Purpose of review

Cardiac magnetic resonance imaging provides radiation-free, 3-dimensional soft tissue visualization with adjunct hemodynamic data, making it a promising candidate for image-guided transcatheter interventions. This review focuses on the benefits and background of real-time magnetic resonance imaging (MRI)-guided cardiac catheterization, guidance on starting a clinical program, and recent research developments.

Recent findings

Interventional cardiac magnetic resonance (iCMR) has an established track record with the first entirely MRI-guided cardiac catheterization for congenital heart disease reported nearly 20 years ago. Since then, many centers have embarked upon clinical iCMR programs primarily performing diagnostic MRI-guided cardiac catheterization. There have also been limited reports of successful real-time MRI-guided transcatheter interventions. Growing experience in performing cardiac catheterization in the magnetic resonance environment has facilitated practical workflows appropriate for efficiency-focused cardiac catheterization laboratories. Most exciting developments in imaging technology, MRI-compatible equipment and MRI-guided novel transcatheter interventions have been limited to preclinical research. Many of these research developments are ready for clinical translation. With increasing iCMR clinical experience and translation of preclinical research innovations, the time to make the leap to radiation-free procedures is now.

Despite therapeutic advances over the past decades, pulmonary arterial hypertension (PAH) and related pulmonary vascular diseases continue to cause significant morbidity and mortality in neonates, infants, and children. Unfortunately, an adequate understanding of underlying biology is lacking. There has been a growing interest in the role that genetic factors influence pulmonary vascular disease, with the hope that genetic information may aid in identifying disease etiologies, guide therapeutic decisions, and ultimately identify novel therapeutic targets. In fact, current data suggest that genetic factors contribute to ~42% of pediatric-onset PH compared to ~12.5% of adult-onset PAH. We report a case in which the knowledge that biallelic ATP13A3 mutations are associated with malignant progression of PAH in young childhood, led us to alter our traditional treatment plan for a 21-month-old PAH patient. In this case, we elected to perform a historically high-risk Potts shunt before expected rapid deterioration. Short-term follow-up is encouraging, and the patient remains the only known surviving pediatric PAH patient with an associated biallelic ATP13A3 mutation in the literature. We speculate that an increased use of comprehensive genetic testing can aid in identifying the underlying pathobiology and the expected natural history, and guide treatment plans among PAH patients.