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BackgroundDilated 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 summaryWe 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.DiscussionPercutaneous 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.

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.

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.

Pediatric drugs 23(2), 195-202 (2021). doi:10.1007/s40272-021-00438-2, Published by Springer, Berlin [u.a.]

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.

Neonatal Marfan syndrome (nMFS) is a rare and severe form of Marfan syndrome (MFS) with a poor prognosis, that presents with a highly variable phenotype, particularly regarding skeletal, ocular, and cardiovascular manifestations. Mutations in the fibrillin-1 (

Basierend auf Erfahrungen vorwiegend in den USA berichtet dieser Artikel uber den komplikationslosen Verschluss eines hamodynamisch relevanten persistierenden Dutus arteriosus (PDA) bei einem Fruhgeborenen mit bronchopulmonaler Dysplasie (BPD) und pulmonaler Hypertension mit einem Medtronic Micro Vascular Plug® (MVP) und zeigt mogliche Vorteile des MVP gegenuber anderen PDA-Verschluss-Systemen auf.

Background: Congenital duodenal obstruction (CDO) can be complete (CCDO) or incomplete (ICDO). To date there is no outcome analysis available that compares both subtypes. Aim: To quantify and compare the association between CCDO and ICDO with outcome parameters. Methods: We retrospectively reviewed all patients who underwent operative repair of CCDO or ICDO in our tertiary care institution between January 2004 and January 2017. The demographics, clinical presentation, preoperative diagnostics and postoperative outcomes of 50 patients were compared between CCDO (n = 27; atresia type 1-3, annular pancreas) and ICDO (n = 23; annular pancreas, web, Ladd´s bands). Results: In total, 50 patients who underwent CDO repair were enrolled and followed for a median of 5.2 and 3.9 years (CCDO and ICDO, resp.). CCDO was associated with a significantly higher prenatal ultrasonographic detection rate (88% versus 4%; CCDO vs ICDO, P < 0.01), lower gestational age at birth, lower age and weight at operation, higher rate of associated congenital heart disease (CHD), more extensive preoperative radiologic diagnostics, higher morbidity according to Clavien-Dindo classification and comprehensive complication index (all P ≤ 0.01). The subgroup analysis of patients without CHD and prematurity showed a longer time from operation to the initiation of enteral feeds in the CCDO group (P < 0.01). Conclusion: CCDO and ICDO differ with regard to prenatal detection rate, gestational age, age and weight at operation, rate of associated CHD, preoperative diagnostics and morbidity. The degree of CDO in mature patients without CHD influences the postoperative initiation of enteral feeding.

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.

Aim The purpose of this study is to describe the special aspects of perimembranous ventricular septal defects (pmVSD) closure by utilizing Amplatzer Duct Occluder II (ADO II) devices with a rational request for bigger ADO-II sizes, based on our experience in transcatheter device closure of pmVSD. Methods and results At our institution, placement of an ADO II device was used in 15 patients with pmVSD; the patients' age ranged between 6 months and 20 years. The indications for closure were CHF (n = 4), hemodynamically significant shunt (n = 7), tricuspid regurgitation (n = 3), and high risk for infective endocarditis (n = 2), respectively. The location of the VSD was infracristal in 13 patients, supracristal in 1, and a postsurgical Gerbode VSD in another one. Implantation of the device was successfully performed without embolization, any evidence of an AV block, or other conductance abnormalities during implantation and follow-up in the mean of 2.5 years (range 2 months-6.5 years). Conclusions Transcatheter closure of a pmVSD with ADO II is feasible in all pediatric and young adult age groups, by considering the device diameter limitations. The off-label use of ADO II implantation seems to be safe for VSDs closure up to 6 mm of size and feasible for various locations including unusual morphology such as postsurgical Gerbode defect.

