BACKGROUND:Myocarditis progressing to a dilated cardiomyopathy (DCM) phenotype carries a high risk of end-stage heart failure in early childhood. OBJECTIVES:Given the age-dependent regenerative capacity of the immature myocardium, we evaluated a staged, pathophysiology-guided strategy combining left atrial decompression and pulmonary artery banding (PAB) to promote myocardial recovery in selected infants and young children. METHODS:We retrospectively analyzed 31 children <3 years (55% female; median age 312 days) with biopsy-proven myocarditis and a DCM phenotype treated between 2013 and 2024. All underwent standardized multimodal assessment, including echocardiography, cardiac magnetic resonance imaging, invasive hemodynamic assessment, and endomyocardial biopsy. RESULTS:At presentation, all patients were in Ross functional class > III; median BNP was 2,258 pg/mL (IQR: 770-4331), and 90% required inotropic support. The mean echocardiography-derived left ventricular (LV) ejection fraction at admission was 24% ± 6.2% with marked LV dilatation (mean z-score +5). The median LV end-diastolic pressure (was 20 mm Hg (IQR: 15-23), indicating elevated filling pressures. A restrictive atrial communication (restrictive atrial septal defect or patent foramen ovale dilatation) was created in 17 patients for atrial decompression. Seventeen patients met the predefined criteria for surgical PAB, including severely reduced LV ejection fraction (mean 19% ± 6%), marked LV dilatation (z-score +6 ± 1.6), and preserved right ventricular function. During a median follow-up of 2 years (IQR: 1-7) overall survival was 90%. Heart transplantation was required in 19%; mechanical circulatory support in 6%. Among patients selected for PAB, 76% achieved sustained functional recovery. CONCLUSIONS:A staged, pathophysiology-guided strategy incorporating left atrial decompression and PAB may promote myocardial recovery in selected young children with myocarditis-associated DCM.

The Norwood procedure was the first therapeutic approach for neonates with hypoplastic left heart syndrome (HLHS), enabling survival through subsequent Fontan circulation. Shortly thereafter, heart transplantation emerged as a biventricular alternative. The Giessen Hybrid approach was developed in response to the high Norwood mortality, offering a survival chance even for high-risk HLHS patients. Based on convincing Hybrid-results, some centers replaced the complex neonatal Norwood surgery. Unlike the North American approach, the Giessen Hybrid combines surgical pulmonary branch banding with percutaneous ductal stenting, designed to evolve into a fully percutaneous Stage-1-Procedure (S1P). The designated transcatheter S1P, referred to as "DIDI" (Double Intention, Double Intervention) was introduced as a dual intervention with placement of pulmonary flow restrictors (PFRs) followed by ductal stenting for newborns with duct dependent systemic blood flow and aims at the "No Norwood, No Hybrid" strategy. This became feasible with CE-approved Nitinol-based stents for ductal stenting and the development of transcatheter pulmonary flow restrictors (PFRs), suitable even for neonates. Recent advancements have enabled the safe neonatal use of PFRs through manual manipulation of CE-approved microvascular plugs via 4 and 5 Fr standard diagnostic catheters. Though off-label, their feasibility and effectiveness in neonatal transcatheter S1P are evident. Various institutions now perform transcatheter S1P with slight technique variations. Our method makes the S1P almost atraumatic for selected newborns with severe left ventricular obstructions. The experiences of 27 newborns treated with the "No Norwood, No Hybrid" strategy, including transcutaneous pulmonary artery banding and ductal stenting, are critically reviewed by experts from Germany.

