Pink tetralogy of Fallot

Case contributed by Chris Newman

Presentation

Hypoxic and polycythemic. ? Pulmonary embolism

Patient Data

Age: 35 years
Gender: Male

Chest x-ray

x_ray
  • slightly lobulated cardiac silhouette with mild transverse cardiomegaly but no definite boot-shape
  • symmetric lung appearance
ct
  • no pulmonary embolism
  • multiple features of tetralogy of Fallot including ventricular septal defect, overriding aorta, right ventricular outflow obstruction (minimal), and right ventricular hypertrophy
  • the lungs and pleural spaces are clear

Case Discussion

Uncorrected adult tetralogy of Fallot is rare. 

This patient had periods of high oxygen demand in the emergency requirement and was noted to have polycythemia as well as a heart murmur. 

Pink Tetralogy of Fallot occurs because the degree of right ventricular outflow obstruction is minimal and does not result in a right to left shunt (therefore acyanotic). 

The chronic hypoxia causes the acquired / secondary polycythemia.

The patient underwent an outpatient correctional procedure for the cardiac anomaly including a pulmonary valve replacement, patch augmentation of the main pulmonary artery and right ventricular outflow tract.

Case with thanks to Corey Thompson.

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