796–800 (2008).

    Article  Google Scholar 

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Acknowledgements

Written consent for publication was obtained from the patient.

Author information

Authors and Affiliations

  1. The Heart Hospital, London, UK

    Ferdinando Pasquale

  2. The Heart Hospital and Great Ormond Street Hospital, London, UK

    Maria Teresa Tomé-Esteban

  3. Tor Vergata Hospital, Rome, Italy

    Riccardo Morgagni

  4. The Heart Hospital and The Institute of Child Health, Great Ormond Street Hospital, London, UK

    Perry Elliott

Authors
  1. Ferdinando Pasquale
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  2. Maria Teresa Tomé-Esteban
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  3. Riccardo Morgagni
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  4. Perry Elliott
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Corresponding author

Correspondence to Perry Elliott.

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The authors declare no competing financial interests.

Supplementary information

Supplementary movie

Resting echocardiogram on presentation. This loop shows the angulated shape of the septum at parasternal long-axis view. In spite of the normal thickness, the septum protrudes in the left ventricular outflow tract causing a mechanical narrowing. Furthermore it is possible to appreciate the elongated anterior mitral leaflet and the absence of systolic anterior motion at rest. (AVI 8896 kb)

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Pasquale, F., Tomé-Esteban, M., Morgagni, R. et al. Provokable left ventricular outflow tract obstruction in a patient without hypertrophy. Nat Rev Cardiol 6, 313–316 (2009). https://doi.org/10.1038/nrcardio.2009.7

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  • DOI: https://doi.org/10.1038/nrcardio.2009.7

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