AbstractAimsHeritable dilated cardiomyopathy (DCM) or DCM associated with congenital or acquired left ventricular diseases carries a significant mortality risk. Pulmonary artery banding (PAB) has been proposed as an alternative to heart transplantation. This study aimed to delineate the clinical development, ventricular reverse remodelling, and functional regeneration of the dilated left ventricle, presenting as a pioneering approach in China.Methods and resultsThis prospective study was initiated in November 2021, involving paediatric patients with a significant dilated left ventricle and preserved right ventricle who underwent surgical PAB. The baseline characteristics and clinical information during follow‐up were collected. Seven patients (five boys) with a median age of 240 (148, 1028) days have been included thus far. No procedural or follow‐up mortality was observed. The modified Ross functional class improved from treatment to follow‐up of 348 (200, 629) days, and the median left ventricular ejection fraction increased from 27.0 (15.0, 34.0) % before surgery to 61.0 (52.0, 68.0) % (P < 0.05); the median left ventricular end‐diastolic diameter and corresponding Z‐scores decreased from 43.0 (40.0, 55.0) mm [+9.4 (+7.7, +11.7)] to 33.0 (29.0, 39.0) mm [+1.8 (+1.3, +3.8)] (P < 0.05). Functional regeneration of the left ventricle was observed in five patients. Three of them underwent balloon dilation of the PAB to relieve excessively elevated right ventricular pressures.ConclusionsThe application of PAB should adhere to strict criteria. Initial results are promising for infants and even toddlers with a dilated left ventricle and limited probability of spontaneous recovery. PAB can be an alternative when there is a shortage of donor transplants and assist devices, especially for low‐ and middle‐income countries.

Arterial duct stenting, pioneered in the early 1990s for newborns with a duct-dependent pulmonary and systemic circulation, has evolved significantly over the past decades. This progressive technique has led to the development of novel therapeutic strategies, including the Hybrid approach introduced three decades ago, and more recently, a complete transcatheter approach for treating newborns with hypoplastic left heart syndrome (HLHS). Subsequently, the transcatheter method has been extended to bi-ventricular lesions and patients with pulmonary hypertension, establishing a reverse Potts-shunt pathophysiology. Considering current experiences, this review aims to assess the strengths, weaknesses, and complications associated with ductal stenting, which represents a critical component of these complex treatment strategies. Despite advancements, the mortality rate of Norwood and Hybrid stage-1 procedures has plateaued, underscoring the importance of enhancing the quality of life of affected patients as the primary therapeutic goal. The prerequisite is a gentle, almost atraumatic medicine, particularly during the newborn period. It is essential to recognize that both the Hybrid and total transcatheter approaches demand comparable experience to Norwood surgery. Successful outcomes hinge on much more than merely inserting a stent into the duct; they require meticulous attention to detail and comprehensive management strategies.

Dose-dependent in vitro effects of aspirin on platelet inhibition and predictors of non-responsiveness have led to the recommendation of significantly higher doses of aspirin (5 mg/kg/day) in newborns and infants. The results are inconsistent with the pharmacodynamic effects of clopidogrel in newborns, where approximately 30

Background In patients after heart transplantation, systemic arterial hypertension and enhanced central aortic stiffness contribute to increased ventricular afterload, which might lead to graft dysfunction. The aim of our study was to characterize systemic arterial elastance and its impact on left ventricular function and ventriculo‐arterial coupling in a cohort of children, adolescents, and young adults after heart transplantation using invasive conductance catheter technique. Methods and Results Thirty patients who had heart transplants (age, 20.0±6.5 years, 7 female) underwent invasive cardiac catheterization including pressure‐volume loop analysis. Load‐independent parameters of systolic (ventricular elastance [Ees]) and diastolic (ventricular compliance) function as well as systemic arterial elastance (Ea, end‐systolic pressure/stroke volume) and ventriculo‐arterial coupling (Ea/Ees) were assessed at baseline level and during dobutamine infusion (10 μg/kg/min). Ees showed an appropriate increase under inotropic stimulation from 0.43 (0.11–2.52) to 1.00 (0.20–5.10) mm Hg/mL/m 2 ( P <0.0001), whereas ventricular compliance remained rather unchanged (0.16±0.10 mm Hg/mL/m 2 to 0.12±0.07 mm Hg/mL/m 2 ; P =0.10). Ventriculo‐arterial coupling Ea/Ees was abnormal at rest and did not improve significantly under dobutamine (1.7 [0.6–6.7] to 1.3 [0.5–4.9], P =0.70) due to a simultaneous rise in Ea from 0.71 (0.37–2.82) to 1.10 (0.52–4.03) mm Hg/mL/m 2 ( P <0.0001). Both Ees and ventricular compliance were significantly associated with Ea at baseline and under dobutamine infusion. Conclusions Patients who underwent heart transplantation show impaired ventriculo‐arterial coupling at rest and under inotropic stimulation despite preserved left ventricular contractile reserve. An abnormal response in vascular function resulting in increased afterload seems to represent an important factor that may play a role for the development of late graft failure.

