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Kardiologie > Hypertrophe Kardiomyopathie (HCM) > Flashcards

Hypertrophe Kardiomyopathie (HCM) Flashcards

1
Q

Was ist Myokardhypertrophie?

A

Vergrößerung des Herzmuskels, oft in Verbindung mit Herzkrankheiten

Myokardhypertrophie ist eine häufige Reaktion auf erhöhte Belastungen des Herzens.

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2
Q

Was ist der Unterschied zwischen HCM mit und ohne Obstruktion?

A

HCM mit Obstruktion: systol. intraventrikulärer Druckgradient; HCM ohne Obstruktion: kein Druckgradient

Der Druckgradient ist pathognomonisch für die obstruktive Form.

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3
Q

Nenne zwei Hauptformen der hypertrophischen Kardiomyopathie.

A
  • Hypertrophische obstruktive Kardiomyopathie (HOCM)
  • Hypertrophische nichtobstruktive Kardiomyopathie (HNCM)

Diese Formen unterscheiden sich in der Präsenz einer Obstruktion im Ausflusstrakt.

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4
Q

Was sind typische Symptome der hypertrophischen Kardiomyopathie?

A
  • Dyspnoe
  • Angina pectoris
  • Schwindel
  • Synkopen

Viele Patienten sind asymptomatisch oder haben nur geringe Symptome.

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5
Q

Worin besteht die klinische Differenzialdiagnose bei HCM?

A

Die Differenzierung zur stenosierenden KHK ist oft nicht möglich

HCM kann auch gleichzeitig mit einer koronaren Herzkrankheit auftreten.

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6
Q

Was beschreibt das SAM-Phänomen?

A

Systolische Vorwärtsbewegung von Strukturen des Mitralapparats

Das SAM-Phänomen ist ein typisches Zeichen der obstruktiven hypertrophischen Kardiomyopathie.

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7
Q

Wie wird die Diagnose der hypertrophischen Kardiomyopathie gestellt?

A

Echokardiografie ist das diagnostische Mittel der Wahl

Sie hilft bei der Beurteilung der Hypertrophieverteilung und -lokalisation.

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8
Q

Was sind die EKG-Befunde bei hypertropher Kardiomyopathie?

A
  • Ventrikelhypertrophie
  • ST-T-Veränderungen
  • Pathologische Q-Zacken

EKG-Befunde können variieren und sind oft untypisch.

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9
Q

Was ist der Erbgang der familiären HCM?

A

Autosomal-dominanter Erbgang

Etwa die Hälfte der Fälle von HCM ist familiär bedingt.

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10
Q

Fülle die Lücke: Bei HCM ohne erkennbare Ursache ist die Erkrankung _______ verdächtig.

A

HCM

Unklare Myokardhypertrophie sollte immer auf HCM untersucht werden.

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11
Q

Was ist die Prävalenz von HCM in der Bevölkerung?

A

1 : 500 Erwachsene

HCM gehört zu den häufigsten genetischen Erkrankungen.

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12
Q

Nenne zwei Klassifikationen der hypertrophischen Kardiomyopathie.

A
  • Hypertrophische obstruktive Kardiomyopathie (HOCM)
  • Hypertrophische nichtobstruktive Kardiomyopathie (HNCM)

Diese Klassifikationen basieren auf der Hypertrophielokalisation und klinisch-funktionellen Aspekten.

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13
Q

Was sind die Befunde bei der Auskultation von HCM?

A
    1. Herzton normal
    1. Herzton mit normaler Spaltung
  • systolisches Austreibungsgeräusch

Die Befunde können variieren, insbesondere bei asymptomatischen Patienten.

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14
Q

Worin besteht die Bedeutung der Echokardiografie bei HCM?

A

Diagnose der HCM, Hypertrophieverteilung, systol. und diastol. Ventrikelfunktion

Sie ist wichtig für die Beurteilung und das Screening von Familienangehörigen.

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15
Q

Wie wird die LV-Füllung bei HCM beurteilt?

