DR. MUHAMMAD BIN ZULFIQARPGR IV FCPS SIMS/SHLradiombz@gmail.com

20 Congenital Heart Disease:General Principles and Imaging Grainger and Allison

• FIGURE 20-1 Perioperative CXR. (A) A 3-year-old patient following total ■cavopulmonary connection surgery, postoperative CXR demonstrating tube positions in intensive care. Note two chest and one mediastinal drains, endotracheal tube and veno-venous collateral occluder device (right upper zone). (B) Third postoperative day following extubation and removal of mediastinal drain. Note change in cardiomediastinal contour caused by large pericardial clot, requiring evacuation.

• FIGURE 20-2 Physiological assessment using CXR. (A) ■Pulmonary plethora in a patient with a VSD. Note the increased number and size of discrete vessels without haziness. (B) Pulmonary oedema in a supine patient with cor triatriatum (membranous obstruction to LA outflow) resulting in increased pulmonary venous pressure. Note cardiomegaly, perihilar alveolar haziness/consolidation and peribronchial cuffing

• FIGURE 20-3 Pulmonary oligaemia. (A) Supine AP CXR, in an 8-week-old ■patient with tetralogy of Fallot with severe pulmonary stenosis and cyanosis. Note black lungs with sparse, small-calibre vessels. (B) Supine AP CXR, in the same patient following construction of a right modified Blalock–Taussig (BT) shunt on the next day. Note the increased size of the left cardiac contour due to increased LV filling, increased pulmonary vascular markings, now plethoric, suggestive of high pulmonary blood flow arising from the shunt. Indeed, the patient had compromised systemic perfusion due to redistribution of cardiac output to the lungs, necessitatin clipping the shunt to reduce its calibre

• FIGURE 20-4 Atrial septal defects. (A) Schematic drawing of ASD positions. (B) b-■SSFP CMR image. Four-chamber view showing a large secundum ASD with posterior extension. The absence of a posterior rim (arrow) precludes insertion of an ASD closure device. Note the dilated right atrium (RA), and right ventricle (RV), and flattened interventricular septum. (C) b-SSFP CMR image. Axial view showing a large superior sinus venosus defect, with PAPVD of the right upper and right middle pulmonary veins, straddling the deficient atrial septum (arrow). (D) Plot of instantaneous flow (measured by velocity-encoded phase-contrast MRI) as a function of time showing a left-to-right shunt through an ASD; note increased pulmonary blood flow

• FIGURE 20-4 Atrial septal defects. (A) Schematic drawing of ASD positions. ■(B) b-SSFP CMR image. Four-chamber view showing a large secundum ASD with posterior extension. The absence of a posterior rim (arrow) precludes insertion of an ASD closure device. Note the dilated right atrium (RA), and right ventricle (RV), and flattened interventricular septum. (C) b-SSFP CMR image. Axial view showing a large superior sinus venosus defect, with PAPVD of the right upper and right middle pulmonary veins, straddling the deficient atrial septum (arrow). (D) Plot of instantaneous flow (measured by velocity-encoded phase-contrast MRI) as a function of time showing a left-to-right shunt through an ASD; note increased pulmonary blood flow

• FIGURE 20-5 Atrioventricular septal defects. (A) Schematic drawing ■of orthogonal views of a common atrioventricular valve: shortaxis view from below (left), long-axis (top right), 4-chamber (bottom right). (B) Valve view showing a complete AVSD in a patient with right atrial isomerism and double outlet RV. Valve leaflets: SB = superior bridging leaflet, RAS = right anterosuperior leaflet, RI = right inferior (mural) leaflet, IB = inferior bridging leaflet, LM = left mural leaflet. (C) b-SSFP CMR image showing 4-chamber view of a balanced complete AVSD. There are large atrial and ventricular components. Note the VSD (arrow) and moderate left AV valve regurgitation (arrowhead).

• FIGURE 20-6 Ventricular septal defects. (A) ■Schematic drawing of VSD positions viewed from the right ventricular aspect. (B) b-SSFP CMR image of a VSD (arrow) with overriding aorta in a patient with tetralogy of Fallot. (C) Coronal oblique view following correction with VSD patch (arrowhead).

