The Role of Novel Transcatheter Procedures in Patients With Congenital Heart Disease.

The development of percutaneous structural interventions in patients with acquired heart disease is happening at an exponential rate, and some of this technology is being used to treat patients with congenital heart disease. This review describes the pathophysiology of valvular abnormalities specific to congenital heart disease and discusses the application of structural procedures in this population. Although the overall experience has been encouraging, especially in high-risk patients, this article will highlight the reasons that a cautious approach to adoption of this technology is necessary in these patients.

Associations between anthropometric indices and outcomes of congenital heart operations in infants and young children: An analysis of data from the Society of Thoracic Surgeons Database.

BACKGROUND: Children with congenital heart disease are at risk for growth failure due to inadequate nutrient intake and increased metabolic demands. We examined the relationship between anthropometric indices of nutrition (height-for-age z-score [HAZ], weight-for-age z-score [WAZ], weight-for-height z-score [WHZ]) and outcomes in a large sample of children undergoing surgery for congenital heart disease. METHODS: Patients in the Society of Thoracic Surgeons Congenital Heart Surgery Database having index cardiac surgery at age 1 month to 10 years were included. Indices were calculated by comparing patients' weight and height to population norms from the World Health Organization and Centers for Disease Control and Prevention. Outcomes included operative mortality, composite mortality or major complication, major postoperative infection, and postoperative length of stay. For each outcome and index, the adjusted odds ratio (aOR) (for mortality, composite outcome, and infection) and adjusted relative change in median (for postoperative length of stay) for a 1-unit decrease in index were estimated using mixed-effects logistic and log-linear regression models. RESULTS: Every unit decrease in HAZ was associated with 1.40 aOR of mortality (95% CI 1.32-1.48), and every unit decrease in WAZ was associated with 1.33 aOR for mortality (95% CI 1.25-1.41). The relationship between WHZ and outcome was nonlinear, with aOR of mortality of 0.84 (95% CI 0.76-0.93) for 1-unit decrease when WHZ ≥ 0 and a nonsignificant association for WHZ < 0. Trends for other outcomes were similar. Overall, the incidence of low nutritional indices was similar for 1-ventricle and 2-ventricle patients. Children between the age of 1 month and 1 year and those with lesions associated with pulmonary overcirculation had the highest incidence of low nutritional indices. CONCLUSIONS: Lower HAZ and WAZ, suggestive of malnutrition, are associated with increased mortality and other adverse outcomes after cardiac surgery in infants and young children. Higher WHZ over zero, suggestive of obesity, is also associated with adverse outcomes.

Current perspectives on treatment of perioperative hemodynamic instability and hypotension.

Overall, there are numerous causes of hypotension in the perioperative period. The approach to definitive treatment must be tailored to the child's unique anatomy and physiology, as well as the current factors presumed to be eliciting the hypotensive state. It is imperative to consider both routine and lesion-specific etiologies to the current hypotensive episode. Lastly, when employing pharmacologic therapy for hypotension, there are often multiple combinations of medications that can reasonably be used to achieve the desired hemodynamic effects.

Understanding cardiac shunts.

Most patients with congenital heart disease have a cardiac shunt whose direction and magnitude can have a major impact on cardiorespiratory physiology and function. The dynamics of the shunt can be significantly altered by anesthetic management and must be understood in order to provide optimal anesthetic care. Given that there are now more adults than children with congenital heart disease and that the majority of nonpediatric patients are cared for in centers without special expertise in congenital heart disease, it is imperative that all anesthesia providers have a general understanding of the subject. This educational review describes a technique to explain this complex subject using simple pictorial diagrams.

Preoperative malnutrition is associated with increased mortality and adverse outcomes after paediatric cardiac surgery.

BACKGROUND: Malnutrition is common in children with CHD and is likely to place them at an increased risk for adverse surgical outcomes. We sought to evaluate the impact of preoperative malnutrition on outcomes after paediatric cardiac surgery. METHODS: We conducted a retrospective analysis of patients from age 0 to 5 years undergoing cardiac surgery at Seattle Children's Hospital from 2006 to 2015. We used regression modelling to examine the impact of malnutrition on surgical outcomes. RESULTS: We found a non-linear relationship between low height-for-age and weight-for-age z-scores and mortality after surgery. In the range of z-score ⩽-2, each additional unit decrease in height-for-age or weight-for-age z-score was associated with a 2.9 or 2.1% increased risk for mortality, respectively. Each unit decrease in height-for-age z-score was associated with a 1.2% increased risk for cardiac arrest, 1.1% increased risk for infection, and an average of 1.7 additional hours of mechanical ventilation, 6 hours longer ICU stay, and 13 hours longer hospital stay. Each unit decrease in weight-for-age z-score was associated with a 0.7% increased risk for cardiac arrest, 0.8% increased risk for infection, and an average of 1.9 additional hours of mechanical ventilation and 5.3 additional hours of ICU stay. CONCLUSIONS: This study is unique in demonstrating a significant association between malnutrition and 30-day mortality and other adverse outcomes after paediatric cardiac surgery in a mixed population of CHD patients. By evaluating nutritional status as a continuous variable, we were able to clearly distinguish the point at which malnutrition begins to affect mortality.

