Outcomes of extracorporeal membrane oxygenation and cardiopulmonary bypass in children after drowning-related resuscitation.

Drowning is one of the leading causes of accidental deaths in children worldwide. However, the use of long-term extracorporeal life support (ECLS) in this setting is not widely established, and rewarming is often achieved by short-term cardiopulmonary bypass (CPB) treatment. Thus, we sought to add our experience with this means of support as a bridge-to-recovery or to-decision. This retrospective single-center study analyzes the outcome of 11 children (median 23 months, minimum-maximum 3 months-6.5 years) who experienced drowning and subsequent cardiopulmonary resuscitation (CPR) between 2005 and 2016 and who were supported by veno-arterial extracorporeal membrane oxygenation (ECMO), CPB, or first CPB then ECMO. All but one incident took place in sweet water. Submersion time ranged between 10 and 50 minutes (median 23 minutes), water temperature between 2°C and 28°C (median 14°C), and body core temperature upon arrival in the emergency department between 20°C and 34°C (median 25°C). Nine patients underwent ongoing CPR from the scene until ECMO or CPB initiation in the operating room. The duration of ECMO or CPB before successful weaning/therapy withdrawal ranged between 2 and 322 hours (median 19 hours). A total of four patients (36%) survived neurologically mildly or not affected after 4 years of follow-up. The data indicate that survival is likely related to a shorter submersion time and lower water temperature. Resuscitation of pediatric patients after drowning has a poor outcome. However, ECMO or CPB might promote recovery in selected cases or serve as a bridge-to-decision tool.

Retrieval of a Dislocated Stent Using a Grasping Forceps and a Steerable Sheath in a Fontan Patient.

In Fontan patients, any pulmonary stenosis may impede free passive inflow into the pulmonary circuit and elevate central venous pressure. When stenting such pulmonary stenosis, dislocation of a stent is a feared complication. Here, we report on a successful retrieval of a dislocated Cook Formula stent into the peripheral left pulmonary artery in a Fontan patient with protein-losing enteropathy using a steerable sheath and a grasping forceps. Furthermore, a successful stent implantation for treating the stenosis was possible. Subsequently, we simulated the retraction technique ex vivo. To manage stent dislocation during complex pediatric catheter interventions, we can recommend the use of a steerable sheath guiding a grasping forceps. A dislocated stent can be grasped, completely longitudinally refolded, and safely retrieved.

Autologous Tissue Technique to Repair Unilateral Isolated Absence of a Pulmonary Artery.

Isolated unilateral absence of a pulmonary artery (UAPA) is a rare congenital anomaly in which one branch pulmonary artery has no connection to the main pulmonary trunk (most often there is ductal origin). Without treatment, it may lead to ipsilateral pulmonary hypoplasia and contralateral pulmonary artery hypertension. To avoid these complications, early surgical repair of UAPA is necessary. Surgical strategies include direct anastomosis between the "isolated" branch pulmonary artery (PA) and the main pulmonary trunk or creation of an interposition graft using prosthetic material or flap techniques. We describe a surgical technique using a totally autologous interposition tube graft.

Narrative review of Ebstein's anomaly beyond childhood: Imaging, surgery, and future perspectives.

Ebstein's anomaly is a rare congenital heart disease with malformation of the tricuspid valve and myopathy of the right ventricle. The septal and inferior leaflets adhere to the endocardium due to failure of delamination. This leads to apical displacement of their hinge points with a shift of the functional tricuspid valve annulus towards the right ventricular outflow tract with a possibly restrictive orifice. Frequently, a coaptation gap yields tricuspid valve regurgitation and over time the "atrialized" portion of the right ventricle may dilate. The highly variable anatomy determines the clinical presentation ranging from asymptomatic to very severe with need for early operation. Echocardiography and magnetic resonance imaging are the most important diagnostic modalities to assess the tricuspid valve as well as ventricular morphology and function. While medical management of asymptomatic patients can be effective for many years, surgical intervention is indicated before development of significant right ventricular dilatation or dysfunction. Onset of symptoms and arrhythmias are further indications for surgery. Modified cone reconstruction of the tricuspid valve is the state-of-the-art approach yielding the best results for most patients. Alternative procedures for select cases include tricuspid valve replacement and bidirectional cavopulmonary shunt depending on patient age and other individual characteristics. Long-term survival after surgery is favorable but rehospitalization and reoperation remain significant issues. Further studies are warranted to identify the optimal surgical strategy and timing before adverse right ventricular remodeling occurs. It is this article's objective to provide a comprehensive review of current literature and an overview on the management of Ebstein's Anomaly. It focuses on imaging, cardiac surgery, and outcome. Additionally, a brief insight into arrhythmias and their management is given. The "future perspectives" summarize open questions and fields of future research.

