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.

Anomalous origin of coronary arteries from the pulmonary artery: A single-center experience.

Background: The aim of the study was to evaluate the clinical and diagnostic findings, treatment, and follow-up of cases of anomalous origin of coronary arteries from the pulmonary artery. Methods: Between January 1998 and June 2021, a total of 14 patients (5 males, 9 females; median age: 15 months; range, 3 to 156 months) diagnosed with anomalous origin of coronary arteries from the pulmonary artery were retrospectively analyzed. Demographic and clinical data of the patients, electrocardiographic, echocardiographic, angiographic, surgical, and follow-up findings were evaluated. Results: The most common symptoms were respiratory distress (n=6) and murmur (n=3). With the exception of three cases, all other patients were diagnosed by echocardiography in the first examination. Severe mitral valve insufficiency was detected in four patients and four other patients had moderate mitral insufficiency on echocardiography. Ejection fraction values ranged between 38 and 79%. Eleven patients underwent direct implantation of the coronary artery into the aorta, and three underwent a Takeuchi procedure. Mortality occurred in only one case. After surgery, mitral insufficiency and ejection fraction values improved. Median follow-up was 62 (range, 5 to 170) months and all patients were asymptomatic, except one who required redo surgery. Conclusion: Anomalous origin of the coronary arteries from the pulmonary artery is an uncommon congenital anomaly. Echocardiography is the main diagnostic tool. If all echocardiographic findings of the coronary anomaly are not investigated thoroughly, the diagnosis may be overlooked.

Camptodactyly-Arthropathy-Coxa Vara-Pericarditis Syndrome Resembling Juvenile Idiopathic Arthritis: A Single-Center Experience from Southern Turkey.

Camptodactyly-arthropathy-coxa vara-pericarditis (CACP) syndrome, caused by biallelic pathogenic mutations in the PRG4 gene, is characterized by early-onset camptodactyly, noninflammatory arthropathy, coxa vara deformity, and rarely, pericardial effusion. Herein, we report 3 patients with CACP syndrome from 2 unrelated families. All patients are female, born to consanguineous parents, and had camptodactyly since the first years of their lives. Two patients had a prior diagnosis of juvenile idiopathic arthritis. Hip changes were present in 2 patients, and 2 of 3 patients had undergone surgery for camptodactyly. Routine echocardiographic evaluations were normal during the 2-year follow-up. This paper represents the third study including CACP patients from Turkey. Clinically, all 3 patients resembled juvenile idiopathic arthritis cases and received unnecessary medication. There is also an ongoing need for improving awareness of CACP and an effective treatment focusing on the lubrication of the joint space in CACP patients.

What are the non-cardiac prognostic factors affecting mortality in neonates with aortopulmonary shunt.

Background/aim: Systemic to pulmonary shunts (SPS) have proven to be highly effective for the palliation of neonates with cyanotic congenital heart disease. Mortality after SPS surgery in neonates has multifactorial basis. We aimed to investigate the clinical results of the SPS in relation to the underlying cardiac disease and to identify the risk factors contributing to an adverse outcome.Material and method: All neonates who underwent first shunt insertion for cyanotic congenital heart disease during the study period from 1 January 2014 to 31 December 2017 were included. A retrospective review of patient records was done. Patients were grouped into two different categories: survived with or without any reintervention and death before or after any reintervention till discharge.Result: During the study period, 47 patients underwent SPS shunt placement. Patients who survived with or without any reintervention were in Group 1 and patients who died before or after any reintervention till discharge were in Group 2. Preoperative epinephrine requirement and mechanical ventilation and postoperative erythrocyte transfusion need were statistically significant.Conclusion: Although primary cardiac pathology is the most important prognostic factor, some other preoperative and postoperative factors like preoperative epinephrine requirement, and postoperative erythrocyte transfusion might also affect the prognosis. As there are very few centers in the region that specialize in pediatric cardiac surgery, a multicenter approach will be helpful in reaching reliable conclusions.

Acute rheumatic fever in south-east of Turkey: clinical features and epidemiological evaluation of the patients over the last 25 years.

