Biventricular function after Ebstein's anomaly repair from a single-center echocardiography study.

BACKGROUND: We aimed to examine bi-ventricular remodeling and function following Ebstein's anomaly (EbA) surgical correction using echocardiographic techniques, particularly the relations between the bi-ventricular changes and the EbA types. METHODS: From April 2015 to August 2022, 110 patients with EbA were included in this retrospective study based on the Carpentier classification. Echocardiography assessments during the preoperative, early, and mid-term postoperative periods were performed. RESULTS: The 54 patients with types A and B EbA were included in group 1, whereas the 56 with types C and D were in group 2. Seventy-eight patients underwent surgical correction of EbA. The median age at operation was 8.8 years. During the mid-term follow-up, only 9.1% of the patients had moderate or severe tricuspid regurgitation. Right ventricular (RV) systolic function worsened in group 2 at discharge (fractional area change: 27.6±11.2 versus 35.4±11.5 [baseline], P<0.05; global longitudinal strain: -10.8±4.4 versus -17.9±4.7 [baseline], P=0.0001). RV function slowly recovered at a mean of 12-month follow-up. Regarding left ventricular (LV) and RV systolic function, no statistical difference was found between before and after surgery in group 1. CONCLUSIONS: A high success rate of surgical correction of EbA with an encouraging durability of the valve was noted. Bi-ventricular systolic function was maintained fairly in most patients with types A and B postoperatively. A late increase in RV systolic function after an initial reduction and unchanged LV systolic function were observed in the patients with types C and D postoperatively.

Platelet Aggregation Before Aspirin Initiation in Pediatric Patients With Congenital Heart Disease at High Risk of Thrombosis.

Background: Aspirin following unfractionated heparin is the most common anticoagulation strategy for pediatric patients who experienced cardiac surgery at high risk of thrombosis. The platelet aggregation test is the golden method to evaluate the aspirin effect on platelet function. However, the platelet aggregation basal status before postoperative aspirin initiation and the related clinical influencing factors hasn't been investigated systemically in this population. Methods: In a prospective cohort of 247 children, arachidonic acid-induced platelet aggregation (PAG-AA) was measured by means of light transmission aggregometry (LTA) before the first dose of aspirin after cardiac surgical procedure and the perioperative variables were also collected. Distribution of this population's PAG-AA basal status was described. Univariate and multivariate logistic regression analysis were performed to identify the main influencing factors of PAG-AA. Results: The median time of aspirin administration was 2 (1-27) days after surgery and the corresponding median value of basal PAG-AA was 20.70% (1.28-86.49%), with 67.6% population under 55% and 47.8% population under 20%. Patients undergoing cardiopulmonary bypass (CPB) had a significantly lower basal PAG-AA than those without (30.63 ± 27.35 vs. 57.91 ± 27.58, p = 0.013). While patients whose test done within 3 days after CPB had a significantly lower PAG-AA than those out of 3 days (25.61 ± 25.59 vs. 48.59 ± 26.45, p = 0.001). Univariate analysis implied that the influencing factors of the basal PAG-AA including CPB use, test time point, cyanosis, and platelet count. Multivariate regression analysis indicated that only CPB use, test time point, and platelet count were the main independent influencing factors for the basal PAG-AA. Conclusion: The majority of children have impaired basal platelet aggregometry responses before postoperative aspirin initiation. The main influencing factors are CPB use, test time point, and platelet count. To establish the platelet aggregometry baseline prior to commencement of aspirin therapy, testing should be performed 3 days later following the procedure when effect of CPB is basically over.

Echocardiography in the diagnosis of Shone's complex and analysis of the causes for missed diagnosis and misdiagnosis.

