Takotsubo syndrome

Acute stress cardiomyopathy syndrome characterized by transient and reversible regional left ventricular systolic dysfunction (typically apical and mid-ventricular ballooning with compensatory contractile hyperkinesia of basal segments).

Definition

Acute stress cardiomyopathy syndrome characterized by transient and reversible regional left ventricular systolic dysfunction (typically apical and mid-ventricular ballooning with compensatory contractile hyperkinesia of basal segments), typically triggered by intense emotional or physical stress factors and mediated by a massive catecholamine surge, mimicking an acute coronary syndrome in the absence of culprit obstructive atherothrombotic coronary disease [C1] [G5-03] [G5-04] [G5-16].

Severity criteria

  • Transient regional dysfunction: Transient regional wall motion abnormalities (akinesia, hypokinesia, or dyskinesia) of apical and/or mid-ventricular LV segments extending beyond the distribution of a single epicardial coronary artery [G5-03].
  • Systolic function depression: Depressed left ventricular ejection fraction in the acute phase (typically 30–45%), with full ad integrum recovery of wall motion within weeks [G5-03] [G5-04].
  • Dynamic LVOT obstruction: Emergence of a dynamic intraventricular or LVOT gradient (peak LVOT gradient ≥ 25–30 mmHg, reaching values > 40–50 mmHg in severe cases) in 12–25% of patients due to basal hyperkinesia and systolic anterior motion (SAM) of the anterior mitral leaflet [G5-04] [G5-16].
  • Filling pressure elevation: Transiently elevated mean pulmonary capillary wedge pressure (> 18–20 mmHg) in the presence of acute pulmonary edema or acute heart failure [G5-04].

Findings by layer

Hemodynamics

  • LV ejection fraction (left ventricular ejection fraction): Reduced in acute phase to 30–45% [G5-03] [G5-04].
  • Dynamic LVOT gradient: Elevated (25–50 mmHg) in the subgroup of patients (15–20%) who develop functional intraventricular obstruction due to basal hyperkinesia [G5-04] [G5-16].
  • Pulmonary capillary wedge pressure: Moderately elevated during the acute episode [G5-04].

Rhythm

  • Sinus tachycardia as a reflex response to massive endogenous sympathetic adrenergic surge [G5-03].
  • Ventricular or supraventricular arrhythmias possible during the acute phase of electrical instability [G5-03] [G5-04].

ECG

  • Acute phase: ST-segment elevation (diffuse ST-segment elevation / persistent ST-segment elevation) in anterior precordial leads (V2 to V6) and lead I/aVL, typically without reciprocal ST depression in inferior leads (distinctive feature distinguishing it from classic anterior STEMI) [G5-03] [G5-04].
  • Subacute/evolutionary phase: Deep, symmetric, and diffuse T-wave inversion in precordial and limb leads [G5-03].
  • QT interval prolongation: Markedly prolonged QTc interval (prolonged QT interval: QTc > 480–500 ms) in the subacute phase, associating risk of polymorphic ventricular arrhythmias such as torsades de pointes [G5-03] [G5-04].

Heart sounds and murmurs

  • Ejection systolic murmur: Mesosystolic murmur at left sternal border and apex when dynamic intraventricular/LVOT gradient develops from mitral SAM and outflow tract narrowing [G5-04] [G5-16].
  • Mitral regurgitation murmur: Apical holosystolic murmur of secondary mitral regurgitation generated by SAM or leaflet tethering in the setting of ventricular geometric dysfunction [G5-04] [G5-16].
  • Third and fourth heart sounds (S3 and S4): Auscultated transiently during the acute congestive phase at the apex [G1-12] [G1-13].

Arterial pulse

  • Tachycardic pulse with preserved or diminished volume; in the presence of dynamic LVOT gradient it may acquire pulsus bisferiens characteristics [B4] [G5-16].

Jugular venous pulse

  • Generally normal or with moderate elevation of venous pressure in cases of acute biventricular congestion [B3].

Maneuvers

  • Valsalva maneuver: Increases ejection murmur intensity in patients presenting with dynamic LVOT obstruction by reducing end-diastolic volume [B1] [G5-16].

Simulator severity grades

Simulator severity grades: Single grade
Baseline rhythm: Sinus Tachycardia

Also known as: Takotsubo cardiomyopathy · Stress-induced cardiomyopathy · Broken heart syndrome · Apical ballooning syndrome · Stress cardiomyopathy

Other pathologies in this group

Sources

  1. [B1] Bedside diagnosis of systolic murmurs . N Engl J Med (1988) . DOI: 10.1056/NEJM198806163182404 PubMed: 2897627
  2. [B3] The jugular venous pressure revisited . Cleve Clin J Med (2013) . DOI: 10.3949/ccjm.80a.13039 PubMed: 24085809
  3. [B4] The Carotid Pulse . Clinical Methods: The History, Physical, and Laboratory Examinations (1990) . PubMed: 21250154
  4. [C1] 2023 ESC Guidelines for the management of cardiomyopathies . Eur Heart J (2023) . DOI: 10.1093/eurheartj/ehad194 PubMed: 37622657
  5. [G1-12] The Third Heart Sound . Clinical Methods: The History, Physical, and Laboratory Examinations (1990) . PubMed: 21250184
  6. [G1-13] The Fourth Heart Sound . Clinical Methods: The History, Physical, and Laboratory Examinations (1990) . PubMed: 21250185
  7. [G5-03] International Expert Consensus Document on Takotsubo Syndrome (Part I): Clinical Characteristics, Diagnostic Criteria, and Pathophysiology . Eur Heart J (2018) . DOI: 10.1093/eurheartj/ehy076 PubMed: 29850871
  8. [G5-04] International Expert Consensus Document on Takotsubo Syndrome (Part II): Diagnostic Workup, Outcome, and Management . Eur Heart J (2018) . DOI: 10.1093/eurheartj/ehy077 PubMed: 29850820
  9. [G5-16] Prevalence and characteristics of left ventricular outflow tract obstruction in Tako-Tsubo syndrome . Am Heart J (2008) . DOI: 10.1016/j.ahj.2008.05.002 PubMed: 18760139