Morphology Of Congenital Heart Disease Research Articles

A novel non-contrast agent-enhanced 3D whole-heart magnetic resonance sequence for congenital heart disease patients: the REACT Study.

The three-dimensional balanced-steady-state-free-precession (3D bSSFP) whole-heart (WH) technique has long been used to depict cardiac morphology in congenital heart disease (CHD) but is prone to banding artifacts. The Relaxation Enhanced Angiography without Contrast and Triggering (REACT) sequence is an alternative method that is resistant to off-resonance effects. To evaluate cardiac structures and great vessels in CHD patients using 3D WH REACT sequence and compare it to 3D WH bSSFP sequence. This study was approved by the Institutional Review Board. Thirty CHD patients were prospectively enrolled. Contrast-to-noise ratio (CNR), image quality, and cross-sectional area (CSA) were analyzed. Categorical data were compared with a Wilcoxon signed-rank test and normally distributed variables with a t-test. Thirty patients (16 females) participated in this study (median age 17, range 5months to 52years). REACT showed higher CNR in all pulmonary veins (all P<0.05), while 3D bSSFP had higher CNR in the right ventricle (P<0.001) and right pulmonary artery, (P=0.04). Image quality favored 3D bSSFP in the right atrium and ventricle (both P<0.001), main pulmonary artery (P=0.02), and coronary arteries (left: P<0.001, right: P=0.01). REACT outperformed 3D bSSFP for the pulmonary veins (all P<0.05) from image quality perspective. CSA measurements were not significantly different between REACT and 3D bSSFP (all P≥0.05). The REACT method is associated with improved image quality and CNR for pulmonary veins, with CSA measurements concordant with 3D bSSFP in CHD patients, while bSSFP shows better performance for imaging cardiac chambers and coronary arteries.

Novel TrueVue series of 3D echocardiography: Revealing the pathological morphology of congenital heart disease.

Aims: This study explored the advantages and limitations of novel series of three-dimensional (3D) echocardiographic techniques and summarized their application methods for congenital heart diseases (CHDs). Method and result: Two-dimensional (2D), traditional 3D echocardiography, and TrueVue plus light and/or Glass novel 3D technologies were performed on 62 patients with CHD, and a clinical survey was designed to judge whether the novel 3D images were more helpful for understanding the cardiac condition and guide treatment than traditional 3D images. TrueVue increased the visual resolution and simulated the true texture of cardiac tissue, significantly improving the display ability of abnormal anatomical structures in CHDs. TrueVue Glass displayed the blood channel and the internal structure of cardiac cavity more intuitively, indicating a new observation aspect not shown by conventional echocardiography. The clinical survey results showed that the new 3D imaging methods effectively increased the diagnostic confidence of echocardiographers, enabled surgeons to better understand the details of lesions, promoted efficient communication, and improved the confidence of both doctors and patients in treatment. Conclusion: The combined application of TrueVue, TrueVue Light, and TrueVue Glass more closely simulated real anatomical features, showed more comprehensive and subtle blood flow in the lumen, not only increased the visual effect but also provided more useful diagnostic information, improved the accuracy of evaluation and treatment of CHD when compared to traditional imaging techniques, indicating that this combined application has significant clinical value.

C22. Cardiac Morphology in Congenital Heart Disease with 3D Modeling, 3D Printing and Augmented Reality Smartphone Application: a Case Series of Cardiac Computed Tomography Assessment of Right Ventricular Outflow Tract Obstruction with Shunt

