Illustrated Imaging Essay on Congenital Heart Diseases: Multimodality Approach Part III: Cyanotic Heart Diseases and Complex Congenital Anomalies

Abstract

From the stand point of radiographic analysis most of the complex cyanotic congenital heart diseases (CHD), can be divided into those associated with decreased or increased pulmonary vascularity. Combination of a specific cardiac configuration and status of lung vasculature in a clinical context allows plain film diagnosis to be predicted in some CHD. Correlation of the position of the cardiac apex in relation to the visceral situs is an important information that can be obtained from the plain film. This information helps in gathering information about the atrio-ventricular, ventricular arterial concordance or discordance. Categorization of the cyanotic heart disease based on vascularity is presented below. Thorough understanding of cardiac anatomy by different imaging methods is essential in understanding and interpreting complex cardiac disease. Basic anatomical details and background for interpretation are provided in the previous parts of this presentation.

Keywords: Cardiac imaging; Ebsteins anomaly; Echocardiography; Pulmonary atresia; Tetralogy; Transposition of great arteries; Truncus arteriosus.

[Table/Fig-3a]:

TOF Severe outflow obstruction. A three-month-old female presented with cyanosis on feeding, diagnosed as Tetralogy on echocardiography, confirmed on MDCT examination; (a) Diagrammatic representation of cardiac anatomy in TOF; (b) Plain radiograph shows moderate cardiac enlargement with upturned apex (RV configuration) (triangle) and decreased pulmonary arterial markings; (c) Echocardiography long axis view demonstrates a large malaligned perimembraneous VSD (arrow); (d) Colour Doppler examination of RV outflow demonstrates narrowing of pulmonary artery with turbulent flow.

[Table/Fig-3b]:

A three-month-old female presented with cyanosis on feeding, diagnosed as Tetralogy on echocardiography, confirmed on MDCT examination: (e) axial CT images shows a large subaortic ventricular septal defect (arrow) and RVH; (f) Sagittal CT demonstrates large aorta(triangle) with normal branching pattern; (g) axial CT image at a slightly higher level shows severe narrowing of pulmonary infundibulum and proximal pulmonary artery (arrow). Right and left pulmonary arteries are relatively small. Also, there are enlarged collateral arteries in chest wall (open arrow) (h) 3D reconstruction of aorta shows enlarged major aorto-pulmonary collateral arteries (MAPCA) (arrows).

[Table/Fig-4]:

TOF Moderate outflow obstruction. A 21-year-old female presented with palpitations, right ventricular outflow obstruction on echocardiography diagnosed with PS; (a) Diagrammatic representation of cardiac anatomy in TOF (b) Plain radiograph shows normal cardiac size and configuration. Pulmonary arterial markings are decreased; (c) RVOT reconstructed CT; and (d) axial CT image at a slightly higher level shows severe narrowing of pulmonary infundibulum, pulmonary valve and proximal PA (arrows). RPA and LPA are relatively small; (e) Reconstructed CT view shows a large sub-aortic VSD (arrow) and large overriding aorta. There is biventricular enlargement and hypertrophy.

[Table/Fig-5a]:

Pulmonary Atresia. An eight-month-old male with cyanosis at birth, feeding difficulty; failure to demonstrate pulmonary artery on echocardiography diagnosed with pulmonary atresia: (a) Diagrammatic representation of cardio-vascular anatomy in pulmonary atresia; (b) Plain radiograph shows mild cardiac enlargement with upturned apex (RV configuration) and decreased pulmonary arterial markings. Collateral vessel around right hilar region are prominent (open arrow); (c) axial CT images shows a large sub-aortic ventricular septal defect (arrow) and RVH; (d) Reconstructed outflow CT view shows overriding of aorta and large sub-aortic VSD (arrow).

[Table/Fig-5b]:

An eight-month-old male with cyanosis at birth, feeding difficulty and failure to demonstrate pulmonary artery on echocardiography: Diagnosed with pulmonary atresia: (e) Axial CT slightly at a lower level shows blind ending pulmonary infundibulum (angled arrow). Main pulmonary artery is absent. Right and left pulmonary arteries reconstituted by collaterals (triangles); (f) 3D reconstruction of aorta shows enlarged collateral arteries (arrow) (MAPCA).

[Table/Fig-6a]:

Pulmonary atresia with bronchial compression by collateral artery. A three-year-old female presenting with exertional dyspnoea, diagnosed with a pulmonary atresia on echocardiography: (a) Diagrammatic representation of vascular anatomy in Pulmonary atresia; (b) Plain radiograph shows mild cardiac enlargement with upturned apex(arrow) (RV configuration) and decreased pulmonary arterial markings.

[Table/Fig-6b]:

A three-year-old female presenting with exertional dyspnoea, diagnosed with a pulmonary atresia on echocardiography: (a) axial CT images shows a atretic main pulmonary artery (star) and small right and left pulmonary arteries; (b,c) Reconstructed outflow CT views shows overriding of aorta and large sub-aortic VSD(circles).

