Total Aortic Arch and Neoaortic Root Replacement Following Stage III Palliation for Hypoplastic Left Heart Syndrome.

Progressive dilatation of the neoaortic root and reconstructed aortic arch is a serious complication after the Norwood procedure. There are no clear guidelines on the management of this complex anatomy in the setting of single ventricle physiology, and the surgical treatment of such an entity remains anecdotal. We describe a successful surgical repair in a 15-year-old girl presenting with a severely dilated neoaortic root and aortic arch causing compression and narrowing of the left pulmonary artery after successful three-stage palliation for hypoplastic left heart syndrome.

Ventricular Septal Defect Exposure by Tricuspid Valve Chordal Detachment-A Retrospective Matched Study.

Background: Transatrial approach is the standard method in repairing ventricular septal defects (VSD) in the pediatric population. However, the tricuspid valve (TV) apparatus might obscure the inferior border of the VSD risking the adequacy of repair by leaving residual VSD or heart block. Detachment of the TV chordae has been described as an alternative technique to TV leaflet detachment. The aim of this study is to investigate the safety of such a technique. Methods: Retrospective review of patients who underwent VSD repair between 2015 and 2018. Group A (n = 25) had VSD repair with TV chordae detachment were matched for age and weight to group B (n = 25) without tricuspid chordal or leaflet detachment. Electrocardiogram (ECG) and echocardiogram at discharge and at 3 years of follow-up were reviewed to identify new ECG changes, residual VSD, and TV regurgitation. Results: Median ages in groups A and B were 6.13 (IQR 4.33-7.91) and 6.33 (4.77-7.2) months. New onset right bundle branch block (RBBB) was diagnosed at discharge in 28% (n = 7) of group A versus 56% (n = 14) in group B (P = .044), while the incidence dropped to 16% (n = 4) in group A versus 40% (n = 10) in group B (P = .059) in the 3 years follow-up ECG. Echocardiogram at discharge showed moderate tricuspid regurgitation in 16% (n = 4) in group A and 12% (n = 3) in group B (P = .867). Three years of follow-up echocardiography revealed no moderate or severe tricuspid regurgitation and no significant residual VSD in either group. Conclusion: No significant difference in operative time was observed between the two techniques. TV chordal detachment technique reduces the incidence of postoperative RBBB without increasing the incidence of TV regurgitation at discharge.

Ventricular septal defect: diagnosis and treatments in the neonates: a systematic review.

BACKGROUND: Medical advancements have encouraged minimally invasive surgical repair of congenital heart defects such as ventricular septal defects (VSDs), and the diagnostic process can now be carried out using non-traditional techniques such as pulse oximetry. This, in turn, has improved clinical outcomes with reduced complication rates post-surgery. However, the variations in type of VSDs, age of patient, comorbidities, and access to closure devices may limit the efficacy of surgical advancements. METHODS: Articles were identified amongst Scopus, MEDLINE, and PubMed using various relevant search strings using PRISMA guidelines. Of the 115 articles initially extracted, 10 were eventually reviewed after duplicates and irrelevant studies were removed. RESULTS: Of the 24 eligible articles, 10 papers were selected for analysis. Minimally invasive approaches to VSD repair was associated with satisfactory short-term outcomes when compared to open repair. For diagnosis of congenital VSD, whilst recent advances such as pulse oximetry method and genome analysis are more sensitive, the limited availability and access to such investigatory methods must be recognised. CONCLUSION: Pulse oximetry and fetal echocardiography are established non-invasive diagnostic tools for VSD. The recent advances in minimally invasive treatment options including periventricular approach and transcatheter techniques have improved patient outcomes, yet at the expense of higher residual rates. Careful patient selection for each technique and follow-up should be planned through multidisciplinary team meetings.

COVID-19 and congenital heart disease: an insight of pathophysiology and associated risks.

OBJECTIVE: We aimed to examine the literature to determine if both paediatric and adult patients diagnosed with congenital heart disease (CHD) are at a higher risk of poor outcomes if they have the coronavirus disease 2019 (COVID-19), compared to those without CHD. METHODS: A systematic review was executed using the Preferred Reporting Items for Systematic Reviews and Meta-analysis (PRISMA) guidelines. To identify articles related to COVID-19 and CHD, an extensive literature search was performed on EMBASE, Medline, Scopus, and Global Health databases using keywords and MeSH terms. RESULTS: A total of 12 articles met the inclusion criteria and were included for analysis in this systematic review. Two themes were identified for data extraction: evidence supporting higher risks in CHD patients and evidence against higher risks in CHD patients. After combining the data, there were 99 patients with CHDs out of which 12 required admissions to ICU. CONCLUSION: This systematic review suggests that CHD may increase the risk of poor outcomes for those with COVID-19, but also highlights the necessity for more research with larger sample sizes in order to make a more justified conclusion, as the majority of papers that were analysed were case series and case reports. Future research should aim to quantify the risks if possible whilst accounting for various confounding factors such as age and treatment history.

