| Objective through retrospective study on patients of pulmonary atresia with ventricular septal defect, We classified this disease according to the blood supply of pulmonary circulation and the development and distribution of pulmonary artery. According to it's type,we took individualized treatment measures to the patient.We summarized the clinical experience for finding a suitable treatment and evaluated the treatment effect of various methods.Methods from April 2011 to December 2015, 71 cases of pulmonary atresia with ventricular septal defect were treated in our hospital. All patients were divided into 4 types according to the source of blood supply of pulmonary circulation and the development degree of the pulmonary artery. Type I:Patients without arterial duct and major aortopulmonary collateral arteries, It's true pulmonary arteries are hypoplastic.blood supply of pulmonary circulation comes from blood capillary proliferated from the parietal pleura and mediastinum; Type IIa:Patents have ductus arterioles and no major aortopulmonary collateral arteries, Their blood supply of pulmonary circulation is depended on arterial duct. the development of pulmonary artery is good, the pulmonary arteries is separated from the right ventricle by an imperforate membrane;Type IIb:The blood flow of pulmonary circulation of this type patients is depended on patent ductus arteriosus.Patients of this type have no major aortopulmonary collateral arteries and their left and pulmonary artery are well developed, and their proximal main pulmonary arteries atresia are like mouse tail.A section of fibrous tissue is lie between main pulmonary artery and right ventricle outflow. Type IIc:Patients of this type have patent ductus arterioles and no major aortopulmonary collateral arteries.Their pulmonary blood flow supplied by duct arteries. left and right pulmonary artery are dysplasia.Their proximal main pulmonary arteries are like mouse tail. A section of fibrous tissue is lie between main pulmonary artery and right ventricle outflow. Type IIIa:The blood flow of pulmonary circulation of this type patients is depended on patent ductus arteriosus.Patients of this type have no major aortopulmonary collateral arteries and their left and pulmonary artery are well developed.They have no main pulmonary arteries. Type IIIb:Patients of this type have patent ductus arterioles and no major aortopulmonary collateral arteries.Their pulmonary blood flow supplied by duct arteries. left and right pulmonary artery are dysplasia. They have no main pulmonary arteries.Type IVa:This type patients have no patent ductus arteriosus or have a small duct artery or have severe stenosis of the duct artery. Their pulmonary blood flow supplied by the major aortopulmonary collateral arteries. Their native pulmonary arteries are well developed because of good communication between native pulmonary arteries andmajor aortopulmonary collateral arteries. Type IVb:This type patients have no patent ductus arteriosus or have a small duct artery or have severe stenosis of the duct artery. Their pulmonary blood flow supplied by the major aortopulmonary collateral arteries or double supply. Their native pulmonary arteries are dysplasia. 5 cases among 71 cases is belong to type I,2 male and 3 female. Theyare aged from 6 months to 25 years old, and 7 years old on average. Their arterial oxygen saturation(Sa O2) is(47±4.2)%, hemoglobin(HB) is(198±17) g/L, Nakata index is(45±11) mm2/m2.All patients had excellent “arborization”.One patient did Melbourne shunt,the other 4 patients did Sano shunts, 3 cases had achieved surgical anatomic correction at two-stage operation. 1 case is waiting for the two-stage surgery. 3 cases among 71 cases is belong to type IIa,1male and 2 female.The age,respectively was 2months,4 months and 1 years old,3years old.The Sa O2,respectively was 81%,78% and 74%.The hemoglobin,respectively was126 g/l,131g/l and 148g/l, The Nakata index respectively was231 mm2/m2,215 mm2/m2 and 189 mm2/m2.All patients had achieved surgical anatomic correction at first stage operation. 26 cases among 71 cases was belong to type IIb,11 male and 15 female.They were aged from 1 month to 9 years old, and(2.4±2.0)years old on average.Their Sa O2 was(81.8±6.1)%, HB was(140.4±17.0) g/L, Nakata index was(191.1±23.4) mm2/m2.All patients had achieved surgical anatomic correction at first stage operation.4 cases among 71 cases is belong to type IIc,3 male and 1 female.They are aged from 2 months to 5 years old, and 0.9 years old on average.Their arterial Sa O2 is(67±3.9)%, HB is(174±11) g/L, Nakata index is(98±7) mm2/m2.All patients had done Sano shunt at first stage, surgical anatomic correction at second stage operation. 