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.

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.

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.

Case report: nonarrhythmogenic right ventricular dysplasia presenting with severe right ventricular failure in an adolescent.

Right ventricular dysplasia is usually discovered by the presence of ventricular arrhythmia. As arrhythmia is an epiphenomenon, the first presentation of some cases can be primarily heart failure. We describe an adolescent girl who presented with progressive right heart failure and whose hallmark was fibrofatty replacement of ventricular muscle, especially of the right side, without ventricular arrhythmia. The patient was successfully treated by orthotopic heart transplantation.

[Transcatheter closure of atrial septal defects in a patient with lipomatous hypertrophy of the interatrial septum]. / Interatriyal septumda lipomatöz hipertrofinin eslik ettigi atriyal septal defektlerin transkateter yolla kapatilmasi: Olgu sunumu.

Lipomatous hypertrophy of the interatrial septum is a rare benign pathology characterized by fatty deposits in the septum and is mostly diagnosed incidentally. This accumulation mostly causes a globular thickening of the interatrial septum, commonly sparing the fossa ovalis. We report on a 65-year-old female patient who underwent successful transcatheter closure of atrial septal defects (ASD) accompanied by lipomatous hypertrophy of the septum. Both transthoracic and transesophageal echocardiography showed enlargement of the right heart cavities, thickening of the interatrial septum (16 mm) with bright echogenicity, and two separate secundum ASDs measuring 17 mm and 4 mm, respectively. Transcatheter closure of the defects was performed using a 24-mm Amplatzer septal occluder. There was no residual shunt and Holter monitoring was normal after the procedure. During a three-year follow-up, no complications were observed pertaining to the procedure or lipomatous tissue.

Resistive index in febrile urinary tract infections: predictive value of renal outcome.

In the absence of specific symptomatology in children, the early diagnosis of acute pyelonephritis is a challenge, particularly during infancy. In an attempt to differentiate acute pyelonephritis from lower urinary tract infection (UTI), we measured intrarenal resistive index (RI). We evaluated its ability to predict renal involvement as assessed by dimercaptosuccinic acid (DMSA) scintigraphy. In total 157 patients admitted to the pediatric department of the Sisli Etfal Hospital with clinical signs of febrile UTI were included in the study. The children were divided into groups according to their age at the time of ultrasonography (US). RI was measured from the renal arteries with Doppler US in the first 72 h in all 157 children. Renal involvement was assessed by (99m)Tc-DMSA scintigraphy in the first 7 days after admission. The examination was repeated at least 6 months later if the first result was abnormal. All available patients with an abnormal scintigraphy underwent voiding cystourethrography 4-6 weeks after the acute infection. All patients with vesicoureteral reflux and scarred kidneys were excluded from the study. DMSA scintigraphy demonstrated abnormal changes in 114 of 157 children and was normal in the remaining 43 children. Of these 114 children, 104 underwent repeat scintigraphy, of whom 77 showed partially or totally reversible lesion(s). Of these 77 children, 17 children (22%) with vesicoureteral reflux were excluded. Thus, we compared the 43 children with lower UTI with the 60 children with definite acute pyelonephritis at admission. Kidneys with changes of acute pyelonephritis had a mean RI of 0.744+/-0.06 in infants, 0.745+/-0.03 in preschool children, and 0.733+/-0.09 in patients of school age with upper UTI. However, the mean RI was 0.703+/-0.06 in infants, 0.696+/-0.1 in preschool children, and 0.671+/-0.09 in school-aged patients with lower UTI. The mean RI values were significantly higher in patients with upper UTI ( P<0.001). There was a highly significant correlation between RI values and the severity of the renal lesion as ranked by DMSA scintigraphy ( P<0.001). When the cut-off RI value was 0.715, there was an 80% sensitivity and a 89% specificity for diagnosing upper UTI. Refluxing kidneys and scarred kidneys also had higher RI values. In conclusion, RI values were increased significantly in children with febrile UTI when renal parenchymal involvement (assessed by DMSA scintigraphy) was present. Our results also support the view that the children with high RI values are at a high risk of reflux, scarring, or both, which was frequently observed in febrile UTI. This might allow identification of patients at risk for severe renal lesions that require more aggressive therapy, investigation, and follow-up than those with lower UTI.