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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.

Introduction: Previous studies have demonstrated the predictive value of the neutrophil-to-lymphocyte ratio (NLR) in many cardiovascular disorders. The aim of this study was to assess whether NLR is associated with echocardiographic or electrocardiographic parameters, or with predicted five-year risk of sudden cardiac death (SCD), in patients with hypertrophic cardiomyopathy (HCM). Methods: This prospective observational study included 74 controls and 97 HCM patients. Three years of follow-up results for HCM patients were evaluated. Results: NLR was significantly higher in patients with fragmented QRS, ventricular tachycardia, and presyncope than in those without (p=0.031, 0.030, and 0.020, respectively). NLR was significantly higher in patients whose predicted five-year risk of SCD was more than 6% and whose corrected QT interval was greater than 440 ms (p=0.022 and 0.001, respectively). It was also significantly higher in patients whose left ventricular ejection fraction (LVEF) was 60% (p=0.017). Conclusion: NLR was significantly higher in patients with HCM compared to the control group. A high NLR is associated with a higher five-year risk of SCD in patients with HCM. Resumo: Introdução: Estudos prévios demonstraram o valor preditivo da relação neutrófilos/linfócitos (RNL) em muitas alterações cardiovasculares. O objetivo deste estudo foi avaliar se a RNL está associada a parâmetros ecocardiográficos ou eletrocardiográficos, ou com o score de risco de morte súbita cardíaca (MSC) a cinco anos em doentes com miocardiopatia hipertrófica (MCH). Métodos: Este estudo prospetivo observacional incluiu 74 controlos e 97 doentes com MCH. Foram avaliados os resultados dos doentes com MCH ao longo de três anos de seguimento. Resultados: O valor da RNL foi significativamente superior nos doentes com QRS fragmentado, com taquicardia ventricular e com pré-síncope do que nos que não revelaram esses sinais (valores p: 0,031, 0,030, 0,020, respetivamente). O valor RNL foi estatística e significativamente superior nos doentes com risco de MSC previsível a cinco anos superior a 6%, e com um intervalo QT corrigido superior a 440 ms (valores p: 0,022, 0,001, respetivamente). O valor da RNL foi significativamente superior nos doentes com fração de ejeção (FE) 60% (valor p = 0,017). Conclusão: A RNL foi significativamente superior nos doentes com MCH quando comparada com o grupo controlo. Uma RNL alta está associada a um score de risco de MSC elevado aos cinco anos em doentes com MCH. Keywords: Hypertrophic cardiomyopathy, Neutrophil-to-lymphocyte ratio, Five-year risk of sudden cardiac death, Palavras-chave: Miocardiopatia hipertrófica, Relação neutrófilos/linfócitos, Score de risco a cinco anos de morte súbita cardíaca

Substance use, including cannabis, has been documented among women during pregnancy, particularly during the 1st trimester. Recreational drugs well known to induce Myocardial Infarction (MI) include cannabis, cocaine, amphetamines and ecstasy even in new born. Their effects on the cardiovascular system are predominantly related to stimulation of the sympathetic nervous system; thus they are known to cause MI secondary to coronary vasospasm. Here we showed that transient coronary vasospasm in a new born due to maternal cannabis use during pregnancy. Smoking cannabis is known to be a rare cause of MI. Cannabis use during pregnancy can induce transient coronary vasospasm and ischemia in new born. These patients should be followed closely in intensive care unit. If necessary a coronary angiography should design to evaluate coronary arteries.

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.

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). 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.

The use of contraband cigarettes is a serious public health problem. We present a case of atrial fibrillation in a healthy adolescent suspected to be caused by smoking contraband cigarettes. A 15-year-old man was admitted to our emergency department experiencing syncope and palpitations. He was a cigarette smoker, but he had never smoked any illicit tobacco products before. He had finished a pack of counterfeit cigarettes (20 pieces) in 1.5 h. His electrocardiogram showed atrial fibrillation with a rapid ventricular response and irregular RR intervals. The patient had no history of alcohol use, surgery, palpitations, hypertension, chronic bronchitis, or any infectious diseases. His atrial fibrillation was converted to a normal sinus rhythm after the cardioversion treatment. Our patient was discharged from the pediatric cardiology service and advised to quit smoking cigarettes, strictly warning against illicit tobacco products. In conclusion, intensive smoking of counterfeit cigarettes may lead to occurrences of atrial fibrillation.

