Ten-year-old boy with congenital long QT syndrome type 2 (LQTS2) and life-threatening electrical storm: a case report of successful treatment with mexiletine.

We present a case of a boy with long QT syndrome type 2, who was admitted after an out of hospital cardiac arrest due to ventricular fibrillation. Subsequently, all treatments - intravenous magnesium, optimisation of electrolytes, an isoproterenol infusion - failed to terminate his electrical storm. As a last option before left-sided cardiac sympathetic denervation, mexiletine was started and the electrical storm resolved completely.

Invasive Bacterial and Fungal Infections After Pediatric Cardiac Surgery: A Single-center Experience.

BACKGROUND: Discrimination of infectious and noninfectious complications in children with inflammatory signs after cardiac surgery is challenging. Given the low prevalence of infectious complications after heart surgery, there might be a risk of excessive antibiotic usage. We performed this study to determine the rate of invasive bacterial or fungal infections in children after cardiac surgery at our institution and to evaluate our postoperative management. METHODS: This single-center retrospective observational cohort study included children 16 years of age or younger who underwent cardiac surgery at our institution between January 2012 and December 2015. RESULTS: We analyzed 395 surgical procedures. Thirty-five postoperative invasive bacterial or fungal infections were detected in 29 episodes (7%, 0.42 per 100 admission days). Among bacterial infections, the most common infection sites were bacteremia and pneumonia, accounting for 37% (13/35) and 23% (8/35) of infections respectively. The rate of postoperative infections was associated with surgical complexity score and length of postoperative pediatric intensive care unit (PICU) stay. In 154 (43%) of 357 episodes without microbiologically documented infection, uninterrupted postoperative antibiotic administration was continued for more than 3 days and in 80 (22%) for more than 5 days. CONCLUSIONS: The rate of postoperative bacterial or fungal infection at our institution is comparable to current literature. High surgical complexity score and prolonged length of PICU stay were risk factors for bacterial or fungal infections in this patient population.

Symptomatic Coronary Anomalies and Ischemia in Teenagers - Rare but Real.

Three cases of teenagers with anomalous aortic origin of the coronary arteries (AAOCA) are presented with typical exercise induced symptoms (chest pain, syncope or dizziness). Using multimodal imaging, diagnoses was confirmed showing interarterial and/or intramural course of the coronary artery explaining the ischemia induced symptoms. Successful surgical correction with unroofing of the AAOCA was performed in all three cases with a favorable outcome. Even though AAOCA are rare, some variants may be relevant and potentially life threatening, therefore treating physicians should be aware of correctly diagnosing and treating these individuals.

Risk Factors for Postprocedural Arterial Ischemic Stroke in Children With Cardiac Disease.

BACKGROUND AND PURPOSE: Cardiac pathologies are the second most frequent risk factor (RF) in children with arterial ischemic stroke (AIS). This study aimed to analyze RFs for AIS in children with cardiac disease and cardiac intervention. METHODS: Data were drawn from the Swiss Neuropediatric Stroke Registry. Patients with cardiac disease and postprocedural AIS registered from 2000 until 2015 were analyzed for the cause of cardiac disease and for potential RFs. RESULTS: Forty-seven out of 78 children with cardiac disease had a cardiac intervention. Of these, 36 presented a postprocedural AIS. Median time from cardiac intervention to symptom onset was 4 days (interquartile range, 2-8.5); time to diagnosis of AIS was 2 days (interquartile range, 0-5.8). Main RFs for postprocedural AIS were hypotension, prosthetic cardiac material, right-to-left shunt, arrhythmias, low cardiac output, and infections. CONCLUSIONS: In children with postprocedural AIS, time to diagnosis was delayed. Most patients presented multiple potentially modifiable RFs as hemodynamic alterations and infections.

Three-Dimensional Rotational Angiography during Catheterization of Congenital Heart Disease - A ten Years' experience at a single center.

This paper aims to assess the usability and advantages of three-dimensional rotational angiography (3DRA) in patients with congenital heart disease (CHD) and its application in the cath lab. Up to now, its use in CHD is not widespread or standardized. We analyzed all patients with CHD who underwent a 3DRA at our facility between January 2010 and May 2019. The 3DRAs were evaluated for radiation exposure, contrast dye consumption, diagnostic utility and image quality. We performed 872 3DRAs. 3DRA was used in 67.1% of the cases for interventional procedures and in 32.9% for diagnostic purposes. Two different acquisition programs were applied. The median dose-area product (DAP) for all 872 rotations was 54.1 µGym2 (21.7-147.5 µGym2) and 1.6 ml/kg (0.9-2.07 ml/kg) of contrast dye was used. Diagnostic utility of the generated 3D-model was rated superior to the native 3D angiography in 94% (819/872). 3DRA is an excellent and save diagnostic and interventional tool. However, 3DRA has not become a standard imaging procedure in pediatric cardiology up to now. Effort and advantage seems to be unbalanced, but new less invasive techniques may upgrade this method in future.

