Wolf-Parkinson-White Syndrome: Diagnosis, Risk Assessment, and Therapy-An Update.

Wolf-Parkinson-White (WPW) syndrome is a disorder characterized by the presence of at least one accessory pathway (AP) that can predispose people to atrial/ventricular tachyarrhythmias and even sudden cardiac death. It is the second most common cause of paroxysmal supraventricular tachycardia in most parts of the world, affecting about 0.1-0.3% of the general population. Most patients with WPW syndrome have normal anatomy, but it may be associated with concomitant congenital heart disease or systemic diseases. Although many individuals are asymptomatic, during supraventricular arrhythmia episodes, they may experience severe symptoms, including syncope or even sudden cardiac death (mainly due to pre-excited atrial fibrillation over rapidly conducting AP). In addition to arrhythmia-related symptoms, for some specific locations of the APs with overt anterograde conduction, there might be a reduction in exercise capacity mediated by a reduction in LV systolic performance due to anomalous LV depolarization. Although it is typically diagnosed through electrocardiography (ECG), additional tests are necessary for risk assessment. Management of WPW syndrome may be quite challenging and can vary from only acknowledging the presence of the accessory pathway to pharmacological treatment or radiofrequency ablation. Early diagnosis, risk assessment, and appropriate treatment are critical steps in the management of WPW syndrome, aiming to improve the quality of life and reduce the risk of life-threatening arrhythmias.

Nanotechnology Innovations in Pediatric Cardiology and Cardiovascular Medicine: A Comprehensive Review.

(1) Background: Nanomedicine, incorporating various nanoparticles and nanomaterials, offers significant potential in medical practice. Its clinical adoption, however, faces challenges like safety concerns, regulatory hurdles, and biocompatibility issues. Despite these, recent advancements have led to the approval of many nanotechnology-based products, including those for pediatric use. (2) Methods: Our approach included reviewing clinical, preclinical, and animal studies, as well as literature reviews from the past two decades and ongoing trials. (3) Results: Nanotechnology has introduced innovative solutions in cardiovascular care, particularly in managing myocardial ischemia. Key developments include drug-eluting stents, nitric oxide-releasing coatings, and the use of magnetic nanoparticles in cardiomyocyte transplantation. These advancements are pivotal for early detection and treatment. In cardiovascular imaging, nanotechnology enables noninvasive assessments. In pediatric cardiology, it holds promise in assisting the development of biological conduits, synthetic valves, and bioartificial grafts for congenital heart defects, and offers new treatments for conditions like dilated cardiomyopathy and pulmonary hypertension. (4) Conclusions: Nanomedicine presents groundbreaking solutions for cardiovascular diseases in both adults and children. It has the potential to transform cardiac care, from enhancing myocardial ischemia treatment and imaging techniques to addressing congenital heart issues. Further research and guideline development are crucial for optimizing its clinical application and revolutionizing patient care.

Applicability of the PedsQLTM 4.0 Generic and Fatigue Modules in Romanian Children with Inflammatory Bowel Disease: Pilot Study.

The Pediatric Quality of Life (PedsQL) Inventory is a tool used to measure Health Related Quality of Life (HRQoL) in children aged 2 to 18 years. The aim of the present study was to investigate the feasibility and reliability of the Romanian version of two PedsQL modules, the Generic Core and the Multidimensional Fatigue Scales, in children with inflammatory bowel disease (IBD). Children diagnosed with IBD in our clinic and their parents completed a total of 26 Romanian version PedsQL forms, while a control group of healthy children and their caregiver filled in 86 identical online forms. We compared total and dimensional scores between controls and subjects, along with age, sex, and active versus inactive disease differences. The results indicated that the PedsQL total and summary scores differentiated between subjects and controls, with lower HRQoL and higher levels of fatigue being reported in children with chronic IBD. The Romanian version of the PedsQL was found to be feasible and reliable, with good internal consistency higher than 0.70 and minimum missing responses. However, the limited number of participants meant that clinical activity severity indices correlated poorly with fatigue and generic scores. Further validation of these models requires larger, multi-centric studies.

Postoperative Cardiac Arrhythmias in Pediatric and Neonatal Patients with Congenital Heart Disease-A Narrative Review.

Cardiac arrhythmias are a frequent complication in the evolution of patients with congenital heart disease. Corrective surgery for these malformations is an additional predisposition to the appearance of arrhythmias. Several factors related to the patient, as well as to the therapeutic management, are involved in the etiopathogenesis of cardiac arrhythmias occurring post-operatively. The risk of arrhythmias in the immediate postoperative period is correlated with the patient's young age and low weight at surgery. The change in heart geometry, hemodynamic stress, and post-surgical scars represent the main etiopathogenic factors that can contribute to the occurrence of cardiac arrhythmias in the population of patients with operated-on congenital heart malformations. Clinical manifestations differ depending on the duration of the arrhythmia, underlying structural defects, hemodynamic conditions, and comorbidities. The accurate diagnosis and the establishment of specific management options strongly influence the morbidity and mortality associated with arrhythmias. As such, identifying the risk factors for the occurrence of cardiac arrhythmias in the case of each patient is essential to establish a specific follow-up and management plan to improve the life expectancy and quality of life of children.