Abstract BACKGROUND Obstruction of the right ventricular outlet may include stenosis of the Right Ventricular Outflow Tract (RVOT) or the presence of atresia in the valve or pulmonary artery. The Cardiac Computed Tomography (CCT) examination for RVOT obstruction with a shunt has several indications, including pulmonary and coronary artery assessment, and lesions accompanying RVOT obstruction with a shunt. CASE DESCRIPTION In this report, we present five cases with RVOT obstruction with shunts in the form of Tetralogy of Fallot (ToF), pulmonary atresia with Ventricle Septal Defect (VSD), and pulmonary stenosis with Double Outlet Right Ventricle (DORV). The first patient with ToF and infective endocarditis. The second patient with atypical ToF, cardiac dextroposition, and suspect Scimitar Syndrome. The third patient with pulmonary atresia, VSD, and post-R-BT shunt. The fourth patient with pulmonary stenosis, DORV, remote VSD, post-BT shunt, and suspect Criss Cross Heart. The fifth patient with pulmonary stenosis, DORV, RA isomerism, malposition of the great artery, bilateral SVC, and dextrocardia. DISCUSSION The CCT assessment can be carried out in a segmental sequential to systematically and comprehensively approach. For complex lesions, CCT provides 3D printing of the heart and may help to understand the cardiac morphology easily. CCT is a recommended examination for RVOT obstruction with shunt, shows a good sensitivity for assessing BT-shunt, and provides good spatial resolution for assessing the pulmonary and coronary arteries. 3D printing and augmented reality can improve practitioners' understanding of RVOT obstruction with shunt, including the accompanying lesions.

C22. Cardiac Morphology in Congenital Heart Disease with 3D Modeling, 3D Printing and Augmented Reality Smartphone Application: a Case Series of Cardiac Computed Tomography Assessment of Right Ventricular Outflow Tract Obstruction with Shunt

Abstract BACKGROUND Obstruction of the right ventricular outlet may include stenosis of the Right Ventricular Outflow Tract (RVOT) or the presence of atresia in the valve or pulmonary artery. The Cardiac Computed Tomography (CCT) examination for RVOT obstruction with a shunt has several indications, including pulmonary and coronary artery assessment, and lesions accompanying RVOT obstruction with a shunt. CASE DESCRIPTION In this report, we present five cases with RVOT obstruction with shunts in the form of Tetralogy of Fallot (ToF), pulmonary atresia with Ventricle Septal Defect (VSD), and pulmonary stenosis with Double Outlet Right Ventricle (DORV). The first patient with ToF and infective endocarditis. The second patient with atypical ToF, cardiac dextroposition, and suspect Scimitar Syndrome. The third patient with pulmonary atresia, VSD, and post-R-BT shunt. The fourth patient with pulmonary stenosis, DORV, remote VSD, post-BT shunt, and suspect Criss Cross Heart. The fifth patient with pulmonary stenosis, DORV, RA isomerism, malposition of the great artery, bilateral SVC, and dextrocardia. DISCUSSION The CCT assessment can be carried out in a segmental sequential to systematically and comprehensively approach. For complex lesions, CCT provides 3D printing of the heart and may help to understand the cardiac morphology easily. CCT is a recommended examination for RVOT obstruction with shunt, shows a good sensitivity for assessing BT-shunt, and provides good spatial resolution for assessing the pulmonary and coronary arteries. 3D printing and augmented reality can improve practitioners' understanding of RVOT obstruction with shunt, including the accompanying lesions.

Clinical value of patient-specific three-dimensional printing of congenital heart disease: Quantitative and qualitative assessments.

ObjectiveCurrent diagnostic assessment tools remain suboptimal in demonstrating complex morphology of congenital heart disease (CHD). This limitation has posed several challenges in preoperative planning, communication in medical practice, and medical education. This study aims to investigate the dimensional accuracy and the clinical value of 3D printed model of CHD in the above three areas.MethodsUsing cardiac computed tomography angiography (CCTA) data, a patient-specific 3D model of a 20-month-old boy with double outlet right ventricle was printed in Tango Plus material. Pearson correlation coefficient was used to evaluate correlation of the quantitative measurements taken at analogous anatomical locations between the CCTA images pre- and post-3D printing. Qualitative analysis was conducted by distributing surveys to six health professionals (two radiologists, two cardiologists and two cardiac surgeons) and three medical academics to assess the clinical value of the 3D printed model in these three areas.ResultsExcellent correlation (r = 0.99) was noted in the measurements between CCTA and 3D printed model, with a mean difference of 0.23 mm. Four out of six health professionals found the model to be useful in facilitating preoperative planning, while all of them thought that the model would be invaluable in enhancing patient-doctor communication. All three medical academics found the model to be helpful in teaching, and thought that the students will be able to learn the pathology quicker with better understanding.ConclusionThe complex cardiac anatomy can be accurately replicated in flexible material using 3D printing technology. 3D printed heart models could serve as an excellent tool in facilitating preoperative planning, communication in medical practice, and medical education, although further studies with inclusion of more clinical cases are needed.

