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Tunneled left anterior descending artery in a child with hypertrophic cardiomyopathy

Nature Clinical Practice Cardiovascular Medicine volume 6, pages 134–139 (2009)Cite this article

Abstract

Background A 10-year-old boy presented with a history of severe angina on exertion. A two-dimensional echocardiogram showed mild asymmetric left ventricular (LV) hypertrophy localized to the interventricular septum, consistent with nonobstructive hypertrophic cardiomyopathy. A maximal treadmill exercise test was terminated early owing to marked downsloping of the ST–T segment on all precordial leads, associated with mild chest discomfort. Cardiac MRI and coronary angiography showed that the left anterior descending (LAD) artery was 'tunneled' from its origin to the junction of the middle and lower segments, causing systolic obliteration. PET showed diffusely blunted myocardial blood flow after dipyridamole infusion. A beating-heart technique was used to perform surgical mobilization of the superficial and lateral surfaces of the LAD artery. The patient was free from angina at 6 months after surgery. A repeat exercise test showed considerable improvement in exercise tolerance, which was associated with a marked decrease in ST–T changes on exertion.

Investigations Physical examination, laboratory tests, 12-lead electrocardiography, two-dimensional echocardiography, exercise testing, cardiac MRI, coronary angiography, PET, Holter electrocardiographic monitoring.

Diagnosis Angina caused by extensive myocardial tunneling of the LAD artery in nonobstructive hypertrophic cardiomyopathy.

Management Bisoprolol therapy and surgical mobilization of the tunneled LAD artery.

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Figure 1: Results of 12-lead electrocardiography.
Figure 2: Preoperative two-dimensional echocardiography (parasternal short-axis view).
Figure 3: Cardiac MRI of the left anterior descending artery at end-diastole showing the extended intramural course of the proximal and middle segments of the artery.
Figure 4: Coronary angiography.
Figure 5: Nitrogen-13 ammonia PET polar map that illustrates the regional values of myocardial blood flow after dipyridamole injection.
Figure 6: Surgical correction of myocardial tunneling.

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Acknowledgements

Written consent for publication was obtained from the patient's mother. The authors are grateful to Professor Roberto Sciagrá, who performed the PET scan and to Dr Luigi Festa, who performed the cardiac MRI.

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Authors and Affiliations

  1. I Olivotto is a Staff Physician and F Cecchi is Head of the Referral Center for Cardiomyopathies in the Cardiology Department of Careggi University Hospital, Florence, Italy.,

    Iacopo Olivotto & Franco Cecchi

  2. R Bini is Chief and Silvia Favilli is a Staff Physician at the Pediatric Cardiology unit, Meyer Pediatric Hospital, Florence, Italy.,

    Roberta Bini & Silvia Favilli

  3. B Murzi is Chief of the Pediatric Cardiac Surgery unit at the Ospedale Pediatrico Apuano G. Pasquinucci in Massa, Italy.,

    Bruno Murzi

  4. I El-Hamamsy is a Research Fellow in Cardiac Surgery at the Harefield Heart Science Center, London, UK,

    Ismail El-Hamamsy

  5. MH Yacoub is Professor of Cardiac Surgery at Imperial College London, Director of Research at the Harefield Heart Science Centre, Harefield, UK, and Professor of Surgery at the University of Florence, Italy.,

    Magdi H Yacoub

Authors
  1. Iacopo Olivotto
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  2. Franco Cecchi
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  3. Roberta Bini
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  4. Silvia Favilli
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  5. Bruno Murzi
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  6. Ismail El-Hamamsy
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  7. Magdi H Yacoub
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Corresponding author

Correspondence to Iacopo Olivotto.

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The authors declare no competing financial interests.

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Olivotto, I., Cecchi, F., Bini, R. et al. Tunneled left anterior descending artery in a child with hypertrophic cardiomyopathy. Nat Rev Cardiol 6, 134–139 (2009). https://doi.org/10.1038/ncpcardio1420

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