Neonatal Outcomes of Critical Congenital Heart Defects: A Multicenter Epidemiological Study of Turkish Neonatal Society : Neonatal Outcomes of CCHD.

Critical congenital heart disease (CCHD) is one of the leading causes of neonatal and infant mortality. We aimed to elucidate the epidemiology, spectrum, and outcome of neonatal CCHD in Türkiye. This was a multicenter epidemiological study of neonates with CCHD conducted from October 2021 to November 2022 at national tertiary health centers. Data from 488 neonatal CCHD patients from nine centers were entered into the Trials-Network online registry system during the study period. Transposition of great arteria was the most common neonatal CHD, accounting for 19.5% of all cases. Sixty-three (12.9%) patients had extra-cardiac congenital anomalies. A total of 325 patients underwent cardiac surgery. Aortic arch repair (29.5%), arterial switch (25.5%), and modified Blalock-Taussig shunt (13.2%). Overall, in-hospital mortality was 20.1% with postoperative mortality of 19.6%. Multivariate analysis showed that the need of prostaglandin E1 before intervention, higher VIS (> 17.5), the presence of major postoperative complications, and the need for early postoperative extracorporeal membrane oxygenation were the main risk factors for mortality. The mortality rate of CCHD in our country remains high, although it varies by health center. Further research needs to be conducted to determine long-term outcomes for this vulnerable population.

Use of percutaneous carotid artery access for performing pediatric cardiac interventions: Single-center study.

BACKGROUND: Carotid artery (CA) access allows a more straight route for many left heart lesions. This has previously been achieved via a surgical cut-down approach in certain pediatric cardiac interventions. However, there are little data considering CA access in pediatric cases, percutaneously. AIM: We hypothesized that there would be notable improvements in efficiency as well as overall success when using the CA for access in selected cases. METHODS: Between November 2016 and January 2019, records of patients undergoing attempted percutaneous CA access under ultrasound guidance for cardiac catheterization were reviewed. RESULTS: Thirty patients underwent 36 catheterizations; median age 17 days (range, 6 days-9 months) and median weight 3.2 kg (1.2-7.8). Procedures performed were stenting or stent redilatation of the patent ductus arteriosus in 23, stenting or angioplasty of modified Blalock-Taussig shunts in four, aortic valvuloplasty in three, angioplasty for coarctation of the aorta in four, renal angioplasty in one, and diagnostic catheterization in one case. The intended intervention was unsuccessful in two patients despite successful CA access. Follow-up imaging showed a normal carotid in 28 of 30 (94%), with mild luminal narrowing with normal Doppler velocities in two instances. No patient had clinically apparent neurological sequelae attributable to CA access. CONCLUSIONS: Our data indicate that CA access should be employed when dealing with a select group of infants requiring vertical approach for left-sided cardiac lesions. Percutaneous CA access, even in very small preterm infants, is safe and feasible with negligible vascular injury and no neurological adverse events.

Surgical ductal stent implantation in total anomalous pulmonary venous connection to vena porta with right pulmonary sequestration in a mature newborn.

In many cases of total anomalous pulmonary venous connection (TAPVC), the four pulmonary veins (PV) join together behind the left atrium, where they form a collector. This collector can drain into the right atrium directly through the innominate vein into the superior vena cava (SVC), into the coronary sinus (CS), or through the diaphragm to the venous structures of the abdomen. In our case, a mature newborn had TAPVC draining into the vena porta along with severe pulmonary hypertension. Additionally, there were right pulmonary sequestration, dextrocardia, transposition of the great arteries, severe pulmonary stenosis, and single ventricular pathology in echocardiographic examination. Clinical signs manifested in the first 7 days of life. Diagnostic tools used were echocardiography and angiography. A ductal stent was surgically implanted into the ductus arteriosus by angiography. TAPVC was found to be nonobstructive. Therefore, we would like to emphasize the rareness and hardness to perform the surgical ductal implant technique in our particular case of TAPVC with pulmonary sequestration draining into the vena porta. The prognosis in TAPVC is poor and related mainly to the existence of pulmonary venous obstruction.

Ultrasound-Guided Femoral Arterial Cannulation in Neonates Undergoing Cardiac Surgery or Catheterization: Comparison of 0.014-Inch Floppy Versus 0.019-Inch Straight Guidewire.