Perioperative morbidity in children with elastin arteriopathy.

BACKGROUND: Children with elastin arteriopathy (EA), the majority of whom have Williams-Beuren syndrome, are at high risk for sudden death. Case reports suggest that the risk of perioperative cardiac arrest and death is high, but none have reported the frequency or risk factors for morbidity and mortality in an entire cohort of children with EA undergoing anesthesia. AIM: The aim of this study was to present one institution's rate of morbidity and mortality in all children with EA undergoing anesthesia and to examine patient characteristics that pose the greatest risk. METHODS: We reviewed medical records of children with EA who underwent anesthesia or sedation for any procedure at our institution from 1990 to 2013. Cardiovascular hemodynamic indices from recent cardiac catheterization or echocardiography were tabulated for each child. The incidence, type, and associated factors of complications occurring intraoperatively through 48 h postoperatively were examined. RESULTS: Forty-eight patients with confirmed EA underwent a total of 141 anesthetics. There were seven cardiac arrests (15% of patients, 5% of anesthetics) and nine additional intraoperative cardiovascular complications (15% of patients, 6% of anesthetics). Extracorporeal life support was initiated in five cases. There were no perioperative deaths. All children having a cardiac arrest or complication were <3 years old and had biventricular outflow tract obstruction (BVOTO). Subgroup analysis demonstrated high rates of cardiac arrest in two groups: children with BVOTO (44%) and age <3 years old (21%). CONCLUSIONS: We have confirmed that the rate of cardiac arrest and complications is significantly elevated in children with EA undergoing anesthesia. Children <3 years old and with BVOTO were at the greatest risk in our population.

Perioperative and Anesthetic Considerations in Vascular Rings and Slings.

Vascular rings represent an increasingly prevalent and diverse set of congenital malformations in which the aortic arch and its primary branches encircle and constrict the esophagus and trachea. Perioperative management varies significantly based on the type of lesion, its associated comorbidities, and the compromise of adjacent structures. Multiple review articles have been published describing the scope of vascular rings and relevant concerns from a surgical perspective. This review seeks to discuss the perioperative implications and recommendations of such pathology from the perspective of an anesthesia provider.

Novel Transcatheter Approach to Treat Primum Atrial Septal Defects.

•Degenerative common AVC defect can mimic rheumatic MV stenosis.•Closure of primum ASD can be achieved percutaneously.•Live 3D multiplanar TEE is crucial for procedural guidance.

Encouraging results of blood conservation in neonatal open-heart surgery.

OBJECTIVE: To report early outcomes of blood conservation in neonatal open-heart surgery. METHODS: Ninety-nine patients undergoing neonatal open-heart surgery during the implementation of a blood conservation program between May 2021 and February 2023 were reviewed. Patients either received traditional blood management (blood prime, n = 43) or received blood conservation strategies (clear prime, n = 56). Baseline characteristics and outcomes were compared between groups. RESULTS: There was no difference in body weight (median, 3.2 kg vs 3.3 kg; P = .83), age at surgery (median, 5 days vs 5 days; P = .37), distribution of The Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery Congenital Heart Surgery Mortality Categories categories or duration of cardiopulmonary bypass. Patients in the clear prime group had higher preoperative hematocrit (median, 41% vs 38%; P < .01), shorter postoperative mechanical ventilation time (median, 48 hours vs 92 hours; P = .02) and postoperative intensive care unit length of stay (median, 6 days vs 9 days; P < .01) than patients in the blood prime group. Fourteen patients (25%) in the clear prime group, including 1 Norwood patient, were discharged without any transfusion. Among patients within the clear prime group, hospitalizations without blood exposure were associated with higher preoperative hematocrit (median, 43% vs 40%; P = .02), shorter postoperative mechanical ventilation times (median, 22 hours vs 66 hours; P = .01) and shorter postoperative hospital stays (median, 10 days vs 15 days; P = .02). CONCLUSIONS: Bloodless surgery is possible in a significant proportion of neonates undergoing open-heart surgery, including the Norwood operation, even in the early stages of experience. Early clinical results are favorable but long-term follow-up and continued efforts are warranted to prove safety and reproducibility.

High-frequency micro-ultrasound for vascular access in young children--a feasibility study by the High-frequency UltraSound in Kids studY (HUSKY) group.

BACKGROUND: Cannulation of small arteries and veins in young children can be challenging. Although anesthesiologists frequently use ultrasound for placement of central venous lines and nerve blocks, its use for cannulation of small, peripheral vessels is less helpful. Ultrasound systems (7-15 MHz) currently used in clinical practice focus poorly at the sub-10-mm space and thus lack the resolution to allow accurate ultrasound-guided cannulation of small vessels. High-frequency micro-ultrasound (HFMU) is a new technology that allows higher resolution (15-50 MHz) compared with conventional ultrasound. Limited human studies have been performed thus far with HFMU, and none have been performed in young children or for vascular access. METHODS: This study was conducted to determine the feasibility of using HFMU to visualize and cannulate peripheral arteries and central veins in children under the age of 6 years old. The diameter of radial and ulnar arteries was also measured. RESULTS: The anesthesiologists involved in this study found the 50 MHz HFMU probe useful for cannulation of peripheral arteries, especially in the youngest children. The higher-frequency probes were less helpful for internal jugular vein cannulation because it was not always possible to view the carotid artery while cannulating the vein. CONCLUSIONS: The experience gained in this feasibility study suggests that HFMU could be a valuable addition to our armamentarium for difficult vascular access in the future.

