Adult coronary artery bypass grafting by congenital surgeons-a propensity matched analysis.

OBJECTIVES: Surgical myocardial revascularization will be increasingly needed in adult patients with congenital heart disease. We investigated the results of coronary artery bypass grafting (CABG) performed on adults by congenital cardiac surgeons at our institution. METHODS: We conducted a retrospective, single-centre study. Adults undergoing isolated or combined CABG from 2004 to 2017 were included. Early and late outcomes were analyzed for the whole cohort. Furthermore, a propensity matched analysis was conducted comparing the results of isolated CABG between congenital and adult surgeons. RESULTS: A total of 514 and 113 patients had isolated and combined CABG for acquired heart disease, respectively. A total of 33 patients had myocardial revascularization at the time of surgery for congenital heart disease. Overall early mortality was 1.2%, the rate of re-exploration for bleeding was 4.5%, and an internal mammary artery to left anterior descending artery graft was used in 85.6% patients. One-year survival was 97.5% (96.2-98.8%), and 5-year survival was 88.0% (84.8-91.3%). After propensity matching (468 pairs), early mortality (0.6% vs 1.2%, P = 0.51), re-exploration for bleeding (3.6% vs 3.0%, P = 0.72), use of internal mammary artery to left anterior descending artery graft (92.7% vs 91.9%, P = 0.70) and late survival did not differ between congenital surgeons and adult surgeons, respectively. CONCLUSIONS: Surgical myocardial revascularization can be required for adult congenital patients in a broad spectrum of clinical situations. Despite lower volumes, congenital cardiac surgeons perform CABG safely and with results that are comparable to those of the adult surgeons at our centre.

Age over 35 years is associated with increased mortality after pulmonary valve replacement in repaired tetralogy of Fallot: results from the UK National Congenital Heart Disease Audit database.

OBJECTIVES: Many adults with repaired tetralogy of Fallot will require a pulmonary valve replacement (PVR), but there is no consensus on the best timing. In this study, we aim to evaluate the impact of age at PVR on outcomes. METHODS: This is a national multicentre retrospective study including all patients >15 years of age with repaired tetralogy of Fallot who underwent their first PVR between 2000 and 2013. The optimal age cut-off was identified using Cox regression and classification and regression tree analysis. RESULTS: A total of 707 patients were included, median age 26 (15-72) years. The mortality rate at 10 years after PVR was 4.2%, and the second PVR rate of 6.8%. Age at PVR of 35 years was identified as the optimal cut-off in relation to late mortality. Patients above 35 years of age had a 5.6 fold risk of death at 10 years compared with those with PVR under 35 years (10.4% vs 1.3%, P < 0.001), more concomitant tricuspid valve repair/replacement (15.1% vs 5.7%, P < 0.001) and surgical arrhythmia treatment (18.4% vs 5.9%, P < 0.001). In those under 50 years, there was an 8.7 fold risk of late death compared with the general population, higher for those with PVR after 35 than those with PVR below 35 years (hazard ratio 9.9 vs 7.4). CONCLUSIONS: Patients above 35 years of age with repaired tetralogy of Fallot have significantly worse mortality after PVR, compared with younger patients and a higher burden of mortality relative to the general population. This suggests that there are still cases where the timing of initial PVR is not optimal, warranting a re-evaluation of criteria for intervention.

Direct Arterial Cannulation Allows Easy and Safe Continuous Selective Cerebral Perfusion During Repair of Interrupted Aortic Arch Even for Low Birth Weight Neonates.

