Imaging of congenital heart disease in adults

Abstract

Imaging is fundamental to the lifelong care of adult congenital heart disease (ACHD) patients. Echocardiography remains the first line imaging for inpatient, outpatient, or perioperative care. Cross-sectional imaging with cardiovascular magnetic resonance (CMR) or computed tomography (CT) provides complementary and invaluable information on cardiac and vascular anatomy and other intra-thoracic structures. Furthermore, CMR provides quantification of cardiac function and vascular flow. Cardiac catheterization is mostly reserved for assessment of pulmonary vascular resistance, ventricular end-diastolic pressure, and percutaneous interventions. There have been further advances in non-invasive imaging for ACHD including the application of advanced echocardiographic techniques, faster automated CMR imaging, and radiation dose reduction in CT. As a result ACHD, a heterogeneous population, benefit from appropriate application of multiple imaging modalities matched with tertiary ACHD expertise.

Keywords: Chest X-ray; Computed tomography; Congenital heart disease; Echocardiography; Imaging; Magnetic resonance imaging.

Figure 1

Acute presentation of endocarditis with vegetation (asterisk) related to previous VSD surgical repair patch leak shown in apical four-chamber 2D echocardiography view (A) and magnified from apical five-chamber view (B).

Figure 2

Echocardiography indices tricuspid annular plane systolic excursion <15 mm, right atrial area ≥25 cm², right atrial to left atrial area ratio ≥1.5, and right ventricular systolic over diastolic ratio ≥1.5 are all predictive of survival in Eisenmenger syndrome. Images show right atrial enlargement (A), prolonged tricuspid regurgitation on Doppler compromising filling (D = diastole, S = systole in B), biventricular hypertrophy and a flattened interventricular septum (C), and impaired tricuspid annular plane systolic excursion of 11 mm (D), in this patient with longstanding Eisenmenger syndrome.

Figure 3

Speckle tracking of the left ventricular (LV) from apical four-chamber (A), apical three-chamber (B), and apical two-chamber (C) views in adult patient with repaired anomalous coronary artery from the pulmonary artery syndrome. Despite normal LV ejection fraction, speckle tracking shows LV myocardial dysfunction. The bullseye plot (D) shows that the anterior and inferior segments are akinetic. Total global longitudinal strain is −14%.

Figure 4

Large atrial septal defect (asterisk) at 3D transoesophageal echocardiography (A) with relative lack of a posterior rim (B). Fenestrated atrial septal defect from left atrial aspect (C) and from right atrial aspect (D); in these cases, 2D echocardiography could potentially underestimate the size of the overall defect/shunt if the plane sampled is perpendicular to the small defect only (dotted arrow).

Figure 5

Anomalous coronary artery from the pulmonary artery (Ai and Aii) studied with computed tomography preoperatively. Post-operative computed tomography (Bi and Bii) shows patent left internal mammary graft to mid left anterior descending artery (images courtesy of Dr Mike Rubens). Ao, aorta; LAD, left anterior descending; LCA, left coronary artery; PA, pulmonary artery; RCA, right coronary artery.

Figure 6

Anomalous right pulmonary venous drainage to the inferior vena cava creating a curvilinear ‘scimitar’ silhouette (arrows) on chest X-ray (A) with corresponding images from computed tomography (B and C) (images courtesy of Dr Mike Rubens).

Figure 7

Contrast-enhanced cardiovascular magnetic resonance angiography (CE-CMRA) in an adult presenting with systemic hypertension due to severe aortic coarctation (A). Computed tomography imaging following endovascular stenting (B). Aneurysm related to endovascular stenting of coarctation studied with cine CMR (C; dotted arrow), and aneurysm related to Dacron patch coarctation repair studied with CE-CMRA (D; arrow).

Figure 8

Cardiovascular magnetic resonance (or computed tomography) may be useful in the diagnosis of sinus venosus defects, which can be at the orifice of the superior (or less commonly inferior caval veins) and to delineate anomalous pulmonary venous drainage. Cardiovascular magnetic resonance images showing sinus venosus atrial septal defect (asterisk) in (A) and the anomalous drainage of the right upper pulmonary vein to superior vena cava (B). IVC, inferior vena cava; LA, left atrium; RA, right atrium; RUPV, right upper pulmonary vein; PA, pulmonary artery; RCA, right coronary artery; SVC, superior vena cava.

Figure 9

Diastolic still image from cardiovascular magnetic resonance cine pre-pulmonary (A) and post-pulmonary (B) valve replacement for pulmonary regurgitation status post repaired tetralogy of Fallot. Reduction in RV volume and increased LV filling in (B). Late gadolinium enhancement cardiovascular magnetic resonance evidence of ventricular fibrosis/scarring is seen in (C); block arrows point to bright areas of scar in the right ventricular outflow tract and dotted arrows to the ventricular septal defect patch site. (D) Derived from 3D cardiovascular magnetic resonance acquisition after segmentation of chambers, outflows, and scar using Mimics, Materialise NV (courtesy of collaboration with Drs Veronica Spadotto and Jennifer Keegan). LV, left ventricle; RV, right ventricle.

Figure 10

Cardiac computed tomography in a patient with RV–PA conduit (A), showing virtually single origin coronary arteries passing between the aorta and narrow segment of conduit (B–D) (images courtesy of Dr Mike Rubens). Ao, aorta; Cx, circumflex; LAD, left anterior descending; PA, pulmonary artery; RCA, right coronary artery; RV, right ventricle.

Figure 11

Cardiovascular magnetic resonance images of simple transposition of the great arteries (TGA) (A and B) and congenitally corrected TGA (ccTGA) (C and D) showing parallel discordant outflows (A and C) and hypertrophic systemic RV (B and D). Late gadolinium enhancement in TGA with atrial redirection surgery (E) and in ccTGA (F); bright areas of enhancement within the RV (arrows) suggestive of fibrosis. Ao, aorta; LA, left atrium; LV, left ventricle; PA, pulmonary artery; PVAC, pulmonary venous atrial compartment; RV, right ventricle; RA, right atrium.

Figure 12

(A and B) Patent Fontan pathways status post-atriopulmonary Fontan. In patient (C), thrombus has formed (arrows) due to sluggish flow in the dilated right atrium. In (D), late gadolinium enhancement cardiovascular magnetic resonance evidence of rudimentary endocardial right ventricular fibrosis is seen (arrows). (E and F) Patent total cavopulmonary pathways (asterisks). Ao, aorta; IVC, inferior vena cava; LA, left atrium; LPA, left pulmonary artery; LV, left ventricle; RA, right atrium; RPA, right pulmonary artery; SVC, superior vena cava.