German Registry for Cardiac Operations and Interventions in Congenital Heart Disease: Annual Report 2022.

BACKGROUND: The German Registry for Cardiac Operations and Interventions in Patients with Congenital Heart Disease is a voluntary registry initiated by the German Society for Thoracic and Cardiovascular Surgery and the German Society for Pediatric Cardiology and Congenital Heart Defects. Since 2012, the registry collects data for the assessment of treatment and outcomes of surgical and interventional procedures in patients with congenital heart disease (CHD) of all age groups. METHODS: This real-world, prospective all-comers registry collects clinical and procedural characteristics, adverse events (AEs), mortality, and medium-term outcomes (up to 90 days) of patients undergoing surgical and interventional. A unique pseudonymous personal identifier (PID) allows longitudinal data acquisition in case of further invasive treatment in any participating German heart center. Prior to evaluation, all data sets are monitored for data completeness and integrity. Evaluation includes risk stratification of interventional and surgical procedures and classification of AEs. Each year's data are summarized in annual reports containing detailed information on the entire cohort, all subgroups, and 15 index procedures. In addition, each participating center receives an institutional benchmark report for comparison with the national results. This paper presents a comprehensive summary of the annual report 2021. RESULTS: In 2021, a total of 5,439 patients were included by 22 participating centers. In total, 3,721 surgical, 3,413 interventional, and 34 hybrid procedures were performed during 6,122 hospital stays. 2,220 cases (36.3%) could be allocated to the 15 index procedures. The mean unadjusted in-hospital mortality ranged from 0.4% among interventional and 2% among surgical cases up to 6.2 % in cases with multiple procedures. In-hospital mortality among index procedures accounted for 2.3% in total cavopulmonary connection, 20.3% in Norwood procedures, and 0.4% following interventional closure of patent ductus arteriosus. For the remaining seven surgical and five interventional index procedures, no in-hospital deaths were recorded. The 10-year longitudinal evaluation of 1,795 patients after tetralogy of Fallot repair revealed repeat interventional or surgical procedures in 21% of the patients. Over the same period, 31.1% of 2,037 patients, following initial treatment of native coarctation, required at least one additional hospital admission, 39.4% after initial interventional, and 21.3% after initial surgical therapy. CONCLUSION: The annual report 2021 of the German Registry for Cardiac Operations and Interventions in CHD shows continuously good results in accordance with previous data of the registry. Compared to international registries on CHD, it can be ascertained that in Germany invasive treatment of CHD is offered on a high medical level with excellent quality. The proven fact that patients with various malformations like tetralogy of Fallot and coarctation of the aorta require repeat procedures during follow-up confirms the urgent requirement for longitudinal assessment of all patients presenting with complex lesions.

Socio-demographic parameters and non-cardiac comorbidity related to self-perceived quality of life in young adults after neonatal arterial switch operation for transposition of the great arteries.

Evaluating the relation of non-cardiac comorbidity and socio-demographic factors to physical and mental health-related quality of life (QOL) which has been partially found at elevated risk in young adults after neonatal arterial switch operation (ASO) for transposition of the great arteries (TGA). In a prospective reassessment study, results of 92 unselected young adults (22.8 ± 2.6 years) having undergone evaluation of QOL (SF-36) were related to non-cardiac comorbidity with special respect to neurologic and psychiatric comorbidity and to socio-demographic parameters. Neurologic (14%) contrary to psychiatric comorbidities (6.5%) were more frequent than in the general population. The educational level was higher, the rate of unemployment was double as high compared to the average German population. Significant inverse relations (p = 0.006 to 0.033) existed between physical health domains (physical functioning and general health perception) and non-cardiac, neurologic, and psychiatric comorbidity, as well as correlations between the latter domains and socio-economic status, educational level, and worse employment status (Spearman 0.22-0.41, p < 0.0001 to 0.036). Mental health domains (vitality, social functioning, psychical health) were significantly inversely related with neurologic and psychiatric comorbidity (p = 0.002 to 0.048) and correlated with higher educational level (Spearman 0.25, p = 0.019). Neurologic and psychiatric comorbidities and socio-demographic parameters are significant risk factors for a reduced QOL concerning physical and mental health in young adults with TGA after ASO. Standardized QOL measurement should be part of routine screening programs to detect subclinical physical, neurodevelopmental, and psychosocial comorbidity.