OBJECTIVES: Percutaneous femoral artery cannulation can be technically challenging in small infants. DESIGN: We designed a prospective randomized trial to compare the use of two different guidewires for femoral arterial cannulation in neonates undergoing cardiac surgery or catheterization. SETTINGS: Cardiac ICU in a university hospital. PATIENTS: One-hundred twenty-four children were enrolled in this prospective study, with 64 being randomized to the 0.019-inch straight guidewire group and 60 to the 0.014-inch floppy guidewire group. INTERVENTIONS: Femoral artery cannulation. MEASUREMENTS AND MAIN RESULTS: The study period was limited to 10 minutes at the first site of arterial puncture. The time to complete cannulation, number of successful cannulation on first attempt, number of attempts, and number of successful cannulations were compared. The number of successful cannulations and successful cannulations on first attempt were higher in 0.014-inch floppy guidewire group (p = 0.001; p = 0.002, respectively). The time to complete cannulation was significantly shorter, and the number of attempts was lower in 0.014-inch floppy guidewire group (p = 0.001). Among the neonates less than 2000g, the number of attempts and time to complete cannulation were significantly lower (p < 0.001), and number of successful cannulation on first attempt and number of successful cannulations were significantly higher (p < 0.028; p < 0.001, respectively) in the 0.014-inch floppy guidewire CONCLUSIONS:: Using 0.014-inch floppy guidewire for femoral arterial cannulation in particularly very small neonates provides significant improvement in first attempt success, number of successful cannulations, number of attempts, time to complete cannulation.

Giant Aneurysm of the Ascending Aorta Requiring Emergency Repair in a Newborn Baby.

Congenital aneurysm of the ascending aorta is a rare cardiovascular pathology and usually associated with well-known connective tissue disorders. We present an idiopathic ascending aortic aneurysm extending to the aortic arch in an antenatally diagnosed newborn who required immediate surgical treatment due to the rapid progression of aneurysm size at the age of 1 day.

Stenting the vertical ductus arteriosus via axillary artery access using "wire-target" technique.

OBJECTIVES: To retrospectively review the outcome of stent placement in neonates with a vertical ductus, present a technique of ductal stenting via the axillary artery and compare it to ductal stening via the femoral venous access. DESIGN: Nineteen patients with duct-dependent pulmonary circulations through a vertical ductus arteriosus were treated with stent implantation. Those patients were retrospectively included in the study. In the first nine of these cases, stent delivery was done transvenously. In the latter ten cases, we favored the axillary artery access to the transvenous approach for stenting the vertical ductus arteriosus. Wire-target technique was used to attain access to axillary artery. RESULTS: PDA stenting was successful in six out of nine cases in whom the procedure was done anterogradely via the femoral vein and in all cases in whom the procedure was done via axillary artery (P = .047). Wire-target technique was used successfully in all ten cases in whom the procedure was done via axillary artery. Fluoroscopy time and total procedure time were significantly shorter in patients in whom the procedure was done via axillary artery (P < .001) CONCLUSIONS: Stenting of a vertical ductus arteriosus via the axillary artery using wire-target technique is feasible and safe in selected patients.

Transcatheter closure of ventricular septal defects using the Amplatzer Duct Occluder II device: a single-center experience.

INTRODUCTION: Off-label use of different devices has been described for percutaneous closure of ventricular septal defects (VSD) because of the unacceptable rate of post-procedure heart block associated with special VSD devices. AIM: To describe the early single-center clinical experience with closure of a VSD using the Amplatzer Duct Occluder II (ADO II) device in children. MATERIAL AND METHODS: Between May 2013 and June 2015, 26 patients between 13 days and 16 years of age underwent percutaneous closure of a VSD with an ADO II device at our institute. The decision to use the ADO II device was based primarily on lower risk of total atrioventricular block (TAVB) after VSD closure reported in the literature, reduction of the cost of the procedure and the need to close symptomatic VSDs in young patients. RESULTS: The location of the VSD was perimembranous in 21 patients, postsurgical residual in 2, midmuscular in 2 and apical muscular in 1. Complex procedures including newborn, combined procedures and postsurgical residual VSD were performed in 5 (19%) patients. The median duration of follow-up was 12 months. The complete VSD closure rate was 81% immediately after the procedure, 85% at 24 h, and 93% at the last follow-up. There was no device embolization. During the entire follow-up period, TAVB did not develop. CONCLUSIONS: The ADO II constitutes a safe and effective therapeutic alternative for morphologically varied VSDs in all pediatric age groups. A larger range of sizes and configurations of this occluder may be required to successfully occlude a wider range of VSDs.

