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.
This is a preview of subscription content, access via your institution
Access options
Subscribe to this journal
Receive 12 print issues and online access
$209.00 per year
only $17.42 per issue
Buy this article
- Purchase on Springer Link
- Instant access to full article PDF
Prices may be subject to local taxes which are calculated during checkout
References
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).
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).
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).
Maron, M. S. et al. Hypertrophic cardiomyopathy is predominantly a disease of left ventricular outflow tract obstruction. Circulation 114, 2232–2239 (2006).
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).
Bos, J. M. et al. Relationship between sex, shape, and substrate in hypertrophic cardiomyopathy. Am. Heart J. 155, 1128–1134 (2008).
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).
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).
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).
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).
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).
Acknowledgements
Written consent for publication was obtained from the patient.
Author information
Authors and Affiliations
Corresponding author
Ethics declarations
Competing interests
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)
Rights and permissions
About this article
Cite this article
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
Issue Date:
DOI: https://doi.org/10.1038/nrcardio.2009.7