Aortic balloon valvuloplasty and mid-term results in newborns: a single center experience.

BACKGROUND AND OBJECTIVES: Aortic balloon valvuloplasty (ABV) has become the first-line treatment for critical aortic valve stenosis in infants. We aimed to evaluate the short- and mid-term results of patients who underwent ABV during neonatal period, the factors affecting the success and complications of the procedure. METHODS: We retrospectively examined 65 patients who underwent ABV during the neonatal period between 1998 and 2017. All hospital records including cardiac catheterization reports, echocardiographic information, and angiographic views were reviewed. RESULTS: Forty five (69.2%) of the patients were male and mean follow-up was 6.2 ± 4.9 years (range: 6 months - 19 years). The mean age of the patients at the first ABV was 14.5 ± 10.6 days (range: 1-30 days) and body weight was 3.25 ± 0.6 kg (range: 1.5-4.8 kg). The peak systolic gradient measured during pre-valvuloplasty cardiac catheterization was 73.3 ± 22.7 mmHg (range: 30-142 mmHg), and it decreased to 29.2 ± 12.2 mmHg (range: 5-55 mm Hg) after the procedure. Valvuloplasty was successful in 59 (90.7%) patients. There was no more than mild aortic regurgitation in any patient before valvuloplasty. There was mild aortic regurgitation in 21 patients before the valvuloplasty. In the acute phase after valvuloplasty, 30 patients had mild, 15 had moderate and two had severe aortic regurgitation. There was a significant increase in the degree of aortic regurgitation related to valvuloplasty (p < 0.05). The most important complication of ABV was increased aortic regurgitation (26.2%). Another important complication was femoral artery occlusion; and was detected early after valvuloplasty (61.6%). There was no serious complication or death in the acute phase. CONCLUSIONS: In newborns with valvular aortic stenosis, balloon valvuloplasty has become the first choice in many centers due to its high success rate, low mortality and morbidity, and increased clinical experience. Aortic regurgitation and femoral artery occlusion were the most important complications. Although reintervention for residual or recurrent aortic valve stenosis is common during the first year after valvuloplasty, these patients are able to reach advanced ages without the need for surgical intervention. Surgical valvotomy is a good alternative treatment for a small number of patients in whom valvuloplasty fails.

Complications of cardiac catheterization in children with congenital heart disease.

Tokel K, Gümüs A, Ayabakan C, Varan B, Erdogan I. Complications of cardiac catheterization in children with congenital heart disease. Turk J Pediatr 2018; 60: 675-683. Catheterization procedures for congenital heart disease include a broad range of procedures with a large spectrum of potential adverse outcomes. We aimed to determine the incidence of various complications during pediatric cardiac catheterizations and to designate the relative risk factors for such complications. All pediatric patients undergoing cardiac catheterizations between January 2005-December 2010 were included. Data are collected prospectively by filling out computerized catheterization reports. Patient records were scanned for potential risk factors retrospectively. Groups were divided based on cardiac diagnosis; type of procedure. Adverse events were categorized into major or minor events. A total of 2662 cardiac catheterizations were performed during this period. The mean age of patients was 53.2±64.3 months. Diagnostic catheterizations were done for 1797 (67.5%) patients, and interventional procedures were done in 865 (32.5%) cases. Adverse events were observed in 688 patients (26%) during 941 procedures. Minor and major events were seen in 21.4% and 7.1% of the procedures respectively. Most frequent major complications were anesthesia related (6%), most frequent minor complications were vascular complications (45.2%). Complications were more frequent in younger patients (p=0.0001), during interventional procedures (p=0.0001). Thirteen patients died after a cardiac catheterization; they were younger and had longer procedures compared to those who survived (p=0.0001). Vascular complications were frequent among younger patients, with prolonged procedure time and vessel access (p < 0.0001). Cyanotic patients had more complications (p < 0.05; OR for major and minor complications: 3.5 and 2 respectively). Minor complications were 2.7 times more likely in ventricular outflow obstructions (p < 0.05). The complication rates of cardiac catheterization in children are low, but not negligible. Defining risk factors will help anticipate adverse events, which will guide in preparation for rescue procedures and improvement of patient safety systems in catheterization laboratories.

Ictal electrocardiographic changes in children presenting with seizures.

