Miocardiopatía Hipertrófica

Instituto Mexicano del Seguro Social

Centro Médico Nacional del BajíoUnidad Médica de Alta Especialidad

>> Departamento de Cardiología <<

Tema:“Miocardiopatía Hipertrófica (MCH)”

Dr. Ricardo Mora MorenoMédico Residente 1er Año de Cardiología (R1C)

León, Guanajuato

17 / Mayo / 2016

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Objetivos:

• Definición• Epidemiología• Etiología• Cuadro clínico• Diagnostico• Tratamiento• Muerte súbita

Miocardiopatía Hipertrófica


Definición:

• La miocardiopatía hipertrófica (MHC) se define por la presencia de aumento del grosor de la pared del ventrículo izquierdo (VI) que no se puede explicar únicamente por condiciones de carga anómalas.


Guía de práctica clínica de la ESC 2014 sobre el diagnóstico y manejo de la miocardiopatía hipertrófica; Rev Esp Cardiol, 2015; 68 (1): 63. e1-e52


Definición:


The 50-year History, controversy, and clinical implications of left ventricular outflow tract obstruction in hypertrophic cardiomyopathy: from idiopathic hypertrophic subaortic stenosis to hypertrophic cardiomyopathy.

Journal of the American College of Cardiology, Volume 54, Issue 3, 2009, e 191- 200

Historia:


Epidemiología


Epidemiología:

• Prevalencia a nivel mundial en adultos del 0.02-0.23% (1:500)

• Incidencia anual en niños 0.3-0.5/100,000

• MCH se transmite como un rasgo autosómico dominante

• Genero: Masculino>Femenino




Epidemiología:




Epidemiología:




Etiología


Etiología:Miocardiopatía Hipertrófica



Etiología:Miocardiopatía Hipertrófica



Cuadro clínico


Cuadro clínico:Miocardiopatía Hipertrófica




Moya A, Sutton R, Ammirati F, Blanc JJ, Brignole M, Dahm JB, et al. Guidelines forthe diagnosis and management of syncope (version 2009): the Task Force for the

Diagnosis and Management of Syncope of the European Society of Cardiology(ESC). Eur Heart J. 2009;30:2631-71.



Brignole M, Vardas P, Hoffman E, Huikuri H, Moya A, Ricci R, et al. Indications forthe use of diagnostic implantable and external ECG loop recorders. Europace.

2009;11:671-87.


Diagnostico


Diagnostico:Miocardiopatía Hipertrófica



Diagnostico:Miocardiopatía Hipertrófica

Rapezzi C, Arbustini E, Caforio AL, Charron P, Gimeno-Blanes J, Helio T, et al.Diagnostic work-up in cardiomyopathies: bridging the gap between clinicalphenotypes and final diagnosis. A position statement from the ESCWorking

Group on Myocardial and Pericardial Diseases. Eur Heart J. 2013;34:1448-58.


Diagnostico EKG:





Diagnostico EKG:


Consenso de Miocardiopatía hipertrófica; Consenso Argentino SAC; Revista Argentina de Cardiología; Vol 77 No 2, Marzo-Abril, 2009


Las anomalías mas frecuentes incluyen:*Aumento del voltaje por hipertrofia del VI.*Cambios en ST con inversión pronunciada de la onda T en derivaciones precordiales laterales.*Ondas Q profundas y estrechas, con más frecuencia en las derivaciones de cara inferior y lateral del VI.*Ondas R pequeñas en derivaciones precordiales laterales.




Diagnostico EKG:



1. Muestra la ausencia de la onda Q en DI, aVL , en V5 y V62. La presencia de ondas R altas desde la derivación V23. Un segmento ST rectificado y ascendente, fundamentalmente en la

derivación V2.4. Ondas T negativas gigantes seudoisquémicas en la cara anterolateral.� � � �

MCH APICAL


Diagnostico Ecocardiograma:








Diagnostico Ecocardiograma:Miocardiopatía Hipertrófica

Maron BJ, Gottdiener J, Epstein S: Patterns and significance of distribution of left ventricular hypertrophy in hypertrophic cardiomyopathy. Am J Cardiol 1981; 48: 418-428.