Long-term outcome of perimembranous VSD closure using the Nit-Occlud® Lê VSD coil system.

OBJECTIVE: This study presents data from the admission trial to show the feasibility, safety and effectiveness of the Nit-Occlud® Lê VSD in the treatment of perimembranous ventricular septal defects with an aneurysmal configuration and a diameter up to 8 mm. BACKGROUND: The majority of ventricular septal defects (VSD) are still closed surgically, while a less invasive transcatheter treatment by closure devices is available. Device-based closure is reported to be associated with the risk of complete atrio-ventricular block, especially with double-disc devices in perimembranous defects. METHODS: In six tertiary centers in Germany and Israel, an interventional closure of a periembranous VSD was attempted in 88 patients using the Nit-Occlud® Lê VSD. RESULTS: The interventional VSD closure was performed in 85 patients. Patients had a median age of 8.0 (2-65) years and a median body weight of 26.7 (10-109) kg. A complete closure of the defects was achieved in 85.4% 2 weeks after device implantation, in 88.9% after three months and in 98.6% at the 5-year follow-up. There was no incidence of death during the study nor did any patient suffer of permanent atrio-ventricular block of higher degree. Serious adverse events, by definition, are potentially life-threatening or require surgery to correct, while major serious events require medical or transcatheter intervention to correct. The study results exhibit a serious adverse event rate of 3.5% (3/85 patients) and a major adverse event rate of 5.9% (5/85 patients). CONCLUSION: The Nit-Occlud® Lê VSD coil offers the possibility of an effective and safe approach in patients with aneurysmal perimembranous ventricular septal defects.

Genotype-Phenotype Correlation in Children: The Impact of FBN1 Variants on Pediatric Marfan Care.

Currently, no reliable genotype-phenotype correlation is available for pediatric Marfan patients in everyday clinical practice. We investigated correlations of FBN1 variants with the prevalence and age of onset of Marfan manifestations in childhood and differentiated three groups: missense/in-frame, splice, and nonsense/frameshift variants. In addition, we differentiated missense variants destroying or generating a cysteine (cys-missense) and alterations not affecting cysteine. We categorized 105 FBN1-positive pediatric patients. Patients with cys-missense more frequently developed aortic dilatation (p = 0.03) requiring medication (p = 0.003), tricuspid valve prolapse (p = 0.03), and earlier onset of myopia (p = 0.02) than those with other missense variants. Missense variants correlated with a higher prevalence of ectopia lentis (p = 0.002) and earlier onset of pulmonary artery dilatation (p = 0.03) than nonsense/frameshift, and dural ectasia was more common in the latter (p = 0.005). Pectus excavatum (p = 0.007) appeared more often in patients with splice compared with missense/in-frame variants, while hernia (p = 0.04) appeared earlier in the latter. Findings on genotype-phenotype correlations in Marfan-affected children can improve interdisciplinary therapy. In patients with cys-missense variants, early medical treatment of aortic dilatation seems reasonable and early regular ophthalmologic follow-up essential. Patients with nonsense/frameshift and splice variants require early involvement of orthopedic specialists to support the growing child.

Severe heart failure and the need for mechanical circulatory support and heart transplantation in pediatric patients with myocarditis: Results from the prospective multicenter registry "MYKKE".