Combined methylmalonic aciduria and homocystinuria is a very rare disease caused by a defect in the synthesis of two cofactors in cobalamin dependent reactions, i.e. adenosylcobalamin and methylcobalamin, active forms of vitamin B12. Early onset disease consists of neurological, hematological and gastrointestinal abnormalities seen in the first year of life and some minor face abnormalities (long philtrum, wide forehead, big and low set ears, long face). Here we report a 4-month-old male with failure to thrive, pancytopenia and neurological disturbance.

Stenting of patent ductus arteriosus is an alternative to palliative cardiac surgery in newborns with duct-dependent or decreased pulmonary circulation; however, the use of this technique in patients with an aortic arch abnormality presents a challenge. Tetralogy of Fallot is a congenital heart defect that is frequently associated with anomalies of the aortic arch and its branches. The association is even more common in patients with chromosome 22q11 deletion. We present the case of an 18-day-old male infant who had cyanosis and a heart murmur. After an initial echocardiographic evaluation, the patient was diagnosed with tetralogy of Fallot and right-sided aortic arch. The pulmonary annulus and the main pulmonary artery and its branches were slightly hypoplastic; the ductus arteriosus was small. Conventional and computed tomographic angiograms revealed a double aortic arch and an aberrant left subclavian artery. The right aortic arch branched into the subclavian arteries and continued into the descending aorta, whereas the left aortic arch branched into the common carotid arteries and ended with the patent ductus arteriosus. After evaluation of the ductal anatomy, we implanted a 3.5 × 15-mm coronary stent in the duct. Follow-up injections showed augmented pulmonary flow and an increase in oxygen saturation from 65% to 94%. The patient was also found to have chromosome 22q11 deletion.

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.

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.

Background We aimed to determine the correlation between noninvasive testing (exercise stress testing [EST] and adenosine responsiveness of accessory pathway [AP] ) and invasive electrophysiology study (EPS) for assessment antegrade conduction of the AP in Wolff–Parkinson–White syndrome. Patients and Method This prospective, observational study enrolled 40 children (58% male children, median age of 13 years, and median weight of 47.5 kg) with Wolff–Parkinson–White syndrome. Conduction through the AP to a cycle length of ≤250 ms was considered rapid or high-risk; otherwise, patients were nonrapid or low-risk. Results The sudden disappearance of the delta-wave was seen in 10 cases (25%) during EST. Accessory pathway was found to be high-risk in 13 cases (13/40, 32.5%) while the accessory path was identified as low-risk in 27 cases; however, six patients (15%) had blocked AP conduction with adenosine during EPS. Low-risk classification by EST alone to identify patients with nonrapid conduction in baseline EPS had a specificity of 93% and a positive predictive value of 90% (accuracy 54%). Blocked AP conduction with adenosine as a marker of nonrapid baseline AP conduction had a specificity of 93% and a positive predictive value of 84%. Finally, AP was adenosine nonresponsive in the majority of patients (28/30, 93%) with persistent delta-waves, 40% of those who had a sudden disappearance of delta-waves had an adenosine-responsive AP (P value: .028). Conclusion Abrupt loss of preexcitation during EST and blocked AP conduction with adenosine had high specificity and positive predictive value for nonrapid and low-risk antegrade conduction during baseline invasive EPS. Successful risk stratification of pediatric patients with Wolff–Parkinson–White is possible through the use of EST and the adenosine responsiveness of AP.

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.

Congenital supravalvar aortic stenosis (SVAS) is an arteriopathy associated with Williams-Beuren syndrome (WBS) and other isolated elastin gene deletions. Cardiovascular manifestations associated with WBS are characterized by obstructive arterial lesions such as SVAS and pulmonary artery stenosis in addition to bicuspid aortic valve and mitral valve prolapse. However, coronary artery ostial stenosis may be associated with SVAS, and it increases the risk of sudden death and may complicate surgical management. In this report, we present our experience with two patients having SVAS and left coronary artery ostial stenosis with associated left ventricular dysfunction.

