Discussion:
In this case report, we have demonstrated successful cardiac
resynchronisation using His-bundle pacing in a patient with Scimitar
syndrome with subsequent improvement in the patient’s heart failure
symptoms. Utilization of integrated image modalities using CARTOSEG™ and
CARTOUNIVU™ software helped us to achieve successful cardiac
resynchronization despite the difficult anatomy with respect to the
cardiac chambers as well as the CS tributaries. To the best of our
knowledge, our case is the first description of His-CRT implantation in
Scimitar syndrome with image integration tools.
Image integration using three-dimensional electro-anatomical mapping
systems was crucial for understanding the Scimitar syndrome complex
cardiac anatomy given the right lung hypoplasia and right sided
dextroposition of the heart1 , and in advancing the
lead to the key cardiac landmarks. In our patient, L-transposition of
great arteries (L-TGA) and left sided valvular abnormalities were
specifically ruled out.
The use of CARTOSEG™ and CARTOUNIVU™ image integration modules has
previously been shown to reduce fluoroscopy time and procedural
complications.2 In our case, planning with CARTOSEG™
allowed orientation to the inter-ventricular septum in unconventional
fluoroscopic views. Predefining and integrating the location of the
His-bundle potentially reduced the procedural time.
Cardiac resynchronization with His-bundle pacing (His-CRT) is evolving
rapidly as a viable strategy and as an alternative to the LV lead
implanted through the CS (conventional CRT).3, 4 This
has added a new dimension to patients where placement of an LV lead may
be technically challenging as in our case. It is possible to correct the
focal and proximal LBBB at the level of the His conduction system by
pacing distal to the block as shown in our case.
In congenital heart diseases, the perceived difficulties of His-bundle
pacing are related either to the cardiac malrotation as observed in our
case or due to varied positions of the AV node conduction axis.
Nevertheless, His-bundle pacing for complete heart block has been
achieved in congenital anomalous hearts like in persistent left superior
vena cava (LSVC) 5, dextrocardia 6,
Ebstein’s anomaly 7 and in L-TGA 8.
In a recent case series of patients with intraventricular conduction
defects and L-TGA, His-bundle pacing was preferred over conventional CRT
given the CS may be impossible to reach without compromise to procedural
success rates and subsequent functional improvements.9As the technology evolves, we will expect successful His-bundle pacing
in all ranges of congenital heart diseases. The extent and duration of
the desired reverse remodelling of the LV remains to be seen in our
case.