Isolated right ventricular noncompaction caused ventricular tachycardia and pulmonary embolism

Abstract

Isolated ventricular noncompaction is an unclassified cardiomyopathy due to intrauterine arrest of compaction of the loose interwoven meshwork. Its mortality and morbidity are high, including heart failure, thromboembolic events, and ventricular arrhythmias. Isolated right ventricular noncompaction was reported rarely, especially that causes pulmonary embolism and ventricular tachycardia. We describe a case of isolated noncompaction of the right ventricular causing pulmonary embolism and ventricular tachycardia.

A case report of isolated right ventricular noncompaction with mutation of ACVRL1: a new cause of noncompaction of ventricular myocardium?

“…NVM could be associated with congenital heart disease, other primary cardiomyopathy or metabolic diseases. Most cases of iRVNC reported exist independently or combined with congenital heart disease [ 13 – 15 ]. And results of this case, for example, cardiac imaging, thyroid function and autoantibodies could exclude congenital heart disease, other primary cardiomyopathy and metabolic diseases.…”

“…Cardiac imaging suggested enlargement of right heart and moderate-severe tricuspid regurgitation, so iRVNC combined with HHT2 and pulmonary hypertension was confirmed. iRVNC can show complications such as right heart insufficiency, arrhythmia and pulmonary embolism [ 5 , 15 , 16 ], and pulmonary hypertension can be found in secondary pulmonary embolism [ 15 , 16 ]. But the child's oxygen saturation was normal, pneumonia could be utilized to explain dyspnea, D-dimer and fibrinogen are within the normal range.…”