Pectus excavatum causing dynamic right ventricular outflow tract obstruction: increased obstruction during expiration and decreased during inspiration

Pectus excavatum causing dynamic right ventricular outflow tract obstruction: increased... Right heart metrics and exercise performance in dilated cardiomyopathy 925 31. Myers J, Zaheer N, Quaglietti S, Madhavan R, Froelicher V, Heidenreich P. Model in a transplant referral population. J Heart Lung Transplant 2012;31: Association of functional and health status measures in heart failure. J Card Fail 817–24. 2006;12:439–45. 33. Kirklin JK, Naftel DC, Pagani FD, Kormos RL, Stevenson LW, Blume ED et al. 32. Levy WC, Aaronson KD, Dardas TF, Williams P, Haythe J, Mancini D. Prognostic Seventh INTERMACS annual report: 15,000 patients and counting. J Heart Lung impact of the addition of peak oxygen consumption to the Seattle Heart Failure Transplant 2015;34:1495–504. IMAGE FOCUS doi:10.1093/ehjci/jey053 Online publish-ahead-of-print 30 March 2018 .................................................................................................................................................... Pectus excavatum causing dynamic right ventricular outflow tract obstruction: increased obstruction during expiration and decreased during inspiration 1 1 1 2 1 Dhaval Desai , M. Fuad Jan , Renuka Jain , Farouk Mookadam , and A. Jamil Tajik * Aurora Cardiovascular Services, Aurora Sinai/Aurora St. Luke’s Medical Centers, University of Wisconsin School of Medicine and Public Health, 2801 W. Kinnickinnic River Parkway, Ste. 840, Milwaukee, WI 53215, USA; and Department of Cardiovascular Diseases, Mayo Clinic Arizona, Mayo Clinic, 13400 E. Shea Blvd., Scottsdale, AZ 85259, USA * Corresponding author. Tel: 11 (414) 649 3909; Fax: 11 (414) 649-3578. E-mail: publishing14@aurora.org Pectus excavatum can result in external compression of right-sided chambers. These findings might be exacerbated in the forward-leaning sitting position. A 27-year-old woman with pectus excavatum was evaluated. Chest computed tomography showed marked pectus excava- tum (Panel A), Haller index of 4.4 and Ghent score of 7. She was genotype-positive for a fibrillin gene variant of unknown significance. Electrocardiography showed sinus rhythm with a RSR’ pattern. In the supine position, a Grade 1–2/6 systolic ejection murmur was auscul- tated along the left upper sternal border, radiating towards the left infraclavicular fossa. Recumbent Doppler across the right ventricular outflow tract (RVOT) showed a minimally increased flow velocity of 1.2 m/s (Panels B–D). In the forward-leaning sitting position, murmur intensity increased during expiration to Grade 3–4/6 and diminished to Grade 1–2/6 with inspiration. Doppler echocardiography with res- pirometer showed a velocity of 2.5 m/s during expiration (peak gradient 25 mmHg) and 1.7 m/s (peak gradient 16 mmHg) during inspiration (Panels E and F, white arrow). This observation relates to the relationship between the sternum and RVOT in pectus excavatum and the changes in volumes with respiration. Compression of the RVOT by the sternum in pectus excavatum is exacerbated in the sitting, forward- leaning position, resulting in increased obstruction of the RVOT, and higher velocities. Inspiration leads to an increase in intrathoracic vol- ume resulting in a decrease of obstruction and decreased velocities across the RVOT. This mechanism accounts for the auscultatory para- dox of a right-sided murmur decreasing during inspiration and increasing during expiration, a novel observation. LA, left atrium; LV, left ventricle; RA, right atrium; RV, right ventricle. Published on behalf of the European Society of Cardiology. All rights reserved. V The Author(s) 2018. For permissions, please email: journals.permissions@oup.com. Downloaded from https://academic.oup.com/ehjcimaging/article-abstract/19/8/925/4956823 by Ed 'DeepDyve' Gillespie user on 24 July 2018 http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png European Heart Journal – Cardiovascular Imaging Oxford University Press

Pectus excavatum causing dynamic right ventricular outflow tract obstruction: increased obstruction during expiration and decreased during inspiration