Acute myocardial infarction due to spontaneous coronary artery dissection in a 6-year-old boy with ADHD on the third day of treatment with methylphenidate.

Methylphenidate (MPH) is an indirect-acting sympathomimetic drug and structurally related to amphetamine. It is widely used to treat children aged 6 years and older, as well as adolescents who have attention-deficit/hyperactivity disorder (ADHD). We report on a 6-year-old boy who presented with typical angina symptoms occurring several hours after intake of an increased dose of MPH, which had been initiated for ADHD treatment 2 days earlier. Despite typical angina symptoms, the diagnosis of myocardial infarction due to spontaneous coronary artery dissection of the right coronary artery was delayed. Most epidemiological studies could not detect an increased risk for cardiovascular events in association with ADHD medications. However, the direct temporal relationship in our case indicates the possibility that MPH may trigger spontaneous coronary artery dissection in predisposed patients. Since myocardial infarction in children is rare but comprises various etiologies, awareness of this possible catastrophic event among medical staff may be lower and may delay immediate life-saving diagnostic and therapeutic measures.

Late outcome, therapy and systemic ventricular function in patients with a systemic right ventricle: data of the German National Register for Congenital Heart Defects.

BACKGROUND: Adults with systemic right ventricle have a significant risk for long-term complications such as arrhythmias or heart failure. METHODS: A nationwide retrospective study based on the German National Register for Congenital Heart Disease was performed. Patients with transposition of the great arteries after atrial switch operation or congenitally corrected TGA were included. RESULTS: Two hundred and eight-five patients with transposition of the great arteries after atrial switch operation and 95 patients with congenitally corrected transposition of the great arteries were included (mean age 33 years). Systolic function of the systemic ventricle was moderately or severely reduced in 25.5 % after atrial switch operation and in 35.1% in patients with congenitally corrected transposition. Regurgitation of the systemic atrioventricular valve was present in 39.5% and 43.2% of the cases, respectively. A significant percentage of patients also had a history for supraventricular or ventricular arrhythmias. However, polypharmacy of cardiovascular drugs was rare (4.5%) and 38.5 % of the patients did not take any cardiovascular medication. The amount of cardiovascular drugs taken was associated with NYHA class as well as systemic right ventricular dysfunction. Patients with congenitally corrected transposition were more likely to receive pharmacological treatment than patients after atrial switch operation. CONCLUSION: A significant portion of patients with systemic right ventricle suffer from a relevant systemic ventricular dysfunction, systemic atrioventricular valve regurgitation, and arrhythmias. Despite this, medication for heart failure treatment is not universally used in this cohort. This emphasises the need for randomised trials in patient with systemic right ventricle.

Hemodynamic Evaluation of Children with Persistent or Recurrent Pulmonary Arterial Hypertension Following Complete Repair of Congenital Heart Disease.

