Evaluation of ventricular function and myocardial deformation in children with repaired tetralogy of Fallot by real-time three-dimensional (four-dimensional) echocardiography.

AIM: The left and right ventricular dysfunction are important clinical course indicators in patients with repaired tetralogy of Fallot. This study aimed to evaluate ventricular volumes, functions, and myocardial deformation in children with repaired tetralogy of Fallot by real-time three-dimensional (four-dimensional) echocardiography and compared with healthy children. It also aimed to investigate the relationships between ventricular volumes, functions, and myocardial deformation parameters in the patients. MATERIALS AND METHODS: In this cross-sectional study, 35 patients (mean age 15.1 ± 2.8 years, 54% male) and 35 healthy controls of similar age, gender, and body measurements underwent echocardiography. End-diastolic volume index, end-systolic volume index, and ejection fractions of both ventricles; global longitudinal, circumferential, radial strain, twist, and torsion of the left ventricle; the longitudinal strain of the right ventricle free wall and septum were measured. RESULTS: Left ventricular ejection fraction, global circumferential and radial strain, twist and torsion were significantly lower in patients compared with controls. Left ventricular ejection fraction correlated with global circumferential (r = -0.446, p < 0.001) and radial strain (r = -0.433, p < 0.001) in the patients. Right ventricular volumes were significantly higher, and ejection fraction was significantly lower in patients compared with controls. All right ventricular parameters correlated with each other in the patients. CONCLUSION: Left ventricular contraction pattern was changed, circumferential and radial fibres were most affected in the patients. Right ventricular dilatation and dysfunction were detected, and right ventricular ejection fraction correlated well with strain measurements of the right ventricle.

Transcatheter closure of ventricular septal defect with two different devices.

Transcatheter closure of a multi-hole perimembranous ventricular septal defect with an aneurysm is challenging. Specific ventricular septal defect closure devices have been developed, but some occluders are reportedly used in an off-label manner. This report describes a child who had a multi-hole perimembranous ventricular septal defect with an aneurysm and underwent successful transcatheter closure using two different occluders: the Occlutech Duct Occluder (Occlutech, Helsingborg, Sweden) and the Amplatzer Duct Occluder II (St. Jude Medical, Saint Paul, Minnesota, United States of America). Transcatheter closure of a multi-hole perimembranous ventricular septal defect with an aneurysm using these two different devices can be performed safely by an experienced interventionist in selected patients.

Percutaneous Ventricular Septal Defect Closure in Patients Under 1 Year of Age.

Untreated ventricular septal defect (VSD) is an important cause of congestive heart failure in early infancy. Growth is impaired in this population, and surgical closure is challenging because of congestion in the lungs, making infants prone to respiratory infection, and because of their poor nutritional status. The aim of this study is to share our experience with percutaneous VSD closure in patients under 1 year of age. Patients with hemodynamically significant left-to-right shunt, less than 1 year of age, and with VSD diameter ≤ 6 mm were retrospectively included in the study between December 2014 and January 2017. The median length of follow-up was 8.5 (4-14.2) months. Twelve patients from 2 to 12 months of age, with a median weight of 6.75 (5.4-8) kg, were included. The mean VSD diameter as measured by angiography from the left ventricle side was 4.7 ± 0.25 mm, and from the right ventricle side was 3.4 ± 1.1 mm. All were of a perimembranous type except three, which were muscular. All defects were closed with the Amplatzer Duct Occluder II (ADO II) or the ADO II-additional size. The mean fluoroscopy duration and total radiation dose were 22.6 ± 18.7 min and 1674 ± 851 cGy/min, respectively. No aortic regurgitation associated with device closure was seen in any of the patients. Complete atrioventricular block occurred in one patient 6 months after the procedure, and was treated with a permanent pacemaker. VSD closure is challenging, regardless of whether a surgical or percutaneous procedure is used. The risks are higher for children younger than 1 year with low body weight. Percutaneous closure, which carries similar risks but is less invasive than surgery, may be the preferred alternative in early infancy.

