Imaging Pearls ❯ Cardiac ❯ Congenital Heart Disease
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- "Thoracic vascular anomalies in the pediatric population are a heterogeneous group of diseases, with varied clinical presentations and imaging findings. High-resolution computed tomography is widely available and has become a standard part of the workup of these patients, often with three dimensional images. Cinematic rendering is a novel 3D visualization technique that utilizes a new, complex global lighting model to create photorealistic images with enhanced anatomic detail. The purpose of this pictorial review is to highlight the advantages of cinematic rendering compared to standard 2D computed tomography and traditional volume-rendered 3D images in the evaluation of thoracic vascular anomalies. Although cinematic rendering remains a new visualization technique under continued study, the improved anatomic detail and photorealistic quality of these images may be advantageous for surgical planning in cases of complex vascular abnormalities. Cinematic rendering may also help improve communication among clinicians, trainees, and patients and their families.”
3D CT cinematic rendering of pediatric thoracic vascular anomalies
Hannah S. Recht, Edmund M. Weisberg, Elliot K. Fishman
European Journal of Radiology Open 10 (2023) 100485 - "Congenital intrathoracic vascular anomalies are a heterogeneous group of diseases with varied clinical presentations, ranging from asymptomatic to life-threatening. Cross-sectional imaging with 3D visualization has been the standard of care in diagnosis of aortic arch anomalies, aortic coarctation, pulmonary slings, as well as bronchopulmonary malformations with vascular involvement, such as pulmonary sequestration.”
3D CT cinematic rendering of pediatric thoracic vascular anomalies
Hannah S. Recht, Edmund M. Weisberg, Elliot K. Fishman
European Journal of Radiology Open 10 (2023) 100485 - “The examples in this case series highlight the advantages of CR over standard VR reconstructions. The ability of CR to extract additional data from standard CT datasets may obviate the need for additional testing and radiation exposure, which is particularly advantageous in the pediatric population, for whom minimizing radiation is of foremost concern. The 3D visualization and textural contrast provided by CR can improve visualization of complex vascular anatomy, as shown in the above cases, which is essential for preoperative evaluation. One small study assessing the value of CR for extracardiac anatomy in patients with congenital heart defects indicated that pediatric cardiac surgeons preferred the CR images to VR images due to improved “spatial impression in general” and “depth perception”
3D CT cinematic rendering of pediatric thoracic vascular anomalies
Hannah S. Recht, Edmund M. Weisberg, Elliot K. Fishman
European Journal of Radiology Open 10 (2023) 100485 - "Beyond potential diagnostic and treatment benefits, CR offers photorealistic representation of complex anatomy and pathology that can be more intuitively understood by those without a medical background, potentially improving communication between clinicians and patients and/or their families. For the same reasons, CR also may have a role in trainee education, as virtual anatomy becomes increasingly relevant. Although CR remains a relatively new technique under much study, it brings new possibilities for improving diagnosis and treatment in the pediatric population.”
3D CT cinematic rendering of pediatric thoracic vascular anomalies
Hannah S. Recht, Edmund M. Weisberg, Elliot K. Fishman
European Journal of Radiology Open 10 (2023) 100485
- Ebstein's anomaly
- Ebstein's anomaly is a congenital heart defect in which the septal and posterior leaflets of the tricuspid valve are displaced towards the apex of the right ventricle of the heart. It is classified as a critical congenital heart defect accounting for <1% of all congenital heart defects presenting in ≈1 per 200,000 live births. Ebstein anomaly is the congenital heart lesion most commonly associated with supraventricular tachycardia. - Ebstein anomaly accounts for <1% of all congenital heart anomalies. In this disease, a malformation of the tricuspid valve and right ventricle is characterized by adherence of the septal and posterior leaflets to the underlying myocardium with a failure of delamination. One of the most common associated structural cardiac findings with Ebstein anomaly is an interatrial communication in most patients. Additional cardiac abnormalities in patients with Ebstein anomaly include ventricular septal defect and Wolff-Parkinson-White syndrome.
- “ Multidetector CT is an alternative tool helpful in establishing the primary diagnosis, defining anatomic landmarks and their relationships, and identifying associated cardiovascular anomalies. It is also an adjunct in the evaluation of complications during follow-up.”
Uncommon Congenital and Acquired Aortic Disease: Role of Multidetector CT Angiography
Kimura-Hayama ET, et al
RadioGraphics 2010; 30;79-98 - “ With its high spatial resolution and isotropic and volumetric information multidetector CT performed with or without ECG-gated technique allows accurate and fast noninvasive characterization of aortic pathologic conditions.”
Uncommon Congenital and Acquired Aortic Disease: Role of Multidetector CT Angiography
Kimura-Hayama ET, et al
RadioGraphics 2010; 30;79-98 - Patent Ductus Arteriosus: Facts
- Connects the proximal descending aorta, immediately distal to the origin of the left subclavian artery, with the proximal left pulmonary artery at the junction of the main pulmonary artery
- Closes functionally 18-24 hours after birth and anatomically at one month
- If it is patent after 3 months it is a PDA - Patent Ductus Arteriosus: Facts
- PDA occurs in 1 of 2,000 children born full term
- More common in woman (2-1)
- Treatment includes surgical closure or percutaneous placement of an occluder devise
- “ Multidetector CT is an alternative tool helpful in establishing the primary diagnosis, defining anatomic landmarks and their relationships, and identifying associated cardiovascular anomalies. It is also an adjunct in the evaluation of complications during follow-up.”