Parental emotional and hands-on work-Experiences of parents with a newborn undergoing congenital heart surgery: A qualitative study.

PURPOSE: To explore both mothers' and fathers' experiences from prenatal or postnatal diagnosis of their newborn's congenital heart disease (CHD) to the first discharge after heart surgery in a Swiss university children's hospital. DESIGN AND METHOD: A qualitative research approach, based on a constructivist paradigm, was applied to explore participants' experiences and perceptions. Parents of nine children with moderate to severe CHD participated in semistructured joint couple interviews. Data were analyzed inductively via an iterative process, following the steps of thematic analysis. RESULTS: Between the child's CHD diagnosis and hospital discharge after neonatal cardiac surgery, the overarching theme for parents was being confronted with demanding emotional and hands-on work. This parental work included four themes with subthemes: Parents had to tackle a route through an unknown hospital world from receiving the CHD diagnosis and experiencing delivery to attending to their child in the pediatric intensive care unit and during surgery, as well as during the transfer to and the stay on the pediatric cardiac unit. They experienced an at times challenging interplay with health care professionals, performed teamwork as the nuclear family and managed concerned relatives and friends. PRACTICE IMPLICATIONS: Health care professionals should establish trustful relationships with parents, while accompanying families continuously, providing consistent, straightforward information, and expressing appreciation for the parents' exceptional emotional and hands-on work. Health care professionals' awareness of parent's experiences is vital to compassionate family-centered care.

Late correction of tetralogy of Fallot in children.

AIM OF STUDY: To report our experience of late correction after infancy in patients with tetralogy of Fallot (ToF). METHODS: Observational single-centre retrospective analysis of the surgical techniques and perioperative development of patients from developing countries undergoing total surgical correction of ToF after infancy, between 1 November 2011 and 30 November 2016. Variables are presented as numbers with percentages or as mean ± standard deviation. Due to the setting of the humanitarian programme, clinical and echocardiographic follow-up procedures could be conducted for only one month postoperatively. RESULTS: Twenty-five children (mean age: 70.8 ± 42 months, range 23-163; 44% female) underwent total surgical correction of ToF. Two patients (0.8%) initially received a Blalock-Taussig shunt and underwent subsequent correction 24 and 108 months later, respectively. Preoperative mean right ventricular/pulmonary artery (RV/PA) gradient was 84 ± 32 mm Hg, with a Nakata index of 164 ± 71 mm2/m2. Major aortopulmonary collateral arteries (MAPCAs) were observed in eight children (32%), six (26%) of whom underwent transcatheter closure before surgery. 24 children (96%) underwent a valve-sparing pulmonary valve repair and one patient received a transannular patch (TAP). There were no cases which saw major adverse cardiac and cerebrovascular events (MACCE). Mean duration of mechanical ventilation was 28 ± 19.6 hours (range 7-76). Pre-discharge echocardiography demonstrated a mean RV/PA gradient of 25 ± 5.7 mm Hg, with left ventricular ejection fraction >60% in all cases. Overall length of hospital stay was 11.7 ± 4.5 days. There were no in-hospital mortality cases. CONCLUSIONS: Late surgical correction of ToF can be safely performed and produce highly satisfying early postoperative results comparable to those of classical “timely” correction. A valve-sparing technique can be applied in the majority of children.

Vertical Right Axillary Mini-Thoracotomy for Correction of Ventricular Septal Defects and Complete Atrioventricular Septal Defects.

BACKGROUND: Vertical right axillary mini-thoracotomy (VRAMT) is the standard approach for correction of atrial septal defect and partial atrioventricular septal defect at our institution. This observational single-center study compares our initial results with the VRAMT approach for the repair of ventricular septal defect (VSD) and complete atrioventricular septal defect (CAVSD) in infants and children to an approach using standard median sternotomy (MS). METHODS: The perioperative courses of patients undergoing VSD and CAVSD correction through either a VRAMT or an MS were analyzed retrospectively. The surgical technique for the VRAMT involved a 4- to 5-cm vertical incision in the right axillary fold. RESULTS: Of 84 patients, 25 (VSD, n = 15; CAVSD, n = 10) underwent correction through a VRAMT approach, whereas 59 (VSD, n = 35; CAVSD, n = 24) had repair through MS. VSD and CAVSD groups were comparable with respect to age and weight. No significant differences were observed for aortic cross-clamp duration, intensive care unit stay, hospital stay, and echocardiographic follow-up. There was no need for any conversion from VRAMT to MS in any case. Neither wound infections nor thoracic deformities were observed in both groups. CONCLUSIONS: VRAMT can be considered as a safe and effective approach for the repair of VSD and CAVSD in selected patient groups, and the outcome data appear comparable to those of MS.

