Imaging of ventricular septal defect: Native and post-repair

Abstract

Cardiac imaging using a combination of multiple modalities has become an essential tool in the management of adults with congenital heart disease with the potential to improve the outcome. Knowledge of the potential advantages and limitations of each modality allows their correct use to ensure timely diagnosis and a patient-tailored treatment approach in a variety of clinical settings. We provide herewith an expert consensus on the role of cardiovascular imaging in the assessment of ventricular septal defect and its hemodynamic consequences in adults.

Keywords: Adult congenital heart disease; Cardiovascular resonance; Echocardiography; Multimodality imaging; VSD; Ventricular septal defect.

Fig. 1

Diagram illustrating the anatomy of the right ventricle (A) and the classification of VSDs based on the margins and localization in the right ventricle (B). From Jacobs JP, Burke RP, Quintessenza JA et al. Congenital heart surgery nomenclature and database project: ventricular septal defect. Ann Thorac Surg. 2000; 69:S25–S35.).

Fig. 2

TTE views to assess VSDs. A central perimembranous VSD is showed in parasternal short axis view (A) and 5-chamber view (B). Panel C demonstrates a doubly-committed VSD: proximity of the defect to the pulmonary valve (yellow arrow) should be noted. Panel D shows a parasternal long-axis view of a perimembranous VSD with septal malalignment. E) and F) illustrates muscular VSDs as visualized from a short axis at the ventricle level and an apical muscular defect on off-axis TTE view. G) Right oblique subcostal view of a perimembranous defects. H) Gerbode type defect: the 4-chamber view with colour Doppler shows the blood shunt from the LV into the RA. Asterisks ​= ​VSD, Ao ​= ​aorta, LV ​= ​left ventricle, PA ​= ​pulmonary artery, RA ​= ​right atrium, RV ​= ​right ventricle.

Fig. 3

Doppler assessment and role of CMR. Panels A,B,D and E show diastolic function assessment on TTE and VSD Doppler profile in the same patient. Continous-wave Doppler demonstrates high-velocity left-to-right shunt during systole with a persisting flow occurring during diastole (yellow arrow) (A), in the context of increased left ventricular end-diastolic pressure, as revealed by increased left atrial volume (B), pulsed-wave mitral flow pattern (D), and tissue Doppler analysis (E). Panels C illustrates a central perimembranous VSDs as visualized by CMR. In panel D there is a CMR image of a muscular VSD. Asterisks ​= ​VSD, Ao ​= ​aorta, LV ​= ​left ventricle, RV ​= ​right ventricle.

Fig. 4

Added value of novel echocardiographic techniques. Panel A shows a dilated LV with normal systolic function on 3D TTE, however, the same patient showed subclinical signs of impaired systolic and diastolic function as demonstrated by reduced LV global longitudinal strain (B) and reduction in the 3 components of LA function assessed by atrial strain (C). Panel D shows a muscular VSD with a funnel shape on 3D TTE, which allows to evaluate accurately the course of the defect in the ventricular septum as well as to visualize the shape and size of the defect “en-face” from the RV perspective (E). Panel F shows 3D TTE with colour Doppler of a perimembranous VSD, as seen from the LV perspective.

Fig. 5

Role of TOE. A) Inlet VSD visualized on mid oesophageal view at 0°, B) Apical muscular VSD showed at 120°. Panels C,D,F,G show intraprocedural images during VSD transcatheter closure. VSD sizing using 3D images with en face visualization of the defect (C). In panel D we can observe the wire passing across a muscular defect (yellow arrow) in transgastric view at 0°. Pericardial effusion (D, yellow arrow), device embolization (in panel E the yellow arrow demonstrates device embolization into the LV) and residual shunt (in panel F the yellow arrow points to a periprosthetic residual shunt). Asterisks ​= ​VSD, Ao ​= ​aorta, LA ​= ​left atrium, LV ​= ​left ventricle, RV ​= ​right ventricle.

Fig. 6

Aortic valve dysfunction. A) TTE parasternal short axis view demonstrating prolapse of the right coronary cusp (yellow arrow) with associated aortic regurgitation in a patient with perimembranous VSD. B) Zoomed parasternal long-axis TTE view clearly demonstrates a fibrotic valve with an eccentric jet of regurgitation directed posteriorly, suggesting prolapse of the right or non-coronary cusp. C) TOE image on mid-oesophageal view at 120° in a patient with perimembranous VSD, showing a subaortic ridge (yellow arrow), which causes LV outflow obstruction as demonstrated by colour flow Doppler. Asterisks ​= ​VSD, Ao ​= ​aorta, LV ​= ​left ventricle, RV ​= ​right ventricle.

Fig. 7

Double chambered right ventricle. Panel A) Right oblique subcostal view showing an anomalous muscular bundle (yellow arrow) in the RV infundibulum, causing an accelerated anterograde flow with a peak gradient of 73 ​mmHg measured with CW Doppler (B). In panel C we can observe a CMR short-axis view of the same patient, in which the DCRV jet is visible. At the bottom of the figure, another case of DCRV is reported: Panel D shows clearly a hypertrophied muscular bundle (yellow arrow) in the RV infundibulum. This structure is not well visualized in left oblique subcostal view, which only captures the accelerated forward flow during systole (E). The pulmonary regurgitation jet is used as a reference for the pulmonary valve site allowing differential diagnosis with pulmonary valve stenosis (F). LV ​= ​left ventricle, PA ​= ​pulmonary artery, PV ​= ​pulmonary valve.

Fig. 8

Postoperative follow-up. A) TOE RV inflow-outflow view shows a gross vegetation (yellow arrow) attached to the VSD closure patch (asterisk). Colour flow Doppler demonstrates a residual VSD (asterisk) close to the area of the vegetation. B) TTE parasternal short axis view showing partial detachement (yellow arrow) of the VSD patch (asterisk) resulting in a residual shunt at the distal edge of the patch. C) Zoomed 5-chamber TTE view demonstrating an aneurysmal VSD patch (asterisk) causing aortic valve cusp prolapse giving rise to an eccentric jet of aortic regurgitation. D) Severe tricuspid regurgitation following VSD surgical closure is demonstrated by EROA measurement. 3D TTE with tricuspid valve view from the RV perspective allows visualization of the coaptation gap (yellow arrow) secondary to tethering of septal leaflet. Asterisks ​= ​VSD/VSD patch, A ​= ​anterior leaftlet of tricuspid valve, Ao ​= ​aorta, LA ​= ​left atrium, LV ​= ​left ventricle, P ​= ​posterior leaflet of tricuspid valve, RA ​= ​right atrium, RV ​= ​right ventricle, S ​= ​septal leaflet of tricuspid valve.