Persistent or recurrent pulmonary arterial hypertension (PAH) following complete surgical repair of congenital heart disease (CHD) represents one of the largest group of PAH associated with CHD (PAH-CHD) in recent registry studies and seems to have a particularly poor prognosis. However, little is known about this fourth clinical subclass of PAH-CHD, especially in children. The purpose of this study was to assess specific characteristics of invasive hemodynamics of this disease in children, including acute vasodilator testing (AVT) and pulmonary endothelial function (PEF) and to compare to patients with idiopathic PAH (IPAH), who usually present with a similar fatal clinical course. Thirty-two children with PAH were included in the study, twelve of these patients had PAH-CHD subclass 4 (mean age 8.0 ± 3.4 years) and twenty children had IPAH (mean age 8.6 ± 4.4 years). Cardiac catheterization was performed in all children, including AVT and PEF. PEF was assessed by changes in pulmonary blood flow in response to acetylcholine (Ach) using Doppler flow measurements. Pulmonary flow reserve (PFR) was calculated as the ratio of pulmonary blood flow velocity in response to Ach relative to baseline values. At baseline, the ratio of mean PA pressure to mean systemic arterial pressure (mPAP/mSAP) was comparably high in both groups (0.78 ± 0.32 vs. 0.80 ± 0.22), while the indexed pulmonary vascular resistance (PVRI) was significantly lower in the PAH-CHD group (12.6 ± 6.8 WUxm2) compared to IPAH patients (19.9 ± 10.6 WUxm2) (p = 0.04). Cardiac index was significantly higher in the PAH-CHD group (4.19 ± 1.09 l/min/m2 vs. 3.23 ± 0.76) (p = 0.017). However, AVT revealed a significantly larger maximum response (percentage of fall of PVR/SVR ratio during AVT) in the IPAH group (37 ± 22%) compared to the PAH-CHD group (13 ± 23%) (p = 0.017). PEF showed no significant difference between both patient groups (PFR 1.69 ± 0.71 vs. 1.73 ± 0.68) (p = 0.76). Our study demonstrates significant pulmonary vascular disease in children with persistent or recurrent PAH following complete surgical repair of CHD similar to IPAH patients. Although baseline measures appeared to be more favorable, pulmonary vasoreactivity was markedly impaired in PAH-CHD subclass 4, which may contribute to its negative impact on the long-term outcome of this patient group.

Internal Cardioverter Defibrillator Indications and Therapies after Atrial Baffle Procedure for d-Transposition of the Great Arteries: A Multicenter Analysis.

BACKGROUND: Sudden cardiac death (SCD) is the most important cause of late mortality after atrial baffle procedure for d-transposition of the great arteries (d-TGA). Experience with internal cardioverter defibrillator (ICD) therapy in this population is limited. We conducted a multicenter cohort study to determine the current state of ICD therapy in individuals after atrial baffle procedure. METHODS: Demographic and clinical data as well as data on device implantation, programming, ICD discharges, and complications after atrial baffle procedure for d-TGA from four German centers were analyzed retrospectively. RESULTS: ICD implantation was undertaken in 33 subjects. ICD implantation was undertaken as primary prevention in 29 (88%) and secondary prevention in four (12%) individuals. There were 21 (64%) subjects with atrial reentrant tachycardia (IART). During a median follow-up of 4.8 years, seven appropriate ICD therapies were delivered in three (10%) individuals with primary prevention indication. No appropriate shocks were documented in subjects with secondary prevention indication. A total of 12 inappropriate ICD discharges occurred in eight (24%) individuals due to IART (n = 6) or lead failure (n = 2). ICD-related complications were noted in seven individuals (21%): lead dislodgement/failure in five (15%) and ICD infection in two subjects (6%). CONCLUSIONS: The majority of individuals received an ICD for primary prevention of SCD, thus representing a liberal attitude of physicians for ICD implantation. During a median follow-up of 4.8 years, the rate of appropriate ICD therapies was low and clearly exceeded by inappropriate ICD discharges. Lead failure and IART were present in >20% of the individuals and were frequent reasons for inappropriate ICD discharges. Facing these results, rigorous treatment of IART and careful ICD programming seems mandatory.

Effect of Inhaled Nitric Oxide on Blood Flow Dynamics in Patients After the Fontan Procedure Using Cardiovascular Magnetic Resonance Flow Measurements.

