Neo-aortic posterior sinus of Valsalva reduction and closed coronary artery transfer as a method to approach aortopulmonary mismatch in transposition of great arteries.

OBJECTIVE: In transposition of great arteries (TGA), aortopulmonary mismatch (APM) can determine postoperative neo-aortic insufficiency after arterial switch operation (ASO). The distortion of sinu-tubular junction may be the geometric mechanism responsible. We developed a strategy able to reduce the mismatch at the timing of ASO, and in this study, we aimed to describe our indications and results. METHODS: Preoperative root circumferences at the level of the mid-portion of sinus of Valsalva and ascending aorta circumference were used to define APM. Indication to surgery was a neo-aortic root (NAR) to ascending aorta ratio ≥ 1.4. Along with standard ASO, posterior neo-aortic sinus inverted conal resection and punch technique for coronary reimplantation was used in all patients to re-establish the more geometric ratio possible between the two components. Hypoplastic aortic arch (HAA) and aortic coarctation (CA) were managed by aortic arch enlargement with an autologous pericardial patch. RESULTS: Twenty patients (20 male), 19 with diagnoses TGA (17 with ventricular septal defect, 85%) and 1 with Taussig-Bing anomaly underwent ASO. HAA was present in three (15%) and CA in two (10%). The mean preoperative neo-aortic to ascending aorta ratio was 1.8 versus 1.1 postoperatively (p < .01). No moderate or severe neo-aortic insufficiency was observed before discharge and at a mean follow-up of 4.3 years (interquartile range = 0.5-12 years). CONCLUSION: Neo-aortic reduction plasty with coronary reimplantation by punch technique is an effective strategy to approach preoperative APM in TGA. This technique confers a more harmonious geometry to NAR that can improve neo-aortic valve function.

Retrosternal Clots After Fontan Surgery by Systematic Evaluation With Transthoracic Ultrasound.

OBJECTIVES: Bleeding is a common, serious, and often subtle complication after total cavopulmonary connection surgery. The aim of the present study was to assess the incidence of retrosternal clots after surgery, which were searched for systematically with transthoracic ultrasound. DESIGN: Retrospective study. SETTING: Single center. PARTICIPANTS: Total cavopulmonary surgeries were reviewed from January 2016 to May 2019. INTERVENTIONS: Thoracic ultrasound with careful evaluation of the retrosternal area was performed at different postoperative times (12-36 hours, 5-7 days, and before discharge) as completion of routine echocardiography. MEASUREMENTS AND MAIN RESULTS: Among 37 children undergoing total cavopulmonary connection (mean age 5.5 ± 1.8 years [range 2.4-11.7]; mean body surface area 0.7 ± 0.1 m2 [range 0.3-1.6 m2]), retrosternal clots were detected in 18 (48.6%). Of these, 7 (13.5%) had small clots (<1 cm), 2 (5.4%) small to moderate sized clots (>1 cm-<2 cm), 3 (8.1%) moderate sized clots (>2-<3 cm), and 6 (16.2%) large clots (>3 cm). Four of the 6 detected large clots required surgical revision, and in the other 2 patients, the clots were not treated because the patients' conditions were clinically stable. When 3 major groups (group 1-no or small clots, group gropu 2 are small to moderate or moderate, group 3-large clots) were evaluated, no significant differences were noted in age, body surface area, CPB time, conduit type, or the number of previous surgeries. CONCLUSIONS: With thoracic ultrasound diagnosis, existence of retrosternal clots was found to be very common after total cavopulmonary connection. Most clots were small or moderate with no clinical effect; however, large clots that required redo surgery also were detected.

Tracheal surgery in children: outcome of a 12-year survey.

