Tetralogy of Fallot(TOF)with total anomalous pulmonary vein connections(TAPVC)is a rare type of complex congenital heart disease among all TOF cases.Co-presentation of major aortopulmonary collateral arteries(MAPCAs)c...Tetralogy of Fallot(TOF)with total anomalous pulmonary vein connections(TAPVC)is a rare type of complex congenital heart disease among all TOF cases.Co-presentation of major aortopulmonary collateral arteries(MAPCAs)compensates for the lack of central pulmonary bloodflow and decreases the severity of right-to-left shunting in TOF.We present a case of a 2-year-old child with complex diagnoses of TOF,TAPVC,a large secun-dum atrial septal defect(ASD),and intraoperatively identified MAPCAs.She underwent surgery to repair the TAPVC,valve-sparing reconstruction of the right ventricular outflow tract,interventricular defect closure,and the creation of patent foramen ovale(PFO).After the operation,hemodynamic instability happened along with sudden blood pressure drop,desaturation,and increased central venous pressure,which subsided after adminis-tering inhalational nitric oxide(NO).A postoperative pulmonary hypertension crisis was suggested when the patient experienced recurrent symptoms after the termination of NO.Echocardiographicfindings of a D-shaped left ventricle(LV),right-to-left PFO shunt and high tricuspid valve gradientfirmly established the diagnosis.It was subsequently managed with continuous NO inhalation and sildenafil,which rendered a satisfactory outcome.Repaired TOF and TAPVC could be at particular risk of developing pulmonary hypertension crisis,especially in the presence of MAPCAs due to possible remodeling of the pulmonary vasculature.Furthermore,a relatively non-compliant LV function and small left atrial size may exacerbate the risk of developing postcapillary pulmonary hypertension after TAPVC repair.A successful postoperative outcome calls for a meticulous preoperative analysis of the anatomical lesions,as well as careful monitoring.展开更多
Objective To evaluate surgical methods and results of extracardiac conduit total cavopulmonary anastomosis (EC-TCPA) without cardiopulmonary bypass (CPB). Methods From May 2000 to April 2003, 11 patients with function...Objective To evaluate surgical methods and results of extracardiac conduit total cavopulmonary anastomosis (EC-TCPA) without cardiopulmonary bypass (CPB). Methods From May 2000 to April 2003, 11 patients with functional univentricle underwent off-pump EC-TCPA (no-CPB group). Their postoperative outcome was retrospectively compared with a 17-patient group who underwent EC-TCPA with cardiopulmonary bypass (CPB group) over a concurrent time period. Results There was 1 operative death in no-CPB group and 2 in CPB group; early postoperative hemodynamics appe-ared to significantly improve in no-CPB group. Blood and platelet transfusions decreased and blood plasma transfusion significantly lowered in no-CPB group compared with CPB group (P = 0.036). Postoperative courses of patients in no-CPB group were smooth and event free, and extubation time was substantially short. Intensive cares unit stay (P = 0.04) and hospital stay (P = 0.02) postoperation were significantly shorter, hospital costs were significantly reduced (P = 0.004) in no-CPB group compared with CPB group. Conclusions EC-TCPA without use of CPB is not a difficult procedure; the procedure results in improvement in postoperative hemodynamics, and decreased use of blood and blood products. It is a more efficient operation with more short recovery time and reduced hospital stay.展开更多
基金The report was conducted in accordance with the Nuremberg Code and Declaration of Helsinki,and the protocol was approved by the Institutional Review Board of National Cardiovascular Center Harapan Kita(No.LB.02.01/VII/037/KEP037/2022).
文摘Tetralogy of Fallot(TOF)with total anomalous pulmonary vein connections(TAPVC)is a rare type of complex congenital heart disease among all TOF cases.Co-presentation of major aortopulmonary collateral arteries(MAPCAs)compensates for the lack of central pulmonary bloodflow and decreases the severity of right-to-left shunting in TOF.We present a case of a 2-year-old child with complex diagnoses of TOF,TAPVC,a large secun-dum atrial septal defect(ASD),and intraoperatively identified MAPCAs.She underwent surgery to repair the TAPVC,valve-sparing reconstruction of the right ventricular outflow tract,interventricular defect closure,and the creation of patent foramen ovale(PFO).After the operation,hemodynamic instability happened along with sudden blood pressure drop,desaturation,and increased central venous pressure,which subsided after adminis-tering inhalational nitric oxide(NO).A postoperative pulmonary hypertension crisis was suggested when the patient experienced recurrent symptoms after the termination of NO.Echocardiographicfindings of a D-shaped left ventricle(LV),right-to-left PFO shunt and high tricuspid valve gradientfirmly established the diagnosis.It was subsequently managed with continuous NO inhalation and sildenafil,which rendered a satisfactory outcome.Repaired TOF and TAPVC could be at particular risk of developing pulmonary hypertension crisis,especially in the presence of MAPCAs due to possible remodeling of the pulmonary vasculature.Furthermore,a relatively non-compliant LV function and small left atrial size may exacerbate the risk of developing postcapillary pulmonary hypertension after TAPVC repair.A successful postoperative outcome calls for a meticulous preoperative analysis of the anatomical lesions,as well as careful monitoring.
文摘Objective To evaluate surgical methods and results of extracardiac conduit total cavopulmonary anastomosis (EC-TCPA) without cardiopulmonary bypass (CPB). Methods From May 2000 to April 2003, 11 patients with functional univentricle underwent off-pump EC-TCPA (no-CPB group). Their postoperative outcome was retrospectively compared with a 17-patient group who underwent EC-TCPA with cardiopulmonary bypass (CPB group) over a concurrent time period. Results There was 1 operative death in no-CPB group and 2 in CPB group; early postoperative hemodynamics appe-ared to significantly improve in no-CPB group. Blood and platelet transfusions decreased and blood plasma transfusion significantly lowered in no-CPB group compared with CPB group (P = 0.036). Postoperative courses of patients in no-CPB group were smooth and event free, and extubation time was substantially short. Intensive cares unit stay (P = 0.04) and hospital stay (P = 0.02) postoperation were significantly shorter, hospital costs were significantly reduced (P = 0.004) in no-CPB group compared with CPB group. Conclusions EC-TCPA without use of CPB is not a difficult procedure; the procedure results in improvement in postoperative hemodynamics, and decreased use of blood and blood products. It is a more efficient operation with more short recovery time and reduced hospital stay.