Invasive hemodynamic studies have shown that nitric oxide (NO), a selective pulmonary vasodilator, can lower pulmonary vascular resistance in Fontan patients. Because oximetry-derived flow quantification may be unreliable, we sought to detect changes in blood flow within the Fontan circulation after inhalation of NO using cardiovascular magnetic resonance (CMR). Thirty-three patients (mean age 12.8 ± 7.0 years) after the Fontan procedure underwent CMR as part of their routine clinical assessment. Standard two-dimensional blood flow measurements were performed in the Fontan tunnel, superior vena cava (SVC) and ascending aorta (AAO) before and after inhalation of 40 ppm NO for 8-10 min. Systemic-to-pulmonary collateral (SPC) flow was calculated as AAO - (SVC + tunnel). Heart rate (82 ± 18 to 81 ± 18 bpm; p = 0.31) and transcutaneous oxygen saturations (93 ± 4 to 94 ± 3 %; p = 0.13) did not change under NO inhalation. AAO flow (3.23 ± 0.72 to 3.12 ± 0.79 l/min/m(2); p = 0.08) decreased, tunnel flow (1.58 ± 0.40 to 1.65 ± 0.46 l/min/m(2); p = 0.032) increased, and SVC flow (1.01 ± 0.39 to 1.02 ± 0.40 l/min/m(2); p = 0.50) remained unchanged resulting in higher total caval flow (Qs) (2.59 ± 0.58 to 2.67 ± 0.68 l/min/m(2); p = 0.038). SPC flow decreased significantly from 0.64 ± 0.52 to 0.45 ± 0.51 l/min/m(2) (p = 0.002) and resulted in a significant decrement of the Qp/Qs ratio (1.23 ± 0.23 to 1.15 ± 0.23; p = 0.001). Inhalation of NO in Fontan patients results in significant changes in pulmonary and systemic blood flow. The reduction in SPC flow is accompanied by a net increase in effective systemic blood flow suggesting beneficial effects of pulmonary vasodilators on cardiac output, tissue perfusion and exercise capacity.

Patterns of monocyte subpopulations and their surface expression of HLA-DR during adverse events after hematopoietic stem cell transplantation.

Human leukocyte antigen DR surface expression in "classical" CD14++CD16- (M1), "intermediate" CD14++CD16+ (M2), and "non-classical" CD14+CD16++ (M3) monocytes reflects the activation state of these cells. The full spectrum of monocyte and its function is still unknown. The present pilot study describes the monocyte subpopulations and their human leukocyte antigen DR expression during the post-transplant period as well as during transplant-related adverse events of 30 pediatric patients and young adults with hemato-oncological malignancies and immunodeficiency disorders in comparison to healthy children and young adults. A significant change of the human leukocyte antigen DR expression in all three monocyte subpopulations during the period after bone marrow transplantation depending on the time after transplantation and adverse events could be recognized. Prior to and during sepsis or bacterial infection, a significant decrease in human leukocyte antigen DR expression occurred. A significant increase on CD14++CD16- monocytes could be observed during graft-versus-host disease. The alterations of human leukocyte antigen DR expression on the monocyte subpopulations during adverse events after hematopoietic stem cell transplantation may be a sign of changes in the capacity of these subpopulations. Moreover, human leukocyte antigen DR expression in monocyte subpopulations may be used to monitor treatment responses in these entities.

Human leukocyte antigen DR surface expression on CD14+ monocytes during adverse events after hematopoietic stem cell transplantation.

The human leukocyte antigen DR surface expression on CD14+ monocytes reflects the degree to which these cells have been activated. Given the central role monocytes and macrophages play in the immune system, a decreased human leukocyte antigen DR expression on CD14+ monocytes results in a hallmark of altered immune status during systemic inflammatory response syndrome. We hypothesize that human leukocyte antigen DR expression might be similarly altered after hematopoietic stem cell transplantation and during post-transplant complications. Using flow cytometry, this study investigates the human leukocyte antigen DR surface expression of CD14+ monocytes in 30 pediatric and young adult patients up to 1 year after hematopoietic stem cell transplantation. Normal values were derived from a control group of healthy children, adolescents, and young adults. Human leukocyte antigen DR expression decreased significantly prior and during bacterial infection or sepsis. By contrast, human leukocyte antigen DR expression levels were elevated before and at the time of viremia. Human leukocyte antigen DR expression was also elevated during acute graft-versus-host disease. In contrast, the expression was reduced when patients had hepatic veno-occlusive disease. A significant decrease of human leukocyte antigen DR expression was associated with a relapse of the underlying disease and before death. Human leukocyte antigen DR expression on CD14+ monocytes appears to be a promising parameter that might allow identification of patients at risk after hematopoietic stem cell transplantation.

