Stephen Pophal

A novel approach to neonatal management of tetralogy of Fallot, with pulmonary atresia, and multiple aortopulmonary collaterals.

Tetralogy of Fallot (TOF), pulmonary atresia (PA), and multiple aortopulmonary collateral arteries (MAPCAs) need complex interventions, and pre-natal diagnosis allows for appropriate peri-partum planning [(1)][1]. Traditionally the post-natal echocardiogram is followed by cardiac catheterization to

Fontan-associated protein-losing enteropathy and heart transplant: A Pediatric Heart Transplant Study analysis

BACKGROUND Post-Fontan protein-losing enteropathy (PLE) is associated with significant morbidity and mortality. Although heart transplantation (HTx) can be curative, PLE may increase the risk of morbidity before and after HTx. This study analyzed the influence of PLE influence on waiting list and post-HTx outcomes in a pediatric cohort. METHODS Fontan patients listed for HTx and enrolled in the Pediatric Heart Transplant Study from 1999 to 2012 were stratified by a diagnosis of PLE, and the association of PLE with waiting list and post-HTx mortality, rejection, and infection was analyzed. RESULTS Compared with non-PLE Fontan patients (n = 260), PLE patients listed for HTx (n = 96) were older (11.9 years vs 7.6 years; p = 0.003), had a larger body surface area (1.1 m(2) vs 0.9 m(2); p = 0.0001), had lower serum bilirubin (0.5 vs 0.9 mg/dl; p = 0.01), lower B-type natriuretic peptide (59 vs 227 pg/ml; p = 0.006), and were less likely to be on a ventilator (3% vs 13%; p = 0.006). PLE patients had lower waiting list mortality than non-PLE Fontan patients (p < 0.0001). There were no intergroup differences for post-HTx survival or times to the first infection or rejection. PLE was not independently associated with increased post-HTx mortality at any time point. CONCLUSIONS In this multicenter cohort, the diagnosis of PLE alone was not associated with increased waiting list mortality or post-HTx morbidity or mortality. Given the limitations of our data, this analysis suggests that PLE patients in the pediatric age group have outcomes similar to their non-PLE counterparts. Additional multicenter studies of PLE patients with targeted collection of PLE-specific information will be necessary to fully delineate the risks conferred by PLE for HTx.

Total artificial heart in the pediatric patient with biventricular heart failure.

Mechanical circulatory support emerged for the pediatric population in the late 1980s as a bridge to cardiac transplantation. The Total Artificial Heart (TAH-t) (SynCardia Systems Inc., Tuscon, AZ) has been approved for compassionate use by the Food and Drug Administration for patients with end-stage biventricular heart failure as a bridge to heart transplantation since 1985 and has had FDA approval since 2004. However, of the 1,061 patients placed on the TAH-t, only 21 (2%) were under the age 18. SynCardia Systems, Inc. recommends a minimum patient body surface area (BSA) of 1.7 m2, thus, limiting pediatric application of this device. This unique case report shares this pediatric institution’s first experience with the TAH-t. A 14-year-old male was admitted with dilated cardiomyopathy and severe biventricular heart failure. The patient rapidly decompensated, requiring extracorporeal life support. An echocardiogram revealed severe biventricular dysfunction and diffuse clot formation in the left ventricle and outflow tract. The decision was made to transition to biventricular assist device. The biventricular failure and clot formation helped guide the team to the TAH-t, in spite of a BSA (1.5 m2) below the recommendation of 1.7m2. A computed tomography (CT) scan of the thorax, in conjunction with a novel three-dimensional (3D) modeling system and team, assisted in determining appropriate fit. Chest CT and 3D modeling following implantation were utilized to determine all major vascular structures were unobstructed and the bronchi were open. The virtual 3D model confirmed appropriate device fit with no evidence of compression to the left pulmonary veins. The postoperative course was complicated by a left lung opacification. The left lung anomalies proved to be atelectasis and improved with aggressive recruitment maneuvers. The patient was supported for 11 days prior to transplantation. Chest CT and 3D modeling were crucial in assessing whether the device would fit, as well as postoperative complications in this smaller pediatric patient.

Color-coded patient-specific physical models of congenital heart disease

Purpose – The purpose of this study was to develop and apply new physical heart defect models (PHDMs) that are patient-specific and color-coded with an optimized map. Design/methodology/approach – Heart defect anatomies were segmented from medical images and reconstructed to form virtual models, which were then color-coded and rapid prototyped. The resulting PHDMs were used in a medical educational study to evaluate their pedagogical efficacy and in clinical case studies to investigate their utility in surgical planning. Findings – A growing library of 36 PHDMs (including the most common defects) was generated. Results from the educational study showed that the PHDMs enabled uniquely effective learning, and the clinical case studies indicated that the models added value as surgical planning aids. Research limitations/implications – The education study involved a limited number of students, so future work should consider a larger sample size. The clinical case studies favored use of the PHDMs in surgical p...