BACKGROUND: Shone's complex is a rare syndrome characterized by congenital left heart defects that can differ among the patients. AIM: To use echocardiography in the diagnosis of Shone's complex and analyze the causes of missed diagnosis and misdiagnosis. METHODS: This was a retrospective study of patients who underwent echocardiography and repair surgery from February 14, 2008, to November 22, 2019. The patients were followed once a year at the outpatient clinic after surgery. RESULTS: Sixty-six patients were included. The patients were 2.7 (0.8-5.6) years of age, and 54.5% were male. Ten (15.2%) had a history of heart surgery. The most common heart defect was the Annulo-Leaflet mitral ring (ALMR) (50/66, 75.8%), followed by coarctation of the aorta (CoA) (43/66, 65.2%). The patients had a variety of combinations of defects. Only two (3.0%) patients had all four defects. None of the patients had a family history of congenital heart disease. The preoperative echocardiographic findings were examined against the intraoperative findings. Echocardiography missed an ALMR in 31 patients (47.0%), a parachute mitral valve (PMV) in one patient (1.5%), subaortic stenosis in one patient (1.5%), and CoA in two patients (3.0%). CONCLUSION: Echocardiography is an effective method to diagnose the Shone's complex. Due to this disease's complexity and interindividual variability, Improving the understanding of the disease can reduce misdiagnosis and missed diagnosis.

Handmade tri-leaflet ePTFE conduits versus homografts for right ventricular outflow tract reconstruction.

BACKGROUND: This study aimed to investigate the performance of handmade tri-leaflet expanded polytetrafluoroethylene (ePTFE) conduits in the absence of a suitable homograft. METHODS: Patients who underwent right ventricular outflow tract reconstruction with tri-leaflet ePTFE conduits or homografts between December 2016 and August 2020 were included. The primary endpoint was the incidence of moderate or severe conduit stenosis (≥ 36 mmHg) and/or moderate or severe insufficiency. The secondary endpoint was the incidence of severe conduit stenosis (≥ 64 mmHg) and/or severe insufficiency. RESULTS: There were 102 patients in the ePTFE group and 52 patients in the homograft group. The median age was younger [34.5 (interquartile range: 20.8-62.8) vs. 60.0 (interquartile range: 39.3-81.0) months, P = 0.001] and the median weight was lower [13.5 (10.0-19.0) vs. 17.8 (13.6-25.8) kg, P = 0.003] in the ePTFE group. The conduit size was smaller (17.9 ± 2.2 vs. 20.5 ± 3.0 mm, P < 0.001) and the conduit Z score was lower (1.48 ± 1.04 vs. 1.83 ± 1.05, P = 0.048) in the ePTFE group. There was no significant difference in the primary endpoints (log rank, P = 0.33) and secondary endpoints (log rank, P = 0.35). Multivariate analysis identified lower weight at surgery [P = 0.01; hazard ratio: 0.75; 95% confidence interval (CI) 0.59-0.94] and homograft conduit use (P = 0.04; hazard ratio: 8.43; 95% CI 1.14-62.29) to be risk factors for moderate or severe conduit insufficiency. No risk factors were found for moderate or severe conduit stenosis or conduit dysfunction on multivariate analysis. CONCLUSION: Handmade tri-leaflet ePTFE conduits showed acceptable early and midterm outcomes in the absence of a suitable homograft, but a longer follow-up is needed.

Two approaches for newborns with critical congenital heart disease: a comparative study.

BACKGROUND: Prenatal diagnosis and planned peripartum care is an unexplored concept in China. This study aimed to evaluate the effects of the "prenatal diagnosis and postnatal treatment integrated model" for newborns with critical congenital heart disease. METHODS: The medical records of neonates (≤ 28 days) admitted to Fuwai Hospital were reviewed retrospectively from January 2019 to December 2020. The patients were divided into "prenatal diagnosis and postnatal treatment integrated group" (n = 47) and "non-integrated group" (n = 69). RESULTS: The age of admission to the hospital and the age at surgery were earlier in the integrated group than in the non-integrated group (5.2 ± 7.2 days vs. 11.8 ± 8.0 days, P < 0.001; 11.9 ± 7.0 days vs. 16.5 ± 7.7 days, P = 0.001, respectively). The weight at surgery also was lower in the integrated group than in the non-integrated group (3.3 ± 0.4 kg vs. 3.6 ± 0.6 kg, P = 0.010). Longer postoperative recovery time was needed in the integrated group, with a median mechanical ventilation time of 97 h (interquartile range 51-259 h) vs. 69 h (29-168 h) (P = 0.030) and with intensive care unit time of 13.0 days (8.0-21.0 days) vs. 9.0 days (4.5-16.0 days) (P = 0.048). No significant difference was observed in the all-cause mortality (2.1 vs. 8.7%, P = 0.238), but it was significantly lower in the integrated group for transposition of the great arteries (0 vs. 18.8%, log rank P = 0.032). CONCLUSIONS: The prenatal diagnosis and postnatal treatment integrated model could significantly shorten the diagnosis and hospitalization interval of newborns, and surgical intervention could be performed with a lower risk of death, especially for transposition of the great arteries.