Creation of a transcatheter fenestration in children with failure of Fontan circulation: Focus on extracardiac conduit connection.

OBJECTIVES: We report our experience with a transcatheter technique to bypass the lung and to thus improve single-ventricle preload and reduce venous congestion in Fontan patients. BACKGROUND: In the absence of a dedicated power source to serve the pulmonary circulation and a significantly elevated transpulmonary pressure gradient, fenestration of the Fontan circulation is an option to improve hemodynamics in patients by relieving excessive systemic venous pressure. METHODS AND RESULTS: From 2005 to 2011, 22 transcatheter fenestrations were performed without any major complications in 19 patients (median age 3.2 years, interquartile range (IQR) 2.7-3.7 years)) with failing Fontan circulation and exceeding systemic venous pressure. In 16 patients, the procedure was performed for acute postoperative failure 1-24 days after surgery. After perforation of the conduit and atrial wall by a Brockenbrough needle and gradual balloon dilation, premounted stents were expanded to create a diabolo configuration with flaring stent edges, leaving a slight but definitive central waist. The procedure resulted in regression of pleural effusions and a significant decrease in systemic venous pressure. Clinical improvement was observed in 16 of the 19 treated patients. Follow-up demonstrated sustained fenestration in 85% of treated patients for at least 24 months. CONCLUSION: Transcatheter creation of a Fontan fenestration is a safe approach despite the anatomic gap between the extracardiac conduit cavity and the atrial wall. Stent implantation allows defining the diameter of the fenestration, reduces spontaneous occlusion, and ensures sustained clinical improvement.

Transcatheter Closure of Perimembranous Ventricular Septal Defects with Left Ventricular to Right Atrial Shunt.

During the development of so-called aneurysmal transformation of perimembranous ventricular septal defects (pmVSD), tricuspid valve (TV) morphology and function may be altered resulting in left ventricular (LV) to right atrial (RA) shunting. The feasibility and outcome of interventional closure of these pmVSD has not been investigated so far. Interventional closure of pmVSD associated with mild to moderate LV-to-RA shunt was performed in four patients (aged 6.5-12.5 years). pmVSD were closed under fluoroscopic guidance by establishing an arteriovenous wire loop via a femoral artery and advancing the delivery sheath from a femoral vein. Before device release (or withdrawal if necessary), residual shunting across the device and TV valve function was investigated by transthoracic echocardiography and LV angiography. pmVSD sizes of 4, 5.5, 8 and 8.5 mm were closed with a 4/4 and 6/6 Amplatzer duct occluder II and an 8- and 10-mm Amplatzer muscular VSD occluder device, respectively. There were no or only minor residual postinterventional LV-to-RA shunts. No atrioventricular blocks were observed during a mean follow-up of 12.5 months (range 6.5-17 months). Transthoracic echocardiography indicated that the elimination of the VSD jet pushing the antero-superior TV leaflet open is the key mechanism for LV-to-RA shunt reduction after transcatheter pmVSD closure. Interventional closure in pmVSD associated with mild to moderate indirect LV-to-RA shunting is feasible and results in significant reduction in or elimination of LV-to-RA shunting.

Permanent cardiac pacing in children: choosing the optimal pacing site: a multicenter study.