1 cases among 71 cases was belong to type IIIa, male, 2 months and 4 years old, weight 15.5Kg, Sa O278%, Hb136g/l, Nakata index 201mm2/m2, intracardiac malformation was like tetralogy of Fallot in pathological anatomy, He underwent one stage complete repair of cardiac malformation.2 cases among 71 cases was belong to type IIIb,1 male,7 months old,2famale,9 months old.The Sa O2,respectively was 61% and 59%.The hemoglobin,respectively was185g/l and 188g/l, The Nakata index respectively was 111 mm2/m2 and 97 mm2/m2. One patient did modified B-T shunt,the other patients did Sano shunt, 1 cases had achieved surgical anatomic correction at two-stage operation. 1 case is waiting for the two-stage surgery. 9 cases among 71 cases was belong to type IVa,5 male and 4 female.They were aged from 2 month to 6 years old, and(2.4±2.1)years old on average.Their Sa O2 was(84.3±5.3)%, HB was(113.9±16.5) g/L, Nakata index was(185.6±13.7) mm2/m2.All patients had achieved surgical anatomic correction at first stage operation. Type IVb 21 cases, 11 male, 10 female, age from 2 months to 11 years old,(3±2.7) years old on average, weight from 4.5Kg to 31 Kg,(12.8 + 6.2) Kg on average. All patient were divided into group A and B(Nakata index 40 mm2/m2 as the boundary), 16 cases in group A, male 7 cases, female 9, age 3 months to 6 years old,(2.7±1.8)years old on average, weight(12.9±4.3) kg, Sa O2(68.1±8.5)%, Hb(146.3±20.3)g/L, Nakata index(76.7±23.5) mm2/m2, in which 5 cases were surgical treatment withunifocalization procedure + Sano shunt and 3 cases had done complete repair at 2-stage operationg;in which 4 cases wereonly done Sano shunt,all of them had acceptedcomplete repair at 2-stage operation;in which 7 cases were done collateral arteries ligature orembolizationalone with Sano shunt, 5 cases had finished the second stage of complete repairmen and 2 patients is waiting for the second stage operation. Group B(n = 5), 4 male, 1 female, aged from 2 months to 11 years,(3.7±4.8) years old on everage, Sa O2(77.4±15.1)%, HB(147.6±53.3) g/L, Nakata index(25.4±8.8) mm2/m2, in which 2 patients had obstinate congestive heart failure and were done completerepairmen at 1-stage operation; 1 case had done unifocalization and Sano shunt,2 cases had done unifocalization and Melbourne shunt, in which 1 patient had received completerepairmen at 2-stage operation, 1 patient is waiting for the 2-stage operation.Results71 cases were treated with 92 times of surgical procedure.41 patients had done complete repairmen at 1-stage operation,1 patient died.30 patients had done all kinds of shunts procedure at 1-stage operation,2 patients died.20 patients had done complete repairmen at 2-stage operation,1 patient died.Left and right ventricular pressure ratio of all patients who underwent complete repairmentwas(44.5±12.8)%.Eight patients waiting for 2-stage operation don't come up to the standard of complete repairmen.They will be followed up continuously. According to the observation groups: group I: patients' postoperative Sa O2 increased to(69±2.4)%.1 patient aged 25 years old suffered massive hemorrhage of alimentary tractat the fifth day after Melbourne shunt, and died for hemorrhagic shock,The other 4 patients had done Sano shunt,Among which 3 patients underwent second stage complete surgical repair after(12±1.9) months.1 patient of which appeared cardiac arrest in the prone position, resuscitation, 3 patients all recovered smoothly. Patients of IIa group all survived, 1 patient occurred with postoperative pulmonary edema, supporting with ventilator, cure after peritoneal dialysis.The remaining patients recovered smoothly.Their Sa O2are(99.6±0.6)%.Shown by color Doppler ultrasound pulmonary valve have mild regurgitation in 2 cases and moderate regurgitation in 1 case, the rest of the valves were not seen more than mild regurgitation and residual shunt. Patients of IIb group all survived, 1 patient occurred with postoperative pulmonary edema, supporting with ventilator, cure after peritoneal dialysis. 