Myocarditis represents an important cause for acute heart failure. MYKKE, a prospective multicenter registry of pediatric patients with myocarditis, aims to gain knowledge on courses, diagnostics, and therapy of pediatric myocarditis. The role of mechanical circulatory support (MCS) in children with severe heart failure and myocarditis is unclear. The aim of this study was to determine characteristics and outcome of patients with severe heart failure requiring MCS and/or heart transplantation. The MYKKE cohort between September 2013 and 2016 was analyzed. A total of 195 patients were prospectively enrolled by 17 German hospitals. Twenty-eight patients (14%) received MCS (median 1.5 years), more frequently in the youngest age group (0-2 years) than in the older groups (P < 0.001; 2-12 and 13-18 years). In the MCS group, 50% received a VAD, 36% ECMO, and 14% both, with a survival rate of 79%. The weaning rate was 43% (12/28). Nine (32%) patients were transplanted, one had ongoing support, and six (21%) died. Histology was positive for myocarditis in 63% of the MCS group. Patients within the whole cohort with age <2 years and/or ejection fraction <30% had a significantly worse survival with high risk for MCS, transplantation, and death (P < 0.001). Myocarditis represents a life-threatening disease with an overall mortality of 4.6% in this cohort. The fulminant form more often affected the youngest, leading to significantly higher rate of MCS, transplantation, and mortality. MCS represents an important and life-saving therapeutic option in children with myocarditis with a weaning rate of 43%.

Effectiveness of a physical activity intervention in preschoolers: A cluster-randomized controlled trial.

Several activity interventions in preschool settings exist, but little attention has been paid to effects on hemodynamic factors. The study aimed to assess the effectiveness of an exercise program on health-related outcomes including blood pressure (BP) and markers of vascular function in preschoolers, with focus on socioeconomic background. This study is a cluster-randomized controlled trial, with preschool as unit of randomization and children as unit of analysis. Preschools with 3- to 6-year-old children, stratified by social area, were randomly allocated to: intervention (three clusters, n = 92) including 2 d·wk-1 /45 min (6 months) exercise lessons or control (two clusters, n = 43). In total, 135 children (4.8 ± 0.8 y) had minimum one outcome measurement at baseline and follow-up. Primary outcome: peripheral BP. Secondary outcomes: central BP, pulse wave velocity (PWV), BMI, waist circumference, physical activity measures, motor skills. Maternal education was used as an indicator of socioeconomic status. Mixed models were applied to evaluate differences in mean change. Group allocation had no effect on primary or secondary outcomes. However, the intervention was effective in reducing increases in peripheral systolic BP (-3.4 mm Hg; 95% CI: -6.6; -0.2; P = 0.037), central systolic BP (-3.8 mm Hg; -6.4; -1.1; P = 0.006), and PWV (-0.1 m/s; -0.2; -0.0; P = 0.045) among children whose mothers had the lowest educational level. We found no evidence for effectiveness of a 6-months preschool-based exercise program on hemodynamics, anthropometrics, activity, or motor skills, but lack of process evaluations and poor fidelity preclude interpretation of the causal relation. However, the results indicate that children from lower social backgrounds could benefit from early exercise-promoting interventions.

"Tailored" endovascular pulmonary valve and root replacement for rupture of a dilated homograft.

Current balloon expandable and self-expanding valves have limitations for the treatment of the enlarged right ventricular outflow tract. We report the first use of a tailored Zenith graft in composition with an Edwards Sapien S3 valve as an alternative to high-risk surgery for the treatment of a spontaneously ruptured homograft in an adult congenital heart disease patient.

Stenting of Native Right Ventricular Outflow Tract Obstructions in Symptomatic Infants.

OBJECTIVE: To assess feasibility, safety and effectiveness of right ventricular outflow tract (RVOT) stenting in symptomatic young infants. METHODS: Multicentre evaluation of 35 patients intended to undergo RVOT stenting in 11 pediatric cardiac centres from 2009 to August 2011. RESULTS: Median age and weight at the time of first stent implantation were 8 weeks and 3.3 kg, with 40% of patients <3 kg. A total of 19 patients had suffered from hypoxemic spells, 8 patients were ventilated, 6 on inotropic support and 5 on prostaglandin infusion. Severe concomitant malformations were present in 11 patients, and acute infections in 2. Stenting of the RVOT was successfully performed in 33 patients, improving oxygen saturation from a median of 77 to 90% 2 days after intervention. Besides the 2 patients in whom RVOT stenting was not successful for technical reasons, there were no procedural complications. In 17 of 33 patients, 1-3 reinterventions were performed during follow-up, less than half of those were reinterventions in the RVOT. A total of 27 patients have undergone successful surgical repair 4-162 (median 19.5) weeks after initial RVOT stent implantation, 2 patients are still waiting. There were no perioperative deaths. CONCLUSIONS: Stenting of the RVOT provides a safe and effective management strategy for initial palliation in symptomatic young infants, including those patients not suitable or at higher risk for surgical therapy.

