Adult congenital heart disease
Tetralogy, transposition, Fontan, Ebstein, AV canal, ductus and a persistent left SVC — segmental anatomy first, then the haemodynamics.
Numbers to remember
- Repaired tetralogy: follow pulmonary regurgitation and RV size — CMR volumes decide valve replacement [25]
- In a systemic right ventricle (Mustard, congenitally corrected TGA), watch systemic AV-valve regurgitation and RV function [25]
- A dilated coronary sinus usually means a persistent left SVC — confirm with agitated saline from the left arm [25]
Questions echo must answer
Adult congenital heart disease needs a segmental approach: atrial situs, AV connections, ventriculo-arterial connections, then shunts, valves and outflow tracts [25].
- What is the anatomy, and what operation was done?
- Residual lesionsShunts, outflow obstruction, valve regurgitation, conduit or baffle problems [25].
- Ventricular functionOf the systemic and the subpulmonary ventricle.
- Pulmonary pressure[8,25].
Acquisition protocol
- SubcostalSitus and the atrial septum.
- Apical four-chamberOffset of the AV valves identifies the morphological tricuspid valve (more apical) and the right ventricle (moderator band).
- OutflowsWhich great artery arises from which ventricle; crossing or parallel.
- Colour and agitated salineShunts, baffle leaks, persistent left SVC [25].
Diagnosis & severity
| Lesion | Key echo finding |
|---|---|
| Tetralogy of Fallot (unrepaired) | Overriding aorta, large outlet VSD, RVOT obstruction, RV hypertrophy |
| Repaired tetralogy | Pulmonary regurgitation, RV dilatation, residual RVOT obstruction |
| Congenitally corrected TGA | AV and VA discordance; systemic RV with tricuspid regurgitation |
| Mustard/Senning | Systemic RV; baffle obstruction or leak |
| Fontan | Laminar low-velocity venous flow to the pulmonary arteries; single ventricle function |
| Ebstein anomaly | Apical displacement of the septal tricuspid leaflet, atrialised RV |
| AV septal defect | Common AV valve level, primum ASD and/or inlet VSD, cleft left AV valve |
| PDA | Continuous flow into the pulmonary artery |
| Persistent left SVC | Dilated coronary sinus |
Thresholds that change management
- Follow-up and intervention thresholds are lesion-specific and set by an ACHD centre; the ESC 2020 guideline lists them [25].
- Significant shunt with RV volume overload and acceptable PVR → closure [25].
- Severe PR with RV dilatation after tetralogy repair → pulmonary valve replacement [25].
Pitfalls & mimics
- Assuming normal anatomy In a scarred, operated heart the "LV" may be a right ventricle.
- Dilated coronary sinus mistaken for a pericardial effusion or a mass.
- Silent PDA found only with colour in the parasternal short axis.