Clinical and Immunologic Features of a Patient With Homozygous FNIP1 Variant.

Agammaglobulinemia represents the most profound primary antibody deficiency, stemming from early cessation of B-cell development. Deficiency in folliculin-interacting protein 1 (FNIP1) is a novel inborn error of immunity characterized by a severe defect in B-cell development, agammaglobulinemia, variable neutropenia, and hypertrophic cardiomyopathy. FNIP1 plays a critical role in B-cell development and metabolic homeostasis, establishing a metabolic checkpoint that ensures pre-B cells possess sufficient metabolic capacity to undergo division while concurrently limiting lymphogenesis due to abnormal growth. Disruption of FNIP1 functionality affects the fundamental metabolic regulators adenosine monophosphate-activated protein kinase and mTOR, culminating in a severe B-cell deficiency alongside hypogammaglobulinemia, hypertrophic cardiomyopathy, preexcitation syndrome, and intermittent neutropenia. This case report presents an 11-month-old male patient with FNIP1 deficiency who, in addition to classical features, exhibited posterior cerebellar hypoplasia.

Impact of high-flow nasal oxygen therapy on postoperative atelectasis and reintubation rate after paediatric cardiac surgery.

INTRODUCTION: Airway problems emerging after congenital cardiac surgery operations may have an impact on mortality and morbidity. Recently, to improve alveolar gas exchange and reduce respiratory effort, high-flow nasal cannula (HFNC) has started to be used in paediatric cases. This study aimed to evaluate the potential effects of high-flow nasal oxygen therapy on postoperative atelectasis development and reintubation rate in paediatric cardiac surgery patients. METHODS: This study was conducted retrospectively in term newborns and infants younger than six months of age who underwent congenital cardiac surgery operation from 1 November 2022 to 1 November 2023 and were followed in the paediatric cardiac ICU. Patients who were receiving mechanical ventilator support at least 12 hours postoperatively were evaluated for the development of postoperative atelectasis and reintubation in the first 3 days of extubation. The patients were grouped as HFNC and non-HFNC users. Demographic characteristics, surgery type, and ICU clinical follow-up data were obtained from medical records. The results were statistically evaluated. RESULTS: A total of 40 patients who did not use HFNC in the early postoperative period and 40 patients with HFNC in the late period during the study period were included in the study. The median age was 1 month (IQR 15 days-2 months) with equal gender distribution. Among patients, 70% of them were in the neonatal age group. Reintubation rates in the first 72 hours in HFNC users and non-HFNC users were 2.5% and 12.5%, respectively (p < 0.05). The median postoperative atelectasis scores at 24, 48, and 72 hours of extubation were 2 versus 2.5 (p > 0.05), 1.5 versus 3.5 (p < 0.05), and 1 versus 3 (p < 0.05) in HFNC users and non-HFNC users, respectively. CONCLUSION: HFNC therapy may have a positive effect on preventing atelectasis and reducing the reintubation rate in the early postoperative period.

Assessment of the frequency and risk factors of gastrointestinal bleeding after cardiopulmonary bypass in paediatric cases.

