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. Additionally, we delineate areas of uncertainty in various aspects of care, discuss gaps in current evidence, and review current status in less privileged countries and propose initiatives to reduce disease burden. Finally, we propose the application of emerging technologies to enhance the delivery and quality of health care related to ES and beyond.

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. Drug therapies have an effect in systemic left ventricle failure and are employed in acute HF for symptomatic relief. Data on elective drug therapy for the failing systemic right ventricle and/or Fontan circulation is currently lacking. Drugs such as angiotensin receptor blockers with neprilysin inhibitors or sodium-glucose co-transporter-2 inhibitors may show benefit. Cardiac resynchronization therapy, in appropriately selected patients, is considered a treatment option. Mechanical circulatory support and transplantation remain the last resource in highly selected patients. As the congenital heart disease population continues to grow and age, both outpatient and inpatient service for HF will continue to play a major role in the care of adult patients with congenital heart disease.

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. surgical closure, consideration of pre-closure ablation for patients with atrial tachycardia and suitability for closure or/and targeted therapy for patients with PAH. This review serves to update the clinician on the latest evidence, the nuances of optimal diagnostics, treatment options, and long-term follow-up care for patients with an ASD.

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. RESULTS: For cardiac surgery the primary efficacy endpoint of total allogeneic blood products (units vs mL/kg for pediatric patients) administered intraoperatively and postoperatively through day 5 or hospital discharge is recommended. For mechanical circulatory support outside the perioperative period the recommended primary outcome for extracorporeal membrane oxygenation is a 5-point ordinal score of thrombosis and bleeding severity adapted from the Common Terminology Criteria for Adverse Events version 5.0. The recommended primary endpoint for ventricular assist device is freedom from disabling stroke (Common Terminology Criteria for Adverse Events AE ≥ grade 3) through day 180. CONCLUSIONS: The proposed composite risk scores could impact the design of upcoming clinical trials and enable comparability of future investigations. Harmonizing and disseminating global consensus definitions and management guidelines can also reduce patient heterogeneity that would confound standardized primary outcomes in future research.

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. They presented with severely depressed left ventricle function and underwent heart transplantation with excellent outcomes. Given the incidence of heart defects and cardiomyopathy in 1p36 deletion syndrome, it should be recommended that children with this genetic condition have screening for cardiac disease. These cases add to the current literature by demonstrating the potential therapeutic options for non-compaction in 1p36 deletion syndrome and showed the favorable outcomes.

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. METHODS: We used a left anterior mini-thoracotomy in six male patients (15 ± 1.94 years of age) who developed progressive right ventricular enlargement due to chronic PR. RESULTS: Primary diagnoses were tetralogy of Fallot in five patients and pulmonary atresia with an intact septum in another. Four patients had previous median sternotomy with transannular patch repair. The mean right ventricular end-diastolic volume index was 189 ± 27.13 ml/m2 . The procedure was feasible in all patients. All patients had satisfactory adult size pulmonary bioprosthesis (25 or 27 mm valve), with a mean peak gradient of 18 ± 2.40 mmHg across the prosthesis at discharge. All patients were extubated intraoperatively at the end of the procedure and required no intraoperative transfusions. There were no early or late mortalities. Early morbidities included left hemidiaphragm paralysis in one patient, and re-sternotomy for prosthetic valve endocarditis in one. One patient required late reoperation for a common femoral artery pseudoaneurysm. CONCLUSIONS: Minimally invasive access for PVR is feasible in both primary and repeat settings, through a limited anterior left minithoracotomy in the absence of intracardiac shunts and the need for other concomitant cardiac procedures. Longer-term studies with a larger number of patients are needed to compare the efficacy of this approach to standard sternotomy.

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. This report summarizes the outcomes of patients supported with EXCOR from the Advanced Cardiac Therapies Improving Outcomes Network (ACTION) registry. These data were part of the PMA surveillance study (PSS) required by the Food and Drug Administration. METHODS: ACTION is a learning collaborative of over 40 pediatric heart failure programs worldwide, which collects data for all VAD implantations as one of its initiatives. All patients in North America with EXCOR implants reported to ACTION from 2018 to 2020 (n = 72) who had met an outcome were included in the EXCOR PSS group. This was compared with a historical, previously reported Berlin Heart EXCOR study group (Berlin Heart study [BHS] group, n = 320, 2007‒2014). RESULTS: Patients in the PSS group were younger, were smaller in weight/body surface area, were more likely to have congenital heart disease, and were less likely to receive a bi-VAD than those in the BHS group. Patients in the PSS group were less likely to be in Interagency Registry for Mechanically Assisted Circulatory Support Profile 1 and were supported for a longer duration. The primary anticoagulation therapy for 92% of patients in the PSS group was bivalirudin. Success, defined as being transplanted, being weaned for recovery, or being alive on a device at 180 days after implantation, was 86% in the PSS group compared with 76% in the BHS group. Incidence of stroke was reduced by 44% and the frequency of pump exchange by 40% in the PSS group compared with those in the BHS group. Similarly, all other adverse events, including major bleeding, were reduced in the PSS group. CONCLUSIONS: The PSS data, collected through ACTION, highlight the improvement in outcomes for patients supported with EXCOR compared with the outcomes in a historical cohort. These findings may be the result of changes in patient care practices over time and collaborative learning.