Hallazgo casual de origen anómalo de la arteria coronaria derecha desde la arteria pulmonar en un lactante / An incidentally detected anomalous origin of the right coronary artery from the pulmonary artery in an infant

El origen anómalo aislado de la arteria coronaria derecha desde la arteria pulmonar principal es una anomalía congénita rara y se han notificado pocos casos en la población pediátrica. En este artículo informamos el caso asintomático de un lactante varón de dos meses de edad al que se le diagnosticó origen anómalo de la arteria coronaria derecha desde la arteria pulmonar principal durante la evaluación realizada para detectar anomalías cardíacas. Debido a la sospecha durante una ecocardiografía, se realizaron un cateterismo cardíaco y una angiografía coronaria para verificar el diagnóstico del origen anómalo de la arteria coronaria derecha desde la arteria pulmonar principal. El paciente se sometió a la cirugía y estaba en buen estado en el seguimiento a los dos meses. El diagnóstico temprano podría evitar que los pacientes tengan complicaciones cardiovasculares.

Isolated anomalous origin of the right coronary artery from the main pulmonary artery is a rare congenital anomaly, and few cases have been reported in the pediatric age group. Here in, we report an asymptomatic case of a 2-month-old male infant who has been diagnosed as anomalous origin of the right coronary artery from the main pulmonary artery during the evaluation for cardiac abnormalities. For a suspicion on echocardiography, cardiac catheterization and coronary angiography performed to verify the diagnosis of anomalous origin of the right coronary artery from the main pulmonary artery. The patient underwent surgery and did well after two months follow up. Early diagnosis may prevent patients from cardiovascular complications.

An incidentally detected anomalous origin of the right coronary artery from the pulmonary artery in an infant. / Hallazgo casual de origen anómalo de la arteria coronaria derecha desde la arteria pulmonar en un lactante.

Isolated anomalous origin of the right coronary artery from the main pulmonary artery is a rare congenital anomaly, and few cases have been reported in the pediatric age group. Here in, we report an asymptomatic case of a 2-month-old male infant who has been diagnosed as anomalous origin of the right coronary artery from the main pulmonary artery during the evaluation for cardiac abnormalities. For a suspicion on echocardiography, cardiac catheterization and coronary angiography performed to verify the diagnosis of anomalous origin of the right coronary artery from the main pulmonary artery. The patient underwent surgery and did well after two months follow up. Early diagnosis may prevent patients from cardiovascular complications.

El origen anómalo aislado de la arteria coronaria derecha desde la arteria pulmonar principal es una anomalía congénita rara y se han notificado pocos casos en la población pediátrica. En este artículo informamos el caso asintomático de un lactante varón de dos meses de edad al que se le diagnosticó origen anómalo de la arteria coronaria derecha desde la arteria pulmonar principal durante la evaluación realizada para detectar anomalías cardíacas. Debido a la sospecha durante una ecocardiografía, se realizaron un cateterismo cardíaco y una angiografía coronaria para verificar el diagnóstico del origen anómalo de la arteria coronaria derecha desde la arteria pulmonar principal. El paciente se sometió a la cirugía y estaba en buen estado en el seguimiento a los dos meses. El diagnóstico temprano podría evitar que los pacientes tengan complicaciones cardiovasculares.

Transcatheter correction of Scimitar syndrome: occlusion of abnormal pulmonary venous drainage and vascular supply in an infant.

Treatment of Scimitar syndrome is usually surgical; however, if there is "dual drainage" - that is, one to the inferior caval vein and the other to the left atrium - it is possible to successfully treat this anomaly via a less-invasive transcatheter approach. We report a case of Scimitar syndrome in a 21-month-old, male infant successfully treated with transcatheter embolisation.

Antegrade transvenous balloon angioplasty for coarctation of the aorta in infants with ventricular septal defect.

For a newborn, surgical correction has been the primary treatment of native coarctation at most centers; however, there has been an increased use of balloon angioplasty (BA). The anterograde transvenous (AT) technique is another alternative way for coarctation (AoC) angioplasty in low weight patients with large ventricular septal defect (VSD). Four, 5-day-old to 7-month-old, infants weighing 2500, 2700, 2800, and 3400g, respectively presented to emergency unit (EU) with cyanosis, tachypnea, and loss of weight. Echocardiography demonstrated AoC and VSD. All four children were admitted to the EU with hemodynamic compromise and critically ill status. We used femoral vein for sheath and used VSD to enter left ventricle from right antegrade route, and performed BA without any complication. AT described in this report is another alternative way for coarctation angioplasty in patients with large VSD. We suggest that AT BA can be applied to small infants in situations where surgery might have been hazardous.

Cardiovascular side effects related with use of synthetic cannabinoids "bonzai" : two case reports.

