Heart failure therapy based on interventricular mechanics and cardio-vascular communications.

The heart should not be divided in right and left, whether in health nor in disease. However, the morphological and functional differences between the right and left ventricle should be known and the impact of the ventricle's position considered. Further, the parameters beyond heart rate, contractility, pre- and afterload guaranteeing a sufficient systemic cardiac output have to be integrated in therapeutic measures; preferentially the influence of interventricular mechanics. Despite of recent developments of specific drug therapies, heart failure is associated with a high rate of morbidity and mortality in children. During the progression of heart failure, pulmonary vascular disease is the consequence or the reason for further failing. Clinical symptoms are associated with congestion and low cardiac output at rest or exercise. Improved understanding of the pathophysiological mechanisms particularly of ventricular failure has resulted in the development of innovative therapies that target atrial/ventricular/arterial interactions. Recent advances in interventional and surgical approaches provide promising new strategies to deal with right and left ventricular deterioration. These techniques may delay listing for heart and (heart-) lung transplantation or even make redundant in individual cases. The beneficial effects of these ventricular interaction strategies are mainly based on the mechanics of the interventricular septum and improvement of systolic and diastolic ventricular performance.

Hypoplastic Left Heart: Stage-I Will be Performed Interventionally, Soon.

The hypoplasia of left-sided heart structures shows great variability and complexity. What the many variants have in common is that their heart structures are neither fully developed before nor after birth. Fetuses and newborns require an individual therapy depending on anatomy and function of the heart. Fetal interventions focus on improving left heart structures by catheter-based interventions and maternal hyperoxygenation which promotes growth as the left ventricular preload and blood flow within the cavity increase. Stage-I management of newborns with single ventricle physiology is usually based on the Norwood/Sano surgery or the Hybrid approach. Two more steps are required to ultimately achieve a Fontan circulation. Some centers also use the Hybrid approach for subsequent Norwood operation beyond the neonatal period. After the Hybrid approach, a comprehensive stage-II or corrective surgery is performed, the latter if a bi-ventricular circulation is possible. With progressively improved catheter-based interventions, particularly ductal stenting and manipulations of the atrial septum, the next advance is to develop a bespoke flow restrictor that can be easily inserted into the branches of the pulmonary artery. The main goal is to avoid complex heart operations under general anesthesia, followed by substantial intensive care in the neonatal period, especially for patients with complex heart defects. Based on the current state of the art of surgical treatment of hypoplastic left heart syndrome and variants with the Norwood surgery or the Hybrid approach, our main focus is on an alternative percutaneous transcatheter technique in the sense of a completely non-surgical stage-I approach.

Cardiovascular Drug Therapy during Interstage After Hybrid Approach: A Single-Center Experience in 51 Newborns with Hypoplastic Left Heart.

BACKGROUND: Newborns with hypoplastic left heart (HLH) are usually palliated with the Norwood procedure or a hybrid stage I procedure. Hybrid is our preferred approach. Given the critical relationship between stage I, interstage, and comprehensive stage II or advanced biventricular repair, we hypothesized that appropriate drug treatment is a significant therapeutic cornerstone, especially for the management of the high-risk interstage. METHODS: We report a single-center observational study addressing the cardiovascular effects of, in particular, oral ß-blockers and the additional use of angiotensin-converting enzyme (ACE) and mineralocorticoid inhibitors. RESULTS: In total, 51 newborns-30 with HLH syndrome (HLHS) and 21 with HLH complex (HLHC)-with a median bodyweight of 3.0 kg (range 1.9-4.4; nine with bodyweight ≤ 2500 g) underwent an uneventful "Giessen hybrid approach" using a newly approved duct stent. All patients were discharged home with a single, double or triple therapy consisting of ß-blockers, ACE and mineralocorticoid inhibitors; 90% of the patients received bisoprolol, 10% received propranolol, 72% received lisinopril, and 78% received spironolactone. Resting heart rate decreased from 138 bpm (range 112-172; n = 51) at admission to 123 bpm (range 99-139; n = 51) at discharge and 110 bpm before stage II/biventricular repair/heart transplantation (range 90-140; n = 37) accompanied by favorable bodyweight gain. No side effects were evident. CONCLUSION: In view of drug risk/benefit profiles, as well as the variable morphology and hemodynamics, the highly selective ß1-adrenoceptor blocker bisoprolol is our preferred drug for treatment of HLHS/HLHC in the interstage. We avoid using ACE inhibitor monotherapy and exclude potential risks for coronary and cerebral perfusion pressure beforehand.

