Illustrated Imaging Essay on Congenital Heart Diseases: Multimodality Approach Part II: Acyanotic Congenital Heart Disease and Extracardiac Abnormalities

Abstract

Acyanotic heart disease constitutes a significant majority of patient who may present with non-cardiac symptoms. Either they are detected incidentally or present with respiratory complaints. Equipped with knowledge of anatomy by echocardiography and radiographic methods described in previous part of this presentation, diagnosis may be confidently attempted. On plain radiography acyanotic congenital heart diseases have variable appearance depending upon severity of disease. Cardiac size, chamber enlargement and pulmonary vascular pattern are key elements. Typically left to right shunts with large volume flow are associated with pulmonary plethora. Plain radiography has an important role in detecting manifestation of pulmonary arterial hypertension. Severe stenosis of pulmonary valve is associated with pulmonary oligemia. Small intra-cardiac shunts and anomalies of coronary arteries generally present with normal cardiac size and pulmonary arterial pattern. Disease spectrum presented in this illustration demands thorough scrutiny of pulmonary, osseous and abdominal abnormalities. This section illustrates some commonly encountered spectrum of acyanotic cardiac disease.

Keywords: Absent right pulmonary artery; Anomalous pulmonary venous drainage; Coarctation of aorta; Coronary A-V fistula; Imaging.

[Table/Fig-4]:

A 13-year-old male patient presenting with exertional dyspnoea, diagnosed with atrial septal defect on echocardiography and MDCT: (a) Diagrammatic representation of secondum of atrial septal defect; (b) Plain radiograph shows mild cardiac enlargement, increased pulmonary arterial markings (arrows) and mildly dilated main pulmonary arterial segment(open arrow).

[Table/Fig-5]:

(c) Grey scale image of the right atrium illustrates sinus venosus ASD (arrow) (d) Colour Doppler image of the right atrium illustrates sinus venosus type of ASD (arrow) with the anomalous upper lobe vein (e) Reconstructed CT 4-CH view shows secundum ASD (arrows). There is enlargement of right atrium and ventricle (star); (f) 4-CH MR image in another patient shows a large septal defect (triangles).

[Table/Fig-6]:

Six-month-old male patient diagnosed with a left-to-right shunt, VSD, confirmed on echocardiography: (a) Diagrammatic representation of ventricular septal defect; (b) Chest radiograph shows pulmonary plethora (open arrow) and moderately dilated main pulmonary arterial segment (arrow). There is right upper lobe collapse; (c) Grey scale and the colour doppler images of the right ventricular outflow shows the perimembranous septal defect (arrow) and the jet illustrated by the Doppler examination; (d) Axial CT view shows grossly dilated pulmonary artery (arrow); (e) Coronal CT reconstruction demonstrates enlarged left atrium (star). Increased arterial shadows; (f) Demonstrates peri-membraneous ventricular septal defect in 4CH MRI examination (open arrow).

[Table/Fig-7]:

Eight-month-old, female diagnosed with patent ductus arteriosus on echocardiography, confirmed on MDCT: (a) Diagrammatic representation of patent ductus; (b) Plain radiograph shows moderate cardiac enlargement, increased pulmonary arterial markings, prominent aortic knuckle and left atrial enlargement (arrow); (c) Short axis Doppler view at the pulmonary arterial level shows the turbulent flow in the region of ductus (arrow). Pulmonary artery is noted medially (triangle). The continuous flow pattern is illustrated in the jet (C1). MDCT coronal (d) and axial; (e) views shows redundant patent ductus arteriosus (open arrows); (d) Size and length of ductus can vary considerably; (f) 3-D MDCT reconstruction demonstrates large ductus (angled arrow) connecting LPA and aorta.

[Table/Fig-8]:

L-R shunt with PAH, bronchial compression: Six-month-old female with feeding difficulty, irritability diagnosed with VSD, PDA with PAH. Echo demonstrated dilated MPA and VSD, PDA, PAH: (a) Plain radiograph shows moderate cardiomegaly with prominent MPA and proximal RPA (arrows); (b) axial CT image shows grossly dilated MPA and branches (arrows); (c) Coronal MIP CT image demonstrates, grossly dilated RPA; (d) Sagittal MIP images demonstrates severe dilatation of MPA and LPA (arrows); (e) 3D reconstruction of airway shows left bronchial compression(open arrow) by enlarged LPA.

