MioAtlas
G9: Masses and tumors

Cardiac rhabdomyoma

Most common primary cardiac tumor in neonates and children, of benign hamartomatous nature, characterized by myocardial accumulations of glycogen-rich vacuolated cardiomyocytes, closely linked to tuberous sclerosis complex.

Open in 3D simulator

Definition

Most common primary cardiac tumor in neonates and children, of benign hamartomatous nature, characterized by myocardial accumulations of glycogen-rich vacuolated cardiomyocytes, closely linked to tuberous sclerosis complex; manifests as solid intramyocardial nodular masses typically multiple distributed in interventricular septum and ventricular walls, capable of producing acceleration or mild obstruction to outflow tract and arrhythmias or ventricular pre-excitation (Wolff-Parkinson-White syndrome) [C10] [G810-09].

Severity criteria

  • Literal statement from multicenter study of Padalino, Basso et al. [G810-09, Abstract]:
    • «Rhabdomyomas are the most frequent surgical histotypes»
  • Obstructive gradient: Peak gradient across ventricular outflow tract typically mild or absent (< 20–25 mmHg) in vast majority of cases [C10] [G810-09].
  • Pre-excitation and arrhythmias: Frequency of accessory atrioventricular pathways and ventricular pre-excitation associated with mass [C10] [G810-09].

Findings by layer

Hemodynamics

  • Hemodynamics globally preserved at rest; mild dynamic outflow tract gradient (< 25 mmHg) if mass protrudes into ejection pathway [C10] [G810-09].
  • Blood pressure and cardiac output within normal limits (systolic blood pressure 110–120 mmHg, cardiac output 4.5–5.5 L/min) [C10].

Rhythm

  • Normal sinus rhythm with alternative of Wolff-Parkinson-White pattern ventricular pre-excitation (Wolff-Parkinson-White type pre-excitation) originating from conducting myocardial strands within or adjacent to mass [C10] [G810-09] [E2].

ECG

  • In normal sinus rhythm: ECG within physiological limits or with mild non-specific repolarization abnormalities [E1].
  • In variant with pre-excitation (Wolff-Parkinson-White type pre-excitation): Short PR interval (< 120 ms), initial delta wave on QRS complex (delta wave), and QRS widening [C10] [G810-09] [E2].

Heart sounds and murmurs

  • Heart sounds: S1 and S2 normal [B1].
  • Systolic murmur: Soft low-intensity midsystolic ejection murmur (grade 1–2/6) at sternal border or aortic/pulmonary area in case of intracavitary septal protrusion [C10] [B1].

Arterial pulse

  • Pulse of normal physiological characteristics [B4].

Jugular venous pulse

  • Normal parameters [B3].

Maneuvers

  • Conventional physiological hemodynamic responses [B1].

Simulator severity grades

Simulator severity grades: Single grade
Baseline rhythm: Normal Sinus Rhythm

Also known as: Cardiac rhabdomyoma · Rhabdomyoma · Cardiac rhabdomyomatosis · Heart rhabdomyoma

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. [C10] Primary Cardiac Tumors: JACC: CardioOncology State-of-the-Art Review . JACC CardioOncol (2020) . DOI: 10.1016/j.jaccao.2020.05.009 PubMed: 34396236
  5. [E1] AHA/ACCF/HRS recommendations for the standardization and interpretation of the electrocardiogram: part V: electrocardiogram changes associated with cardiac chamber hypertrophy: a scientific statement from the American Heart Association Electrocardiography and Arrhythmias Committee, Council on Clinical Cardiology; the American College of Cardiology Foundation; and the Heart Rhythm Society: endorsed by the International Society for Computerized Electrocardiology . Circulation (2009) . DOI: 10.1161/CIRCULATIONAHA.108.191097 PubMed: 19228820
  6. [E2] AHA/ACCF/HRS recommendations for the standardization and interpretation of the electrocardiogram: part III: intraventricular conduction disturbances: a scientific statement from the American Heart Association Electrocardiography and Arrhythmias Committee, Council on Clinical Cardiology; the American College of Cardiology Foundation; and the Heart Rhythm Society: endorsed by the International Society for Computerized Electrocardiology . Circulation (2009) . DOI: 10.1161/CIRCULATIONAHA.108.191095 PubMed: 19228822
  7. [G810-09] Surgery for primary cardiac tumors in children: early and late results in a multicenter European Congenital Heart Surgeons Association study . Circulation (2012) . DOI: 10.1161/CIRCULATIONAHA.111.037226 PubMed: 22626745