Single Nuclei Sequencing Reveals Novel Insights Into the Regulation of Cellular Signatures in Children With Dilated Cardiomyopathy.

BACKGROUND: Dilated cardiomyopathy (DCM) is a leading cause of death in children with heart failure. The outcome of pediatric heart failure treatment is inconsistent, and large cohort studies are lacking. Progress may be achieved through personalized therapy that takes age- and disease-related pathophysiology, pathology, and molecular fingerprints into account. We present single nuclei RNA sequencing from pediatric patients with DCM as the next step in identifying cellular signatures. METHODS: We performed single nuclei RNA sequencing with heart tissues from 6 children with DCM with an age of 0.5, 0.75, 5, 6, 12, and 13 years. Unsupervised clustering of 18 211 nuclei led to the identification of 14 distinct clusters with 6 major cell types. RESULTS: The number of nuclei in fibroblast clusters increased with age in patients with DCM, a finding that was confirmed by histological analysis and was consistent with an age-related increase in cardiac fibrosis quantified by cardiac magnetic resonance imaging. Fibroblasts of patients with DCM >6 years of age showed a profoundly altered gene expression pattern with enrichment of genes encoding fibrillary collagens, modulation of proteoglycans, switch in thrombospondin isoforms, and signatures of fibroblast activation. In addition, a population of cardiomyocytes with a high proregenerative profile was identified in infant patients with DCM but was absent in children >6 years of age. This cluster showed high expression of cell cycle activators such as cyclin D family members, increased glycolytic metabolism and antioxidative genes, and alterations in ß-adrenergic signaling genes. CONCLUSIONS: Novel insights into the cellular transcriptomes of hearts from pediatric patients with DCM provide remarkable age-dependent changes in the expression patterns of fibroblast and cardiomyocyte genes with less fibrotic but enriched proregenerative signatures in infants.

Interrelations of Intraoperative Changes in Cerebral Tissue Oxygen Saturation with Brain Volumes and Neurodevelopment Outcome After the Comprehensive Stage II Procedure in Infants With Hypoplastic Left Heart Syndrome: A Retrospective Cohort Study.

OBJECTIVES: The monitoring of cerebral tissue oxygen saturation by near-infrared spectroscopy (ScerebO2) is used widely in pediatric cardiac anesthesia. However, little information is available on the effects of changes in perioperative ScerebO2 on brain morphology and neurologic outcome. The primary hypothesis tested in this study was that intraoperative ScerebO2 during the comprehensive stage II procedure correlated with brain volumes assessed by magnetic resonance imaging and neurodevelopmental scores. DESIGN: Retrospective observational cohort study. SETTING: University Hospital, Pediatric Heart Centre. PATIENTS AND MEASUREMENTS: In 19 infants, the intraoperative course of ScerebO2 during the comprehensive stage II procedure was examined. Minimal ScerebO2 and integrated ScerebO2 below 45% (AUC) during surgery, as well as cerebral magnetic resonance imaging (MRI) examinations and Bayley III test at the ages of two-to-three years, were analyzed. MAIN RESULTS: A positive correlation between minimal ScerebO2 and intracranial volume (p = 0.0243), total brain volume (p = 0.0243), and white matter volume (p = 0.0276) was observed, as was a negative correlation between AUC and intracranial volume (p = 0.0454) and white matter volume (p = 0.0381), respectively. No association was found between ScerebO2 and Bayley-III Score. CONCLUSION: The correlation between ScerebO2 and brain volumes measured by MRI pointed out a possible importance of neuroprotective strategies aimed at optimizing ScerebO2 during complex congenital heart surgery. That no correlation between ScerebO2 and Bayley III Score was found suggested multifactorial causes for neurologic outcome in children with congenital heart defects.

Anesthesia for bilateral pulmonary banding as part of hybrid stage I approach palliating neonates with hypoplastic left heart syndrome.

