Outcome of Staged Repair of Tetralogy of Fallot with Pulmonary Atresia and a Ductus-dependent Pulmonary Circulation: Should Primary Repair Be Considered?원문보기
Kim, Hyung-Tae
(Department of Thoracic and Cardiovascular Surgery, Pusan National University Yangsan Hospital)
,
Sung, Si-Chan
(Department of Thoracic and Cardiovascular Surgery, Pusan National University Yangsan Hospital)
,
Chang, Yun-Hee
(Department of Thoracic and Cardiovascular Surgery, The Catholic University of Korea Seoul St. Mary's Hospital)
,
Jung, Won-Kil
(Department of Thoracic and Cardiovascular Surgery, Pusan National University School of Medicine)
,
Lee, Hyoung-Doo
(Department of Pediatrics, Pusan National University Yangsan Hospital)
,
Park, Ji-Ae
(Department of Pediatrics, Pusan National University Yangsan Hospital)
,
Huh, Up
(Department of Thoracic and Cardiovascular Surgery, Pusan National University Yangsan Hospital)
Background: The 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 1...
Background: The 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 Methods: Forty-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 $40.8{\pm}67.5$ days (range: 0~332 days). Thirty-one patients (31/44, 70%) were neonates. The average weight was $3.5{\pm}1.6$ 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 $24.2{\pm}13.3$ months (range: 7.3~68 months) after the first palliative operation. The average age at the time of definitive repair was $25.4{\pm}13.5$ months (range: 7.6~68.6 months) and their average weight was $11.0{\pm}2.1$ 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 $72{\pm}37$ months (range: 4~160 months). Results: Ten 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. Conclusion: There 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.
Background: The 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 Methods: Forty-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 $40.8{\pm}67.5$ days (range: 0~332 days). Thirty-one patients (31/44, 70%) were neonates. The average weight was $3.5{\pm}1.6$ 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 $24.2{\pm}13.3$ months (range: 7.3~68 months) after the first palliative operation. The average age at the time of definitive repair was $25.4{\pm}13.5$ months (range: 7.6~68.6 months) and their average weight was $11.0{\pm}2.1$ 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 $72{\pm}37$ months (range: 4~160 months). Results: Ten 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. Conclusion: There 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.
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문제 정의
However, staged repair has several disadvantages: longer exposure to hypoxemia, abnormal growth of pulmonary alveoli and vascular structures, pulmonary artery distortion, right ventricular hypertrophy or even fibrosis due to pressure and volume load on the right ventricle, and a significant attrition rate due to the shunt problem between stages. The aim of this study is to determine whether our surgical policy for the TOF with PA and ductus-dependent pulmonary circulation should be changed based on the results of staged repair over the last 19 years.
제안 방법
Four patients required another systemic-pulmonary shunt or revision before the definitive repair was performed. Three of them required a second procedure due to hypoplastic LPA: one underwent an LMBT shunt as a second shunt operation at 17.4 months after the first shunt operation, and the other two patients underwent a shunt revision (change) with LPA angioplasty at 5 months and 8 months after the first shunt operation respectively due to re-stenosis of the concomitant LPA angioplasty site of the first RMBT shunt procedure. The remaining one patient was the case with absence of intrapericardial pulmonary artery.
대상 데이터
A retrospective study was conducted based on the medical records of 44 patients with TOF/PA with PDA treated with staged repair in the Department of Thoracic and Cardiovascular Surgery at Dong-A University Hospital and Pusan National University Hospital between June 1991 and October 2010. The patients with major aorto-pulmonary collateral arteries (MAPCAs) were excluded.
Ventricular septal defect (VSD) patch fenestration was required in 2 patients. Polystan porcine pericardium valved conduits were used in 10 patients, Selhigh porcine valved conduits in 4 patients, and homemade Gore-Tex valved conduits in 11 patients (Table 2). Various sizes of conduits were used: 14 mm in 2 patients, 16 mm in 11 patients, and 18 mm in 12 patients.
One of them underwent a central shunt and reimplantation of the LPA for subcarinal LPA sling with left main bronchus stenosis. She was a premature low birth weight baby (1,190 g at birth, and 2,100 g at operation). Soon after arrival at the intensive care unit (ICU) after surgery, bradycardia and hypotension developed and in the end she died despite cardio-pulmonary resuscitation (CPR).
One patient had absence of intrapericardial pulmonary arteries with bilateral PDA and situs inversus. The main pulmonary artery and pulmonary annulus could be inspected in 32 patients. Among them, thirty patients had a main pulmonary artery and 27 of these 30 patients had no pulmonary valve.
Polystan porcine pericardium valved conduits were used in 10 patients, Selhigh porcine valved conduits in 4 patients, and homemade Gore-Tex valved conduits in 11 patients (Table 2). Various sizes of conduits were used: 14 mm in 2 patients, 16 mm in 11 patients, and 18 mm in 12 patients. Four patients required an RVOT conduit change at 54, 61, 84, and 100 months after the definitive repair (Table 3).
이론/모형
All results are expressed as means±standard deviations and the Kaplan-Meier model was used to analyze the survival rate and freedom from extra-cardiac conduit change.
성능/효과
One patient died from dilated cardiomyopathy caused by myocarditis at 8 years and 3 months after the definitive repair. In the 25 patients who had undergone the extra-cardiac conduit technique at our center, freedom from extra-cardiac conduit change was 94.7% at 5 years and 64.6% at 10 years (Fig. 1). Overall, the five-year survival rate was 76.
참고문헌 (16)
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