Uncommon Congenital and Acquired Aortic Disease: Role of Multidetector CT Angiography
Kimura-Hayama ET, et al
RadioGraphics 2010; 30;79-98 - “ With its high spatial resolution and isotropic and volumetric information multidetector CT performed with or without ECG-gated technique allows accurate and fast noninvasive characterization of aortic pathologic conditions.”
Uncommon Congenital and Acquired Aortic Disease: Role of Multidetector CT Angiography
Kimura-Hayama ET, et al
RadioGraphics 2010; 30;79-98 - Patent Ductus Arteriosus: Facts
- Connects the proximal descending aorta, immediately distal to the origin of the left subclavian artery, with the proximal left pulmonary artery at the junction of the main pulmonary artery
- Closes functionally 18-24 hours after birth and anatomically at one month
- If it is patent after 3 months it is a PDA - Patent Ductus Arteriosus: Facts
- PDA occurs in 1 of 2,000 children born full term
- More common in woman (2-1)
- Treatment includes surgical closure or percutaneous placement of an occluder devise - “Patients who have a pericardial defect without associated congenital abnormalities are often asymptomatic. Complications of congenital pericardial defect may include herniation and entrapment of a cardiac chamber, especially the left atrial appendage. Surgical closure or enlargement of the defect is sometimes necessary to alleviate herniation.”
CT and MR Imaging of Pericardial Disease
Wang ZJ et al.
RadioGraphics, October 2003 23, S167-S180. - “Patients with pericardial defects also may have one or more associated congenital abnormalities, including atrial septal defect, patent ductus arteriosus, mitral valve stenosis, or tetralogy of Fallot (41), which also are detectable on CT or MR images. Patients who have a pericardial defect without associated congenital abnormalities are often asymptomatic. Complications of congenital pericardial defect may include herniation and entrapment of a cardiac chamber, especially the left atrial appendage. Surgical closure or enlargement of the defect is sometimes necessary to alleviate herniation.”
CT and MR Imaging of Pericardial Disease
Wang ZJ et al.
RadioGraphics, October 2003 23, S167-S180. - “ Congenital absence of the pericardium is rare. Most pericardial defects are partial and occur on the left side. Infrequently, defects also occur on the right side or at the diaphragmatic surface. Normally, the aortopulmonary window is covered by pericardium and contains some fat. Left-sided absence of the pericardium allows interposition of lung tissue between the aorta and the main segment of the pulmonary artery, and, occasionally, bulging of the left atrial appendage through the defect. As a result of these abnormalities, the heart usually rotates toward the left.”
CT and MR Imaging of Pericardial Disease
Wang ZJ et al.
RadioGraphics, October 2003 23, S167-S180. - “ Congenital absence of the pericardium is rare. Most pericardial defects are partial and occur on the left side. Infrequently, defects also occur on the right side or at the diaphragmatic surface. Normally, the aortopulmonary window is covered by pericardium and contains some fat. Left-sided absence of the pericardium allows interposition of lung tissue between the aorta and the main segment of the pulmonary artery, and, occasionally, bulging of the left atrial appendage through the defect. As a result of these abnormalities, the heart usually rotates toward the left. Although radiographs may show evidence of this condition, a definitive diagnosis can be obtained with either CR or MR imaging.”
CT and MR Imaging of Pericardial Disease
Wang ZJ et al.
RadioGraphics, October 2003 23, S167-S180. - Ebstein Anomaly: CT Findings
- Marked right atrial enlargement / right ventricle dilatation
- Reflux of contrast media into the hepatic veins due to tricuspid regurgitation
- Apical displacement of septal and posterolateral tricuspid valve leaflets; apical displacement of the septal leaflet at least 8 mm is considered diagnostic
- Atrialized proximal right ventricle
- Shunt may be present at the atrial level (50%):
- Effacement or displacement of left ventricle by massive right heart size - “ The embryological development of tricuspid valve leaflets and chordae involves undermining of the right ventricular free wall. This process continues to the level of the atrioventricular (AV) junction. In Ebstein anomaly, this process of undermining is incomplete and falls short of reaching the level of the AV junction. In addition, the apical portions of the valve tissue, which normally undergo resorption, fail to resorb completely. This results in distortion and displacement of the tricuspid valve leaflets, and a part of the right ventricle becomes atrialized. In one study involving 50 hearts with the anomaly, the entire right ventricle was found to be morphologically abnormal.”
Ebstein Anomaly Author: Kamran Riaz, MD; Chief Editor: Park W Willis IV, MD - Ebstein Anomaly: Facts
Ebstein anomaly can present at various stages of life.