Left Hemitruncus Associated with Tetralogy of Fallot: Fetal Diagnosis and Postnatal Echocardiographic and Cardiac Computed Tomographic Confirmation

Anomalous origin of one pulmonary artery from the aorta, or hemitruncus, is a rare cardiac malformation. We report a case of left hemitruncus (aortic origin of the left pulmonary artery) associated with tetralogy of Fallot diagnosed in utero. To the authors’ knowledge, this is the first such case diagnosed by fetal echocardiography to be described in the literature. The condition was documented by postnatal echocardiogram and cardiac computed tomography. Prompt recognition of this lesion is essential for early repair to improve outcome.

The failing Fontan: what's NEXT…?

The Fontan procedure represents the final stage of the transition to single ventricle physiology. Conversion of very complex congenital heart anatomy, such as hypoplastic left heart syndrome, double-outlet right ventricle or double-inlet left ventricle, to a single ventricle has grown in popularity as morbidity and mortality have improved. As these patients grow, survivors are at risk for impaired ventricular dysfunction, plastic bronchitis, protein-losing enteropathy and late failure. Late failing Fontan patients represent a particularly vexing scenario for clinicians, as the only durable treatment option is cardiac transplantation. However, in the short-term, some of these patients require support beyond medical management, with mechanical circulatory support via extracorporeal life support or a ventricular assist device. We report the successful bridge of an adolescent female post-Fontan conversion with late severe cardiac failure. The patient was initially resuscitated with extracorporeal life support, transitioned to a single Berlin Heart EXCOR® ventricular assist device and, subsequently, underwent successful cardiac transplantation.

Three-dimensional printing: changing clinical care or just a passing fad?

Purpose of review Advances in medical imaging and three-dimensional (3D) reconstruction software have enabled a proliferation of 3D modeling and 3D printing for clinical applications. In particular, 3D printing has garnered an extraordinary media presence over the past few years. There is growing optimism that 3D printing can address patient specificity and complexity for improved interventional and surgical planning. Will this relatively untested technology bring about a paradigm shift in the clinical environment, or is it just a transient fad? Recent findings Case studies and series centered around 3D printing are omnipresent in clinical and engineering journals. These primarily qualitative studies support the potential efficacy of the emerging technology. Few studies analyze the value of 3D printing, weighing its potential benefits against increasing costs (e.g., institutional overhead, labor, and materials). Summary Clinical integration of 3D printing is growing rapidly, and its adoption into clinical practice presents unique workflow challenges. There are numerous clinical trials on the horizon that will finally help to elucidate the measured impact of 3D printing on clinical outcomes through quantitative analyses of clinical and economic metrics. The contrived integration of 3D printing into clinical practice seems all but certain as the value of this technology becomes more and more evident.

Alternative methods for virtual heart transplant-Size matching for pediatric heart transplantation with and without donor medical images available

Listed pediatric heart transplant patients have the highest solid‐organ waitlist mortality rate. The donor‐recipient body weight (DRBW) ratio is the clinical standard for allograft size matching but may unnecessarily limit a patients donor pool. To overcome DRBW ratio limitations, two methods of performing virtual heart transplant fit assessments were developed that account for patient‐specific nuances. Method 1 uses an allograft total cardiac volume (TCV) prediction model informed by patient data wherein a matched allograft 3‐D reconstruction is selected from a virtual library for assessment. Method 2 uses donor images for a direct virtual transplant assessment.

Tetralogy of Fallot with Major Aortopulmonary Collateral Arteries

Tetralogy of Fallot (TOF) with major aortopulmonary collateral arteries (MAPCAs) is one of the most severe forms of TOF, creating significant challenges for surgeons and interventionalists due to complex, patient-specific morphology. Furthermore, MAPCAs are highly variable, with morphology differing in number, tortuosity, stenosis, and branching locations. Echocardiography and catheter-based evaluation are typically performed preoperatively. These imaging modalities have limitations, however, in representing volumetric data. Three-dimensional (3D) visualization and 3D printing from computed tomography (CT) or magnetic resonance (MR) angiography can provide a unique and powerful mechanism to aid in the evaluation and planning of surgical or interventional repair of TOF with MAPCAs. The various current 3D printing technologies all have strengths and limitations in regard to representing patient-specific morphology, especially as it relates to the TOF/MAPCA congenital lesion subset. 3D reconstruction and 3D printing technologies and techniques are discussed to benefit evaluation and planning for the repair of TOF/MAPCA.

Integration of Hybrid and Single Ventricle Rehabilitation Techniques to Treat a Neonate After Iatrogenic Mitral Injury.

Neonatal critical aortic stenosis is life threatening and requires intervention shortly after birth. Early percutaneous aortic valvuloplasty is generally considered to be an effective way to obtain reliable prograde flow. We describe the integrated use of hybrid, open surgical, and single ventricle rehabilitation techniques to successfully treat a neonate with borderline left-sided cardiac structures, who sustained a complication from balloon aortic valvuloplasty.