BACKGROUND: The aim of this study was to determine electrocardiographic changes in children during seizures. METHODS: We assessed heart rate changes, RR intervals and QT changes during 47 seizures in children. Consecutive QT and RR intervals were measured for 60 s before the seizures, during the seizures and 60 s after the seizures during video electroencephalography monitoring. RESULTS: There were 47 seizures in 18 patients. Five patients had generalized seizures and 13 patients had focal seizures. Twelve patients were male. The mean age during monitoring was 10.1 years (range 4 months-19 years). Ictal tachycardia was seen in every seizure. No ictal bradycardia was noted. There was only one dropped beat in a patient. The mean ictal heart-rate-corrected QT (QTc) interval was significantly higher than the postictal measurements (P= 0.005). Mean ictal QTc variation tended to increase during seizures and then decreased below the pre-ictal measurements in the postictal period. However these changes were statistically insignificant (P > 0.05). RR variance was significantly decreased during seizures compared to the postictal period and the standard deviation of the RR intervals was significantly decreased in the ictal period compared to the pre-ictal period (P= 0.014 and P= 0.001, respectively). CONCLUSION: Tachycardia is the main finding in seizures in children. Ictal bradycardia and cardiac arrhythmias are very rare despite being more frequent in adults with seizures.

A prospective study of temporal course of phrenic nerve palsy in children after cardiac surgery.

The aim of the study is to determine the temporal course of phrenic nerve palsy in children after cardiac surgery. Prospective electrophysiological measurement of phrenic nerve latencies and diaphragmatic action potential (DAP) amplitudes before and after operation were recorded in 59 children who underwent open heart surgery for congenital heart disease. In patients with phrenic nerve dysfunction, the procedure was repeated at 1 week and 2 weeks after the operation to determine the temporal course. In the early postoperative period, 12 patients had abnormal left phrenic nerve latencies and/or DAP amplitudes, 11 had abnormal right phrenic nerve latencies and/or DAP amplitudes, and 3 had abnormal bilateral phrenic nerve latencies and/or DAP amplitudes. After 2-week follow-up, only one of these patients had persistent left phrenic nerve palsy. Prolonged phrenic nerve latencies and decreased DAP amplitudes often occur in the early postoperative phase in children who undergo cardiac surgery. This palsy is usually transient, and electrophysiologic studies should be repeated at least up to 1 week after surgery before diaphragmatic plication is considered.

Fenestration between the left atrial appendage and left superior vena cava in Kawashima operation.

The benefits of a baffle fenestration in essentially high-risk Fontan patients have been demonstrated. Described here is the use of a new fenestration between the left atrial appendage and the left superior vena cava after Kawashima operation in a patient with a double-outlet right ventricle with hypoplastic left ventricle, left atrial isomerism, bilateral superior vena cavae with no bridging vein, an interrupted inferior vena cava, and continuation of the hemiazygos vein to the left superior vena cava.

Thrombosis in children with cardiac pathology: analysis of acquired and inherited risk factors.

The present study was conducted to analyze the features and risk factors of childhood thrombotic events in patients with cardiac defect followed-up at our hospital. The clinical and laboratory findings of 59 patients diagnosed with cardiac defects and thromboses between 1997 and 2006 were retrospectively analyzed. Thirty-one children (52.5%) had venous system thromboses, 21 (35.6%) had arterial system thromboses, and seven (11.9%) had venous and arterial system thromboses. Presence of congenital heart disease and cardiomyopathy (CMP) were significant risk factors for developing intracardiac thrombosis. In addition, presence of congenital heart disease was the significant statistical risk factor for developing left atrium and right ventricle thromboses. Presence of congenital heart disease was a significant risk factor for developing a central nervous system thrombosis. Presence of pulmonary stenosis and aortic coarctation were significant risk factors for developing a peripheral arterial system thrombosis. Acquired risk factors including major surgery, angiography, central venous catheter, systemic infection, and hypoxia were identified in 49 of the 59 patients. Many patients had more than one of these acquired risk factors. Analysis of the relationship between thrombosis and type of major surgery demonstrated a statistically significant relationship between an intracardiac thrombosis and total correction of tetralogy of Fallot and a peripheral venous system thrombosis and a Blalock Taussig shunt. Twenty-three of the 52 patients (44.2%) had at least one thrombophilic mutation. Overall, a heterozygous factor V Leiden mutation was found in nine patients (17.3%), a methylenetetrahydrofolate reductase 677C-T mutation in 15 patients (28.8%), and a PT 20210G-A mutation in three patients (5.8%). Our data suggest that cardiac defects are common risk factors for developing a childhood thrombosis. The type of disorder determines the site of thrombosis. Acquired risk factors may contribute to the development of a thrombosis. The results of this study also indicate that to ensure early diagnosis, routine screening for thrombosis should be performed in patients with a cardiac defect and that screening for factor V Leiden and PT 20210G-A mutations and other genetic risk factors should be included when assessing all patients with cardiac defects who present with a thrombosis, whether or not a predisposing factor has been identified.