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Oxford University Press
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Published on behalf of the European Society of Cardiology. All rights reserved. © The Author(s) 2018. For permissions, please email: journals.permissions@oup.com.
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2047-2404
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10.1093/ehjci/jey053
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Abstract

Right heart metrics and exercise performance in dilated cardiomyopathy 925 31. Myers J, Zaheer N, Quaglietti S, Madhavan R, Froelicher V, Heidenreich P. Model in a transplant referral population. J Heart Lung Transplant 2012;31: Association of functional and health status measures in heart failure. J Card Fail 817–24. 2006;12:439–45. 33. Kirklin JK, Naftel DC, Pagani FD, Kormos RL, Stevenson LW, Blume ED et al. 32. Levy WC, Aaronson KD, Dardas TF, Williams P, Haythe J, Mancini D. Prognostic Seventh INTERMACS annual report: 15,000 patients and counting. J Heart Lung impact of the addition of peak oxygen consumption to the Seattle Heart Failure Transplant 2015;34:1495–504. IMAGE FOCUS doi:10.1093/ehjci/jey053 Online publish-ahead-of-print 30 March 2018 .................................................................................................................................................... Pectus excavatum causing dynamic right ventricular outflow tract obstruction: increased obstruction during expiration and decreased during inspiration 1 1 1 2 1 Dhaval Desai , M. Fuad Jan , Renuka Jain , Farouk Mookadam , and A. Jamil Tajik * Aurora Cardiovascular Services, Aurora Sinai/Aurora St. Luke’s Medical Centers, University of Wisconsin School of Medicine and Public Health, 2801 W. Kinnickinnic River Parkway, Ste. 840, Milwaukee, WI 53215, USA; and Department of Cardiovascular Diseases, Mayo Clinic Arizona, Mayo Clinic, 13400 E. Shea Blvd., Scottsdale, AZ 85259, USA * Corresponding author. Tel: 11 (414) 649 3909; Fax: 11 (414) 649-3578. E-mail: publishing14@aurora.org Pectus excavatum can result in external compression of right-sided chambers. These findings might be exacerbated in the forward-leaning sitting position. A 27-year-old woman with pectus excavatum was evaluated. Chest computed tomography showed marked pectus excava- tum (Panel A), Haller index of 4.4 and Ghent score of 7. She was genotype-positive for a fibrillin gene variant of unknown significance. Electrocardiography showed sinus rhythm with a RSR’ pattern. In the supine position, a Grade 1–2/6 systolic ejection murmur was auscul- tated along the left upper sternal border, radiating towards the left infraclavicular fossa. Recumbent Doppler across the right ventricular outflow tract (RVOT) showed a minimally increased flow velocity of 1.2 m/s (Panels B–D). In the forward-leaning sitting position, murmur intensity increased during expiration to Grade 3–4/6 and diminished to Grade 1–2/6 with inspiration. Doppler echocardiography with res- pirometer showed a velocity of 2.5 m/s during expiration (peak gradient 25 mmHg) and 1.7 m/s (peak gradient 16 mmHg) during inspiration (Panels E and F, white arrow). This observation relates to the relationship between the sternum and RVOT in pectus excavatum and the changes in volumes with respiration. Compression of the RVOT by the sternum in pectus excavatum is exacerbated in the sitting, forward- leaning position, resulting in increased obstruction of the RVOT, and higher velocities. Inspiration leads to an increase in intrathoracic vol- ume resulting in a decrease of obstruction and decreased velocities across the RVOT. This mechanism accounts for the auscultatory para- dox of a right-sided murmur decreasing during inspiration and increasing during expiration, a novel observation. LA, left atrium; LV, left ventricle; RA, right atrium; RV, right ventricle. Published on behalf of the European Society of Cardiology. All rights reserved. V The Author(s) 2018. For permissions, please email: journals.permissions@oup.com. Downloaded from https://academic.oup.com/ehjcimaging/article-abstract/19/8/925/4956823 by Ed 'DeepDyve' Gillespie user on 24 July 2018

Journal

European Heart Journal – Cardiovascular ImagingOxford University Press

Published: Mar 30, 2018

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