INTRODUCTION AND AIM: Gastrointestinal bleeding is a potential complication in paediatric patients undergoing cardiopulmonary bypass, as it develops secondary to low gastrointestinal perfusion. This study aimed to examine the incidence of gastrointestinal bleeding and identify its risk factors in these patients. METHOD: This retrospective study was undertaken to examine the demographic features, clinical findings, and operative data of paediatric patients under years old who had undergone congenital heart surgery with cardiopulmonary bypass between November 1, 2021, and November 1, 2023. The study aimed to investigate the incidence of gastrointestinal bleeding associated with cardiopulmonary bypass and to identify potential risk factors for gastrointestinal bleeding. The obtained results were statistically evaluated. RESULTS: The study period included 1100 patients who underwent congenital heart surgery with cardiopulmonary bypass. Fifty-two percent of the total participants were male. The median weight of the patients was 4.4 kg, with an interquartile range of 3.5-5.8 kg. The patients were categorised by age, revealing that 62% were newborns, 24% were infants, and 14% were children. Forty-four (4.2%) of the total number of patients experienced gastrointestinal bleeding. Newborns had a significantly higher incidence of bleeding (6% or 34 patients) compared to infants (3% or 8 patients) and children (1.5% or 2 patients) (p < 0.05). Patients who experienced gastrointestinal bleeding had a longer median hospital stay of 24 days compared to those who did not, with a median hospital stay of 14 days. Moreover, patients who suffered from bleeding had a significantly higher mortality rate (30%) in comparison to those who did not (9.9%) (p < 0.05). The incidence of gastrointestinal bleeding was found to be associated with several risk factors, such as low operative age and weight, high surgical score, presence of low cardiac output syndrome, extracorporeal membrane oxygenation (ECMO) usage, high lactate levels, and low platelet count. CONCLUSION: Gastrointestinal bleeding is a potential complication for patients who undergo cardiopulmonary bypass. It is particularly relevant for newborns who have undergone prolonged surgery, have a high surgical complexity score, exhibit high lactate levels, display low cardiac output, utilise ECMO, and possess low platelet counts. In such cases, there may be a heightened incidence of gastrointestinal bleeding. It is important to consider this possibility in order to ensure the best possible patient outcomes.

A rare ventriculoarterial connection: double outlet of both ventricles.

Ventriculoarterial connection is one of the important points of the segmental approach to congenital cardiac malformations. Double outlet of both ventricles is a rare form where both great arterial roots override the interventricular septum. In this article, we aimed to draw attention to this very rare form of ventriculoarterial connection by presenting an infant case diagnosed using echocardiography, CT angiography, and 3-dimensional modelling.

Radiofrequency ablation of fast ventricular tachycardia causing an ICD storm in an infant with hypertrophic cardiomyopathy.

An implantable cardioverter defibrillator (ICD) storm involves very frequent arrhythmia episodes and ICD shocks, and it is associated with poor short-term and long-term prognosis. Radiofrequency catheter ablation can be used as an effective rescue treatment for patients with an ICD storm. To our knowledge, this is the first report of an infant with hypertrophic cardiomyopathy presenting with an ICD storm and undergoing successful radiofrequency catheter ablation salvage treatment for the fast left posterior fascicular ventricular tachycardia.

Junctional ectopic tachycardia in late period after early postoperative complete atrioventricular block: Messenger of return to normal sinus rhythm? : Explanation with four case series.

Junctional ectopic tachycardia(JET) is a rare childhood arrhythmia originating from the area adjacent to the atrioventricular(AV) node. It often occurs after surgical procedures like repair of Tetralogy of Fallot, atrioventricular septal defect and ventricular septal defect, which are all performed in that area. While AV block (AVB) can occur after JET, it is very rare for late JET occurring after early postoperative AVB to be followed by normal sinus rhythm (NSR). There is no information in the literature related to the pathophysiology of this phenomenon. In this text, we present 4 patients who developed complete AV block(CAVB) in the early postoperative period (within the first 24h) after JET in late period (>72h) and returned to NSR with first-degree AV block and then NSR during follow-up. Based on these cases, we hypothesize that there is a link between late JET after early postoperative CAVB and return to NSR.

Evaluation of Permanent or Transient Complete Heart Block after Open Heart Surgery for Congenital Heart Disease.

