Zhongqun Zhu

Total Anomalous Pulmonary Venous Connection: The Current Management Strategies in A Pediatric Cohort of 768 Patients

Background: Total anomalous pulmonary venous connection (TAPVC) is a rare form of congenital heart disease. This study describes current surgical treatment strategies and experiences in a cohort of patients from 2 congenital cardiac centers in Shanghai and Guangdong in China. Methods: This retrospective study included 768 patients operated on between 2005 and 2014. Although most patients (n=690) underwent conventional repair, a sutureless technique was used in 10% (n=78) of cases. A multilevel mixed-effects parametric survival model and a competing-risk analysis were used to analyze associated risk factors for death and recurrent pulmonary venous obstruction (PVO), respectively. Kaplan-Meier analysis was used to analyze the overall survival. The Nelson-Aalen cumulative risk curve was used to compare distributions of time with recurrent PVO. Results: The mean surgical age and weight were 214.9±39.2 days and 5.4±3.6 kg, respectively. Obstructed TAPVC (PVO) was documented in 192 (25%) of the 768 patients. There were 38 intraoperative deaths and 13 late deaths. A younger age at the time of repair (P=0.001), mixed (P=0.004) and infracardiac (P=0.035) TAPVC, preoperative PVO (P=0.027), prolonged cardiopulmonary bypass time (P<0.001), and longer duration of ventilation (P=0.028) were associated with mortality. The median follow-up was 23.2 months (range; 1–112 months). Among the 717 survivors, recurrent PVO was observed in 111 patients (15%). Associated risk factors for recurrent PVO included preoperative PVO (P<0.001), infracardiac TAPVC (P<0.001), mixed TAPVC (P=0.013), and prolonged cardiopulmonary bypass time (P<0.001). Sutureless technique was associated with a lower restenosis rate compared with conventional repair in patients with preoperative PVO (P=0.038), except in newborn patients (P=0.443). Reintervention for restenosis was performed in 24 patients. The function of most survivors (91%) was classified according to the New York Heart Association as functional class I or II. Conclusions: Surgical correction in patients with TAPVC with a biventricular anatomy can achieve an acceptable outcome. Risk factors such as a younger age at the time of repair, infracardiac and mixed TAPVC, and preoperative PVO were associated with a poorer prognosis.

Cone reconstruction of the tricuspid valve in Ebstein anomaly with or without one and a half ventricle repair

OBJECTIVE We sought to review our institutional experience and midterm results with cone reconstruction in Ebstein anomaly with or without one and a half ventricle repair to evaluate these 2 important surgical methods for the treatment of Ebstein anomaly. METHODS From January 1, 2004, to October 1, 2009, 30 consecutive patients with Ebstein anomaly underwent cone reconstruction. All patients underwent cone reconstruction, and 20 patients with severe Ebstein anomaly had a bidirectional cavopulmonary shunt constructed. The median age was 60 months (range, 2-192 months). Our criteria to define severe Ebstein anomaly include a severely enlarged right-sided chamber, significant abnormality of the septal leaflet of the tricuspid valve, and hemodynamic instability after cardiopulmonary bypass. Clinical or echocardiographic characteristics were studied both preoperatively and postoperatively. RESULTS There was 1 (3.3%) hospital death. Before the operation, tricuspid incompetence was moderate in 8 and severe in 22 patients. Postoperative early echocardiographic analysis showed that tricuspid incompetence was mild in 26 patients, moderate in 3 patients, and severe in 1 patient. After a median follow-up time of 22 months, tricuspid incompetence of 20 patients with bidirectional cavopulmonary shunt was mild in 15 and moderate in 4. Tricuspid incompetence of 10 patients without a bidirectional cavopulmonary shunt from the latest echocardiogram (median follow-up time, 32 months) was mild in 4, moderate in 4, and severe in 2 patients. For patients whose postoperative tricuspid incompetence was beyond mild among the 10 patients without a bidirectional cavopulmonary shunt, preoperative echocardiographic analysis shows a severely dilated right ventricle. CONCLUSIONS Addition of a bidirectional cavopulmonary shunt to cone reconstruction of the tricuspid valve should be considered for young patients with severe Ebstein anomaly who are at high risk of right ventricular failure after the operation.