[Table/Fig-6c]:

A three-year-old female presenting with exertional dyspnoea, diagnosed with a pulmonary atresia on echocardiography: (a) 3D reconstruction of aorta and airways (red) shows anatomy of pulmonary arteries, enlarged collateral arteries (MAPCA) (open arrows) impressing on the airways (triangle); (b).

[Table/Fig-7]:

TOF absent Pulmonary valves: A One-day-old female child presenting with respiratory distress, cyanosis, diagnosed with TOF with absent pulmonary valve on echo and CT: (a) Diagrammatic representation of vascular anatomy in TOF with absent pulmonary valve: (b) Plain radiograph shows hyperinflated left lung (arrow) due to partial left bronchial compression; (c) Coronal 3-D airway reconstruction demonstrates left bronchial compression(triangle); (d) Axial MIP CT image shows gross dilatation of pulmonary arteries with relative narrowing at valve level (arrow); (e) CT coronal MIP view shows severe dilatation of RPA and LPA (triangles); (f) Axial CT images reveals subaortic VSD (circle).

[Table/Fig-10]:

Pseudotruncus. A 10-year-male patient presenting with exertional dyspnoea, investigated for cardiac disease diagnosed with severe pulmonary atresia: (a) Diagrammatic representation patent of vascular anatomy in severe pulmonary atresia (pseudo-truncus); (b) axial CT images shows a large ascending aorta (arrow) and absent pulmonary artery. Large collateral artery is seen arising from descending aorta (open arrow); (c) Reconstructed coronal CT view shows collateral arteries reconstituting pulmonary arteries.(triangles); (d) 3D reconstruction of aorta shows enlarged collateral arteries (open arrow) (MAPCA).

[Table/Fig-12]:

Double outlet right ventricle (DORV) with aberrant right subclavian artery. One-year-old male referred to exclude CHD, diagnosed with a DORV in association with ARSA; (a) Diagrammatic representation of vascular anatomy in DORV, both great arteries arising from RV; (b) Plain radiograph reveal cardiomegaly with oligemic lungs; (c) Sagittal oblique MIP views shows dilated aortic arch with normal branching. There is a small PDA(triangle); (d)Axial MIP images shows infundibular and valvular stenosis(arrows); (e) Outflow view shows both aorta and PA arising from RV, LV communicating with a large VSD(star); (f) CT Coronal MIP view shows ARSA (open arrow) and narrowing of origin of LPA (arrow).

[Table/Fig-13]:

DORV, anomalons conus branch. One-year-old male presented with the cyanosis on feeding and feeding difficulty, suspected as TOF on echocardiography , diagnosed with DORV: (a) Diagrammatic representation patient of vascular anatomy in DORV; (b) Plain radiograph shows right ventricular cardiac configuration, oligemic lungs and right aortic arch (arrow); (c) Outflow view shows both aorta and PA arising from RV, LV communicating with a large VSD(arrow); (d) Axial CTA reveals normal size pulmonary arteries and right aortic arch (arrow) (e) Axial CT at aortic root show conus branch crossing RTOT(open arrow); (f) Coronal CT reconstruction shows tight infundibular stenosis (open arrow).

[Table/Fig-15a]:

TGA. A two-month-old male cyanotic child, suspected on echocardiography to have TGA, large VSD PS, diagnosed with transposition of great arteries, VSD and PS: (a) diagrammatic illustration of TGA with narrowing of the pulmonary outflow; (b) Plain radiography demonstrate mild biventricular cardiac enlargement. Lung fields are unremarkable; (c) Axial MIP image showing anteriorly located large aorta (arrow) and posteriorly located pulmonary artery (and open arrows); (d) CT axial image demonstrates biventricular enlargement with large ventricular septal defect (circle).

[Table/Fig-15b]:

A two-month-old male cyanotic child, suspected on echocardiography to have TGA, large VSD PS, diagnosed with transposition of great arteries, VSD and PS; (e) Lateral MIP CT image demonstrates anterior location of aorta (arrow). There is a narrowing of the pulmonary arterial origin (open arrow) (f). Modified oblique view shows large ventricular septal defect, anteriorly located aorta (arrow) and posterior pulmonary artery with narrowing at the origin. (triangle).

[Table/Fig-17a]:

Truncus arteriosus. A three-month-old male with breathing difficulty, recurrent chest infection, diagnosed with truncus arteriosus: (a) Diagrammatic representation of vascular anatomy in truncus arteriosus; (b) Plain radiograph shows moderate cardiac enlargement with increased pulmonary arterial markings, especially on right side(open arrow); (c) echocardiography shows a single trunk(open arrow) providing origin to pulmonary artery and aorta; (d-f) Reconstructed outflow CT images shows a single large ascending aorta overriding large septal defect(circle); (e) Reconstructed axial oblique CT view shows pulmonary arteries arising from posterior aspect of aorta (triangle).