The etiology of pediatric heart failure is heterogenous. Missing evidence-based studies lead to symptom-based therapies. Regarding children’s life expectancy, heart regenerative strategies are required. Pathophysiological differences such as the dependence of the ventricles on the preload, the tendency towards peripheral centralization with predominantly catecholamine-controlled circulatory regulation as well as age and disease-related differences in the myocardial s-receptor physiology require individualized precision medicine with the concept of a “responder phenotype”. First-line therapy with diuretics for chronic heart failure is not indicated. Inadequate diuretic therapy is a common reason that anti-adrenergic and renin-angiotensin-aldosterone antagonists are not or only insufficiently administered. Regarding pediatric dilated cardiomyopathy, selective s1-blockers and tissue ACE-inhibitors are indicated primarily for their activity and side-effect profile, as well as for reasons of heart regeneration. A high-selective s1-blocker, (bisoprolol) protects against noradrenaline-associated myocytic apoptosis and necrosis, but allows β2-receptor mediated myocardial regeneration. A reduced heart rate lowers myocardial oxygen consumption and extends the diastolic filling and coronary perfusion time. Despite the fact that the risk of cardiac fibrosis in infants is lower, the combination of bisoprolol-lisinopril-spironolactone (B-L-S) is still beneficial. Effective B-L-S doses can reduce systemic vascular resistance by ensuring myocardial perfusion pressure due to an adequate intravascular volume (preload). Almost identical daily doses of bisoprolol and lisinopril (0.1-0.2 (0.4) mg/kg/day) and 0.5-1 (- 2) mg / kg spironolactone enable a high level of compliance in the treatment by the parents or young patients. In summary, individualized heart failure therapy is the prerequisite for further regenerative strategies.

Approximately 5% of percutaneous pulmonary valve implantations (PPVIs) are at risk for coronary compression. Therefore, PPVI is contraindicated if coronary anomalies and tested coronary flow impairment are observed. Simultaneous right ventricular outflow tract ballooning and coronary angiography are mandatory elucidating contraindications for PPVI. We present the case of a 22-year-old woman who had Rastelli repair with chronic right heart failure. Weighing the risk of several offered surgical options, she underwent successful PPVI after minimally invasive direct coronary artery bypass procedure.

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.

Objectives We investigated whether transcatheter aortic valve replacement (TAVR) results in a short-term decrease in left atrium (LA) size and whether such decrease may predict patients' clinical outcome. Background Increased LA size is a hallmark of severe aortic stenosis (AS) and is associated with adverse patients' cardiovascular outcome. Whether TAVR may lead to a decrease in LA size is not known. Methods and results Hundred and four patients with severe symptomatic AS and dilated LA undergoing TAVR were enrolled. LA volume was assessed by echocardiography before and shortly after TAVR (median time: 7 days). Composite rate of death and hospitalization for acutely decompensated heart failure (HF) was recorded and clinical status was assessed through NYHA-class evaluation at 12 months median follow-up. After TAVR, 49 patients (47%) demonstrated a decrease in LA volume. Despite a similar baseline NYHA class, patients with decrease in LA size had significant better improvement in clinical status respect to patients with unvaried LA size (NYHA post: 1.2 ± 0.6 vs. 1.8 ± 1.1, p = .001; NYHA reduction: -1.6 ± 0.9 vs. -0.9 ± 1.0, p = .002, respectively). Moreover, these patients had a significantly reduced rate of death or HF-hospitalization (4 vs. 29%, p = .001) and a significantly longer event-free-survival from Kaplan-Meier curves (p = .003). COX regression analysis showed that, among echocardiographic parameters, decrease in LA size was an independent predictor of clinical outcome (HR: 0.149, CI: 0.034-0.654, p = .012). Conclusions The lack of decrease in LA size shortly after TAVR is associated with significantly higher rates of death and HF-hospitalization, as well as with impaired improvement in clinical status during long-term follow-up.

Background Increased left atrium (LA) size is a hallmark of severe aortic stenosis (AS) and is associated with adverse patients' cardiovascular outcome. Whether transcatheter aortic valve replacement (TAVR) may lead to a decrease in LA size is not known. Aim: We investigated whether TAVR results in a short-term decrease in LA size and whether such decrease may predict patients' clinical outcome. Methods 104 consecutive patients with severe symptomatic AS and dilated LA undergoing TAVR were enrolled. LA volume was assessed by echocardiography before and shortly after TAVR (median time: 7 days). Composite rate of death and hospitalization for acutely decompensated heart failure (HF) was recorded and clinical status was assessed through NYHA- class evaluation at 12 months median follow-up. Results After TAVR, 49 patients (47%) demonstrated a decrease in LA volume. Despite a similar baseline NYHA class, patients with decrease in LA size had significant better improvement in clinical status respect to patients with unvaried LA size (NYHA post: 1.2±0.6 vs 1.8±1.1, p=0.001; NYHA reduction: −1.6±0.9 vs −0.9±1.0, p=0.002, respectively). Moreover, these patients had a significantly reduced rate of death or HF-hospitalization (4 vs 29%, p=0.001) and a significantly longer event-free-survival from Kaplan-Meier curves (p=0.003). COX regression analysis showed that, among echocardiographic parameters, decrease in LA-size was an independent predictor of clinical outcome (HR: 0.149, CI: 0.034–0.654, p=0.012). Conclusions The lack of decrease in LA size shortly after TAVR is associated with significantly higher rates of death and HF-hospitalization, as well as with impaired improvement in clinical status during long-term follow-up.