Objective We evaluated autonomic behavior by examining heart rate variability (HRV) in the time domain and frequency domain in pediatric patients who underwent transcatheter closure of atrial septal defect (ASD). Methods A prospective study design was used. Holter ECG was performed in a control group of 30 healthy subjects and a group of 47 patients who underwent transcatheter ASD closure. ECG was taken one day before, one day after, and six months after the procedure to evaluate changes in the time domain [SDNN, rMSSD, NN, pNN50(%), and SDANN] and frequency domain (VLF, LF, HF, VHF, and LF/HF) in the patient group. Student's t-test was used to evaluate changes prior to and after the procedure. Results There were 28 females (60%) in the patient group and 21 females (70%) in the control group. The mean age and weight of the participants in the patient group were 9.61±4.72 years and 32.40±19.60 kg, respectively; the mean age and weight of the control subjects were 10.43±5.31 years and 32.83±13.00 kg, respectively. In both the time domain and frequency domain analyses, the patient group values were found to be lower than those in the control group prior to the procedure; the values in the patient group were found to approach the values in the control group following the procedure. By the sixth month, the values in the patient group reached the control levels with no statistically significant difference (SDNN: 145±0.84, 137.50±42.50; rMSSD: 72.18±48.22, 58.14±28.49; SDANN: 125.13±13.50, 122.40±41.06; VLF: 112.85±29.07, 114.41±98.39; LF: 50.40±24.09, 45.69±15.13; HF: 39.28±19.86, 44.29±13.14; VHF: 10.29±4.24, 9.99±6.47; LF/HF: 1.90±1.44, 1.24±0.81; p>0.05). Conclusion The transcatheter closure of secundum ASDs was found to have a positive effect on HRV. Consequently, it may contribute to reduced mortality and morbidity. We can conclude that in children, HRV recovers approximately six months after transcatheter ASD closure.

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.

Objectives: The radiation dose from interventional cardiac catheterization is particularly relevant when treating children because of their greater radiosensitivity compared to adults. The transcatheter closure of patent ductus arteriosus (PDA), as well as other more complex pediatric interventions, have raised concerns regarding radiation exposure, particularly relevant when treating children. The purpose of this study is to show how to perform the transcatheter closure of PDA in children while giving less ionized radiation and to prove that the amount of radiation and contrast material can be reduced. Study design: Following appropriate device selection based on PDA morphology and diameter, transthorasic echocardiography images and control aortography findings were analyzed. The following devices were used during the procedure: Gianturco coils (10/63), an Amplatzer Duct Occluder (ADO, 31/63), Flipper coils (19/63), and an Amplatzer vascular plug (3/63). Results: The scopy time, radiation dose, and contrast were 12+-6.4 mins, 28.1+-14.7 cmGy/cm2/kg, and 4.2 +- 2.3 cc/ kg, respectively. In the control aortography shortly after the procedure, residual shunt was detected at various levels in 39.7% of patients, and 9.5% demonstrated residual shunt in real-time echocardiography. In the control aortography, the exposure to radiation was 13.3% of the total, and the amount of infused contrast was 27.2% of the total. Conclusion: Patients may be exposed to less radiation and contrast material if an echocardiographic evaluation, instead of a final control aortography injection, is performed after the transcatheter closure of PDA.

Patients who are diagnosed with Brugada syndrome (BS) usually experience sudden cardiac arrest (SCA) and arrhythmia when they have a high fever, consume alcohol, and, more frequently, during their night sleep. In some rare cases, an SCA can be seen depending on a possible vagal stimulus, such as eating a large bite of food. We describe a 9-year-old patient who had a sudden cardiac attack while he was eating a large hot dog. After successful resuscitation, a suspicious ST elevation in V2 was seen in his electrocardiographic evaluation. He was diagnosed with BS after the ajmaline test and an implantable cardioverter defibrillator was implanted. Vagal stimulus–dependent SCA after eating a large bite of food may be the first symptom of BS. For this reason, the electrocardiographic results of the children who had a cardiac arrest after eating a large meal with big bites should be evaluated in detail.

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. (PACE 2014; 00:1‐5)

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.

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.

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.

Sinus pericranii is a small circumscribed fluctuating vascular swelling of the scalp that directly communicates with the intracranial venous system. Sinus pericranii is not a single clinicopathologic entity, rather a symptom complex with diverse clinical manifestations. The exact diagnosis of sinus pericranii is made by angiography and percutaneous sinusography. We report an 8-month-old child with sinus pericranii who was diagnosed with a non-invasive method, magnetic resonance venography.