BACKGROUND: Neonatal management of patients with hypoplastic left heart syndrome and complex remains a challenging task, whereby the "hybrid" palliation is often reserved for high-risk patients as a "rescue" procedure. AIM: This study documents the anesthetic challenges and potential complications associated with the Giessen hybrid stage I approach. METHODS: The Giessen hybrid stage I approach is focused on surgical bilateral pulmonary artery banding. Retrospective perioperative data were analyzed. Contrary to a stable group A, inotropic treatment before surgery for treatment of postnatal shock classified patients as unstable (Group B). Clinical outcomes considered were inhospital mortality, duration of postoperative mechanical ventilation, postoperative time at the intensive care unit, perioperative vasoactive medication requirements, and red blood cell transfusion. RESULTS: From June 1998 to December 2015, 185 patients were allocated to Group A (n = 165) and Group B (n = 20). The inhospital mortality was 2.2% with no difference between the groups. There was also no difference in the postoperative time on mechanical ventilation and the time in the intensive care unit. Vasoactive medication was more often required in Group B (100%) compared to Group A (19%). In Group B, more red blood cells were transfused 6.0 ± 8.3 vs 2.0 ± 5.8 mL/kg in Group A (P < .05, 95% CI 0.0 - 2.6). CONCLUSION: Considering a learning curve, anesthesia for surgical bilateral pulmonary artery banding palliating patients with hypoplastic left heart syndrome and complex can safely be performed, independent from the preoperative clinical status.

Anesthesia for Pediatric Heart Transplantation: Are Patients With a Failing Hemi-Fontan- or Fontan-Physiology Different?

Background. The absolute number of patients presenting with failure of the hemi-Fontan or Fontan circulation for cardiac transplantation (cTx) will continuously rise. We aimed to analyze the intraoperative differences in patients undergoing cTx for a failing hemi-Fontan or Fontan circulation (group A) with those undergoing cTx for cardiomyopathy and congenital heart disease (group B). Methods. Data of patients undergoing cTx during a 10-year period were compared between group A and group B patients. Results. cTx was performed in 83 patients (group A, n = 21; group B, n = 62). Surgical times including median incision-suture time (549 minutes vs 386 minutes, P < .05) and median organ ischemia time (305 minutes vs 233 minutes, P < .05) were longer in group A patients. After weaning off cardiopulmonary bypass a higher median modified Vasoactive Inotropic Score (16 vs 10, P < .05) was necessary in group A patients. During surgery more fresh frozen plasma (44 mL/kg vs 20 mL/kg, P < .05), platelet concentrates (20 mL/kg vs 14 mL/kg, P < .05), and coagulation factor concentrates were given in group A patients. Mortality during the first 90 days after cTx was higher in group A (23.8% vs 6.5%, P < .05). Conclusion. Patients undergoing cTx for a failing hemi-Fontan or Fontan circulation are challenging. They require an intensive vasoactive and inotropic support; furthermore, special attention should be paid to the management of bleeding complications. cTx for this group of patients is associated with higher 90 days mortality.

Percutaneous pulmonary valve implantation (PPVI) in non-obstructive right ventricular outflow tract: limitations and mid-term outcomes.

BACKGROUND: Percutaneous pulmonary valve implantation (PPVI) has been established as a safe and effective alternative to surgery treating patients with a failing pulmonary valve conduit. Nevertheless, the majority of patients in need of a valve have a native, non-obstructive right ventricular outflow tract (RVOT). The current approved stent-valves have a balloon-expandable design. Pre-stenting of the RVOT to create a landing zone and also protect the valve stability is usually mandatory; large, non-obstructive RVOTs need pre-stenting to reduce the RVOT-diameter for a balloon-expandable valve implantation. METHODS: A retrospective study design was used to analyze the medium-term outcome after PPVI in a series of 26 patients with native or reconstructed RVOT. RESULTS: PPVI was successfully performed in all, but 1 (96%). Within the follow-up of a minimum of 2 years, the percutaneous implanted valves remained competent; a significant pressure gradient was not detected. Furthermore, no PPVI-related complications such as endocarditis, migration or stent fractures were observed. The electrocardiogram at rest, in particular the QRS duration remained unchanged immediate post-PPVI as well as at medium-term follow-up of 24 months. However, ventricular arrhythmias were documented in 3 patients (11.5%); all patients were successfully treated with antiarrhythmic drugs, utilizing metoprolol. A trial of an invasive catheter based RVOT-ablation in one remained unsuccessful; pre-stented RVOT did not allow a successful intervention. CONCLUSIONS: Medium-term follow-up showed excellent results of the mechanical valve function. PPVI utilizing balloon-expandable stent-valves in a native RVOT remains an off-label use. Despite our encouraging results, advanced manipulations of the patched or native RVOT might be associated with significant ventricular arrhythmias. There is a need for less invasive RVOT reduction devices.

