Outcomes and re-interventions after one-stage repair of transposition of great arteries and aortic arch obstruction

Objectives: One-stage repair of transposition of great arteries (TGA) and aortic arch obstruction (AAO) is currently advocated, but carries formidable surgical challenges. This report presents our experience and re-interventions for residual lesions over the last 10 years. Methods: Twenty-two patients (19.5±42.4 days; range 2-206; median 10 days, 3.5±0.6kg) diagnosed with TGA (nine patients) or double outlet right ventricle (DORV) (13 patients) and AAO underwent one-stage repair. Of the nine TGA patients (two with intact ventricular septum), AAO were: two patients hypoplastic arch, one patient discrete coarctation, four patients hypoplastic arch with coarctation and two patients interrupted aortic arch. The 13 DORV patients were all of Taussig-Bing type and one showed multiple ventricular septal defects (VSDs). The degree of AAO ranged from hypoplastic arch in five patients, coarctation two patients, combined four patients and interrupted aortic arch (IAA) two patients. Arterial switch with Lecomte±VSD repair was performed during cooling, and aortic arch repair was performed under deep hypothermic circulatory arrest (DHCA) (35±14min at 16.9±0.7°C). Our preference was to use homograft patch-plasty for arch and direct end-to-side anastomosis for coarctation repair. Aortic-cross-clamp time was 124±24min and cardiopulmonary bypass (CPB) time 215±84min. Results: Early survival was 19/22 (86%) up to 30 days without mortality in the second half of our series. Three patients required extracorporeal membrane oxygenation (ECMO) support and renal support was needed in three and preferred permanent pace maker (PPM) implantation in two. Length of stay was 21.9±22.1 days. There was one late death and overall survival was 18/22 (82%) for the follow-up period of 4.8 years (0.2-9.8 years). Eight patients (44%) required re-intervention for re-coarctation. Four patients required right ventricular outflow tract (RVOT)/pulmonary artery re-interventions. At follow-up, there was no requirement for aortic valve replacement, residual VSD closure and no evidence of ventricular dysfunction. Conclusions: One-stage repair of TGA/DORV and AAO can be performed safely with a good survival rate. Three important lessons that we have learnt are as follows: (1) the subpulmonary VSD may have a perimembraneous component, (2) late re-coarctation is not infrequent and (3) late residual right-sided cardiac lesions remain an issue in complex TGA repai

VIRTUAL 3-D MODELLING OF AIRWAYS IN CONGENITAL HEART DEFECTS

The involvement of the airway is not uncommon in the presence of complex cardiovascular malformations. In these cases, a careful inspection of the relationship between the airway and the vasculature is paramount to plan the surgical procedure. Three-dimentional printing enhanced the visualization of the cardio-vascualr structure. Unfortunately IT does not allow to remove selected anatomy to improve the visualization of the surrounding ones. Computerized modelling (CM) of has the potential to fill this gap by allowing a dynamic handling of different anatomies, increasing the exposure of vessels or bronchi to show their relationship.. We started to use this technique to plan the surgical repair in these complex cases where the airway is affected. This technique is routinely used in our Institution as an additional tool in the pre-surgical assessment. We report 4 cases in which the airways were compressed by vascular structures : ascending aorta in 1, left pulmonary artery sling in 1, Patent ductus arteriosus (PDA) in 1 and major aorto-pulmonary collateral artery in 1. We believe this technique can enhance the understanding of the causes of airway involvement and facilitate the creation of an appropriate surgical plan

Outcomes and re-interventions after one-stage repair of transposition of great arteries and aortic arch obstruction

Objectives: One-stage repair of transposition of great arteries (TGA) and aortic arch obstruction (AAO) is currently advocated, but carries formidable surgical challenges. This report presents our experience and re-interventions for residual lesions over the last 10 years. Methods: Twenty-two patients (19.5 ± 42.4 days; range 2–206; median 10 days, 3.5 ± 0.6 kg) diagnosed with TGA (nine patients) or double outlet right ventricle (DORV) (13 patients) and AAO underwent one-stage repair. Of the nine TGA patients (two with intact ventricular septum), AAO were: two patients hypoplastic arch, one patient discrete coarctation, four patients hypoplastic arch with coarctation and two patients interrupted aortic arch. The 13 DORV patients were all of Taussig–Bing type and one showed multiple ventricular septal defects (VSDs). The degree of AAO ranged from hypoplastic arch in five patients, coarctation two patients, combined four patients and interrupted aortic arch (IAA) two patients. Arterial switch with Lecomte ± VSD repair was performed during cooling, and aortic arch repair was performed under deep hypothermic circulatory arrest (DHCA) (35 ± 14 min at 16.9 ± 0.7 °C). Our preference was to use homograft patch-plasty for arch and direct end-to-side anastomosis for coarctation repair. Aortic-cross-clamp time was 124 ± 24 min and cardiopulmonary bypass (CPB) time 215 ± 84 min. Results: Early survival was 19/22 (86%) up to 30 days without mortality in the second half of our series. Three patients required extracorporeal membrane oxygenation (ECMO) support and renal support was needed in three and preferred permanent pace maker (PPM) implantation in two. Length of stay was 21.9 ± 22.1 days. There was one late death and overall survival was 18/22 (82%) for the follow-up period of 4.8 years (0.2–9.8 years). Eight patients (44%) required re-intervention for re-coarctation. Four patients required right ventricular outflow tract (RVOT)/pulmonary artery re-interventions. At follow-up, there was no requirement for aortic valve replacement, residual VSD closure and no evidence of ventricular dysfunction. Conclusions: One-stage repair of TGA/DORV and AAO can be performed safely with a good survival rate. Three important lessons that we have learnt are as follows: (1) the subpulmonary VSD may have a perimembraneous component, (2) late re-coarctation is not infrequent and (3) late residual right-sided cardiac lesions remain an issue in complex TGA repair

Is decellularized porcine small intestine sub-mucosa patch suitable for aortic arch repair?