Thrombocytosis in the neonatal intensive care unit: Experience at a single center.

Although thrombocytosis is frequently detected in newborns, few reports have focused on its risk factors. This report documents the characteristics of 89 neonates with thrombocytosis followed up in a neonatal intensive care unit (NICU). We reviewed the patients' medical and laboratory records retrospectively to determine the associated conditions and risk factors for neonatal thrombocytosis, and complications related to thrombocytosis. We also discussed the differences of neonatal thrombocytosis from that of childhood in the light of literature. The mean platelet count of these newborns was 579.7 ± 111.5 (451-936) x 109/L. Associated conditions included anemia (73.7%), high- isk pregnancies (56. %), prematurity (51.7%), infections (37.1%), antenatal drug use (22.7%), indirect hyperbilirubinemia (20.2%), cardiac disorders (14.6%), respiratory distress syndrome (14.6%), history of hypoxia (13.5%), surgery (12.4%), and hemorrhage (3.4%). In most cases, more than one risk factor for thrombocytosis existed in the same newborn. No hemorrhagic complications related to thrombocytosis were observed; however, 1 newborn had portal vein thrombosis associated with intestinal malrotation. In summary, risk factors and associated conditions for thrombocytosis in newborns differed from those in children. In light of associated disorders, the mechanism of thrombocytosis in newborns may differ from that of childhood thrombocytosis.

Ventricular arrhythmia and tetralogy of Fallot repair with transannular patch.

OBJECTIVE: Life-threatening arrhythmias and sudden death remain to be serious late complications after correction of tetralogy of Fallot. The aim of this study was to detect ventricular arrhythmia incidence and to find out the relationship between ventricular arrhythmia and the transannular and infundibular patch repair techniques to correct tetralogy of Fallot. That is still unpredictable exactly. METHODS: Thirty-nine patients with mean age of 12.1+/-3.1 years were studied prospectively for 7.1+/-2.1 years after operation. They were all investigated with electrocardiography, echocardiography, treadmill and Holter monitorization. Right ventricular functions, exercise capacity and arrhythmia patterns were assessed. Lown criteria were used for grading the arrhythmia patterns. QRS duration, QT dispersion and QT dispersion indexes were calculated. RESULTS: Follow up time was 5 to 13 years in both groups. All QT dispersion times and indexes were within normal ranges and there were no differences between two groups. Holter and treadmill studies did not reveal during any ventricular arrhythmia risk in the study and control groups. CONCLUSION: Seven years of follow-up after correction of tetralogy of Fallot revealed that transannular patch reconstruction is not a cause of tendency for ventricular arrhythmia according to Lown criteria, QT dispersion, QT dispersion indexes and QRS duration do support the results of previous studies.

Topical EMLA cream versus prilocaine infiltration for pediatric cardiac catheterization.

OBJECTIVE: The aim of this study was to compare the anesthetic efficacy of prilocaine infiltration and a eutectic mixture of local anesthetics (EMLA) in cream for femoral vessel catheterization during pediatric cardiac catheterization and to evaluate whether EMLA cream application improves cannulation success. DESIGN: Prospective, randomized clinical trial. SETTING: A university hospital. PARTICIPANTS: Forty American Society of Anesthesiologists class III and IV children scheduled for cardiac catheterization via the femoral route were included. INTERVENTIONS: The children were randomly assigned to 2 groups. The EMLA group (n = 20) had EMLA cream applied to the groin 60 minutes before the procedure, and the control group (n = 20) had prilocaine infiltrated at the site 5 minutes before the procedure. Boluses of intravenous midazolam, 0.1 mg/kg, and/or ketamine, 1 mg/kg, were given to achieve and maintain a predetermined sedation score of 2-3 (0 = deeply sedated, 5 = agitated) throughout the procedure (sedation monitored every 5 minutes). The groups were compared with respect to demographic data, hemodynamic and respiratory parameters/complications, amounts of additional sedative-analgesics required, cannulation time, and cannulation results (first-attempt success [right groin], second-attempt success [left groin], or failure on both attempts). Each group's "overall cannulation success rate" was calculated as the proportion of cases in which cannulation was achieved on the first or second attempt. MEASUREMENTS AND MAIN RESULTS: The demographic data and the group findings for hemodynamic and respiratory parameters/complications, additional amounts of sedative-analgesics needed, cannulation times, and overall cannulation success rate were similar. The mean sedation score during femoral puncture in the EMLA group was significantly lower than that in the control group (3 +/- 1 v 4 +/- 1, respectively, p = 0.001). There were no other significant differences between the groups with respect to sedation scores during the procedure. The respective frequencies of first-attempt cannulation success in the EMLA and control groups were 75% and 45% (p = 0.05). CONCLUSION: The study showed that EMLA cream provides adequate topical anesthesia for femoral vessel cannulation during pediatric cardiac catheterization and may also increase the likelihood of cannulation success. However, use of this cream has no effect on sedative-analgesic requirements or on the risks of hemodynamic and respiratory complications during this procedure.

