Minimal invasive approaches for pediatric & congenital heart surgery: safe, reproducible, more cosmetic than through sternotomy, and here to stay.

Minimal invasive approaches through small thoracic incisions for the isolated repair of the most common congenital heart defects have been around for decades. However, the lack of belonging in established surgical training curriculums compared to the traditional median sternotomy, the requirement for more technical expertise and a certain learning curve, has limited their use, being routinely performed only by certain surgeons in specialized centers. More recently, through cumulated and increasingly mediatized shared experience, remote teaching potential through universally accessible surgical videos and simulation, the approach has gained traction and acceptance, and even established itself as the new norm in many centers. In this review, we present technically focused aspects of our own experience and protocols which have evolved over time, along with a brief overview of the literature pertaining to other right thoracic approaches, and some comparison to established results using the traditional median sternotomy. An increasing body of literature, produced more frequently and across all continents, seems to suggest that repairs of congenital heart defects through a minimal invasive right thoracic approach are becoming the new norm, as they are reported to be safe and reproducible, with excellent surgical results, and an obvious superior and more desirable cosmetic result. This comes at a cost of additional training and learning curve by surgeons, who are not offered the technique as part of their standard professional training curriculum.

Mini right axillary thoracotomy for congenital heart defect repair can become a safe surgical routine.

OBJECTIVE: Owing to its obvious cosmetic appeal, minimal invasive repair of congenital heart defects (CHDs) through the mini right axillary thoracotomy is becoming routine in many centres. Besides cosmesis, and before becoming a new norm, it is important to establish its outcomes as safe compared to repairs through traditional median sternotomy. METHODS: Between 2013 and 2021, 116 consecutive patients underwent defect repairs through mini right axillary thoracotomy. Patient, operative data, and hospital outcomes were compared to contemporary mini right axillary thoracotomy and sternotomy series. RESULTS: There was no mortality or need for approach conversion (mean age 4.3 years, range 0.17-17, mean weight 18.6 kg, range 4.8-74.4) in 118 repairs for atrial septal defect, ventricular septal defect, partial anomalous pulmonary venous return, partial atrioventricular canal with mitral cleft, scimitar syndrome, double-chambered right ventricle, cor triatriatum, and tricuspid valve repair. Protocol included on-table extubation, achieved in 97 children, with 23 outliers leading to 0.7 average hours of mechanical ventilation (range 0-66 hours), indwelling chest drain time of 2.6 days (range 1-9 days), intensive care stay of 1.8 days (range 1-10 days), and hospital stay of 3.9 days (range 2-18 days). Late revisions were required in one patient after scimitar repair for scimitar vein stenosis at 2 weeks, and in another for repair of superior caval vein stenosis after a Warden operation at 2 months; reoperations (5/116 = 4.3%) were successfully performed through the same mini right axillary incision. CONCLUSIONS: While providing obvious cosmetic advantages, the minimally invasive right axillary thoracotomy approach for the surgical repair of common CHDs yields excellent results and is safe compared to the benchmark median sternotomy approach.

Advantages of a mini right axillary thoracotomy for congenital heart defect repair in children.

OBJECTIVES: The mini right axillary thoracotomy is an alternative surgical approach to repair certain congenital heart defects. Quality-of-life metrics and clinical outcomes in children undergoing either the right axillary approach or median sternotomy were compared. METHODS: Patients undergoing either approach for the same defects between 2018 and 2020 were included. Demographic details, operative data, and outcomes were compared between both groups. An abbreviated quality of life questionnaire based on the Infant/Toddler/Child Health Questionnaires focused on the patient's global health, physical activity, and pain/discomfort was administered to all parents/guardians within two post-operative years. RESULTS: Eighty-seven infants and children underwent surgical repair (right axillary thoracotomy, n = 54; sternotomy, n = 33) during the study period. There were no mortalities in either group. The right axillary thoracotomy group experienced significantly decreased red blood cell transfusion, intubation, intensive care, and hospital durations, and earlier chest tube removal. Up to 1 month, parents' perception of their child's degree and frequency of post-operative pain was significantly less after the right axillary thoracotomy approach. No difference was found in the patient's global health or physical activity limitations beyond a month between the two groups. CONCLUSIONS: With the mini right axillary approach, surrogates of faster clinical recovery and hospital discharge were noted, with a significantly less perceived degree and frequency of post-operative pain initially, but without the quality of life differences at last follow-up. While providing obvious cosmetic advantages, the minimally invasive right axillary thoracotomy approach for the surgical repair of certain congenital heart lesions is a safe alternative to median sternotomy.