Background The rapid expansion of pediatric cardiovascular surgery, along with the increase in the number of centers and operations, has also increased the use of intraoperative transesophageal echocardiography (TEE). Preoperative TEE allows for the confirmation of diagnoses established by transthoracic echocardiography and angiography, and also identifies possible additional pathologic conditions. TEE is also used to follow up on surgical repairs, determine the need for reintervention, and evaluate myocardial performance and the need for inotropic treatment. Our intention is to share the TEE findings before and after intervention for congenital heart disease in patients who received operations at our center, and the conclusions reached. Patients and Method From October 2009 to February 2013, 1008 patients underwent surgery for congenital heart disease. Of these, 265 were subjected to TEE in the operating room. Each patient entering the operating room with TEE planned had the echocardiography probe placed while intubated under general anesthesia. TEE studies used a GE Vivid S5. Different investigations, according to the patients' particular pathologic conditions, were also performed in addition to standard TEE studies. Results Of the 265 patients for whom surgery was indicated by transthoracic echocardiography and other appropriate examinations, 260 had the diagnosis confirmed by preoperative TEE and the indicated intervention was performed. For the remaining 5 patients (1.8%), the intervention plan was changed following preoperative TEE. Cardiopulmonary bypass was reinitiated in 12 (4.5%) patients because of residual defects identified by postoperative TEE. Thus, the preoperative plan was changed in 17 (6.4%) patients out of a total of 265 owing to preoperative and postoperative TEE findings. Conclusion The use of intraoperative TEE in surgical centers for congenital heart disease allows for a significant reduction in mortality and morbidity. Intraoperative TEE performed by experienced pediatric cardiologists is therefore an absolute necessity.

ObjectivesCurative therapy of idiopathic ventricular tachycardia remains a challenge in interventional electrophysiology. The aim of this study was to demonstrate the utility of an EnSite NavX system in the catheter ablation of idiopathic ventricular tachycardia in children.Patients and methodsIn all, 17 children with idiopathic ventricular tachycardia underwent electrophysiological studies using the EnSite NavX system guidance.ResultsThe mean patient age was 13 ± 2.4 years (range: 7.8–17.9) and the mean patient weight was 52.3 ± 11.9 kg (range: 32–75). The origin of ventricular tachycardia was in the right ventricular outflow tract in nine patients, in the left ventricle in six, near the bundle of His/right bundle branch in one, and in the left aortic cusp in one. The mean procedure and fluoroscopy times were 169.3 ± 43.2 minutes and 8 ± 10.8 minutes, respectively. No fluoroscopy was used in six patients. The mean radiation exposure was 33.1 ± 56.4 mGy. Acute success was achieved in 14 patients (82%). The focus of ventricular tachycardia was epicardial in two failed procedures. During a mean follow-up of 8.5 ± 7.6 months, ventricular tachycardia recurred in three patients, two of whom underwent a second procedure. Except for one patient who developed transient right bundle branch block, no complications were seen.ConclusionCatheter ablation of idiopathic ventricular tachycardia in children can be performed safely and effectively with low fluoroscopy exposure using the EnSite NavX system.

Background Epicardial implantation of implantable cardioverter defibrillators (ICDs) is considered in the presence of intracardiac shunt, venous access issue, or small body size. We report our experience with epicardial ICD coil implantation using a minimally invasive method. Methods Nine patients who underwent epicardial ICD implantation were included. The median age was 7.4 years (3.9–9.6 years) and the median weight was 15 kg (12–24 kg). Diagnosis at the time of implantation included long QT syndrome (n = 5), catecholaminergic polymorphic ventricular tachycardia (n = 2), hypertrophic cardiomyopathy (n = 1), and fast monomorphic ventricular tachycardia (n = 1). The minimally invasive method involved a subxiphoid incision to place the epicardial pacing leads. The ICD coil was placed in the transverse sinus in four patients using an access path posterior to the heart. The second approach involved a path anterior to the heart to reach the epicardial location posterior to the left atrial appendage in the five remaining patients. No fluoroscopy was used in either approach. The median defibrillation threshold (DFT) at implantation was 7.5 J. Results Lower DFTs were observed in the anterior approach (10 J vs 5 J). Appropriate ICD shocks were observed in three patients during the median 18-month follow-up period (0.3–28 months). No inappropriate shocks were noted. One patient developed pericardial tamponade 39 days after the procedure and was surgically drained. Conclusions Minimally invasive epicardial ICD coil implantation in children with low DFT values is possible. The anterior implantation approach appears superior to the posterior approach.

