7. Congenital Heart Disease/Cyanotic Heart Disease

Question 7: In persons with congenital heart disease or associated conditions with possible requirements for oxygen therapy who are considering driving, what is the incremental risk of SCI posed by the presence of cyanosis, separate and apart from the restrictions required by the hemodynamic, structural, and electrical consequences of these conditions?

Congenital heart diseases comprise a variety of entities as well as clinical manifestations. These manifestations include HF, valvular problems, and rhythm disorders, many of which will require implantation of a device. Any driving restrictions on this patient population will therefore more likely be a result of these manifestations or complications. For this reason, the fitness to drive of these patients should be addressed by consulting the relevant sections of this document.

Cyanosis in patients with congenital heart disease presents a different challenge and is secondary to right to left shunting of unoxygenated blood, not poor alveolar oxygen tension. These patients have chronic oxygen desaturation and developed adaptations with a rightward shift of the oxygen-hemoglobin dissociation curve. This shift does not affect oxygen binding in the lungs but favours oxygen release at the tissue level.

Patients with cyanosis and Eisenmenger syndrome can require supplemental oxygen with advanced NYHA functional class (III and IV), but there are no specific evidence-based analyses regarding their fitness to drive. Existing data for supplemental oxygen exists in the field of chronic obstructive pulmonary disease and hypoxia, but this is of limited applicability in the congenital heart disease population.

As such, there are limited restrictions to driving, and each patient should be assessed according to the related comorbidities and complications related to their underlying condition (Table 11).