[Table/Fig-10]:

Six-month-old female presenting with recurrent chest infection, abnormal chest radiograph, diagnosed with PAPCV: (a) Diagrammatic representation of vascular anatomy in infra-cardiac PAPVC. Anomalous right inferior pulmonary vein is shown draining IVC (red); (b) Plain radiograph shows dextro-positon of cardiac shadow with increased pulmonary arterial markings in left lung. Right lung is hypoplastic; (c) Coronal oblique CT and sagittal; (d) reconstruction shows anomalous right inferior pulmonary vein joining distal IVC (arrow). Sequestrated lung is seen at right lung base (solid arrow).

[Table/Fig-11]:

(e) 3D CT image shows anomalous vein joining IVC (triangle); Additional 3D CT image in oblique view shows union of anomalous vein. Arteries from SMA supplying sequestrated lung segment (open arrows).

[Table/Fig-12]:

One-year-old female admitted to hospital with severe chest infection, breathlessness diagnosed with PAPVC: (a) Diagrammatic representation of vascular anatomy in infra-cardiac PAPVC. Anomalous right inferior pulmonary vein is shown draining IVC (colored red); (b) Coronal image shows anomalous pulmonary vein (arrow) with increased pulmonary arterial markings.

[Table/Fig-13]:

(c) Coronal CT reconstruction shows anomalous right inferior pulmonary vein joining distal IVC (triangle); (e) Axial CT images demonstrate a large secundum atrial septal defect (black arrow). There is biventricular and right atrial enlargement arrow; There are consolidative changes in sequestrated lower lobe (triangle) (d, f) Sagittal CT MIP image and 3D images demonstrates anomalous pulmonary vein (arrow).

[Table/Fig-14]:

Three-year-old female presenting with a failure to thrive, left SVC on echocardiography diagnosed with partial anomalous pulmonary venous drainage of the left upper lobe: (a) Diagrammatic representation of vascular anatomy in supracardiac PAPVC. Anomalous left superior pulmonary veins is shown joining vertical vein and subsequently SVC (colour); (b) Scanogram shows moderate cardiac enlargement with increased pulmonary arterial markings and wide mediastinum (triangles); (c) axial-oblique CT images shows path of right superior pulmonary vein, vertical vein, innominate vein and SVC (arrow heads).

[Table/Fig-15]:

(d) Coronal MIP CT reconstruction shows anomalous superior pulmonary veins joining vertical vein; (e) Coronal and (f) sagittal CT image showing associated segmentation anomalies in vertebrae (arrows).

[Table/Fig-16a]:

Absent right pulmonary artery. Seven-month-old male child presenting with recurrent chest infections, diagnosed with an absent right pulmonary artery: (a) Chest radiograph demonstrating the decrease in the volume of right lung and the decrease in the visualization of the pulmonary arterial branches; (b,c) axial and sagittal MIP images shows increased pulmonary arterial markings on the left side. There are thin pulmonary arterial branches on the right side; (d,e) oblique views illustrate absence of right pulmonary artery (within circle) and dilatation of the left pulmonary artery (triangle).

[Table/Fig-16b]:

MIP Reconstructions in (f) coronal and (g) sagittal planes demonstrate large collaterals (black arrows, triangles) reconstituting right pulmonary artery (open arrow).

[Table/Fig-17]:

Young female with a cardiac murmur suspected on echo to have a vascular anomaly: (a) Contrast axial CT demonstrate enlarged tortuous circumflex coronary artery (open arrow); (b) Axial image at a slightly higher level show circumflex arterial branches extending to SVC with luminal opacification, (white arrow) due to fistulous connection.

[Table/Fig-18]:

Seventeen-year-old male presenting with chest pain. Axial contrast CT images at three levels (a-c) demonstrate gross dilatation of right (white arrow) and left coronary artery divisions. (open arrows). Colour display of CT angiography; (d) clearly demonstrates origin of LCA from pulmonary artery (ALCAPA).

[Table/Fig-19]:

Young adult, suspected with aortic coarctation on clinical examination and echocardiography: (a) Plain radiograph shows post stenotic dilation of aorta (black arrow). Aortic knuckle is not dilated. No rib notching was noted; (b) Oblique sagittal MIP images shows the site and extent of coarctation (open arrow); (c) Volumetric 3-D rendered images illustrates site of coarctation (open arrow) and intercostal collaterals (white triangle) and dilated internal mammary arteries (open triangles).

[Table/Fig-20]:

Heterotaxy: Asplenia features: (a) Right sided bronchial divisions; (b) bilateral right atrial appendage configuration; (c) Midline, bridged liver, situs inverses; (d) Levocardia.

[Table/Fig-21]:

Heterotaxy: Polysplenia features: (a) Bilateral left atrial appendage configuration; (b) Polyspenia; (c) Left sided bronchial divisions; (d) Dextrocardia.