The Retrospective Evaluation of the Patients in Pediatric Cardiac Intensive Care Unit of Cardiac Surgery Center

Introduction:In this study, demographic and epidemiologic features, clinic and prognosis of patients admitted to the pediatric cardiac intensive care (PCICU) unit of a cardiac surgery center were analysed retrospectively.Methods:Patients followed in the PCICU between January 2017 and January 2018 were included in the study. Patient files were analysed for medical data. The patients were divided into 4 major categories as postoperative patients (group 1), patients followed after cardiac catheterization (group 2), patients followed due to arrythmias (group 3), and others (myocarditis, pneumonia, tamponade) (group 4). The demographic variables, such as age and gender, echocardiographic diagnosis, kind and condition of the transfer, reason for PCICU follow-up, and discharge status were evaluated in detail.Results:Eighty hundred ninety five patients were followed in the PCICU during the study period.The median age was 5.5 months (1 day-18 years). 53% of patients (n=474) were male and 47% (n=421) were female. The ortanca weight of the patients was 7.2 kg (1.8-80 kg). 16% of the patients were younger than 1 month of age and 54% of the patients were under the age of 6 months. 12% (n=107) of the patients had a diagnosis of a genetic condition such as Down syndrome, DiGeorge syndrome, etc. 40% of the patients were non-residents. 25% was transferred by air ambulance or ground ambulance. 22% of the patients were foreigners. There were 610 patients in group 1, 130 patients in group 2, 55 patients in group 3, and 100 patients in group 4. The RACHS-1 scores in group 1 were as follows: 0.8% undetermined, 11.2% category 1, 42.9% category 2, 29.6% category 3, 12.6% category 4,(-) category 5, and 2.6% category 6. Overall mortality was 5.9% (n=53) and morbidity was 22% (n=197).Conclusion:We believe that by the means of this kind of epidemiological studies, we may detect the types of cardiac problems in children who needed intensive care follow-up and help to decrease mortality and morbidity in childhood due to congenital cardiac diseases in our country

Single-center experience with routine clinical use of 3D technologies in surgical planning for pediatric patients with complex congenital heart disease

PURPOSEThis study was planned to assess the application of three-dimensional (3D) cardiac modeling in preoperative evaluation for complex congenital heart surgeries.METHODSFrom July 2015 to September 2019, 18 children diagnosed with complex congenital heart diseases (CHDs) were enrolled in this study (double outlet right ventricle in nine patients, complex types of transposition of the great arteries in six patients, congenitally corrected transposition of the great arteries in two patients, and univentricular heart in one patient). The patients’ age ranged from 7 months to 19 years (median age, 14 months). Before the operation, 3D patient-specific cardiac models were created based on computed tomography (CT) data. Using each patient’s data, a virtual computer model (3D mesh) and stereolithographic (SLA) file that would be printed as a 3D model were generated. These 3D cardiac models were used to gather additional data about cardiac anatomy for presurgical decision-making.RESULTSAll 18 patients successfully underwent surgeries, and there were no mortalities. The 3D patient-specific cardiac models led to a change from the initial surgical plans in 6 of 18 cases (33%), and biventricular repair was considered feasible. Moreover, the models helped to modify the planned biventricular repair in five cases, for left ventricular outflow tract obstruction removal and ventricular septal defect enlargement. 3D cardiac models enable pediatric cardiologists to better understand the spatial relationships between the ventricular septal defect and great vessels, and they help surgeons identify risk structures more clearly for detailed planning of surgery. There was a strong correlation between the models of the patients and the anatomy encountered during the operation.CONCLUSION3D cardiac models accurately reveal the patient’s anatomy in detail and are therefore beneficial for planning surgery in patients with complex intracardiac anatomy

A rare association with suffered cardiac arrest, long qt interval, and syndactyly: Timothy syndrome (LQT-8)

Timothy syndrome (TS), also referred to as syndactylyassociated long QT syndrome (LQTS) or LQT8, is a multi-system disorder characterized by developmental defects causing dysmorphic facial features, congenital heart abnormalities, neurocognitive impairment, and webbing of the toes and fingers (syndactyly) (1). TS is caused by mutations of the CACNA1C gene, which encodes L-type calcium channel Ca (V) 1.2. Two types of TS have been defined according to the mutation sites: G406R in exon 8A (TS1) and G402S/G406R in exon 8 (TS2). These gain-offunction mutations result in an impaired open-state and voltagedependent inactivation of the L-type calcium channel, ultimately 672 Case Reports Anatol J Cardiol 2015; 15: 671-4 leading to a markedly prolonged myocardial action potential (delayed ventricular repolarization) (1-4)

