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. Comparisons were performed between those receiving component therapy (CT) versus those receiving LTOWB plus CT (LTOWB+CT). Outcomes included drainage tube (DT) output and total transfusion volumes. Optimization-based weighting was used for adjusted analyses between groups. RESULTS: There were 117 patients transfused with only CT and 127 patients transfused with LTOWB+CT. In the LTOWB+CT group, 66 were Group non-O and 61 were Group O. Total transfusion volumes given from the start of the operation until the first 24 h in the cardiac intensive care unit was a median (IQR) 41 (10, 93) mL/kg in the CT group and 48 (28, 77) mL/kg in the LTOWB+CT group, (p = .28). Median (IQR) DT output was 22 (15-32) in CT versus 22 (16-28) in LTOWB+CT groups, (p = .27). There were no differences in death, renal failure and a composite of death and renal failure between the two groups, but there were statistically fewer re-explorations for bleeding in the LTOWB+CT group (p < .001). CONCLUSIONS: The use of LTOWB appears to be safe in <2 years old undergoing cardiac surgery and may reduce re-explorations for severe bleeding. Large trials are needed to determine the efficacy and safety of LTOWB in this population with severe bleeding.

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.

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.

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.

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.

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.

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).

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.

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.

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. His cardiac function subsequently recovered, and the device was decommissioned. We believe this to be the youngest HVAD recipient and the only child to have recovered and had the device decommissioned.

Management of the failing Fontan.

With and increasing number of early survivors after the palliation of the single ventricle physiology there is a burgeoning Fontan population worldwide that will pose unique challenges because of the inevitable sequelae related to the absence of the alleged "needless" sub-pulmonic ventricle. The increasing number and older-age single-ventricle patients highlights the results of successful contemporary surgical palliation in children, leading to the development of an adult single-ventricle population with unpredictable socio-economic and health service impacts. The wide variability in clinical status of patients with Fontan circulation reflects not only the broadened spectrum of morphological substrates involved, but also the evolving surgical techniques during the last four decades. This has come in the wake of a gradual understanding of an incredibly tricky physiology. The magnitude of the disease, the physio-pathological mechanisms, and the therapeutic options to optimize the "failing Fontan" status and to delay the irreversible deterioration of "Fontan failure" condition are discussed in this review.

Outcome of mechanical cardiac support in children using more than one modality as a bridge to heart transplantation.

OBJECTIVES: Mechanical cardiac support (MCS) can successfully be applied as a bridging strategy for heart transplantation (OHTx) in children with life-threatening heart failure. Emergent use of MCS is often required before establishing the likelihood of OHTx. This can require bridge-to-bridge strategies to increase survival on the waiting list. We compared the outcome of children with heart failure who underwent single MCS with those who required multiple MCS as a bridge to OHTx. METHODS: A retrospective study of patients aged less than 16 years was conducted. From March 1998 to October 2005, we used either a veno-arterial extracorporeal membrane oxygenator (VA-ECMO), or the Medos® para-corporeal ventricular assist device (VAD). From November 2005 onwards, the Berlin Heart EXCOR® (BHE) device was implanted in the majority of cases. Several combinations of bridge-to-bridge strategies have been used: VA-ECMO and then conversion to BHE; BHE and then conversion to VA-ECMO; left VAD and then upgraded to biventricular support (BIVAD); conversion from pulsatile to continuous-flow pumps. RESULTS: A total of 92 patients received MCS with the intent to bridge to OHTx, including 21 (23%) supported with more than one modality. The mean age and weight at support was similar in both groups, but multimodality MCS was used more often in infancy (P = 0.008) and in children less than 10 kg in weight (P = 0.02). The mean duration of support was longer in the multiple MCS group: 40 ± 48 vs 84 ± 43 days (P = 0.0003). Usage of multimodality MCS in dilated cardiomyopathy (19%) and in other diagnoses (29%) was comparable. Incidence of major morbidity (haematological sequelae, cerebrovascular events and sepsis) was similar in both groups. Survival to OHTx/explantation of the device (recovery) and survival to discharge did not differ between single MCS and multiple MCS groups (78 vs 81% and 72 vs 76%, respectively). CONCLUSION: Bridge to OHTx with multiple MCS does not seem to influence the outcome in our population. Infancy and body weight less than 10 kg do not tend to produce higher mortality in the multiple MCS group. However, children receiving more than one modality are supported for longer durations.

