Outcome of Staged Repair of Tetralogy of Fallot with Pulmonary Atresia and a Ductus-dependent Pulmonary Circulation: Should Primary Repair Be Considered?

Abstract

BackgroundThe tetralogy of Fallot (TOF) with pulmonary atresia (PA) and a ductus-dependent pulmonary circulation (no major aorto-pulmonary collateral arteries (MAPCAs)) has been treated with staged repair or primary repair depending on the preference of surgeons or institutions. We evaluated the 19-year outcome of staged repair for this anomaly to find out whether our surgical strategy should be changed.Materials and MethodsForty-four patients with TOF/PA with patent ductus arteriosus (PDA) who underwent staged repair from June 1991 to October 2010 were included in this retrospective study. The patients with MAPCAs were excluded. The average age at the first palliative shunt surgery was 40.8±67.5 days (range: 0~332 days). Thirty-one patients (31/44, 70%) were neonates. The average weight was 3.5±1.6 kg (range: 1.6~8.7 kg). A modified Blalock-Taussig (BT) shunt was performed in 38 patients, classic BT shunt in 4 patients, and central shunt in 2 patients. Six patients required concomitant procedures: pulmonary artery angioplasty was performed in 4 patients, pulmonary artery reconstruction in one patient, and re-implantation of the left pulmonary artery to the main pulmonary artery in one patient. Four patients required a second shunt operation before the definitive repair was performed. Thirty-three patients underwent definitive repair at 24.2±13.3 months (range: 7.3~68 months) after the first palliative operation. The average age at the time of definitive repair was 25.4±13.5 months (range: 7.6~68.6 months) and their average weight was 11.0±2.1 kg. For definitive repair, 3 types of right ventricular outflow procedures were used: extra-cardiac conduit was performed in 30 patients, trans-annular patch in 2 patients, and REV operation in 1 patient. One patient was lost to follow-up after hospital discharge. The mean follow-up duration for the rest of the patients was 72±37 months (range: 4~160 months).ResultsTen patients (10/44, 22.7%) died before the definitive repair was performed. Four of them died during hospitalization after the shunt operation. Six deaths were thought to be shunt-related. The average time of shunt-related deaths after shunt procedures was 8.7 months (range: 2 days~25.3 months). There was no operative mortality after the definitive repair, but one patient died from dilated cardiomyopathy caused by myocarditis 8 years and 3 months after the definitive repair. Five-year and 10-year survival rates after the first palliative operation were 76.8% and 69.1%, respectively.ConclusionThere was a high overall mortality rate in staged repair for the patients with TOF/PA with PDA. Majority of deaths occurred before the definitive repair was performed. Therefore, primary repair or early second stage definitive repair should be considered to enhance the survival rate for patients with TOF/PA with PDA.

Outcome of neonatal palliative procedure for pulmonary atresia with ventricular septal defect or tetralogy of Fallot with severe pulmonary stenosis: experience in a single tertiary center

“…Modified BT shunt placement is one of the systemic to pulmonary shunt methods that connects the innominate or subclavian artery to ipsilateral PA with a synthetic interposition graft. It is a traditional palliative procedure used prior to complete repair for maintaining proper pulmonary blood flow and PA growth in cyanotic heart disease group, including PA VSD and TOF with severe PS [ 4 ].…”

“…Although modified BT shunt placement is still widely used for patients with ductal dependent pulmonary circulation, various studies have reported the disadvantages of modified BT shunt placement. It may increase the incidence of hypoplasia and distortion in the adjoining PA, which can lead to PA stenosis or occlusion, and when the shunt size is too big, excessive pulmonary blood flow can increase the probability of heart failure, which may require the use of diuretics or other medications to treat heart failure [ 4 , 5 ]. In addition, overshunting and shunt thrombosis can increase interstage mortality between BT shunt operation and complete surgical repair [ 5 , 6 ], with interstage mortality for infants reported to range between 10% and 15% [ 11 ].…”

“…Recently, early primary complete repair was introduced to reduce ventricular hypertrophy-mediated risks related to traditional palliative procedures in the TOF patient group, if the size of pulmonary artery (PA) would allow it [ 3 , 4 ]. Early primary repair provides avoidance of shunt-related complications, early relief of hypoxia, and normal lung development [ 5 , 6 ].…”

Surgical repair of Tetralogy of Fallot following primary palliation: Right ventricular outflow track stenting versus modified Blalock-Taussig shunt

“…(MAPCA) are candidates for primary palliation as a first step to avert hypoxia, improvement of oxygen saturation and PAs development until the complete repair [2,7, 8, 9].…”

Systemic to Pulmonary Artery Versus Right Ventricular to Pulmonary Artery Shunts for Patients With Pulmonary Atresia, Ventricular Septal Defect, and Hypoplastic Pulmonary Arteries

“…Currently, the treatment for pulmonary atresia with ventricular septal defect (PA/VSD) remains a challenge because of the heterogeneity of their pulmonary arteries and major aortopulmonary collaterals (MAPCAs) . Systemic‐pulmonary shunts (SPS) and right ventricular to pulmonary artery connection (RV‐PA connection) are the main procedures to promote pulmonary artery growth .…”