Pulmonary atresia with intact ventricular septum (PAIVS) is characterized by a broad spectrum of heterogeneous morphologies. Perforation of the atretic valve, balloon dilatation, and stenting of the patent ductus arteriosus are the percutaneous techniques that are used with increasing frequency in our clinic as well. They have some advantages over surgery, including short hospital stay and short intensive care unit stay. The main goal of the primary interventional approach is to avoid surgery. However, a group of patients with PAIVS still need surgery due to poor right ventricular growth. Therefore, the final achievement of the initial percutaneous treatment strategies is still debatable. In this article, we present the early- and mid-term results of the percutaneous approach utilized at our clinic in order to investigate the final effects of interventional therapy according to initial morphology. Between May 2010 and May 2012, 15 neonates diagnosed with PAIVS underwent transcatheter intervention. Detailed echocardiographic examination focused on right ventricle size, and tricuspid valve morphology and coronary sinusoids were performed in all the patients before the intervention. Nine of the patients were boys and six were girls. The mean age was 11.40 ± 12.87 days and mean weight was 3.34 ± 0.46 kg. Only one procedure-related mortality occurred. The mean follow-up period was 10.05 ± 3.42 months (1-26 months). The mean duration of intensive care was 7.19 ± 5.14 days. The mean follow-up time was 10.05 ± 3.42 months. After this period, survival rate was 66% (10/15). Two of the patients achieved biventricular physiology after pulmonary valve perforation. Two patients still have univentricular physiology. Six patients have been followed as a one and half ventricle repair candidate. Five out of 15 patients had stent patency during 6 months of follow-up, while restenosis developed in one patient (1/5, 20%), who had undergone the Glenn operation at 5 months of age. Transcatheter management for PAIVS is a feasible, safe, and effective primary palliative treatment in newborns. Shunt surgery may be considered in cases where cyanosis occurs despite transcatheter intervention. Right ventricular size determines the type of intervention. The early outcomes can be comparable with surgical palliation. However, a group of PAIVS, particularly with severe right ventricular hypoplasia, needs surgery even after a successful primary percutaneous intervention.

Objective: Experience with administration of inhaled nitric oxide (iNO) in pediatric cardiac intensive care unit was retrospectively reviewed. Methods: Data from 32 pediatric patients treated with iNO between 2011 and 2012 were collected. Patients were divided into 3 groups: Group I comprised postoperative patients, Group II comprised newborns with persistent pulmonary hypertension (PPH), and Group III comprised patients with primary pulmonary hypertension (PH) or Eisenmenger's syndrome. Age, sex, weight, primary diagnosis, arterial blood sample, pulmonary artery pressure (PAP), systemic arterial pressure (SAP), and oxygen saturation levels were analyzed. Results: Groups I, II, and III included 25, 3, and 4 patients, respectively. Median weight was 8 kg (range: 3–40 kg), and median age was 7 months (range: 2 days–10 years). On average, iNO treatment was initiated at the 12th hour after admission to the unit (range: 1–48 hours) and continued for a median duration of 24 hours (range: 12–168 hours). Systolic PAP was 40+-15 mmHg, mean SAP was 57+-18 mmHg, PAP/SAP ratio was 0.69, and oxygen saturation levels were 88% prior to iNO treatment. Following iNO treatment, PAP decreased to 24+-9 mmHg (p