Diagnostico Ecocardiograma:Miocardiopatía Hipertrófica









Sasson Z, Henderson M, Wilansky S y col: Casual relation between the pressure gradient and left ventricular ejection time in hypertrophic cardiomyopathy. J Am Coll Cardiol 1989; 13: 1275-1279.

















Klues HG, Schiffers A, Maron BJ. Phenotypic spectrum and patterns of leftventricular hypertrophy in hypertrophic cardiomyopathy: morphologic

observations and significance as assessed by two-dimensional echocardiographyin 600 patients. J Am Coll Cardiol. 1995;26:1699-708.




Flachskampf FA, Badano L, Daniel WG, Feneck RO, Fox KF, Fraser AG, et al.Recommendations for transoesophageal echocardiography: update 2010. Eur J

Echocardiogr. 2010;11:557-76.


Diagnostico RMN:


Burton H, Alberg C, Stewart A. Heart to heart: inherited cardiovascular conditionsservices — a needs assessment and service review. London: PHG Fundation; 2009


Diagnostico RMN:


Burton H, Alberg C, Stewart A. Heart to heart: inherited cardiovascular conditionsservices — a needs assessment and service review. London: PHG Fundation; 2009


Diagnostico Gammagrafía Nuclear:


Rapezzi C, Quarta CC, Guidalotti PL, Pettinato C, Fanti S, Leone O, et al. Role of(99m)Tc-DPD scintigraphy in diagnosis and prognosis of hereditary transthyretin

related cardiac amyloidosis. JACC Cardiovasc Imaging. 2011;4:659-70.


Diagnostico Biopsia endomiocárdica:


Leone O, Veinot JP, Angelini A, Baandrup UT, Basso C, Berry G, et al. 2011consensus statement on endomyocardial biopsy from the Association for

European Cardiovascular Pathology and the Society for Cardiovascular Pathology.Cardiovasc Pathol. 2012;21:245-74.


Diagnostico Laboratorio:




Diagnostico Genético:


Godard B, ten Kate L, Evers-Kiebooms G, Ayme S. Population genetic screeningprogrammes: principles, techniques, practices, and policies. Eur J Hum Genet.

2003;11 Suppl 2:S49-87


Diagnostico Genético:




Diagnostico Cateterismo cardiaco:


Zipes DP, Camm AJ, Borggrefe M, Buxton AE, Chaitman B, Fromer M, et al. ACC/AHA/ESC 2006 guidelines for management of patients with ventricular arrhythmias and the prevention of sudden cardiac death: a report of the American College of Cardiology/American Heart Association Task Force and the European Society of

Cardiology Committee for Practice Guidelines (Writing Committee to Develop guidelines for management of patients with ventricular arrhythmias and the prevention of sudden cardiac death) developed in collaboration with the European Heart Rhythm Association and the Heart Rhythm Society. Europace. 2006;8:746-837.


Diagnostico Electrofisiología:


Brignole M, Auricchio A, Baron-Esquivias G, Bordachar P, Boriani G, Breithardt OA, et al. 2013 ESC Guidelines on cardiac pacing and cardiac resynchronization therapy: the Task Force on cardiac pacing and resynchronization therapy of the European Society of

Cardiology (ESC). Developed in collaboration with the European Heart Rhythm Association (EHRA). Eur Heart J. 2013;34:2281-329.


Tratamiento


Tratamiento:




Tratamiento:


Stauffer JC, RuizV, Morard JD. Subaortic obstruction after sildenafil in a patientwith hypertrophic cardiomyopathy. N Engl J Med. 1999;341:700-1


Tratamiento:


Sherrid MV, Shetty A, Winson G, Kim B, Musat D, Alviar CL, et al. Treatment of obstructive hypertrophic cardiomyopathy symptoms and gradient resistant to first-line therapy with beta-blockade or verapamil. Circ Heart Fail. 2013;6: 694-702.


