Utilisation of a post-Fontan management pathway reduces chest tube drainage and hospital readmission rates.

Background: Complications following the Fontan procedure include prolonged pleural drainage and readmission for effusions. To address these complications, a post-Fontan management pathway was implemented with primary goals of reducing chest tube duration/reinsertion rates and decreasing hospital length of stay and readmissions.

Methods: Fontan patients were identified by retrospective chart review (2017-2019) to obtain baseline data for chest tube duration/reinsertion rates, hospital length of stay, and readmission rates for effusion.

Management of Ebstein Anomaly in the Current Era: The Story of One Fetus and the Collaboration of Many-A Case Report.

Collaborative multicenter research has significantly increased our understanding of fetal Ebstein anomaly, delineating risk factors for adverse outcomes as well as predictors of postnatal management. These data are incorporated into prenatal care and therapeutic strategies and inform family counseling and delivery planning to optimize care. This report details the translation of findings from multicenter studies into multidisciplinary prenatal care for a fetus with Ebstein anomaly, supraventricular tachycardia, and a circular shunt, including transplacental therapy to control arrhythmias and achieve ductal constriction, informed and coordinated delivery room management, and planned univentricular surgical palliation.

Evaluating threshold for donor fraction cell-free DNA using clinically available assay for rejection in pediatric and adult heart transplantation.

Background: The aims of the study were to assess the performance of a clinically available cell-free DNA (cfDNA) assay in a large cohort of pediatric and adult heart transplant recipients and to evaluate performance at specific cut points in detection of rejection.

Methods: Observational, non-interventional, prospective study enrolled pediatric and adult heart transplant recipients from seven centers. Biopsy-associated plasma samples were used for cfDNA measurements.

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

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).

Validation of donor fraction cell-free DNA with biopsy-proven cardiac allograft rejection in children and adults.

Objectives: Donor-specific cell-free DNA shows promise as a noninvasive marker for allograft rejection, but as yet has not been validated in both adult and pediatric recipients. The study objective was to validate donor fraction cell-free DNA as a noninvasive test to assess for risk of acute cellular rejection and antibody-mediated rejection after heart transplantation in pediatric and adult recipients.

Methods: Pediatric and adult heart transplant recipients were enrolled from 7 participating sites and followed for 12 months or more with plasma samples collected immediately before all endomyocardial biopsies.

Congenitally Corrected Transposition Cardiac Surgery: Society of Thoracic Surgeons Database Analysis.

Background: Congenitally corrected transposition of the great arteries (ccTGA) has many management strategies, with the emergence of anatomic repair increasing the available surgical options. Contemporary surgical practices have not been described in multicenter analyses. This study describes the distribution of heart surgery in patients with ccTGA and defines contemporary outcomes in a large multicenter cohort.

Relationship between donor fraction cell-free DNA and clinical rejection in heart transplantation.

Background: Clinical rejection (CR) defined as decision to treat clinically suspected rejection with change in immunotherapy based on clinical presentation with or without diagnostic biopsy findings is an important part of care in heart transplantation. We sought to assess the utility of donor fraction cell-free DNA (DF cfDNA) in CR and the utility of serial DF cfDNA in CR patients in predicting outcomes of clinical interest.

Methods: Patients with heart transplantation were enrolled in two sequential, multi-center, prospective observational studies.

Misclassification of Pulmonary Hypertension in Partial Anomalous Pulmonary Venous Return.

Partial anomalous pulmonary venous return is a rare congenital heart defect that can escape detection until adulthood and can be misdiagnosed as pulmonary hypertension and managed with vasodilators before the anomaly is identified. This report describes the cases of 3 patients with pulmonary hypertension whose hemodynamics and symptoms improved after repair of anomalous veins. Anomalous pulmonary veins are difficult to identify on a transthoracic echocardiogram, so a high index of suspicion and early use of additional imaging modalities are important to avoid a delayed diagnosis and progression to irreversible disease.

Impact of obesity on post-operative arrhythmias after congenital heart surgery in children and young adults.

Background: Obesity increases the risk of post-operative arrhythmias in adults undergoing cardiac surgery, but little is known regarding the impact of obesity on post-operative arrhythmias after CHD surgery.

Methods: Patients undergoing CHD surgery from 2007 to 2019 were prospectively enrolled in the parent study. Telemetry was assessed daily, with documentation of all arrhythmias.

Increase in nuclear cell-free DNA is associated with major adverse events in adult and pediatric heart transplant recipients.

Background: Cell-free DNA is an emerging biomarker. While donor fraction may detect graft events in heart transplant recipients, the prognostic value of total nuclear cell-free DNA (ncfDNA) itself is largely unexplored.

Objective: Explore the relationship between ncfDNA and clinical events in heart transplant recipients.

Autograft Root Dilation After the Ross Procedure Is Not Benign.

Autograft root dilation is common after the unsupported Ross procedure. In the absence of valvar incompetence, and with the perception that dissection is exceedingly rare, expectant management of autograft aneurysm is common practice. Autograft dissection may not be as rare as thought though, as at this point 7 case reports have accrued that describe autograft dissection requiring urgent operative intervention.

No survival benefit associated with waiting for non-lung donor heart transplants for adult recipients with congenital heart disease.

Background: Adults with congenital heart disease (CHD) awaiting heart transplant (HT) have higher mortality and waitlist removal due to clinical deterioration than those without CHD. The selective use of non-lung donors (NLD) to recover donor pulmonary vasculature to assist in graft implantation may be a contributing factor and is supported by consensus statements despite the recent use of pericardium or graft material as an alternative in pulmonary vascular reconstruction. The impact of selecting NLD for CHD recipients on wait time and mortality has not been evaluated.

Spatial N-glycomics of the human aortic valve in development and pediatric endstage congenital aortic valve stenosis.

Congenital aortic valve stenosis (AS) progresses as an obstructive narrowing of the aortic orifice due to deregulated extracellular matrix (ECM) production by aortic valve (AV) leaflets and leads to heart failure with no effective therapies. Changes in glycoprotein and proteoglycan distribution are a hallmark of AS, yet valvular carbohydrate content remains virtually uncharacterized at the molecular level. While almost all glycoproteins clinically linked to stenotic valvular modeling contain multiple sites for N-glycosylation, there are very few reports aimed at understanding how N-glycosylation contributes to the valve structure in disease.

Vaping Contributing to Postoperative Acute Respiratory Distress Syndrome.

We report the case of an 18-year-old male patient with a history of bicuspid aortic valve with severe aortic insufficiency who had undergone a Ross procedure 1 year prior but subsequently developed stenosis of the pulmonary homograft necessitating conduit replacement. His postoperative course was complicated by acute respiratory distress syndrome. Bronchoscopy revealed significant mucus plugging without identification of contributing pathogen.

Association of chest tube position with phrenic nerve palsy after neonatal and infant cardiac surgery.

Background: Diaphragm paralysis (DP) complicates the postoperative course of neonates and infants undergoing cardiac surgery. Events causing DP remain poorly understood, and preventive strategies remain elusive. This retrospective cohort analysis aims to test the hypothesis that chest tubes in contact with the phrenic nerve in the pleural apex may cause pressure palsy.