Use of low titer O whole blood in infants and young children undergoing cardiac surgery with cardiopulmonary bypass.

Background: Low titer group O whole blood (LTOWB) is commonly used for severe bleeding in trauma patients. LTOWB may also benefit young children requiring cardiac surgery with cardiopulmonary bypass (CPB) at risk of severe bleeding.

Study Design And Methods: In this retrospective study, children <2 years old who underwent cardiac surgery with CPB were included.

Eisenmenger Syndrome: JACC State-of-the-Art Review.

Although major breakthroughs in the field of pediatric cardiology, cardiac surgery, intervention, and overall care improved the outlook of congenital heart disease, Eisenmenger syndrome (ES) is still encountered and remains a complex clinical entity with multisystem involvement, including secondary erythrocytosis, increased thrombotic and bleeding diathesis, high arrhythmogenic risk, progressive heart failure, and premature death. Clearly, care for ES is best delivered in multidisciplinary expert centers. In this review, we discuss the considerable recent progress in understanding the complex pathophysiology of ES, means of prognostication, and improvement in clinical outcomes achieved with pulmonary arterial hypertension-targeted therapies.

Heart failure in adults with congenital heart disease.

Heart failure (HF) represents the leading cause of morbidity and mortality in adult patients with congenital heart disease. The nature of underlying congenital heart disease has bearing on timing and severity of HF and impacts on short- and long-term outcomes. HF can be subclinical, underscoring the need for close follow-up at tertiary centres with timely management of target hemodynamic lesions.

Consensus Statement: Hemostasis Trial Outcomes in Cardiac Surgery and Mechanical Support.

Background: Research evaluating hemostatic agents for the treatment of clinically significant bleeding has been hampered by inconsistency and lack of standardized primary clinical trial outcomes. Clinical trials of hemostatic agents in both cardiac surgery and mechanical circulatory support, such as extracorporeal membrane oxygenation and ventricular assist devices, are examples of studies that lack implementation of universally accepted outcomes.

Methods: A subgroup of experts convened by the National Heart, Lung, and Blood Institute and the US Department of Defense developed consensus recommendations for primary outcomes in cardiac surgery and mechanical circulatory support.

Atrial septal defect in adulthood: a new paradigm for congenital heart disease.

Atrial septal defects (ASDs) represent the most common congenital heart defect diagnosed in adulthood. Although considered a simple defect, challenges in optimal diagnostic and treatment options still exist due to great heterogeneity in terms of anatomy and time-related complications primarily arrhythmias, thromboembolism, right heart failure and, in a subset of patients, pulmonary arterial hypertension (PAH). Atrial septal defects call for tertiary expertise where all options may be considered, namely catheter vs.

1p36 Deletion Syndrome and Left Ventricular Non-compaction Cardiomyopathy-Two Cases Report.

1p36 deletion is the most common terminal deletion syndrome in humans. Herein, we report two cases, a 5-month-old female and a 14.5-year-old female, both with 1p36 deletion and left ventricular non-compaction cardiomyopathy.

Berlin Heart EXCOR and ACTION post-approval surveillance study report.

Background: The Berlin Heart EXCOR Pediatric (EXCOR) ventricular assist device (VAD) was introduced in North America nearly 2 decades ago. The EXCOR was approved under Humanitarian Device Exemption status in 2011 and received post-market approval (PMA) in 2017 from Food and Drug Administration. Since the initial approval, the field of pediatric mechanical circulatory support has changed, specifically with regard to available devices, anticoagulation strategies, and the types of patients supported.

Pulmonary valve replacement via left anterior minithoracotomy: Lessons learned and early experience.

Objective: Median sternotomy has been the standard for pulmonary valve replacement (PVR) in patients with free pulmonary regurgitation (PR) and right ventricular enlargement. With the introduction of transcatheter therapy, the search for an alternate to sternotomy is mandated. We present our early experience with a limited anterior left thoracotomy approach.

Berlin Excor Cannulation of Left Atrial Appendage in Left Ventricular Restrictive Physiology: A Novel Bailout Strategy.

Ventricular assist device (VAD) management continues to be a challenge in the presence of restrictive physiology. Left atrial (LA) decompression is not satisfactory even with good function and position of the left ventricular cannula. We describe an alternate approach with LA cannulation via the left atrial appendage (LAA) as a rescue strategy in a patient who had restrictive physiology, in our case was secondary to viral myocarditis acute systolic heart failure with subsequent insidious diffuse endomyocardial fibrosis and superimposed massive calcification, causing inadequate emptying of the left ventricle despite optimal VAD apical cannula position.

Angiographic evidence of backward compression wave: systolic compression of septal perforators in a child with hypertrophic cardiomyopathy.

Intracoronary wave intensity analysis in hypertrophic cardiomyopathy has shown a large backward compression wave due to compressive deformation of the intramyocardial coronary arteries in systole. The authors describe the angiographic evidence of this backward compression wave, which has not been described in this physiological context and can be a marker of poor prognosis.

Orthotopic Heart Transplantation in a Child with Single Ventricle after Pneumonectomy.