Cardiovascular computed tomography angiography in newborns and infants with suspected congenital heart disease: retrospective evaluation of low-dose scan protocols.

Twelve infants (body weight, 3.59±1.36 kg) with congenital heart disease underwent a clinically indicated cardiovascular computed tomography angiography (CTA) study. Retrospectively, we investigated the feasibility and diagnostic capability of different non-electrocardiogram-triggered CTA protocols and assessed radiation doses. Scans were performed on a 256-multislice CT (MSCT) scanner with the vendor-preset helical protocol at 120 kV for babies, with axial single-shoot scans at 120 kV and 80 kV. The 80-kV protocol led to significantly reduced mean effective doses of 0.29±0.08 mSv (P=.017) and renders diagnostic image quality. All major cardiovascular defects were detected on MSCT, and all images were of diagnostic quality.

The impact of shunt type on palliative outcomes in neonates and infants with diminished pulmonary blood flow.

We analyzed early and intermediate outcomes in cyanotic neonates (n = 43) and infants (n = 26) requiring palliation with either a modified Blalock-Taussig shunt (MBT) or a central aortopulmonary shunt (CAP). Between 1995 and 2009, 69 consecutive patients underwent an MBT (n = 42) or CAP (n = 27) for tetralogy of Fallot (n = 21), pulmonary atresia (n = 25), severe pulmonary valve stenosis (n = 22), and 2-stage repair of transposition of the great arteries (n = 1). The groups were similar with regard to age, weight, pulmonary artery diameter, and preoperative saturations. Postoperative mortality was 3 after CAP (11.1%) versus 1 after MBT (2.4%; P = .0203). Shunt size/weight index was comparable for both groups. MBTs had shorter surgical times (P = .002), required less inotropes (inotropic index, 103 ± 18 vs 889 ± 199; P = .0069), less blood product transfusions (P = .01), and had shorter duration of ventilation (P = .026) and intensive care unit (ICU) stay (P = .042). Children with MBTs had higher saturations at hospital discharge (P = .018). Prior to complete repair, 2 patients with a CAP and 10 patients after an MBT needed pulmonary artery dilation or stent implantation (P = .23). At the time of complete repair and shunt takedown, 3 MBT patients needed surgical patch augmentation of the pulmonary artery. The MBT is a safer and more expeditious operation and more frequently avoids cardiopulmonary bypass. Patients require less inotropes, blood products, and ICU time but may require more interventional therapy to treat pulmonary artery stenosis in the interval to complete repair. Surgical treatment of shunt-related pulmonary artery distortion may be addressed at the time of complete repair.

Identification of 29 novel and nine recurrent fibrillin-1 (FBN1) mutations and genotype-phenotype correlations in 76 patients with Marfan syndrome.

Marfan syndrome (MFS) is an autosomal-dominant disorder of the fibrous connective tissue that is typically caused by mutations in the gene coding for fibrillin-1 (FBN1), a major component of extracellular microfibrils. The clinical spectrum of MFS is highly variable and includes involvement of the cardiovascular, skeletal, ocular, and other organ systems; however, the genotype-phenotype correlations have not been well developed. Various screening methods have led to the identification of about 600 different mutations (FBN1-UMD database; www.umd.be). In this study we performed SSCP and/or direct sequencing to analyze all 65 exons of the FBN1 gene in 116 patients presenting with classic MFS or related phenotypes. Twenty-nine novel and nine recurrent mutations were identified in 38 of the analyzed patients. The mutations comprised 18 missense (47%), eight nonsense (21%), and five splice site (13%) mutations. Seven further mutations (18%) resulted from deletion, insertion, or duplication events, six of which led to a frameshift and subsequent premature termination. Additionally, we describe new polymorphisms and sequence variants. On the basis of the data presented here and in a previous study, we were able to establish highly significant correlations between the FBN1 mutation type and the MFS phenotype in a group of 76 mutation-positive patients for whom comprehensive clinical data were available. Most strikingly, there was a significantly lower incidence of ectopia lentis in patients who carried a mutation that led to a premature termination codon (PTC) or a missense mutation without cysteine involvement in FBN1, as compared to patients whose mutations involved a cysteine substitution or splice site alteration.