A

Mit pw-Doppler

Die Füllungsgeschwindigkeiten E und A sind wichtige Parameter.

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16
Q

Was sind die typischen EKG-Veränderungen bei asymptomatischen Patienten mit HCM?

A

Gelegentlich normales EKG

Häufiger bei Kindern und nimmt mit dem Alter ab.

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17
Q

Was sind häufige rhythmische Störungen bei HCM?

A
  • Vorhofflimmern
  • Ventrikuläre Arrhythmien

Diese können die klinische Prognose erheblich beeinflussen.

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18
Q

Was beschreibt die diastolische Funktionsstörung bei HCM?

A

Gestörte Ventrikelrelaxation und abnorme Kammersteifigkeit

Dies führt zu hohen enddiastolischen Ventrikeldrücken.

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19
Q

What is the significance of E/E’ values in the assessment of cardiac function?

A

E/E’ < 10 indicates normal relaxation, while E/E’ > 10 indicates compliance disturbance

E/E’ values are used in echocardiography to assess diastolic function.

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20
Q

How can systolic ejection and obstruction be assessed using Doppler?

A

Use cw-Doppler to determine pressure gradients and the level of obstruction (mesoventricular, subaortal)

Doppler ultrasound helps visualize blood flow and detect obstructions.

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21
Q

What does a typical velocity profile of left ventricular ejection look like?

A

Saber-shaped with a late-systolic maximum

This profile is characteristic of the left ventricular ejection pattern.

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22
Q

What is the main indication for performing a cardiac MRI?

A

Differential diagnosis of myocardial diseases with the phenotype of HCM (e.g., amyloidosis, sarcoidosis)

Cardiac MRI provides detailed imaging and tissue characterization.

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23
Q

What factors are evaluated in cardiac MRI regarding myocardial hypertrophy?

A

LV mass index, myocardial collagen percentage, and myocardial scarring

Higher LV mass index correlates with poorer prognosis.

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24
Q

What are the indications for invasive diagnostics in hypertrophic obstructive cardiomyopathy (HOCM)?

A

Symptomatic HOCM requiring intervention, unclear obstruction severity, and suspected additional coronary artery disease

Invasive methods include catheterization and pressure measurements.

25
Q

True or False: In HOCM, prominent a-waves in atrial pressures are observed.

A

True

These a-waves indicate increased atrial pressure.

26
Q

What are common findings in hemodynamics for patients with HOCM?

A

Increased LVEDP and RVEDP, potential elevated PCWP and PAP

These measurements reflect the pressures in the left and right ventricles.

27
Q

What is the Brockenbrough phenomenon?

A

Provocation of a systolic gradient during rest or Valsalva maneuver in the absence of obstruction

It indicates dynamic obstruction in HOCM.

28
Q

What does cine-angiography reveal in patients with HCM?

A

Essential part of invasive diagnostics, displays LV in two planes and evaluates septum thickness and shape

Cine-angiography is crucial for assessing chamber anatomy.

29
Q

Fill in the blank: The typical angiographic appearance in mid-ventricular obstruction is described as _______.

A

hourglass shape

This shape indicates a characteristic narrowing of the LV cavity.

30
Q

What is the recommended management for asymptomatic patients with HCM?

A

General behavioral guidelines, possible treatment for significant family history or symptoms

Asymptomatic patients typically do not require specific medical treatment.

31
Q

What is the first-line medication for symptomatic patients with HCM?

A

Beta-blockers

Beta-blockers help reduce heart rate and improve diastolic function.

32
Q

What surgical intervention is indicated for severe outflow tract obstruction in HOCM?

A

Septal myotomy/myectomy

This procedure aims to relieve obstruction and improve ventricular relaxation.

33
Q

True or False: Pregnancy is considered a high-risk condition for patients with HCM.

A

True

Pregnancy increases blood volume and requires careful monitoring in HCM patients.

34
Q

What complications can arise from atrial fibrillation in HCM patients?

