p.3 抽出テキスト

6.8.3.1. Genetic counselling in children ........................................ 3534 6.8.3.2. Pre- and post-test genetic counselling (proband) .... 3535 6.8.3.3. Genetic counselling for cascade testing ........................ 3535 6.8.3.4. Pre-natal or pre-implantation genetic diagnosis ....... 3536 6.9. Diagnostic approach to paediatric patients ................................ 3537 6.9.1. Infantile and early childhood-onset cardiomyopathy ...... 3538 6.10. General principles in the management of patients with cardiomyopathy ............................................................................................... 3539 6.10.1. Assessment of symptoms ......................................................... 3539 6.10.2. Heart failure management ........................................................ 3539 6.10.2.1. Preventive heart failure medical therapy of asymptomatic carriers/early disease expression ....................... 3540 6.10.2.2. Cardiac transplantation ..................................................... 3540 6.10.2.3. Left ventricular assist devices ......................................... 3540 6.10.3. Management of atrial arrhythmias ........................................ 3541 6.10.3.1. Anticoagulation ..................................................................... 3541 6.10.3.2. Rate control ........................................................................... 3541 6.10.3.3. Rhythm control .................................................................... 3543 6.10.3.4. Comorbidities and risk factor management ............ 3543 6.10.4. Management of ventricular arrhythmias ............................ 3544 6.10.5. Device therapy: implantable cardioverter defibrillator 3544 6.10.6. Routine follow-up of patients with cardiomyopathy ... 3546 6.11. Family screening and follow-up evaluation of relatives ....... 3546 6.11.1. Special considerations in family screening ......................... 3547 6.12. Psychological support in cardiomyopathy patients and family members ............................................................................................................. 3548 6.13. The patient pathway .......................................................................... 3549 7. Specific cardiomyopathy phenotypes ...................................................... 3549 7.1. Hypertrophic cardiomyopathy ......................................................... 3549 7.1.1. Diagnosis ............................................................................................ 3549 7.1.1.1. Diagnostic criteria .................................................................. 3549 7.1.1.2. Diagnostic work-up ............................................................... 3549 7.1.1.3. Echocardiography ................................................................... 3549 7.1.1.4. Cardiac magnetic resonance ............................................. 3550 7.1.1.5. Nuclear imaging ...................................................................... 3551 7.1.2. Genetic testing and family screening ...................................... 3551 7.1.3. Assessment of symptoms ........................................................... 3552 7.1.4. Management of symptoms and complications ................... 3552 7.1.4.1. Management of left ventricular outflow tract obstruction ............................................................................................... 3553 7.1.4.1.1. General measures .......................................................... 3553 7.1.4.1.2. Drug therapy ................................................................... 3553 7.1.4.1.3. Invasive treatment of left ventricular outflow tract (septal reduction therapy) ................................................. 3555 7.1.4.2. Management of symptoms in patients without left ventricular outflow tract obstruction ........................................... 3557 7.1.4.2.1. Heart failure and chest pain ...................................... 3557 7.1.4.2.2. Cardiac resynchronization therapy ........................ 3557 7.1.5. Sudden cardiac death prevention in hypertrophic cardiomyopathy ........................................................................................... 3558

7.1.5.1. Left ventricular apical aneurysms .................................... 3559 7.1.5.2. Left ventricular systolic dysfunction ............................... 3559 7.1.5.3. Late gadolinium enhancement on cardiac magnetic resonance imaging ................................................................................. 3559 7.1.5.4. Abnormal exercise blood pressure response ............ 3559 7.1.5.5. Sarcomeric variants ............................................................... 3560 7.1.5.6. Prevention of sudden cardiac death .............................. 3560 7.2. Dilated cardiomyopathy ...................................................................... 3562 7.2.1. Diagnosis ............................................................................................ 3562 7.2.1.1. Index case .................................................................................. 3562 7.2.1.2. Relatives ..................................................................................... 3562 7.2.1.3. Diagnostic work-up ............................................................... 3562 7.2.1.4. Echocardiography ................................................................... 3562 7.2.1.5. Cardiac magnetic resonance ............................................. 3563 7.2.1.6. Nuclear medicine ................................................................... 3563 7.2.2. Genetic testing and family screening ...................................... 3563 7.2.2.1. Genetic testing ........................................................................ 3563 7.2.3. Assessment of symptoms ........................................................... 3564 7.2.4. Management ...................................................................................... 3564 7.2.5. Sudden cardiac death prevention in dilated cardiomyopathy ........................................................................................... 3564

7.2.5.1. Secondary prevention of sudden cardiac death ....... 3564 7.2.5.2. Primary prevention of sudden cardiac death ............. 3564 7.3. Non-dilated left ventricular cardiomyopathy ............................. 3566 7.3.1. Diagnosis ............................................................................................ 3566 7.3.1.1. Index case .................................................................................. 3566 7.3.1.2. Relatives ..................................................................................... 3566 7.3.1.3. Diagnostic work-up ............................................................... 3566 7.3.1.4. Electrocardiographic features ........................................... 3567 7.3.1.5. Echocardiography ................................................................... 3567 7.3.1.6. Cardiac magnetic resonance ............................................. 3567 7.3.1.7. Nuclear medicine ................................................................... 3567 7.3.1.8. Endomyocardial biopsy ........................................................ 3567 7.3.2. Genetic testing ................................................................................. 3567 7.3.3. Assessment of symptoms ........................................................... 3567 7.3.4. Management ...................................................................................... 3567 7.3.5. Sudden cardiac death prevention in non-dilated left ventricular cardiomyopathy .................................................................... 3568 7.3.5.1. Secondary prevention of sudden cardiac death ....... 3568 7.3.5.2. Primary prevention of sudden cardiac death ............. 3568 7.4. Arrhythmogenic right ventricular cardiomyopathy ................. 3569 7.4.1. Diagnosis ............................................................................................ 3569 7.4.1.1. Index case .................................................................................. 3569 7.4.1.2. Relatives ..................................................................................... 3569 7.4.1.3. Diagnostic work-up ............................................................... 3569 7.4.1.4. Electrocardiography and Holter monitoring .............. 3569 7.4.1.5. Echocardiography and cardiac magnetic resonance 3569 7.4.1.6. Endomyocardial biopsy ........................................................ 3569 7.4.1.7. Nuclear medicine ................................................................... 3569 7.4.1.8. Arrhythmogenic right ventricular cardiomyopathy phenocopies ............................................................................................. 3570 7.4.2. Genetic testing and family screening ...................................... 3570 7.4.3. Assessment of symptoms ........................................................... 3570 7.4.4. Management ...................................................................................... 3570 7.4.4.1. Antiarrhythmic therapy ....................................................... 3570 7.4.5. Sudden cardiac death prevention in arrhythmogenic right ventricular cardiomyopathy .................................................................... 3570 7.4.5.1. Secondary prevention of sudden cardiac death ....... 3571 7.4.5.2. Primary prevention of sudden cardiac death ............. 3571 7.5. Restrictive cardiomyopathy ............................................................... 3572 7.5.1. Diagnosis ............................................................................................ 3572 7.5.2. Genetic testing ................................................................................. 3572

ESC Guidelines                                                                                                                                                                                          3505

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