• FIGURE 20-7 Severe coarctation of the aorta. (A) PA CXR showing characteristic ■bilateral rib-notching (arrow), secondary to the development of collateral circulation. (B) Black-blood, spin-echo, oblique sagittal image through the aorta showing a tight discrete coarctation (arrow). (C) Volume-rendered 3D reconstruction of MR angiography showing a tight coarctation (arrowhead), and multiple enlarged collateral vessels. (D) Echocardiographic continuous-wave Doppler profile of the coarctation region, demonstrating increased velocity across the stenosis, 4.18 m/s (blue cross), corresponding to a pressure gradient of 70 mmHg from the simplified Bernoulli equation. There is also markedly increased diastolic velocity, characteristic in coarctation, termed ‘diastolic tail’ (red star

• FIGURE 20-7 Severe coarctation of the aorta. (A) PA CXR showing characteristic ■bilateral rib-notching (arrow), secondary to the development of collateral circulation. (B) Black-blood, spin-echo, oblique sagittal image through the aorta showing a tight discrete coarctation (arrow). (C) Volume-rendered 3D reconstruction of MR angiography showing a tight coarctation (arrowhead), and multiple enlarged collateral vessels. (D) Echocardiographic continuous-wave Doppler profile of the coarctation region, demonstrating increased velocity across the stenosis, 4.18 m/s (blue cross), corresponding to a pressure gradient of 70 mmHg from the simplified Bernoulli equation. There is also markedly increased diastolic velocity, characteristic in coarctation, termed ‘diastolic tail’ (red star

• FIGURE 20-8 Coronary artery anomalies. Schematic diagram of the ■coronary arteries viewed in the axial oblique plane on CMR. RA = right atrium, LA = left atrium, LV = left ventricle, RVOT = right ventricular outflow tract, LAD = left anterior descending artery, RCA = right coronary artery, LCX = left circumflex artery. (A) Anomalous LCX from RCA. (B) Anomalous RCA from left main stem (LMS), with interarterial course between pulmonary artery and aorta. (C) Anomalous RCA from LMS passing posteriorly between the aorta and atria. (D) Anomalous left coronary artery arising from RCA with interarterial course between the pulmonary trunk and aorta. (E) Anomalous left coronary artery arising from RCA passing anterior to pulmonary trunk. (F) Anomalous left coronary artery arising from RCA passing posteriorly between aorta and atria

• FIGURE 20-8 Coronary artery anomalies. Schematic diagram of the ■coronary arteries viewed in the axial oblique plane on CMR. RA = right atrium, LA = left atrium, LV = left ventricle, RVOT = right ventricular outflow tract, LAD = left anterior descending artery, RCA = right coronary artery, LCX = left circumflex artery. (A) Anomalous LCX from RCA. (B) Anomalous RCA from left main stem (LMS), with interarterial course between pulmonary artery and aorta. (C) Anomalous RCA from LMS passing posteriorly between the aorta and atria. (D) Anomalous left coronary artery arising from RCA with interarterial course between the pulmonary trunk and aorta. (E) Anomalous left coronary artery arising from RCA passing anterior to pulmonary trunk. (F) Anomalous left coronary artery arising from RCA passing posteriorly between aorta and atria

• FIGURE 20-9 Tetralogy of Fallot. (A, B) Right ventricular outflow tract, ■morphological specimen and corresponding black-blood spin-echo image in coronal view. The deviated outlet septum (asterisk), aortic root (arrowhead) and hypertrophied septoparietal trabeculations (arrow) are shown. (C) b-SSFP images of unrepaired tetralogy of Fallot: inflow/outflow view of the left ventricle (LV) shows a VSD with overriding aorta (Ao)—note the severe hypertrophy of the right ventricle (RV). (D) Black-blood, spin-echo image of right modified Blalock–Taussig shunt; 3.5-mm gortex tube from innominate artery to right pulmonary artery (arrow).

• FIGURE 20-9 Tetralogy of Fallot. (A, B) Right ventricular outflow tract, ■morphological specimen and corresponding black-blood spin-echo image in coronal view. The deviated outlet septum (asterisk), aortic root (arrowhead) and hypertrophied septoparietal trabeculations (arrow) are shown. (C) b-SSFP images of unrepaired tetralogy of Fallot: inflow/outflow view of the left ventricle (LV) shows a VSD with overriding aorta (Ao)—note the severe hypertrophy of the right ventricle (RV). (D) Black-blood, spin-echo image of right modified Blalock–Taussig shunt; 3.5-mm gortex tube from innominate artery to right pulmonary artery (arrow).

