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Objective:To explore the value of current indications for fetal pulmonary valvuloplasty (FPV) by summarizing the postnatal diagnosis, treatment, and prognosis of fetuses with pulmonary atresia with intact ventricular septum (PA/IVS) and right ventricular hypoplasia (RVH).Methods:This prospective study was conducted at the Heart Center of Women and Children's Hospital, Qingdao University from September 2018 to March 2021, which included pregnant women who were (1) with fetal PA/IVS and RVH; (2) unable to receive FPV due to fetal position or gestational age despite the indications; (3) given integrated pre- and postnatal management. Prenatal fetal echocardiography assessment, postnatal diagnosis, treatment, and follow-up were summarized using Wilcoxon matched-pair signed-rank test.Results:A total of 35 singleton pregnant women were diagnosed with fetal PA/IVS and RVH by ultrasonic cardiogram and admitted during the study period. Among the 28 fetuses meeting the FPV indications, 18 underwent FPV, while the other 10 did not due to inappropriate fetal position or gestational age. After excluding four terminated pregnancies, the rest six cases were enrolled. The median gestational age at the initial prenatal fetal echocardiography diagnosis was 28.9 weeks (28.3-30.4 weeks). Compared with the initial evaluation, the fetal right ventricular to left ventricular length/diameter ratio [0.8 (0.6-0.9) vs 0.6 (0.5-0.8)] and tricuspid regurgitation velocity [4.7 m/s (3.2-5.1 m/s) vs 4.1 m/s (3.3-4.8 m/s)] were increased, while tricuspid valve Z value [－0.8(－1.6-0.8) vs 0.4 (－0.3-1.9)] and single-ventricular predictive score [0.5 (0.0-2.0) vs 2.0 (1.0-3.0)] were decreased when re-evaluated six weeks later ( T were－2.21, 2.00,－2.20, and 2.00; all P<0.05). All of the six fetuses were born alive with a median gestational age of 38.9 weeks (37.3-40.1 weeks). The median weight was 3 425 g (3 100-4 160) g after being transferred to cardiac intensive care unit. The median age was 12.5 d (0.0-20.0 d) at the first surgical intervention. The median follow-up duration was 15 months (11.8-18.5 months). At initial diagnosis, the single-ventricular predictive score was 1-2 points in four fetuses, and =3 points in two fetuses. There was no death during follow-up. Four patients achieved anatomical biventricular circulation, one achieved clinical biventricular circulation, and one still needed further follow-up, with single-ventricular predictive score at initial diagnosis of 1-3, 3, and 2 points, respectively. Conclusions:The prognosis is good in fetuses with PA/IVS and RVH who have FPV indications but do not receive intrauterine intervention, which suggests that the current FPV indications may be too broad, and a more suitable FPV indication need to be further explored given the difficulty of implementing FPV.
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Objective:Postoperative venous obstruction (PVO) is the most severe complication of total anomalous pulmonary venous connection (TAPVC), and facing challenging re-intervention with high mortality. We aimed to review and analyze the follow-up and management of postoperative PVO in our center.Methods:We conducted a retrospective study of the patients with isolated TAPVC admitted in our center from October 2013 to October 2019. All available data and images of PVO patients were reviewed, such as the initial perioperative medical records, patients’ follow-up records, results of patients’ echo and CT angiography. Re-intervention including hybrid technique, sutureless technique, and patch augmentation, were carried out for postoperative PVO patients. The results were reviewed and analyzed to find the risk factors for adverse prognosis.Results:A series of 174 isolated TAPVC patients were admitted in our center and 169 received surgical treatment and 26 (26/169, 15.4%) had postoperative PVO. The diagnosis was made at a median time of 11.5 (0-77) weeks after initial operation and within 6 months of surgery in 22 (22/26, 84.6%) of the 26 patients. The subtype of TAPVC patients with postoperative PVO were: supracardiac 11 cases (11/26, 42.3%), cardiac 7 cases (7/26, 26.9%), infracardiac 5 cases (5/26, 19.2%), and mixed 3 cases (3/26, 11.5%). Bilateral obstruction and stenosis with diffusely small pulmonary veins were in 12 (12/26, 46.2%) and 3 cases (3/26, 11.5%) respectively. PVO progressed to worse condition in all the 26 cases during follow-up period. 8 (8/26, 30.8%) postoperative PVO patients underwent 10 re-interventions: one cases had 3 re-interventions. Five-year survival for patients with postoperative PVO was worse than those without postoperative PVO ( HR=6.46, 95% CI: 2.34-17.85, P<0.01). Risk factors for death or re-intervention in postoperative PVO patients were earlier presentation after TAPVC repair ( HR=0.85, 95% CI: 0.73-0.99, P=0.04) and an increased number of lung segments affected by obstruction ( HR=1.74, 95% CI: 1.01-2.99, P=0.04). Conclusion:Risk factors for death or re-intervention in postoperative PVO patients were earlier presentation after TAPVC repair and an increased number of lung segments affected, which should be focused on during strict follow-up period. Early re-intervention should be taken before irreversible secondary changes occur in these patients.