Multicenter Comparison of Percutaneous and Surgical Pulmonary Valve Replacement in Large RVOT.

BACKGROUND: A percutaneous approach for pulmonary valve replacement (PVR) is a feasible alternative to surgical PVR in selected patients with severe pulmonary regurgitation after repair of tetralogy of Fallot. However, large right ventricular outflow tract (diameter ≥ 25 mm) remains challenging. METHODS: This retrospective multicenter study enrolled consecutive patients with large right ventricular outflow tract who underwent percutaneous PVR (Venus P-valve, Venus MedTech Inc, Hangzhou, China) (n = 35) or surgical PVR (homograft valve; n = 30) between May 2014 and April 2017. Patients were followed up at 1, 3, 6, and 12 months, and yearly thereafter. Main study outcomes were pulmonary valve function and right ventricular function at discharge and midterm follow-up. RESULTS: PVR was successful in all patients. Percutaneous compared with surgical PVR group had: similarly distributed baseline characteristics; shorter hospitalization, intensive care unit stay, and endotracheal intubation duration; lower cost; lower pulmonary valve gradient before discharge; lower pulmonary valve regurgitant grade (mean difference, -0.63; 95% CI -1.11 to -0.20, P = .022), pulmonary valve gradient (mean difference, -5.7 mm Hg; 95% CI -9.4 to -2.2 mm Hg, P = .005), and right ventricular end-diastolic volume index (mean difference, -9.5 mL/m2; 95% CI -16.9 to -3.1 mL/m2, P = .022); and greater right ventricular ejection fraction (mean difference, 5.4%; 95% CI 2.4%-8.3%, P = .002) at median 36 months follow-up, without deaths in either group. CONCLUSIONS: Percutaneous PVR using Venus P-valve appeared to be a safe, efficacious and minimally invasive alternative to surgical PVR in selected patients with large right ventricular outflow tract yielding better right ventricular and pulmonary valve function at midterm follow-up.

Surgical outcome after complete repair of tetralogy of Fallot with absent pulmonary valve: comparison between bovine jugular vein-valved conduit and monocusp-valve patch.

BACKGROUND: The prognosis of tetralogy of Fallot with absent pulmonary valve (TOF/APV) without operation is poor. We evaluated the surgical outcome of TOF/APV in a single center. METHODS: Twenty-two TOF/APV patients underwent complete surgical correction in our hospital. Right ventricular outflow tract reconstruction was performed using bovine jugular vein (BJV)-valved conduit implantation (n = 10), homograft-valved conduit implantation (n = 2), or monocusp-valve patch (n = 10). Health-related quality of life (QOL) was evaluated during follow-up. RESULTS: The overall survival at 5 and 10 years was 86.4 ± 7.3% (confidence interval 69.4-97.2%). The survival rates were significantly different between patients with and without bronchial stenosis (40 and 100%, P = 0.0003, log-rank test). The survival of patients aged > 6 months was higher than those ≤ 6 months (100 vs. 40%, P = 0.0003, log-rank test). Patients with BJV-valved conduits had higher systolic gradients from the right ventricle to the pulmonary artery (RV-PA) compared to those with monocusp-valve patches. BJV-valved conduit implantation was a risk factor for post-operative pulmonary-valve stenosis. The QOL score for patients with BJV-valved conduits was lower than those with monocusp-valve patches (P < 0.05). No reoperation was performed during follow-up. CONCLUSIONS: Bronchial stenosis and lower age (≤ 6 months) were the main factors influencing post-operative survival. The use of a BJV-valved conduit was a main reason for RV-PA restenosis; thus, the use of a BJV-valved conduit may increase the need for repeat intervention and decrease the post-operative quality of life.

Hybrid Balloon Valvuloplasty for the Treatment of Severe Congenital Aortic Valve Stenosis in Infants.