Süt çocuğunda kalp yetersizliği ile başvuran dilate kardiyomiyopatinin düzeltilebilir bir nedeni: ALCAPA sendromu

Anomalous origin of the left coronary artery arising from pulmonary artery ALCAPA syndrome is a rare congenital heart disease seen in children. If untreated, it may lead to congestive heart failure, dilated cardiomyopathy (DCM), ischemic and arrhythmic complications may lead to patient’s death. ALCAPA is diagnosed with echocardiography; in the patients of suspected diagnosis, computerized tomography, magnetic resonance imaging and cardiac catheterization are used for further testing. Surgically correctable ALCAPA syndrome must be considered as etiology of DCM in children. In this report, we presented the case of an infant that was referred to our center with the diagnosis of DCM, who was echocardiographically diagnosed with ALCAPA syndrome and successfully treated with surgery, as well as a review of recent literature.Sol koroner arterin pulmoner arterden çıkış anomalisi olarak tanımlanan ALCAPA sendromu, çocuklarda nadir görülen bir konjenital kalp hastalığıdır. Tedavi edilmediği zaman konjestif kalp yetmezliği, dilate kardiyomiyopati (DKM), iskemik ve aritmik komplikasyonlarla hasta kaybedilebilir. Tanı ekokardiyografi bulgularıyla koyulur, tanıdan şüphelenilen olgularda bilgisayarlı tomografi, manyetik rezonans görüntüleme ve kalp kateterizasyonu gibi ileri tetkiklerden faydalanılabilir. Çocukluk çağında DKM tanısı alan hastalarda etiyolojide, cerrahi olarak düzeltme şansı olan ALCAPA sendromu mutlaka düşünülmelidir. Bu yazıda, merkezimize DKM tanısıyla sevk edilen, ekokardiyografi ile ALCAPA sendromu tanısı konulup, başarılı cerrahi tamir yapılan hasta olgu sunumu yapıldı ve son literatür gözden geçirildi

The use of Chronic Total Occlusion (CTO) wires for perforation of atretic pulmonary valve; two centers experience

Pulmonary valve atresia with intact ventricular septum (PA-IVS) can be treated either surgically or transcatheterly for eligible patients. Perforation of pulmonary valves using chronic total occlusion (CTO) guidewires has been reported as an alternative to radiofrequency (RF) perforation. We sought to report our experience with CTO guidewires for perforation of atretic pulmonary valves and subsequent balloon dilatation (with or without patent ductus arteriosus stenting) in patients with PA-IVS from two centers. A retrospective study was carried out on PA-IVS patients who underwent intervention between March 2014 and September 2019, in which CTO guidewire was employed for pulmonary valve perforation. A total of 26 patients were identified. The median age and weight of the patients were 5.2 days (range 1-21 days) and 3.1 kg (range 2.2-3.8 kg), respectively. All patients were situs solitus, except one patient with left atrial isomerism. The right ventricle (RV) morphology was bipartite in 22/26 patients and tripartite in 4/26 patients. Before the procedure, the mean saturation was 76% (range 70-86%) while the patients were under prostaglandin infusion. The pulmonary valve perforation attempt was performed with the Asahi Conquest Pro 9 CTO wire (n = 6) or Asahi Pro 12 CTO wire (n = 18) and/or Asahi Miracle CTO wire (n = 2). The procedure was successful in 20/26 (77%) patients using CTO wires. We analyzed the efficiency of CTO wire based on the subtypes: Conquest Pro 9 in 6/6 (100%) patients, Conquest Pro 12 in 12/18 (67%) patients, and Miracle in 2/2 (100%) patients. Before CTO wire usage in 3 patients, radiofrequency (RF) perforation was unsuccessful. Among these 3 patients, pulmonary valve perforation was successful in 2 patients with CTO wire; hence, in the remaining patient, perforation was also unsuccessful with CTO wire. After CTO wire perforation was unsuccessful in 6 patients, RF perforation was attempted in 3 patients (2 successful attempts and 1 unsuccessful attempts), and one patient as referred to surgery. Desaturation was persistent in 19 cases, which necessitated ductus arteriosus stenting. Early procedural complication was observed in 3/26 (11%) patients. Two of these patients had vascular complications due to the sheath, which was treated with heparin infusion and streptokinase, and the remaining patient had sudden bradycardia and cardiac arrest during the procedure and did not respond to cardiac resuscitation. CTO wires should be keep in mind for atretic pulmonary valve perforation as a first choice or when RF perforation is unsuccessful