Isolated right ventricular noncompaction caused recurrent pulmonary embolism

Ventricular noncompaction (VNC) is an unusual cause of cardiomyopathy. The most common site of involvement is the left ventricle, with isolated right ventricular noncompaction (iRVNC) involvement being reported in a few cases. No iRVNC caused pulmonary embolism (PE) has been reported so far. In this report, we present one case of iRVNC caused PE. The patient was admitted in my hospital due to aggravated chest tightness after activities, accompanied by breathlessness, repeated amaurosis, and syncope. Coronary and left ventricular angiography revealed no abnormality. Pulmonary angiography suggested bilateral PE and pulmonary arterial hypertension, and noncompaction of right ventricular myocardium was ocationally found in main pulmonary artery angiography, which was confirmed by right ventricular angiography and echocardiography and was diagnosed as iRVNC. Since no thrombus was found in venography in both lower limb varices, it was thought that the PE was caused by situ embolus of the right ventricle. This patient was applied thrombolysis, anticoagulation, and ventricular remodeling improvement, and appeared improved conditions. The very rare case warns us when PE occurs while without significant risk factors or lower extremity deep venous thrombosis (DVT), iRVNC should be considered.