BACKGROUND: Surgical or percutaneous interventional treatment of severe congenital aortic valve stenosis (CAS) in early infancy remains challenging. This single-center, retrospective study analyzed midterm outcomes of a hybrid balloon valvuloplasty procedure through the ascending aorta by way of median sternotomy, including cases with improved technique. METHODS: Included were 45 consecutive infants (aged <90 days) with CAS and selected for biventricular repair who underwent hybrid balloon valvuloplasty in a hybrid or ordinary operating room from October 2010 to March 2016. Patients were assessed at 1, 3, 6, and 12 months and yearly thereafter. RESULTS: Hybrid balloon valvuloplasty was successful in all patients, with the last 8 treated in an ordinary operating room under only echocardiography guidance with a new sheath. Thirty-two patients were successfully rescued from low heart rate or left ventricular systolic dysfunction, or both, by cardiac massage under direct visualization; none required cardiopulmonary bypass. The degree of new aortic insufficiency was mild in 7 patients and changed from mild to moderate in 1 patient. Aortic valve pressure gradient decreased from 70.6 ± 17.5 mm Hg preoperatively to 15.2 ± 4.2 mm Hg immediately postoperatively (p < 0.001). Fluoroscopy time was 4.8 ± 2.3 minutes. At a median of 32.1 months (range, 1 to 68 months) follow-up, all patients were alive and healthy. Aortic valve pressure gradient remained low (19.1 ± 5.2 mm Hg). Left ventricular ejection fraction increased from 0.515 ± 0.134 (range, 0.21 to 0.70) preoperatively to 0.633 ± 0.035 (range, 0.58 to 0.75; p < 0.001). No aortic insufficiency developed, and no patient required reintervention. CONCLUSIONS: For infants with severe CAS, hybrid balloon valvuloplasty through the ascending aorta by way of a median sternotomy appears efficacious and safe up to midterm follow-up.

Preoperative Evaluation and Midterm Outcomes after the Surgical Correction of Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery in 50 Infants and Children.

BACKGROUND: Anomalous origin of the left coronary artery (LCA) from the pulmonary artery (ALCAPA) is a rather rare congenital anomaly that has a profound effect on heart function. This study aimed to retrospectively illustrate the perioperative clinical features, therapy experience, and midterm outcomes after surgical correction, and to determine the value of left ventricular ejection fraction (LVEF) and myocardial viability in differentiating critically ill patients among infants and children with ALCAPA. METHODS: From April 1999 to March 2013, infants and children patients diagnosed with ALCAPA in Beijing Fuwai Hospital were analyzed. Clinical data of patients were summarized and retrospectively analyzed. All patients were divided into two groups according to LVEF level (Group 1: LVEF >50%, or Group 2: LVEF ≤50%) to compare perioperative and follow-up variables. Effect of myocardial viability evaluated according to myocardial perfusion/18F-fluorodeoxyglucose (FDG) imaging on the clinical variables was also analyzed. RESULTS: A total of 50 patients with ALCAPA (male/female: 29/21; median age: 3.1 years [range: 4 months to 18 years]) were included. Younger age, lower weight, intercoronary collaterals (ICC) dysplasia, ratio of the proximal right coronary artery diameter to the aortic root diameter <0.2, and larger cardiothoracic ratio (CTR) were more frequently found in Group 2 than those in Group 1. Forty-seven patients underwent cardiac surgery. The times of cross-clamp and cardiopulmonary bypass were not different between the two groups; however, the duration of mechanical ventilation and postoperative Intensive Care Unit stay were longer in Group 2 than those in Group 1. Follow-ups were possible in 38 patients (80.9%); median time: 84.5 months (range: 49 months to 216 months). There was one late sudden death with simple ligation of the LCA at 8 months after surgery. No severe complications and reoperation occurred. The relationship of the grades of myocardial viability and clinical features was analyzed in 15 patients with myocardial perfusion/18F-FDG imaging, and the results showed that myocardial viability correlated well with LVEF, CTR, abnormal Q waves, and left ventricular end-diastolic dimension. It was not correlated with age, mitral regurgitation, and ICC. Heart implantation was decided in one patient with little viable myocardium; however, this patient died 2 months after the diagnosis while waiting for transplantation. Two patients with no viable myocardium in the area of aneurysm had aneurysmectomy concomitantly. CONCLUSIONS: In infants and children with ALCAPA, heart function and myocardial viability are closely related to clinical features. LVEF and the grades of myocardial viability can differentiate high-risk patients before surgery and in the early stage of recovery after surgery. The area and extent of myocardial infarction are also crucial in making preoperative clinical decisions. However, even in patients with depressed ventricular function and severe myocardial infarction, the midterm follow-up showed satisfactory recovery of cardiac function after the successful restoration of a dual-coronary arterial system.

