Rastelli Procedure for Double Outlet Right Ventricle With Pulmonary Atresia

We present to you a complete repair of double outlet right ventricle (DORV) with pulmonary atresia (precisely a single outlet) in an 8-year-old girl. This is a demonstration of the Rastelli procedure. The patient was palliated with a central shunt followed by stent placement in a right sided major aortopulmonary collateral artery (MAPCA) which was further dilated and restented but the patient never underwent complete repair.

She presented with cyanosis, polycythemia, and recurrent respiratory tract infection. Preoperative assessment confirmed the presence of a DORV with pulmonary atresia and subaortic ventricular septal defect (VSD), bilateral superior venae cave with no bridging vein. The ascending aorta was quite large with right aortic arch and mirror image branching. There was an additional small apical muscular VSD. CT scan confirmed the anatomy and the right main branch pulmonary artery and its upper lobar branch appeared to be obstructed by the previously placed stent. No flow was visualized in the central shunt. Peripheral pulmonary arteries were satisfactory. The patient underwent preoperative catheterization to assess her suitability for complete repair, and to evaluate the possibility of device closure of the apical VSD.

A repeat median sternotomy was performed, and cardiopulmonary bypass was initiated via aortic and tricaval cannulation. The branch pulmonary arteries were controlled to avoid steal. The MAPAC was transected and the stent was extracted. The central pulmonary arteries were augmented with a pulmonary homograft. The heart was then arrested and a right ventriculotomy was created. Rastelli baffle was created using a large bovine pericardial patch. An aortic homograft was then used to restore the right ventricular to pulmonary confluence continuity.

The patient was weaned off cardiopulmonary bypass without difficulty. Post bypass transesophageal echocardiography showed widely patent right and left ventricular outflow tracts and no baffle leak. The apical VSD did not seem to be of significance. Hemodynamic measurements were satisfactory.

The patient was extubated on the first postoperative day and the remaining postoperative course was uneventful. Pre-discharge echocardiogram and CT scan showed good flow in branch pulmonary arteries, good biventricular functions, and satisfactory conduit position with a mean gradient of 15 mmHg across the left ventricular outflow tract.

References

1. Rastelli GC, McGoon DC, Wallace RB. Anatomic correction of transposition of the great arteries with ventricular septal defect and subpulmonary stenosis. J Thorac Cardiovasc Surg. 1969 Oct;58(4):545-52
2. Huang ES, Herrmann JL, Rodefeld MD, Turrentine MW, Brown JW. Rastelli Operation for D-Transposition of the Great Arteries, Ventricular Septal Defect, and Pulmonary Stenosis. World J Pediatr Congenit Heart Surg. 2019 Mar;10(2):157-163
3. Barron DJ, Mehsood DK, Kutty RS, Stickley J, Botha P, Khan NE, Jones TJ, Brawn WJ. Fate of the Left Ventricular Outflow Tract After Rastelli With Selective Infundibular Muscle Resection. Ann Thorac Surg. 2019 Apr;107(4):1226-1231
4. Van Praagh R, Pérez-Trevino C, Reynolds JL, Moes CA, Keith JD, Roy DL, Belcourt C, Weinberg PM, Parisi LF. Double outlet right ventricle (S,D,L) with subaortic ventricular septal defect and pulmonary stenosis. Report of six cases. Am J Cardiol. 1975 Jan;35(1):42-53

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