OBJECTIVES:Prenatal prediction of postnatal univentricular versus biventricular circulation in patients with borderline left ventricle (bLV) remains challenging. This study investigated prenatal fetal echocardiographic parameters and postnatal outcome of patients with a prenatally diagnosed bLV.METHODS:We report a retrospective study of bLV patients at four prenatal centers with a follow-up of one year. BLV was defined as z-scores of the left ventricle (LV) between -2 and -4. Single-ventricle palliation (SVP), biventricular repair (BVR), and no surgical or catheter-based intervention served as the dependent outcome. Prenatal ultrasound parameters were used as independent variables. Cut-off values from receiver operating characteristic curves (ROC) were determined for significant discrimination between outcomes.RESULTS:A total of 54 patients were diagnosed with bLV from 2010 to 2018. All were live births. Out of the entire cohort, 8 (15 %) received SVP, 34 (63 %) BVR, and 12 (22 %) no intervention. There was no significant difference with regard to genetic or extracardiac anomalies. There were significantly more patients with endocardial fibroelastosis (EFE) in the SVP group compared to the BVR group (80 % vs. 10 %), (p < 0.001). Apex-forming LV (100 % vs. 70 %) and lack of retrograde arch flow (20 % vs. 80 %) were associated with no intervention (p < 0.001). With respect to BVR vs. SVP, the LV sphericity index provided the highest specificity (91.7 %) using a cutoff value of ≤ 0.5.CONCLUSION:The majority of bLV patients maintained biventricular circulation. EFE, retrograde arch flow, and LV sphericity can be helpful parameters for counseling parents and further prospective studies can be developed.

Background Trisomy 18 syndrome, the second most common autosomal numerical chromosomal disorder, is associated with multiple-organ abnormalities. Cardiovascular malformations are just one of those affecting prenatal and postnatal life expectancy. In general, the type and scope of treatment are still controversial and are handled very differently. Palliative care usually focuses on improving quality of life or life-threatening conditions. Objective Based on parental treatment preferences for their child, we report the anticongestive drug treatment and consecutive transcatheter therapy of cardiac shunt lesions in a girl with trisomy 18 who was born small-for-date. Methods Anticongestive drug treatment was switched form diuretic-directed drug treatment to our standard care for chronic heart failure in infants, which consists of a ß1-specific beta-blocker (bisoprolol) in combination with an ACE-inhibitor (lisinopril) and a low-dose mineralocorticoid-blocker (spironolactone). In addition, the strategy and technique of semi-invasive transcatheter treatment of significant cardiovascular shunt lesions is reported. At 3 months of age and weighing 2.4kg, a significant arterial duct was first occluded percutaneously with an MVP-5Q device, followed by transcatheter closure of a slightly misaligned perimembranous VSD six weeks later. Both interventions were performed on a spontaneously breathing only sedated baby. Results The device strategy for treating the hemodynamically significant cardiovascular shunt lesions was technically feasible and effective in combination with medication. However, the medium- or long-term outcome cannot be defined due to the limitations caused by the syndrome. Conclusion As part of a medical risk-benefit assessment, the parents should make the decision as to whether their child should be treated or not; the responsible physicians have individually to offer the best treatment option.

The Giessen hybrid strategy is used for initial palliation of HLHS and variants when the intent is to pursue further staged palliative reconstruction toward Fontan circulation. It is also used for initial palliation of HLHC and other anomalies with potential for eventual biventricular repair. From June 1998 - October 2021, 197 patients with HLHS and related variants underwent hybrid stage 1. Follow-up is complete (median 8.3 [range 0-23.3] years). Operative mortality for hybrid stage I was 3.6% (7/197); reduced to 2.8% since 2010 (4/141). Interstage mortality was 4.1% (8/197). Operative mortality for comprehensive stage II was 5.8% (10/172), and since 2010 was 1.8% (2/113 patients). Fontan completion has been accomplished in 117 patients, and 33 are still awaiting stage III. Twelve patients underwent heart transplantation. Over 23 years, overall survival is 77.7% and transplant-free survival is 73.6%.