The purpose of this study was to evaluate the feasibility of multislice computed tomographic (MSCT) angiography as a noninvasive method for detecting ostial, proximal, and middle segment coronary stenosis or occlusion and anatomy in patients with transposition of the great arteries who had undergone arterial switch operation (ASO). Sixteen-detector-row MSCT angiography was performed in 16 patients treated with ASO for transposition of the great arteries. The median age was 10.3 years (range, 6.2-16.3 years). Sixteen-detector-row MSCT angiography was performed in 16 patients who had undergone ASO. CT imaging was performed in the craniocaudal direction from 2 cm above the carina up to the heart basis. Noninvasive assessment of coronary artery stenosis and anatomy were investigated by MSCT angiography. Two patients were excluded from the study because of artifacts. Of 14 evaluated patients, 1 patient had ostial stenosis (7.1%). A coronary artery anatomy variant was present in six patients: left main artery (LMA) and right coronary artery (RCA) originating from the right sinus as a single orifice (n = 2); left circumflex artery (LCX) originating from the RCA (n = 1); LMA and RCA, after branching to the LCX, originating separately from the right sinus (n = 1); and LMA (n = 1) and left anterior descending artery (LADA; n = 1) originating directly from the right sinus. Intramural bridging in the LAD (n = 2) was detected. Five patients were normal. In conclusion, MSCT angiography, as a noninvasive, feasible technique for assessing coronary stenosis or occlusion and anatomy, can be used in the follow-up of patients who have undergone ASO.

Systemic capillary leak syndrome (SCLS) is a rare disorder of unknown pathophysiology, characterised by episodic life-threatening hypotension, haemoconcentration and hypo-albuminaemia. A 5-month-old child presented with episodes of relapsing diarrhoea, vomiting and hyponatraemia and developed generalised oedema during treatment. Clinical and laboratory findings were consistent with acute SCLS. Treatment with careful fluid replacement, fresh frozen plasma and aminophylline in the acute phase and teophylline and gingko biloba in the chronic phase led to sustained remission during follow-up for over 1 year. To our knowledge, this is the youngest case of SCLS in the literature and is unusual in that it presented with diarrhoea.

A full-term 38-day-old female infant weighing 4 kg was admitted to our clinic with cardiac murmur. Oxygen saturation was 85%. Physical examination revealed a 2/6 systolic murmur along the left sternal border. Transthoracic echocardiography revealed a d-transposition of the great arteries. The two ventricles were balanced with a preserved left ventricle systolic function (LVEF = 70%). A 2 mm ventricular septal defect, a 6 mm secundum atrial septal defect, and a large patent ductus arteriosus were detected. In addition, there were peripheral pulmonary artery stenoses (Figure A, Video 1*) with a continuous-wave Doppler gradient of 50 mmHg in both, and arcus hypoplasia. The main pulmonary artery was dilated (21 mm); right and left pulmonary arteries were small in size (3.5 mm and 3 mm respectively). The proximal transverse arch (5 mm, z-score: -3.41) and distal transverse arch (4.5 mm, z-score:-2.11) were hypoplastic. Isthmus was of normal size (5.5 mm, z-score:-0.41). Computerized tomography (CT) angiogram and 3D reconstruction confirmed severe right and left pulmonary artery stenosis and arcus aorta hypoplasia (Figure B-D). The patient was operated for arterial switch with bilateral pulmonary artery and aortic arch reconstruction. Transposition of the great arteries is a common congenital heart defect that causes cyanosis in neonates. It has been reported as occurring concomitantly with many congenital heart malformations. However, to the best of our knowledge, transposition of the great arteries associated with a peripheral pulmonary artery stenosis with arcus hypoplasia has not been reported. Careful echocardiographic assessment with CT confirmation may be the most important factor in developing the operative plan. 411

The features of pediatric patients with postoperative transient and permanent complete atrioventricular (AV) block (CAVB) were compared.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.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 (P0.05). 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 (P0.05). 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.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.

Background: Congenital complete atrioventricular block without any structural heart disease and anti-Ro/La negativity is very rare. Discordant complete atrioventricular block, which is more frequently defined in the literature as an autoimmune mechanism, is much more rare in monozygotic twins. Case Report: The 26-year-old healthy mother had given birth in her first spontaneous, uneventful pregnancy to monozygotic twins at week 35. While the first twin’s physical examination proved her to be normal with a pulse rate consistent with her age, the second twin had a pulse rate of approximately 40 beats/minute.The patient was confirmed to have congenital complete atrioventricular block. Conclusion: Despite this case appears to be an isolated one, a discordant complete atrioventricular block regression without any autoimmune evidence should be included in the differential diagnosis of bradycardia in infants.