OBJECTIVES: Despite the fact that team management has improved the results in recent years, perioperative deaths and complications remain high in paediatric tracheal surgery. We reviewed our institutional experience by comparing our results with those in the literature. METHODS: Between 2005 and 2017, 30 children underwent surgery for tracheal disease. Fifteen were boys and fifteen were girls (50% vs 50%). The median age at operation was 7 months (15 days-9.6 years), and the median weight was 5.2 kg (2.8-34 kg). Congenital tracheal stenosis was diagnosed in 25 children (83.3%), and 5 (16.7%) had acquired lesions. The mean internal diameter in congenital tracheal stenosis was 1.5 mm, with complete tracheal rings present in all patients. Associated malformations were bronchopulmonary in 11 cases (36.7%) and cardiovascular in 16 (53.3%). RESULTS: No in-hospital deaths occurred in our data set. Overall mortality was 4 of 30 cases (13.3%). Twenty-four endoscopic reinterventions were required in 19 children (63%) and consisted of stent positioning in 13 (43.3%), balloon dilatation in 5 (16.7%), granulation removal in 4 (13.3%) and tracheostomy in 2 (6.7%). Of the survivors (26 of 30, 86.7%), 11 children (42.3%) did not require further examination on adequate tracheal diameter for age and absence of symptoms after a median follow-up period of 3.5 years. CONCLUSIONS: The result of paediatric tracheal surgery depends on several factors. The number of cases treated at a particular centre is an important one, but our experience, although limited, can be compared with that at centres with a higher volume of cases. We emphasize the need for applying a multidisciplinary approach to master the surgical command of different reconstructive tracheal procedures, to manage associated defects, particularly cardiovascular defects, and to manage complications under endoscopic guidance. These can be considered the mainstays of building a successful tracheal programme.

Arterial Switch Operation and Plasma Biomarkers: Analysis and Correlation with Early Postoperative Outcomes.

The aims of our study were to describe plasma brain natriuretic peptide (BNP), Troponin I (TnI), and Cystatin C (Cys-C) concentration kinetics in the postoperative period after arterial switch operation in neonate, and to test the correlation between the plasma biomarkers and early clinical outcomes. We prospectively enrolled 29 neonates who underwent ASO. All patients received Custodiol cardioplegia. Blood samples were collected preoperatively (one day before) and in the ICU immediately after admission, and then 6, 12, 24, and 48 h after surgery. TnI peak (mean 17.23 ± 7.0 ng/mL) occurred between the arrival in the ICU and the 6th hour, then we had a constant decrease. TnI had a good correlation with the inotropic support time (r = 0.560, p = 0.0015) and ICU time (r = 0.407, p = 0.028), less than with ventilation and Hospital stay (r = 0.37, p = 0.0451 and r = 0.385, p = 0.0404). BNP peak (mean 4773.79 ± 2724.52 ng/L) was in the preoperative time with a constant decrease after the operation and it had no significant correlations with clinical outcomes. The CyS-C had the highest preoperative values, which decreased during the operating phase, and then constantly increased upon arrival to the ICU with a peak at 48 h (mean 1.76 ± 0.35 mg/L). CyS-C peak had a good correlation with a plasmatic creatinine peak (r = 0.579, p = 0.0009) but not with other clinical outcomes. Our study demonstrated significant correlations between the Tnl peak and early clinical outcomes in neonates undergoing arterial switch operation. Other plasma biomarkers such as the BNP and CyS-C had no direct correlation.

Diagnostic accuracy and prognostic valued of plasmatic Cystatin-C in children undergoing pediatric cardiac surgery.

BACKGROUND: Diagnosis and treatment of acute kidney injury (AKI) is often delayed in children after cardiac surgery due to the lack of an early biomarker of renal damage. Our aim was to evaluate the diagnostic accuracy of plasma cystatin-C as an early biomarker of AKI and its prognostic value in pediatric cardiac surgery. METHODS: Cystatin-C and creatinine were measured pre-operatively and at 2-6-12h post-surgery. The primary outcome was: AKI (defined as an increase of ≥1.5 of plasma creatinine from baseline) and a composite marker, including major complications and/or extubation time>15days. Risk was evaluated using Cox proportional hazards regression analysis, considering some continuous predictors in the basal model (i.e., age, body surface area and Aristotle-score) to which cystatin-C peak values were added. Discrimination, calibration, and reclassification tests were also performed. RESULTS: 248 children (140 males) undergoing cardiac surgery (median age 6.5months; IQR: 1.7-40.1months; range 0-17years) have been enrolled. Post operatory Cystatin-C values were found to be an early diagnostic marker of AKI showing the best area under the ROC curve value (AUC) at 12h (0.746, CI 95% 0.674-0.818). In the multivariable analyses, peak cystatin-C values showed a significant hazard ratio (HR=2.665, CI 95% 1.750-4.059, p<0.001). Finally, post operatory cystatin-C at 12h significantly improved the AUC (p=0.017) compared to basal model, resulting a net gain in reclassification proportion (NRI=0.417, p<0.001). CONCLUSIONS: Our data show that cystatin-C should be considered an early biomarker of AKI, improving the risk prediction for complicated outcome in pediatric cardiac surgery.