Background Right ventricular outflow tract (RVOT) stenosis after repair of tetralogy of Fallot has been linked with favorable right ventricular remodeling but adverse outcomes. The aim of our study was to assess the hemodynamic impact and prognostic relevance of right ventricular pressure load in this population. Methods and Results A total of 296 patients with repaired tetralogy of Fallot (mean age, 17.8±7.9 years) were included in a prospective cardiovascular magnetic resonance multicenter study. Myocardial strain was quantified by feature tracking technique at study entry. Follow‐up, including the need for pulmonary valve replacement, was assessed. The combined end point consisted of ventricular tachycardia and cardiac death. A higher echocardiographic RVOT peak gradient was significantly associated with smaller right ventricular volumes and less pulmonary regurgitation, but lower biventricular longitudinal strain. During a follow‐up of 10.1 (0.1–12.9) years, the primary end point was reached in 19 of 296 patients (cardiac death, n=6; sustained ventricular tachycardia, n=2; and nonsustained ventricular tachycardia, n=11). A higher RVOT gradient was associated with the combined outcome (hazard ratio [HR], 1.03; 95% CI, 1.00–1.06; P=0.026), and a cutoff gradient of ≥25 mm Hg was predictive for cardiovascular events (HR, 3.69; 95% CI, 1.47–9.27; P=0.005). In patients with pulmonary regurgitation ≥25%, a mild residual RVOT gradient (15–30 mm Hg) was not associated with a lower risk for pulmonary valve replacement. Conclusions Higher RVOT gradients were associated with less pulmonary regurgitation and smaller right ventricular dimensions but were related to reduced biventricular strain and emerged as univariate predictors of adverse events. Mild residual pressure gradients did not protect from pulmonary valve replacement. These results may have implications for the indication for RVOT reintervention in this population.

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Decision-making for biventricular repair (BVR) or univentricular palliation is challenging in neonates with hypoplastic left heart complex (HLHC). Hybrid strategy can be used successfully to achieve BVR in some of these patients. Between June 1998 and January 2022, 342 patients with a diagnosis of HLHS/variants, ductal-dependent lower body circulation with two ventricles, or HLHC with borderline left ventricle (LV) underwent initial bilateral pulmonary artery banding and ductal stenting in our institution. Among these 342 patients, 224 patients were defined as HLHS/variants and underwent univentricular palliation. 118 patients were determined to have borderline LV and hypoplastic left ventricular structures (HLHC, n = 48) or ductal-dependent lower body circulation with two ventricles (n = 70) considered suitable for BVR. 48 patients had multilevel obstructions including both aortic and mitral valve hypoplasia, aortic arch hypoplasia, and borderline LV. These were considered to have HLHC. These 48 HLHC patients are the subject of this report. Neonatal hybrid palliation at a median age of seven days was performed in 48 patients and 46 patients underwent BVR at a median age of 156 days. In 46 patients who underwent BVR, hospital mortality was not observed. The median follow-up was 66 months, with no late mortality. Heart transplant-free survival at 5, 10, and 15 years was 95.7%. 12 patients (26%) required reoperation and 9 (19.5%) required catheter reinterventions. Hybrid palliation may increase the chances for biventricular circulation in patients with borderline LV and small left-sided structures. Giessen hybrid approach as a left ventricular recruitment strategy achieves excellent early and long-term results.

In this week’s issue of EJCTS, Staehler et al. [1] report a retrospective single-centre study in which perioperative data from a large cohort of 322 newborns with hypoplastic left heart syndrome (HLHS) and variants were analysed who underwent a Norwood procedure in a period of 18 years between 2001 and 2019. The unvarnished study confirmed known risk factors (i.e. mortality) for neonatal Norwood surgery [stage 1 procedure (S1P)] such as additional extracardiac anomalies, birth weight <2.5 kg, restrictive atrial septum communication, pulmonary vein abnormalities and presence of a small ascending aorta [2]. In addition, clinical and haemodynamic variables related to length of stay (LOS) in the intensive care unit (ICU) and subsequent adverse outcomes were shown. Systolic and in part diastolic blood pressure reactions increased postoperative serum lactate levels and ventricular dysfunction of the single right (systemic) ventricle were associated with the LOS and as striking outcome factors. With a focus on the perioperative strategy of a neonatal Norwood procedure, which is still the most common S1P for patients with HLHS, it should be noted that the treatment of this congenital heart defect is still the procedure with the highest mortality and early and late morbidity despite improvements over the last 2 decades [2]. The treatment of newborns with HLHS also means, that of all congenital malformations, including non-cardiac congenital ones, the highest expenditure, including financial resources, is required, despite the highest mortality rate [3].