We report a 6-year-old with single ventricle physiology secondary to tricuspid atresia who had cardiorespiratory failure who was not a candidate for further single ventricle palliation. The patient underwent planned staged left pneumonectomy for recurrent pneumonias secondary to bronchomalacia followed by orthotopic heart transplantation. This aggressive approach improved the patient candidacy for heart transplantation by removing the source of recurrent infection and respiratory failure (left lung).

Outcomes of Surgical Repair of Vascular Rings and Slings in Children: A Word for the Asymptomatic.

Vascular rings (VRs) are rare aortic arch anomalies that may present with a wide variety of symptoms related to esophageal and/or airway compression. We reviewed our surgical experience in both symptomatic and asymptomatic children. All children (n = 58) who underwent surgical repair of VRs or slings (mean age 27.

Off-Pump "Clamp and Sew" Extracardiac Fontan With Inverted Bifurcated Graft for Dextrocardia With Heterotaxy Syndrome.

Avoiding cardiopulmonary bypass during palliation of single ventricle has the advantages of minimizing transfusions, pulmonary vascular resistance, and avoiding the inflammatory response from cardiopulmonary bypass. It is however not always straightforward, and the technique may be faced with challenges.

Anomalous Aortic Origin of the Right Pulmonary Artery From the Ascending Aorta With Spontaneous Dissection and Thrombosis.

Anomalous aortic origin of the pulmonary artery is rare. It can affect either of the main branches and can be an important cause of neonatal respiratory distress. Early diagnosis and surgical repair is associated with improved survival and long-term outcomes.

HeartMate III as a Bridge to Transplantation in an Adolescent With Failed Fontan Circulation.

HeartMate III is an emerging, small-sized centrifugal ventricular assist device. Its lower pump thrombosis and stroke rates make it favorable for use in pediatrics. We report the use of HeartMate III as a bridge to transplantation in an adolescent with failed Fontan circulation.

Hidden Complexity in Routine Adult and Pediatric Arrhythmias Interpretation: The Future of Precision Electrocardiology.

Classic ECG interpretation is based on identification of waveforms and deductive analysis of the electrical events the waveforms represent. The more in depth the understanding of electrophysiologic cellular interactions, the more precise the interpretation of ECG tracing. Surface ECG has limitations; yet, it is accurate in representing myocytes' pathologic behaviors.

Mechanical Circulatory Support for Single Ventricle Failure.

Mechanical circulatory support (MCS) for failing single ventricle (SV) physiology is a complex and challenging problem, which has not yet been satisfactorily addressed. Advancements in surgical strategies and techniques along with intensive care management have substantially improved the outcomes of neonatal palliation for SV physiology, particularly for hypoplastic left heart syndrome (HLHS). This is associated with a steady increase in the number of SV patients who are susceptible to develop heart failure (HF) and would potentially require MCS at a certain stage in their palliation.

Closing the gap in paediatric ventricular assist device therapy with the Berlin Heart EXCOR® 15-ml pump.

Objectives: The Berlin Heart EXCOR ® (EXCOR) paediatric ventricular assist device is used worldwide for mechanical support of infants and small children with end-stage heart failure. A clinically important gap between the smallest EXCOR blood pump (10 ml) and the next larger size (25 ml) limited the choice of pump size in patients with a body surface area (BSA) between 0.33 and 0.

An Extended Role of Continuous Flow Device in Pediatric Mechanical Circulatory Support.

Background: Mechanical circulatory support in the pediatric population is currently limited to pulsatile ventricular assist devices (VAD). In recent years, the use of durable, newer generation, continuous flow devices have increased substantially among adults with end-stage heart failure. We examined the extended role of this device in the pediatric population (aged less than 18 years).

Successful HeartWare Bridge to Recovery in a 3-Year Old: A Game Changer?

We report a 3-year-old boy weighing 13.5 kg who presented with intractable cardiac failure resulting from myocarditis and was treated by implantation of a HeartWare (HVAD) device. He was discharged home with the device.

Hybrid transatrial stent insertion for left atrial decompression in hypoplastic left heart syndrome with intact atrial septum.

Objectives: To describe a novel strategy for the management of infants with hypoplastic left heart syndrome (HLHS) and intact atrial septum. Antenatally diagnosed infants are delivered in cardiac theatre and immediate left atrial decompression (LAD) using hybrid transatrial stent insertion (HTSI) via sternotomy is carried out.

Background: HLHS with intact atrial septum is a condition incompatible with life following placental separation.

Effect of persistent versus transient donor-specific HLA antibodies on graft outcomes in pediatric cardiac transplantation.

Background: De novo donor-specific HLA antibodies (DSA) are a risk for poor graft outcomes, but there is little evidence of their long-term effect in pediatric cardiac transplantation or of the effect of transient versus persistent DSA found using newer antibody testing methods.

Methods: Archived serum samples were obtained from patients <18 years of age who underwent primary cardiac transplantation during the period from 1996 to 2009. Luminex antibody testing was performed at 3 months, 6 months and 1 year post-transplant, and then annually.