anesthesia for the patients with congenital heart disease

Shone's complex is a congenital cardiac disease consisting of the following four lesions: parachute mitral valve, supravalvar mitral ring, subaortic stenosis, and aortic coarctation. Though not all components are required for a diagnosis, the end result is both left ventricular inflow and outflow obstruction, which typically present in patients as congestive heart failure. The complex pathology requires careful management and surgical decision-making to ensure an optimal outcome. This review will focus on the anatomy, physiology, and perioperative anesthetic management of patients with Shone's complex...

INTRODUCTION: Patients with Fontan palliation are susceptible to congestive hepatopathy and Fontan-associated liver disease (FALD) because of hemodynamic changes. The staging of liver fibrosis involves various methods, including invasive biopsy. Transjugular liver biopsy (TJLB) offers a less invasive alternative, enhancing liver disease surveillance in routine cardiac catheterization. We detail the technical aspects, share initial outcomes, and discuss existing literature. METHODS/RESULTS: During routine follow-up cardiac catheterization indicated by hemodynamic or clinical alterations, four patients aged between 16 and 26 years with univentricular Fontan circulation and three patients with biventricular circulation underwent TJLB during routine surveillance catheterization...

BACKGROUND: Tetralogy of Fallot (TOF) is a complex cyanotic congenital heart disease. As most patients with TOF undergo palliative or radical surgical repair during childhood, cardiac surgery under cardiopulmonary bypass (CPB) for adult survivors with unrepaired TOF is exceedingly rare. CASE PRESENTATION: A 41-year-old woman with unrepaired TOF, pulmonary atresia (PA), and major aortopulmonary collateral arteries (MAPCAs) developed acute infectious endocarditis (IE)...

The goal of this article is to provide an up-to-date and comprehensive review of the current perioperative anesthetic management of pediatric patients with congenital heart disease (CHD) undergoing non-cardiac surgery. This report discusses a case of a nine-month-old female with Tetralogy of Fallot who was scheduled for non-cardiac surgery for anorectal malformation stage 1 and stage 2 repair. This case study discusses how to adjust perioperative anesthesia care in cases of left-to-right shunt, right-to-left shunt, and complex cardiac disease...

The number of patients with congenital heart disease (CHD) undergoing ambulatory surgery is increasing. Deciding whether a CHD patient is suitable for an ambulatory procedure is still challenging. Several factors must be considered, including the type of planned procedure, the complexity of the underlying pathology, the American Society of Anesthesiologists' Physical Status classification of the patient, and other patient-specific factors, including comorbidity, chronic complications of CHD, medication, coagulation disorders, and issues related to the presence of a pacemaker (PM) or cardioverter-defibrillator...

INTRODUCTION: Cerebral oxygen desaturation during pediatric surgery has been associated with adverse perioperative outcomes. The aim of this pilot study was to analyze the frequency and severity of intraoperative cerebral oxygen desaturations and their impact on postoperative cerebral oxygen metabolism in neonates and infants undergoing pediatric surgery. METHODS: In a prospective pilot study, intra- and postoperative regional cerebral oxygen saturation and blood flow were measured noninvasively using a device combining laser Doppler flowmetry and white-light-spectrometry...

BACKGROUND: Eisenmenger's syndrome (ES) is a rare complication of congenital heart disease that includes pulmonary artery hypertension and reversed or bidirectional shunts. The mortality rate of pregnant women with ES is 30%-70% due to pathophysiological deterioration. Successful perioperative management of a pregnant patient with ES is a challenge for anesthesiologists. CASE SUMMARY: A 38-year-old pregnant woman was admitted to the cardiology department of our hospital at 22 wk of gestation with complaints of chest tightness and shortness of breath for 3 wk...

BACKGROUND: Four-dimensional flow (4D flow) MRI has become a clinically utilized cardiovascular flow assessment tool. However, scans can be lengthy and may require anesthesia in younger children. Adding compressed sensing can decrease scan time, but its impact on hemodynamic data accuracy needs additional assessment. OBJECTIVE: To compare 4D flow hemodynamics acquired with and without compressed sensing. MATERIALS AND METHODS: Twenty-seven patients (median age: 13 [IQR: 9...

Although complex congenital heart disease (CHD) patients usually present in childhood, it is not rare to see adults well past middle age. These patients undergo continuous pathophysiological changes in their heart and blood vessels, making anesthetic management more challenging if surgery is required. Herein, we report a case in which understanding the anatomy and pathophysiology helped optimally manage a patient with a double outlet right ventricle (DORV) who underwent plating and fixation for a hangman's fracture in the prone position...

Congenitally corrected transposition of the great arteries (CCTGA) is a rare form of congenital heart disease often associated with other cardiac defects. The adaptations and physiologic changes in pregnancy can present maternal challenges and complications; multidisciplinary care allows for the safest management of pregnancy and delivery in these patients. We present a case of the anesthetic management of cesarean delivery in a woman with CCTGA with her pregnancy complicated by recurrent volume overload, pulmonary hypertension, and dysrhythmias...