Given the heterogenous etiology of pediatric heart failure (pHF), evidence-based studies improving pHF are unlikely. A paradigm shift towards updated medicine-based evidence is therefore necessary. In view of the life expectancy of children, cardiac regeneration strategies are required. Therefore, age- and disease-related differences in myocardial (receptor) physiology require individualized precision medicine. First-line diuretic therapy, adopted from the treatment of adults with HF with no chance for recovery, should be questioned in the treatment of pHF with potential for recovery. Inadequate use of diuretics is a common reason for additional stimulation of the neurohumoral axis. Consecutive intravascular volume depletion led to an inadequate treatment with β-blocker and renin–angiotensin–aldosterone antagonists. Given the age-related catecholamine-driven cardiovascular (patho-) physiology, highly selective β1-blockers (bisoprolol) protect against β1-(noradrenaline)-related myocytic apoptosis and necrosis, but allow β2-receptor-mediated myocardial regeneration. Based on its high safety–efficacy profile with rarely seen adverse effects but easily monitorable efficacy by the surrogate of heart rate (reduction), bisoprolol is our first-line drug in infancy. Reduced heart rate economizes the heart and full body oxygen consumption and extends the diastolic filling and coronary perfusion time. Based on our many years of institutional experience, physicians should be encouraged to use β1-selected blockers in infants with dilated cardiomyopathy and hypoplastic left heart syndrome after stage-1 procedure, but also to treat ventricular septal defects with a significant left-to-right shunt. In summary, individualized pHF therapy is the prerequisite for a causal treatment to improve HF symptoms, but above all for the most functional regeneration possible.

BACKGROUND:Transcatheter pulmonary valve (TPV) replacement (TPVR) has become the standard therapy for postoperative pulmonary outflow tract dysfunction in patients with a prosthetic conduit/valve, but there is limited information about risk factors for death or reintervention after this procedure. OBJECTIVES:This study sought to evaluate mid- and long-term outcomes after TPVR in a large multicenter cohort. METHODS:International registry focused on time-related outcomes after TPVR. RESULTS:Investigators submitted data for 2,476 patients who underwent TPVR and were followed up for 8,475 patient-years. A total of 95 patients died after TPVR, most commonly from heart failure (n = 24). The cumulative incidence of death was 8.9% (95% CI: 6.9%-11.5%) 8 years after TPVR. On multivariable analysis, age at TPVR (HR: 1.04 per year; 95% CI: 1.03-1.06 per year; P < 0.001), a prosthetic valve in other positions (HR: 2.1; 95% CI: 1.2-3.7; P = 0.014), and an existing transvenous pacemaker/implantable cardioverter-defibrillator (HR: 2.1; 95% CI: 1.3-3.4; P = 0.004) were associated with death. A total of 258 patients underwent TPV reintervention. At 8 years, the cumulative incidence of any TPV reintervention was 25.1% (95% CI: 21.8%-28.5%) and of surgical TPV reintervention was 14.4% (95% CI: 11.9%-17.2%). Risk factors for surgical reintervention included age (0.95 per year [95% CI: 0.93-0.97 per year]; P < 0.001), prior endocarditis (2.5 [95% CI: 1.4-4.3]; P = 0.001), TPVR into a stented bioprosthetic valve (1.7 [95% CI: 1.2-2.5]; P = 0.007), and postimplant gradient (1.4 per 10 mm Hg [95% CI: 1.2-1.7 per 10 mm Hg]: P < 0.001). CONCLUSIONS:These findings support the conclusion that survival and freedom from reintervention or surgery after TPVR are generally comparable to outcomes of surgical conduit/valve replacement across a wide age range.