BACKGROUND: We evaluated the effects of the site of ventricular pacing on left ventricular (LV) synchrony and function in children requiring permanent pacing. METHODS AND RESULTS: One hundred seventy-eight children (aged <18 years) from 21 centers with atrioventricular block and a structurally normal heart undergoing permanent pacing were studied cross-sectionally. Median age at evaluation was 11.2 (interquartile range, 6.3-15.0) years. Median pacing duration was 5.4 (interquartile range, 3.1-8.8) years. Pacing sites were the free wall of the right ventricular (RV) outflow tract (n=8), lateral RV (n=44), RV apex (n=61), RV septum (n=29), LV apex (n=12), LV midlateral wall (n=17), and LV base (n=7). LV synchrony, pump function, and contraction efficiency were significantly affected by pacing site and were superior in children paced at the LV apex/LV midlateral wall. LV dyssynchrony correlated inversely with LV ejection fraction (R=0.80, P=0.031). Pacing from the RV outflow tract/lateral RV predicted significantly decreased LV function (LV ejection fraction <45%; odds ratio, 10.72; confidence interval, 2.07-55.60; P=0.005), whereas LV apex/LV midlateral wall pacing was associated with preserved LV function (LV ejection fraction ≥55%; odds ratio, 8.26; confidence interval, 1.46-47.62; P=0.018). Presence of maternal autoantibodies, gender, age at implantation, duration of pacing, DDD mode, and QRS duration had no significant impact on LV ejection fraction. CONCLUSIONS: The site of ventricular pacing has a major impact on LV mechanical synchrony, efficiency, and pump function in children who require lifelong pacing. Of the sites studied, LV apex/LV midlateral wall pacing has the greatest potential to prevent pacing-induced reduction of cardiac pump function.

Cardiovascular magnetic resonance assessment of ventricular function and myocardial scarring before and early after repair of anomalous left coronary artery from the pulmonary artery.

BACKGROUND: In patients with anomalous left coronary artery from the pulmonary artery (ALCAPA) left ventricular (LV) dilatation and dysfunction evolves due to diminished myocardial perfusion caused by coronary steal phenomenon. Using late gadolinium enhanced cardiovascular magnetic resonance (LGE-CMR) imaging, myocardial scarring has been shown in ALCAPA patients late after repair, however the incidence of scarring before surgery and its impact on postoperative course after surgical repair remained unknown. METHODS: 8 ALCAPA-patients (mean age 10.0 ± 5.8 months) underwent CMR before and early after (mean 4.9 ± 2.5 months) coronary reimplantation procedures. CMR included functional analysis and LGE for detection of myocardial scars. RESULTS: LV dilatation (mean LVEDVI 171 ± 94 ml/m2) and dysfunction (mean LV-EF 22 ± 10 %) was present in all patients and improved significantly after surgery (mean LVEDV 68 ± 42 ml/m2, p=0.02; mean LV-EF 58 ± 19 %, p<0.001). Preoperative CMR revealed myocardial scarring in 2 of the 8 patients and did not predict postoperative course. At follow-up CMR, one LGE-positive patient showed delayed recovery of LV function while myocardial scarring was still present in both patients. In two patients new-onset transmural scarring was found, although functional recovery after operation was sufficient. One of them showed a stenosis of the left coronary artery and required resurgery. CONCLUSIONS: Despite diminished myocardial perfusion and severely compromised LV function, myocardial scarring was preoperatively only infrequently present. Improvement of myocardial function was independent of new-onset scarring while the impact of preoperative scarring still needs to be defined.

Systemic arterial endothelial function in children and young adults with idiopathic pulmonary arterial hypertension: is there a relation to pulmonary endothelium-dependent relaxation?