Patterns of paediatric end-of-life care: a chart review across different care settings in Switzerland.

BACKGROUND: Paediatric end-of-life care is challenging and requires a high level of professional expertise. It is important that healthcare teams have a thorough understanding of paediatric subspecialties and related knowledge of disease-specific aspects of paediatric end-of-life care. The aim of this study was to comprehensively describe, explore and compare current practices in paediatric end-of-life care in four distinct diagnostic groups across healthcare settings including all relevant levels of healthcare providers in Switzerland. METHODS: In this nationwide retrospective chart review study, data from paediatric patients who died in the years 2011 or 2012 due to a cardiac, neurological or oncological condition, or during the neonatal period were collected in 13 hospitals, two long-term institutions and 10 community-based healthcare service providers throughout Switzerland. RESULTS: Ninety-three (62%) of the 149 reviewed patients died in intensive care units, 78 (84%) of them following withdrawal of life-sustaining treatment. Reliance on invasive medical interventions was prevalent, and the use of medication was high, with a median count of 12 different drugs during the last week of life. Patients experienced an average number of 6.42 symptoms. The prevalence of various types of symptoms differed significantly among the four diagnostic groups. Overall, our study patients stayed in the hospital for a median of six days during their last four weeks of life. Seventy-two patients (48%) stayed at home for at least one day and only half of those received community-based healthcare. CONCLUSIONS: The study provides a wide-ranging overview of current end-of-life care practices in a real-life setting of different healthcare providers. The inclusion of patients with all major diagnoses leading to disease- and prematurity-related childhood deaths, as well as comparisons across the diagnostic groups, provides additional insight and understanding for healthcare professionals. The provision of specialised palliative and end-of-life care services in Switzerland, including the capacity of community healthcare services, need to be expanded to meet the specific needs of seriously ill children and their families.

Challenges of congenital heart disease in grown-up patients.

Nowadays, more than 90% of all children born with congenital heart disease (CHD) reach adult life. Although initially considered to be cured, the majority of them continue to need specialised follow-up because they require re-do interventions or are at increased risk of cardiovascular complications and premature death. Arrhythmias are the most common cause of unscheduled hospital visits for grown-up CHD (GUCH) patients, accounting for one third of emergency admissions in these patients. Some GUCH patients are also at increased risk for sudden cardiac death. The principles of arrhythmia management and the prevention of sudden cardiac death in GUCH patients are similar to those used in adults with acquired heart disease, but are not evidence based. Decompensated heart failure is the other leading cause of death. Conventional medical heart-failure therapy for left ventricular dysfunction is not effective in GUCH patients at highest risk of heart failure, i.e., those with right or single ventricular failure. Careful haemodynamic assessment and structural interventions are the first step to consider in GUCH patients presenting with heart failure symptoms. Adults with moderate or complex CHD and regular follow-up in specialised GUCH centres have a survival benefit compared with patients without such follow-up. Cardiac surgery in GUCH patients should be performed by surgeons trained in treatment of CHD, i.e., surgeons also operating on paediatric patients. A structured transition programme with a defined transfer of care from the paediatric to the adult care environment is important to avoid lapses of care in today's adolescents with CHD. For GUCH patients with an intervention performed decades ago and no specific cardiac follow-up in later life, referral to a specialised GUCH centre is recommended and may save lives.

Approaching atrial septal defects in pulmonary hypertension.

Atrial septal defects (ASDs) are one of the most frequent congenital cardiac malformations, accounting for about 8-10% of all congenital heart defects. The prevalence of pulmonary arterial hypertension (PAH) in adults with an ASD is 8-10%. Different clinical PAH scenarios can be encountered. At one end of the spectrum are adults with no or only mild pulmonary vascular disease and a large shunt. These are patients who can safely undergo shunt closure. In the elderly, mild residual pulmonary hypertension after shunt closure is the rule. At the other end of the spectrum are adults with severe, irreversible pulmonary vascular disease, shunt reversal and chronic cyanosis, that is, Eisenmenger syndrome. These are patients who need to be managed medically. The challenge is to properly classify ASD patients with PAH falling in between the two ends of the spectrum as the ones with advanced, but reversible pulmonary vascular disease amenable to repair, versus the ones with progressive pulmonary vascular disease not responding to shunt closure. There are concerns that adults with progressive pulmonary vascular disease have worse outcomes after shunt closure than patients not undergoing shunt closure. Due to the correlation of pulmonary vascular changes and pulmonary hemodynamics, cardiac catheterization is used in the decision-making process. It is important to consider the hemodynamic data in the context of the clinical picture, the defect anatomy and further noninvasive tests when evaluating the option of shunt closure in these patients.