Biventricular Repair of Double Outlet Right Ventricle: Preoperative Echocardiography and Surgical Outcomes.

OBJECTIVE: To discuss the key anatomic features of double outlet right ventricle (DORV) assessed by preoperative echocardiography among patients treated with different types of biventricular repair. METHODS: Surgical and echocardiographic databases were queried to identify patients who had undergone biventricular repair for DORV and had adequate preoperative echocardiographic imaging. All patients underwent pre- and postoperative echocardiography and clinical evaluation following discharge. RESULTS: Two hundred sixty-two patients with DORV met the inclusion criteria of the study. The patients were divided into two groups-intraventricular tunnel repair (IVR) to the aorta (194 [74%] patients) or to the pulmonary artery with either concomitant arterial switch operation or double-root translocation (68 [26%] patients). Among 68 patients undergoing IVR to the pulmonary artery, 50 patients with transposition of the great arteries (TGA) type of DORV and 7 patients with remote ventricular septal defect (VSD) type underwent IVR plus arterial switch operation and 6 patients with TGA type and 5 patients with remote VSD type underwent IVR plus double-root translocation. There were three hospital deaths and one late death (overall operative mortality: 1.5%). CONCLUSION: Preoperative echocardiography provided crucial data to estimate the feasibility of intraventricular tunnel creation to either the aorta or the pulmonary artery and to guide the selection of either arterial switch or double-root translocation. Biventricular repair could be achieved with favorable outcomes in most patients with DORV.

Echocardiographic Classification and Surgical Approaches to Double-Outlet Right Ventricle for Great Arteries Arising Almost Exclusively from the Right Ventricle.

Selecting an appropriate surgical approach for double-outlet right ventricle (DORV), a complex congenital cardiac malformation with many anatomic variations, is difficult. Therefore, we determined the feasibility of using an echocardiographic classification system, which describes the anatomic variations in more precise terms than the current system does, to determine whether it could help direct surgical plans. Our system includes 8 DORV subtypes, categorized according to 3 factors: the relative positions of the great arteries (normal or abnormal), the relationship between the great arteries and the ventricular septal defect (committed or noncommitted), and the presence or absence of right ventricular outflow tract obstruction (RVOTO). Surgical approaches in 407 patients were based on their DORV subtype, as determined by echocardiography. We found that the optimal surgical management of patients classified as normal/committed/no RVOTO, normal/committed/RVOTO, and abnormal/committed/no RVOTO was, respectively, like that for patients with large ventricular septal defects, tetralogy of Fallot, and transposition of the great arteries without RVOTO. Patients with abnormal/committed/RVOTO anatomy and those with abnormal/noncommitted/RVOTO anatomy underwent intraventricular repair and double-root translocation. For patients with other types of DORV, choosing the appropriate surgical approach and biventricular repair techniques was more complex. We think that our classification system accurately groups DORV patients and enables surgeons to select the best approach for each patient's cardiac anatomy.

Effect analysis of repeat sternotomy in pediatric cardiac operations.

BACKGROUND: Reoperation for congenital heart disease may be associated with cardiac or vascular injuries during repeat sternotomy, resulting in increased mortality and/or morbidity rates. The aim of this study was to determine the frequency of these cardiac injuries and the associated outcome. METHODS: Between January 2012 and December 2013, 4256 sternotomy procedures were performed at the Pediatric Cardiac Center in Fuwai Hospital, including 195 repeat sternotomy procedures (RS). We retrospectively studied the clinical data of 195 RS patients and 250 randomly selected primary sternotomy (PS) patients. Demographic and operative details, major injures (MI), and clinical outcomes were compared between the two groups. We also assessed the risk factors for major injury and in-hospital mortality and morbidity. RESULTS: Significant differences were observed between the RS and PS groups in terms of skin incision to cardiopulmonary bypass(CPB) time, overall CPB time, cross-clamp time and blood requirement, and ventilation time (p < 0.001). MI during RS occurred in 7 of the 195 patients (3.6 %), while operative mortality was 1.0 % (2/195). However, in the RS patients, mortality and morbidity rates were not significantly different between the MI subgroup and the non-MI subgroup (p = 1.000 and 0.556, respectively). Additionally, no significant difference was found between the RS and PS groups in terms of mortality (p = 1.000) and morbidity (p = 0.125). CONCLUSIONS: Both RS and MI are not associated with increased risk of operative mortality and morbidity. Outcomes for reoperative pediatric operations in contemporary practice are similar with those for primary operations.