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.

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.

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 27.4 ± 45.60 months; 36 males [62%]) between March 2000 and April 2020 were included. The most common anatomic variant was a right aortic arch (RAA) with aberrant left subclavian artery (ALSCA) (n = 29; 50%). Kommerell's diverticulum was present in 23 of these patients (79%). The second most common variant was a double aortic arch (n = 22; 38%), followed by pulmonary artery sling (n = 4; 6%), RAA with mirror image branching and left ligamentum arteriosum (n = 3; 5.2%), and left aortic arch (LAA) with aberrant right subclavian artery (n = 1; 1.7%). One patient had a double ring with pulmonary artery sling and RAA with ALSCA. Symptoms were present in 42 patients (72%). Left lateral thoracotomy was the approach in 50 patients (86%), while sternotomy was used in 8 (14%). Symptomatic improvement occurred in the majority of symptomatic patients (93%). There was one perioperative mortality (1.7%) in the symptomatic group which was non-VR related. Morbidities included recurrent laryngeal nerve injury in three patients (5.2%) and transient chylothorax in two (3.4%). Persistence/recurrence of symptoms resulted in one early and one late reoperation. The mean follow-up was 3 ± 5 years. In the current era, VR repair in children including asymptomatic ones can be performed with excellent results. We recommend complete repair of RAA with aberrant LSCA by resection of Kommerell's diverticulum and translocation of the ALSCA to avoid recurrence.

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.

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. Recent advances have improved understanding of arrhythmias by reconstructing their mechanisms of induction and maintenance and exploring cellular channel dysfunction. Translating this knowledge to ECG analysis will create the link that allows ECG interpretation to reach the level of precision electrocardiology. This article presents cases illustrating new techniques for electrophysiologists.

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. (Level of Difficulty: Advanced.).

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. We have reviewed the literature regarding the reported modalities of MCS use in the management of SV patients. This includes analysis of various devices and strategies used for failing circulation at distinct stages of the SV pathway: after neonatal palliation, after the superior cavo-pulmonary connection (SCPC), and after total cavo-pulmonary connection (TCPC).

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 0.5 m 2 . We present the first clinical experience from the early product surveillance (EPS) of the new EXCOR 15-ml blood pump. METHODS: After CE and U.S. Food and Drug Administration approval in January 2013, 20 patients with a mean age of 1.6 years (range 0.5-3.5 years) and a mean BSA of 0.45 m 2 (range 0.33-0.59 m 2 ) were enrolled in the EPS. The main diagnosis was idiopathic cardiomyopathy in 13 patients; the majority ( n = 16) of children were in INTERMACS level 1 or 2. Data from high-volume paediatric transplant centres were collected prospectively for a defined follow-up period of 60 days after device implantation. RESULTS: Mean time on the EXCOR 15-ml blood pump was 43 days; the survival rate was 100% at the end of the EPS period. Seven patients underwent a heart transplant from the device; 2 children were weaned; and 11 patients remained on support. Infection of cannula exit sites occurred in 3 patients. Two patients had minor thromboembolic strokes but made a complete neurological recovery. CONCLUSIONS: The new EXCOR 15-ml blood pump demonstrated optimal ventricular assist device support of children with a BSA of 0.33-0.5 m 2 .

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). METHODS: Between 2010 and 2015, 12 patients (median age 7.1 years; range, 3.7 to 17.0; one third of patients were aged 5 years or less) received a HeartWare ventricular assist device (HVAD; HeartWare, Framingham, MA), 11 for cardiomyopathy and 1 for posttransplant rejection. Right VAD support (n = 5; 42%) was provided by a short-term device (Levitronix, Zurich, Switzerland). RESULTS: Overall, 1 patient died (day 638), 8 patients (67%) underwent transplantation, 1 patient (8.3%) recovered, and 2 patients (17%) remain on HVAD. The mean length of support was 150 days (range, 16 to 638). Four patients (33.3%) were discharged home (all left VAD). In the left VAD group (n = 7), 3 patients subsequently received transplants (days 185, 201, and 234, respectively), 1 recovered (day 149), 1 died (day 638), 1 remained on HVAD (day 198), and 1 needed conversion to biventricular assist device (BIVAD [day 73]). In the BIVAD group (n = 5), right VAD was weaned in 3 (60%), all subsequently received transplants, and 2 remained on BIVAD support until transplant (days 16 and 17, respectively). One BIVAD patient required conversion to central cannulation for longer-term support. Four BIVAD patients (80%) were in Interagency Registry for Mechanically Assisted Circulatory Support level 1 before VAD compared with 2 (29%) in the left VAD group (p = not significant). The actuarial survival rate was 100% at 1 year with no neurologic events. CONCLUSIONS: The third-generation, continuous flow device can provide durable support in the pediatric population. The selection strategy for patients who benefit most from the device continues to evolve. It is anticipated that a smaller design in the future will benefit an even wider pediatric population with heart failure.