Perioperative and Anesthetic Considerations in Pediatric Valvar and Subvalvar Aortic Stenosis.

Aortic stenosis (AS) is a common form of left ventricular outflow tract obstruction (LVOTO) in children with congenital heart disease. This review specifically considers the perioperative features of valvar (VAS) and subvalvar AS (subAS) in the pediatric patient. Although VAS and subAS share some clinical features and diagnostic approaches, they are distinct clinical entities with separate therapeutic options, which range from transcatheter intervention to surgical repair. We detail the pathophysiology of AS and highlight the range of treatment strategies with a focus on anesthetic considerations for the care of these patients before, during, and after intervention.

The Year in Review: Anesthesia for Congenital Heart Disease 2021.

This review focuses on the literature published during the calendar year 2021 that is of interest to anesthesiologists taking care of children and adults with congenital heart disease. Four major themes are discussed, including cardiovascular disease in children with COVID-19, aortic valve repair and replacement, bleeding and coagulation, and enhanced recovery after surgery (ERAS).

Perioperative and Anesthetic Considerations in Tetralogy of Fallot With Pulmonary Atresia.

Tetralogy of Fallot with pulmonary atresia (ToF-PA) is a rare diagnosis that includes an extraordinarily heterogeneous group of complex anatomical findings with significant implications for physiology and prognosis. In addition to the classic findings of ToF, this particular diagnosis is characterized by complete failure of forward flow from the right ventricle to the pulmonary arterial system. As such, pulmonary blood flow is entirely dependent on shunting from the systemic circulation, most frequently via a patent ductus arteriosus, major aortopulmonary collaterals, or a combination of the two. The pathophysiology of ToF-PA is largely attributable to the abnormalities of the pulmonary vasculature. Ultimately, these patients require operative intervention to create a reliable, controlled source of pulmonary blood flow and ideally complete intracardiac repair. Even after operative correction, these patients remain at risk for pulmonary arterial stenoses and pulmonary hypertension. Although there have been significant advances in surgical and interventional management of ToF-PA leading to dramatic improvements in survival and long-term functional status, there is ongoing debate about the optimal management strategy given the risk of development of irreversible abnormalities of the pulmonary vasculature and the morbidity and mortality associated with sometimes multiple, complex operative interventions often occurring early in infancy. This review will discuss the findings in patients with ToF-PA with a focus on the perioperative and anesthetic management and will highlight challenges faced by the anesthesiologist in caring for these patients.

The Year in Review: Anesthesia for Congenital Heart Disease 2020.

This review focuses on the literature published during the calendar year 2020 that is of interest to anesthesiologists taking care of children and adults with congenital heart disease. Five major themes are discussed, including COVID-19 in children with heart disease, race and outcome disparities in congenital heart disease, Norwood procedure and outcomes, Fontan procedure and outcomes, and neurotoxicity/neurologic outcomes. A total of 59 peer-reviewed articles are discussed.

Perioperative and Anesthetic Management of Coarctation of the Aorta.

Isolated coarctation of the aorta is a relatively common form of congenital heart disease that is characterized by variable degrees of obstruction to aortic outflow. The clinical presentation varies from asymptomatic arterial hypertension to cardiogenic shock. The treatment options include surgical repair or interventional therapy with aortic balloon dilation and stent placement. This article will summarize the pathophysiology as well as describe the surgical and interventional procedures. The anesthetic management for those interventions will be reviewed.

Perioperative and Anesthetic Considerations in Truncus Arteriosus.

Truncus arteriosus is a congenital cardiac lesion in which failure of embryonic truncal septation results in a single semilunar valve and single arterial trunk providing both pulmonary and systemic circulations. Most patients with this lesion are symptomatic in the neonatal period with cyanosis and/or congestive heart failure and undergo complete repair in the first weeks of life. This review will focus on the anatomy, physiology, and perioperative anesthetic management of patients with truncus arteriosus.

Perioperative and Anesthetic Considerations in Pulmonary Atresia With Intact Ventricular Septum.

Pulmonary atresia with intact ventricular septum (PA/IVS) is a rare right-heart obstructive lesion with a wide anatomic and physiologic spectrum of disease, ranging from simple membranous pulmonary valve atresia with a fully developed right ventricle (RV) to a severely hypoplastic RV and ventriculocoronary (RV-coronary) fistulas. Affected neonates are dependent on prostaglandin for adequate pulmonary blood flow. Depending on the severity of disease, treatment options range from transcatheter pulmonary valve perforation and ultimate biventricular repair to staged single-ventricle palliation. Cardiac transplantation is recommended in the most severe cases. This review will discuss the perioperative and anesthetic management of patients with PA/IVS and highlight the challenges in management.