The Rastelli procedure is a complex reconstructive cardiac operation employed to correct certain forms of congenital heart disease that involve malposition of the great arteries and associated ventricular septal defects (VSD) with obstruction of the right ventricular outflow tract (RVOT). First described by the cardiac surgeon Tomas F. Rastelli in the late 1960s, the technique redirects left ventricular blood flow to the aorta through a VSD and creates a conduit to channel right ventricular blood to the pulmonary arteries.
Indications
The procedure is primarily indicated for:
- Transposition of the great arteries (TGA) with a large VSD and subpulmonary (infundibular) stenosis or atresia.
- Double‑outlet right ventricle (DORV) in which the aorta arises predominantly from the left ventricle but the VSD is not amenable to direct biventricular repair.
- Certain types of truncus arteriosus or complex single‑ventricle physiology when a biventricular repair is preferred.
Surgical technique
- Ventricular septal defect enlargement – The existing VSD is enlarged or a new opening is created to allow unobstructed left ventricular (LV) flow into the aorta.
- Intracardiac baffle formation – A patch (usually made of autologous pericardium or synthetic material) is sutured within the right ventricle to direct LV blood from the VSD to the aortic valve, effectively routing systemic circulation.
- RV‑to‑pulmonary artery conduit – Because the native RVOT is obstructed, a valved conduit (often a homograft or a prosthetic valved tube) is implanted between the right ventricle and the main pulmonary artery to re‑establish pulmonary blood flow.
- Closure of residual defects – Any remaining atrial or ventricular septal defects are closed, and the heart is weaned from cardiopulmonary bypass.
Variations and related procedures
- The modified Rastelli or Rastelli‑type repair incorporates advances such as the use of expanded polytetrafluoroethylene (ePTFE) conduits, which may have longer durability.
- In cases where the RV‑to‑PA conduit is expected to outgrow the patient, a staged approach may be planned, with conduit replacement later in childhood or adolescence.
- The Nikaidoh procedure and the LeCompte maneuver are alternative surgical strategies for similar anatomical lesions; they differ principally in the method of aortic translocation and RVOT reconstruction.
Outcomes and complications
- Early mortality rates in contemporary series range from 5 % to 10 % and are lower in high‑volume pediatric cardiac centers.
- Long‑term follow‑up identifies conduit degeneration, stenosis, or regurgitation as the most common late complications, often necessitating re‑intervention (surgical conduit replacement or percutaneous valve implantation).
- Other potential complications include arrhythmias, residual VSD, left ventricular outflow tract obstruction, and right ventricular dysfunction.
- Overall survival beyond 20 years after the operation exceeds 80 % in most reported cohorts, provided that conduit surveillance and timely re‑operations are performed.
Historical context
The operation is named after Dr. Tomas F. Rastelli, an Italian‑Brazilian cardiac surgeon who published the initial description of the technique in 1968 (J Thorac Cardiovasc Surg 1968;55:527‑534). His contribution provided a biventricular repair option for a subset of patients who previously required single‑ventricle palliation (e.g., the Fontan pathway).
Current usage
While advances in imaging, catheter‑based interventions, and alternative surgical repairs have refined patient selection, the Rastelli procedure remains a standard option in contemporary congenital cardiac surgery programs for anatomically suitable lesions. Ongoing research focuses on improving conduit longevity and reducing the need for re‑operation.