Assessment of left ventricular diastolic function by Doppler tissue imaging in children with end-stage renal disease.

AIM: To evaluate left ventricular (LV) diastolic function in children with end-stage renal disease (ESRD) using conventional pulsed-Doppler echocardiography and Doppler tissue imaging (DTI), and to compare the findings with these two modalities. METHODS: Twenty-four children with ESRD and 22 healthy age- and sex-matched control subjects were assessed with conventional Doppler echocardiography and DTI. The scans of the renal disease patients were done after a dialysis session. Parameters related to LV systolic and diastolic function were compared in the ESRD and control groups. RESULTS: The ESRD patients had lower mean mitral E/A ratio both according to conventional Doppler echocardiography and TDI than the control subjects. The ESRD group also had significantly longer isovolumetric relaxation time (116+/-31 ms vs 97+/-3.1 ms, respectively; p<0.001), and significantly longer deceleration time (235+/-44 ms vs 202+/-35 ms, respectively; p<0.01) than the control group. CONCLUSION: DTI findings correlate well with conventional Doppler echocardiography findings. Children with ESRD show, after dialysis, echocardiographic signs of LV diastolic dysfunction.

Effect of congenital heart disease on renal function in childhood.

BACKGROUND: Nephropathy is a well-known complication of congenital heart disease (CHD), and the risk of developing renal impairment is particularly high in patients with cyanotic CHD. Most investigations of renal impairment in CHD have involved patients 20 years and older. This study investigated renal tubule function in pediatric patients with CHD, and compared findings in cyanotic and acyanotic groups. METHODS: Twenty children with acyanotic CHD, 23 children with cyanotic CHD, and 13 healthy children were enrolled. Blood and early morning urine samples were collected from each subject to measure urinary concentrations of sodium, microalbumin, creatinine, beta(2)-microglobulin, and N-acetyl-beta-D-glucosaminidase (NAG). RESULTS: The age and sex distributions in the three groups were similar. Median fractional excretion of sodium (FeNa) and urinary NAG/creatinine were significantly higher in the cyanotic group than in the control group (p = 0.022 and p = 0.002, respectively). There were no statistically significant differences among the groups with respect to urinary beta(2)-microglobulin/creatinine, urinary microalbumin/creatinine or glomerular filtration rate. CONCLUSION: Tubular injury can be detected before glomerular injury occurs even within the first decade of life in patients with cyanotic CHD.

Endobronchial stenting in a two-month-old infant with bronchial compression secondary to tetralogy of Fallot and absent pulmonary valve.

Bronchial compression due to pulmonary artery dilation is an important problem in infants with congenital heart disease, and can complicate the postoperative course. In recent years, airway stenting has become a popular treatment for these cases. We achieved success with endobronchial stenting in a two-month-old infant with bronchial compression caused by a dilated pulmonary artery.

Dysplastic changes in the peripheral blood of children with microdeletion 22q11.2.

Myelodysplasia refers to abnormal morphology of the bone marrow and/or peripheral blood. This condition is frequently due to myelodysplastic syndrome; however, myelodysplasia is caused by a variety of factors and is not always a sign of pre-malignant disease. We observed myelodysplastic changes in peripheral blood smears and bone marrow specimens from a patient who had chromosomal microdeletion 22q11.2 (del22q11.2). We then investigated such changes in several other patients who were newly diagnosed with del22q11.2 (n = 5 total, including the index case) and compared the findings to those in four sets of controls without this chromosomal abnormality. Specifically, the controls were children with conotruncal heart defects (n = 3); otherwise healthy children with bacterial (n = 4) or viral infection (n = 4); and healthy children (n = 4). The myelodysplasia scores in the myeloid cells and eosinophils of the children with del22q11 were higher than those in all four of the control groups. Myelodysplastic changes in the peripheral blood of children with del22q11.2 have not been reported previously. We believe that certain gene(s) in the deleted region may be responsible for the myelodysplastic changes that we observed in our patients with this chromosomal abnormality.

