Final Pr. ID: Poster #: CR-001
Limited experience with 3D print technology has been reported for surgical planning in infants with congenital heart disease. We present 5 infants with complex intracardiac anatomy for whom 3D cardiac models from gated cardiac CT angiograms facilitated surgical planning.
Case 1: Term 2-day-old infant with d-transposition of the great arteries (TGA), large anterior malalignment ventricular septal defect (VSD), and severe aortic arch hypoplasia. The 3D cardiac model confirmed feasibility of biventricular repair using a Yasui-type operation, with Norwood arch reconstruction, Rastelli VSD closure, and right ventricle to pulmonary artery (RV-PA) conduit placement.
Case 2: 2-month old, ex-34-week premature infant with double-outlet right ventricle (DORV), side-by-side great arteries, large remote predominantly-subaortic VSD, and pulmonary stenosis (PS). The 3D cardiac model highlighted the remoteness of the VSD from the outflow tracts, prompting right ventricular outflow tract patch augmentation without VSD closure when the patient developed hypercyanotic spells.
Case 3: Term 7-day-old infant with Goldenhar syndrome, D-TGA, large posterior malalignment VSD with inlet extension, and PS. The first 3D cardiac model raised concern that VSD closure would be difficult as a newborn due to VSD size, prompting placement of a BT shunt and pulmonary artery band. The second 3D model at 11 months of age demonstrated feasibility of biventricular repair with Rastelli VSD closure and RV-PA conduit placement.
Case 4: Term 2-day-old infant with 22q11.2 deletion syndrome, type B interruption of a right aortic arch, aberrant left subclavian artery, posterior malalignment VSD, small bicuspid aortic valve, and superior-inferior branch pulmonary artery relationships. The 3D cardiac model demonstrated need for VSD enlargement to enable complete repair with Yasui operation with LeCompte maneuver.
Case 5: Term 3-week-old infant with mesocardia, ventricular inversion, DORV, L-malposed great arteries, subpulmonic VSD, multilevel PS, and mitral chordal attachments to the crest of the ventricular septum. The 3D cardiac model suggested that VSD enlargement might enable a double switch operation with Rastelli VSD baffle. However, during the operation at 20 months old, mitral chordal apparatus prevented VSD enlargement, necessitating bidirectional Glenn instead. Read More
Final Pr. ID: Paper #: 040
4D flow MRI with full coverage of the heart and proximal vessels is a powerful tool to understand hemodynamic pathology arising from congenital heart disease (CHD) and subsequent surgery; however, scan times often exceed 10 minutes thereby limiting widespread adoption in pediatrics. Decreasing scan time using compressed-SENSE (CS), a combination of conventional parallel imaging (SENSE) and randomized spatial undersampling, shows promise over SENSE alone. However, both the feasibility and its effect on accurate flow quantification in pediatrics has not been evaluated. This study investigated the feasibility of CS for pediatric 4D flow MRI to quantify flow indices used in risk stratification of CHD such as coarctation or pulmonary regurgitation, in comparison with SENSE. Read More
Final Pr. ID: Poster #: EDU-090
The structure of the heart is more complex than 4 major chambers, 4 major valves, venous inflow and arterial outflow.
Muscular and soft tissue ridges, perforated membranes, tissue flaps, electrical pathways, external grooves, endocardial surface characteristics, and a core architecture structured of fibrous tissue and embryonic tissue remnants form distinguishing anatomic landmarks. These structures provide insight into cardiac development, serve physiologic purpose, define structural and functional elements, as well as offer prognostic information.
Specific details of cardiac anatomy may not be recognized on cardiac MR and CT examinations. Structural anomalies that affect cardiac function and anatomic or physiologic relationships may be abnormal in hearts with congenital malformations. The fine structural details of the heart are typically not part of an imagers search pattern and are therefore overlooked or underreported. Each minute structure and anatomic characteristic provides clues to errors in cardiac formation and may provide clinically relevant diagnostic information. After palliation of CHD defining anatomic structure may be lost, displaced, interrupted or even restored.
Important processes, corresponding anatomic landmarks, and effects of malformation in defining congenital heart disease anatomy, physiology and functional variation from normal include basal structures such as the central fibrous body of the heart, atrial structures including the crista terminalis, the sinus venarum, and eustacian valve, and ventricular structures such as the crista supraventricularis.
Each structure is described along with its normal location, anatomy functional and physiologic importance, the effect of congenital malformation, and imaging findings as in the example of the central fibrous body (table 1).
