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.

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.

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.

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.

Successful two-stage correction of ventricular septal defect and patent ductus arteriosus in a patient with fixed pulmonary hypertension.

We report a case of a 6-year-old boy with fixed severe pulmonary artery hypertension secondary to a ventricular septal defect (VSD) together with a patent ductus arteriosus (PDA). As a preliminary step, PDA embolization was performed following therapy with inhaled prostacyclin over a period of 6 months. Further, the patient underwent successful surgical VSD closure. We postulate that a staged procedure with long-term prostaglandin therapy might be capable of reducing pulmonary artery resistance and permitting total correction in a patient once considered to have inoperable pulmonary arteriopathy.

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.

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.

Figulla ASD occluder versus Amplatzer Septal Occluder: a comparative study on validation of a novel device for percutaneous closure of atrial septal defects.

OBJECTIVES: Occlutech Figulla ASD Occluder (FSO) is an alternative device to Amplatzer Septal Occluder (ASO) with some structural innovations including increased flexibility, minimizing the amount of material implanted, and absence of the left atrial clamp. We aimed to report our experiences with FSO and compare the outcomes of this novel device versus ASO. INTERVENTIONS: Between December 2005 and February 2009, 75 patients diagnosed with secundum atrial septal defects underwent transcatheter closure. The FSO device was used in 33 patients, and the ASO was used in 42. RESULTS: Patient characteristics, stretch size of the defect, device left disc size, procedure, and fluoroscopy time were similar between the groups. However, the difference between device waist size and stretched diameter of the defect was significantly higher, and device delivery sheath was significantly larger in FSO group and device left disc size was significantly lower in the FSO group. In all subjects, the residual shunt was small to trivial during follow-up and the reduction in prevalence of residual shunt with time was similar in both groups (P = 0.68). We found no differences in complication rate between the two devices; however, device embolization to the pulmonary bifurcation in one patient was recorded as major complication in FSO device group. CONCLUSIONS: Both devices are clinically safe and effective in ASD closure. FSO device has similar outcomes when compared to ASO device. Difficulties in selecting the correct device size in larger defects and larger venous sheath requirement need to be evaluated in further studies.

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.

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.

Correlation of abdominal fat accumulation and stiffness of the abdominal aorta in obese children.

AIM: To evaluate abdominal adipose tissue accumulation in obese children by ultrasound measurement and compare with the elastic properties of the abdominal aorta. CHILDREN AND METHODS: A total of 56 obese children and a control group of 30 non-obese children had an ultrasound examination for measurements of thickness of visceral, preperitoneal, and subcutaneous fat as well as abdominal aorta stiffness parameters. Anthropometric measurements and metabolic risk profile were assessed by physical examination and blood tests. RESULTS: Abdominal aorta stiffness parameters were all significantly higher in the obese children than in the controls. Among the adipose tissue compartments, visceral fat thickness was the strongest correlate of abdominal aorta stiffness parameters. Similarly, the correlation between visceral abdominal adipose tissue and fasting insulin levels, HOMA score, and pulse pressure also approached significance. CONCLUSIONS: This study suggests that abdominal adipose tissue accumulation is closely associated with cardiovascular risk factors in obese children, and among abdominal adipose tissue compartments, visceral fat thickness was strongly correlated with the elastic properties of the abdominal aorta.

[Coexistence of partial anomalous pulmonary venous drainage and pulmonary atresia with ventricular septal defect: a report of two cases]. / Ventriküler septal defektli pulmoner atreziye eslik eden kismi pulmoner venöz dönüs anomalisi: Iki olgu sunumu.

Coexistence of partial anomalous pulmonary venous drainage (PVD) and pulmonary atresia with ventricular septal defect (PA-VSD) is very rare with the exception of heterotaxy syndromes. We presented two cases of PA-VSD in which partial anomalous PVD was detected during pulmonary artery angiography. One was a six-month-old girl who underwent cardiac catheterization to evaluate the chance of unifocalization. There were true pulmonary arteries and three major aortopulmonary collaterals. It was noted that the right upper pulmonary vein was in direct continuity with the right atrium. The second case was a 12-year-old girl who was previously diagnosed as having PA-VSD and absence of true pulmonary arteries, for which she had undergone two subsequent aortopulmonary shunt operations four years before. During cardiac catheterization, confluent but hypoplastic true pulmonary arteries and multiple minor aortopulmonary collaterals were detected. The right and left upper pulmonary veins were draining to the right atrium. It should be known that, albeit very rare, partial anomalous PVD may be present in PA-VSD cases, requiring careful evaluation of pulmonary venous drainage during echocardiographic and angiographic studies.

Successful transcatheter balloon dilatation of coarctation of aorta and coil occlusion of patent ductus arteriosus in a single catheterization procedure.

Simultaneous transcatheter coil occlusion of patent ductus arteriosus and balloon angioplasty of coarctation of aorta is avoided since intimal tearing after angioplasty is believed to increase the risk for dissection of the aorta during catheter manipulation. We report a 14-month-old boy, with weight of 7.6 kg, with aortic coarctation and patent ductus arteriosus with a large left-to-right shunt who underwent successful simultaneous balloon angioplasty of native coarctation and coil embolization of the patent ductus arteriosus. Coarctation gradient decreased from 30 to 4 mmHg after balloon angioplasty and complete occlusion of the ductus arteriosus was achieved without any complication.

Stent implantation into the patent ductus arteriosus in cyanotic congenital heart disease with duct-dependent or diminished pulmonary circulation.

We assessed the efficacy and safety of stent implantation into the ductus arteriosus in infants with cyanotic heart disease. Ten patients with duct-dependent or diminished pulmonary circulation underwent cardiac catheterization with the aim of stent implantation. Coronary stents of 3.5-4 mm in diameter were used and successfully implanted in eight. All patients in whom stent implantation was successful had adequate relief of their cyanosis and were discharged home. During a mean follow-up of 9.3 months (median 10 months), one patient had evidence of restenosis of the stent at five months. Re-dilatation of the stenosed stent was attempted but was unsuccessful and a modified left Blalock-Taussig shunt was placed surgically. Stent implantation of ductus arteriosus in neonates and infants with a duct-dependent pulmonary circulation is an effective and safe palliative procedure. It may be an alternative to surgical systemic-pulmonary artery shunt in a select group of patients.

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.