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Objective@#To reviewe our clinical experience on biventricular surgical repair of Swiss-cheese ventricular septal defects with two-patch and right ventricle apex excluding technique in 9 infants.@*Methods@#From March 2014 to March 2019, a series of 9 patients(M/F=2/7) with Swiss-cheese ventricular septal defects were admitted in our center to receive surgical treatment, with a median age of 4.8(2-12) months and a median body weight of 4.5(3.7-6.8) kg. All the 9 cases were symptomatic with shortness of breath and sweating. All the patients received echocardiography, ECG and chest X-ray. 3 patients with other intracardiac anomalies received CT angiography and 1 with severe pulmonary hypertension had transcatheter angiography. 4 cases of 9 had antenatal echocardiography but with no positive findings. The median cardiothoracic ratio was 0.63(0.58-0.72). 8 cases underwent one-stage surgical repair with the two-patch and right ventricle apex excluding technique and 1 case received a second-stage surgical repair with the same technique following a previous pulmonary artery banding procedure. The surgical repair was carried out with cardiopulmonary bypass under moderate hypothermia and using HTK cardioplagia to stop the heart in all the 9 cases. 2 fresh autologous pericardium patchs were used to closure defects of the outflow tract area and the apex trabecular defects respectively by excluding the apex of the right ventricle from the right ventricular inflow. Other intracardiac anomalies were corrected simultaneously. Patients were strictly followed up with a stanard protocol.@*Results@#All the operations were successful. Median cardiopulmonary bypass time and aortic clamping time were 96(68-167)min and 68(43-122)min respectively. Delayed chest closure were done in 2 cases within 48-72 hours postoperatively. The time of mechincal ventilation and ICU stay were 131.3(32-328)hours and 8.7(5-31)days respectively. All the patients were discarged in 11.5(9-42)days after operation. There was no mortality and major complication except for 1 case of low cardiac output syndrome, 2 cases of ventilator associated pnumonia and 2 cases of residual shunt(less than 2 mm). All the patients were followed up for 3.2 years(1 month-9 years). There was no death and major complication. The latest echocardiography results showed that the left and right heart function was normal in all the cases.@*Conclusion@#Biventricular surgical repair of Swiss-cheese ventricular septal defects with two-patch of fresh autologous pericardium and right ventricle apex excluding technique in infants can be relatively easy with favorable early and mid-term results. Long term results need to be evaluated with more cases.
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Objective: To summarize the experience for treating pulmonary venous obstruction in patients after total anomalous pulmonary venous connection (TAPVC) operation. Methods: A total of 16 patients with post-TAPVC pulmonary venous obstruction in our hospital from 2011-01 to 2015-12 were retrospectively analyzed including10 male. All patients received echocardiography, electrocardiogram and chest X-ray examinations at pre-discharge, 1, 3, 6, 12 and 24 months post-operation. Pulmonary venous obstruction was diagnosed by echocardiography measured pulmonary vein (PV) lfow speed>2m/s. The time of re-operation was determined by clinical manifestations as recurrent heart failure and growth retardation; sutureless technique and conventional patch enlarge technique were used in the second operation. Results: No one lost contact in all 16 patients. There were 7/16 patients with anastomotic stenosis (1 mixed type, 3 infracardiac type, 2 supracardiac type and 1 cardiac type), 7 patients with one PV stenosis, 2 with two PV stenosis and nobody with three or more PV stenosis. Based on per-operative Darling classiifcation, there were 2 patients with mixed type, 5 with infracardiac type, 5 with supracardiac type and 4 with cardiac type. Most post-operative PV stenosis occurred at 3-6 months after the surgery. There were 5 patients receive re-operation, 4 with sutureless technique, 1 with conventional patch enlarge technique and all of them suffered from anastomotic stenosis. 2 patients died and 3 were followed-up. Conclusion: Post-operative anastomotic stenosis was the main indication for re-operation in patients after TAPVC; early operation could better improve the clinical condition.
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Objective: To evaluate the feasibility ofin vitro balloon dilatation for treating aortic coarctation infants younger than 6 months. Methods: A total of 13 aortic coarctation infants treated in our hospital from 2011-01 to 2015-12 were summarized. The patients were at the mean age of 3.4 months (from 20 days to 6 months), with the mean body weight of 4.8 kg (from 3.6 kg to 6.2 kg) including 9 male. Aortic coarctation segments were resected during the operation and the resected segments were expanded by percutaneous transluminal angioplasty (PTA) via (2-4) times diameter balloon dilatation. The diameters of resected aortic segments were recorded before and after expansion; the change of vascular middle smooth muscle layer was observed by HE staining. Results: There were 8 cases with non-restricted ventricular septal defect (VSD) and 3 of them received staging operation, 2 with atrial septal defect (ASD), 3 with bicuspid aortic valve, 3 with patent ductus arteriosus (PDA) and 1 with pericardial effusion. The resected aortic segments presented that 9 patients had diaphragm type narrow and 4 had tube type narrow. After PTA, the diameters of resected aortic segments were slightly increased for the mean of 0.2 mm and no obvious change was observed; HE staining showed that the vascular inner mucosa was shed, while the middle smooth muscle layer was intact. Conclusion: Balloon dilatation had the poor effect for treating aortic coarctation infants younger than 6 months; surgical procedure could be applied for treating the relevant patients.