Early predictive echocardiographic features of hemodynamically significant patent ductus arteriosus in preterm VLBW infants.

BACKGROUND: Patent ductus arteriosus (PDA) is associated with increased morbidity and mortality in very low-birthweight (VLBW) preterm infants due to significant left-to-right shunting, which leads to pulmonary edema/hemorrhage, intracranial hemorrhage, acute renal failure and necrotizing enterocolitis. In this prospective study, echocardiography was carried out in VLBW preterm infants soon after birth and at the end of 72 h to evaluate the relationship between early ductal anatomic features and significant ductal shunt during follow up. METHODS: Preterm infants with a gestational age ≤ 28 weeks, birthweight < 1000 g and who had ductal patency during the first 6-12 h of life underwent color Doppler echocardiograms through the first 3 days after birth. RESULTS: Fifty-eight patients were enrolled. The DA remained open in 42 preterm infants (72.4%) and was hemodynamically significant in 36 (62%) at the end of 72 h postnatal age. The preterm infants with hemodynamically significant PDA (hsPDA) had shorter ductal length from aortic to pulmonary insertion and from ductal constriction to pulmonary insertion in the initial exam. Cut-offs for these lengths were 5.2 and 1.7 mm, respectively. These parameters had significant univariate correlation with ductal closure time after treatment. CONCLUSIONS: Echocardiographic features such as short ductal length and short or absent ductal constriction area can be used to predict hsPDA for early decision making strategies in VLBW preterm infants.

The use of basic parameters for monitoring the haemodynamic effects of midazolam and ketamine as opposed to propofol during cardiac catheterization.

OBJECTIVE: Our aim is to compare the haemodynamic and adverse effects of propofol versus the mixture of midazolam and ketamine as used in sedation for cardiac catheterization in children. METHODS: In a prospective randomized trial, we divided patients needing sedation into 72 receiving a mixture of midazolam and ketamine and 42 receiving propofol. Their ages ranged from 6 months to 12 years, and 1 year to 16 years, respectively. We collected data relative to heart rate, mean arterial pressure, respiratory rate, peripheral saturations of oxygen, and adverse effects. We assessed cyanotic patients to establish any relationship between the haemodynamic data and peripheral arterial saturations of oxygen. RESULTS: Demographic data, including age, gender, and weight, was not statistically different between the groups. In those receiving midazolam and ketamine, mean systemic arterial pressures before, and 30 minutes after, sedation were 64.3, with standard deviation of 9.8, and 62.5, with standard deviation of 10.2, millimetre of mercury (p equals to 0.237). Heart rates were 131.3, with standard deviation of 13.5, and 109.2, with standard deviation of 17.3 beats per minute, (p less than 0.001) whereas in those given propofol the comparable values were 71.2, with standard deviation of 14.4, and 53.6 with standard deviation of 9.7 millimetres of mercury (p less than 0.001), and 115.2, with standard deviation of 13.6, and 100.5 with standard deviation of 20.1 beats per minute (p less than 0.01), respectively. Mean systemic arterial pressures in the subgroups of cyanotic patients before and 30 minutes after sedation were 74.8, with standard deviation of 14.6, and 72.7, with standard deviation of 12.4 millimetres of mercury for those receiving midazolam and ketamine (p equals to 0.544), and heart rates were 119.3, with standard deviation of 12.2, and 104.6 with standard deviation of 16.1 beats per minute (p equals to 0.001). In those given propofol, the comparable values were 71.1 with deviation of 15.5 and 53.9 with deviation of 9.2 millimetres of mercury (p equals to 0.001), and 126.7 with deviation of 20.8 and 107.2 with deviation of 13.5 beats per minute (p equals to 0.001), respectively. CONCLUSIONS: In cyanotic children, propofol used as a sedative agent during cardiac catheterization causes a decrease in mean arterial pressure and arterial desaturation. Ketamine produces more stable haemodynamic data in children with congenitally malformed hearts.