Surgical repair for primary pulmonary vein stenosis: Single-institution, midterm follow-up

BACKGROUND Primary pulmonary vein stenosis (PVS) is a rare congenital heart condition and carries a poor prognosis. METHODS A retrospective review of 18 patients who underwent surgical therapy for primary PVS (2006-2014) was conducted. According to the degree of stenosis severity, the involved pulmonary veins (PVs) were divided into 3 categories: mild (34 veins), moderate (8), and severe (3). Pericardial patch venoplasty was used in 10 involved veins, endarterectomy in 11, and sutureless pericardial marsupialization in 19. RESULTS Median surgical age and weight were 19.8 (range: 7-100) months and 7.7 (range: 5.3-20.3) kg, respectively. Bilateral PVS was found in 10 patients (56%), and unilateral in 8 (44%). Moderate or severe stenosis was found more frequently in PVs on the left side (P = .035). Multivein involvement was more common in patients age ≤18 months than in older patients (75% vs 20%, P = .054). No early operative death occurred. Median length of hospital stay was 16 (range: 8-60) days. One subsequent death occurred (6%), at the 2-month follow up after discharge. Median follow-up time for the remaining patients was 29 (range: 2-91) months. Three of the 6 PVs treated for moderate stenosis, compared with 7 of the 34 treated for mild stenosis, developed restenosis, irrespective of the surgical strategy (P = .153). Most surviving patients remained in relatively good condition, in New York Heart Association functional class I or II. CONCLUSIONS Detailed morphologic evaluation of each PV involved is a consideration for surgery, and is closely related to the prognosis. Moderate or severe primary PVS is worse than mild PVS, and no differences were found in effectiveness among endarterectomy, pericardial patch venoplasty, and sutureless pericardial marsupialization in treating primary PVS.

Extracardiac Fontan with direct cavopulmonary connections: midterm results

OBJECTIVES The aim of this study was to analyse the midterm results following superior and inferior direct cavopulmonary connections (DCPC) to create a Fontan circulation in patients with functionally univentricular hearts. METHODS A retrospective review of patients operated on between January 2005 and December 2011 was carried out. RESULTS Thirty consecutive patients who underwent this type of operation were retrospectively reviewed. There were 18 (60%) males and 12 (40%) females, with a median age of 69 months (range 16-150 months) and median weight of 23 kg (range 11-46 kg). Aortic cross-clamping was used in 10 patients, with a median cross-clamp time of 40 min (range 23-99) and a median cardiopulmonary bypass (CPB) time of 135 min (range 76-179 min). The remaining 20 patients were operated on without aortic cross-clamping. Their median CPB time was 104 min (range 78-139 min). Fenestration was performed in 16 patients. The associated intracardiac procedures were performed in 10 patients. The follow-up period ranged from 2 months to 6 years. Operative mortality and late mortality after discharge was zero. The major postoperative complications included supraventricular tachycardia in one patient, oliguria and peritoneal dialysis in one and chest drainage (>30 ml/day) persisting >7 days in five (20%). One patient developed sinus bradycardia in association with sinus pauses 2 months after discharge. One patient developed pericardial effusion 1 month after discharge. A computational fluid dynamic study was performed in one patient. The computational fluid dynamic study showed that DCPC may have a better power efficiency. CONCLUSIONS Superior and inferior DCPCs to create a Fontan circulation in appropriately selected patients with functionally univentricular hearts can be performed with a low risk and a low rate of reinterventions. The midterm results are favourable.

Surgical results for pulmonary atresia with intact ventricular septum: a single-centre 15-year experience and medium-term follow-up.

OBJECTIVES The optimal surgical strategies for pulmonary atresia with intact ventricular septum (PAIVS) are still not well established. This study reviewed our 15-year experience in the management of PAIVS. METHODS Between July 1999 and June 2014, 170 patients were treated for PAIVS in our heart centre. Based on the morphology of the right ventricle (RV), age and surgical approaches, the patients were divided into two groups: the one-stage surgery group (n = 33) and the staged surgery group (n = 137), in which patients received definitive repair, including biventricular repair, 1.5 ventricular repair and univentricular palliation without or with initial intervention. The median follow-up time was 6.6 years (range: 1-15 years); survival rates, risk factors for death and clinical status after operation were assessed. RESULTS In the one-stage surgery group, there were three deaths post operation; the estimated 1-, 5- and 15-year survival rates were 97.0, 93.7 and 88.5%, respectively. In the staged surgery group, 23 patients died, including 15 in the waiting period after initial intervention. The estimated 1-, 5- and 15-year survival rates of the staged group were 89.8, 88.2 and 69.1%, without significant difference when compared with the one-stage surgery group (P > 0.05). Independent predictors of mortality were severe RV hypoplasia (P < 0.05) and lower tricuspid valve Z-scores (P < 0.01). At the latest follow-up, most of the patients in both groups had a good clinical status after definitive repair. The re-operation rate was 16.0% (4/25) in the one-stage surgery group compared with 15.4% (6/39) in the staged surgery group. CONCLUSIONS Both one-stage repair and the staged surgical procedure had acceptable surgical outcomes in this retrospective study. Initial intervention is suitable for neonates or younger patients to promote the growth of the RV, and one-stage definitive repair is a beneficial choice for older patients with PAIVS, in whom the growth potential of the RV is limited.