Heart failure (HF) is usually defined by the dominantly affected heart chamber; therefore, termed right or left HF (RHF or LHF). Pulmonologists understand RHF as a complex syndrome characterized by insufficient delivery of blood from the right ventricle associated with elevated systemic venous pressure at rest or exercise. Cardiologists specify LHF by its clinical functional class and the relation to a reduced (HFrEF), preserved (HFpEF) or mid-range ejection fraction (HFmrEF). Pediatric cardiologist, dealing also with patients with a failing single ventricle, define HF as a condition of insufficient systemic oxygen delivery (DO(2)). Certainly, pediatricians do not think of the right and left heart, or even a single ventricle as an isolated, independently acting entity. Because of the importance of cardiac interactions, the creation of a restrictive atrial communication aims at a palliative approach with the goal to diminish the congestive consequences of a dysfunctional ventricle; further to serve as a pop-off valve in order to prevent syncope and cardiovascular collapse. This review covers the background, the particular indications, the techniques and preliminary results achieved following the creation of a restrictive atrial septum defect (rASD) in different pathophysiological settings. Based on the institutional experience, percutaneous trans-catheter perforation of the atrial septum, followed by gradual balloon dilatation can be performed at any age and location worldwide. Medical institutions in low resource countries can make use of such palliating procedures in the setting of right as well as LHF independent of their pharmacological facilities.

BackgroundLeft 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.ConclusionsPercutaneous left atrial decompression is an age-independent, effective palliation treating patients with dilated cardiomyopathy.

A cytomegalovirus-associated heart failure in a young infant with atrial and ventricular septal defects is reported in this case report. The patient recovered by an anti-congestive and anti-viral therapy with an extra percutaneous transcatheter treatment strategy. In the context of bi-ventricular predominant right heart failure associated with supra-systemic pulmonary hypertension, the already closed arterial duct was re-opened and stented to unload the right ventricle and thereby augment the systemic blood flow. Either the left-to-right shunting atrial septal defect or bi-directional shunting ventricular septal defect was involved in the disease process and was not able to avoid global heart failure. After clinical improvement, the stented duct was shunted left-to-right and was occluded with an ADO-II-AS. During the same procedure the atrial septal defect was closed with an Amplatzer-ASD occluder, while the peri-membranous ventricular septal defect was closed with an ADO-II occluder 2 months later.

Objectives Somatic development is impaired in children with congenital heart defects (CHDs), and head circumference seems to be a strong predictor of neurodevelopmental prognosis. The aim of this study was to generate up-to-date reference values for the somatic development (head circumference, body weight, and length/height) of children with CHDs. Study design Our study population consisted of all patients included in the PAN study (Pravalenz angeborener Herzfehler bei Neugeborenen in Deutschland), which was conducted prospectively over a 3-year study period by the Competence Network for Congenital Heart Defects. All children with mild, moderate, and severe CHDs born in 2006-2009 in Germany were enrolled. For computing of z-scores, only children with the following characteristics were included: appropriate for gestational age, nonsyndromic disease, term or post-term delivery, and no cardiac surgery. Results There were 2818 patients included. New z-scores for the described somatic measures of children with mild, moderate, and severe CHDs were computed. Comparisons with the KiGGS study (Gesundheit von Kindern und Jugendlichen in Deutschland) and the Berlin Longitudinal Study revealed significantly lower measurements for all measures—most notably in children with severe CHDs and/or cardiac surgery. In our cohort, no catch-up growth was seen after cardiac surgery. Conclusion Children with severe CHDs demonstrated the most abnormal pattern in growth, including head circumference before and after cardiac surgery, which is indicative of accompanying brain pathology unrelated to operative injury.