Tissue Doppler imaging in rheumatic carditis.

OBJECTIVE: Our study was undertaken to assess cardiac functions by tissue Doppler echocardiography in patient with primary episode of rheumatic carditis. METHODS: We divided 82 patients with rheumatic carditis were divided in two groups; 50 patients with mild and 32 patients with mitral regurgitation of grade two or more. A control group consisted of 30 healthy children free of any disease. All children underwent conventional and tissue Doppler echocardiography initially and at the time of the follow-up examination. RESULTS: Myocardial systolic wave velocity of the mitral annulus was significantly higher in patients with mitral regurgitation of grade two or more when compared to the control group, but was not different between patients with mild mitral regurgitation and healthy subjects at the time of the initial attack. Myocardial precontraction time, myocardial contraction time, and the ratio of myocardial precontraction and contraction times were significantly prolonged, and the systolic myocardial velocity of the mitral annulus was significantly decreased in patients with mitral regurgitation of grade two or more at the time of the follow-up examination. The myocardial systolic wave velocity was significantly lower, and myocardial precontraction time, myocardial contraction time, and the ratio of the precontraction and contraction times, were significantly longer or greater between patients with grade two or more mitral regurgitation and the control group at follow-up examination. CONCLUSION: We detected subclinical systolic dysfunction of the left ventricle in children with a primary episode of rheumatic carditis due to ongoing ventricular volume overload. Tissue Doppler imaging provides a quantifiable indicator useful for cardiac monitoring of disease during the period of follow up.

Long-term follow-up results of lung perfusion studies after transcatheter closure of patent ductus arteriosus.

OBJECTIVE: This study presents the long-term follow-up of patients who developed left lung perfusion (LLP) abnormalities following patent ductus arteriosus (PDA) closure with various device types. DESIGN: The study includes 23 adult and pediatric patients who had undergone transcatheter PDA closure and were shown to have decreased LLP (<40%) by the first scintigraphy performed within the average follow-up period of 14.0 ± 8.12 months (2.0-30 months). For PDA closure, the Amplatzer duct occluder was used in 12 patients, and coils were used in 11. Within the average period of 58.91 ± 12.93 months (37-85 months) after transcatheter PDA closure, a second lung perfusion scintigraphy was performed. RESULTS: In 13 out of 23 patients (56.5%), LLP improved by the time of the second scintigraphy. Improved and unimproved patients did not differ with regard to age, weight, body surface area, PDA diameter, ampulla diameter, and PDA length at the time of PDA closure and the second scintigraphy. There was no significant difference with regard to the percent of improved patients between the different device types (P =.88). The left pulmonary artery indexes were also insignificantly different (P =.446). Patients with persistent LLP abnormality had significantly higher average Doppler velocity index [(LPA blood flow velocity--RPA blood flow velocity) / MPA blood flow velocity] × 100 (P =.007) and PDA diameter/length. If Doppler velocity index ≥50% is taken as the cutoff value, it is possible to predict persisting LLP abnormality with 80% sensitivity and 76% specificity. Left lung perfusion abnormality was found to persist in patients with PDA diameter/length ≥0.5 with 80% sensitivity and 92.3% specificity. CONCLUSIONS: The LLP abnormalities seen after PDA closure with various devices eventually improve to normal in the majority of patients during long-term follow-up. Patients whose PDA length is shorter than its diameter are at risk of developing LLP abnormalities that persist long-term.

Ischemia-modified albumin levels in children with chronic liver disease.