We aimed to determine the correlation between noninvasive testing (exercise stress testing [EST] and adenosine responsiveness of accessory pathway [AP] ) and invasive electrophysiology study (EPS) for assessment antegrade conduction of the AP in Wolff-Parkinson-White syndrome.This prospective, observational study enrolled 40 children (58% male children, median age of 13 years, and median weight of 47.5 kg) with Wolff-Parkinson-White syndrome. Conduction through the AP to a cycle length of ≤250 ms was considered rapid or high-risk; otherwise, patients were nonrapid or low-risk.The sudden disappearance of the delta-wave was seen in 10 cases (25%) during EST. Accessory pathway was found to be high-risk in 13 cases (13/40, 32.5%) while the accessory path was identified as low-risk in 27 cases; however, six patients (15%) had blocked AP conduction with adenosine during EPS. Low-risk classification by EST alone to identify patients with nonrapid conduction in baseline EPS had a specificity of 93% and a positive predictive value of 90% (accuracy 54%). Blocked AP conduction with adenosine as a marker of nonrapid baseline AP conduction had a specificity of 93% and a positive predictive value of 84%. Finally, AP was adenosine nonresponsive in the majority of patients (28/30, 93%) with persistent delta-waves, 40% of those who had a sudden disappearance of delta-waves had an adenosine-responsive AP (P value: .028).Abrupt loss of preexcitation during EST and blocked AP conduction with adenosine had high specificity and positive predictive value for nonrapid and low-risk antegrade conduction during baseline invasive EPS. Successful risk stratification of pediatric patients with Wolff-Parkinson-White is possible through the use of EST and the adenosine responsiveness of AP.

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.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).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.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.

We evaluated the preoperative, operative and postoperative risk factors affecting early mortality in patients who underwent total correction of tetralogy of Fallot (TOF).One hundred and twenty-two TOF patients who underwent reparative surgery between January 2010 and November 2013 were enrolled in the study.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).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.

A 2.5-month-old female patient presented for closure of a ventricular septal defect (VSD). Transthoracic echocardiography showed a large muscular ventricular septal defect. After perventricular closure of the defect was performed with an Amplatzer muscular VSD occluder, peroperative transesophageal echocardiography revealed that the device had been implanted in the wrong area of the ventricular septum. The device was retrieved and a large mid-muscular defect with extension to the outlet septum was closed with a patch of Dacron which was secured with 5-0 sutures. A perforation in the ventricular septum due to attempted perventricular device delivery was seen, and it was repaired primarily. In the eighth month of follow-up, transthoracic echocardiography revealed an aneurysm in the posterior wall of the left ventricle. The patient’s electrocardiogram showed pathological Q waves and ST-segment elevation in leads DII, DIII, and aVF consistent with subacute inferior myocardial infarction. At subsequent surgery, this was found to be a true aneurysm, located in area of distribution of the obtuse marginal branch of the left circumflex coronary artery in the posterior wall of the left ventricle. The aneurysm was closed off using a Dacron patch, and the sac was resected. Development of a true aneurysm is a rare but important complication of attempted perventricular VSD closure.

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.

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.A retrospective review was carried out of clinical data, indications, findings, and a final management strategy in patients who underwent ILR implantation.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.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.

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.

Patients who have pulmonary atresia with intact ventricular septum have been shown to have a number of various myocardium anomalies like ischemia, fibrosis, infarction, rupture, disarray, spongious myocardium and ventricular endocardial fibroelastosis. Multiple connections have been found between right ventricular myocardial sinusoids and small branches of intramural coronary arteries. Noncompation of ventricular myocardium has been shown to be the result of myocardial ischemia or excessive pressure preventing the reduction of embryonic sinusoids. The persistence of intertrabecular recesses that are connected to both the ventricular cavity and coronary circulation is the result of this process. In this text, we describe a PA-IVS patient who underwent patent ductus arteriosus stenting and pulmonary valve perforation to create antegrade flow and later developed left ventricular noncompaction. We posit that there is a connection between right ventricular coronary sinusoids and noncompaction sinusoids. As our patient’s RV outflow tract stenosis and RV pressure increased, the coronary circulation connected to coronary sinuses became sufficient and LV function improved, which further supports our hypothesis.