Pulmonary artery banding in dilative cardiomyopathy of young children: review and protocol based on the current knowledge.

Dilated cardiomyopathy (DCM) is a leading cause of cardiac death in children. Current therapeutic strategies are focused on improving symptoms of congestive heart failure (CHF); the potentials of cardiac regeneration especially in infants and young children are neglected in particular when DCM is classified as "end-stage". Heart transplantation (HTx) serves as the only life-saving option, despite is palliative character with limited survival time. Therapeutic alternatives are strongly needed, but already existing though less used; presupposed, that cardiac dysfunction and its treatment are not reduced to the four components of heart rate (rhythm), myocardial contractility, preload and afterload. A paradigm shift in the treatment of pediatric heart failure can be achieved by modifying ventricular afterload with improving contra-lateral ventricular function. Adverse ventricular-ventricular interactions (VVI) have the potential to harness them for therapeutic benefit. Surgical placement of a pulmonary artery banding (PAB) utilized in infants and young children with LV-DCM and preserved RV function are able to improve LV function via VVI; it is hypothesized, that functional recovery can be achieved in almost 80% especially of infants with LV-DCM despite criteria for listing to orthotopic HTx. The review summarizes details of the current perioperative treatment enabling each pediatric heart center to utilize rPAB as a strategy for functional recovery, even in centers without the option for Htx. Of course, future studies are needed to delineate the geometrical, temporal and molecular mechanisms of PA-banding-induced ventricular crosstalk and to examine their potential modulation through mechanical, electrophysiological and pharmacological interventions, but our patients are born, now.

Ideal intensive care unit course following comprehensive stage II in hypoplastic left heart syndrome.

Comprehensive stage II is the advanced surgical part of the staged treatment of a newborn with hypoplastic left heart syndrome (HLHS) palliated initially by a Giessen-Hybrid approach. We report an almost ideal course following comprehensive stage II operation with focus on postoperative intensive care strategy. Following a short introduction of the postnatally performed Giessen-Hybrid approach, in which the surgical part is focused on bilateral pulmonary banding and duct stenting as well as manipulation of the atrial septum is postponed to transcatheter approach, it should be emphasized, that the quality of inter-stage I is eminently important for the success of the following comprehensive stage II. Furthermore, the interplay of the responsible surgeon, anesthesiologist, cardiologist and intensivist is mandatory for working as a team with a similar pathophysiological background. Presupposed a sophisticated surgical and anesthesiologic management, the immediate post-operative intensive care is crucial for the patient's final outcome, not only in terms of mortality but even morbidity (long-term neurological condition). Detailed treatment strategies are presented by pathophysiological reasonable hypotheses and the current pharmacological knowledge. Aiming to improve systemic and regional oxygen delivery and lowering oxygen consumption, as a sine qua none for a favorable patient's outcome.

Reduced Biventricular Volumes and Myocardial Dysfunction Long-term After Pediatric Heart Transplantation Assessed by CMR.