Safety, feasibility, and long-term results of percutaneous closure of atrial septal defects using the Amplatzer septal occluder without periprocedural echocardiography.

OBJECTIVES: We sought to assess the safety and efficacy of percutaneous closure of atrial septal defects (ASDs) under fluoroscopic guidance only, without periprocedural echocardiographic guidance. BACKGROUND: Percutaneous closure of ASDs is usually performed using simultaneous fluoroscopic and transthoracic, transesophageal (TEE), or intracardiac echocardiographic (ICE) guidance. However, TEE requires deep sedation or general anesthesia, which considerably lengthens the procedure. TEE and ICE increase costs. METHODS: Between 1997 and 2008, a total of 217 consecutive patients (age, 38 ± 22 years; 155 females and 62 males), of whom 44 were children ≤16 years, underwent percutaneous ASD closure with an Amplatzer ASD occluder (AASDO). TEE guidance and general anesthesia were restricted to the children, while devices were implanted under fluoroscopic guidance only in the adults. For comparison of technical safety and feasibility of the procedure without echocardiographic guidance, the children served as a control group. RESULTS: The implantation procedure was successful in all but 3 patients (1 child and 2 adults; 1.4%). Mean device size was 23 ± 8 mm (range, 4-40 mm). There was 1 postprocedural complication (0.5%; transient perimyocarditis in an adult patient). At last echocardiographic follow-up, 13 ± 23 months after the procedure, 90% of patients had no residual shunt, whereas a minimal, moderate, or large shunt persisted in 7%, 1%, and 2%, respectively. Four adult patients (2%) underwent implantation of a second device for a residual shunt. During a mean follow-up period of 3 ± 2 years, 2 deaths and 1 ischemic stroke occurred. CONCLUSION: According to these results, percutaneous ASD closure using the AASDO without periprocedural echocardiographic guidance seems safe and feasible.

Dimensions of the ascending aorta in conotruncal heart defects.

Dilatation of the ascending aorta is an important sequel in conotruncal anomalies, such as tetralogy of Fallot (TOF) or d-transposition of the great arteries (TGA). We measured dimensions and their progression at different levels of the ascending aorta in 80 patients. In TOF patients, mean z-score for aortic annulus was 1.65 (range -3.16-6.47), for sinus 1.93 (range -2.28-5.39), for st-junction 4.15 (range 0.0-8.18), and for ascending aorta 3.51 (range -1.23-6.36). Over time, annulus z-scores increased in the univariate analysis [0.07/year, 95 % confidence interval (CI) 0.01-0.14; p = 0.02], and this was unique to male patients (0.08/year, 95 % CI 0.00-0.15; p = 0.05). z-scores of the ascending aorta decreased (-0.1/year, 95 % CI -0.18 to -0.02; p = 0.02), and this was confined to patients without aortic regurgitation (AR; -0.09/year, 95 % CI -0.18 to -0.01; p = 0.04). In TGA, mean z-score for the aortic annulus was 2.13 (range -3.71-8.39), for sinus 1.77 (range -3.04-6.69), for st-junction 1.01 (range -5.44-6.71), and for ascending aorta 0.82 (range -4.91-6.46). In bivariate analysis, annulus z-scores decreased in females (-0.14/year, 95 % CI -0.25 to -0.03; p = 0.01) and in patients without AR (-0.07/year, 95 % CI -0.14-0.0; p = 0.03). z-scores of the ascending aorta increased significantly in males (0.08/year, 95 % CI 0.0 to 0.16; p = 0.05) and in patients with AR (0.12/year, 95 % CI 0.03-0.21; p = 0.01). In conclusion, TOF and TGA z-scores of the ascending aorta differ significantly from those of the normal population. Progression of z-scores over time is influenced by diagnosis, sex, and presence of AR.

Persistent left superior vena cava in cardiac congenital surgery.