Durable ventricular assist device support for failing systemic morphologic right ventricle: early results.

BACKGROUND: The systemic morphologic right ventricle (RV) in congenitally corrected transposition of the great arteries or after atrial switch for transposition of the great arteries is associated with late ventricular failure. Although the role of the left ventricular assist device (LVAD) in supporting the failing LV is established, the indications and outcomes of using LVAD in a systemic RV remain unclear. We assessed the role of a third-generation LVAD for systemic RV support. METHODS: Seven patients (mean age, 36 years) received the HeartWare (HeartWare International Inc, Framingham, MA) VAD for systemic RV failure (congenitally corrected transposition of the great arteries in 1 and after atrial switch in 6). Four patients (57%) had severe subpulmonic LV failure, and aggressive perioperative diuresis with or without hemofiltration was used to offload the subpulmonic LV. The indications of VAD were (1) bridge to transplant in 3 and (2) bridge to decision for a high transpulmonary gradient in 4. Transplantation outcome was compared with systemic RV failure without VAD bridge in 19 patients (years 1989 to 2013). RESULTS: Systemic RV support alone was achieved in all patients, with no early deaths (≤30 days). Overall, 6 (86%) returned home, 3 (44%) received a transplant, 2 (28%) died of noncardiac causes, and 2 (28%) continue on VAD support (median support, 232 days). Repeat catheterization (n = 4) showed an improved median transpulmonary gradient in 3 patients (median 18.5 mm Hg pre-VAD vs 8.0 mm Hg post-VAD). Two bridge-to-decision patients received transplants at 640 and 685 days. The stroke rate on VAD support was 43% (2 thromboembolic and 1 hemorrhagic; 3 with satisfactory recovery). De novo aortic regurgitation was 29% (n = 2; 1 valve replacement). All patients (n = 3) survived transplantation (vs 10.5% early mortality without VAD bridge; p = 1.00) and were well at follow-up (range, 53 to 700 days). CONCLUSIONS: The third-generation VAD provides durable support for systemic RV failure as a bridge to transplant and as a strategy to reduce pulmonary vascular resistance. Although concomitant subpulmonic LV failure is common, systemic RV support alone was achieved in all patients.

Mechanical circulatory support in pediatrics.

There is no reliable published data on the overall prevalence or incidence of heart failure (HF) in children. However, the success of mechanical circulatory support (MCS) in management of HF has raised the prospect of a previously unavailable treatment modality. Orthotopic heart transplant (OHTx) remains the gold standard treatment, but the number of patients requiring this treatment far outweighs the donor availability. It is therefore not surprising to see the popularity of various MCS modalities, with different devices ranging from veno-arterial extra corporeal membrane oxygenation (VA-ECMO) to ventricular assist devices (VADs), which are either para-corporeal or intra-corporeal, with pulsatile or continuous flow. Indication, timing and the choice of the type of mechanical support are crucial so in order to avoid potential lethal complications such as hemorrhage, thrombo-embolism and infections. In the pediatric population, MCS is used mainly as bridge to transplantation but can be used as bridge to recovery in patients with acute myocarditis or following open-heart surgery. Active research is currently underway to develop newer and more durable devices that will assist the pediatric population across all age groups. This research will support different pathologies that have lower incidences of major morbidities, particularly as greater durations of MCS are expected due to a paucity of donors for OHTx. The combined experience developed through the usage of different devices in pediatric and adult populations has led to the to the application of MCS in some subgroups of grown-up congenital heart diseases (CHDs) patients, particularly those with systemic right ventricular failure.

Four-year outcomes with third-generation centrifugal left ventricular assist devices in an era of restricted transplantation.