BACKGROUND: The features of pediatric patients with postoperative transient and permanent complete atrioventricular (AV) block (CAVB) were compared. METHODS: Patients who developed CAVB in postoperative period after congenital cardiac surgery between 2010-2015 were included in the study. They were classified as patients with transient CAVB and with permanent CAVB. The demographics and perioperative and postoperative variables of the groups were evaluated. RESULTS: A total of 1,550 patients underwent surgery during the study period. CAVB was determined in 96 patients (6.2%) in the early postoperative period: 66 had transient CAVB, 30 had permanent CAVB that necessitated pacemaker implantation. The median body weight and age at surgery were similar in both groups. The most frequent diagnosis was tetralogy of Fallot (TOF, n = 22), complete AV septal defect (AVSD, n = 15), and ventricular septal defect (n = 13). Junctional ectopic tachycardia (JET) developed in 27 patients with transient CAVB and in four with permanent CAVB (P < 0.05). There were no significant differences in the congenital cardiac pathology, the cardiopulmonary bypass time, cross-clamp time, and the presence of preoperative arrhythmia between the groups (P > 0.05). The duration of intensive care unit stay was 6 days (range 2-25) for patients with transient CAVB and 13 days (range 4-90) for patients with permanent CAVB. The duration of hospital stay was 10 days (range 2-33) for patients with transient CAVB and 20 days (range 10-90) for patients with permanent CAVB. Both were significantly longer in the patients with permanent CAVB. CONCLUSIONS: Complete AVSD and TOF are the most risky operations for the development of postoperative AV block. Ninety-seven percent of the patients with transient CAVB regained AV conduction within the first 10 postoperative days. The high incidence of JET in patients with transient CAVB was striking.

Using a Cardiac Event Recorder in Children with Potentially Arrhythmia-Related Symptoms.

BACKGROUND: In this study, we reported our experience with the use of cardiac event recorders in pediatric patients. METHODS: We evaluated 583 patients fitted with an event recorder (15-30 days) between March 2010 and November 2014 at our clinic. Excluded from the study were 117 patients with no recorded events and six with records contaminated by electrocardiogram artifacts. All of the patients received electrocardiograms, Holter monitoring, and echocardiography before the cardiac event recording. RESULTS: The patient sample consisted of 460 patients (64% female). The mean age was 12.8 ± 4.1 years. The median number of recorded events was 7. The indications included palpitations in 336 (73%) patients, syncope in 27 (6%) patients, and chest pain and palpitations in 97 (21%) patients. Whereas 64 patients (14%) had structural heart disease according to echocardiographic examination, the remaining patients had normal echocardiographic examination results. The most frequent cardiac comorbidities were mitral valve prolapse (6%), operated tetralogy of Fallot (1.5%), and complicated congenital heart diseases with single ventricle physiology (1%). The recorded events were sinus tachycardia in 113 (25%) patients, supraventricular tachycardia in 35 (8%) patients, ventricular extrasystole in 20 (4%) patients, supraventricular extrasystole in nine (2%) patients, and ventricular tachycardia in two (0.4%) patients. Based on the event recorder and follow-up electrocardiogram findings, 46 patients received an electrophysiology study/ablation. The symptom-rhythm correlation was 39%. CONCLUSION: In the presence of possible arrhythmia-related symptoms in children, a cardiac event recorder can be considered a useful primary diagnostic method. More research on this topic is needed.

Electrocardiographic and electrophysiologic effects of dexmedetomidine on children.

BACKGROUND: Dexmedetomidine (DEX) is a highly selective alpha-2-adrenergic agonist approved for short-term sedation and monitored anesthesia care in adults. Its effects on the electrocardiography and cardiac conduction tissue are not well described in the literature. Therefore, we aimed to characterize the electrocardiographic and electrophysiologic effects of DEX in children. METHODS: Twenty children (11 boys and nine girls) between the ages of eight and 17 undergoing electrophysiology study and ablation of the supraventricular tachycardia had hemodynamic and cardiac electrophysiologic variables measured before and during the administration of DEX (1 microgram/kg IV over 10 minutes followed by a 10-minute continuous infusion of 0.5 microgram/kg/h). RESULTS: A significant decrease in heart rate was seen after the administration of DEX, but the systolic-diastolic-mean arterial pressure, respiratory rate, and end-tidal carbon dioxide did not change. Corrected sinus node recovery times and baseline sinus cycle lengths, which are markers of sinus nodal function, were both lengthened with the administration of DEX. Atrioventricular (AV) nodal function, as evidenced by the Wenckebach cycle length, the ventriculoatrial block cycle length, and AV nodal effective refractory periods, was lengthened significantly. We also found that DEX increased the atrial refractory period and diminished atrial excitability. CONCLUSIONS: DEX significantly depressed sinus and AV nodal function in pediatric patients without significant electrocardiogram interval changes, other than a trend toward lower heart rates. Although no spontaneous AV nodal block and no clinically significant bradycardia were seen, we recommend that DEX be used with caution in patients at risk for bradycardia and/or AV nodal dysfunction due to its associated comorbidities.