Introduction: We reviewed our experience with decellularized porcine small intestine sub-mucosa (DPSIS) patch, recently introduced for congenital heart defects. Materials and Methods: Between 10/2011 and 04/2016 a DPSIS patch was used in 51 patients, median age 1.1 months (5 days to 14.5 years), for aortic arch reconstruction (45/51 = 88.2%) or aortic coarctation repair (6/51 = 11.8%). All medical records were retrospectively reviewed, with primary endpoints interventional procedure (balloon dilatation) or surgery (DPSIS patch replacement) due to patch-related complications. Results: In a median follow-up time of 1.5 ± 1.1 years (0.6-2.3years) in 13/51 patients (25.5%) a re-intervention, percutaneous interventional procedure (5/51 = 9.8%) or re-operation (8/51 = 15.7%) was required because of obstruction in the correspondence of the DPSIS patch used to enlarge the aortic arch/isthmus, with median max velocity flow at Doppler interrogation of 4.0 ± 0.51 m/s. Two patients required surgery after failed interventional cardiology. The mean interval between DPSIS patch implantation and re-intervention (percutaneous procedure or re-operation) was 6 months (1-17 months). While there were 3 hospital deaths (3/51 = 5.9%) not related to the patch implantation, no early or late mortality occurred for the subsequent procedure required for DPSIS patch interventional cardiology or surgery. The median max velocity flow at Doppler interrogation through the aortic arch/isthmus for the patients who did not require interventional procedure or surgery was 1.7 ± 0.57 m/s. Conclusions: High incidence of re-interventions with DPSIS patch for aortic arch and/or coarctation forced us to use alternative materials (homografts and decellularized gluteraldehyde preserved bovine pericardial matrix)

Comparison of Early Outcomes for Normothermic and Hypothermic Cardiopulmonary Bypass in Children Undergoing Congenital Heart Surgery.

Objective: Comparison of early outcomes of normothermic cardiopulmonary bypass (N-CPB, ≥35°C) with hypothermic cardiopulmonary bypass (H-CPB, 28-34°C) for congenital heart defects. Methods: Data from 99 patients <2 years operated with N-CPB (n = 48) or H-CPB (n = 51) were retrospectively reviewed: aortic X-clamping and CPB duration, vasoactive inotropic score (VIS), arterial lactate, pH and base excess, urine output, extubation, PICU stay, transfusion requirements, chest drain losses, costs of transfusions, and costs of PICU stay. Results: The two groups were homogeneous for diagnosis, risk factors, surgery and demographic variables: N-CPB age 7.7 ± 6.1 months, weight 6.2 ± 2.4 kg, and H-CPB age 6.6 ± 6.5 months, weight 6.1 ± 2.4 kg. There were no hospital deaths in either group. VIS in N-CPB was lower than H-CPB on PICU arrival (9.7 ± 5.9 vs. 13.4 ± 7.9, P < 0.005), after 4 h (7.0 ± 5.2 vs. 11.1 ± 7.3, P < 0.001) and 24 h (2.8 ± 3.6 vs. 5.6 ± 5.6, P < 0.003); arterial pH was better at PICU arrival (7.33 ± 0.09 vs. 7.30 ± 0.09, P = 0.046) after 4 h (7.35 ± 0.07 vs. 7.32 ± 0.07, P = 0.022) and after 24 h (7.37 ± 0.05 vs. 7.35 ± 0.05, P = 0.01). Extubation was earlier in N-CPB than in H-CPB (22 ± 27 vs. 48 ± 57 h, P = 0.003) as PICU discharge (61 ± 46 h vs. 87 ± 69 h, P = 0.021). Transfusion requirements in operating room were lower in N-CPB vs. H-CPB for RBC, FFP, cryoprecipitate, and platelets, while during the first 24 h in PICU were lower only for cryoprecipitate and platelets. Chest drain losses (mL/kg) on PICU arrival, after 4 and 24 h were lower with N-CPB vs. H-CPB (respectively 1.5 ± 1.4 vs. 2.5 ± 2.7, P = 0.013, 7.8 ± 6.0 vs. 10.9 ± 8.7, P = 0.025, and 23.0 ± 12.0 vs. 27.9 ± 15.2, P = 0.043). Tranexamic acid infusion was required in 7/48 (14.6%) patients with N-CPB vs. 18/51(= 35.3%) in H-CPB (P = 0.009). The average total costs/patient of blood and blood products (RBC, FFP, cryoprecipitate, platelets) were lower in N-CPB vs. H-CPB for both the first 24 h after surgery (£204 ± 169 vs. £306 ± 254, P = 0.011) as well as during the total duration of PICU stay (£239 ± 193 vs. £427 ± 337, P = 0.001). The average cost/patient/day of stay in PICU was lower in N-CPB than in H-CPB (£4,067 ± 3,067 vs. £5,800 ± 4,600, P = 0.021). Conclusions: N-CPB may reduce inotropic and respiratory support, shorten PICU stay, and decrease peri-operative transfusion requirements, with subsequent costs reduction, compared to H-CPB. Future studies are needed to validate and support wider use of N-CPB