Information about the effects of synthetic cannabinoids "bonzai" on the cardiovascular system is limited. In this article, two patients in whom different cardiological side effects were observed following use of synthetic cannabinoids 'bonzai' were presented. Our first patient who was a 16-year old boy presented to pediatric emergency department with severe chest pain which had started one hour before. On electrocardiographic examination, ST segment elevations greater than 3 mm were observed in DII, DIII, AVF and V5-V6 derivations which caused to suspicious myocardial infarction. Cardiac catheterization was performed and coronary angiographic findings were found to be normal. When the patient was questioned again, it was learned that he used synthetic cannabinoid 'bonzai'. The second patient who was aged 18 years and addicted to synthetic cannabinoid 'bonzai' was brought to our emergency department by his friends because of clouding of consciousness. Dopamine treatment was started in the patient whose cardiac apical beat reduced to 40/min and who was found to develop hypertension, because bradycardia worsened. However, the blood pressure increased rapidly and dopamine treatment was stopped. Heart rate and blood pressure monitoring was continued. Use of synthetic cannabinoids 'bonzai' should be kept in mind in patients who present to emergency departments with chest pain and complaints related with the cardiovascular system.

Relationships between left heart chamber dilatation on echocardiography and left-to-right ventricle shunting quantified by cardiac catheterization in children with ventricular septal defects.

Left atrium and/or left ventricle dilatation on echocardiography is considered to be an indication for closure of ventricular septal defects (VSD). No study has addressed the accuracy of using dilated left heart chambers when defining significant left-to-right shunting quantified by cardiac catheterization in isolated small or moderate VSDs. In this study, the relation between dilated left heart chambers, measured by echocardiography, and left-to-right ventricle shunting, quantified by cardiac catheterization, was evaluated in patients with isolated VSD. The medical records of all patients with isolated VSD who had undergone catheterization from 1996 to 2010 were examined retrospectively. Normative data for left heart chambers adjusted for body weight (BW) and body surface area (BSA) were used. The pulmonary-to-systemic flow ratio (Qp:Qs) was calculated by an oximetry technique. A total of 115 patients (mean age 7.3 ± 5 years) fulfilled the inclusion criteria. There was a statistically significant difference in terms of Qp:Qs between the patient groups with normal and dilated left heart chambers, when adjusted for BW and BSA (p = 0.001 and p = 0.002, respectively). But the relationships between Qp:Qs and left heart chamber sizes on echocardiography were not strong enough to be useful for making surgical decisions, as left heart chamber dilatation was not significantly associated with Qp:Qs ≥ 2 (p = 0.349 when adjusted for BW, p = 0.107 when adjusted for BSA). Left heart chamber dilatation was significantly associated with Qp:Qs ≥ 1.5 only when it was adjusted for BSA (for BW p = 0.022, for BSA p = 0.006). As a result, left heart chamber dilatation measured by echocardiography does not show significant left-to-right ventricle shunting, as quantified by catheterization. We still advocate that catheter angiography should be undertaken when left heart chambers are dilated in echocardiography in order to make decisions about closing small- to moderate-sized VSD.

[Late presentation of posttraumatic pulmonary arteriovenous fistulea occlusion with septal occluder device]. / Travma sonrasi geç prezente olmus pulmoner arteriyovenöz fistülün septal tikayici cihaz ile kapatilmasi

Posttraumatic pulmonary arteriovenous fistula is a rare complication of penetrating injury. Cases may remain asymptomatic for years prior to diagnosis. We present a case of pulmonary arteriovenous fistula related to a penetrating injury of the thorax 6 years previously that was occluded with a septal occluder device. Consistent use of CT or conventional catheter pulmonary angiography following penetrating injury to the thorax is essential to the early diagnosis of pulmonary arteriovenous fistula during the asymptomatic stage. Modern occlude devices facilitate the transcatheter approach as a viable alternative to surgery for the closure large, high pressure defects.

QT dispersion and cardiac involvement in children with Familial Mediterranean fever.