Echocardiographic Guided Closure of Perimembranous Ventricular Septal Defects.

BACKGROUND: Transesophageal echocardiogram-guided minimally invasive periventricular device closure of perimembranous ventricular septal defects (pmVSDs) without cardiopulmonary bypass is a treatment option for pmVSDs. We introduce our improvements to this technique and mid-term follow-up results. METHODS: From May 2011 to May 2014, 187 patients with pmVSDs aged 6 months to 31 years (8.2 ± 10.2 years) were enrolled in this study. The procedure was performed through a new transthoracic approach of 1 to 2 cm without sternotomy. Device selection and the operative procedure were monitored by transesophageal echocardiogram. The patients underwent follow-up examinations of echocardiography and electrocardiogram at 1 month, 3 months, 6 months, and 1 year after the operation and annually thereafter. RESULTS: The defects were closed successfully in 179 patients (95.7%), and in 8 patients the operation was converted to conventional surgical repair. Six patients (3.4%) had an incomplete right bundle branch block. One patient experienced an intermittent complete atrioventricular block on the fourth day after the operation, and sinus rhythm was restored by corticosteroid therapy after 5 days. A trivial residual shunt was observed in 8 patients (4.5%) during the procedure. The average hospital stay was 3.1 ± 0.9 days. Follow-up in all patients ranged from 1 month to 36 months (median, 12.6 months), and aortic regurgitation, malignant arrhythmia, and device dislocation were not observed in any patients. However, 3 patients (1.7%) still had a trivial residual shunt at their last follow-up. CONCLUSIONS: Periventricular device closure through a modified transthoracic approach without sternotomy is a potentially safe and effective treatment option for pmVSDs. Controlled studies with long-term follow-up are necessary.

Protective efficacy of recombinant canine adenovirus type-2 expressing TgROP18 (CAV-2-ROP18) against acute and chronic Toxoplasma gondii infection in mice.

BACKGROUND: The use of recombinant viral vectors expressing T. gondii antigens is a safe and efficient approach to induce immune responses against the parasite, as well as a valuable tool for vaccine development. We have previously prolonged the survival time of mice challenged with the RH strain of T. gondii by immunizing the mice with a eukaryotic vector expressing the protein ROP18 of T. gondii. We are now looking for ways to improve this vaccination strategy and enhance protection. METHODS: In this study, we constructed and characterized a novel recombinant canine adenovirus type 2 expressing ROP18 (CAV-2-ROP18) of T. gondii by cytopathic effect (CPE) and indirect immunofluorescence assay (IFA) following transfection into MDCK cells. Intramuscular immunization of Kunming mice with CAV-2-ROP18 was carried out to evaluate humoral and cellular immune responses. RESULTS: The vaccination of experimental mice with CAV-2-ROP18 elicited antibody production against ROP18, including high levels of a mixed IgG1/IgG2a and significant production of IFN-γ or IL-2, and displayed a significant bias towards a helper T cell type 1 (Th1) profile. Furthermore, the presence of T. gondii-specific IFN-γ-production and TNF-α-production T cells was elicited in both CD4+ and CD8+ T cell compartments. Significantly higher survival rates (40%) occurred in the experimental group, and a reduction in brain cyst burden was detected in vaccinated mice. CONCLUSION: These results demonstrate the potential use of a CAV vector harboring the ROP18 gene in the development of a vaccine against acute and chronic toxoplasmosis.

Hybrid balloon valvuloplasty through the ascending aorta via median sternotomy in infants with severe congenital valvular aortic stenosis: feasibility of a new method†.