OBJECTIVES: To review the outcomes of direct innominate artery cannulation for continuous cerebral perfusion used for repair of interrupted aortic arch (IAA) in a consecutive cohort of neonates regardless of weight. METHODS: Between September 1999 and April 2016, forty-four children with IAA (18 type A and 26 type B) underwent repair using continuous, hypothermic (18°C) low-flow cerebral perfusion via direct innominate artery cannulation. Associated cardiac lesions were truncus arteriosus (TA; 5), ventricular septal defect (VSD; 30), transposition of the great arteries (TGA; 1), unbalanced atrioventricular septal defect (1), double-inlet left ventricle (1), double-outlet right ventricle (3), and aortopulmonary window (APW; 5). Truncus arteriosus, single VSD, TGA, and APW were corrected while the other patients were palliated. RESULTS: Age at the time of surgery was 7 days (4-120 days) and weight 3.1 kg (2.1-5.8 kg). Selective cerebral perfusion was maintained in all patients. During the selective cerebral perfusion, perfusion flow rate was maintained at 30 mL/kg/min. Aortic cross-clamp time, low-flow, and total cardiopulmonary bypass time were 63 (40-116), 28 (17-41), and 108 (80-217) minutes, respectively. There were no deaths nor clinical evidence of neurological injury. Postoperative ventilation time, length of intensive care unit, and hospital stay were 3 (2-14), 5 (3-21), and 13 (6-27) days, respectively. Follow-up, complete at 84 months (24-221), revealed no late clinically evident neurologic sequelae nor innominate artery complications. CONCLUSIONS: Direct innominate arterial cannulation with continuous selective cerebral perfusion can be safely applied for repair of IAA even in low birth weight neonates. It is technically simple and associated with excellent clinical outcomes.

Primary repair versus surgical and transcatheter palliation in infants with tetralogy of Fallot.

OBJECTIVES: Treatment of infants with tetralogy of Fallot (ToF) has evolved in the last two decades with increasing use of primary surgical repair (PrR) and transcatheter right ventricular outflow tract palliation (RVOTd), and fewer systemic-to-pulmonary shunts (SPS). We aim to report contemporary results using these treatment options in a comparative study. METHODS: This a retrospective study using data from the UK National Congenital Heart Disease Audit. All infants (n=1662, median age 181 days) with ToF and no other complex defects undergoing repair or palliation between 2000 and 2013 were considered. Matching algorithms were used to minimise confounding due to lower age and weight in those palliated. RESULTS: Patients underwent PrR (n=1244), SPS (n=311) or RVOTd (n=107). Mortality at 12 years was higher when repair or palliation was performed before the age of 60 days rather than after, most significantly for primary repair (18.7% vs 2.2%, P<0.001), less so for RVOTd (10.8% vs 0%, P=0.06) or SPS (12.4% vs 8.3%, P=0.2). In the matched groups of patients, RVOTd was associated with more right ventricular outflow tract (RVOT) reinterventions (HR=2.3, P=0.05 vs PrR, HR=7.2, P=0.001 vs SPS) and fewer pulmonary valve replacements (PVR) (HR=0.3 vs PrR, P=0.05) at 12 years, with lower mortality after complete repair (HR=0.2 versus PrR, P=0.09). CONCLUSIONS: We found that RVOTd was associated with more RVOT reinterventions, fewer PVR and fewer deaths when compared with PrR in comparable, young infants, especially so in those under 60 days at the time of the first procedure.

Aortic Valve Replacement and the Ross Operation in Children and Young Adults.

BACKGROUND: There are several options available for aortic valve replacement (AVR), with few comparative reports in the literature. The optimal choice for AVR in each age group is not clear. OBJECTIVES: The study sought to report and compare outcomes after AVR in the young using data from a national database. METHODS: AVR procedures were compared after advanced matching, both in pairs and in a 3-way manner, using a Bayesian dynamic survival model. RESULTS: A total of 1,501 patients who underwent AVR in the United Kingdom between 2000 and 2012 were included. Of these, 47.8% had a Ross procedure, 37.8% a mechanical AVR, 10.9% a bioprosthesis AVR, and 3.5% a homograft AVR, with Ross patients being significantly younger when compared to the other groups. Overall survival at 12 years was 94.6%. In children, the Ross procedure had a 12.7% higher event-free probability (death or any reintervention) at 10 years when compared to mechanical AVR (p = 0.05). We also compared all procedures except the homograft in a matched population of young adults, where the bioprosthesis had the lowest event-free probability of 78.8%, followed by comparable results in mechanical AVR and Ross, with 86.3% and 89.6%, respectively. Younger age was associated with mortality and pulmonary reintervention in the Ross group and with aortic reintervention in the mechanical AVR. Of all 3 options, only the patients undergoing the Ross procedure approached the survival of the general population. CONCLUSIONS: AVR in the young achieves good results, with the Ross being overall better suited for this age group, especially in children. Although freedom from aortic valve reintervention is superior after the Ross procedure, the need for homograft reinterventions is an issue to take into account. All methods have advantages and limitations, with reinterventions being an issue in the long term for all, more crucially in smaller children.

