Cardiovascular Magnetic Resonance Imaging has evolved during the last 2 decades as the reference standard imaging modality to assess the anatomic and functional sequelae in patients with repaired ToF.
Tetralogy of Fallot is the most common type of cyanotic congenital heart disease.
It consists of a right ventricle (RV) outflow tract obstruction,
a malalignment ventricular septal defect,
an overriding aorta,
and right ventricular hypertrophy.
In addition,
ToF is associated with other congenital cardiac abnormalities in about 40% of patients.
Some of the more common anomalies are a right sided aortic arch (13–34%),
atrial septal defect (~15%),
atrioventricular septal defect (1.7–7%),
anomalous coronary arteries (most commonly a prominent conus artery or the left anterior descending artery from the right coronary artery or right sinus of Valsalva).
The history of surgical treatment of ToF is closely linked with the origins of cardiac surgery.
Before the era of cardiac surgery,
most ToF patients died in childhood; While early surgical mortality decreased from 50% in the late 1950’s to less than 2% in the modern surgical era,
residual anatomic and hemodynamic abnormalities are nearly universal.
As a result,
the number of patients with repaired TOF,
many of whom with considerable cardiac and non-cardiac disease burden,
is growing rapidly.
Right ventricular (RV) dilatation from pulmonary regurgitation (PR),
residual atrial and/or ventricular septal defect,
tricuspid regurgitation,
right ventricular outflow tract (RVOT) aneurysm,
pulmonary artery stenosis,
and tachyarrhythmias are some of the abnormalities frequently encountered in patients with repaired TOF.
Cardiac myxoma is a benign neoplasm arising within the heart.
Most frequently affect adults with a slight predominance in females.
The mean age at diagnosis is 50,
and 90% of patients are aged 30-60 years.
It is sometimes referred to as an atrial myxoma,
since the vast majority arise within the atrial septum.
5% of these tumors are located in the left or right ventricle.
For many years,
there was a debate on the origin of this tumor.
Some thought it represented an organizing thrombus.
Today,
it has clearly been shown that this is a true neoplasm.
Cardiovascular MRI is an extremely useful technique to follow up patients with repaired ToF,
independent of the acoustic window,
not associated with exposure to ionizing radiation that in many centers has become the preferred method of noninvasive imaging in patients with repaired ToF.
Delayed gadolinium enhanced sequences are extremely helpful for the diagnosis of tumors and differential diagnosis between thrombi.
We report a first (to our knowledge) case of Myxoma in RV in this category of patients with a very atypical location in the apical part.We describe the MR and US findings