Pulmonary arterial endothelial function is known to be affected in patients with idiopathic pulmonary arterial hypertension (IPAH). Current reports also detected peripheral systemic arterial dysfunction in IPAH patients. The purpose of this study was to assess whether there is a relation between pulmonary arterial and systemic arterial endothelial function. Pulmonary arterial endothelium-dependent relaxation was assessed by changes in pulmonary blood flow in response to acetylcholine which were determined using intravascular Doppler flow measurements. Pulmonary flow reserve (PFR) was calculated as the ratio of pulmonary blood flow velocity in response to acetylcholine relative to baseline values. Systemic arterial endothelial function was assessed by the vascular response to reactive hyperemia, and was recorded non-invasively by peripheral arterial finger tonometry under standardized conditions. Thirteen children and young adults [mean age 16.7 (±5.6) years] with IPAH and 13 age-/gender-matched controls were included in the study. Digital reactive hyperemic index (RHI) of the IPAH patients was 1.54 (±0.69), and of the controls was 1.67 (±0.66) [p = 0.64]. The mean baseline flow velocity in the segmental pulmonary artery of all patients was 18.5 (±5.5) cm/s, increasing to 27.4 (±12.3) cm/s (p = 0.003) during acetylcholine infusion. The calculated mean PFR was 1.48 (±0.4). There was no significant correlation between the PFR and RHI (r = 0.19; p = 0.54). According to our results, systemic arterial endothelial function assessed by peripheral arterial finger tonometry was not significantly impaired in children and young adults with IPAH compared with age-/gender-matched controls. There was no correlation between systemic arterial and pulmonary arterial endothelial function, suggesting that different mechanisms may contribute to their pathogenesis and progression.

Functional regeneration of dilated cardiomyopathy by transcatheter bilateral pulmonary artery banding: first-in-human case series.

Background: Dilated cardiomyopathy (DCM) is a leading cause of heart transplantation (HTx) in children. Surgical pulmonary artery banding (PAB) is used worldwide to achieve functional heart regeneration and remodelling. Case summary: We report for the first-time successful bilateral transcatheter implantation of bilateral pulmonary artery flow restrictors in a case series of three infants with severe DCM based on left-ventricular non-compaction morphology associated with Barth syndrome in one and a non-classified syndrome in another. Functional cardiac regeneration was observed in two patients after almost 6 months of endoluminal banding, and in the neonate with Barth syndrome already after 6 weeks. Accompanied by an improvement in functional class (Class IV to Class I), the left ventricular end-diastolic dimensions z-score normalized, as did the elevated serum brain natriuretic peptide levels. A listing for HTx could be avoided. Discussion: Percutaneous bilateral endoluminal PAB is a novel minimally invasive approach that enables functional cardiac regeneration in infants with severe DCM and preserved right ventricular function. Interruption of the ventriculo-ventricular interaction, the key mechanism for recovery, is avoided. Intensive care for these critically ill patients is reduced to a minimum. However, investing in 'heart regeneration to avoid transplantation' remains a challenge.

Left Ventricular Physiology and Ventricular-Vascular Interactions in Young Patients After Heart Transplantation.

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/m2 (P<0.0001), whereas ventricular compliance remained rather unchanged (0.16±0.10 mm Hg/mL/m2 to 0.12±0.07 mm Hg/mL/m2; 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/m2 (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.

A novel technique for zero-fluoroscopy catheter ablation used to manage Wolff-Parkinson-White syndrome with a left-sided accessory pathway.

Conventional catheter ablation of cardiac arrhythmias is associated with the potential adverse effects of low-dose ionizing radiation on both patients and laboratory personnel. Due to the greater radiation sensitivity and the longer life expectancy of children, reduction of radiation exposure for them is of particular importance. A novel technique for zero-fluoroscopy catheter ablation is described using real-time tissue-tip contact force measurements for a 10-year-old boy who had Wolff-Parkinson-White syndrome with a left-sided accessory pathway.

Rare Case of an Adult With Double-Chambered Left Ventricle.

The rare case of an adult with a double-chambered left ventricle was revealed using multimodality imaging using echocardiography and cardiac magnetic resonance imaging in a 38-year-old asymptomatic male patient. The congenital malformation was dominated by a second, coarsely trabeculated muscular shelf dividing the left ventricle into 2 chambers without signs for left ventricular inflow or outflow tract obstruction. The partition wall did not show any signs for intramyocardial fibrosis in late gadolinium enhancement cardiovascular magnetic resonance imaging. Flow measurements excluded a relevant intracardial shunt across the additive perimembranous ventricular septal defect. There were no signs for global right and left ventricular dysfunction with left and right ventricular volumes and ejection fraction within normal limits. A conservative approach was recommended. In summary, we are able to present the case of an adult with a double-chambered left ventricle with a second muscular "septum" partially dividing the left ventricular cavity without causing a relevant impact on cardiac function or clinical signs for heart failure.

