Effect of main pulmonary artery dilation and stiffness on right ventricular volume overload and function in patients with repaired tetralogy of Fallot

Survivors of Tetralogy of Fallot (TOF) constitute a large and growing population of patients. Although post-surgical outcome is generally favorable, as these patients move into adulthood, late morbidity is becoming more prevalent. Recent evidence suggests that adverse long-term post-surgical outcome is related to chronic pulmonary regurgitation (PR) and RV dilation. Repeat surgery with pulmonary valve replacement (PVR) may be necessary to preempt RV functional deterioration and malignant ventricular arrhythmias. However, optimal time of PVR is still debatable. We speculate that main pulmonary artery area and stiffness are associated with RV dilation and impaired function and might contribute to the referral of PVR.

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