Investigations
The ECG will show right atrial enlargement with “Himalayan” P waves, prolonged PR interval and right bundle branch block. There may be low volume QRS complexes, pre-excitation in the form of delta waves and arrhythmias including atrial fibrillation and flutter may be present.
The diagnosis can be confirmed with echocardiography showing apical displacement of the septal tricuspid leaflet. The degree of displacement affects the severity of the disease. Other findings include tricuspid regurgitation, right atrium and ventricular dilatation and associated structural defects such as PFO, ASD and VSD.
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