Isolated hypoplastic right ventricle - a challenge in medical practice.

Isolated right ventricle hypoplasia (IRVH) is a disease characterized by an underdeveloped right ventricle. It is a congenital heart disease than can associate heterogeneous structural defects and nonspecific clinical features, which can often present a challenging therapeutic management. In this article, there are presented diagnostic methods and treatment options for right ventricle hypoplasia (RVH) according to clinical features, patients age and associated structural heart defects. RVH has a different prognosis in accordance with the severity of the heart defects and the patient's age at which the diagnosis is established. Thus, isolated forms of RVH generally present mild structural and functional defects that can be associated with the onset of symptoms in adolescence or even in adulthood. In these cases, atrial septal defect closure with or without superior cavo-pulmonary anastomosis can be the only procedures needed to correct the hemodynamic abnormalities and relief the symptomatology. Patients with severe form of RVH associated with complex cardiac malformations and onset of the symptoms in the neonatal period require prompt intervention and necessitate palliative procedures. In the long term, these patients could need multiple reinterventions. The family physician should be aware of the cardiac origin of isolated symptoms or clinical signs, such as exertional dyspnea or clubbing fingers, and send the patient for pediatric cardiological evaluation.

Cocoon devices for transcatheter closure of atrial septal defect and patent ductus arteriosus in children: Single center experience.

Closure of the atrial septal defect (ASD) and patent ductus arteriosus (PDA) are among the most frequent cardiac interventional procedures. This was a prospective study, which started together with the implementation of a national program of pediatric interventional cardiology in Romania. We used Cocoon devices in 83 consecutive cases from 92 implantations for ASD and PDA. 27 cases were ASD closure and 56 cases PDA closure. Regarding the ASD closure, the median age was 8.5 years (range 3-25 years) and median weight 25 kg (range 11.5-63 kg). The mean follow-up was 17.4 ±â€Š6.7 months (range 3-26 months). The mean ASD diameter by transesophageal echocardiography was 15.2 ±â€Š4.1 mm (range 8-26 mm). The mean device diameter used was 17.3 ±â€Š5.6 mm (range 8-32 mm). Regarding the PDA closure, the median age was 36 months (range 4-192 months) and median weight 14 kg (range 5-58 kg). The mean follow-up was 15 ±â€Š8 months (range 3-28 months). The mean PDA minimum diameter was 2.5 ±â€Š0.8 mm. The success implantation rate for both groups was 97.6% (2 cases of withdrawn for ASD and PDA), while the complication rate was 2.3% (including 2 ASD device embolization). In the first 24 hours, the closure rates were 96.3% for ASD, 98.2% for PDA, and 100% at 1-month follow-up for both procedures. On short and intermediate follow-up (3-28 months), no device-related complications were noted.The Cocoon devices are safe for transcatheter closure of both ASD and PDA, and the initial experience with their use in our emerging center is encouraging.

Unroofed coronary sinus: update on diagnosis and treatment.

Unroofed coronary sinus (UCS) is among the rarest congenital heart malformations in pediatric practice. Often, they are associated with persistent left superior vena cava (PLSVC) draining into the left atrium, and in this situation, there is a double intracardiac shunt, right-to-left and left-to-right. They are asymptomatic in the majority of the cases. Each ordinary imagistic method (transthoracic echocardiography, contrast transesophageal echocardiography, angiography) can be helpful in finding the diagnosis, but enhanced computed tomography scan can give an irrefutable diagnosis. The surgical intervention may consist in the recreation of a roof for the coronary sinus, and anastomosis of the PLSVC to the right atrial appendage.

Complex atrial septal defect closure in children.

Atrial septal defect (ASD) is one of the most common congenital heart diseases (CHDs) diagnosed in children. Symptoms in ASD may be absent, but palpitations and dyspnea in children sometimes reveal a complex structural and CHD. Diagnosis is established usually by transthoracic echocardiography, but in more complex cases such as coronary sinus defect, enhanced computed tomography or cardiac magnetic resonance may be used. Indication for closure depends on the dimension and on the hemodynamical impact. There are two methods for closure: surgical and interventional. Surgery may be applied to all types of ASD, while transcatheter closure may be used only for ostium secundum ASD. The most important issue in the interventional closure is the anatomical aspect related to the position of the defect. Both methods may have complications. Complex cases in children usually are not recommended for interventional closure, surgery being the recommended method. Oversizing of the device to be implanted according to the sizing balloon and not to the initial defect diameter may give a solution for large ASDs. Interventional closure of large defects in small children with an aneurysmal, multi-fenestrated, malaligned septum, or with other CHD associated may be challenging. Complications are present for both types of closure, but they are relatively different.

Scimitar syndrome associated with aberrant right subclavian artery, diaphragmatic hernia, and urinary anomalies - case report and review of the literature.