Minute cardiac anatomy will be shown in illustration as well as in CT and MR images (figure 1). Read More
Final Pr. ID: Poster #: CR-049
To describe the radiographic appearance of subclinical calcified brown fat necrosis and to delineate the associated clinical and laboratory findings. While brown fat necrosis has been described in infants with underlying cardiac disease treated with prostaglandins, we emphasize hypotension from cardiac or respiratory arrest as a primary risk factor. Read More
Final Pr. ID: Poster #: EDU-002
Characteristic chest radiographic findings of patients with congenital heart disease have been reported for some decades ago. For beginner, it may be hard to detect these findings and to understand reasons why these findings depict. Recently, radiologists can interpret specific findings of congenital heart disease because technique of CT have been developed. Read More
Final Pr. ID: Poster #: SCI-086
Tracheobronchial anomalies including tracheobronchial stenosis, tracheal bronchus, cardiac bronchus and bronchial isomerism are common in congenital heart disease. Cardiovascular anomaly is the principal extrinsic lesion causing tracheobronchial stenosis. MRI has the advantage of being non-ionizing and providing excellent soft tissue contrast for the diagnosis of congenital heart disease and tracheobronchial anomalies
Purpose: To define diagnostic accuracy of three-dimensional turbo field echo (3D-TFE) to detect tracheobronchial anomalies in patients with congenital heart disease Read More
Final Pr. ID: Paper #: 011
Three dimensional magnetic resonance angiography (3DMRA) and two-dimensional phase contrast are commonly used for assessment of anatomy and flow in congenital heart disease. Time-resolved, three-dimensional phase contrast MRI (4D flow) provides complete volumetric coverage of the chest with flow assessment in any plane. How the addition of ferumoxytol (Fe), a blood pool contrast, changes 4D flow magnitude image quality relative to extracellular gadolinium-based contrast agents (GB) and non-contrast (NC) exams is unknown. We compare image quality measurements in Fe, GB and NC 4D flow with ECG-gated, respiratory-navigated 3DMRA. Read More
Final Pr. ID: Paper #: 100
Purpose is to describe how FCMR is instrumental in diagnosis of congenital heart disease (CHD) and to present imaging findings and correlation in 50 fetuses with CHD. Read More
Keywords: fetal cardiac MRI, congenital heart disease
Final Pr. ID: Poster #: EDU-006
The purpose of this educational exhibit is to show FCMR images of congenital heart disease (CHD) and anatomical findings associated with CHD. We will describe currently available FCMR image acquisition techniques and use a case-based approach to show a variety of CHD diagnosis with teaching points to highlight the added value of FCMR in diagnosis and prognosis. Read More
Keywords: Fetal cardiac MRI, congenital heart disease
Final Pr. ID: Poster #: EDU-010
1. Review the safety, limitations and advantages of fetal cardiac MR (CMR)
2. Discuss potentialindications of fetal CMR
3. Describe the technique of fetal CMR, including imaging sequences, imaging planes, the method
of overcoming motion artifact
4. Illustrate the appearance of normal fetal cardiac structures and present a modified anatomic
segmental approach of congenital heart disease (CHD) at prenatal CMR
5. Demonstrate examples of fetal CMR in the evaluation of various cardiovascular anomalies Read More
Authors: Dong Su-zhen
Final Pr. ID: Poster #: SCI-069
Computed Tomography Angiography (CTA) is a valuable non-invasive tool for assessment of congenital heart disease in neonates. Though it requires less radiation, contrast and sedation than catheter angiography, which is the current standard of care, traditional CTA can still necessitate significant radiation dose, sedation, breath-holding and/or low heart-rates to achieve diagnostic studies. In this frequently imaged neonatal patient population, it is imperative to minimize cumulative radiation to limit the lifetime risk of cancer. At our institution, we have successfully used a new fast-acquisition FLASH CTA protocol to achieve high quality imaging of neonates with complex congenital heart disease with minimal radiation dose and without sedation. Read More
Final Pr. ID: Poster #: EDU-004
CHD (congenital heart disease) occurs in approximately 1% of all live births with more than 90% survival into adulthood. Prevalence of coronary artery disease has been reported to be similar to general adult population. Patients with complex CHD may be subjected to non-atherosclerotic premature coronary artery disease due to anomalous coronaries, peri-coronary region interventions, or coronary re-implantation. Cardiac MRI (CMR) may provide valuable myocardium health information with pointers towards a coronary distribution in unsuspected chronic or acute cases. Acquisition of delayed enhancement sequences (LGE) and T1 mapping should be considered routine in CMR studies for assessment of interval ischemic events. Patients with complex CHD are also at risk for sudden cardiac arrest and LGE data may assist in further risk stratification of these patients. Three complex CHD patients who all suffered myocardial infarctions at a young age as a result of their multifaceted cardiac history are highlighted to illustrate the importance of this (see included table, Figure 1). Two CMR images (Figures 2,3) demonstrate delayed enhancement throughout the septum at the base to mid-cavity in a patient with truncus arteriosus type 1 who presented with a non-ST-elevation myocardial infarction (NSTEMI). Focal area of low-signal sub-endocaridum within the enhancement on Figure 3 favors microvascular obstruction.
CMR is a vital component of surgical planning and post-operative care of patients with CHD, providing accurate anatomical, functional, and flow information that assists in clinical management. Delayed enhancement sequences and post-gadolinium T1 mapping allow assessment of ischemic injury or infarct, and therefore should be considered part of routine follow up CMR studies in patients with complex CHD. Read More
Final Pr. ID: Poster #: EDU-003
The ductus arteriosus is normal structure of fetal circulation derived from the embryologic left 6th aortic arch that connects the aorta to the pulmonary arterial circulation. Patent ductus arteriosus (PDA) is a congenital cardiac anomaly in which the ductus arteriosus fails to undergo physiologic closure after birth. PDA is associated with prematurity, trisomy 21, and other diseases. When isolated, a PDA acts as a left to right cardiac shunt. In the setting of a right aortic arch, a PDA completes the vascular ring. A PDA may be treated with medical closure with indomethacin, endovascular closure, or surgical closure.