Diagnosis of pentacuspid aortic valve with severe regurgitation using three-dimensional transesophageal echocardiography.

Monocuspid and quadricuspid aortic valves are a rare congenital valve malformation, and less common is the pentacuspid aortic valve (PAV). Echocardiography is a helpful noninvasive technique for their diagnosis. In this article, we describe a 48-year-old female patient with a PAV and severe aortic regurgitation that was diagnosed using three-dimensional transesophageal echocardiography.

Evaluation rhythm problems in unexplained syncope etiology with implantable loop recorder.

BACKGROUND: Syncope is a frequent complaint in children and adolescents and may be a significant sign of serious pathology. Although patient history, family history, and physical examination are sufficient to reach a diagnosis in most cases of syncope, the cause of syncope still cannot be determined after initial investigation in one-third to half of all patients. The aim of this study was to evaluate the diagnostic yield of implantable loop recorder (ILR) in children with unexplained syncope. METHODS: A retrospective review was carried out of clinical data, indications, findings, and a final management strategy in patients who underwent ILR implantation. RESULTS: A total of 12 patients with a mean age of 9.4 ± 4.5 years underwent ILR (Reveal Plus; Medtronic) implantation. ILR implantation indication was syncope in all of the patients. Family history, routine cardiac assessment, including resting 12-lead electrocardiogram, transthoracic echocardiography, 24 h Holter recording, and event recorder findings, were normal with the exception of one patient with (previously corrected) tetralogy of Fallot. After an average of 20 months (range, 1-36 months), six patients developed symptoms. ILR memory showed torsades de pointes-ventricular fibrillation (n = 3), catecholaminergic polymorphic ventricular tachycardia (n = 1), asystole and ventricular tachycardia (n = 1), and normal sinus rhythm (n = 1). At the time of writing six patients were still in follow up with no symptoms after an average of 25.2 months. CONCLUSION: Implantable loop recorder plays an important role in the diagnosis of life-threatening arrhythmias in which syncope is otherwise unexplained. ILR implantation should be remembered in children whose symptoms are strongly correlated with rhythm disturbances.

Extracorporeal cardiopulmonary resuscitation for breath-holding spells followed by cardiac arrest due to left main coronary artery stenosis.

Non-syndromic congenital supravalvular aortic stenosis (SVAS) leads to ventricular hypertrophy and increased oxygen consumption, and when combined with other factors reduces coronary blood flow, potentially resulting in myocardial ischemia and sudden cardiac death. While the anatomic obstruction of coronary circulation is as common in non-syndromic SVAS as in Williams syndrome, it often remains unacknowledged. Extracorporeal membrane oxygenation (ECMO) is an elective procedure that can be used to support patients with cardiac arrest during diagnosis as a way to reduce cardiopulmonary load in preparation for surgery or further treatment. In this report, we describe the rare case of an infant with severe SVAS and mild valvular pulmonary and left main coronary artery stenosis, as well as breath-holding spells. After multiple cardiac arrests, the infant underwent diagnostic catheter angiography on ECMO and had the pathology surgically corrected.

Factors affecting perioperative mortality in tetralogy of Fallot.