Familial Mediterranean fever is a hereditary disease characterised by recurrent and self-terminated attacks of fever and polyserositis. An earlier study found that adult patients of Familial Mediterranean fever had an abnormally longer QT dispersion and corrected QT dispersion, markers for ventricular arrhythmogenicity. QT dispersion is a simple non-invasive arrhythmogenic marker that can be used to assess homogeneity of cardiac repolarisation; however, it has not been studied in children with Familial Mediterranean fever before. The aim of this study was to assess QT dispersion and corrected QT dispersion, and their relationship with systolic and diastolic function of the left ventricle in a group of children with Familial Mediterranean fever. We performed electrocardiography and Doppler echocardiography on patients and controls. Maximum QT, minimum QT, QT dispersion, corrected QT, maximum corrected QT, minimum corrected QT, and corrected QT dispersion intervals were measured from standard 12-lead electrocardiography. No statistically significant differences were found between the groups in QT dispersion, corrected QT dispersion, and systolic-diastolic function of the left ventricle parameters. During the 12 months of follow-up, no ventricular arrhythmias were documented in either group.

P wave dispersion in juvenile idiopathic arthritis patients with diastolic dysfunction.

OBJECTIVE: Cardiac involvement as pericarditis, myocarditis and valvular disease is common in juvenile idiopathic arthritis (JIA). However, there are few studies concerning systolic and diastolic functions of the left ventricle in children with JIA. P wave dispersion is a sign for the prediction of atrial fibrillation. A recent study found that rheumatoid arthritis patients had an abnormally high P wave duration and P wave dispersion, markers for supraventricular arrhythmogenicity. In this study, we aimed to evaluate P wave dispersion and its relation with diastolic dysfunction of the left ventricle in patients with JIA. METHODS: We performed electrocardiography and Doppler echocardiography on patients and controls. Maximum and minimum P wave duration were obtained from electrocardiographic measurements. P wave dispersion defined as the difference between maximum and minimum P wave duration was also calculated. FINDINGS: No statistically significant differences were found between the patients and controls in minimum, maximum P wave duration and P wave dispersion. Among the diastolic parameters in patients group, increased late flow velocity, decreased early flow velocity and prolonged isovolumic relaxation time reflected diastolic dysfunction. CONCLUSION: During 12 months of follow-up, no supraventricular arrhythmias were documented in JIA with diastolic dysfunction. JIA with diastolic dysfunction has normal atrial conduction parameters and therefore seemingly do not have an increased risk of atrial fibrillation.

Acquired origin of the left anterior descending coronary artery from the pulmonary artery: A complication of the arterial switch operation.

The prevalence of coronary anomalies in the transposition of the great arteries is high. Transfer of the coronary arteries during arterial switch operation is the principle step and incomplete transport of the coronary arteries to the neoaortic root results into iatrogenic coronary problems. We present a case with the residual left anterior descending coronary artery originating from the pulmonary artery as a complication of the failure of transfer during the arterial switch operation.

Sequence variations of NKX2-5 and HAND1 genes in patients with atrial isomerism.

OBJECTIVE: Atrial isomerism is a congenital disorder, which is characterized by lateralization defects in normally asymmetrical developing organs like the heart. Atrial isomerism is supposed to be caused by molecular defects during early development. The NKX2-5 is a cardiac specific transcription factor, which initiates and regulates downstream transcriptional cascades of cardiogenesis. The HAND1 is another transcription factor expressed in the heart, and it is characterized by an asymmetrical pattern of expression. In this study, we aimed to test whether mutations in NKX2-5 and HAND1 genes play a role in the etiology of atrial isomerism. METHODS: This case-control study consisted of 70 patients who underwent surgical treatment for congenital heart defects including atrial isomerism, 80 healthy subjects (HAND1 gene) and 40 healthy subjects (NKX2-5 gene). All exons and exon-intron boundaries of NKX2-5 and HAND1 genes were analyzed by SSCP, and suspected samples were sequenced for mutation analysis. Digestion with appropriate restriction enzymes was performed for analysis of known mutations and polymorphisms. The frequencies of the alleles and the genotypes were compared among patient and control groups using the Chi-square and the Fisher tests when appropriate. RESULTS: In intronic region of HAND1 gene, we identified a C>G substitution both in patients and controls. Frequency of mutant allele (11, 42%) was found higher (p=0.046) in patient group than that of the control group (2.5%). Association between atrial isomerism and genotypes with mutant allele was found borderline significant (p=0.054). In NKX2-5 gene, we identified heterozygous Q170X (Gln170ter) mutation in one patient. We did not found any correlation between defined sequence variations and clinical properties of the patients. CONCLUSION: Our results suggest that mutations or sequence variations in HAND1 or NKX2-5 genes may play role in etiology or pathogenesis of atrial isomerism.

