Hypertrophe Kardiomyopathie (HCM) Flashcards
Was ist Myokardhypertrophie?
Vergrößerung des Herzmuskels, oft in Verbindung mit Herzkrankheiten
Myokardhypertrophie ist eine häufige Reaktion auf erhöhte Belastungen des Herzens.
Was ist der Unterschied zwischen HCM mit und ohne Obstruktion?
HCM mit Obstruktion: systol. intraventrikulärer Druckgradient; HCM ohne Obstruktion: kein Druckgradient
Der Druckgradient ist pathognomonisch für die obstruktive Form.
Nenne zwei Hauptformen der hypertrophischen Kardiomyopathie.
- Hypertrophische obstruktive Kardiomyopathie (HOCM)
- Hypertrophische nichtobstruktive Kardiomyopathie (HNCM)
Diese Formen unterscheiden sich in der Präsenz einer Obstruktion im Ausflusstrakt.
Was sind typische Symptome der hypertrophischen Kardiomyopathie?
- Dyspnoe
- Angina pectoris
- Schwindel
- Synkopen
Viele Patienten sind asymptomatisch oder haben nur geringe Symptome.
Worin besteht die klinische Differenzialdiagnose bei HCM?
Die Differenzierung zur stenosierenden KHK ist oft nicht möglich
HCM kann auch gleichzeitig mit einer koronaren Herzkrankheit auftreten.
Was beschreibt das SAM-Phänomen?
Systolische Vorwärtsbewegung von Strukturen des Mitralapparats
Das SAM-Phänomen ist ein typisches Zeichen der obstruktiven hypertrophischen Kardiomyopathie.
Wie wird die Diagnose der hypertrophischen Kardiomyopathie gestellt?
Echokardiografie ist das diagnostische Mittel der Wahl
Sie hilft bei der Beurteilung der Hypertrophieverteilung und -lokalisation.
Was sind die EKG-Befunde bei hypertropher Kardiomyopathie?
- Ventrikelhypertrophie
- ST-T-Veränderungen
- Pathologische Q-Zacken
EKG-Befunde können variieren und sind oft untypisch.
Was ist der Erbgang der familiären HCM?
Autosomal-dominanter Erbgang
Etwa die Hälfte der Fälle von HCM ist familiär bedingt.
Fülle die Lücke: Bei HCM ohne erkennbare Ursache ist die Erkrankung _______ verdächtig.
HCM
Unklare Myokardhypertrophie sollte immer auf HCM untersucht werden.
Was ist die Prävalenz von HCM in der Bevölkerung?
1 : 500 Erwachsene
HCM gehört zu den häufigsten genetischen Erkrankungen.
Nenne zwei Klassifikationen der hypertrophischen Kardiomyopathie.
- Hypertrophische obstruktive Kardiomyopathie (HOCM)
- Hypertrophische nichtobstruktive Kardiomyopathie (HNCM)
Diese Klassifikationen basieren auf der Hypertrophielokalisation und klinisch-funktionellen Aspekten.
Was sind die Befunde bei der Auskultation von HCM?
- Herzton normal
- Herzton mit normaler Spaltung
- systolisches Austreibungsgeräusch
Die Befunde können variieren, insbesondere bei asymptomatischen Patienten.
Worin besteht die Bedeutung der Echokardiografie bei HCM?
Diagnose der HCM, Hypertrophieverteilung, systol. und diastol. Ventrikelfunktion
Sie ist wichtig für die Beurteilung und das Screening von Familienangehörigen.
Wie wird die LV-Füllung bei HCM beurteilt?
Mit pw-Doppler
Die Füllungsgeschwindigkeiten E und A sind wichtige Parameter.
Was sind die typischen EKG-Veränderungen bei asymptomatischen Patienten mit HCM?
Gelegentlich normales EKG
Häufiger bei Kindern und nimmt mit dem Alter ab.
Was sind häufige rhythmische Störungen bei HCM?
- Vorhofflimmern
- Ventrikuläre Arrhythmien
Diese können die klinische Prognose erheblich beeinflussen.
Was beschreibt die diastolische Funktionsstörung bei HCM?
Gestörte Ventrikelrelaxation und abnorme Kammersteifigkeit
Dies führt zu hohen enddiastolischen Ventrikeldrücken.
What is the significance of E/E’ values in the assessment of cardiac function?
E/E’ < 10 indicates normal relaxation, while E/E’ > 10 indicates compliance disturbance
E/E’ values are used in echocardiography to assess diastolic function.
How can systolic ejection and obstruction be assessed using Doppler?
Use cw-Doppler to determine pressure gradients and the level of obstruction (mesoventricular, subaortal)
Doppler ultrasound helps visualize blood flow and detect obstructions.
What does a typical velocity profile of left ventricular ejection look like?
Saber-shaped with a late-systolic maximum
This profile is characteristic of the left ventricular ejection pattern.
What is the main indication for performing a cardiac MRI?
Differential diagnosis of myocardial diseases with the phenotype of HCM (e.g., amyloidosis, sarcoidosis)
Cardiac MRI provides detailed imaging and tissue characterization.
What factors are evaluated in cardiac MRI regarding myocardial hypertrophy?
LV mass index, myocardial collagen percentage, and myocardial scarring
Higher LV mass index correlates with poorer prognosis.
What are the indications for invasive diagnostics in hypertrophic obstructive cardiomyopathy (HOCM)?
Symptomatic HOCM requiring intervention, unclear obstruction severity, and suspected additional coronary artery disease
Invasive methods include catheterization and pressure measurements.