Tratamiento:


Agarwal S, Tuzcu EM, Desai MY, Smedira N, Lever HM, Lytle BW, et al. Updated meta-analysis of septal alcohol ablation versus myectomy for hypertrophic cardiomyopathy. J Am Coll Cardiol. 2010;55:823-34


Tratamiento:


Agarwal S, Tuzcu EM, Desai MY, Smedira N, Lever HM, Lytle BW, et al. Updated meta-analysis of septal alcohol ablation versus myectomy for hypertrophic cardiomyopathy. J Am Coll Cardiol. 2010;55:823-34


Tratamiento:




Tratamiento:


Kappenberger L, Linde C, Daubert C, McKenna W, Meisel E, Sadoul N, et al. Pacing in hypertrophic obstructive cardiomyopathy. A randomized crossover study. PIC Study Group. Eur Heart J. 1997;18:1249-56.


Tratamiento:


Rosing DR, Condit JR, Maron BJ, Kent KM, Leon MB, Bonow RO, et al. Verapamil therapy: a new approach to the pharmacologic treatment of hypertrophic cardiomyopathy: III. Effects of long-term administration. Am J Cardiol.

1981;48:545-53.


Tratamiento:


McMurray JJ, Adamopoulos S, Anker SD, Auricchio A, Bohm M, Dickstein K, et al. ESC Guidelines for the diagnosis and treatment of acute and chronic heart failure 2012: The Task Force for the Diagnosis and Treatment of Acute and Chronic Heart Failure 2012 of the European Society of Cardiology. Developed in collaboration with the Heart Failure Association (HFA) of the

ESC. Eur Heart J. 2012;33:1787-847


Tratamiento:




Tratamiento:


Rogers DP, Marazia S, Chow AW, Lambiase PD, Lowe MD, Frenneaux M, et al. Effect of biventricular pacing on symptoms and cardiac remodelling in patients with end-stage hypertrophic cardiomyopathy. Eur J Heart Fail.

2008;10:507-13.


Tratamiento:


Kato TS, Takayama H, Yoshizawa S, Marboe C, Schulze PC, Farr M, et al. Cardiac transplantation in patients with hypertrophic cardiomyopathy. Am J Cardiol. 2012;110:568-74.





Muerte Súbita



Barry J. Maron, Contemprary Insights and Strategies for risk stratification and prevention of sudden death in hypertrophic cardiomyopathy; Circulation, 2010: 121: 445-456



















GRACIAS!!!



Fuentes de Información:

• Guía de práctica clínica de la ESC 2014 sobre el diagnóstico y manejo de la miocardiopatía hipertrófica; Rev Esp Cardiol, 2015; 68 (1): 63. e1-e52• Consenso de Miocardiopatía hipertrófica; Consenso Argentino SAC; Revista Argentina de Cardiología; Vol 77 No 2, Marzo-Abril, 2009• Rapezzi C, Arbustini E, Caforio AL, Charron P, Gimeno-Blanes J, Helio T, et al. Diagnostic work-up in cardiomyopathies: bridging the gap between clinical phenotypes and

final diagnosis. A position statement from the ESCWorking Group on Myocardial and Pericardial Diseases. Eur Heart J. 2013;34:1448-58.• Moya A, Sutton R, Ammirati F, Blanc JJ, Brignole M, Dahm JB, et al. Guidelines for the diagnosis and management of syncope (version 2009): the Task Force for the

Diagnosis and Management of Syncope of the European Society of Cardiology (ESC). Eur Heart J. 2009;30:2631-71.• Brignole M, Vardas P, Hoffman E, Huikuri H, Moya A, Ricci R, et al. Indications for the use of diagnostic implantable and external ECG loop recorders. Europace.

2009;11:671-87.• Klues HG, Schiffers A, Maron BJ. Phenotypic spectrum and patterns of left ventricular hypertrophy in hypertrophic cardiomyopathy: morphologic observations and significance

as assessed by two-dimensional echocardiography in 600 patients. J Am Coll Cardiol. 1995;26:1699-708.• Flachskampf FA, Badano L, Daniel WG, Feneck RO, Fox KF, Fraser AG, et al. Recommendations for transoesophageal echocardiography: update 2010. Eur J Echocardiogr.