Duration and dispersion of the P wave after the Senning operation.

We studied the duration and dispersion of the P wave in patients after a Senning operation, assessing its value in detecting the risk of atrial tachycardias.We measured the duration and dispersion of the wave in surface 12 lead electrocardiograms obtained from 18 patients with sinus rhythm, having a mean age of 12.8 years, with 13 being males and 5 females, who had undergone a Senning operation, comparing the values obtained in 35 age and gender-matched healthy people. The patients had undergone repair at a mean age of 13.4 months, and had a mean duration of follow-up of 12.8 years after the procedure. We also made 24 hour Holter recordings.The maximal duration of the P wave, at a mean of 129.3 milliseconds, and dispersion with a mean of 78 milliseconds, were both significantly increased in the patients compared with their controls, the mean values for the normal subjects being 103.7 and 54 milliseconds. Supraventricular tachycardia was detected in 1 of 3 patients with dispersion greater than 100 milliseconds, and in 2 of 15 patients (13%) with dispersion less than 100 milliseconds as measured from the Holter recordings (p > 0.05).Thus, the maximum duration and dispersion of the P wave were increased in patients after a Senning operation, but we were unable to establish any relationship between these measurements and atrial tachycardias as observed using Holter monitoring.

Left ventricular noncompaction associated with Ebstein's anomaly.

Left ventricular (LV) noncompaction is a congenital dysfunction of endomyocardial morphogenesis characterized by excessively prominent trabeculations and deep intratrabecular recesses of the myocardium. Its association with Ebstein's anomaly is very rare. A 13-year-old boy presented to the outpatient clinic for a cardiac evaluation before enrolling in a sports activity. On physical examination, there was a grade 1/6 systolic murmur at the tricuspid valve location. His electrocardiogram was normal. Transthoracic echocardiography revealed numerous large trabeculations and deep intratrabecular recesses at the apex, lateral wall, and the apical part of the interventricular septum. Color flow Doppler examination confirmed the presence of blood flow within the trabeculae. The size and ejection fraction of the LV was normal (65%). The attached margins of the septal and inferior leaflets of the tricuspid valve were apically displaced and there was mild tricuspid regurgitation. The distance between the mitral and tricuspid annuli was 20 mm. The right ventricle showed no dilatation and had normal systolic function, but the right atrium was moderately enlarged. As the patient was asymptomatic and the LV systolic function was preserved, he was scheduled for regular follow-up without medication except for low-dose aspirin (100 mg/day). After five years of follow-up, he was still asymptomatic with normal size and ejection fraction of both ventricles.

Outcome for patients with isolated atrial septal defects in the oval fossa diagnosed in infancy.

OBJECTIVE: Our aim was to investigate the change in diameter of holes within the oval fossa, and the role of aneurismal formation in reducing the size of the hole, in patients diagnosed during infancy with isolated defects in the floor of the fossa. PATIENTS: In a retrospective study, we included 100 patients diagnosed during the first year of life with an isolated defect in the floor of the oval fossa who had subsequently been observed for at least 5 years. There were 56 females and 44 males. They had been admitted to hospital because of a murmur in 65, heart failure in 9, and other reasons in 17. The remaining 9 patients were referred from other institutions with an established diagnosis of defects within the oval fossa. Patients were grouped according to the size of the deficiency in the floor of the fossa. Defects of diameter less than 5 mm were considered to be small, and 20 patients had such defects. Medium sized defects were judged to be between 5 and 8 mm, with 26 patients fulfilling this criterion, with the other 54 patients having large defects with diameters greater than 8 mm. RESULTS: The overall spontaneous rate of closure was 27%. Of those with medium defects, half closed spontaneously, but only 7.5% of those with large defects showed such closure. Of the patients who were diagnosed with heart failure, 9 had defects measuring 7 mm, and of these, 6 required surgical closure, 1 patient had spontaneous closure, while the defect became smaller in the remaining 2. On the other hand, in 9 out of 10 patients who had aneurysms, the diameter of the defect was between 7 and 15 mm. Amongst these patients, the defect closed spontaneously in 3, and reduced in size in the others. CONCLUSION: When holes within the oval fossa measure 8 mm or below, the majority of patients with experience either spontaneous closure or show decrease in size of the defect. In those with larger defects, the size usually increases, and surgery is needed for closure. If there is aneurismal formation, however, even when the defect measures more than 8 mm, the defect usually closes on its own or gets smaller.