Hypertrophic cardiomyopathy Nicolas Mansencal Hpital Ambroise Par, - - PowerPoint PPT Presentation

Unknown indications of stress test Hypertrophic cardiomyopathy

Journées Européennes de la SFC Paris, 17 janvier 2014

Nicolas Mansencal

Hôpital Ambroise Paré, Boulogne Centre de Réfce pour les Mdies Cardques Héréditaires Université de Versailles Saint-Quentin France

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• Bracco Imaging, MSD, Astrazeneca
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I currently have, or have had over the last two years, an affiliation or financial interests or interests of any order with a company or I receive compensation or fees or research grants with a commercial company :

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Cardiomyopathies HCM DCM ARVC Unclassified Familial/Genetic Non-familial/Non-genetic RCM Elliott et al. Eur Heart J 2008

Classification

• Characterized by abnormal wall thickness

(LV, predominating in IVS)

• Most frequent cause of sudden death in

athlete < 35 yo HCM:

August 2011 November 2011

www.has-sante.fr

Why and which explorations?

• Which?

– ECG – Echocardiography (± contrast LVO) – Cardiac magnetic resonance (doubt) – Exercise test / Exercise echocardiography – 24–hour ambulatory (Holter) ECG monitoring

• Why?
• 1. To perform the diagnosis of HCM
• 2. To assess the presence/absence of gradient
• 3. To assess the risk of sudden death

Echo diagnosis

E A E A E A

IVRT DeT

IVRT DeT IVRT DeT

Major criterion  Non familial HCM: Thickness > 15 mm  Familial HCM: Thickness > 13 mm Associated measurements

• LVEDD ≤ 45 mm
• Pattern of hypertrophy

– symmetric LVH: IVS/PW ≤ 1.3 – asymmetric LVH: IVS/PW > 1.3

• Diastolic dysfunction
• Gradient > 30 mmHg

LV outflow tract gradient

• 2/3 had no gradient
• Assessment of SAM
• The gradient is highly variable:

– 12 HCM during 5 days versus 5 AS – Mean coefficient of variation: 0.52 ± 0.33 – Mean coefficient of variation in AS: < 0.10 – A single measurement is inadequate

Kizilbash Circulation 1998

• Gradient varies with exercise, load

conditions and is related to Σ

• Significant gradient: > 30 mmHg

Maron Circulation 2012

Canepa Am J Cardiol 2013

63% without and 37% with >50 mmHg 47% without and 52% with > 30 mmHg

320 HCM

≥50 mmHg

119

30-49 mmHg

11

< 30 mmHg

190

REST: 320

Maron Circulation 2006

76 30

≥50 mmHg 30-49 mmHg < 30 mmHg

95

EXER 201

95 pts (30%) without and 225 pts (70%) with > 30mmHg

Feiner J Am Coll Cardiol 2013

Post-prandial exercise

HCM and gradient assessment

• Examination
• Echocardiography
• Provocation test:

– Physiologic test: Exercise echocardiography – Pharmacologic test (nitrite, Dobutamine infusion)

• CMR imaging

Writing Committee Members Circulation 2011

Argulian Prog Cardiovasc Dis 2012

Patients with HCM and angina-like symptoms

Athlete’s heart or HCM?

Moderate LV hypertrophy No gradient If gradient  HCM

Recommandations SFC 2007

Athlete’s heart or HCM

Athlete’s heart

• IVS < 13 mm
• Symmetrical LV hypertrophy
• LV diameter > 55 mm
• Normal diastolic function
• Normal left atrium
• No symptoms
• No previous history of HCM/SD
• Normal ECG, exercise test,

holter ECG

• Decreased wall thickness with

deconditionning

HCM

• IVS > 15 mm
• Asymmetrical LV hypertrophy
• LV diameter < 45 mm
• Abnormal diastolic function
• Dilated left atrium
• Symptomatic
• Previous history of HCM/SD
• Abnormal ECG, exercise test,

holter ECG

• No decreased wall thickness

with deconditionning Grey zone

• In subjects without HCM:

 Prevalence of gradient: 5 à 13 %

• Predisposing factors of occurrence of this

gradient: Morphology of the left ventricle & mitral valv.

• Interpretation performed according to:
• Type of exercise
• Intensity of exercise
• Abrupt discontinuation (or not) of exercise
• Time of onset of the gradient (during exercise or

recovery)

LVOT gradient & exercise

Zywica et al. Eur J Echocardiogr 2008; Cabrera Bueno Rev Esp Cardiol 2004

Sudden death and HCM

Identification of high-risk patients: 5 variables related to sudden death:

 History of syncope + familial history of sudden death (multivariate SD risk ratios = 5.3)  LV thickness > 30 mm (RR = 2)  NSVT (Holter ECG monitoring) (RR = 1.9)  Abnormal exercise blood pressure (RR = 1.8)

Elliott J Am Coll Cardiol 2000

O’Mahony Heart 2013 Exercise test Syncope SCD NSVT Severe LVH

• Age-sex predicted METs

achieved (p = 0.0001)

• Abnormal heart rate recovery at

1 min (<12-beat drop,p = 0.007)

• AF (p = 0.007)

Desai J Am Coll Cardiol Img 2013

Stress testing

• Treadmill exercise testing is reasonable to determine

functional capacity and response to therapy (LoE: C)

• Treadmill testing with monitoring of an ECG and

blood pressure is reasonable for SCD risk stratification (LoE: B)

• In patients without resting peak instantaneous gradient
• f greater ≥ 50 mm Hg, exercise echo is reasonable for

the detection and quantification of exercise induced dynamic LVOT obstruction (LoE: B) Class IIa

Examinations for follow-up

• Baseline examinations:
• ECG
• Echocardiography
• Exercise test (BP)
• Holter ECG
• No symptoms, no risk factors:
• Echocardiography (/y), exercise test and

Holter ECG (/2-3y)

• At least 1 risk factor or symptoms:
• Echocardiography (/y), exercise test (/y) and

Holter ECG (/y)

Dubourg HAS 2011

Take Home Message

• Hypertrophic cardiomyopathy:
• Use the French recommendations
• http://www.has-sante.fr
• Two stress tests:
• Exercise test
• Exercise echocardiography
• HCM and stress tests:
• Diagnosis
• Treatment
• Prognosis
• Assessment of gradient using exercise echo
• Assessment of prognosis using exercise test