Perioperative Anomalous Pulmonary Venous Return Evaluation With Low-Dose Cardiac Computed Tomography.

BACKGROUND: Low-dose multidetector computed tomographic angiography (MDCTA) is playing an increasingly larger role in the diagnosis of anomalous pulmonary venous return (APVR). Despite advances in new computed tomographic (CT) techniques with radiation dose reduction, there are limited studies describing radiation dose parameters to allow routine use of cardiac CT in infants and children with APVR. This study compares cardiac CT findings with intraoperative findings and describes comprehensive radiation exposure parameters. METHODS: A retrospective analysis of 27 patients compared MDCTA and intraoperative or cardiac catheterization findings of the pulmonary venous anatomy. RESULTS: A total of 32 MDCTA studies were performed on these 27 patients. Of the 28 studies with subsequent intervention, MDCTA accurately diagnosed the anomalous pulmonary venous anatomy in 27 (96.4%) patients. Narrowing of the pulmonary venous confluence entrance to the coronary sinus was missed on cardiac CT in one patient due to motion artifact, but it was noted intraoperatively. Median estimated effective radiation dose was 0.98 mSv (range: 0.39-3.2 mSv), and mean estimated effective radiation dose was 1.1 ± 0.68 mSv. Median total dose length product (DLP) was 25 mGy cm (range: 10-83 mGy cm), and mean total DLP was 28 ± 18 mGy cm. Median CTDI volume was 3.8 mGy (range: 2.5-14.6 mGy), and mean CTDI volume was 5.0 ± 3.2 mGy. CONCLUSIONS: We conclude that modern cardiac MDCTA is the best imaging modality to guide management in both preintervention and postintervention APVR patients. In this study, we describe comprehensive radiation exposure parameters in infants and children with APVR.

Results of Less Than 5-mm Right Ventricle-Pulmonary Artery Conduits in Single Ventricle Palliation.

BACKGROUND: Our programmatic approach to single ventricle (SV) neonatal palliation has evolved to using smaller-size right ventricle to pulmonary artery (RV-PA) conduits for pulmonary blood flow. Therefore, we sought to compare the early outcomes of less than 5-mm versus 5-mm RV-PA conduits in SV patients undergoing neonatal palliation. METHODS: We performed a retrospective review of SV patients who had neonatal palliation with either a less than 5-mm or 5-mm RV-PA connection, constructed using a waterproof, breathable fabric membrane graft (Gore-Tex, Flagstaff, AZ). Data before, during, and after surgery were analyzed using SPSS software. RESULTS: A total of 26 patients in group 1 (n = 11; <5 mm) and group 2 (n = 15; 5 mm) were operated on, with a mean follow-up of 13 months. Statistical differences (P ≤ .05) were noted with renal near-infrared spectroscopy (mean, 56 versus 44), and oxygen saturation at discharge (mean, 80% versus 85%) in groups 1 and 2, respectively. We found no significant difference in patient weight, peak lactate, cerebral near-infrared spectroscopy, length of intubation, length of hospitalization, or need for RV-PA conduit intervention after stage 1 along with age, branch PA sizes, or need for PA plasty at bidirectional Glenn between groups. CONCLUSIONS: Our early results of less than 5-mm conduits support further exploration into the tailoring of the RV-PA conduit size for each patient. A smaller-diameter conduit may improve systemic cardiac output and net oxygen delivery. Larger patient cohorts and longer follow-up with assessment of pulmonary artery growth are warranted to guide clinical practice.

Resuscitating the Chimney Graft to Innominate Artery for Straightforward Cannulation During Infancy.

Arterial cannulation with a chimney polytetrafluoroethylene graft to the innominate artery is commonly used for antegrade cerebral perfusion during neonatal aortic arch surgery. When properly retained and prepared before sternal closure, resuscitation of the polytetrafluoroethylene graft to innominate artery can be performed months later during sternal reentry. It is a safe and reproducible technique for expeditious arterial cannulation at stage II palliation in single-ventricle patients or complete intracardiac repair of biventricular lesions. We report our experience utilizing this technique successfully during reoperation in 90 of 92 patients, with no adverse thromboembolic events identified.

Pulmonary artery banding in complete atrioventricular septal defect.

