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Treating a Structural Heart Disease Using a Non-structural Approach: Role of Cardiac Pacing in Hypertrophic Cardiomyopathy

Hypertrophic cardiomyopathy (HCM) is the most common genetic cardiovascular disease characterized by a thickened non-dilated ventricle in the absence of another cardiac or systemic condition. Its most important hemodynamic consequence is left ventricular outflow tract (LVOT) obstruction. The primary...

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Autores principales: Albano, Bernard Benjamin P., Fadreguilan, Erdie C., Chua, Jeffrey M., Ho, James, Medrano, Ana Beatriz
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Elmer Press 2017
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5340521/
https://www.ncbi.nlm.nih.gov/pubmed/28275421
http://dx.doi.org/10.14740/cr512w
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author Albano, Bernard Benjamin P.
Fadreguilan, Erdie C.
Chua, Jeffrey M.
Ho, James
Medrano, Ana Beatriz
author_facet Albano, Bernard Benjamin P.
Fadreguilan, Erdie C.
Chua, Jeffrey M.
Ho, James
Medrano, Ana Beatriz
author_sort Albano, Bernard Benjamin P.
collection PubMed
description Hypertrophic cardiomyopathy (HCM) is the most common genetic cardiovascular disease characterized by a thickened non-dilated ventricle in the absence of another cardiac or systemic condition. Its most important hemodynamic consequence is left ventricular outflow tract (LVOT) obstruction. The primary management strategy of this condition is surgical septal myectomy, but an acceptable alternative treatment in patients who are not suitable for (or who refuse) surgery is alcohol septal ablation (ASA). However, in patients with unfavorable coronary anatomy which precludes ASA (i.e. absence of major septal perforator branch of the left anterior descending (LAD) artery), another reasonable option is dual chamber pacemaker implantation to decrease LVOT outflow gradient. A 77-year-old female, known hypertensive, diabetic with a history of coronary artery disease, presented with 1-week history of worsening chest pain and shortness of breath. She was admitted as a case of acute coronary syndrome and pneumonia. On workup, 2DED revealed hypertrophic obstructive cardiomyopathy (HOCM) with a demonstrated systolic anterior motion (SAM) of the mitral valve with a peak instantaneous gradient of 194 mm Hg across the basal LV cavity. The patient refused surgical myectomy, and ASA was the preferred treatment option. On coronary angiography, there was an incidental finding of absent major septal perforator branch of the LAD coronary artery, rendering her unsuitable for septal ablation. She was referred to electrophysiology for evaluation. She underwent dual chamber pacemaker implantation and documented significant decrease in the peak instantaneous gradient from 194 to 37 mm Hg, with complete obliteration of SAM and improvement in overall wall motion. She remained stable and asymptomatic after pacemaker insertion until her recent outpatient follow-up (1 year after implantation). We present a case of HCM with congenitally absent major septal perforator branch coronary artery treated with dual chamber pacemaker implantation. To our knowledge, this is the first reported angiographically absent first (major) septal perforator coronary anatomy in the setting of HCM, and also the first description of dual chamber pacemaker implantation to relieve the LVOT obstruction. Although the role of dual chamber pacing has become limited in HCM because surgical myectomy and septal ablation have resulted in better decrease in LV outflow gradient and symptom improvement, this modality remains essential and may still be considered as the treatment strategy-of-choice in patients who are unsuitable for surgical myectomy and ASA.
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spelling pubmed-53405212017-03-08 Treating a Structural Heart Disease Using a Non-structural Approach: Role of Cardiac Pacing in Hypertrophic Cardiomyopathy Albano, Bernard Benjamin P. Fadreguilan, Erdie C. Chua, Jeffrey M. Ho, James Medrano, Ana Beatriz Cardiol Res Case Report Hypertrophic cardiomyopathy (HCM) is the most common genetic cardiovascular disease characterized by a thickened non-dilated ventricle in the absence of another cardiac or systemic condition. Its most important hemodynamic consequence is left ventricular outflow tract (LVOT) obstruction. The primary management strategy of this condition is surgical septal myectomy, but an acceptable alternative treatment in patients who are not suitable for (or who refuse) surgery is alcohol septal ablation (ASA). However, in patients with unfavorable coronary anatomy which precludes ASA (i.e. absence of major septal perforator branch of the left anterior descending (LAD) artery), another reasonable option is dual chamber pacemaker implantation to decrease LVOT outflow gradient. A 77-year-old female, known hypertensive, diabetic with a history of coronary artery disease, presented with 1-week history of worsening chest pain and shortness of breath. She was admitted as a case of acute coronary syndrome and pneumonia. On workup, 2DED revealed hypertrophic obstructive cardiomyopathy (HOCM) with a demonstrated systolic anterior motion (SAM) of the mitral valve with a peak instantaneous gradient of 194 mm Hg across the basal LV cavity. The patient refused surgical myectomy, and ASA was the preferred treatment option. On coronary angiography, there was an incidental finding of absent major septal perforator branch of the LAD coronary artery, rendering her unsuitable for septal ablation. She was referred to electrophysiology for evaluation. She underwent dual chamber pacemaker implantation and documented significant decrease in the peak instantaneous gradient from 194 to 37 mm Hg, with complete obliteration of SAM and improvement in overall wall motion. She remained stable and asymptomatic after pacemaker insertion until her recent outpatient follow-up (1 year after implantation). We present a case of HCM with congenitally absent major septal perforator branch coronary artery treated with dual chamber pacemaker implantation. To our knowledge, this is the first reported angiographically absent first (major) septal perforator coronary anatomy in the setting of HCM, and also the first description of dual chamber pacemaker implantation to relieve the LVOT obstruction. Although the role of dual chamber pacing has become limited in HCM because surgical myectomy and septal ablation have resulted in better decrease in LV outflow gradient and symptom improvement, this modality remains essential and may still be considered as the treatment strategy-of-choice in patients who are unsuitable for surgical myectomy and ASA. Elmer Press 2017-02 2017-03-03 /pmc/articles/PMC5340521/ /pubmed/28275421 http://dx.doi.org/10.14740/cr512w Text en Copyright 2017, Albano et al. http://creativecommons.org/licenses/by-nc/4.0/ TThis article is distributed under the terms of the Creative Commons Attribution Non-Commercial 4.0 International License, which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Case Report
Albano, Bernard Benjamin P.
Fadreguilan, Erdie C.
Chua, Jeffrey M.
Ho, James
Medrano, Ana Beatriz
Treating a Structural Heart Disease Using a Non-structural Approach: Role of Cardiac Pacing in Hypertrophic Cardiomyopathy
title Treating a Structural Heart Disease Using a Non-structural Approach: Role of Cardiac Pacing in Hypertrophic Cardiomyopathy
title_full Treating a Structural Heart Disease Using a Non-structural Approach: Role of Cardiac Pacing in Hypertrophic Cardiomyopathy
title_fullStr Treating a Structural Heart Disease Using a Non-structural Approach: Role of Cardiac Pacing in Hypertrophic Cardiomyopathy
title_full_unstemmed Treating a Structural Heart Disease Using a Non-structural Approach: Role of Cardiac Pacing in Hypertrophic Cardiomyopathy
title_short Treating a Structural Heart Disease Using a Non-structural Approach: Role of Cardiac Pacing in Hypertrophic Cardiomyopathy
title_sort treating a structural heart disease using a non-structural approach: role of cardiac pacing in hypertrophic cardiomyopathy
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5340521/
https://www.ncbi.nlm.nih.gov/pubmed/28275421
http://dx.doi.org/10.14740/cr512w
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