BACKGROUND/AIMS: Ischemia-modified albumin (IMA) levels have been shown to correlate with the severity of liver failure in adults. However, the role of IMA levels has not been evaluated in children with chronic liver disease (CLD). We analyzed the clinical significance of IMA levels in children with CLD. METHODS: Thirty-three children with CLD and 33 healthy children were included in the study. Blood was collected to analyze biochemical parameters, oxidant status, and IMA. Liver biopsies were re-evaluated for liver fibrosis; severe fibrosis (SF) was defined as fibrosis stage ≥4. RESULTS: THE IMA AND AND IMA TO ALBUMIN RATIOS (IMARS) WERE SIGNIFICANTLY HIGHER IN CHILDREN WITH CLD THAN IN THOSE WITHOUT (IMA: 0.545±0.095 vs 0.481±0.062, p=0.003; IMAR: 0.152±0.046 vs 0.126±0.018, p=0.04). The IMAR was positively correlated with the pediatric end-stage liver disease score (p=0.03, r=0.503) and fibrosis score (p=0.021, r=0.400). Patients with SF had higher IMARs compared to patients with mild fibrosis (0.181±0.056 vs 0.134±0.025, p=0.003). The area under the receiver operation curve (AUROC) for predicting SF was 0.78 (p=0.006). Using a cutoff ratio value of 0.140, the sensitivity and specificity were 84% and 70%, respectively. The AUROC for predicting the need for liver transplantation and/or death was 0.82 (p=0.013). With a cutoff value of 0.156, the sensitivity and specificity was 83% and 82%, respectively. Kaplan-Meier analysis revealed increased morbidity and/or mortality in the group with an IMAR>0.156 (50% vs 4.3%, p=0.005). CONCLUSIONS: IMARs have been shown to provide important clues in predicting the fibrosis stage of the disease and determining the outcome in children with CLD.

Effects of salbutamol given by metered-dose inhaler on dispersion of ventricular repolarization.

OBJECTIVE: Salbutamol has previously been shown to increase the QT dispersion (QTd), which may be associated with high risk of cardiac arrhythmia in asthmatics. Cardiac effects of salbutamol occur in dose-related manner and salbutamol dose given by metered-dose inhaler (MDI) during acute asthma attack is commonly lower than the dose given by nebulizer. This prospective cohort study aimed to assess the effect of salbutamol given by MDI on QTd in the course of moderate acute asthma attack. METHODS: Thirty-two children, between 5-15 years of age, who were able to perform spirometric maneuvers and salbutamol administration by MDI through the spacer, were enrolled. Salbutamol was administered at a dose of 50 µg/kg three times at 15-20 minute intervals. Clinical features, spirometric parameters and QT measurements from the standard electrocardiograms were studied at baseline and 15 minute after the third inhalation of salbutamol. The relation between the continuous variables was evaluated by using paired Student's t-test. RESULTS: Overall, treatments were well-tolerated, significant improvement of pulmonary index scores and spirometric parameters were observed after treatment. No significant difference was observed between the pre and post-treatment values in QTd (30.4±5.6 ms; 33.7±6.2 ms, p=0.086) and corrected QTd (38.8±6.4 ms; 40.7±7.7 ms, p=0.18). CONCLUSION: Salbutamol administered using metered dose inhaler showed satisfying clinical improvement with notably lower doses than the dose given by nebulizer and does not affect ventricular repolarization in children with moderate acute asthmatic attack.

[Radionuclide evaluation of lung perfusion after transcatheter closure of patent ductus arteriosus: analysis of frequently used two different types of coils]. / Patent duktus arteriyozus'un kapatilmasi sonrasi akciger perfüzyonunun radyonüklit yöntemle degerlendirilmesi: Sik kullanilan iki farkli koil tipinin analizi.

OBJECTIVE: Impaired left lung perfusion (LLP) has been described after transcatheter closure of the patent ductus arteriosus (PDA). In this study, we aimed to evaluate lung perfusion scans (LPSs) following occlusion of PDA with two frequently used coils: Cook detachable coil and Gianturco coil. METHODS: A prospective study of 89 patients who underwent PDA occlusion using the Cook coils in 49 and the Gianturco coils in 40, was conducted. LPSs were performed after a median period of 18 months. The relationships between the LPSs and continuous variables were assessed by Pearson correlation analysis and the cut-off value of the best correlated parameters to predict abnormal LPSs obtained by the Receiver Operating Characteristic (ROC) analysis. RESULTS: Decreased left lung perfusion (LLP) was found in 13 patients including 10 after using Cook coils and 3 after using Gianturco coils (p=0.077). LLP values were significantly correlated with the loops deployed at the pulmonary side, coil/ductal diameter ratio and number of coils deployed (p=0.002, p=0.006 and p=0.009, respectively). Number of loops deployed at the pulmonary side >1.4 (specificity 77%, sensitivity 85%, area under the ROC curve 0.804, 95%CI 0.661-0.947, p<0.001) and first coil/ductal diameter ratio >1.85 (specificity 70%, sensitivity 77%, area under the ROC curve 0.747, 95%CI 0.423-0.790, p<0.005) were the best discriminative cut-off values of decreased LLP. CONCLUSION: Impaired LLP may appear following transcatheter closure of PDA with either Cook detachable coils or Gianturco coils. This situation may be complicated with loops deployed at the pulmonary side, the use of relatively large size of coil with respect to the ductal diameter and the use of multiple coils.