OBJECTIVES: To analyze outcomes after pulmonary artery banding (PAB) in complete atrioventricular septal defect (AVSD), with a focus on surgical pathway outcome and timing, survival, and atrioventricular valve function. METHODS: PAB was performed in 50 of 474 infants (11%) from 28 institutions between 2012 and 2018 at a median age of 1.1 months. The median duration of follow-up was 2.1 years. Atrioventricular valve function was assessed by review of pre-PAB and predischarge echocardiograms (median, 9 days postoperatively). Competing-risks methodology was used to analyze the risks for biventricular repair, univentricular repair, and death. RESULTS: At 2 years, the proportions of patients who underwent biventricular repair, univentricular repair, and death were 68%, 13%, and 12%, respectively, with 8% awaiting definitive repair. After PAB, atrioventricular valve regurgitation decreased in 14 infants and increased in 10, but the distribution of regurgitation severity did not change significantly in the total cohort or subgroups. The intended management plan at PAB was deferred biventricular/univentricular decision (23 infants), 2-stage biventricular repair (24 infants), and univentricular repair (3 infants). Among the 24 infants intended for biventricular repair, 23 achieved biventricular repair and 1 died before repair. Survival at 4 years after biventricular repair among patients with previous PAB (93%) was similar to the 4-year survival of the patients who underwent primary biventricular repair (91%; n = 333). CONCLUSIONS: PAB is a successful strategy in complete AVSD to bridge to biventricular repair and has similar post-biventricular repair survival to primary biventricular repair. Changes in atrioventricular valve regurgitation after PAB were variable.

A Severe Form of Arterial Tortuosity Syndrome Presenting With Significant Airway Obstruction in an Infant.

We describe a severe form of arterial tortuosity syndrome in a newborn, in which the tortuous course of the aorta masqueraded as a pulmonary artery sling on fetal echocardiogram. The newborn presented with respiratory distress after birth. The clinical course was complicated by extrinsic airway obstruction requiring cardiopulmonary resuscitation. Timely diagnostic work-up in patients with arterial tortuosity syndrome is necessary to plan eventual intervention, and hopefully to prevent complications related to the abnormal vasculature.

Transfusing Platelets During Bypass Rewarming in Neonates Improves Postoperative Outcomes: A Randomized Controlled Trial.

BACKGROUND: In neonates, transfusion of platelets after hemodilution from cardiopulmonary bypass (CPB) has been standard. We hypothesize that platelet administration during the rewarming phase before termination of CPB would reduce coagulopathy, enhance hemostasis, reduce transfusion, and improve postoperative outcomes after neonatal cardiac surgery. METHODS: A prospective, randomized trial was performed in 46 neonates. Controls received platelets only at the end of bypass with other blood products to assist in hemostasis. The treatment group received 10 mL/kg of platelets during the rewarming phase of bypass after cross-clamp release. After protamine, transfusion and perioperative management protocols were identical and constant among groups. RESULTS: Two neonates in each group were excluded secondary to postoperative need for extracorporeal support. Controls (n = 21) and treatment patients (n = 21) were similar in age, weight, case complexity, associated syndromes, single ventricle status, and CPB times. Compared to controls, the treatment group required 40% less postbypass blood products (58 ± 29 vs 103 ± 80 mL/kg, P = .04), and case completion time after protamine administration was 28 minutes faster (P = .016). The treatment group required fewer postoperative mediastinal explorations for bleeding (P = .045) and had a lower fluid balance (P = .04). The treatment group had shorter mechanical ventilation (P = .016) and length of intensive care unit times (P = .033). There were no 30-day mortalities in either group. CONCLUSION: Platelet transfusion during the rewarming phase of neonatal cardiac surgery was associated with reduced bleeding and improved postoperative outcomes, compared to platelets given after coming off bypass. Further studies are necessary to understand mechanisms and benefits of this strategy.

Validity of Pulmonary Valve Z-Scores in Predicting Valve-Sparing Tetralogy Repairs-Systematic Review †.