Cross-sectional Morphology of Congenital Heart Disease: Fetal Echocardiography Indicating Chromosome 22q11.2 Deletion Syndrome in Rat Model

Cross-sectional Morphology of Congenital Heart Disease: Fetal Echocardiography Indicating Chromosome 22q11.2 Deletion Syndrome in Rat Model

Diagnostic accuracy and clarity of steady-state free precession imaging of cardiac valve morphology in congenital heart disease

Purpose: Evaluation of cardiac valve morphology has not been considered an indication for cardiac magnetic resonance imaging (MRI) due to suboptimal imaging quality. Our study aims to evaluate cine of cardiac magnetic resonance-steady-state free precession (CMR-SSFP) imaging quality and diagnostic accuracy in the assessment of cardiac valve morphology for congenital heart disease. Materials and Methods: We retrospectively reviewed consecutive pediatric/congenital cardiac MRI cases. A 5-grade diagnostic clarity score was assigned to the aortic valve annulus and leaflets, tricuspid valve and mitral valve annuli, leaflets, chordae, and papillary muscles by examination of standard cine CMR-SSFP imaging. Among patients with aortic valve imaging, we compared morphologic diagnosis by CMR-SSFP to echocardiography. High-quality diagnostic imaging was defined as a clarity score of 1 or 2. Results: There were a total of 234 cardiac MRI studies evaluated with a total of 1892 valve components. The majority of valve annuli, leaflets, and papillary muscles had high diagnostic clarity score 64%–80% of the time – the tricuspid valve papillary muscles had a high diagnostic clarity score 53% of the time. Among the 39 cases with aortic valve imaging, CMR-SSFP correctly identified the aortic valve morphology including the affected commissure. Conclusions: CMR-SSFP produces high diagnostic quality imaging of cardiac valve morphology in congenital/pediatric cardiac MRI. The valve components with the highest diagnostic clarity score are tricuspid and mitral valve annuli, leaflets and papillary muscles, and aortic valve annuli and leaflets. Aortic valve morphology can be diagnosed with a high degree of reliability.

Coronary anomalies assessed by whole‐heart isotropic 3D magnetic resonance imaging for cardiac morphology in congenital heart disease

To determine the value of whole-heart three-dimensional magnetic resonance imaging (MRI) for coronary artery imaging in children/adolescents with congenital heart disease (CHD). Forty children/adolescents (median age: 14 years, range 2.6-25.8) with CHD underwent free-breathing navigator-gated isotropic three-dimensional steady-state free-precession (3D-SSFP) MRI for cardiac morphology. Two observers independently evaluated visibility of origin, course, vessel lengths, image quality (IQ), and contrast between coronary lumen and myocardium. A subgroup was compared with cardiac catheter. The total scan time was 6.3 +/- 3.2 minutes (mean +/- SD, at mean heart rate 76 +/- 15/min). The mean vessel length for right coronary artery (RCA) by observer 1 was 97 +/- 43 mm (observer 2: 94 +/- 37 mm), for left main and anterior descending artery (LM/LAD) 91 +/- 40 mm (observer 2: 90 +/- 40 mm), and for left circumflex artery (LCX) 64 +/- 28mm (observer 2: 66 +/- 28 mm). The mean vessel contrast was 0.34 +/- 0.05 (range: 0.23-0.45; maximum = 1, minimum = 0). On a 4-level score (1 = nondiagnostic, 4 = excellent), mean IQ scores ranged between 2.3-2.9 (+/-0.8-1.0). Both observers agreed on the presence/proximal course of RCA in 40/40, LM/LAD in 38/40, and LCX in 36/40 patients. There was complete agreement with invasive coronary angiography available in 12/40 patients (six anomalies). Isotropic whole-heart 3D-MRI for cardiac morphology allows reliable discrimination between normal and abnormal coronary anatomy in children/adolescents with CHD.