BACKGROUND: Long-term cardiac remodeling after heart transplantation (HT) in children has been insufficiently characterized. The aim of our study was to evaluate ventricular size in HT patients using cardiovascular magnetic resonance (CMR) imaging, to find underlying factors related to potentially abnormal cardiac dimensions and to study its impact on functional class and ventricular function. METHODS: Seventy-five pediatric HT recipients (age 14.0 ± 4.2 y) were assessed by using CMR 11.2 ± 5.4 years after HT. Right ventricular (RV) and left ventricular (LV) volumes and mass were derived from short-axis cine images and myocardial strain/strain rate was assessed using myocardial feature tracking technique. Results were compared with a healthy reference population (n = 79, age 13.7 ± 3.7 y). RESULTS: LV end-diastolic ventricular volumes were smaller (64 ± 12 versus 84 ± 12 mL/m; P < 0.001) while mass-to-volume ratio (0.86 ± 0.18 versus 0.65 ± 0.11; P < 0.001) and heart rate (92 ± 14 versus 78 ± 13 beats/min; P < 0.001) were higher in HT patients. LV-ejection fraction (EF) was preserved (66% ± 8% versus 64% ± 6%; P = 0.18) but RV-EF (58 ± 7 versus 62% ± 4%, P = 0.004), LV systolic longitudinal strain (-12 ± 6 versus -15% ± 5%; P = 0.05), diastolic strain rate (1.2 ± 0.6 versus 1.5 ± 0.6 1/s; P = 0.03), and intra and interventricular synchrony were lower in the HT group. Smaller LV dimensions were primarily related to longer follow-up time since HT (ß = -0.38; P < 0.001) and were associated with worse functional class and impaired ventricular systolic and diastolic performance. CONCLUSIONS: Cardiac remodeling after pediatric HT is characterized by reduced biventricular size and increased mass-to-volume ratio. These adverse changes evolve gradually and are associated with impaired functional class and ventricular dysfunction suggesting chronic maladaptive processes affecting allograft health.

Effects of pulmonary artery banding in doxorubicin-induced left ventricular cardiomyopathy.

OBJECTIVE: Central pulmonary banding has been proposed as a novel alternative for the treatment of left ventricular dilated cardiomyopathy in children. We sought to investigate the effects of central pulmonary banding in an experimental model of doxorubicin-induced left ventricular dilated cardiomyopathy. METHODS: Four-month-old sheep (n = 28) were treated with intermittent intracoronary injections of doxorubicin (0.75 mg/kg/dose) into the left main coronary artery. A total dose of up to 2.15 mg/kg of doxorubicin was administered until signs of left ventricular dilation with functional impairment occurred by transthoracic echocardiography evaluation. Animals that survived were treated with surgical central pulmonary banding through a left anterior thoracotomy or sham surgery. Transthoracic echocardiography and pressure-volume loop measurements were used to compare left ventricular function preoperatively and 3 months later. Macroscopic and microscopic histologic examinations followed after hearts were harvested. RESULTS: Nine animals from the central pulmonary banding group and 8 animals from the sham group survived and were included in the final analysis. Both groups showed similar inflammation and fibrosis upon histologic examination consistent with the toxic myocardial effects of doxorubicin. There were no differences in the echocardiographic measurements before central pulmonary banding or sham operation. Baseline measurements before the central pulmonary banding/sham operation were considered as 100%. The central pulmonary banding group had better left ventricular ejection fraction (102.5% ± 21.6% vs 76.7% ± 11.7%, P = .01), with a tendency for smaller left ventricular end-diastolic (101.2% ± 7.4% vs 120.4% ± 10.8%, P = .18) and significantly smaller end-systolic (100.3% ± 12.9% vs 116.5 ± 9.6%, P = .02) diameter of the left ventricle in comparison with the sham animals at 3 months. The end-systolic volume (101.4% ± 31.6% vs 143.4% ± 28.6%, P = .02) was significantly lower in the central pulmonary banding group 3 months postoperatively. Fractional shortening in the long axis (118.5% ± 21.5% vs 85.2% ± 22.8%, P = .016) and short axis (122.5% ± 18% vs 80.9% ± 13.6%, P = .0005) revealed significantly higher values in the central pulmonary banding group. In the conductance catheter measurements, no significant differences were seen between the groups for the parameters of systolic and diastolic function. CONCLUSIONS: Central pulmonary artery banding in the setting of experimental toxic left ventricular dilated cardiomyopathy improved left ventricular echocardiographic function and dimensions.

Transcatheter left atrial decompression in patients with dilated cardiomyopathy: bridging to cardiac transplantation or recovery.