Anomalous Left Coronary From the Pulmonary Artery Presenting as Ventricular Fibrillation After Persistent Ductus Arteriosus Ligation.

An anomalous left coronary artery from the pulmonary artery (ALCAPA) is rarely associated with persistent ductus arteriosus (PDA). A large PDA can maintain perfusion in the left coronary artery, delaying presentation. Assessing the origin of the coronary arteries before PDA ligation is difficult, often being performed in very small or even preterm babies. We present the case of a 5-month-old infant with echocardiographic features of mitral regurgitation and subendocardial ischemia who experienced ischemia and cardiac arrest after PDA ligation. Transesophageal echocardiography demonstrated ALCAPA, and left coronary translocation was performed. The infant was discharged after 10 days.

Normothermic versus hypothermic cardiopulmonary bypass in children undergoing open heart surgery (thermic-2): study protocol for a randomized controlled trial.

BACKGROUND: During open heart surgery, patients are connected to a heart-lung bypass machine that pumps blood around the body ("perfusion") while the heart is stopped. Typically the blood is cooled during this procedure ("hypothermia") and warmed to normal body temperature once the operation has been completed. The main rationale for "whole body cooling" is to protect organs such as the brain, kidneys, lungs, and heart from injury during bypass by reducing the body's metabolic rate and decreasing oxygen consumption. However, hypothermic perfusion also has disadvantages that can contribute toward an extended postoperative hospital stay. Research in adults and small randomized controlled trials in children suggest some benefits to keeping the blood at normal body temperature throughout surgery ("normothermia"). However, the two techniques have not been extensively compared in children. OBJECTIVE: The Thermic-2 study will test the hypothesis that the whole body inflammatory response to the nonphysiological bypass and its detrimental effects on different organ functions may be attenuated by maintaining the body at 35°C-37°C (normothermic) rather than 28°C (hypothermic) during pediatric complex open heart surgery. METHODS: This is a single-center, randomized controlled trial comparing the effectiveness and acceptability of normothermic versus hypothermic bypass in 141 children with congenital heart disease undergoing open heart surgery. Children having scheduled surgery to repair a heart defect not requiring deep hypothermic circulatory arrest represent the target study population. The co-primary clinical outcomes are duration of inotropic support, intubation time, and postoperative hospital stay. Secondary outcomes are in-hospital mortality and morbidity, blood loss and transfusion requirements, pre- and post-operative echocardiographic findings, routine blood gas and blood test results, renal function, cerebral function, regional oxygen saturation of blood in the cerebral cortex, assessment of genomic expression changes in cardiac tissue biopsies, and neuropsychological development. RESULTS: A total of 141 patients have been successfully randomized over 2 years and 10 months and are now being followed-up for 1 year. Results will be published in 2015. CONCLUSIONS: We believe this to be the first large pragmatic study comparing clinical outcomes during normothermic versus hypothermic bypass in complex open heart surgery in children. It is expected that this work will provide important information to improve strategies of cardiopulmonary bypass perfusion and therefore decrease the inevitable organ damage that occurs during nonphysiological body perfusion. TRIAL REGISTRATION: ISRCTN Registry: ISRCTN93129502, http://www.isrctn.com/ISRCTN93129502 (Archived by WebCitation at http://www.webcitation.org/6Yf5VSyyG).

Does the persistence of pulsatile antegrade pulmonary blood flow following bidirectional Glenn procedure affect long term outcome?