Operator-independent isotropic three-dimensional magnetic resonance imaging for morphology in congenital heart disease: a validation study.

Operator-independent isotropic 3D MRI may greatly simplify the assessment of complex morphology in congenital heart disease. We sought to evaluate the reliability of this new approach. In 31 adolescent and adult patients (age, 6 to 42 years; median, 16 years) with congenital heart disease, cardiac morphology was determined with free-breathing (navigator-gated), isotropic, 3D steady-state free-precession (3D SSFP) MRI and independently evaluated by 2 observers. Cardiac diagnoses and multiple distance measurements were compared with conventional MR reference sequences (ie, spin-echo, cine gradient-echo, contrast-enhanced MR angiography) and with echocardiography/cine cardioangiography or surgery. Of the 31 patients, 24 had native congenital heart defects or residual defects after repair that warranted immediate treatment. None of these defects was missed by 3D SSFP. Novel diagnostic issues were discovered in 4 of 31 patients (coronary anomalies, n=3; left juxtaposition of the right atrial appendage in double-outlet right ventricle and transposition of the great arteries, 1). For sizes of valves and vessels, we found minor mean differences of -1.1 to 1.6 mm, with SD ranging from 1.2 to 2.9 mm, demonstrating overall good agreement with standard MRI (Bland-Altman analysis). Interobserver variability of 3D SSFP distance measures was low; mean differences ranged from -1.5 to 1.0 mm, and SD ranged from 0.8 to 2.5 mm. Scatter was lower for extracardiac than intracardiac measures. In adolescents and adults, isotropic 3D SSFP MRI allows reliable assessment of complex cardiac morphology. Distance measurements are accurate and reproducible. Thus, a single operator-independent acquisition may substitute for conventional 2D MRI sequences to accelerate and simplify MR scanning in congenital heart disease.

Paediatric Cardiology, 2nd edition, volumes 1 and 2

Robert H. Anderson, Edward J. Baker, Fergus J. McCartney, et al, eds. 1036 pp (vol 1) and 1036 pp (vol 2). London: Churchill Livingstone; 2002. $399.00. ISBN: 0-4430-7990-0 A long-awaited second edition of Paediatric Cardiology has finally been published, after a period of 15 years. The chief editor, Robert Anderson, is one of the founding fathers of the field of cardiac morphology. The second edition has retained much of its original focus on cardiac morphology, laying out the “heart” of the subject, as it were. Its persisting emphasis on the importance of understanding the morphology of congenital heart disease (CHD) comes from having retained most of the primary authors and many more of the illustrations from the first edition. Indeed, improving non-invasive imaging technology has shifted the illustration of anatomy questions from the autopsy table to the echo laboratory. The new …

Peculiarities of Prevalence and Morphology of Congenital Heart Disease Detected In Utero

Intrauterine echocardiography is changing our knowledge of congenital heart disease; cardiac defects diagnosed in utero have distinctive features of both prevalence and morphology when compared with those observed just after birth. We reviewed a series of 171 fetal heart conditions: 148 were diagnosed at intrauterine echocardiography, the diagnosis being verified at autopsy in 41, and 23 were observed at the postmortem only. Peculiarities of prevalence consisted in an excess of various defects, such as hypoplastic left heart syndrome, atrial isomerism, pulmonary atresia, and atrioventricular and atrial septal defects, and in a reduced number of completely different conditions, such as transposition of great arteries and aortic coarctation. Differences in prevalence have been attributed to difficulties in diagnosing some particular anomalies in utero, to the selection of pregnancies undergoing screening, and to the special intrauterine evidence of some heart defects. Peculiarities in morphology result from the coexistence with extracardiac malformations, from the changes in shape conditioned by fetal hemodynamics, and from the intrauterine evolution of the morphology of some malformations. We concluded that the knowledge of these characteristic traits was helpful to cardiac pathologists, pediatric cardiologists, and obstetricians, and allowed the re-evaluation of the role of hemodynamic factors in remodeling the malformed cardiovascular appara-tus.