Scimitar syndrome is a form of a partially or totally right pulmonary venous return to the inferior vena cava, which may associate variably right lung hypoplasia, right pulmonary artery hypoplasia, pulmonary sequestration together with the presence of aortopulmonary collaterals from the descending aorta towards the right lung. In many cases, there are also other cardiac anomalies associated. We present a unique association of a partially anomalous pulmonary venous return to the inferior vena cava with other vascular and thoracic anomalies: inferior sinus venosus and secundum atrial septal defect, retroesophageal right subclavian artery, obstructed accessory right bronchus, diaphragmatic hernia with ectopic liver, "S"-type thoracic scoliosis and malformations of the urinary tract (duplication of the right ureter and of the left basinet). The patient had a reimplantation of the "scimitar" vein to the left atrium and closure of the inferior sinus venosus and secundum atrial septal defect.

Complex ventricular septal defects. Update on percutaneous closure.

Ventricular septal defects (VSDs) are the most common congenital heart diseases. Sometimes they can be complex because of anatomy, age of the patients or associated diseases. Surgery has been performed for many years and is considered as the gold standard for the treatment of VSD. However, it is associated with morbidity and mortality. Less invasive techniques have been developed in last 15 years. Two types of devices from the Amplatzer family are currently used to close percutaneously muscular and perimembranous VSD. METHODS: Comparative data and technical aspects of percutaneous closure in complex congenital defects (muscular and perimembranous VSDs) and in acquired defects (residual post-surgery, traumatic and postinfarction) are presented and discussed in this review. Hybrid approach to VSD closure is another complex situation and it is presented, too. RESULTS: Successful closure is obtained in around 95% with a rate of major complication of 5.3% for muscular VSD. For the perimembranous VSD, the complete and successful closure is reported in 97.5% of patients, while major acute complications occur in 1.2%. Occurrence of complete atrioventricular block is reported in 1.6% of subjects. Acquired VSD can occur as post-surgical residual leak, traumatic or postinfarction VSD. Procedures are usually complex and different techniques should be used. CONCLUSIONS: Percutaneous closure of complex VSDs is possible, safe and effective procedure in highly specialized centers. Appropriate patient selection is of paramount importance to the success of the procedure.

Partial abnormal drainage of superior and inferior caval veins into the left atrium: two case reports.

Abnormal connection of the right superior caval vein to the left atrium is an uncommon systemic vein drainage anomaly, with only a few cases reported among congenital heart disease (CHD), around 20 cases published in the medical literature. The inferior vena cava connection with the left atrium, also very rare, can appear directly or in heterotaxy. Clinical suspicion arises due to the presence of cyanosis in the absence of other specific clinical signs (without other associated CHD). We present the cases of two children with abnormal superior and inferior systemic venous return. The first case is an abnormal connection of right superior vena cava to the left atrium associated with persistent left superior vena cava draining into the right atrium through the coronary sinus. The second case is an interruption of the inferior vena cava with hemiazygos continuation, drained into the left superior vena cava, which drained into the left atrium. The diagnosis was imagistic - echocardiography and angiography. Surgical treatment solutions vary from one case to another, usually following anatomic correction. Hypoxia accompanied by cyanosis must bring into question the pathology of systemic venous drainage anomaly, after other common causes have been excluded. Surgery is indicated in all cases due to the risk associated with the presence of right-to-left shunt.

Ultrasound Prenatal Diagnosis and Emergency Interventional Radiologic Therapy of Galen Aneurysmal Malformation in a Newborn.

Located under the cerebral hemispheres and draining the anterior and central regions of the brain into the sinus of the posterior cerebral fossa, the vein of Galen aneurysmal malformation is considered to be a rare cause of hydrocephaly. The presence of this condition in the neonatal period typically includes intractable heart failure and a poor prognosis. We report a case of aneurysm of the vein of Galen diagnosed prenatally at 28 weeks of gestation, with the delivery at term by caesarean section of a female infant. Sonographically, the vein of Galen appeared in the mid-sagittal plane, large, supratentorial, non-pulsatile; on color Doppler, the structure filled with bright color, reflecting a turbulent venous flow. A low grade of ventriculomegaly was present during the evolution of pregnancy; regarding the cardiovascular function, an intrauterine right cardiac insufficiency overlapped a tricuspid regurgitation and right atrial dilatation. A multidisciplinary committee decided a neonatal embolization of the aneurysm as an emergency requirement due to increased pulmonary hypertension developed in the next 24 hours after birth. After the embolization of the two main drainage vessels, the cardiac dysfunction persists. Two days later the evolution became unfavorable, leading to the necessity of the second embolization, which resulted in a 48 hours' coma and death, due to a cerebral hemorrhage secondary to thrombosis and fissure of the embolized aneurysm. The prognosis for the neonate with malformation of the Galen vein depends upon the severity of the cardiovascular status. Embolization represents actually the treatment of choice with the best results of these cases, but the mortality remains as high as 50 percent even in the most specialized centers of the world. As far as we know this is the only case of Galen aneurysmal malformation in Romania which beneficiated of embolization by interventional treatment in neonatal period.