Le cas rare d'un adulte présentant un ventricule gauche à double chambre a été révélé par une imagerie multimodale utilisant l'échocardiographie et l'imagerie par résonnance magnétique cardiaque chez un homme asymptomatique de 38 ans. La malformation congénitale était dominée par une deuxième bande musculaire grossièrement trabéculaire divisant le ventricule gauche en deux chambres sans signes d'obstruction des chambres d'admission et d'éjection du ventricule gauche. La cloison de partition ne montrait aucun signe de fibrose intramyocardique à l'imagerie par résonnance magnétique cardiovasculaire avec rehaussement tardif au gadolinium. Les mesures du débit ont exclu un shunt intracardiaque significatif à travers le défaut septal ventriculaire transmembranaire supplémentaire. Il n'y avait pas de signe de dysfonction ventriculaire droite et gauche globale, les volumes ventriculaires gauche et droit et la fraction d'éjection étant dans les limites normales. Une approche conservatrice a été recommandée. En résumé, nous pouvons présenter le cas d'un adulte porteur d'un ventricule à double chambre avec une deuxième « cloison ¼ musculaire divisant partiellement la cavité ventriculaire gauche sans causer d'effet notable sur la fonction cardiaque ou de signes cliniques d'insuffisance cardiaque.

Anxiety and Depression in Adults With Congenital Heart Disease.

Introduction: Anxiety and depression can worsen outcome in patients with heart disease. We elucidate the prevalence of anxiety and depression in a cohort of adults with congenital heart disease (ACHD). Materials and Methods: Prospective screening for anxiety or depression was performed in 204 consecutive patients of the outpatient clinic of our tertiary care center using the Hospital Anxiety and Depression Scale (HADS) questionnaire and the distress thermometer (DT) as a potential ultra-short screening test. Functional data were assessed at liberty of the responsible physician. HADS scores ≥ 8 were considered doubtful and scores ≥ 11 as confirmed cases of anxiety or depression, respectively. HADS results were compared with a historical group of 100 patients with non-Hodgkin Lymphoma (NHL) as well as German reference values from the literature. Results: Patients from the ACHD cohort were 28 ± 10 years old (mean ± SD, 54% male), 34% had a simple, 51% a moderate, including 52 patients with transposition of the great arteries after arterial switch operation, and 15% a heart defect of severe complexity. Prevalence of depression in ACHD was comparable to the German normal population (5.9% ACHD vs. 5.4% control). In contrast, prevalence of anxiety was higher than expected from reference values (12.7% ACHD vs. 5.6% control). There was a positive association between psychological distress and NYHA class [anxiety: OR 2.67 (95% CI, 1.50-4.76) p = 0.001; depression: OR 2.93 (95% CI, 1.60-5.35) p = 0.0005], but not with age, gender, or heart defect severity. Percentages of patients with ACHD with anxiety were significantly higher than in a cohort of patients with indolent non-Hodgkin lymphoma (NHL) but comparable to those with aggressive NHL (HADS-A ≥ 11: ACHD 12.7%, indolent NHL 2.2%, aggressive NHL 13.2%; p = 0.037 ACHD vs. indolent NHL; p = 0.929 ACHD vs. aggressive NHL). The distress thermometer screening test had only a fair discriminatory ability (AUC 0.708; p = 0.002) and is therefore of limited usability. Conclusion: Adults with congenital heart disease exhibit an increased risk for anxiety disorders independently of the severity of the underlying heart defect. Anxiety prevalence was comparable to a historical cohort of patients with aggressive NHL underlining the importance of a routine screening for psychosocial distress in adults with congenital heart disease.