This study evaluates clinical and epidemiological features of acute rheumatic fever using the data of last 25 years in our hospital in south-east of Turkey. The medical records of 377 patients with acute rheumatic fever admitted to Pediatric Cardiology Department of Çukurova University during 1993-2017 were retrospectively analysed. Two hundred and six patients were admitted between 1993 and 2000, 91 between 2001 and 2008, and 80 between 2009 and 2017. The largest age group (52%) were between 9 to 12 years of age and approximately two-thirds of the patients presented in the spring and winter seasons (62.8%). Among the major findings, the most common included carditis 83.6% (n = 315), arthritis at 74% (n = 279), Sydenham's chorea at 13.5% (n = 51), and only two patients (0.5%) had erythema marginatum and two patients (0.5%) had subcutaneous nodule. Carditis was the most common manifestation observed in 315 patients (83.6%). The most commonly affected valve was the mitral valve alone (54.9%), followed by a combined mitral and aortic valves (34%) and aortic valve alone (5.7%). Of the patients with carditis, 48.6% (n = 153) had mild carditis, of which 45 had a subclinical. Sixty-two patients (19.7%) had moderate and 100 patients (31.7%) had severe carditis. At the follow-up, 2 patients died and 16 patients underwent valve surgery. Twenty-eight (7.4%) patients' valve lesions were completely resolved. Conclusion: Although the incidence of acute rheumatic fever decreased, it still is an important disease that can cause serious increases in morbidity and mortality rates in our country.

[Low cardiac output syndrome score to evaluate postoperative cardiac surgery patients in a pediatric intensive care unit]. / Çocuk yogun bakim ünitesinde kardiyak cerrahi sonrasi hastalarin degerlendirilmesinde kullanilan düsük kardiyak debi sendromu skorlamasi.

OBJECTIVE: There is no clear consensus regarding the definition of low cardiac output syndrome (LCOS) or the follow-up of this patient group. Given this lack of a clinical definition, the aim of this study was to use a LCOS score (LCOSs) similar to the low cardiac output score previously presented in the literature and evaluate the relationship between a high LCOSs and poor clinical outcome. METHODS: A total of 54 patients were prospectively evaluated after cardiac surgery. The LCOSs was used to evaluate the deve-lopment of low cardiac output. Each parameter was scored as 1 point. The score was calculated every hour for 24 hours postoperatively and the highest score was recorded as the peak score (pLOCSs). The LOCSs at the time of admission to the pediatric intensive care unit, at the 4th, 8th, and 16th hour were recorded and a cumulative score (cLOCSs) score was calculated. RESULTS: The mean age of the patients was 49.40±53.15 months and 24.07% had LOCS. In the group with LCOS, the cLOCSs, vasoactive-inotropic score (VIS), lactate mean, aortic clamp time, and the total cardiopulmonary bypass time were significantly higher. In this study, a significant and positive correlation was found between the cLOCSs and pLOCSs and the length of hospital stay, length of stay in the pediatric intensive care unit, VIS, lactate mean, and aortic clamp duration. CONCLUSION: The objective of this study was to draw attention to the potential use of a common language in the care of critical pediatric patients undergoing cardiac surgery with a previously defined scoring method that includes parameters indicating poor perfusion in the patient.

Neurofibromatosis type 1 and cardiac manifestations.

OBJECTIVE: Cardiac manifestations of neurofibromatosis type 1 (NF1) may include hypertension, congenital heart disease, and hypertrophic cardiomyopathy. The aim of this study was to evaluate cardiac abnormalities in patients with NF1. METHODS: Sixty-five NF1 patients (mean age: 9±4.48 years) were retrospectively studied. Standard electrocardiography and echocardiography were performed in all patients. RESULTS: Cardiac abnormalities were found in 11 of the 65 patients (15.3%). Five patients had mitral valve regurgitation, 2 patients had secundum atrial septal defect, 1 patient had pulmonary valvular stenosis, 1 patient had ventricular septal defect, 1 patient had tricuspid valve regurgitation, and 1 patient had aortic valve regurgitation. CONCLUSION: Cardiac abnormalities have potential long-term hemodynamic consequences that justify an early diagnosis. Thus, for any patient with NF1, a cardiologic assessment is mandatory at the time of diagnosis and with regular follow-up intervals.