True or False: In HOCM, prominent a-waves in atrial pressures are observed.
True
These a-waves indicate increased atrial pressure.
What are common findings in hemodynamics for patients with HOCM?
Increased LVEDP and RVEDP, potential elevated PCWP and PAP
These measurements reflect the pressures in the left and right ventricles.
What is the Brockenbrough phenomenon?
Provocation of a systolic gradient during rest or Valsalva maneuver in the absence of obstruction
It indicates dynamic obstruction in HOCM.
What does cine-angiography reveal in patients with HCM?
Essential part of invasive diagnostics, displays LV in two planes and evaluates septum thickness and shape
Cine-angiography is crucial for assessing chamber anatomy.
Fill in the blank: The typical angiographic appearance in mid-ventricular obstruction is described as _______.
hourglass shape
This shape indicates a characteristic narrowing of the LV cavity.
What is the recommended management for asymptomatic patients with HCM?
General behavioral guidelines, possible treatment for significant family history or symptoms
Asymptomatic patients typically do not require specific medical treatment.
What is the first-line medication for symptomatic patients with HCM?
Beta-blockers
Beta-blockers help reduce heart rate and improve diastolic function.
What surgical intervention is indicated for severe outflow tract obstruction in HOCM?
Septal myotomy/myectomy
This procedure aims to relieve obstruction and improve ventricular relaxation.
True or False: Pregnancy is considered a high-risk condition for patients with HCM.
True
Pregnancy increases blood volume and requires careful monitoring in HCM patients.
What complications can arise from atrial fibrillation in HCM patients?
Acute hemodynamic decompensation and increased risk of embolism
Management may include emergency cardioversion and long-term anticoagulation.
What is the role of amiodarone in treating ventricular tachyarrhythmias in HCM?
Used for rhythm control and stabilization of sinus rhythm
Amiodarone is an antiarrhythmic drug that can help manage arrhythmias.
What is the recommended procedure for patients with sustained VT and high outflow tract gradients?
Postoperative rhythm analysis including electrophysiological study (EPU), possibly ICD therapy.
What should be done for patients with asymptomatic, short, non-sustained VT without significant gradients?
Electrophysiological study (EPU) to analyze rhythm and possibly review medical antiarrhythmic therapy.
What is the first-line medication for medical antiarrhythmic therapy?
Amiodarone.
When is ICD therapy indicated in patients with symptomatic VT?
Despite effective antiarrhythmic therapy based on electrophysiological criteria.
What tool is used to assess risk for PHT in patients with HCM?
Web-based risk calculator.
What factors are considered in calculating the risk for PHT in the next 5 years?
- Positive family history for PHT
- Unclear syncope
- Age at diagnosis
- Evidence of non-sustained VT
- Degree of hypertrophy
- Atrial size
- LVOT gradient.
What is the threshold for ICD implantation based on risk calculation for PHT?
Risk > 6% indicates ICD implantation; risk < 6% likely has no benefit in primary prophylaxis.
What is the recommended therapy for symptomatic bradycardias?
SM therapy according to indication guidelines.
What stimulation mode is preferred for bradycardia therapy?
AV-sequential stimulation mode (dual chamber SM) for hemodynamic reasons.
What is the risk associated with systemic embolisms?
Increased risk primarily with chronic atrial fibrillation (AF).
What is the treatment for patients with intermittent or chronic-persistent AF?
Lifelong anticoagulation.
What are the signs of progressive heart failure in patients?
- Decrease in intraventricular gradient
- Decrease or disappearance of systolic murmur
- Cavum dilatation
- Decreased ejection fraction
- Frequent AF arrhythmia and symptoms of congestive heart failure.
What are the treatment options for systolic pump dysfunction?
- Digitalis glycosides
- Diuretics
- Possibly ACE inhibitors.
What is contraindicated in patients with normal or hyperdynamic ventricular function and diastolic dysfunction?
Positive inotropic therapy.
What are the differential diagnoses for hypertrophic cardiomyopathy?
- HOCM: Aortic stenosis, pulmonary stenosis, mitral valve disease, functional murmur.
- HNCM: Hypertensive heart disease, mitral regurgitation, storage disease, coronary artery disease, athlete’s heart, functional murmur.
What indicates HCM in athletes?
- Wall thickness > 12mm without LV dilation suggests HCM.
- LVEDD > 55mm indicates athlete’s heart.
What are the criteria for HCM?
- Atypical, segmental LVH
- LVEDD < 45mm
- LA dilation
- Disturbed diastole
- Female gender
- Family history for HCM
- EKG changes.
What is the significance of a pathologic EKG in diagnosing HCM in athletes?
A training pause can reveal normalizing EKG changes, making EKG valuable for diagnosis.
What is the natural course of HCM?
Highly variable; PHT is the most common cause of death (50–90%).
What is the typical prognosis for adults with HCM?
Most are asymptomatic or have mild symptoms with stable course (60–80%).
What is the prognosis for children with HCM?
Very poor, especially if associated with congestive heart failure.
What are the risk factors for identifying patients at risk for PHT?
- Positive family history for HCM or PHT < 45 years
- Survived cardiac arrest
- Previous syncope
- Non-sustained VT on long-term EKG
- Abnormal blood pressure response under stress.
What are the secondary risk factors for PHT in HCM patients?
- Atrial fibrillation/flutter
- LA dilation > 45mm
- High LVOT gradient at rest > 80mmHg.