Background. Pulmonary hypertension (PH) is a common complication of congenital heart disease (CHD). With early surgical intervention, outcomes have improved over the last two decades. Persistent PH, however, may still occur following surgery. Sildenafil has been shown to be beneficial for postoperative pulmonary hypertensive crises. The role of preoperative sildenafil in controlling postoperative PH is poorly elucidated. This study aimed to evaluate the effect of preoperative sildenafil on pediatric patients undergoing congenital heart surgeries...

(1) Transcatheter closure of perimembranous ventricular septal defects (PmVSD) is becoming more attractive and effective with the development of new occluders. The aim of this study was to report a single-center experience in PmVSD closure using the Lifetech Konar-multifunctional occluder (MFO). (2) From March 2019 to October 2022, 43 consecutive patients were enrolled in the study. Among them, 13 had multifenestrated PmVSD including 5 Gerbode-type defects. (3) There were 23 males/20 females, and the median age was 17 years (range 2-68 years)...

OBJECTIVE: Patients undergoing congenital cardiac surgery require induction of anesthesia. Our objective was to identify the median anesthesia ready time and the predictors of this time. METHODS: By using the Society of Thoracic Surgeons Congenital Heart Surgery Database, we identified patients who underwent cardiopulmonary bypass procedures from 2017 to 2021. Univariate and multivariable regression modeling to predict the anesthesia ready time was performed using mixed-effects linear regression...

BACKGROUND: Fontan surgery, a palliative procedure for single ventricle patients, often leads to the need for permanent pacing. Epicardial pacing has limitations, while transvenous pacing carries risks in the Fontan circulation. This case series introduces a novel approach of leadless pacemaker implantation in the extra-cardiac Fontan (ECF) cohort to overcome these limitations. METHODS: The study includes four cases of leadless pacemaker (Micra™) implantation in patients with ECF...

BACKGROUND: Prolonged intensive care unit (ICU) stays consume medical resources and increase medical costs. This study identified risk factors associated with prolonged postoperative intensive care unit (ICU) stay in children with total anomalous pulmonary venous connection (TAPVC). METHODS: The medical records of 85 patients who underwent surgical repair of TAPVC were retrospectively analyzed. The patients were divided into prolonged-stay and standard-stay groups...

Thoracic endovascular aortic repair (TEVAR) is a new alternative surgical treatment for aortic pathologies, which is more minimally invasive. The aim of current study was to summarize the single-center experience of general anesthesia for patients undergoing TEVAR. In adult patients undergoing surgery for congenital heart disease, the strategy of "fast-track" anesthesia with early extubation in theater is associated with a shorter intensive care unit (ICU) stay, and lower health-care-related costs. Fast-track anesthesia has not been assessed in patients under TEVAR...

INTRODUCTION: The transvenous implantable cardioverter defibrillator (ICD) is the treatment of choice for the prevention of sudden cardiac death (SCD). Its use could be restricted when device-related infections occurs or in the pediatric population. In the later, an ICD represents a challenge, due to the minimal dimensions of the venous system in children, the length of the electrodes, the size of the generator, as well as the anatomical complexity in cases with associated congenital heart disease...

BACKGROUND: We evaluated the correlation between regional oxygen saturation (rSO2 ) in the frontal and right renal dorsum (cerebral rSO2 and somatic rSO2 ) measured by near-infrared spectroscopy (INVOS™ 5100C, Medtronic) and central venous oxygen saturation (ScvO2 ) measured with a fiber-optic oximetry catheter (PediaSat™, Edwards Lifesciences) during surgery in order to determine whether noninvasive rSO2 could be used as an alternative to ScvO2 in pediatric cardiac surgery patients...

Single ventricle physiology is a rare form of congenital heart disease and was, historically, a uniformly lethal condition. However, the atriopulmonary Fontan operation, and its successive iterations, the lateral tunnel and extracardiac conduit Fontan, became the fundamental approach to treating single ventricle heart disease. Over time, dysrhythmias are some of the most common complications with Fontan physiology, compounding morbidity and mortality. Atrial arrhythmias are prevalent in the Fontan population and occur in about 15-60% of patients with Fontan palliation, increasingly with age...

Ellis-Van Creveld syndrome (EVC), also known as mesoectodermal dysplasia, is a rare autosomal recessive disorder with a tetrad of clinical features, comprising dwarfism, polydactyly, ectodermal dysplasia with sparse hair, hypoplastic nails and enamel, hypodontia and conical teeth and congenital heart disease (CHD). We report an 18-year-old girl with short stature and polydactyly, who got admitted to our hospital with shortness of breath on exertion for the last 2 years. On echocardiography, a partial atrioventricular canal (AV canal) defect was diagnosed, which was repaired surgically...