We report about a 12-year-old girl who presented with a blood pressure difference between the extremities with the suspicion of an aortic coarctation. After imaging and laboratory tests, the diagnosis of Takayasu arteritis was made. Owing to persistent arterial hypertension despite medical treatment, we initiated a treatment with a balloon angioplasty of the renal arteries with an eluting balloon.

Pediatric heart failure (HF) treatment lagged behind the knowledge of pharmacological research and evidence-based clinical experience in adults. Considering the lack of prospective, double blind randomized studies in children, the review is focused on the preferred indication of specific β1-adrenoreceptor blockers (ARB), mineralocorticoid antagonists and tissue angiotensin-converting enzyme inhibitors (ACE-I). Our recommendations are based on the specificity in children, the effectiveness and the side-effect profile of HF-drugs, the receptor-physiological knowledge and the negative results of the few pediatric HF studies with an "evidence study label". In the interest of our pediatric patients, effective HF treatment has not longer to be postponed by balancing between evidence-based versus pathophysiology-based approach. At our institution, bisoprolol, lisinopril and spironolactone (BLS) are used treating HF in patients with left-right shunt lesions, reduced ejection fraction as well as during the inter-stage after HLHS-Hybrid approach. Chronic use of diuretics and fluid restriction is avoided, if always possible; intravascular volume deficiency stimulates further the neurohumoral axis. Pediatric HF needs to be treated with a strategy respecting the variable pathophysiology and the differences of receptor physiology between children and adult patients. The personalized treatment can be easily proofed by the surrogate parameters as heart rate, breath pattern, weight gain and image-derived parameters as well as biomarkers. Effective HF-therapy is also the basis for novel regenerative strategies in particular for young children with "end-stage" HF avoiding cardiac transplant or death.

Coronary stenting is considered a promising treatment option for patients with coronary artery lesions caused by Kawasaki disease (KD). Here, we report the case of an adolescent with KD who successfully underwent implantation of a fully bioresorbable vascular scaffold (BVS) in a Kawasaki-related, highly obstructed coronary artery. Control coronary angiography 6 months later showed a remaining good result without restenosis or development of aneurysm. Cardial MRI and clinical follow-up revealed stable results at 6 and 18 months without any signs of ischemia. To our knowledge, this is the first report of a successful implantation of a fully bioresorbable vascular scaffold in a young adult with the history of KD. The case illustrates the feasibility and safety of BVS implantation for the treatment of KD. It remains to be confirmed by further studies, if this approach by this novel material might be a therapeutic alternative to coronary bypass grafting or other coronary interventions.

Background Secondary repair of the pulmonary valve after right ventricular outflow tract (RVOT) reconstruction is infrequently reported. This article describes possible techniques of secondary pulmonary valve repair and reports follow-up results. Methods Secondary pulmonary valve repairs in 7 patients (5 children and 2 adults) in our institution were reviewed. All patients presented with a severe pulmonary valve regurgitation associated with RV dilatation and dysfunction after primary RVOT reconstruction. Results The surgical techniques varied in our series, but secondary repair of the incompetent pulmonary valve was possible in all patients. Follow-up was complete, with a mean follow-up of 4.1 ± 2.7 years. There were no operative or late deaths in our group. All valves were repaired successfully, with a mean regurgitation grade of 1.28 ± 0.5 postoperatively. The mean transvalvular gradient was 20 ± 4.1 mm Hg for children and 22.5 ± 3.5 mm Hg for adults, and no significant increase of pulmonary valve regurgitation occurred during follow-up. The mean RV dilatation index (RVDI) decreased significantly from 0.85 ± 0.25 to 0.6 ± 0.2 for children and from 1.4 ± 0.01 to 0.9 ± 0.05 for adults. Conclusions Our results showed functional recovery of the right ventricle after reoperation, with RVDI recovering to almost normal values in children. No significant regurgitation of the secondarily reconstructed pulmonary valve was observed during the 4-year follow-up period. Secondary repair for pulmonary valve incompetence after RVOT procedures might be a valuable alternative to conduit replacement.