OBJECTIVES: To evaluate a novel hybrid balloon valvuloplasty procedure for severe congenital valvular aortic stenosis in low-weight infants, performed through the ascending aorta via median sternotomy. METHODS: Eighteen infants (<90 days of age) with severe congenital aortic stenosis were included in this study. Hybrid balloon valvuloplasty procedures were performed in a hybrid operating room. Patients were followed up at 3 months, 6 months, 1 year and then annually following the procedure. RESULTS: The hybrid balloon valvuloplasty procedure was successful in all patients. Eight patients were successfully rescued from left ventricular systolic dysfunction by cardiac compression under direct vision. The aortic valve pressure gradient decreased from 80.3 ± 20.8 mmHg preoperatively to 16.0 ± 3.6 mmHg immediately postoperatively (P < 0.001). None of the patients developed significant aortic insufficiency. The fluoroscopy time was 6.2 ± 2.9 min. Intraoperative blood transfusions and pacing were not required. The patients were all alive and healthy at the end of the follow-up period (mean 21.3 months; range 3-41 months), and the aortic valve pressure gradient remained low (21.7 ± 5.3 mmHg). Reintervention was not required in any of the patients. CONCLUSIONS: Hybrid balloon valvuloplasty through the ascending aorta via median sternotomy is an effective and safe procedure for infants with severe congenital aortic stenosis.

Postoperative diaphragmatic paralysis after cardiac surgery in children: incidence, diagnosis and surgical management.

BACKGROUND: Cardiac surgery for congenital heart disease covers a wide spectrum from simple to complex cardiac and extracardiac malformations. Innovations in pediatric cardiac surgery and perioperative care over the past decades have allowed surgical correction or at least palliation in almost all complex congenital heart defects in the first years of life. Diaphragmatic paralysis (DP) due to phrenic nerve injury after congenital cardiac surgery is an important respiratory complication resulting with respiratory insufficiency, lung infections, prolonged hospital stay time and even death. METHODS: Between April 2001 and December 2010, among patients undergoing cardiac surgery for congenital heart disease, postoperative DP was diagnosed in 47/10 200 (0.46%) patients. Diaphragmatic placation was performed in 37/47 patients. DP was suspected in children who failed to wean from mechanical ventilation or in those with persistent respiratory distress when there is no cardiac cause. Decreased respiratory sounds in auscultation, paradoxical breathing during spontaneous ventilation and elevated hemidiaphragm on chest X-ray led us to use fluoroscopy, ultrasound and/or electromyogram (EMG). When chest X-rays did not have a diagnostic value in patients with persistent respiratory distress, bilateral DP was suspected and immediate fluoroscopy of EMG was performed for diagnosis. In all patients, diaphragmatic placation was performed using a thoracic approach, through the sixth or seventh intercostals space with lateral thoracotomy. RESULTS: A total of 47 patients (21 females and 26 males) with a median age of 7.21 months (range 0.27-71 months) were diagnosed DP after cardiac surgery. The incidence of DP was 0.46% after cardiac surgery. The paralysed hemidiaphragm was left side in 26/47 (55.3%), right side in 17/47 (36.2%) and bilateral in 4/47 (8.5%) cases. The assisted ventilation time after cardiac surgery was (450±216) (116-856) hours. The median time from cardiac surgery to surgical placation was (24±14) (5-56) days. No patient died in this study. The follow-up period was (26.2±16.8) months. The position of the plicated diaphragm was normal on chest X-ray, in all plicated survivors within the 1st, 6th and 12th months after discharge. CONCLUSIONS: DP caused by phrenic nerve injury during surgical intervention for congenital heart disease is an important risk factor in terms of morbidity during the postoperative period. Diaphragmatic placation appears a good option, especially in newborns and small children, to wean patients from mechanical ventilation and to prevent long-term side effects of mechanical ventilation.

Effect of mitochondrial aldehyde dehydrogenase-2 genotype on cardioprotection in patients with congenital heart disease.