BACKGROUND: We evaluated the preoperative, operative and postoperative risk factors affecting early mortality in patients who underwent total correction of tetralogy of Fallot (TOF). METHODS: One hundred and twenty-two TOF patients who underwent reparative surgery between January 2010 and November 2013 were enrolled in the study. RESULTS: Mean patient age and weight was 2.3 ± 2.5 years and 11.3 ± 6.4 kg, respectively. Cardiac catheterization was performed in 101 patients (83%),and coronary anomalies were found in 11 patients. Mean McGoon index, pulmonary annulus z-score, main pulmonary artery z-score, left pulmonary artery z-score and right pulmonary artery z-score were 2.0 ± 0.4, -1.85 ± 1.54, -2.84 ± 2.06, 1.17 ± 1.73, and 0.74 ± 1.57, respectively. Total reparative surgery with a transannular patch was performed in 97 patients (79.6%); the rest underwent valve-sparing surgery. Median duration of postoperative mechanical ventilation, intensive care and hospital stay were 19 h, 3 days and 9 days, respectively. Extracorporeal membrane oxygenation (ECMO) was required in 10 patients in the postoperative early period. Arrhythmias occurring in the early postoperative period were junctional ectopic tachycardia (n = 13), complete atrioventricular block(n = 10; permanent epicardial pacemaker implanted in four) and ventricular tachycardia (n = 4). Nine patients died in the early postoperative period (7.3%). Parameters found to be associated with increased mortality were low preoperative oxygen saturation; high right ventricular/aortic pressure ratio immediately after surgery; presence of coronary anomaly; requirement of postoperative ECMO; and pacemaker (P = 0.02, P = 0.04, P = 0.01, P = 0.0001, P = 0.03, respectively). CONCLUSIONS: Poor preoperative oxygenation, presence of coronary anomaly, complete AV block in the early postoperative period, high RV pressure and requirement of ECMO appear to be the most significant factors that affect early mortality in the surgical treatment of TOF. Appropriate preoperative assessment, correct surgical strategies and attentive intensive care monitoring are required in order to reduce mortality.

Catecholaminergic Polymorphic Ventricular Tachycardia: A Rare Cause of Cardiac Arrest Following Blunt Chest Trauma.

UNLABELLED: Catecholaminergic polymorphic ventricular tachycardia (CPVT) is an electrophysiological disorder of a physically normal heart that occurs in children when the body is subjected to intense emotional or physical stress that causes adrenergic discharge. This troubling disease can be sporadic (spontaneous) or familial (genetic/inherited). Unfortunately, its associated ventricular tachycardia may cause sudden death, so early diagnosis of CPVT is very important. Treatment modalities include medical treatment, implantation of a cardioverter defibrillator, or surgical sympatectomy; but the implantable cardioverter defibrillator (ICD) should be the first choice in patients with a history of cardiac arrest. We herein present the case of a patient diagnosed with CPVT after a successful cardiopulmonary resuscitation triggered by blunt chest trauma. We implanted an implantable cardioverter defibrillator and started oral B-blocker treatment. During the course of follow-up, flecainide was added to his treatment depending on the patient's status regarding recurrent ICD shock. The patient has now continued follow-up without recurrent ICD shock since flecainide treatment was initiated. In conclusion, in patients with syncope and sudden cardiac arrest secondary to physical stress or blunt chest trauma, CPVT should be considered and an implantable cardioverter defibrillator must be implanted. Additionally, flecainide theraphy should be considered to decrease recurrent ICD shock. KEY WORDS: Blunt chest trauma; Cardiac arrest; CPVT.

Successful transvenous lead extraction using the Evolution System in a 17-kg child.

Despite technological advancements in the field of pacemaker lead extraction, available data on pediatric patients is limited, and risk of failure and severe complications remains high. In this report, we present the case of a male patient who, at five months old, had been transvenously implanted with a single-chamber ventricular pacemaker due to complete atrioventricular block. At 7-year of age, the patient was referred to us with growth-related lead tension, severe tricuspid insufficiency, and weak battery. We extracted the lead using the Evolution System and replaced the unit with a dual-chamber pacemaker with a SelectSecure lead. This new system can be used for lead extraction even in low-weight pediatric patients with congenital heart disease. Using a thin, lumenless SelectSecure lead appears to reduce the risk of venous obstruction.