Three patients resembling Teebi-Shaltout syndrome.

Teebi-Shaltout syndrome (TSS) was first reported by Teebi and Shaltout in 1989. This entity is proposed to be inherited in autosomal recessive manner. The clinical features include characteristic facial features, ectodermal dysplasia, camptodactyly, and caudal appendage. Only one additional paper reporting four additional cases has been published since the first description. Clinical features common to all previously affected individuals diagnosed with TSS are craniofacial, orodental-ectodermal, and skeletal. This report summarizes and discusses the findings of three additional patients from two unrelated families with findings similar to TSS. These findings may be present in a genetically and phenotypically heterogeneous group of disorders similar to TSS. Presence of consanguinity and similarly affected siblings of both genders suggests autosomal recessive inheritance.

Transjugular approach to device closure of atrial septal defect in a child with heterotaxia and interrupted inferior vena cava.

In heterotaxia syndrome with left atrial isomerism, the distinguishing feature is interrupted inferior vena cava with azygos continuation. We report using a transjugular approach to device closure of an atrial septal defect in an 8-year-old boy with heterotaxia syndrome. We found that device closure of the child's atrial septal defect through a jugular venous approach was safe when an inferior vena cava approach was not possible. To our knowledge, ours is the first report of the use of internal jugular vein access to close an atrial septal defect in a child.

[Long term follow-up of 799 children with isolated ventricular septal defects]. / Izole ventriküler septal defektli 799 çocugun uzun süreli takibi.

OBJECTIVES: We evaluated long-term follow-up results and prognosis of pediatric patients with isolated ventricular septal defects (VSD). STUDY DESIGN: The study included 799 patients (368 girls, 431 boys; mean age at diagnosis 24.3±37.4 months; median 6 months) who were monitored by the pediatric cardiology department for VSD. The mean follow-up period was 32.8±30.3 months (median 20 months). RESULTS: The VSDs were classified as perimembranous (n=610, 76.4%), muscular (n=171, 21.4%), doubly committed subarterial (n=10, 1.3%), and multiple (n=8, 1%). Spontaneous closure rates were 42.7%, 13.1%, and 25% in muscular, perimembranous, and multiple VSDs, respectively, which corresponded to a mean age of 18.6±19.9 months (median 12 months) in muscular and 30.2±33.7 months (median 14.5 months) in perimembranous VSDs. Before 2 years of age, 78.1% of muscular and 58.6% of perimembranous VSDs underwent spontaneous closure. Of 256 defects (32%) that required surgical closure, 91.4% were of perimembranous location. The mean age at surgery was 38.8±49.1 months (median 11 months) for muscular, and 43.7±40.9 months (median 24 months) for perimembranous defects. During the follow-up period, the following complications were noted: aortic valve prolapse (0.7%), aortic regurgitation (0.6%), left ventricle-to-right atrium shunt (2.6%), subaortic ridge (3.7%), and infundibular stenosis (1.2%). Aortic regurgitation developed in eight patients (3.7%) after surgical closure. CONCLUSION: Our data on the natural course and prognosis of VSDs may be of relevance with respect to patients' age, defect type, and complications encountered in the follow-up period.

Brain natriuretic peptide levels in pediatric cancer patients with febrile neutropenia.

Brain natriuretic peptide (BNP) is considered as a prognostic marker in patients with sepsis, but no data are available on BNP in pediatric cancer patients with febrile neutropenia (FN). Twenty-five pediatric cancer patients with FN were included in this study. Serum BNP level was measured. The mean BNP level was 330.8 ± 765.3 pg/mL (5.9-3806 pg/mL). BNP levels of 12 patients were found over the normal level. High BNP levels were related to some conditions of the patients, and these were statistically significant (P < .05). These conditions were required erythrocyte suspension, had pneumonia, time stayed in hospital, and neutropenia time. When regression test was done, required erythrocyte suspension for anemia and had pneumonia were found to be statistically significant. In conclusion, this is one of the first studies on BNP levels in pediatric cancer patients with FN. However, further studies with large sample sizes are needed to confirm the results and provide new data about this issue.

Pulmonary hypertension, heart failure and neutropenia due to diazoxide therapy.