The Pathology of Congenital Heart Disease: A Personal Experience With More Than 6300 Congenitally Malformed Hearts

As leaders in a small cadre of cardiovascular clinicians and pathologists with a comprehensive understanding of and experience in the morphology of congenital heart disease, Dr Bharati (over 25 years) and the late Dr Lev (over 60 years) have made immense contributions. This impressive, informative, and richly illustrated two-volume work of more than 1500 pages summarizes their experience with the evaluation of over 6300 hearts. The book has two major sections. The first, only 64 pages long, has nine chapters describing general concepts of external and internal cardiac functional anatomy, embryology, terminology, and methods of examination. The second section has approximately 40 chapters of varying length and complexity, nearly all devoted to specific disease entities. The initial and largest disease-oriented chapter, on tetralogy of Fallot, has 66 pages and 154 illustrations, describing both simple and complex, acyanotic and cyanotic types, with individual consideration of the aorta, pulmonary tract, ventricular septal

Left and right ventricular morphology in complex congenital heart disease defined by two dimensional echocardiography

The ability of two dimensional echocardiography to define right and left ventricular morphology in congenital heart disease was examined in 19 patients with discordant ventricular connections and abnormal relations, but with two ventricles and two unambiguous atrioventricular (A-V) valves. The two dimensional echocardiographic criteria used to identify a chamber as having right morphology were (1) an irregular endocardial surface, (2) insertion of chordae tendineae into the ventricular septum, (3) presence of an infundibulum, (4) a triangular-shaped ventricular cavity, (5) observation of a moderator band, and (6) recognition of the A-V valve as tricuspid. The two dimensional echocardiographic criteria for a left ventricular morphology were (1) a smooth endocardial surface, (2) presence of two discrete papillary muscle groups, (3) an ellipsoid-shaped ventricular cavity, and (4) recognition of the A-V valve as mitral. Identification of the associated A-V valve as mitral or tricuspid was the most reliable criterion, defining each ventricle in all 19 patients. The nature of chordal attachment and papillary muscle insertion successfully identified all left-sided and posteriorly related ventricles and 9 of the 19 anterior ventricular chambers. Other criteria were less useful although, when observed, they confirmed the ventricular type.

Capillary microscopy. Abnormalities in cystic fibrosis, congenital heart disease, and mongolism.

ATTENTION has recently been directed toward the anatomy and physiology of the microvasculature. In humans, the direct observation of capillaries has been limited to but a few areas, namely, the conjunctivae, ocular fundi, and the skin. Because of its unique anatomic structure, the nailbed affords a readily accessible area for microscopic visualization of the capillaries. Capillary microscopy was originally described in 1912 by Lombard 1 and subsequently reviewed in the pediatric literature by Leader. 2 Recently Kontras and Bodenbender 3 described abnormal capillary morphology in congenital heart disease and noted its association with blood coagulation defects. Similar studies have also been reported in mongoloid children. 2 The present investigation was undertaken to extend these observations and to study the morphology of the nailbed capillaries in children with cystic fibrosis. Materials and Methods One hundred and ten children and adolescents were examined. Twenty (age 4 to 16 years) were normal, 23

Abnormal capillary morphology in congenital heart disease

Capillary morphology has been studied in 111 normal children. In 98% of these cases, straight hairpin forms make up over 80% of the capillaries examined. Thirty children with congenital heart disease showed 80% abnormal capillary patterns consisting of dilated, tortuous, and branched loops. These were most marked in cyanotic tetralogy of Fallot and ventricular septal defect with pulmonary hypertension. These cases also were associated with microscopic hemorrhages, increased capillary fragility and actual postoperative hemorrhagic complications. Definitive surgery in two cases, though improving the patient, did not result in changes in the abnormal capillary patterns. It is suggested that the role of the capillary structure in rheology of blood in the microvasculature has largely been ignored and that abnormalities in this vascular compartment may affect perfusion of tissues. The association of abnormalities in coagulation studies, hemorrhage, and thrombosis with congenital heart disease may in part be related to the morphologic vascular abnormalities. The abnormal capillary findings may be due to maturational arrest or dysmaturity of this portion of the cardiovascular system or may be the result of chronic hypoxia.