- Fetal life: Ebstein anomaly is usually diagnosed incidentally by echocardiography.
- Neonatal life and infancy: Ebstein anomaly presents with cyanosis and/or severe heart failure; typically, symptoms present in infancy improve as pulmonary vascular resistance decreases.
- Adult life: Ebstein anomaly presents with fatigue, exertional dyspnea, cyanosis, tricuspid regurgitation and/or right heart failure, and palpitations; arrhythmias are common. (Medscape) - Ebstein Anomaly: Facts
- Ebstein anomaly is a congenital malformation of the heart that is characterized by apical displacement of the septal and posterior tricuspid valve leaflets, leading to atrialization of the right ventricle with a variable degree of malformation and displacement of the anterior leaflet. "A right aortic arch can occur in isolation or in association with congenital heart disease, especially in tetralogy of Fallot and truncus arteriosus. Edwards embryologic double aortic arch model can help explain the embryogenesis of many variations of right aortic arch."
Right Aortic Arch and Its Variants
Kanne JP, Godwin D J
Cardiovascular Comput Tomogr (2010) 4,293-300- Transposition of the Great Vessels (TOGV): Facts
- Two main variant are dextrotransposition (complete) and levotransposition (congenitally corrected)
- In the D-form the aorta connects to the anatomic RV and the pulmonary artery to the LV. Survvival possible due to ASD, VSD and/or PDA
- Surgery is needed in D-form for survival and usually mean arterial switch procedure - Supravalvular Aortic Stenosis: facts
- Focal or diffuse narrowing of the aorta starting at the sinotubular junction and often involves the entire ascending aorta
- SVAS may be associated with Williams-Beuren syndrome which is an autosomal dominant multisystematic disorder that may manifest with SVAS (71% of cases), mitral valve prolapse and pulmonary artery stenosis - Coarctation of the Aorta: Facts
- Male predominance (1.5:1)
- Aortic narrowing in region of ligamentum arteriosum just distal to the left subclavian artery
- Solitary lesion in 82% of cases but can be associated with Turner syndrome, bicuspid aortic valve, intracranial aneurysms, VSD and ASD defects, and Shone complex (left ventricular outflow tract obstruction and parachute mitral valve) - Patent Ductus Arteriosus: Facts
- PDA occurs in 1 of 2,000 children born full term
- More common in woman (2-1)
- Treatment includes surgical closure or percutaneous placement of an occluder device - Patent Ductus Arteriosus: Facts
- Connects the proximal descending aorta, immediately distal to the origin of the left subclavian artery, with the proximal left pulmonary artery at the junction of the main pulmonary artery
- Closes functionally 18-24 hours after birth and anatomically at one month
- If it is patent after 3 months it is a PDA "The primary role of CT is noninvasive assessment of extracardiac systemic and pulmonary arterial and venous structures and post treatment complications, most often as an adjunct to echocardiography ."
Role of CT in the Evaluation of Congenital Cardiovascular Disease in Children
Dillman JR, Hernandez RJ
AJR 2009; 192:1219-1231"CT accurately depicts many forms of congenital cardiovascular disease in children. It is particularly valuable in the assessment of extracardiac vascular anomalies and in the evaluation of postoperative complications."
Role of CT in the Evaluation of Congenital Cardiovascular Disease in Children
Dillman JR, Hernandez RJ
AJR 2009; 192:1219-1231- Coarctation of the Aorta:Facts
Associated with cardiac anomalies
- Bicuspid valve (50%)
- VSD (33%)
- PDA (66%)
- Subaortic and mitral stenosis
- Bicuspid Aortic Valve:Facts
- Incidence of 0.4-2.2%
- More common on Turner’s syndrome, coarctation of the aorta and VSD
- Leads to early valve calcification and aortic stenosis with dilated ascending aorta
- Coarctation of the Aorta: Facts
- 5% of all congenital heart disease
- Narrowing of aorta most commonly just distal to the origin of the subclavian artery
- Simple coarctation means the absence of any other cardiac anomalies
- Complex aortic coarctation refers to associated anomalies plus COA
- Congenital Anomalies of the Thoracic Aorta
- Sequestration
- Vascular rings
- Coarctation of the aorta
- Aberrant vessels
- Right sided arch and associated anomalies
- Transposition of the Great Vessels (TGV): facts
- Classic repair is the Mustard-Senning Procedure with creation of atrial baffle to direct systemic venous blood across mitral valve to left ventricle and pulmonary venous blood flow across the tricuspid valve into the right ventricle
- The Jatene operation is now used - "A systematic approach to the evaluation of the right heart and an understanding of the congenital abnormalities causing right chamber enlargement will allow the radiologist to diagnose unsuspected cardiac abnormalities."
Right Heart Dilatation in Adults:Congenital Causes
Cook AL et al.
AJR 2007;189:592-601 - Pulmonary Sling: Facts:
- Left pulmonary aa arises from the right pulmonary aa and passes between the trachea and esophagus
- Tracheobronchiomalacia and/or stenosis can occur in up to 50% of cases
Medscape