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Objective To discuss one-stage surgical repair of interrupted aortic arch ( IAA) in neonates and young in-fants, summarize follow-up results and subsequent treatments.Methods From September 2010 to December 2014, a series of 38 consecutive neonates and young infants ( M/F =26/12 ) with IAA were admitted in our surgical group to receive surgical treatment, with a median age of 11 days(1 day to 5 months) and a median body weight of 4.1 kg(1.8 to 5.8 kg).IAA was type A in 24 cases, type B in 13 cases and type C in 1 case.Associated cadiovascular anomalies were common except 2 cases, including atrial septal defect(n=23), ventricular septal defect(n=36), left ventricular outlet tract obstruction(LVOTO)(n=7), anomalous left pulmonary artery from aorta(n=1), truncus arteriosus(n=1).There were 5 cases of airway stenosis in 28 cases of CT angiography and 3D airway reconstruction.All the 38 cases were admitted with differential cyanosis.36 cases were recieved one-stage surgical repair except 2 cases died before operation.35 cases of IAA associated with intracardiac anom-alies were repaired through a median sernotomy.Cardiopulmonary bypass(CPB) was performed with two canulations in ascend-ing aorta and main pulmonary artery.Selective cerebral perfusion(n=21) or circulatory arrest(n =14) was carried out with body temperature of 18℃-23℃.After the patent ductus arteriosus tissue was completely resected , a continuous end-to-end or end-to-side suturing with 7/0(8/0) prolene was performed.In 1 type B cases with expected excessive anastomotic tension, the left subclavian artery was cut off to release the aortic arch .The associated anomalies were repaired during the cooling or re-warming period.Chest close was delayed to carry out no more than 24-72 h postoperatively in 16 cases.The other one type A IAA case without intracardiac anomaly was repaired through a standard left posterior lateral incision .Patients were strictly fol-lowed up with a standard protocol.Complications such as aortic arch restenosis, new LVOTO, new airway stenosis, and so on, were specialy focused on during the follow-up period.If needed, reoperation or transcatheter intervention was applied to treat the complications.Results Two cases died before operation and another 2 died in the early period postoperatively because of pulmonary hypertension crisis and severe tracheal stenosis, respectively.Sudden death was in 1 case during the follow-up peri-od.Median CPB time was 138 min(105-208 min) and median selective cerebral perfusion or circulatory arrest time was 24 min(16-35 min) .Mechanical ventilation time was 2-25 days.Blood pressure difference between the upper and lower limbs was less than 20 mmHg in all the patients before discharge.29 cases(85.29%) was followed up for 28 months(9 -60 months).3 cases were received reoperation in 5 cases of newly detected LVOTO.One case received reoperation in the 7 preop-erative LVOTO cases because of newly detected LVOTO(blood pressure gradient more than 40 mmHg).Two cases with aortic arch restenosis received percutaneous transcatheter ballon dilation .One case with new left main bronchus stenosis after opera-tion was strictly followed up.Conclusion IAA is a rare and severe congenital heart disease.Positive surgical repair should be performed after definite diagnosis being made .Although satisfactory results can be obtained with one-stage primary operation , IAA is a kind of progressive chronic disease.New postoperative LVOTO should be focused on becasue it will need reoperation or even the third operation .
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Objective To observe the availability and safety of ventricular septal defect (VSD) occluder in infants and young children with large patent ductus arteriosus (PDA) associated with severe pulmonary hypertension.Methods Five patients (1 male and 4 fomale) of large PDA aged 5 months to 3 years,weighted from 5.1 to 15 kg,body surface area (BSA) 0.37-0.58 m2 underwent transcathter intervention with concentric VSD occluders from June 2008 to May 2009.Arterial ducta were tube-like and their diameters were 5.7 to 8.5 mm,with ulmonary vascular resistance from 4.8 to 5.7 Wood Unit,Qp/Qs 3.4-4.6.Three patients were given Bosentan after intervention.Results The large PDAs were successfully closed with VSD occluders,including 1 concentric perimembranous VSD occluder and 4 muscular VSD occluders.They all discharged 4 to 5 days with hidrosis and weight improved.Echocardiogram indicated VSD occluder was stable,no residue shunt and no stricture of left pulmonary artery and descending aorta were found.According to tricuspid and pulmonary regurgitation,pulmonary arterial pressure decreased differently and returned to normal after 6 months follow-up.Conclusion VSD occluder is available and effective to close large PDA associated with severe pulmonary hypertension in inrants and young children,but more cases and long-term follow-up are necessary.