2010;11:557-76.• Burton H, Alberg C, Stewart A. Heart to heart: inherited cardiovascular conditions services — a needs assessment and service review. London: PHG Fundation; 2009• Rapezzi C, Quarta CC, Guidalotti PL, Pettinato C, Fanti S, Leone O, et al. Role of (99m)Tc-DPD scintigraphy in diagnosis and prognosis of hereditary transthyretin related

cardiac amyloidosis. JACC Cardiovasc Imaging. 2011;4:659-70.• Leone O, Veinot JP, Angelini A, Baandrup UT, Basso C, Berry G, et al. 2011 consensus statement on endomyocardial biopsy from the Association for European

Cardiovascular Pathology and the Society for Cardiovascular Pathology. Cardiovasc Pathol. 2012;21:245-74.• Godard B, ten Kate L, Evers-Kiebooms G, Ayme S. Population genetic screening programmes: principles, techniques, practices, and policies. Eur J Hum Genet. 2003;11

Suppl 2:S49-87• Zipes DP, Camm AJ, Borggrefe M, Buxton AE, Chaitman B, Fromer M, et al. ACC/AHA/ESC 2006 guidelines for management of patients with ventricular arrhythmias and the

prevention of sudden cardiac death: a report of the American College of Cardiology/American Heart Association Task Force and the European Society of Cardiology Committee for Practice Guidelines (Writing Committee to Develop guidelines for management of patients with ventricular arrhythmias and the prevention of sudden cardiac death) developed in collaboration with the European Heart Rhythm Association and the Heart Rhythm Society. Europace. 2006;8:746-837.

• Brignole M, Auricchio A, Baron-Esquivias G, Bordachar P, Boriani G, Breithardt OA, et al. 2013 ESC Guidelines on cardiac pacing and cardiac resynchronization therapy: the Task Force on cardiac pacing and resynchronization therapy of the European Society of Cardiology (ESC). Developed in collaboration with the European Heart Rhythm Association (EHRA). Eur Heart J. 2013;34:2281-329.

• Stauffer JC, RuizV, Morard JD. Subaortic obstruction after sildenafil in a patient with hypertrophic cardiomyopathy. N Engl J Med. 1999;341:700-1• Sherrid MV, Shetty A, Winson G, Kim B, Musat D, Alviar CL, et al. Treatment of obstructive hypertrophic cardiomyopathy symptoms and gradient resistant to first-line therapy

with beta-blockade or verapamil. Circ Heart Fail. 2013;6: 694-702.• Agarwal S, Tuzcu EM, Desai MY, Smedira N, Lever HM, Lytle BW, et al. Updated meta-analysis of septal alcohol ablation versus myectomy for hypertrophic cardiomyopathy.

J Am Coll Cardiol. 2010;55:823-34• Kappenberger L, Linde C, Daubert C, McKenna W, Meisel E, Sadoul N, et al. Pacing in hypertrophic obstructive cardiomyopathy. A randomized crossover study. PIC Study

Group. Eur Heart J. 1997;18:1249-56.• Rosing DR, Condit JR, Maron BJ, Kent KM, Leon MB, Bonow RO, et al. Verapamil therapy: a new approach to the pharmacologic treatment of hypertrophic cardiomyopathy:

III. Effects of long-term administration. Am J Cardiol. 1981;48:545-53.• McMurray JJ, Adamopoulos S, Anker SD, Auricchio A, Bohm M, Dickstein K, et al. ESC Guidelines for the diagnosis and treatment of acute and chronic heart failure 2012:

The Task Force for the Diagnosis and Treatment of Acute and Chronic Heart Failure 2012 of the European Society of Cardiology. Developed in collaboration with the Heart Failure Association (HFA) of the ESC. Eur Heart J. 2012;33:1787-847

• Rogers DP, Marazia S, Chow AW, Lambiase PD, Lowe MD, Frenneaux M, et al. Effect of biventricular pacing on symptoms and cardiac remodelling in patients with end-stage hypertrophic cardiomyopathy. Eur J Heart Fail. 2008;10:507-13.

• Kato TS, Takayama H, Yoshizawa S, Marboe C, Schulze PC, Farr M, et al. Cardiac transplantation in patients with hypertrophic cardiomyopathy. Am J Cardiol. 2012;110:568-74.

• Maron BJ, Gottdiener J, Epstein S: Patterns and significance of distribution of left ventricular hypertrophy in hypertrophic cardiomyopathy. Am J Cardiol 1981; 48: 418-428.



Health & Medicine

Miocardiopatía Hipertrófica