Background— Nonobstructive hypertrophic cardiomyopathy (HCM) has been regarded as the predominant hemodynamic form of the disease on the basis of assessment of outflow gradient under resting conditions. We sought to prospectively define the prevalence, clinical profile, and significance of left ventricular (LV) outflow tract obstruction under resting conditions and with physiological exercise in a large HCM cohort. Methods and Results— We prospectively analyzed 320 consecutive HCM patients (age, 47±17 years), measuring LV outflow gradient at rest, with Valsalva maneuver, and with exercise echocardiography. LV outflow obstruction was present at rest and/or with exercise in 225 patients (70%); 119 had rest gradients ≥50 mm Hg and were not exercised. Of the other 201 patients with gradients <50 mm Hg at rest (average, 4±9 mm Hg), 106 developed mechanical obstruction to LV outflow resulting from mitral valve–septal contact after exercise (80±43 mm Hg), including 76 with marked gradients ≥50 mm Hg and 46 with ...

Hypertrophic Cardiomyopathy Is Predominantly a Disease of Left Ventricular Outflow Tract Obstruction

HCM is a heterogeneous disease genotypically, phenotypically, pathophysiologically, clinically, and therapeutically. In decisions on the management of these patients, it is important to recognize this heterogeneity and to direct therapy at the predominant abnormalities.

Hypertrophic Cardiomyopathy Clinical Spectrum and Treatment

Background: Resting left ventricular outflow tract obstruction (LVOTO) occurs in 25% of patients with hypertrophic cardiomyopathy (HCM) and is an important cause of symptoms and disease progression. The prevalence and clinical significance of exercise induced LVOTO in patients with symptomatic non-obstructive HCM is uncertain. Methods and results: 87 symptomatic patients (43.3 (13.7) years, 67.8% males) with HCM and no previously documented LVOTO (defined as a gradient ⩾30 mm Hg) underwent echocardiography during upright cardiopulmonary exercise testing: 54 patients (62.1%; 95% CI 51.5 to 71.6) developed LVOTO during exercise (latent LVOTO); 33 (37.9%; 95% CI 28.4 to 48.5) had neither resting nor exercise LVOTO (non-obstructive). Patients with latent LVOTO were more likely to have systolic anterior motion of the mitral valve (SAM) at rest (relative risk 2.1, 95% CI 1.2 to 3.8; p = 0.01), and higher peak oxygen consumption (mean difference: 10.3%, 95% CI 2.1 to 18.5; p = 0.02) than patients with non-obstructive HCM. The only independent predictors of Δ gradient during exercise were a history of presyncope/syncope, incomplete/complete SAM at rest and Wigle score (all p Conclusions: Approximately two-thirds of patients with symptomatic non-obstructive HCM have latent LVOTO. This study suggests that all patients with symptomatic non-obstructive HCM should have exercise stress echocardiography.

https://heart.bmj.com/content/heartjnl/94/10/1288.full.pdf

Prevalence of exercise-induced left ventricular outflow tract obstruction in symptomatic patients with non-obstructive hypertrophic cardiomyopathy

Left ventricular systolic dysfunction during exercise and dobutamine stress in patients with hypertrophic cardiomyopathy

To assess the behavior of the subvalvular pressure gradient under physical exercise, 13 patients with obstructive hypertrophic cardiomyopathy were examined during upright bicycle ergometry by means of Doppler echocardiography. Additionally, right-sided cardiac catheterization was performed within 7 days. In 10 patients adequate Doppler tracings could be obtained during exercise. The Doppler-derived systolic pressure gradient increased from 75 ± 24 to 140 ± 42 mm Hg (p

Doppler echocardiographic assessment of the pressure gradient during bicycle ergometry in hypertrophic cardiomyopathy.

Patients with implantable cardioverter/defibrillators (ICD) suffer frequent and various complications during follow-up1. Within a recent multicenter study including 820 patients with a third-generation ICD, 280 patients (34%) experienced adverse events within a follow-up of only 5 ± 5 months2. Thus, a close follow-up of these patients by a physician aware of typical complications should be warranted. The most common adverse event is inappropriate arrhythmia detection typically resolved by programming or drug changes3. Other adverse events which might be resolved by reprogramming are syncopes despite ICD therapy4, ventricular tachycardias below the programmed detection rate5, and painful cardioversion shocks delivered during haemodynamically tolerated ventricular tachycardias6. Further complications, such as infection of the ICD, lead dis-lodgements, insulation failures, lead perforation, and emboli from thrombi attached to the leads which might be detected during follow-up, frequently require surgical intervention1.

Follow-Up Techniques in Patients with Implantable Cardioverter/Defibrillators

Wahrend der letzten zehn Jahre sind die Probleme und Limitierungen der medikamentosen antiarrhythmischen Therapie bewust geworden [1, 2]. Bei Patienten mit dokumentierten anhaltenen Kammertachykardien oder Kammerflimmern, die nicht wahrend der Akutphase eines frischen Infarktes aufgetreten sind, wird die medikamentose antiarrhythmische Therapie ublicherweise durch serielle elektrophysiologische Testungen auf ihre Wirksamkeit uberpruft [3, 4]. Unglucklicherweise sprechen jedoch nur etwa 30% der Patienten wahrend serieller elektrophysiologischer Testungen auf Antiarrhythmika an [1, 3, 4]. Insbesondere bei Patienten mit einer eingeschrankten linksventrikularen Funktion last sich wesentlich seltener ein Antiarrhythmikum finden, welches die Auslosbarkeit der Tachykardien unterdruckt oder zumindest erschwert, als bei Patienten mit guter linksventrikularer Funktion [5]. Aufgrund von Verlaufsbeobachtungen bieten sich serielle elektrophysiologische Untersuchungen dafur an, einerseits diejenigen Patienten zu identifizieren, bei denen unter antiarrhythmischer Therapie eine gute Prognose zu erwarten ist, andererseits solche Patienten, die trotz antiarrhythmischer Therapie eine hohe Sterblichkeit, insbesondere als Folge von rhythmusbedingten Komplikationen aufweisen. Das Bestreben geht dahin, bei letzteren Patienten neue Interventionstechniken wie Katheterablation, gezielte antitachykarde Operation und implantierbare Kardioverter/Defibrillatoren einzusetzen [6, 7, 8].

Nicht-medikamentöse Therapie von ventrikulären Tachyarrhythmien

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Doppler echocardiographic assessment of the pressure gradient during bicycle ergometry in hypertrophic cardiomyopathy.

Follow-Up Techniques in Patients with Implantable Cardioverter/Defibrillators

Nicht-medikamentöse Therapie von ventrikulären Tachyarrhythmien