BACKGROUND: Left atrial congestion results from backward failure in dilated cardiomyopathy. We aimed to evaluate feasibility and efficacy of percutaneous atrioseptostomy to create a restrictive atrial septum defect in management of dilated cardiomyopathy.Methods and resultsFrom June 2009 to December 2016, 27 interventions comprised left atria decompressions in 22 dilated cardiomyopathy patients; 9 females; age: 24 days to 36.9 years; weight: 3-50 kg; NYHA-/Ross class IV (n=16). Mean left ventricular ejection fraction was 21.5±9.7% and brain natriuretic peptide was 2291±1992 pg/ml. Dilated cardiomyopathy was classified as chronic (n=9); acute (n=1) myocarditis; idiopathic (n=5); left ventricular non-compaction (n=4); mitochondriopathy, pacemaker induced, and arrhythmogenic (n=3). Atrioseptostomy was concomitantly performed with myocardial biopsies 6.5 days (±11.7) after admission (n=11). Trans-septal puncture was used in 18 patients; foramen ovale dilatation was done in four patients. Mean balloon size was 11 mm (range 7-14 mm); total procedure time was 133±38 minutes. No procedural complications were observed. Mean left atrial pressure decreased from 15.8±6.8 to 12.2±4.8 mmHg (p=0.005), left/right atrial pressure gradient from 9.6±5.6 to 5±3.5 mmHg; brain natriuretic peptide (n=18) decreased from 1968±1606 to 830±1083 pg/ml (p=0.01). One patient unsuitable for heart transplantation died at home despite additionally performed pulmonary artery banding and three further left atrial decompressions; five patients were bridged to transplantation, two died afterwards. Functional recovery occurred in the remaining 14 patients and in six after additional pulmonary artery banding. No patient required assist device. CONCLUSIONS: Percutaneous left atrial decompression is an age-independent, effective palliation treating patients with dilated cardiomyopathy.

Antithrombotic therapy in pediatric ventricular assist devices: Multicenter survey of the European EXCOR Pediatric Investigator Group.

OBJECTIVES: Mechanical circulatory support for pediatric heart failure patients with the Berlin Heart EXCOR ventricular assist system is the only approved and established bridging strategy for recovery or heart transplantation. In recent years, the burden of thromboembolic events has led to modifications of the recommended antithrombotic therapy. Therefore, we aimed to assess modifications of antithrombotic practice among the European EXCOR Pediatric Investigator Group members. METHODS: We sent a questionnaire assessing seven aspects of antithrombotic therapy to 18 European hospitals using the EXCOR device for children. Returned questionnaires were analyzed and identified antithrombotic strategies were descriptively compared to "Edmonton protocol" recommendations developed for the US EXCOR pediatric approval study. RESULTS: Analysis of 18 received surveys revealed substantial deviations from the Edmonton protocol, including earlier start of heparin therapy at 6-12 h postoperatively and in 50% of surveyed centers, monitoring of heparin effectiveness with aPTT assay, administering vitamin K antagonists before 12 months of age. About 39% of centers use higher international normalized ratio targets, and platelet inhibition is changed in 56% including the use of clopidogrel instead of dipyridamole. Significant inter-center variability with multiple deviations from the Edmonton protocol was discovered with only one center following the Edmonton protocol completely. CONCLUSION: Current antithrombotic practice among European EXCOR users representing the treatment of more than 600 pediatric patients has changed over time with a trend toward a more aggressive therapy. There is a need for systematic evidence-based evaluation and harmonization of developmentally adjusted antithrombotic management practices in prospective studies toward revised recommendations.

Creation of a restrictive atrial communication in heart failure with preserved and mid-range ejection fraction: effective palliation of left atrial hypertension and pulmonary congestion.

BACKGROUND: Left atrial decompression is considered in patients with symptomatic heart failure with preserved ejection fraction (HFpEF). We aimed to evaluate the feasibility and efficacy of transcatheter generation of a restrictive atrial septum communication to manage HFpEF from infancy to adulthood with cardiomyopathy and congenital heart defect. METHODS AND RESULTS: From June 2009 to December 2016, 24 patients (50% with an age less than 16 years) with HFpEF were palliated; NYHA-/Ross class IV (n = 10); median systemic ventricular ejection fraction 64 (range 35-78) %. Cardiomyopathy was classified as a restrictive (n = 4) or hypertrophic (n = 2). (75% related to congenital heart defects) Three patients had a systemic right ventricle; in the majority of patients, HFpEF was associated to complex congenital heart defects (n = 18). Mean pulmonary arterial pressures (PAP systolic/diastolic) were 56/28 (± 24/13), left atrial pressures (LAP, v-, a-wave, mean) 26/25/20 (± 7/10/6). Trans-septal puncture was used in 22 patients; foramen ovale dilatation in 2 patients. Median balloon size was 12 (range 6-18) mm; procedure time including diagnostic measures 125 (83-221) min. No procedural death or complications were observed. Mean LA-pressures decreased significantly to 19/19/15 ± 6/8/5 mmHg (p = 0.05); median brain natriuretic peptide (BNP) decreased from 392 (range 93-4401) pg/ml median BNP to 314 (range 61-1544) pg/ml (p = 0.05). Three patients died; one patient received orthotopic heart and one patient a heart-lung transplantation. No patient required so far an assist device. Clinical improvement occurred in all patients, in some after additional surgical or interventional approach. CONCLUSIONS: Transcatheter LA decompression is an age-independent, effective palliation treating patients with HFpEF.