The use of flecainide in critical neonates and infants with incessant supraventricular tachycardias.

OBJECTIVE: This study aimed to evaluate the efficacy of flecainide therapy in neonates and infants with drug resistant incessant supraventricular tachycardia. METHODS: The study included 11 neonates and infants who received medical and/or ablation therapy between January 2010 and December 2013. Mean patient age and weight were 101.6 ± 96 days and 5.3 ± 1.9 kg respectively. Of the 12 patients, 5 underwent ablation between January 2010 and December 2011, and 6 were treated medically between January 2012 and December 2013. Mean follow-up time was 18 months (6 months-4 years). RESULTS: The antiarrhythmic agent flecainide only became available in Turkey in 2012, and the most noteworthy point was its addition to the therapy administered prior to ablation (adenosine, esmolol-propranolol, propafenone, amiodarone and cardioversion). In all 6 patients admitted between January 2012 and December 2013, refractory SVT was successfully treated with the administration of a triple therapy regimen of esmolol-propranolol, amiodarone and flecainide. One patient with myocarditis developed an atrial flutter complicated by a concealed accessory pathway and was put on extracorporeal membrane oxygenation (ECMO) support due to cardiopulmonary failure. The SVT was terminated, but the patient died on the fifteenth day of ECMO support. One patient with recurrent tachycardia, who had previously undergone ablation for a complex cardiac anomaly and Wolf-Parkinson-White syndrome, was treated with ablation again. No recurrence of tachycardia was observed in any of the other 9 patients. CONCLUSION: It appears that the use of propranolol-esmolol and amiodarone combined with flecainide in the medical treatment of drug-resistant SVT may reduce the need for ablation in critical neonates and infants.

Assessment of atrioventricular conduction following cryoablation of atrioventricular nodal reentrant tachycardia in children.

BACKGROUND: Early-onset transient atrioventricular block (AVB) is a rare occurrence following cryoablation of atrioventricular nodal reentrant tachycardia (AVNRT), despite lack of any AVB at the end of the procedure. The purpose of this prospective study was to assess AVB shortly after successful cryoablation of AVNRT in children. METHODS: A 6-mm-tip cryocatheter was used in 39 procedures. An 8-mm-tip catheter was used in 11 procedures. Twelve-lead electrocardiograms (ECGs) and 24-hour ambulatory ECGs were performed 24 hours prior to the procedure and immediately following the procedure. All procedures were done using the EnSite system (St. Jude Medical, St. Paul, MN, USA) without fluoroscopy. RESULTS: Although nine (18%) patients developed variable degrees of transient AVB during the procedure, all of them had normal atrioventricular (AV) conduction at the end of the procedure and did not require any intervention. Four of these patients had variable degrees of transient AVB following the procedure despite having normal AV conduction at the end of the procedure. One developed Mobitz type I AVB, which lasted for 11.5 hours, and the other three experienced 2:1 AVB, which lasted for 2, 8, and 24 hours, respectively. All patients had complete resolution of the AVB, which was also documented with the 24-hour ambulatory ECGs after the procedure. CONCLUSION: Early transient AVB can develop following AVNRT cryoablation even if AV conduction is normal at the end of the procedure. Despite the transient AVB in the initial 24 hours after the procedure in some cases, there is no evidence for ongoing AV nodal dysfunction.

Holter electrocardiography findings and P-wave dispersion in pediatric patients with transcatheter closure of atrial septal defects.

