Venous Air Embolism Leading to Cardiac Arrest in an Infant with Cyanotic Congenital Heart Disease

Gas emboli, including venous and arterial, are a rare but important complication of pediatric cardiac surgery. They have the potential to have devastating consequences and require prompt recognition and treatment. We present a case of gas embolism occurring in the immediate postoperative period in an infant with cyanotic congenital heart disease after palliative cardiac surgery resulting in cardiopulmonary arrest. The embolism was diagnosed by visualization of air within the vessel creating an airlock and occluding pulmonary blood flow

B-type Natriuretic Peptide: Perioperative Patterns in Congenital Heart Disease

B-type natriuretic peptide (BNP) has diagnostic, prognostic, and therapeutic roles in adults with heart failure. BNP levels in children undergoing surgical repair of congenital heart disease (CHD) were characterized broadly, and distinguishable subgroup patterns delineated.Prospective, blinded, observational case series.Academic, tertiary care, free-standing pediatric hospital.Children with CHD; controls without cardiopulmonary disease.None.Preoperative cardiac medications/doses, CHD lesion types, perioperative BNP levels, intraoperative variables (lengths of surgery, bypass, cross-clamp), postoperative outcomes (lengths of ventilation, hospitalization, open chest; averages of inotropic support, central venous pressure, perfusion, urine output; death, low cardiac output syndrome (LCOS), cardiac arrest; readmission; and discharge medications).Median BNP levels for 102 neonatal and non-neonatal controls were 27 and 7 pg/mL, respectively. Serial BNP measures from 105 patients undergoing CHD repair demonstrated a median postoperative peak at 12 hours. The median and interquartile postoperative 24-hour average BNP levels for neonates were 1506 (782–3784) pg/mL vs. 286 (169–578) pg/mL for non-neonates ( P < 0.001). Postoperative BNP correlated with inotropic requirement, durations of open chest, ventilation, intensive care unit stay, and hospitalization (r = 0.33–0.65, all P < 0.001). Compared with biventricular CHD, Fontan palliations demonstrated lower postoperative BNP (median 150 vs. 306 pg/mL, P < 0.001), a 3-fold higher incidence of LCOS ( P < 0.01), and longer length of hospitalization (median 6.0 vs. 4.5 days, P = 0.01).Perioperative BNP correlates to severity of illness and lengths of therapy in the CHD population, overall. Substantial variation in BNP across time as well as within and between CHD lesions limits its practical utility as an isolated point-of-care measure. BNP commonly peaks 6–12 hours postoperatively, but the timing and magnitude of BNP elevation demonstrates notable age-dependency, peaking earlier and rising an order of magnitude higher in neonates. In spite of higher clinical acuity, non-neonatal univentricular CHD paradoxically demonstrates lower BNP levels compared with biventricular physiologies.Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/79231/1/j.1747-0803.2010.00396.x.pd

Hypoplastic Left Heart Syndrome Sequencing Reveals a Novel NOTCH1 Mutation in a Family with Single Ventricle Defects

Hypoplastic left heart syndrome (HLHS) has been associated with germline mutations in 12 candidate genes and a recurrent somatic mutation in HAND1 gene. Using targeted and whole exome sequencing (WES) of heart tissue samples from HLHS patients, we sought to estimate the prevalence of somatic and germline mutations associated with HLHS. We performed Sanger sequencing of the HAND1 gene on 14 ventricular (9 LV and 5 RV) samples obtained from HLHS patients, and WES of 4 LV, 2 aortic, and 4 matched PBMC samples, analyzing for sequence discrepancy. We also screened for mutations in the 12 candidate genes implicated in HLHS. We found no somatic mutations in our HLHS cohort. However, we detected a novel germline frameshift/stop-gain mutation in NOTCH1 in a HLHS patient with a family history of both HLHS and hypoplastic right heart syndrome (HRHS). Our study, involving one of the first familial cases of single ventricle defects linked to a specific mutation, strengthens the association of NOTCH1 mutations with HLHS and suggests that the two morphologically distinct single ventricle conditions, HLHS and HRHS, may share a common molecular and cellular etiology. Finally, somatic mutations in the LV are an unlikely contributor to HLHS

Retrocardiac mediastinal foregut duplication cyst

AbstractForegut duplications occur at various locations throughout the upper gastrointestinal tract, including the thoracic cavity. Thoracic foregut duplications are typically intimately associated with the esophagus and therefore are located in either the posterior mediastinum or in pleural cavity. Here we report a case of foregut duplication of the middle mediastinum, intimately associated with the pericardium and great vessels that contained gastric mucosa, ciliated respiratory epithelium, bronchial-type epithelium, pancreatic tissue and hepatocytes. The literature of mediastinal foregut duplications is also reviewed

Severe aortopulmonary collaterals are associated with lower transplant-free survival in patients undergoing staged single ventricle palliationCentral MessagePerspective

Objective: To identify risk factors for aortopulmonary collateral (APC) development and assess the impact of severe APCs in children undergoing staged single ventricle palliation. Methods: Children undergoing a bidirectional Glenn operation between January 1, 2016, and March 31, 2021, at our center were included. All underwent angiography prior to Glenn and Fontan; APC flow was graded on a scale of 0 (no appreciable collateral flow) to 4 (severe burden). Demographic data, congenital diagnosis, clinical history, and outcomes were stratified by Glenn assessment; Fontan outcomes were stratified by pre-Fontan grade. Results: Sixty patients met the inclusion criteria, all of whom had angiographic evidence of APCs. There were 7 transplants and 9 deaths in the cohort. There were no significant differences in demographics among the patients. Right ventricular morphology was more common in patients with severe pre-Glenn collaterals (24 of 44 vs 2 of 6 vs 7 of 8; P = .014). Longer stage 1 aortic cross-clamp duration was associated with greater severity pre-Glenn (44 minutes vs 34 minutes vs 66 minutes; P = .023). Patients with grade 3 pre-Glenn collaterals more commonly required transplantation than those with grade 1 collaterals (P < .001) and had lower overall transplant-free survival than those with grade 1 (P = .005) or grade 2 (P = .04) collaterals. Conclusions: The ubiquity of APCs in this study demonstrates their prevalence in single ventricle disease. Right ventricular morphology and prolonged aortic cross-clamp duration are associated with higher burden. Greater severity was associated with decreased transplant-free survival. These data emphasize the negative long-term impact of these collaterals

Case Report Venous Air Embolism Leading to Cardiac Arrest in an Infant with Cyanotic Congenital Heart Disease

Gas emboli, including venous and arterial, are a rare but important complication of pediatric cardiac surgery. They have the potential to have devastating consequences and require prompt recognition and treatment. We present a case of gas embolism occurring in the immediate postoperative period in an infant with cyanotic congenital heart disease after palliative cardiac surgery resulting in cardiopulmonary arrest. The embolism was diagnosed by visualization of air within the vessel creating an airlock and occluding pulmonary blood flow