Relations between human leukocyte antigens and autoimmune hepatitis in Turkish children.

BACKGROUND/AIMS: We aimed to identify the genetic factors associated with increased tendency toward autoimmune hepatitis, a chronic and progressive inflammatory condition. METHODS: A total of 32 children diagnosed with autoimmune hepatitis were included in the present study, and 160 healthy adult blood donors served as controls. In both groups, HLA phenotypes were examined (HLA-A, B, C, DR, DQ) and compared. In addition, the association between the type of autoimmune hepatitis and HLA status was explored. RESULTS: Compared to controls, patients with autoimmune hepatitis had increased frequencies of the following class 1 HLA antigens: A24.9 (28% vs. 9%, p=0.007), A26 (25% vs. 3%, p<0.001), A32 (34% vs. 4%, p<0.001), B38 (9% vs. 0.6%, p=0.015), and B51 (16% vs. 0%, p<0.001). Among class II HLA antigens, DRB1*04 (22% vs. 0%, p<0.001), DRB1*07 (9% vs. 0%, p=0.004), DRB1*11 (12% vs. 0%, p=0.001), DRB1*15 (25% vs. 0%, p<0.001), DRB1*14 (31% vs. 0%, p<0.001), and DR11.5 (9% vs. 0%, p=0.004) were more frequent in patients compared to controls. Type 1 autoimmune hepatitis was associated with high frequencies of A24.9, A26, A32, and DRB1*15, whereas type 2 autoimmune hepatitis was associated with high frequencies of A26, B51, B38, and DRB1*11. On the other hand, frequencies of A32 and DRB1*04 were high among patients with unclassified autoimmune hepatitis. CONCLUSIONS: There seem to be associations between certain HLA antigens and susceptibility to autoimmune hepatitis, but variations among different geographical locations suggest a role for environmental factors.

[Closure of patent ductus arteriosus with duct occluder device in adult patients: evaluation of the approaches to facilitate the procedure]. / Eriskin hastalarda duktus tikayici cihaz ile patent duktus arteriyozus'un kapatilmasi: islemi kolaylastirici yöntemlerin degerlendirilmesi.

OBJECTIVE: The anatomic changes of the patent ductus arteriosus (PDA) in adult patients can complicate its transcatheter occlusion. The aim of the study was to determine procedural approaches to facilitate transcatheter closure of PDA in adult patients using the Amplatzer duct occluder (ADO). METHODS: In this prospective study, 33 consecutive adult patients with a moderate to large-sized PDA underwent transcatheter closure. Conventional antegrade approach was used in 14 patients, while retrograde wire-guided assisted approach in remaining 18 patients to access the PDA antegrade. If the initial procedure failed, snare-assisted technique was used to pass through the ductus antegradely. For comparative statistics, the Student's t-test, Mann-Whitney U test and Chi-square test were used as appropriate. RESULTS: Thirty-two patients had successful PDA occlusion. PDA ostium could be engaged in 10 of 14 patients by conventional approach, while 17 of remaining 18 patients by retrograde wire-guided assisted approach (p=0.07). PDAs could be passed antegradely using the snare-assisted technique in those five patients in whom the initial procedure failed. The mean size of implanted devices was 3.76 mm larger than the narrowest size of the PDA. Spontaneous embolization occurred in one patient in whom smaller device implanted due to his short ductus. All patients attained complete occlusion and there were no complications encountered during follow-up. CONCLUSION: The retrograde wire-guided technique is an effective approach and offers an alternative to access the PDA antegradely in adult patients. Larger than recommended size ADO device can be used safely in all adult patients with feasible ductal ampulla and may prevent device migration.