• FIGURE 20-10 Transposition of the great arteries. (A) b-SSFP CMR image showing an ■oblique sagittal outlet view of the aorta arising from the right ventricle (RV) and pulmonary artery arising posteriorly from the left ventricle (LV). (B) Schematic drawing of the arterial switch repair of TGA, showing the Le Compte manoeuvre with the translocation of the aorta and pulmonary artery. Note sites of coronary artery ‘button’ removal and subsequent reimplantation into the neo-aortic root. (C) b-SSFP CMR image showing the pulmonary arteries straddling the aorta following the arterial switch procedure with Le Compte manoeuvre. (D) Volume-rendered 3D reconstruction of a contrast-enhanced MRA showing bilateral proximal branch pulmonary artery narrowing.

• FIGURE 20-10 Transposition of the great arteries. (A) b-SSFP CMR image showing ■an oblique sagittal outlet view of the aorta arising from the right ventricle (RV) and pulmonary artery arising posteriorly from the left ventricle (LV). (B) Schematic drawing of the arterial switch repair of TGA, showing the Le Compte manoeuvre with the translocation of the aorta and pulmonary artery. Note sites of coronary artery ‘button’ removal and subsequent reimplantation into the neo-aortic root. (C) b-SSFP CMR image showing the pulmonary arteries straddling the aorta following the arterial switch procedure with Le Compte manoeuvre. (D) Volume-rendered 3D reconstruction of a contrast-enhanced MRA showing bilateral proximal branch pulmonary artery narrowing.

• FIGURE 20-11 Congenitally corrected transposition ■of the great arteries. (A) b-SSFP CMR image of CCTGA showing the discordant atrioventricular connection, with anterior LV. Note the apical offset of the left-sided tricuspid valve. (B) Schematic drawing of CCTGA and frequent associated lesions.

• FIGURE 20-11 Congenitally corrected transposition of the great ■arteries. (A) b-SSFP CMR image of CCTGA showing the discordant atrioventricular connection, with anterior LV. Note the apical offset of the left-sided tricuspid valve. (B) Schematic drawing of CCTGA and frequent associated lesions.

• FIGURE 20-12 Total anomalous pulmonary venous drainage. (A) ■PA CXR in a patient with unobstructed supracardiac TAPVD. Note dilated ascending vein (arrow) returning all pulmonary blood to the brachiocephalic vein. The arrowhead shows the dilated SVC. (B) Volume-rendered 3D reconstruction of MR angiography showing total anomalous infracardiac drainage of the pulmonary veins. Note the narrowing of the veins as they pass through the diaphragm (arrow) before draining into the portal vein (arrowhead).

• FIGURE 20-13 Single ventricle. (A) b-SSFP CMR image showing hypoplastic ■left heart syndrome, with severe hypertrophy of the systemic RV. Note the large interatrial communication (arrowed), allowing mixing of systemic and pulmonary venous return. (B) Volume-rendered 3D reconstruction of an MR angiogram showing the Glenn, bidirectional cavopulmonary anastomosis (arrow) and (C) a lateral tunnel total cavopulmonary anastomosis (arrow) to the right pulmonary artery (arrowhead). (D) b-SSFP CMR image showing severe ascites (arrow), and right pleural effusion (arrowhead) in a patient with a failing TCPC circulation and protein-losing enteropathy.

• FIGURE 20-13 Single ventricle. (A) b-SSFP CMR image showing hypoplastic left ■heart syndrome, with severe hypertrophy of the systemic RV. Note the large interatrial communication (arrowed), allowing mixing of systemic and pulmonary venous return. (B) Volume-rendered 3D reconstruction of an MR angiogram showing the Glenn, bidirectional cavopulmonary anastomosis (arrow) and (C) a lateral tunnel total cavopulmonary anastomosis (arrow) to the right pulmonary artery (arrowhead). (D) b-SSFP CMR image showing severe ascites (arrow), and right pleural effusion (arrowhead) in a patient with a failing TCPC circulation and protein-losing enteropathy.

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