CHD affecting the RV Indication for intervention or
medical therapy
Contraindication or pitfalls
Atrial septal defects Closure in the presence of:
- right atrial or RV enlargement (I)
- paradoxical embolism (IIa)
- document orthodoxia or platypnea (IIa)
- if tricuspid valve surgery planned (IIa)
Patients with severe “irreversible” PH
PAP > 2/3 systemic pressures
PVR > 2/3 of SVR Failure occlusion test of defect
Ventricular septal defects Closure if
- Qp/Qs > 2 and signs of LV volume
overload (I)
-Qp/Qs > 1.5 and reversible PH (IIa) or LV systolic or diastolic dysfunction (IIa)
-history of infective endocarditis (I)
Patients with severe “irreversible” PH
cf criteria above
RV outflow tract obstruction Balloon valvotomy for a domed valve if: -asymptomatic with peak Doppler PG > 60mmHg or mean PG > 40 mmHg and less than moderate PR (I)
- symptomatic with peak Doppler PG > 50mmHg or mean PG > 30 mmHg (I) For a dysplasic valve, same criteria but (IIb) recommendation
Balloon valvotomy not recommended if - peak Gradient by Doppler < 50 mmHg in the presence of normal - PS with severe PR - symptomatic patients with peak
gradient < 30 mmHg.
Tetralogy of Fallot Surgery for adults with previous repair of TOF
- symptomatic severe PR (I)
- severe PR with RV enlargement or dysfunction (IIa)
- severe TR (IIa)
- significant residual RVOT stenosis (IIa)
- sustained arrhythmias (IIa)
- residual VSD or severe AR (IIa)
Always ascertain coronary anatomy before surgery Never forget to stratify the risk of sudden death in patients with TOF
Ebstein Anomaly Tricuspid valve repair and closure ASD if
- symptomatic (I)
- cyanosis (I)
- progressive RV dilatation or RV
dysfunction (I)
- paradoxial embolism (I)
Always consider ventricular pre-excitation
ccTGA Moderate or progressive AV valve regurgitation
Refer to guidelines for more specific consideration
Careful use of AV nodal blocking agents
*This table is not intended to summarize the guidelines but mainly as a guideline focusing on criteria of intervention.15 Surgical interval also depends on specific anatomical criteria and should always be clearly individualized. The recommendation in parenthesis are those of the American Heart Association guideline consensus with a class I recommendation being considered beneficial, a class IIa, considered probably beneficial and a class IIb recommendations where the risk benefit ratio is less well established. This table focusses on management of adults with CHD. Most of these recommendations are also valid for patients in childhood.
AV: atrioventricular
PG: pulmonary gradient
Table 2: Management of selected CHD affecting the right heart*.