Prognostic role of BNP in children undergoing surgery for congenital heart disease: analysis of prediction models incorporating standard risk factors.

BACKGROUND: The routine use of brain natriuretic peptide (BNP) in pediatric cardiac surgery remains controversial. Our aim was to test whether BNP adds information to predict risk in pediatric cardiac surgery. METHODS: In all, 587 children undergoing cardiac surgery (median age 6.3 months; 1.2-35.9 months) were prospectively enrolled at a single institution. BNP was measured pre-operatively, on every post-operative day in the intensive care unit, and before discharge. The primary outcome was major complications and length ventilator stay >15 days. A first risk prediction model was fitted using Cox proportional hazards model with age, body surface area and Aristotle score as continuous predictors. A second model was built adding cardiopulmonary bypass time and arterial lactate at the end of operation to the first model. Then, peak post-operative log-BNP was added to both models. Analysis to test discrimination, calibration, and reclassification were performed. RESULTS: BNP increased after surgery (p<0.001), peaking at a mean of 63.7 h (median 36 h, interquartile range 12-84 h) post-operatively and decreased thereafter. The hazard ratios (HR) for peak-BNP were highly significant (first model HR=1.40, p=0.006, second model HR=1.44, p=0.008), and the log-likelihood improved with the addition of BNP at 12 h (p=0.006; p=0.009). The adjunction of peak-BNP significantly improved the area under the ROC curve (first model p<0.001; second model p<0.001). The adjunction of peak-BNP also resulted in a net gain in reclassification proportion (first model NRI=0.089, p<0.001; second model NRI=0.139, p=0.003). CONCLUSIONS: Our data indicates that BNP may improve the risk prediction in pediatric cardiac surgery, supporting its routine use in this setting.

Supra-annular mitral valve implantation in very small children.

OBJECTIVE: Mitral valve replacement (MVR) is a surgical option when mitral valvuloplasty is not feasible/successful. This study reviews our experience with MVR in very young children. METHODS: From July 2004 to January 2014, seven children (mean age 13.3 ± 11.2 months; range 4 months to 35 months; mean body weight 6.0 ± 2.2 kg) underwent MVR with a mechanical prosthesis in the supra-annular position. To provide better exposure in the left atrium, we performed in all but one case a biatrial transeptal incision according to Guiraudon. Six patients had congenital defects of the mitral valve and one had rheumatic. Six patients had undergone previous cardiosurgical procedures. RESULTS: All patients were implanted with a CarboMedics (CarboMedics, Austin, TX, USA) mechanical prosthesis. Mean prosthesis size was 19.0 ± 3.1 mm (range 16 to 25). There were no cases of operative or late mortality. At follow-up (mean 67.1 ± 34.8 months; range 25 to 108 months) two patients (28.6%) required reoperation both for thrombotic pannus formation over the disc at two and three months from first operation, respectively; only in one case was replacement necessary. CONCLUSION: Supra-annular MVR may be considered a feasible secondary surgical option in children with a small annulus when mitral valvuloplasty is unsuccessful or unsuitable. Early and mid-term outcomes are acceptable but complications are not uncommon, especially related to thrombotic events.

Tranexamic acid therapy in pediatric cardiac surgery: a single-center study.