OBJECTIVES: Third-generation ventricular assist devices (VADs) are associated with improved outcomes, though in recent clinical trials bridge-to-transplant (BTT) rates are ∼30% at 6 months, so that transplantation can be used as a 'bail out' for serious complications. In the UK, there was a significant reduction in heart transplantation rates over the last decade, so that transplantation from VADs is much less frequent. The objective of this study was to determine outcomes and their predictors in this situation of low BTT rates, and as patients were exposed to long-term support, the incidence and outcomes of VAD thrombosis. METHODS: We analysed outcomes for 102 consecutive patients between 2009 and 2013 (mean age 47 ± 13; VentrAssist n = 6 and HeartWare n = 96). The median duration of support was 462 ± 426 days. RESULTS: Survival rates on the device were 75 and 66% at 1 and 2 years, respectively. Older age and more acute INTERMACS groups were significantly related to reduced survival within the first 90 days (P = 0.030 and 0.010, respectively). Poor preoperative right ventricular (RV) function had a negative effect on survival after 1 year (P = 0.009), though not earlier. VAD thrombosis (n = 24 HeartWare and n = 1 VentrAssist) occurred at 0.18 events per patient-year for HeartWare and 0.07 for VentrAssist devices at a median time of onset at 404 ± 281 days. There was no significant effect of VAD thrombosis on survival. Only 14 of 102 patients were transplanted at a median of 334 ± 347 days, and only 3 were transplanted within the first 6 months. CONCLUSIONS: Third-generation left ventricular assist device implants with a low rate of transplantation have similar survival to destination therapy, and are susceptible to long-term complications of VAD thrombosis and right heart failure.

Mechanical cardiac support in children with congenital heart disease with intention to bridge to heart transplantation†.

OBJECTIVES: A significant number of children affected by congenital heart disease (CHD) develop heart failure early or late after surgery, and heart transplantation (OHTx) remains the last treatment option. Due to shortage of donor organs in paediatric group, mechanical circulatory support (MCS) is now routinely applied as bridging strategy to increase survival on the waiting list for OTHx. We sought to assess the impact of MCS as intention to bridge to OHTx in patients with CHD less than 16 years of age. METHODS: From 1998 to 2013, 106 patients received 113 episodes of MCS with paracorporeal devices as intention to bridge to OHTx. Twenty-nine had CHD, 15 (52%) with two-ventricle (Group A) and 14 (48%) with single-ventricle physiology (Group B). In Group A, 5 children had venoarterial extracorporeal membrane oxygenation (VA ECMO), 6 left ventricular assist device (LVAD), 2 biventricular assist device (BIVAD), 1 VA ECMO followed by BIVAD and 1 BIVAD followed by VA ECMO. In Group B, VA ECMO was used in 7 children, univentricular assist device (UVAD) changed to VA ECMO in 4, UVAD in 2 and surgical conversion to two-ventricles physiology with BIVAD support changed to VA ECMO in 1. RESULTS: Twenty-one of 29 (72%) children survived to recovery/OHTx. Seven of 29 (59%) survived to discharge. In Group A, 11/15 (73%) survived to recovery/OHTx and 9/15 (60%) survived to discharge. Four of 15 (27%) died awaiting OHTx. One child had graft failure requiring VA ECMO and was bridged successfully to retransplantation. One child dying after OHTx had acute rejection, was supported with VA ECMO and then BIVAD but did not recover. One patient had an unsuccessful second run on BIVAD 1 year after recovery from VA ECMO. In Group B, 10/14 (71%) survived to recovery/OHTx and 8/14 (57%) survived to discharge. Four of 14 (29%) died awaiting OHTx. Of deaths after OHTx, 1 occurred intraoperatively and 1 was consequent to graft failure and had an unsuccessful second run with VA ECMO. CONCLUSIONS: Children with CHD can be successfully bridged with MCS to heart transplantation. Single-ventricle circulation compared with biventricular physiology does not increase the risk of death before transplant or before hospital discharge.

Surgical management of a neonate with congenitally corrected transposition of the great vessels, hypoplastic right aortic arch, and Ebstein anomaly.

We report a neonate with a primary diagnosis of congenitally corrected transposition (ccTGA) of the great vessels, hypoplastic right aortic arch, and a severely regurgitant Ebstein tricuspid valve (TV). During the fetal period, she was listed for heart transplantation, and two weeks after birth due to a deterioration of her general condition, we performed a Norwood-Sano modified procedure. After 58 days a donor heart became available and the baby successfully received a orthotopic heart transplantation.