Several congenital heart defects are dependent on a PDA to maintain circulation either to the systemic or pulmonary circulations. In these cases of ductal-dependent postnatal circulation, the PDA can be kept open with prostaglandins.
- Pulmonary atresia without a ventricular septal defect
- Critical pulmonary stenosis
- Tricuspid atresia
- Severe Ebstein anomaly
- Severe tricuspid regurgitation
- Hypoplastic left heart syndrome
- Critical aortic valve stenosis
- Interruption of the aortic arch
- Severe coarctation of the aorta
Additional important anatomic and physiologic variations of PDA anatomy will be presented:
- Reverse oriented ductus
- To and fro blood flow
- Reversal of PDA blood flow
- Right ductus arteriosus
- Completion of a vascular ring
Other important structures to evaluate:
- Trachea and main bronchi which may be compressed by the PDA
- Main and branch pulmonary arteries which may be stenotic or have isolated origin from the PDA
- Aortic arch sidedness and caliber (arch hypoplasia, coarctation, interruption) which may only become physiologically apparent as the PDA closes
- Vascular ring which may include the PDA or its ligament as a component of the ring
The purpose of this education exhibit is to describe:
- Embryology of the ductus arteriosus
- Normal anatomy and blood flow physiology of the ductus arteriosus
- Anatomy and physiology of the ductus arteriosus in congenital heart disease
- Abnormalities of adjacent mediastinal structures (trachea, bronchi, aorta, pulmonary arteries) due to PDA Read More
Keywords: patent ductus arteriosus, congenital heart disease
Final Pr. ID: Poster #: EDU-009
To provide a working knowledge of normal cardiac anatomy and a systematic guide to findings in common and uncommon congenital heart disease as seen on cardiac CTA. Read More
Authors: Scace Candace
Final Pr. ID: Paper #: 046
Portal hypertension is a manifestation of Fontan-related venous congestion and hepatic fibrosis. We studied the prevalence of radiologic evidence of portal hypertension (RP) and its association with Fontan hemodynamics and adverse outcomes. We also tested the validity of the VAST score, used in a prior study, in our population. Read More
Final Pr. ID: Paper #: 048
Norwood procedure is a 3-stage surgical palliation for patients with functionally single ventricle anatomy, most commonly hypoplastic left heart syndrome. Complications after stage I operation are not uncommon and include Blalock Taussig Thomas (BTT) /Sano shunt abnormalities, Damus-Kaye-Stansel (DKS) anastomotic narrowing, and neo-aortic arch /branch vessel stenosis. Transthoracic echocardiography (TTE) is traditionally the mainstay for evaluation. The purpose of our study is to compare Gated Cardiac Computed Tomographic Angiography (CCTA) with TTE when evaluating post-operative complications after stage I Norwood procedure and implications of CCTA findings on management.
Final Pr. ID: Poster #: EDU-004
The purpose of the educational exhibit is to review the CT imaging features of common congenital heart disease (CHD), using advanced CT technology, including PCD-CT with emphasis on both preoperative imaging and postoperative shunts. A variety of cases will be used to illustrate the features of the most prevalent congenital heart diseases. Read More
Final Pr. ID: Poster #: SCI-050
Infants with one functional cardiac ventricle often undergo the Norwood procedure which seeks to create unobstructed outflow from the single ventricle to aorta using a complex aortic arch reconstruction. The distal region of the patch used to fashion the neo-aorta terminates near the aortic isthmus, and represents an area of extensive challenge and manipulation for cardiothoracic surgeons. Re-coarctation in this region is common after the Norwood procedure and may be linked with longer-term morbidity and mortality. Echocardiography (TTE) is the primary means of surveillance of the aortic isthmus, though measurements are often difficult due to poor acoustic windows and lack of patient cooperation. To date, there have been no studies that assess how accurately TTE measures the aortic isthmus relative to a computed tomography (CT) derived gold standard. Read More
Final Pr. ID: Poster #: SCI-048
Infants with one functional cardiac ventricle undergo operations that yield unobstructed systemic outflow and a controlled source of pulmonary blood flow, often starting with the Norwood operation with Blalock-Taussig (BT) shunt. Infants with shunt dependent pulmonary blood flow are at risk for developing proximal pulmonary artery (PA) stenoses, which may result in morbidity and mortality. Echocardiography (TTE) is the primary means of surveillance for PA narrowing, but is limited by acoustic windows, operator experience and patient cooperation. Computed tomography (CT) offers high spatial resolution, rapid acquisition and relatively low radiation exposure. To date, there have been no studies comparing TTE and CT-derived measurements of the proximal PA anatomy in this high-risk population. Read More