BACKGROUND: This study aimed to determine the frequency of postintervention arrhythmia and factors associated with the development of arrhythmia, including the correlation between arrhythmia and P-wave dispersion, and the effects of the latter on transcatheter closure of atrial septal defects (ASDs). METHODS: Holter ECG recordings were performed before and after the intervention and 6 and 12 months later in 47 of the 59 patients who had undergone transcatheter ASD closure and once in the healthy control subjects. RESULTS: A statistically significant correlation was identified between the patients' arrhythmia grade according to Lown's system and each of the following: the number of defects, the size of the atrioventricular valve rim, the presence of an atrial septal aneurysm. CONCLUSION: The frequency of arrhythmia increases after transcatheter ASD closure, gradually decreases within the next year, and is most frequently of a benign nature. Lown's arrhythmia grading of patients occluded with either the Amplatzer septal occluder (ASO) or the Cardio-O-Fix septal occluder (CSO) were compared, and the arrhythmia frequency was higher with the latter. One day after the intervention, the P maximum (Pmax ) and the P dispersion(Pdis ) values were not increased but in fact slightly reduced in patients occluded with either ASO or CSO. An improvement in the electrical system resulting from early anatomical and mechanical healing following transcatheter ASD occlusion may explain the reduction in the Pmax and Pdis values.

Near infrared spectroscopy monitoring in the pediatric cardiac catheterization laboratory.

Near-infrared spectroscopy (NIRS) is a noninvasive method used to evaluate tissue oxygenation. We evaluated the relationship between cerebral and renal NIRS parameters during transcatheter intervention and adverse events in the catheterization room. Between January 1 and May 31, 2012, 123 of 163 pediatric patients undergoing cardiac catheterization were followed by NIRS. All were monitored by electrocardiography, noninvasive blood pressure measurement, pulse oxymetry, initial and final blood lactate level measurement. The number of interventional procedures was 73 (59%). During the procedures, 39 patients experienced a total of 41 adverse events: 18 (19.5%) had desaturation, 10 (8.1%) arrhythmia, three (2.4%) had respiratory difficulty, six (4.8%) had a situation calling for cardiopulmonary resuscitation, three (2.4%) had anemia necessitating transfusion, and one (0.8%) had a cyanotic spell. Cranial NIRS values worsened in 12 (9.8%) and renal measurements worsened in 13 (12.5%) patients. The sensitivity and specificity of a 9% impairment of cranial values were 90 and 61%, respectively, while the corresponding calculations for a 21% fall in renal measurements were 54% sensitivity and 90% specificity. When arrhythmia developed, NIRS values fell simultaneously, while the development of a desaturation problem was heralded by NIRS falling 10-15 s earlier than changes in pulse oxymetry; on improving saturation, NIRS returned to earlier values 10-15 s before pulse oxymetry readings. NIRS monitoring may provide an early warning with regard to complications likely to develop during a procedure. A fall of 9% in cranial NIRS values, or of 21% in renal measurements, should raise clinician awareness.

Transcatheter closure of perimembranous ventricular septal defects using Nit-Occlud(®) Lê VSD coil: early and mid-term results.

Our objective was to investigate the short- and mid-term results of transcatheter ventricular septal defect (VSD) closure with the Nit-Occlud(®) Lê VSD coil. Retrospective data collection study. Data were collected from 20 patients who underwent transcatheter VSD closure with the Nit-Occlud(®) Lê VSD coil device between October 2011 and June 2013. The mean age of the study subjects was 7.3 ± 4.0 years, and the mean weight was 25.7 ± 11.8 kg. The distance between the defect and the aortic valve, measured using angiography, was an average of 5.1 ± 2.0 mm, and the left ventricular opening averaged 8.2 ± 2.1 mm. The mean value of the Q p/Q s ratio was 1.7 ± 0.4. Intravascular hemolysis developed in the first few hours after the procedure in three patients. In one of these cases, despite medical treatment and the implantation of a detachable coil placed into the Nit-Occlud(®) device transcatheterly, hemolysis persisted. This device was removed and the VSD was closed surgically. In the other two cases, although the residual shunt persisted on echocardiography, the hemolysis regressed spontaneously. There were no rhythm problems or other complications during the follow-up period of 12.3 ± 6.6 months. In the selected cases, for the transcatheter treatment of VSD, the Nit-Occlud(®) Lê VSD coil device can be used. When compared with other VSD closure devices, there was no development of a permanent atrioventricular block, which is an important advantage. However, patients with a residual shunt should be monitored closely for the development of hemolysis during the first few hours.