AIMS: About 40% of East Asians carry an aldehyde dehydrogenase-2*2 (ALDH2*2) allele, and the influence of the ALDH2*2 allele on human cardioprotection has not been studied. This study was designed to evaluate the effect of ALDH2*2 allele on cardioprotection of patients with congenital heart diseases after open-heart surgery. METHODS AND RESULTS: The right atrial appendage was harvested before performing cardiopulmonary bypass in cyanotic and acyanotic congenital heart disease groups (n = 20 per group). Tissues were assayed to determine the impact of cyanosis on metabolic remodelling. A prospective cohort of Tetralogy of Fallot (TOF) patients (n = 118) was recruited to investigate the influence of the ALDH2*2 allele on cardioprotection after surgical repair. Myocardium samples were dissected after cardioplegia. ALDH2 activity, oxidative stress and glutathione (GSH) levels, and activating transcription factor-4 (ATF4) were analysed. After genotyping and grouping, all of the experimental and clinical results were compared between ALDH2*2 carriers and non-carriers. Cyanosis inhibited ALDH2 activity and led to aldehyde accumulation in ALDH2*2 carriers. This accumulation in turn increased expression of ATF4 and resulted in larger myocardium GSH pools. The differences in ALDH2 activity and GSH level between carriers and non-carriers disappeared during cardioplegic arrest, and more aldehydes accumulated in the non-carriers. Consequently, ALDH2*2 carriers showed lower postoperative troponin I, inotrope score, and shorter postoperative length of ICU and hospital stay. CONCLUSIONS: ALDH2*2 carriers with cyanotic congenital heart disease were associated with an induced metabolic remodelling phenotype and a compensatory myocardium GSH pool. When ALDH2 activity was impaired during open-heart surgery, this larger GSH pool could lead to unexpectedly better cardioprotection. This may aid in the prediction of cardioprotection outcomes and identification of individualized cardioprotective strategies.

Modified anastomosis for repair of supracardiac total anomalous pulmonary venous connection in infants.

Recurrent pulmonary venous obstruction after repair of total anomalous pulmonary venous connection (TAPVC) is usually restricted to the anastomosis between the pulmonary venous confluence and the left atrium. We describe a modified technique for repair of supracardiac TAPVC in infants. An L-shaped incision of left atrium is utilized and the right-sided anastomosis is enlarged by using autologous pericardium to create a large and tension-free anastomosis.

Mid-term results after correction of type I and type II persistent truncus arteriosus in older patients.

OBJECTIVE: This study aims to analyze long-term results after correction of type I and type II truncus arteriosus in older patients operated in one institution over five years. METHODS: Between 2006 and 2010, 12 patients, median age 4 years, underwent repair of truncus arteriosus. Repair with reconstruction of the right ventricular to pulmonary artery continuity was performed using a valved conduit in 12 patients. RESULTS: There was no early mortality. All patients are alive with their original conduit 0.6 to 5 years after correction. No patients required reoperations for conduit dysfunction. Recent clinical examination was undertaken in all patients and they are in good condition. CONCLUSIONS: Though mean age at operation was higher in this study than published results, the operation should be performed if the pulmonary vascular resistance is under 8 units.m(2) before operation.

Midterm results of arterial switch operation in older patients with severe pulmonary hypertension.

BACKGROUND: It is widely accepted that patients older than 6 months with dextrotransposition of the great arteries and nonrestrictive ventricular septal defect or Taussig-Bing anomaly and severe pulmonary hypertension usually are not candidates for an arterial switch operation or even repair. This study aimed to evaluate the midterm results of the arterial switch operation in these selected patients. METHODS: The records of 86 patients older than 6 months with dextrotransposition of the great arteries and nonrestrictive ventricular septal defect or Taussig-Bing anomaly and severe pulmonary hypertension undergoing the arterial switch operation at our institution from 2000 to 2008 were reviewed retrospectively. Eighty survivors were followed. RESULTS: There were 6 hospital deaths (7.0%, 95% confidence interval, 1.6% to 12.4%). From 2006 to 2008, 46 were operated without deaths occurring. The median duration of follow-up was 42 months. Two late deaths occurred. Latest follow-up data showed that 2.8% of survivors were in New York Heart Association class II and 97.2% in class I. CONCLUSIONS: Midterm results of the arterial switch operation for patients older than 6 months with dextrotransposition of the great arteries and nonrestrictive ventricular septal defect or Taussig-Bing anomaly and severe pulmonary hypertension are excellent in the current era.