Lung perfusion studies after transcatheter closure of persistent ductus arteriosus with the Amplatzer duct occluder.

BACKGROUND: Reduced left lung perfusion has been described after transcatheter closure of the patent ductus arteriosus (PDA) with several prostheses. Although the Amplatzer ductal occluder (ADO) device is currently the most widely used occluder for closure of large-sized PDAs, the potential consequences of flow distribution to the lungs of this device have not been completely clarified. We evaluated lung perfusion following occlusion of PDA with the ADO device. METHODS: Forty-seven patients underwent successful transcatheter PDA occlusion using the ADO device were included in this study. Lung perfusion scans were performed 6 months after the procedure. RESULTS: Decreased perfusion to the left lung (defined as < 40% of total lung flow) was observed in 17 patients (36%), 5 of whom were low-weight symptomatic infants. Ductal ampulla length was significantly shorter and minimal ductal diameter to ampulla diameter ratio was significantly higher in patients with decreased left lung perfusion and correlated well with left lung perfusion values (r = 0.516 and r = -0.501, respectively). A cut-off value of ≤ 5.8 mm for the ductal ampulla length and ≥ 1.9 for ampulla diameter to ampulla length ratio showed high sensitivity and specificity for reduced lung perfusion. CONCLUSIONS: The incidence of abnormal left lung perfusion is high 6 months after transcatheter closure of PDA with the ADO, more likely in the low weight symptomatic infants and in patients with a short duct or a relatively shallow duct having abrupt narrowing of a large ampulla.

Schistocytic hemolytic anemia owing to central venous catheter in a child with acute lymphoblastic leukemia.

SUMMARY: A 4-year-old boy with acute lymphoblastic leukemia (ALL) was inserted a central venous catheter into right vena jugularis interna, whereas on BFM-ALL (Protocol 1) therapy. He developed progressive anemia, thrombocytopenia, schistocytes with triangular or crescent shapes, microspherocytes and teardrop cells in the peripheric blood smear, elevated lactate dehydrogenase, and decreased haptoglobin. Schistocytic hemolytic anemia was considered. On chest radiogram, the central venous catheter (CVC) tip was seen beneath the expected location. An echocardiography revealed that CVC was within the right atrium, in contact with tricuspite leaflets. So, CVC was overhauled and a new one was inserted. After the revision, hemolysis ceased; haptoglobin level normalized.

Wall motion velocities of ascending aorta measured by tissue Doppler imaging in obese children.

BACKGROUND: Obesity is associated with the development of early cardiovascular abnormalities such as atherosclerotic lesions. Arterial stiffness may be an indicator of early vascular changes signaling the development of vascular disease. The objective of the current study was to screen aortic elastic properties with tissue Doppler imaging in obese children. METHODS: We examined 37 obese children and 30 age- and sex-matched normal subjects. Anthropometric measurements and metabolic risk profile were assessed in a physical examination and with blood taking. The subjects were divided into two subgroups: those with and without metabolic syndrome. Internal aortic systolic and diastolic diameters by M-mode echocardiography and aortic systolic upper-wall tissue velocity by tissue Doppler imaging were measured 3 cm above the aortic valve. Aortic distensibility and aortic stiffness index were calculated using accepted formulae. RESULTS: Aortic stiffness parameters and both tissue Doppler peak systolic and diastolic velocities differed significantly in obese children compared to controls. Among the subgroups, children with metabolic syndrome had a lower aortic stiffness index, aortic distensibility and tissue Doppler velocities. However, only peak diastolic velocities significantly differed between obese children without metabolic syndrome and controls. Homeostatic model scores, diastolic blood pressures and pulse pressures were the strongest to correlate with peak diastolic velocity (r=-0.88, P < 0.001, r=-0.62, P= 0.001 and, r= 0.55, P= 0.001, respectively). CONCLUSIONS: Tissue Doppler imaging is a feasible and sensitive method to identify aortic stiffness in obese children. Reduced aortic diastolic velocity is the most prominent early vascular change detected in obese children before metabolic syndrome occurs.