OBJECTIVES: To evaluate outcomes of the Glenn procedure with or without pulsatile antegrade pulmonary blood flow during palliation of patients with functionally single ventricles. METHODS: The clinical records of 111 consecutive patients who underwent a bidirectional Glenn procedure for palliation of single ventricle morphologies at our institution between 1997 and 2010 were reviewed. We specifically excluded infants with the diagnosis of hypoplastic left heart syndrome. Following the Glenn procedure, there were 57 patients (Group 1) with and 54 (Group 2) without antegrade pulmonary blood flow. We reviewed their long term data from our database to determine whether the presence of forward flow after the Glenn procedure affected outcome. RESULTS: There was 1 early hospital death (in Group 1). The mean SaO2 at discharge was higher in Group 1 (83%±2 vs 78%±4; P<0.001). There was no difference in duration of chest drain insertion, length of intensive care and hospital stay between the two groups. The median follow-up time was 7.1 years (range, 1.7-14.9 years). Sixty-five patients underwent Fontan completion (35 from Group 1) a median of 3.6 years (Group 1) and 3.3 (Group 2) after the Glenn procedure. Three patients died following Fontan completion (1 from Group 1). The 5- and 10-year survival (95% CI) was 96% (84-98%) and 96% (84-98%) in Group 1, and 88% (74-94%) and 82% (66-91%) in Group 2, respectively (log-rank; P=0.03). There was no significant difference in SaO2 levels, or incidence of systemic atrioventricular valve regurgitation or ventricular dysfunction in survivors between groups at the last follow-up. CONCLUSIONS: We conclude that leaving antegrade flow following a Glenn procedure improves oxygen saturation significantly and while it does not impact on short term outcome or hospital stay, long-term outcome is significantly better.

Surgery for simple and complex subaortic stenosis in children and young adults: results from a prospective, procedure-based national database.

OBJECTIVE: To identify the outcomes of surgically treated subaortic stenosis in a national population. METHODS: From 2000 to 2013, 1047 patients aged < 40 years underwent 1142 subaortic stenosis procedures. Of the 1047 patients, 484 (46.2%) were considered to have complex stenosis (CS) because at or before the first operation they had mitral valve (MV) disease, aortic valve disease, aortic coarctation or an interrupted aortic arch. RESULTS: The 30-day mortality was 0.7% for simple stenosis (SS), 2.3% for CS (P = .06), and 1.6% overall. Age < 1 year (P < .01), MV procedure (P = .02) and an interrupted aortic arch at the index procedure (P < .01) were risk factors for early death. Konno-type procedure early mortality was 2.4%. The 12-year survival was 97.1%, with a significant difference between SS and CS (hazard ratio [HR], 4.53; P = .02). Having MV disease alone (HR, 4.11; P = .02), MV disease plus aortic coarctation (HR, 6.73; P = .008), and age < 1 year (HR, 6.72; P < .001) were risk factors for late mortality. Freedom from subaortic reintervention overall was 92.3% and 88.5% at 5 and 12 years, respectively, much greater with CS than with SS (HR, 4.91; P < .0001). The independent risk factors for reintervention were younger age at the index procedure (HR, 0.1/y; P = .002), concomitant MV procedure (HR, 2.68; P = .019), ventricular septal defect plus interrupted aortic arch (HR, 3.19; P = .014), and ventricular septal defect plus aortic coarctation (HR, 2.41; P = .023). Undergoing a concomitant aortic valve procedure at the index procedure was protective (HR, 0.29; P = .025). CONCLUSIONS: Patients with SS had excellent outcomes. However, those with CS had worse long-term survival and freedom from reintervention, with morbidity and mortality greatest in young patients with multiple lesions. Additional evaluation in large-scale prospective studies is warranted.

Controlled reoxygenation during cardiopulmonary bypass decreases markers of organ damage, inflammation, and oxidative stress in single-ventricle patients undergoing pediatric heart surgery.

OBJECTIVE: Single-ventricle patients undergoing pediatric heart surgery are a high-risk group owing to reoxygenation injury during cardiopulmonary bypass (CPB). The present study investigated the effects of controlled reoxygenation CPB on biomarkers of organ damage, inflammation, stress, and long-term functional outcomes in cyanotic patients with either a single or double ventricle during open heart surgery. METHODS: Cyanotic patients with either a single (n = 32) or double (n = 47) ventricle undergoing surgical correction were randomized to receive CPB using either standard oxygen levels or controlled reoxygenation. The markers of cardiac injury, inflammation, stress, and cerebral and hepatic injury were measured preoperatively, at 10 and 30 minutes after starting CPB, and at 10 minutes and 4 and 24 hours after CPB. The data were analyzed using a mixed regression model. RESULTS: No difference was found in the pre- or intraoperative characteristics between the standard and controlled reoxygenation CPB groups for single- or double-ventricle patients. In the single-ventricle patients, controlled reoxygenation CPB significantly (P < .05) decreased the markers of organ damage, inflammation, stress, and oxidative stress. In contrast, the markers of inflammation and cardiac injury were not altered by controlled reoxygenation CPB in the double-ventricle patients. CONCLUSIONS: Controlled reoxygenation CPB decreased the markers of organ damage, stress, inflammation, and oxidative stress in single-ventricle patients undergoing cardiac surgery.