BACKGROUND: We conducted a retrospective study of cyanotic and acyanotic patients undergoing cardiopulmonary bypass to determine the effect of tranexamic acid on blood loss and blood products administered during the operation in pediatric cardiac surgery. METHODS: From January 2008 to December 2011, during 2 different periods, a total of 231 pediatric patients undergoing cardiac surgery with cardiopulmonary bypass (123 cyanotic, 108 acyanotic) were included in this study. A total of 104 patients were in the antifibrinolytic group and exclusively treated with tranexamic acid that was given as a bolus of 20 mg/kg(-1) after anesthetic induction and 20 mg/kg(-1) after protamine. The other 127 patients were in the control group. We analyzed intraoperative and postoperative outcomes of tranexamic acid administration. RESULTS: There were no differences in mortality or operative time, but blood loss in 48 hours was greater in the control group (p=0.0012). A significant difference was found in the amount of intraoperative erythrocyte concentrate transfused (140±55 vs 170±78 mL, p=0.0011) but not in number. The number and amount of erythrocyte concentrate transfused in the first 48 postoperative hours were also greater in the control group (45 vs 77 patients, p=0.012; 100±40 vs 120±55 mL, p=0.0022). There were not many differences in the effect of tranexamic acid between the cyanotic and acyanotic subgroup. CONCLUSIONS: This retrospective study provides evidence that tranexamic acid may be used in the field of congenital cardiac surgery effectively.

Sildenafil therapy for pulmonary hypertension before and after pediatric congenital heart surgery.

Pulmonary hypertension associated with pediatric congenital heart defects is a major cause of postoperative morbidity and death. Sildenafil has been combined with inhaled nitric oxide to treat pulmonary hypertension. We retrospectively studied the pre- and postoperative effects of oral sildenafil as monotherapy in children with pulmonary hypertension who underwent surgery to correct congenital cardiac defects. From September 2005 through November 2009, 38 children with moderate-to-severe pulmonary arterial hypertension (pulmonary arterial/aortic pressure ratio, >0.7) underwent cardiac surgery at our institution. Fifteen patients were given sildenafil (0.35 mg/kg, every 4 hr) orally or through nasogastric tubes 1 week before and 1 week after surgery. Twenty-three patients of comparable medical status were given sildenafil only upon the institution of cardiopulmonary bypass and for 1 week after surgery. Postoperatively, the 15 patients who were given preoperative sildenafil had significantly lower mean pulmonary arterial pressures (25.6 ± 3.1 vs. 30.4 ± 5.7 mmHg; P = 0.005) and pulmonary arterial/aortic pressure ratios (0.35 ± 0.05 vs. 0.42 ± 0.07; P = 0.002) than did the other 23 patients. The preoperative therapy also shortened cardiopulmonary bypass time, mechanical ventilation time, and lengths of intensive care unit and hospital stays. No sildenafil-related hypertensive crises or sequelae occurred. As monotherapy, oral sildenafil in low doses appears to control pulmonary hypertension safely and effectively in children undergoing operations to correct congenital heart defects, particularly when it is given both preoperatively and postoperatively. Further study is warranted.

Anterolateral minithoracotomies for the radical correction of congenital heart diseases.

During the past 10 years, minimally invasive surgical techniques have been applied more and more widely in both adult and pediatric populations, especially in female patients. Right anterolateral minithoracotomy is an alternative to median sternotomy that yields a better cosmetic outcome. From May 1997 through September 2008, 132 patients (107 females, 25 males) underwent open-heart surgery through right anterolateral minithoracotomy. Ages ranged from 1 to 49 years (mean, 10.12 yr). Mean body weight was 21.3 kg (range, 9.4-78 kg). Corrected defects included atrial septal defect, partial anomalous pulmonary venous connection, partial atrioventricular canal defect, ventricular septal defect alone or with pulmonary valve stenosis, tetralogy of Fallot, cor triatriatum, mitral valve defect, and pericardial cyst. The anterolateral skin incision was 5 to 7 cm. Our control group--which in the same period underwent the same interventional procedures through median sternotomy--consisted of 415 patients, 245 of whom were female (59%). There was no early or late death or major illness as a sequela. No patient required conversion to full sternotomy. All patients had gratifying cosmetic results at longer follow-up. Indeed the mortality and morbidity rates obtained through our approach were almost the same as those obtained through median sternotomy, and there were no significant differences in cardiopulmonary bypass time, aortic cross-clamp time, ventilation time, or postoperative hospital stay. We conclude that the right anterolateral minithoracotomy for correction of congenital heart defects offers superior cosmetic results without increasing morbidity or mortality rates and confers upon patients psychological and social satisfaction.