1 case of lung infection was cured, 1 case of postoperative mediastinal infection was cured after reoperation for removal of pus and necrotic tissue, flushing and drainage.The remaining patients recovered smoothly.Their Sa O2 are(98.7±1.6)%.Shown by color Doppler ultrasound pulmonary valve velocity 3.4m/s in1 case, mild regurgitation in 2 cases,severeregurgitation of pulmonary valve and moderate regurgitation of tricuspid valve in 1 case, only tricuspid valve moderate regurgitation in 2 cases.The rest of the valves were not seen more than mild regurgitation and residual shunt. IIc patients' a postoperative Sa O2 increased to(82.6±3.9)%.Balloon dilatation of left pulmonary artery stenosis was done after 1-stage operation in 1 case.3 patients underwent 2-stage operation of complete repairmen after(12.5±2.7) months.They all recovered smoothly.1 patient is waiting for 2-stage operation yet.Patient of IIIa recovered smoothly.His pulmonary valve has mild regurgitation. 1 patient of IIIb group done B-T shunt suffered B-T vascular prosthesis blockage after 12 hours of operation.Emergency surgery was done to replace the vascular prosthesis, He is still waiting for the 2-stage operation.Another patientdone Sano shunt patients recovered smoothly, and underwent complete correction of malformation of heart after 11.5 months.She recovered smoothly after 2-stage operation.In IVa group, 2 patients suffered pulmonary exudation, Of which 1 cases with oliguresis were cured by peritoneal dialysis after operation,and 1 case appeared unexplained low cardiac output syndrome after 12 hours of operation, And we found that myocardial contractility severly decreased and extracorporeal circulation auxiliarydidn't work,then he died.The other patients recovered smoothly.Two cases,young in age,of IVb suffered congestive heart failure were treated with complete repairment,and recovered smoothly after operation.All the other patients did stagedoperation.Complications occurred with re-thoratomy for hemaostsis in 1 case, pulmonary infection in 1 case, bilateral lungs exudation in 1 case and vascular prosthesis of Sano procedure embolized and been replaced in 1 case.They all were cured.1 patient with unifocalization and Sano shunt occurred hyoxemia.His Sa O2 was decreased to 35%, He cann't been weanned from cardiopulmonary bypass.Then we altered procedure to systemic-to-pulmonary artery shunt.Butit still didn't work.Then he died due to hypoxia.The other patients recovered smoothly.With follow-up, except for 5 cases of patients waiting for 2-stage operation other patients all underwent 2-stage operation of complete repairmen.In which 2 patients occurred postoperative pulmonary infiltrates, and recovered after treatment.We corrected different degree stenosis of branch vesselin 11 cases.follow-up results: 64 cases were follow-uped in 71 patients, 3 patients lost to follow-up,follow-up rate was 95.3%, follow-up time was 1-56 months, According to follow-up,all patients' cardiac functions were in grade I to II(NYHA).The Sa O2 was(97.3±1.6)% in patients with complete repairment.1 patient recurred postoperative lung exudation and infection repeatedly, and cured after three months. there was no recurrent pulmonary infection again.2 patients occurred severe regurgitation of pulmonary valves. 3 patients occurred morderate pulmonary valve regurgitation.Pulmonary valve regurgitation of other patients are below mild that. Regurgitation of tricuspid valves are mild in 12 patients and morderate in 6 patients.Aortic valve regurgitation are mild in 4 patients. Velocity through pulmonary valve is(2.1±0.87) m/s. left ventricular ejection fraction(LVEF) is(63.0±3.4)%.Among the groups oxygen saturation and left ventricular ejection fraction have no significant difference. Sa O2 of 8 patients waiting for the 2-stage operation is(71.9±2.9)%.Pulmonary artery branch stenosis occure in 6 cases, Nakata index is(14.3±11.6) mm2/m2.Velocity throughvascular prosthesis of Sano shunt is(4.0±0.5)m/s.Conclusion We appropriately classified patients ofpulmonary atresia with ventricular septal defect according to the developmental condition of pulmonary artery and pulmonary circulation blood suppy.under the guidance of the principle of classification, we can obtain satisfactory therapeutic effect by taking appropriate individualized treatment according to the different types.Operation risk is increased in patients of PA-VSD with MAPCAs. for specific types of patients with PA-VSD/MAPCAs unifocalizationand complete repairment operation can achieve satisfactory results. |
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