Uhl anomaly

Uhl anomaly is an unusual cardiac disorder which affects the right ventricle where there is an almost complete absence of right ventricular myocardium, normal tricuspid valve, and preserved septal and left ventricular myocardium.

Presentation usually occurs in the neonatal and childhood years with clinical aspects of congestive heart failure. The presentation may vary from arrhythmia induced syncope or sudden cardiac death to a syndrome consistent with isolated right-sided heart failure, including 5:

  • hepatomegaly
  • jugular venous distension
  • dyspnea
  • cyanosis
  • sinus rhythm
    • multi-component epsilon waves 6
      • present throughout the limb leads and precordium
      • sharp deflection at the J point (the junction between the QRS complex and the ST segment) 11
      • initial deflection followed by a variable number of oscillations
    • right atrial enlargement
  • arrhythmias
    • monomorphic ventricular tachycardia
      • precordial leads demonstrate a morphology similar to a left bundle branch block (LBBB)
      • the frontal plane QRS axis will be deviated in a superior direction

Non-specific features suggestive of Uhl's anomaly may be detected on a fetal echocardiography examination, including marked dilation of the right ventricle, with reduced wall thickness.

In postnatal life, supportive findings on transthoracic echocardiography include the following:

It is named after Henry S D Uhl, who first described the condition in 1952 3.

Imaging differential considerations include:

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Article information

rID: 24882
System: Cardiac
Synonyms or Alternate Spellings:
  • Uhl's anomaly

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