Effect of normothermic cardiopulmonary bypass on renal injury in pediatric cardiac surgery: a randomized controlled trial.

OBJECTIVE: Hypothermic cardiopulmonary bypass (CPB), although associated with a reduction in oxygen requirement, has a number of disadvantages including detrimental effects on enzymatic function, energy generation, and cellular integrity. Normothermic perfusion is potentially a more physiologic method to maintain the functional integrity of major organ systems. One of the aims of this trial was to compare the effect of normothermic and hypothermic CPB on renal injury in pediatric patients undergoing cardiac surgery. METHODS: Fifty-nine children (median age, 78 months; interquartile range, 39-130) undergoing corrective cardiac surgery were randomized to either hypothermic (28°C) or normothermic (35°C-37°C) CPB. Urinary albumin, retinal binding protein (RBP) and N-acetyl-ß-glucosaminidase (NAG) were measured preoperatively, end of CPB, 4, and 24 hours postoperatively and were expressed as a ratio of urinary creatinine. Serum creatinine was measured preoperatively, end of CPB, and 24 and 48 hours postoperatively. Results are expressed as a difference in means (normotheric - hypothermic) or as a ratio of geometric means (normothermic/hypothermic). RESULTS: Baseline characteristics were similar in both groups. For these biochemical markers no significant interactions between treatment and postintervention time were found. Serum creatinine (-2.10; 95% confidence interval [CI], -6.51-2.31), RBP (ratio, 0.96; 95% CI, 0.65-1.41), and NAG (ratio, 0.86; 95% CI, 0.56-1.36) were similar in the 2 groups (P ≥ .34), but the urinary albumin was significantly lower in the normothermic group (ratio, 0.63; 95% CI, 0.42-0.95, P = .03). CONCLUSIONS: Normothermic CPB is associated with similar renal impairment to hypothermic CPB in children undergoing heart surgery.

Reverse subclavian flap and aorto-pulmonary window technique for repair of interrupted aortic arch and truncus arteriosus.

We report a surgical strategy for repairing an interrupted aortic arch (IAA) with truncus arteriosus (TA) by using a reverse subclavian flap and an aorto-pulmonary (A-P) window technique for preserving the pulmonary artery architecture. A 10-day-old neonate with type B IAA and type I TA with echocardiographic evidence of a significant distance between the ascending and descending aorta underwent surgical repair at the Bristol Royal Hospital for Children. The superior part of the arch was reconstructed using a reverse subclavian flap and the undersurface with a pulmonary homograft patch. The ascending aorta was separated from the pulmonary arteries using a Gore-Tex patch (A-P window type of repair) without disconnecting the branch pulmonary arteries, in order to preserve their architecture. The continuity between the right ventricle and the pulmonary artery bifurcation was established using a 12 mm Contegra conduit. The postoperative course was uneventful, and the neonate was discharged after 12 days. At follow-up, the patient remains well, gaining weight, with no echocardiographic evidences of obstruction. Reverse subclavian flap with homograft patch combined with and 'A-P window' technique for preservation of the pulmonary artery architecture is a useful and effective surgical strategy for neonates presenting with IAA associated with TA.

Influence of tracheobronchomalacia on outcome of surgery in children with congenital heart disease and its management.