[Table/Fig-17b]:

A three -month-old male with breathing difficulty, recurrent chest infection, diagnosed with truncus arteriosus; (a) Axial CT view shows configuration of aortic arch (arrow) and origin of pulmonary artery(arrow); (b) 3 D reconstruction showing larger aorta (arrow) and RPA and LPA arising posteriorly (open arrows). Also, there is an aberrant left subclavian artery (triangle).

[Table/Fig-21a]:

Supracardiac TAPVC. A three-month-old female presenting with cyanosis while feeding, abnormal vessel on echography, diagnosed with TAPVC: (a) diagrammatic representation of confluence of pulmonary veins into a vertical vein, draining subsequently in to left innominate vein; (b) Plain radiography demonstrating biventricular cardiomegaly and plethoric lung fields. Widening of the mediastinum is noted on left side (arrow); (c,d) Grey scale and colour Doppler echocardiography demonstrate ascending vertical vein (triangle and arrows) Both atria are enlarged; (e) MIP image in coronal plane of contrast-enhanced CT demonstrates confluence of all pulmonary veins into vertical vein and innominate vein. (Triangles); (f) Coronal CT MIP image demonstrates pulmonary plethora, dilated MPA and vena-cavae (triangles).

[Table/Fig-21b]:

Supracardiac TAPVC. A three-month-old female presenting with cyanosis while feeding, abnormal vessel on echography, diagnosed with TAPVC (g, h) 3-D CT reconstructed images viewed from posterior aspect demonstrates anomalous drainage of all the pulmonary veins into vertical vein (arrow).

[Table/Fig-22a]:

Intra-cardiac TAPVC. A two-month-old female presenting with cyanosis while crying, shown to have a septal defect on echocardiography diagnosed with intra-cardiac total anomalous pulmonary venous drainage: (a) Diagrammatic illustration showing a confluence of pulmonary artery draining into the enlarged coronary sinus; (b) Echocardiography shows dilated coronary sinus draining in to enlarged right atrium (arrow); (c) Colour Doppler exam shows enhanced flow in to right atrium via coronary sinus; (d) Axial CT MIP image shows confluence of pulmonary veins and dilated coronary sinus, draining into right atrium (open arrows).

[Table/Fig-22b]:

A two-month-old female presenting with cyanosis while crying, shown to have a septal defect on echocardiography diagnosed with intra-cardiac total anomalous pulmonary venous drainage: (a) axial CT images the level of pulmonary artery revealed grossly dilated MPA (open arrow); (b) axial CT image demonstrates grossly enlarged right atrium with a large secundum septal defect (open arrow). There is enlargement of the both ventricular cavities; (c) Coronal CT MIP image shows confluence of pulmonary veins (arrows); and (d) increased pulmonary arterial vascularity.

[Table/Fig-23a]:

Infra-cardiac TAPVC. 3-day-old female neonate presenting with respiratory distress, diagnosed with infra-diaphragmatic TAPVC to portal vein on MDCT. (a) Diagrammatic representation of vascular anatomy in infra-cardiac TAPVC. Anomalous common pulmonary vein (CPV) is shown draining to portal vein (colored red); (b) Plain radiograph shows obscured cardiac shadow with evidence of pulmonary oedema; (c)Coronal MIP CT reconstruction show anomalous CPV traversing the diaphragm and joining portal vein (arrow). Minimal narrowing noted at diaphragmatic level (triangle); (d,e) Axial CT images shows confluence of pulmonary veins (star) and site of union with portal vein (open arrow).

[Table/Fig-23b]:

A three-day-old female neonate presenting with respiratory distress, diagnosed with infra-diaphragmatic TAPVC to portal vein on MDCT: (a) 3 D rendered CT image viewed from posterior aspect shows the course of common pulmonary vein (arrows); (b) Sagittal MIP CT reconstruction show anomalous common pulmonary vein traversing the diaphragm with minimal narrowing at diaphragmatic level (triangle).

[Table/Fig-25]:

Ebsteins Anomaly. A 17-year-old male presenting with cyanosis on diagnosed with Ebsteins anomaly on Echocardiography and MR: (a) Diagrammatic representation of cardiac anatomy in Ebsteins anomaly; (b) Plain radiograph shows enlarged cardiac shadow, RA enlargement (arrow) and pulmonary oligmia; (c) Echocardiography demonstrating enlarged RA with apical displacement of septal tricuspid leaflet (arrow); (d) Axial bright blood MRI examination shows dilated RA displaced dysplastic tricuspid valves (open arrow); (e) Sagittal images shows large RA cavity and RV outflow tract(circle); (f) Coronal images illustrates displaced tricuspid valve (arrow).