Randy Ray Richardson

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

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.

Ivemark syndrome: bronchial compression from anomalous pulmonary venous anatomy

Abstract Ivemark syndrome is a heterotaxy syndrome which affects multiple organs and affects roughly 1 in every 6000 deliveries. Specifically, it can cause total anomalous pulmonary venous return and cardiac defects, which ultimately lead to decreased life expectancy. In order to better understand the nature of cardiac structures, CT angiogram has been heavily relied upon as it also allows for 3D reconstruction and optimal visualization of those features. This specific case presents with an anomalous venous return accompanied by multi-organ right isomerism that was reconstructed with 3D CT angiogram to better visualize and understand the cardiopulmonary system, as well as contribute to a fund of knowledge in hopes of discovering a solution to this condition.

Operations Performed for Patients with Congenital Heart Disease

When dealing with congenital heart disease, it is vital to understand the basic terminology regarding postoperative shunts, procedures, and surgeries.

Scanning Technique for Cardiac CTA in Infants and Small Children

To fully utilize the advantages of cardiac CTA, it is important to consider radiation exposure and to optimize scanning techniques. Recent advances in multidetector CT (MDCT) technology have revolutionized cardiovascular imaging in children with complex congenital heart disease. For infants with congenital heart disease, ECG-gated cardiac CTA is the modality of choice for imaging the coronary arteries, airway, and extracardiac vascular structures. Fast scanning times and high-quality evaluation of both complex cardiac and coronary anatomy have enabled CTA to aid in patient management and treatment planning. Currently, there are two accepted cardiac CTA scanning techniques for infants with congenital heart disease: retrospective and prospective ECG-gated scanning.

Evaluation of the Great Vessels

R.R. Richardson, Atlas of Pediatric Cardiac CTA, DOI 10.1007/978-1-4614-0088-2_7,

Evaluation of the Atria, Atrioventricular Valves, and Veins

One should begin cardiac CT evaluation by assessing the atria, the systemic and pulmonary veins draining to them, and the atrioventricular (AV) valves. Examine the following:

Advantages of Cardiac CTA over Other Imaging Modalities

Congenital heart imaging has changed dramatically over the past several decades. In previous decades, plain x-ray films were a key diagnostic test, with the use of angiocardiography to make specific preoperative diagnoses. However, with several robust cross-sectional imaging modalities now available, this method no longer is the standard. Although plain films still are often obtained, they serve as more of a screening tool, with the first line of imaging being echocardiography. Echocardiography typically does not require sedation and does not expose the patient to ionizing radiation. Echocardiography provides detailed intracardiac anatomy with real-time functional evaluation; however, it may be limited for evaluating extracardiac structure. Similarly, cardiac MRI does not expose the patient to ionizing radiation and offers some of the best tools for functional evaluation of the heart. Cardiac CT angiography (CTA) is another cross-sectional imaging modality that may be used for anatomic and functional evaluation in patients with congenital heart disease. Cardiac CTA does require ionizing radiation; however, with new techniques designed to minimize the patient’s exposure, the radiation can be kept within safe parameters and the advantages of CT may be used.

Advanced Postprocessing of Cardiac CTA

Commercially available workstations have software packages to provide volumetric analysis for cardiac CTA. In children, it is best to obtain the data prospectively to reduce radiation exposure. The rapid heart rate of infants results in data and phases of a larger portion of the cardiac cycle than in adults with the same amount of padding. End-systolic and end-diastolic volumes are obtained easily for both right and left ventricles. The typical part of the phase to obtain the end-diastolic data is at 85–90 % of the cardiac cycle. The typical part of the phase to obtain the end-systolic data is at 45–55 % of the cardiac cycle. Once volumes have been determined for end systole and end diastole, the ejection fraction and stroke volume can be calculated and provided as part of the report. It is helpful to divide the end-diastolic and end-systolic volumes by body surface area to provide indexed volumes. Indexed volumes are obtained by dividing the gross end-diastolic and end-systolic volume of the right and left ventricles by the body surface area (Figs. 3.1, 3.2 and Table 3.1).