BACKGROUND: Patients with complex congenital heart disease associated with tracheobronchomalacia (TBM) remain difficult to manage after cardiac surgery. We studied the influence of TBM on the outcomes of pediatric patients after cardiac surgery for congenital heart disease to determine how to manage these patients better. METHODS: Twenty-two consecutive pediatric patients who had TBM diagnosed by bronchoscopy or dynamic contrast bronchography before or after cardiac surgery for congenital heart disease during a 5.5-year period were compared with an age- and procedure-matched control group operated on during the same period. Patients diagnosed postoperatively were investigated after a second failed extubation. Patients were managed by oxygen administration, endotracheal suctioning, and positive end-expiratory or continuous positive airway pressure through a nasotracheal tube or tracheostomy. RESULTS: There were 4 deaths within 1 year of surgery, all in the study group, with 2 early (neither of which appeared related to TBM) and 2 late. The estimated survival at 5 years was 82% (95% confidence interval, 59% to 93%) for the study group compared with 100% for control patients (p = 0.012). All deaths occurred in patients undergoing palliative procedures (p = 0.0004), and both children who underwent redo operations died (p = 0.02). Postoperatively, 50% of children with TBM required prolonged ventilation and tracheostomy. Compared with control patients the average postoperative ventilation time, pediatric intensive care unit stay, and hospital stay were 6.5, 11.5, and 20 days versus 1, 2, and 6.5 days, respectively (p < 0.001). CONCLUSIONS: Although associated with longer postoperative ventilation time, pediatric intensive care unit stay, hospital stay, and mortality, outcomes after cardiac procedures in children with TBM are acceptable. Palliative and redo procedures in this group of patients are associated with significantly higher risk of death.

The effects of normoxic versus hyperoxic cardiopulmonary bypass on oxidative stress and inflammatory response in cyanotic pediatric patients undergoing open cardiac surgery: a randomized controlled trial.

OBJECTIVES: This study investigates the effects of controlled reoxygenation cardiopulmonary bypass on oxidative stress, inflammatory response, and organ function in children undergoing repair of cyanotic congenital heart defects. METHODS: Sixty-seven cyanotic patients (median age 15 months, interquartile range 6-49 months) undergoing corrective cardiac surgery were randomized to receive either controlled normoxic (50-0 mm Hg; n = 35) or hyperoxic (150-180 mm Hg; n = 32) cardiopulmonary bypass. Troponin I and 8-isoprostane, C3a, interleukins 6, 8, and 10, cortisol, protein S100, and alpha-glutamate transferase were measured preoperatively and 10 and 30 minutes after starting bypass, on removal of the aortic crossclamp, and 12 and 24 hours thereafter. RESULTS: Overall, troponin I and 8-isoprostane levels were lower in the controlled normoxic group (-29%, 95% CI -48% to -3%, P = .03, and -26%, 95% CI -44% to -2%, P = .03, respectively). Protein S100 release was also lower in the normoxic group 10 minutes after starting bypass (-26%, 95% CI -40% to -9%, P = .005) and 10 minutes after aortic crossclamp removal (-23%, 95% CI -38% to -3%, P = .02, respectively), but similar at other time points in the two groups (P >or= .17). The alpha-glutamate transferase release was significantly lower in the normoxic group 10 minutes after aortic crossclamp removal (-28%, 95% CI -44% to -9%, P = .006, respectively) but was similar at other times (P >or= .11). Release of C3a, interleukins 6, 8, and 10, and cortisol was similar in the two groups throughout (P >or= .15). CONCLUSION: Controlled reoxygenation on starting cardiopulmonary bypass is associated with reduced myocardial damage, oxidative stress, and cerebral and hepatic injury compared with hyperoxic bypass and similar whole body inflammatory and stress response in cyanotic children undergoing open cardiac surgery.

The current role of hybrid procedures in the stage 1 palliation of patients with hypoplastic left heart syndrome.

Hypoplastic left heart syndrome is a relatively common cardiac malformation, accounting for 4-9% of children born with congenital heart disease. Since 1981 the mainstay of treatment has been the Norwood series of operations which have been variously modified, most recently using an RV-PA conduit (Sano shunt). With these surgical modifications and with increased experience in perioperative care survival for the surgical approach to completion of stage 2 palliation has improved to 70-80%. However, in 1997 when surgical results were poorer, interventional cardiologists explored the possibility of hybrid palliation of these children using a combined transluminal and closed surgical technique as it was perceived this would be less traumatic. Poor initial results caused many to discontinue this approach but some persevered, and with increasing experience survival to completion of stage 2 following hybrid palliation is now 50-80%. Although these results may superficially appear to be poorer than for surgery, it must be observed that increasingly the hybrid approach has been used by many groups as palliation for the highest risk cases (particularly small size and adverse cardiac factors). This review therefore considers what is the optimal management of children with hypoplastic left heart syndrome currently, and, specifically, what is the role for the hybrid approach in palliation of patients with hypoplastic left heart syndrome?