Primary persistent hyperinsulinaemic hypoglycaemia is characterised by clinical symptoms that occur when blood glucose levels drop below the normal range. Diazoxide treatment remains the mainstay of medical therapy. Tolerance of diazoxide is usually excellent, but several side effects of this drug have been described. We present a 4-month-old girl who developed pulmonary hypertension, heart failure and neutropenia during diazoxide therapy. Diazoxide toxicity was suspected and the drug was withdrawn on day 13. During the next 3 days, respiratory and haemodynamic status dramatically improved and she was weaned from mechanical ventilation. Control white blood cell count was 8800 cells/mm(3) and a new echocardiography showed modreduction of pulmonary artificial pressure to 20 mmHg and resolution of atrial and ventricular enlargement. Paediatric physicians should be in mind of pulmonary hypertension, heart failure and neutropenia developing during diazoxide therapy.

Systemic pulmonary shunt performed with Shelhigh internal mammary artery: early results.

OBJECTIVE: The purpose of this study was to evaluate early findings for aortopulmonary shunts using bovine internal mammary artery grafting. METHODS: Bovine internal mammary artery grafts biomodified with glutaraldehyde and the Shelhigh No-React process were used between May 2005 and April 2006 in our clinic for 20 cases of aorta-pulmonary artery shunts. We implanted 2 sizes of grafts, either a graft with 4-mm proximal and 5-mm distal diameters or a graft with 5-mm proximal and 6-mm distal diameters. Patients were between 20 days and 7 years of age, and the sex distribution was 55% female and 45% male. A Blalock-Taussig shunt with left thoracotomy was performed in patients 2 years of age and older, and a central shunt with sternotomy was performed for patients younger than 2 years. Eight patients underwent operation under emergency conditions. Nine patients had tetralogy of Fallot and/or pulmonary atresia (PA); 3 had transposition of the great arteries, ventricular septal defect, and pulmonary stenosis (PS); 3 had tricuspid atresia; 3 had PS and double-inlet left ventricle; and 2 had PA. RESULTS: After the operation, immediate recovery of oxygen saturation and partial oxygen pressure was observed in all patients. Pulse oximetry measurements showed patient oxygen saturation to be between 84% and 100%. One patient underwent reoperation at the third postoperative hour because of bleeding. Two patients died from causes unrelated to the graft. The hospitalization period was between 7 and 29 days. Echocardiography evaluations showed no shunt obstruction for the early (first postoperative week) or middle (postoperative week 24) period. CONCLUSION: With this study, we assessed the use of the Shelhigh internal mammary artery graft instead of synthetic (polytetrafluoroethylene) tubular grafts in shunt operations for congenital heart diseases with decreased pulmonary blood flow and no evidence of calcification. Further investigation involving a larger number of cases and longer-term results for patency are needed to confirm our findings.

A novel approach for off-pump atrial septostomy applications.

Atrial septostomy or septectomy are required to enable atrial mixture in various congenital cardiac lesions. The aim of this article was to introduce a technique where atrial septostomy application could be employed off pump with the aid of a new device. To our knowledge this is the first report for this technique in the literature. We report the results of 7 patients for whom we employed our technique successfully using a new combined device as an alternative to traditional methods. The major advantage of our approach was avoiding detrimental effects of cardiopulmonary bypass applications.

Congenital aorto-left atrial tunnel--an unusual communication: a case report.

Congenital aortico-cameral communications are rarely seen. We present an asymptomatic patient in whom there was a congenital vascular communication rising from the aortic root and terminating in the left atrium. She had an atrial septal defect (ASD). The diagnosis was made with echocardiography and confirmed by aortography. She was treated by closing the tunnel and the ASD. The outcome was satisfactory in this extremely rare case of a congenital cardiac lesion. Coincidental diagnosis could be made during careful echocardiographic examination.

Right pulmonary artery--left atrial communication presenting with brain abscess: a case report.

Direct communication between the right pulmonary artery and left atrium is a very rare vascular malformation. We report a patient with this anomaly. She presented with unexplained cyanosis and brain abscesses. The diagnosis was made with contrast echocardiography and angiography. We treated this anomaly successfully with surgery. Complete cure for this anomaly can be achieved by ligation.