Congenital Cardiac Surgery and Parental Perception of Risk: A Quantitative Analysis.

INTRODUCTION: Interpretation of risk by parents of children undergoing congenital cardiac surgery is poorly documented. The available evidence highlights a dichotomy where clinicians suggest parents may not grasp the complexity and risk associated with procedures, while some parents suggest risk is unnecessarily overemphasized. AIM: To quantify how risk is perceived by parents. METHODS: One hundred six parents of children undergoing cardiac surgery were recruited and completed a Likert-type scale from 1 (perceived low risk) to 6 (perceived high risk), at 5 points: arrival at preadmission, post discussion with anethetist/surgeon, day of surgery, discharge from intensive care, and at outpatient follow-up. The surgical sample was stratified according to Risk Adjustment in Congenital Heart Surgery level. ANALYSIS: Data were analyzed using Wilcoxon rank tests for differences in distributions of scores and Krippendorff α to examine the level of agreement. RESULTS: Median parental risk scores varied over time, with no consistent risk scores observed. Maternal scores were consistently higher than paternal scores at every time point (P < .001). Postoperative complications resulted in a persistent rise in risk perception at follow-up (P < .001). Analysis of parental risk scores and objective measures of surgical risk highlighted poor agreement that was particularly marked at the extremes of risk. CONCLUSIONS: Parents perceived higher risk scores than those reported by the clinical team. Mothers reported statistically significantly higher scores than their partners, highlighting potential tensions. In addition, the changing perception of risk over time emphasizes the need for flexible levels of support and information as parents navigate uncertainty.

Congenital cardiac surgery and parental perception of risk: a qualitative study.

INTRODUCTION: The way risk is interpreted by parents of children undergoing congenital cardiac surgery is poorly documented. Literature suggests clinicians have concerns that parents may not understand the complexity of procedures. Conversely, some parents perceive an unnecessary over-emphasis of risks. AIM: To explore how risk is encountered by parents of children who are undergoing cardiac surgery, in order to deliver effective and compassionate care. METHODS: A qualitative approach was adopted. Interviews were undertaken with 18 parents (mothers n = 10; fathers n = 8). Recordings were transcribed verbatim and analysed using a constant comparative-based approach. FINDINGS: Three themes emerged from the data: the nature of risk, reflecting the complexity of parental perception of risk and the influence of the doctor-parent relationship; presenting risk, highlighting the way in which risk is presented to and interpreted by parents; and risk and responsibility, examining the way in which parents engaged with risk and the impact of this on their relationship. CONCLUSIONS: The way in which risk is perceived by parents is complex and multi-factorial. The doctor-parent relationship is key to parental engagement. However, parents manage risk and uncertainty through a number of mechanisms, including those perceived as being not rational. This can cause tension, particularly when required to engage in informed decision-making.

Outcome of a repair-oriented strategy for the aortic valve in children.

BACKGROUND: We sought to evaluate the mid-term results of a repair-oriented strategy of the aortic valve in children. METHODS: Between February 2007 and November 2011, we performed 39 aortic valve repairs in children. Median age and weight at surgery were 5.5 years (3 days-18 years) and 16.7 kg (2.7-83.5), respectively. A total of 11 (28%) patients had one or more prior surgical or interventional cardiologic procedures. Median intensive care unit and hospital stay were two (1-96) and five (3-96) days, respectively. Median and cumulative follow-up were 12.7 months (15 days-64 months) and 48.9 patient-years, respectively. RESULTS: There were 3 (7.7%) early deaths all in patients undergoing additional complex cardiac procedures. Two (5.1%) patients required early surgical revision. Twenty-six (66%) patients had isolated or mixed aortic stenosis. The median gradient reduced from 62.4 (range 16-144) to 17.6 mm Hg (range 0-51.8), postoperatively (P = .02). At last follow-up, the median gradient remained largely unchanged at 21.1 mm Hg (0-49; P = .02). Twenty-six (66%) patients had isolated or mixed aortic valve regurgitation (mild in 8 [20%], moderate in 15 [38%], and severe in 6 ([15%] patients). The degree of aortic regurgitation improved in all patients with moderate or severe aortic regurgitation (P = .04). At last follow-up, two patients had moderate aortic regurgitation. Kaplan Meier freedom from reintervention in the survivors was 95% at three years. CONCLUSIONS: A repair-oriented strategy for the aortic valve in children has satisfactory early and mid-term results and remains a promising management option in children with aortic valve disease.

Concomitant congenital heart defect repair and Nuss procedure for pectus excavatum.

The combination of complex congenital cardiac anomalies and pectus excavatum represents a technical challenge. Most concomitant repairs have been performed in adult patients. We report the uncomplicated concomitant repair of double-outlet right ventricle with absent pulmonary valve syndrome and the Nuss procedure for pectus excavatum in a 3-year-old child.