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.

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).

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.

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.

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.

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.

Not for adults only: MitraClip use in a paediatric patient.

We present the primary report of a paediatric patient who had placement of the MitraClip device for severe functional mitral regurgitation. The patient was a 14-year-old boy with symptomatic end-stage non-compaction type cardiomyopathy secondary to a mitochondrial cytopathy. He had severe mitral regurgitation, tricuspid valve regurgitation, a severely dilated LV with apical non-compaction, severe LV dysfunction and severe pulmonary hypertension. Despite optimal medical therapy he developed progressive symptoms of congestive heart failure and he was not a candidate for an assist device or cardiac transplantation. Multimodality imaging with fluoroscopy, two-dimensional and three-dimensional transoesophageal echocardiography was used to guide the procedure. Two MitraClips were placed resulting in an adequate decrease in MR severity to no more than mild-moderate. Five months post MitraClip implantation, he has improved exercise tolerance. Transthoracic echocardiography showed mild to moderate mitral regurgitation and a decrease in left ventricular size. To our knowledge, this is the first reported paediatric patient to be offered this innovative procedure. Percutaneous edge-to-edge MV repair may prove to be a novel and effective palliation to consider in a subgroup of paediatric, adolescent and young adult patients. This case report describes some of the considerations specific to the paediatric patient.

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.

Anesthesia for Potts Shunt in a Child with Severe Refractory Idiopathic Pulmonary Arterial Hypertension.

Childhood idiopathic pulmonary arterial hypertension is a progressive and fatal disease. When pulmonary artery pressures become suprasystemic and refractory to medical management, atrial septostomy can be recommended for bridging patients to lung transplantation. Recently, a surgical Potts shunt has been recommended as an alternative rescue therapy, and initial outcome data are promising. The placement of a Potts shunt converts the child to Eisenmenger physiology, which is anticipated to provide an improved quality and duration of life. We present the first description of anesthetic management of a child undergoing surgical Potts shunt for pulmonary arterial hypertension and summarize the multiple, unique intraoperative considerations.

Anesthetic Considerations and Management of Transposition of the Great Arteries.

Transposition of the great arteries was once an almost uniformly fatal disease in infancy. Six decades of advances in surgical techniques, intraoperative care, and perioperative management have led to at least 90% of patients reaching adulthood, most with a good quality of life. This review summarizes medical and surgical decision making during the neonatal perioperative period, with a special emphasis on factors pertinent to the anesthetic evaluation and care during primary surgical repair of transposition of the great arteries. A review is also provided of anesthetic considerations for noncardiac surgery later in childhood or adulthood, for those survivors of the arterial switch operation, Rastelli procedure, Nikaidoh procedure, and the réparation á l'étage ventriculaire procedure.

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.