[Assessment of atrial pathologies in children using transesophageal echocardiography]. / Cocuklarda Atriyuma Ait Patolojilerin Transözofajiyal Ekokardiyografi ile Degerlendirilmesi.

OBJECTIVE: Transesophageal echocardiography (TEE) is indicated for suspected atrial septal pathology and for monitoring of interventional procedures such as an atrial septal defect (ASD) closure during cardiac catheterization. Transesophageal echocardiography also helps to demonstrate postoperative complications and residual defects of complex congenital cardiac anomalies. METHODS: Transesophageal echocardiography was performed in 112 pediatric patients with or suspected atrial pathology at our institution between 1999-2002, using the standard techniques. The mean age was 8.7+/-4.2 years. RESULTS: In 45 of 112 children the suspected atrial defects were confirmed with the TEE. Patent foramen ovale was correctly predicted in 13.4% of patients by TEE, but only in 8.7% of patients by echocardiography. Multiple ASD's were correctly defined in 4.1%, and high venosus defects were documented in 6.1% of children by the TEE. We used TEE in 13% of patients for detecting atrial vegetations in patients with possible endocarditis, and evaluation of the postoperative care of atrial surgery such as Fontan or Senning operations and total correction of abnormal pulmonary venous return. Successful transcatheter closure of 7 ASD's was accomplished under TEE guidance. CONCLUSION: Transesophageal echocardiography allows a much more detailed evaluation of atrial morphology than transthoracic echocardiography even in infants. Transesophageal echocardiography is also indicated during interventional procedures and postoperative evaluation of the atrial pathology.

Chromosome 22q11.2 deletion and phenotypic features in 30 patients with conotruncal heart defects.

This report describes the dysmorphic features and frequency of 22q11.2 deletion (del22q11) in 30 Turkish patients with conotruncal heart defects (CTHDs). Fluorescence in situ hybridization (FISH) analysis revealed deletions in the 22q11.2 region in nine (30%) individuals. The CTHDs in this group were tetralogy of Fallot (four cases), double-outlet right ventricle (DORV) (two cases), transposition of great arteries (two cases), and ventricular septal defect (VSD) associated with other CTHDs (one case). The frequency of del22q11 in the study group was relatively high because many of the patients with dysmorphic findings also had cardiac anomalies involving the pulmonary artery, ductus arteriosus, or the aortic arch and its main branches. Twenty of the 30 patients exhibited several dysmorphic findings. Two of the nine patients with del22q11 exhibited no apparent dysmorphic features other than sacral dimple. Interestingly, one of the patients with del22q11 had a phenotypic appearance similar to that seen in oculo-auriculo-vertebral spectrum (OAVS). This individual had left microtia, atresia of the external meatus, mandibular asymmetry, and peripheral facial nerve paralysis. His mental development was normal and there were no abnormalities on ophthalmological examination. The CTHDs in this patient were situs inversus dextrocardia, DORV, pulmonary stenosis, and VSD. Radiographs of this patient showed platybasia, complete fusion of C2-C3, and posterior fusion of the T1-T2 vertebrae. This particular case indicates that the phenotypic features of del22q11 and OAVS may overlap.

Co-occurrence of chromosome 22q11.2 microdeletion and trisomy 21 mosaicism.

This report describes a patient who had some phenotypic features of Down syndrome (DS) as well as severe conotruncal cardiac anomalies, including pulmonary atresia with ventricular septal defect (tetralogy of Fallot with pulmonary atresia), confluent pulmonary arteries, a large left-sided ductus arteriosus, left aortic arch, aberrant right subclavian artery, and secundum atrial septal defect. Cytogenetic and fluorescence in situ hybridization (FISH) analysis was carried out on peripheral blood lymphocytes and skin fibroblasts using probes specific for the chromosomal loci 21q22.13 to 21q22.2 and locus 22q11.2. This revealed 47,XX+21/46,XX mosaicism at a rate of 15:85 and the micro-deletion 22q11.2 (del22q11.2). Some patients'congenital cardiac anomalies are atypical for the type of mosaicism or aneuploidy. The case suggests that association of del22q11.2 should be considered in patients with chromosomal mosaicism or aneuploidy who also have particular conotruncal cardiac defects.