Congenital heart disease (CHD) remains the most common type of major congenital
malformation and the leading cause of mortality from birth defects [1-4]. Advances in
effective treatment for these lesions have significantly extended the lifespan of
affected patients, especially for the most complex subtypes of disease. However, these
patients are at higher risk of heart failure (HF) secondary to longer life expectancy.
This includes patients with a systemic right ventricle and a single ventricle circulation
palliated by a Fontan procedure [5, 6]. HF has been documented in up to 30% of patients
with a systemic right ventricle and 40% of patients who have had a Fontan procedure [7].
Ventricular assist devices (VAD) are implanted in patients with HF to improve cardiac
output and prolong life. VAD remains underutilized in patients with CHD and HF in part
due to the highly variable anatomy in this population. This is true despite outcomes
having been shown to be the same for VAD placement in patients with and without CHD
[8-10]. In the absence of VAD placement, however, wait list mortality for patients with
CHD is higher than for those patients without CHD [11, 12].
Advances in imaging techniques have allowed early diagnosis of CHD as well as anatomic
assessment prior to surgical procedures. Given the significant yet often subtle anatomic
differences between CHD patients, it is a substantial challenge to thoroughly depict all
of the components of a complex patient's cardiac anatomy in a two-dimensional imaging
dataset. An innovative technology that is being used with more enthusiasm in the medical
field, is three-dimensional (3D) printing. The investigator and the research team have
previously reported on the best technique that should be used to create 3D printed
cardiac models from MRI and the subtypes of complex CHD's for which 3D printing should be
utilized [13-16]. 3D printing allows creation of patient specific physical anatomic
models from a patient's own imaging data. These models provide a physical guide to
patient-specific anatomic features that often make VAD and cannula placement challenging
in patients with CHD [17]. Factors such as complex cardiac anatomic malformations, heavy
trabeculations or a severely dilated ventricle can distort the usual anatomic landmarks
used to identify the best position for cannula placement. The primary goal is to
establish the utility of this advanced imaging technique, which provides a much more
comprehensive understanding of complex congenital cardiac anatomy. The investigator
hypothesizes that 3D printed models will allow more informed preoperative planning with a
clearer understanding of the best site for inflow and outflow cannula and VAD placement
leading to better surgical preparedness, less operating room time and improved patient
outcomes.
AIM 1: To assess if a 3D printed cardiac model improves perceived visualization of VAD
and cannula placement sites in CHD-HF patients as compared to 2D imaging. The study will
prospectively enroll CHD-HF patients at multiple centers and randomize to Group A (3D
printed models will be used for pre-VAD planning) or Group B (no model-controls). For
both Groups, all of the cardiothoracic surgeons at the participating center will complete
a questionnaire after reviewing 2D imaging data. For Group A, a survey will also be
administered after reviewing a patient specific 3D model. The primary outcome measure
will be better perceived visualization of cannula and VAD sites. The investigator
hypothesizes that the 3D model will more clearly demonstrate sites of cannula and VAD
placement as compared to 2D imaging.
AIM 2: To determine if perioperative factors and outcomes improve in CHD-HF patients with
use of a 3D printed model versus traditional imaging in VAD placement planning. Clinical
characteristics will be collected at time of enrollment including primary diagnosis and
indication for VAD. After VAD placement, information regarding the intraoperative and
postoperative course will be collected including surgical cardiopulmonary bypass time
(CPB) and need for cannula repositioning. Longer CPB increases morbidity and mortality
and is associated with intensive care readmission in patients after LVAD placement
[18-20]. The primary measures of improvement will be CPB. The investigator hypothesizes
that the improved preoperative planning using 3D models will lead to a decrease in CPB
time.
The skill with which patient specific CHD anatomy for pre-procedural planning is assessed
must be improved, especially for the most complex patients. To confirm the clinical
benefit of 3D printed models in pre-surgical planning and justify their use in routine
care, multicenter clinical trials must be conducted. As an expert in the field of 3D
imaging in cardiac disease, the investigator is well poised to lead this body of
research. The goal is to become well versed in conducting high quality multicenter
studies and to become facile in survey tool design through this K23 proposal. The
investigator will then design a prospective multicenter study for an independent R01
proposal focused on assessing the utility of 3D models in pre-procedural planning for all
complex congenital heart diseases. Investigating and reporting on these findings will
result in a paradigm shift in what one considers "standard of care" for advanced imaging
offered to our most complex CHD patients.