A

Acute hemodynamic decompensation and increased risk of embolism

Management may include emergency cardioversion and long-term anticoagulation.

35
Q

What is the role of amiodarone in treating ventricular tachyarrhythmias in HCM?

A

Used for rhythm control and stabilization of sinus rhythm

Amiodarone is an antiarrhythmic drug that can help manage arrhythmias.

36
Q

What is the recommended procedure for patients with sustained VT and high outflow tract gradients?

A

Postoperative rhythm analysis including electrophysiological study (EPU), possibly ICD therapy.

37
Q

What should be done for patients with asymptomatic, short, non-sustained VT without significant gradients?

A

Electrophysiological study (EPU) to analyze rhythm and possibly review medical antiarrhythmic therapy.

38
Q

What is the first-line medication for medical antiarrhythmic therapy?

A

Amiodarone.

39
Q

When is ICD therapy indicated in patients with symptomatic VT?

A

Despite effective antiarrhythmic therapy based on electrophysiological criteria.

40
Q

What tool is used to assess risk for PHT in patients with HCM?

A

Web-based risk calculator.

41
Q

What factors are considered in calculating the risk for PHT in the next 5 years?

A
  • Positive family history for PHT
  • Unclear syncope
  • Age at diagnosis
  • Evidence of non-sustained VT
  • Degree of hypertrophy
  • Atrial size
  • LVOT gradient.
42
Q

What is the threshold for ICD implantation based on risk calculation for PHT?

A

Risk > 6% indicates ICD implantation; risk < 6% likely has no benefit in primary prophylaxis.

43
Q

What is the recommended therapy for symptomatic bradycardias?

A

SM therapy according to indication guidelines.

44
Q

What stimulation mode is preferred for bradycardia therapy?

A

AV-sequential stimulation mode (dual chamber SM) for hemodynamic reasons.

45
Q

What is the risk associated with systemic embolisms?

A

Increased risk primarily with chronic atrial fibrillation (AF).

46
Q

What is the treatment for patients with intermittent or chronic-persistent AF?

A

Lifelong anticoagulation.

47
Q

What are the signs of progressive heart failure in patients?

A
  • Decrease in intraventricular gradient
  • Decrease or disappearance of systolic murmur
  • Cavum dilatation
  • Decreased ejection fraction
  • Frequent AF arrhythmia and symptoms of congestive heart failure.
48
Q

What are the treatment options for systolic pump dysfunction?

A
  • Digitalis glycosides
  • Diuretics
  • Possibly ACE inhibitors.
49
Q

What is contraindicated in patients with normal or hyperdynamic ventricular function and diastolic dysfunction?

A

Positive inotropic therapy.

50
Q

What are the differential diagnoses for hypertrophic cardiomyopathy?

A
  • 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.
51
Q

What indicates HCM in athletes?

A
  • Wall thickness > 12mm without LV dilation suggests HCM.
  • LVEDD > 55mm indicates athlete’s heart.
52
Q

What are the criteria for HCM?

A
  • Atypical, segmental LVH
  • LVEDD < 45mm
  • LA dilation
  • Disturbed diastole
  • Female gender
  • Family history for HCM
  • EKG changes.
53
Q

What is the significance of a pathologic EKG in diagnosing HCM in athletes?

A

A training pause can reveal normalizing EKG changes, making EKG valuable for diagnosis.

54
Q

What is the natural course of HCM?

A

Highly variable; PHT is the most common cause of death (50–90%).

55
Q

What is the typical prognosis for adults with HCM?

A

Most are asymptomatic or have mild symptoms with stable course (60–80%).

56
Q

What is the prognosis for children with HCM?

A

Very poor, especially if associated with congestive heart failure.

57
Q

What are the risk factors for identifying patients at risk for PHT?

A
  • 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.
58
Q

What are the secondary risk factors for PHT in HCM patients?

A
  • Atrial fibrillation/flutter
  • LA dilation > 45mm
  • High LVOT gradient at rest > 80mmHg.

Kardiologie (26 decks)

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