There is a lack of consensus regarding the preoperative pulmonary valve (PV) Z-score "cut-off" in tetralogy of Fallot (ToF) patients to attempt a successful valve sparing surgery (VSS). Therefore, the aim of this study was to review the available evidence regarding the association between preoperative PV Z-score and rate of re-intervention for residual right ventricular outflow tract (RVOT) obstruction, i.e. successful valve sparing surgery. A systematic search of studies reporting outcomes of VSS for ToF was performed utilizing PubMed, EMBASE, and Scopus databases. Patients with ToF variants such as pulmonary atresia, major aortopulmonary collaterals, absent pulmonary valve, associated atrioventricular septal defect, and discontinuous pulmonary arteries were excluded. Out of 712 screened publications, 15 studies met inclusion criteria. A total of 1091 patients had surgery at a median age and weight of 6.9 months and 7.2 kg, respectively. VSS was performed on the basis of intraoperative PV assessment in 14 out of 15 studies. The median preoperative PV Z-score was -1.7 (0 to -4.9) with a median re-intervention rate of 4.7% (0-36.8%) during a median follow-up of 2.83 years (1.4-15.8 years). Quantitatively, there was no correlation between decreasing preoperative PV Z-scores and increasing RVOT re-intervention rates with a correlation coefficient of -0.03 and an associated p-value of 0.91. In observational studies, VSS for ToF repair was based on intraoperative evaluation and sizing of the PV following complete relief of all levels of obstruction of the RVOT, rather than pre-operative echocardiography derived PV Z-scores.

Results of international assistance for a paediatric heart surgery programme in a single Ukrainian centre.

BACKGROUND: Surgery for CHD has been slow to develop in parts of the former Soviet Union. The impact of an 8-year surgical assistance programme between an emerging centre and a multi-disciplinary international team that comprised healthcare professionals from developed cardiac programmes is analysed and presented.Material and methodsThe international paediatric assistance programme included five main components - intermittent clinical visits to the site annually, medical education, biomedical engineering support, nurse empowerment, and team-based practice development. Data were analysed from visiting teams and local databases before and since commencement of assistance in 2007 (era A: 2000-2007; era B: 2008-2015). The following variables were compared between periods: annual case volume, operative mortality, case complexity based on Risk Adjustment for Congenital Heart Surgery (RACHS-1), and RACHS-adjusted standardised mortality ratio. RESULTS: A total of 154 RACHS-classifiable operations were performed during era A, with a mean annual case volume by local surgeons of 19.3 at 95% confidence interval 14.3-24.2, with an operative mortality of 4.6% and a standardised mortality ratio of 2.1. In era B, surgical volume increased to a mean of 103.1 annual cases (95% confidence interval 69.1-137.2, p<0.0001). There was a non-significant (p=0.84) increase in operative mortality (5.7%), but a decrease in standardised mortality ratio (1.2) owing to an increase in case complexity. In era B, the proportion of local surgeon-led surgeries during visits from the international team increased from 0% (0/27) in 2008 to 98% (58/59) in the final year of analysis. CONCLUSIONS: The model of assistance described in this report led to improved adjusted mortality, increased case volume, complexity, and independent operating skills.

Intervention for arch obstruction after the Norwood procedure: Prevalence, associated factors, and practice variability.

OBJECTIVE: Arch obstruction after the Norwood procedure is common and contributes to mortality. We determined the prevalence, associated factors, and practice variability of arch reintervention and assessed whether arch reintervention is associated with mortality. METHODS: From 2005 to 2017, 593 neonates in the Congenital Heart Surgeons' Society Critical Left Heart Obstruction cohort underwent a Norwood procedure. Median follow-up was 3.7 years. Multivariable parametric models, including a modulated renewal analysis, were performed. RESULTS: Of the 593 neonates, 146 (25%) underwent 218 reinterventions for arch obstruction after the Norwood procedure: catheter-based (n = 168) or surgical (n = 50) at a median age of 4.3 months (quartile 1-quartile 3, 2.6-5.7). Interdigitation of the distal aortic anastomosis was protective against arch reintervention. Development of ≥ moderate tricuspid valve regurgitation and right ventricular dysfunction at any point was associated with arch reintervention. Nonsignificant variables for arch reintervention included shunt type and preoperative aortic measurements. Surgical arch reintervention was protective against arch reintervention, but transcatheter reintervention was associated with increased reintervention. Arch reintervention was not associated with increased mortality. There was wide institutional variation in incidence of arch reintervention (range, 0-40 reinterventions per 100 years patient follow-up) and in preintervention gradient (range, 0-64 mm Hg). CONCLUSIONS: Interdigitation of the distal aortic anastomosis during the Norwood procedure decreased the risk of arch reintervention. Surgical arch reintervention is more definitive than transcatheter. Arch reintervention after the Norwood procedure is not associated with increased mortality. Serial surveillance for arch obstruction, integrated with changes in right ventricular function and tricuspid valve regurgitation, is recommended after the Norwood procedure to improve outcomes.