Medical, Interventional, and Surgical Management
Choosing the tool
Management decisions turn on lesion, residual hemodynamics, ventricular function, and symptoms. Transcatheter options include device closure of secundum atrial septal defects, patent ductus arteriosus, and selected VSDs; balloon valvuloplasty for pulmonary and aortic stenosis; and stent or transcatheter valve implantation in right ventricular–pulmonary artery conduits. Surgery remains preferred for many baffles, complex anatomy, and when device closure would leave inadequate rims.
Medical therapy
Evidence in ACHD is thin and extrapolated. Anticoagulation in Fontan palliation (aspirin or warfarin) targets thromboembolism; pulmonary vasodilators are used in Fontan failure and pulmonary hypertension. Heart failure regimens are individualized. Rhythm control, ablation, and device therapy matter as much as drugs.
Endocarditis prophylaxis
Restrict prophylaxis to prosthetic valves or material, prior infective endocarditis, unrepaired cyanotic CHD, and repaired lesions with residual defects at or adjacent to prosthetic material. Native, uncomplicated VSD or atrial septal defect does not require prophylaxis.
Reintervention thresholds
Pulmonary regurgitation after tetralogy repair is the paradigm: symptoms, RV dilation, QRS prolongation, or arrhythmia prompt valve replacement, ideally before irreversible RV dysfunction.
Traps
Offering device closure for a defect without adequate rims. Prescribing prophylaxis for an uncomplicated native defect. Assuming adult heart failure medications have proven benefit. Ignoring that pregnancy planning, arrhythmia, and aortic disease often drive care more than the index lesion.
Rule of thumb
Ask whether the patient is symptomatic, whether the ventricle is under threat, and whether the residual lesion is treatable by catheter or requires surgery - then act before irreversible injury.