Persistent left superior vena cava (LSVC) is a relatively frequent finding in congenital cardiac malformation. The scope of the study was to analyze the timing of diagnosis of persistent LSVC, the timing of diagnosis of associated anomalies of the coronary sinus, and the global impact on morbidity and mortality of persistent LSVC in children with congenital heart disease after cardiac surgery. Retrospective analysis of a cohort of children after cardiac surgery on bypass for congenital heart disease. Three hundred seventy-one patients were included in the study, and their median age was 2.75 years (IQR 0.65-6.63). Forty-seven children had persistent LSVC (12.7 %), and persistent LSVC was identified on echocardiography before surgery in 39 patients (83 %). In three patients (6.4 %) with persistent LSVC, significant inflow obstruction of the left ventricle developed after surgery leading to low output syndrome or secondary pulmonary hypertension. In eight patients (17 %), persistent LSVC was associated with a partially or completely unroofed coronary sinus and in two cases (4 %) with coronary sinus ostial atresia. Duration of mechanical ventilation was significantly shorter in the control group (1.2 vs. 3.0 days, p = 0.04), whereas length of stay in intensive care did not differ. Mortality was also significantly lower in the control group (2.5 vs. 10.6 %, p = 0.004). The results of study show that persistent LSVC in association with congenital cardiac malformation increases the risk of mortality in children with cardiac surgery on cardiopulmonary bypass. Recognition of a persistent LSVC and its associated anomalies is mandatory to avoid complications during or after cardiac surgery.

Pharmacological and non-pharmacological therapy for arrhythmias in the pediatric population: EHRA and AEPC-Arrhythmia Working Group joint consensus statement.

In children with structurally normal hearts, the mechanisms of arrhythmias are usually the same as in the adult patient. Some arrhythmias are particularly associated with young age and very rarely seen in adult patients. Arrhythmias in structural heart disease may be associated either with the underlying abnormality or result from surgical intervention. Chronic haemodynamic stress of congenital heart disease (CHD) might create an electrophysiological and anatomic substrate highly favourable for re-entrant arrhythmias. As a general rule, prescription of antiarrhythmic drugs requires a clear diagnosis with electrocardiographic documentation of a given arrhythmia. Risk-benefit analysis of drug therapy should be considered when facing an arrhythmia in a child. Prophylactic antiarrhythmic drug therapy is given only to protect the child from recurrent supraventricular tachycardia during this time span until the disease will eventually cease spontaneously. In the last decades, radiofrequency catheter ablation is progressively used as curative therapy for tachyarrhythmias in children and patients with or without CHD. Even in young children, procedures can be performed with high success rates and low complication rates as shown by several retrospective and prospective paediatric multi-centre studies. Three-dimensional mapping and non-fluoroscopic navigation techniques and enhanced catheter technology have further improved safety and efficacy even in CHD patients with complex arrhythmias. During last decades, cardiac devices (pacemakers and implantable cardiac defibrillator) have developed rapidly. The pacing generator size has diminished and the pacing leads have become progressively thinner. These developments have made application of cardiac pacing in children easier although no dedicated paediatric pacing systems exist.

Distensibility and diameter of ascending aorta assessed by cardiac magnetic resonance imaging in adults with tetralogy of fallot or complete transposition.

Structural abnormalities of the medial aorta have been described for conotruncal defects (e.g., tetralogy of Fallot [TOF] and complete transposition of the great arteries (dextrotransposition [d]-TGA). In TOF, progressive aortic dilation is a frequent finding. In patients with d-TGA with an atrial switch, this problem is less often described. The aim of the present study was to compare the extent of dilative aortopathy and aortic distensibility in adults with an atrial switch procedure (n = 39) to that in adults with repaired TOF (n = 39) and controls (n = 39), using cardiac magnetic resonance imaging. The groups were matched for age and gender. Diameters of the aorta indexed to the body surface area were significantly increased in the patients with d-TGA and TOF compared to that of the controls at the aortic sinus up to the level of the right pulmonary artery. On multivariate testing, the diagnosis of a conotruncal defect (ß = 0.260; p = 0.003) and aortic regurgitant fraction (ß = 0.405; p <0.001) were independent predictors of an increased aortic sinus diameter. Ascending aorta distensibility was significantly reduced in those with d-TGA and TOF compared to controls: 3.6 (interquartile range 1.5 to 4.4) versus 2.8 (interquartile range 2.0 to 3.7) versus 5.5 (interquartile range 4.8 to 6.9) ×10(-3) mm Hg(-1) (p <0.001). The independent predictors of ascending aorta distensibility were the diagnosis of a conotruncal defect (p <0.001) and age (p = 0.028). In conclusion, intrinsic aortopathy, manifested as increased ascending aortic diameters and reduced ascending aortic distensibility, is not only evident in adults with TOF, but also in adults with d-TGA and an atrial switch procedure. Long-term follow-up is needed to monitor the aortic size in both patient groups.