Primary complete repair of interrupted aortic arch with associated lesions in infants.

Interrupted aortic arch (IAA) is a complicated congenital heart disease requiring an individualized management strategy. We reported the results for surgical repair of IAA with associated anomalies.

Surgical strategies and outcomes of congenital supravalvular aortic stenosis

Various surgical techniques have been introduced to treat supravalvular aortic stenosis (SVAS). However, there is no consensus upon the optimal approach. This study reviewed our institutional experience in the management of SVAS.

Hemodynamic analysis of surgical correction for patient-specific aortic coarctation with aortic arch hypoplasia by end-to-side anastomosis

Coarctation of the Aorta (CoA) is one of the most serious congenital heart defects in newborns. Approximately 30% of cases of CoA associated with aortic arch hypoplasia (AAH). The end-to-side aortic anastomosis (ESAA) is an effective surgical procedure to be performed for correction of CoA with AAH. In this study, the approach of computational fluid dynamics (CFD) was used to investigate the hemodynamics in patient-specific models of CoA with aortic arch hypoplasia before and after the procedure of ESAA. The results showed that the pressure drop and energy loss (EL) were significantly decreased after the surgical correction was done. Uniformed velocity profile and blood flow distribution were surgically created for patients recovery of cardiac function. The approach of CFD can be used to disclose and evaluate the hemodynamics of CoA and its correction procedure.

Extracardiac fontan with direct cavopulmonary connections: midterm results.

Background: The aim of this study was to analyze the midterm results following direct superior and inferior cavopulmonary connections (DCPC) to create Fontan circulation in patients with functionally univentricular hearts. Methods: Retrospective review of patients operated between January 2005 and May 2011. Results: The 25 consecutive patients who underwent this type of operation were retrospectively reviewed. There were 15 (60%) males and ten (40%) females, with median age of 73 months (range: 16-150 months) and median weight of 25 kg (range: 11-46 kg). Aortic cross-clamping used in ten patients, with median cross-clamp time of 40 minutes (range: 23-99) and median cardiopulmonary bypass (CPB) time of 135 minutes (range: 76-179 minutes). The remaining 15 patients were operated without aortic cross-clamping. Their median CPB time was 112 minutes (range: 82-139 minutes). Fenestration was performed in 15 cases. Associated intracardiac procedures were performed on ten patients. The follow-up period ranged from two months to six years. Operative mortality and late mortality after discharge were zero. Major postoperative complications included supraventricular tachycardia in one patient, oliguria and peritoneal dialysis in one patient, chest drainage persisting more than seven days in five patients (20%). One patient developed sinus bradycardia in association with sinus pauses two months after discharge. One patient developed pericardial effusion one month after discharge. Conclusions: Direct superior and inferior cavopulmonary connections to create Fontan circulation in appropriately selected patients with functionally univentricular hearts can be performed with low risk and a low rate of reintervention. The midterm results are favorable.

Inhibition of microglial activation by minocycline reduced preoligodendrocyte injury in a neonatal rat brain slice model

Background: Periventricular leukomalacia is a common white‐matter injury after neonatal cardiac surgery; however, its potential cellular mechanism remains uncertain. There is limited study regarding periventricular leukomalacia treatment. Methods: A neonatal rat brain slice perfusion model was used for reproducing the condition of cardiopulmonary bypass, and oxygen glucose deprivation simulated circulatory arrest. Seven‐day‐old Sprague–Dawley rats were randomly divided into 7 groups: (1) control group with 36°C; (2) 60 minutes of oxygen glucose deprivation group on 15°C, 25°C, 36°C, respectively; and (3) 60 minutes of oxygen glucose deprivation group on 15°C, 25°C, 36°C, plus minocycline (10 &mgr;mol/L), respectively. Immunohistochemistry, Western blot, and inflammatory mediators were compared after the perfusion procedures in the different groups. Results: This neonatal rat brain slice perfusion with oxygen glucose deprivation model could replicate the pathophysiologic process and injury after cardiopulmonary bypass and hypothermic circulatory arrest. With the increase of oxygen glucose deprivation perfusion temperature, we found that both microglia activation and preoligodendrocyte loss increased. The application of minocycline can significantly inhibit microglial activation and preoligodendrocyte cells loss in the normothermic (36°C) and moderate hypothermia (25°C) oxygen glucose deprivation groups (P < .05), with accompanying significant decreasing microglial inflammatory productions; however, no significant improvement was found in the deep hypothermia (15°C) group. Conclusions: The microglial activation may play a key role in preoligodendrocyte injury in the ex vivo neonatal rat brain slice perfusion and circulatory arrest model. Inhibition of microglial activation with minocycline may be an attractive target for white‐matter protection during cardiopulmonary bypass and hypothermic circulatory arrest.