Percutaneous pulmonary valve implantation for reconstruction of a patch-repaired right ventricular outflow tract.

Percutaneous pulmonary valve implantation (PPVI) is nowadays an accepted treatment option to repair post-surgical conduit dysfunction of the right ventricular outflow tract (RVOT). In addition, many patients need a pulmonary valve to reconstruct a hemodynamically incompetent native or conduit free outflow tract. Based on our experience with percutaneous stent-valve placement in a cohort of 125 patients, we report here transvenous reconstruction of a conduit-free, patch repaired outflow tract by utilizing balloon-expandable stent-valves in 23 patients with a median age of 22 years (5-60 years). In 20 patients, the step-by-step procedure was performed uneventful with the aimed success. Severe RVOT dysfunction in term of a clinical relevant regurgitation could be changed to mild, as it was confirmed by follow-up color Doppler echocardiography. In a 5-year-old girl a Melody® valve was placed as a surgical-interventional hybrid approach. In one patient, the procedure was complicated by stent embolization during preparation of the RVOT for stent-valve implantation. Reposition of the embolized stent was nevertheless successful for finishing percutaneous valve-implantation. In one patient, surgical approach became necessary because of the inability to advance the balloon-mounted stent-valve through a pre-stented RVOT. Considering the current available balloon-expandable stent-valves, transvenous pulmonary valve implantation is feasible to treat even an incompetent conduit-free RVOT. However, preparation of the RVOT by pre-stenting, in most patients with more than two stents in telescope technique remains challenging. Reconstruction of RVOT by the current available valves is promising only for a carefully selected group of patients.

Ventricular function and vascular dimensions after Norwood and hybrid palliation of hypoplastic left heart syndrome.

OBJECTIVE: Norwood and hybrid procedure are two options available for initial palliation of patients with hypoplastic left heart syndrome (HLHS). Our study aimed to assess potential differences in right ventricular (RV) function and pulmonary artery dimensions using cardiac magnetic resonance (CMR) in survivors with HLHS. METHODS: 42 Norwood (mean age 2.4±0.8) and 44 hybrid (mean age 2.0±1.0 years) patients were evaluated by CMR after stage II palliation prior to planned Fontan completion. Initial stage I Norwood procedure was performed using a modified Blalock-Taussig shunt, while the hybrid procedure consisted of bilateral pulmonary artery banding and arterial duct stenting. Need for reinterventions and subsequent outcomes were also assessed. RESULTS: Norwood patients had larger RV end-diastolic dimensions (91±23 vs 80±31 mL/m2, p=0.004) and lower heart rate (90±15 vs 102±13, p<0.001) than hybrid patients. Both Norwood and hybrid patients showed preserved global RV pump function (59±9 vs 59%±10%, p=0.91), while RV strain, strain rate and intraventricular synchrony were superior in the Norwood group. Pulmonary artery size was reduced (lower lobe index 135±74 vs 161±62 mm2/m2, p=0.02), and reintervention rate was significantly higher in the hybrid group whereas subsequent outcome did not differ significantly (p=0.24). CONCLUSIONS: Norwood and hybrid strategy were associated with equivalent and preserved global RV pump function while development of the pulmonary arteries and reintervention rate were superior using the Norwood approach. Impaired RV myocardial deformation as a potential marker of early RV dysfunction in the hybrid group may have a negative long-term impact in this population.