Purpose To establish an estimate for the mean pulmonary arterial pressure (mPAP) derived from noninvasive data acquired with magnetic resonance (MR) velocity-encoded sequences. Materials and Methods In seven sedated pigs synchronous catheter-based invasive pressure measurements (IPM) and noninvasive MR were acquired in the main pulmonary artery (MPA) at different severities of pulmonary arterial hypertension (PAH) that were caused by infusion of thromboxane A2 (TxA2). The invasively measured mPAP was correlated with the noninvasive MR velocity data and linear combination equations (LCE) were computed. Results Intravenously applied TxA2 induced a dose dependent level of severity of PAH with an mPAP of up to 54 mmHg without systemic effects. The acceleration time (AT) measured with MR demonstrated the best correlation with the mPAP (r2 = 0.75). The LCE with the highest correlation (R = 0.945, α < 0.01) between IPM and MR revealed a mean difference of 0, a SD of s = 4.66 and a maximal difference of 12.2 mmHg using the Bland-Altman analysis. Conclusion Applying the identified LCE allowed the estimation of the mPAP in an acute and resistance-based model of PAH with high accuracy using noninvasive MR velocity-encoded sequences. J. Magn. Reson. Imaging 2007;26:646–653. © 2007 Wiley-Liss, Inc.

3D-echocardiography using a matrix array transducer was performed in 112 patients. In 98 patients (87.5 %) we were able to acquire adequate 3D-data. The mean time for the 3D-examination was 5.5 min. In valve lesions, septal defects and complex cyanotic heart disease 3D-echocardiography was a confident and helpful innovation for better assessment of the intracardiac morphology in pediatric patients. We conclude that 3D-echocardiography is a useful addition to the conventional 2D-echocardiography.

To evaluate the feasibility of MR-based coronary blood velocity measurements (MRvenc) in patients without coronary artery disease (CAD). Eighty-three patients with angiographically excluded CAD received MRvenc of the proximal segments of both coronary arteries (CAs). Using a retrospectively ECG-gated breath-hold phase-contrast FLASH sequence with high temporal resolution, flow data were technically acquirable in 137/166 (83%) CAs. Quantification and analysis of blood velocities in systole and diastole of both CAs were performed. Biphasic velocity profiles were found in 83/100 CAs. Median systolic and diastolic velocities differed significantly in LCA (19 cm/s, 24 cm/s; P

PURPOSE To provide reference values for MRI-based flow measurements in the main pulmonary artery in healthy children. MATERIALS AND METHODS In 98 healthy children (age: 3 - 17 years, median: 11 years), the main pulmonary artery was examined using MRI-based flow measurements with high temporal resolution (9.6 ms per cardiac phase). RESULTS The acceleration time revealed a distinct age dependency and varied between 90 and 155 ms (mean: 124 ms +/- 14). The relative acceleration time (related to the RR-interval) varied between 12.7 and 27 % (mean: 18 % +/- 2.6). The mean values and the standard deviations for the determined values were as follows: cardiac output (5.4 l/min +/- 1.4), cardiac output normalized to the body surface area (4.2 [l/min]/m(2) +/- 0.8), average systolic flow velocity (66 cm/s +/- 12), maximum systolic flow (309 ml/s +/- 79), mean flow (110 ml/s +/- 30), distensibility of the wall of the main pulmonary artery (79 % +/- 26), peak velocity (96 cm/s +/- 15), pressure gradient along the pulmonary valve (3.8 mm Hg +/- 1.2), stroke volume (63.2 ml +/- 17.9), acceleration volume (23.7 ml +/- 8.7), maximum acceleration of flow (4924 ml/s(2) +/- 1431), and reverse volume (0.2 ml +/- 0.3). CONCLUSIONS The acquired values of reference are applicable to all pediatric patients and serve as a framework for the communication between the radiologist and the pediatric cardiologists. High temporal resolution of the measurement sequence is mandatory. Noticeable deviations of these values should induce additional (probably invasive) evaluation.

The aim of this study was to compare the results of magnetic resonance based shunt volume measurements with the results of the invasive method by the principle of Fick. In 14 children (median age: 16.5 months) with ventricular septal defects the shunt volume was quantified by magnetic resonance flow measurements under spontaneous breathing conditions as well as with invasive angiography during one sedation. A good correlation between both methods was observed (r(2) = 0.8, p0.0001, CI(95%) = 0.62-1.22). A tendency towards higher values in the noninvasive technique was found in the Bland-Altman plot (bias = 3.79). Magnetic resonance based shunt measurements are a reliable alternative to the invasive shunt measurement by cardiac catheterization.