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The number of patient-doctor appointments carried out using telemedicine has surpassed in-person appointments. In spite of this, it is unclear that telemedicine curricula in undergraduate medical education reflect the real importance by means of the effectiveness of these approaches. We aimed to systematically search and review the studies that are on undergraduate telemedicine curricula. We searched the Web of Science, PubMed, and Scopus using the keywords such as telemedicine, medical education, and curriculum. Our search was limited to publication dates between January 1, 2000, and February 1, 2020. We elicited the information of the curricula as to their countries, participants, aims or objectives, teaching methods, and evaluation of effectiveness. We also evaluated the quality of the studies using the Joanna Briggs Institute Qualitative Appraisal and Review Instrument. Out of 461 studies, seven articles were selected based on selection criteria for further review. The studies were mostly from the USA. The participant numbers were between seven and 268. There were several modes of delivery but lectures and patient encounters were used mostly. In four studies, the effectiveness was evaluated only by using satisfaction surveys, and the results were satisfactory. A study reported the acquisition and application of skills as a result. There is no well-established telemedicine curriculum in the undergraduate years. The methods vary but the effectiveness of the educational programs does not have a robust evidence base. It is evident that undergraduate medical education needs a curriculum backed by strong scientific data on its effectiveness.

AbstractHigh take-off coronary artery anomaly is a quite rare anomaly which is usually seen in isolated form and diagnosed incidentally. Association with tetralogy of Fallot is also rare and it is not one of the well-known coronary anomalies seen in this disease. Here, we describe high take-off right coronary artery in a 10-month-old female patient with tetralogy of Fallot which was diagnosed during catheter angiography. It is very important to show this anomaly sometimes with additional imaging techniques as it alters all the surgical approach including aortic cannulation.

AbstractObjectiveThe present study aims to identify the role of inflammatory markers such as C-reactive protein, interleukin-6, and fractalkine in CHD-associated pulmonary hypertension in children.MethodsThis is a prospective review of 37 children with CHD-related pulmonary hypertension, 21 children with congenital heart defects, and 22 healthy children.ResultsSerum C-reactive protein and interleukin-6 levels were significantly higher in the children with CHD-related pulmonary hypertension (respectively, p=0.049 and 0.026). Serum C-reactive protein concentrations correlated negatively with ejection fraction (r=−0.609, p=0.001) and fractional shortening (r=−0.452, p=0.007) in the pulmonary hypertension group. Serum fractalkine concentrations correlated negatively with ejection fraction (r=−0.522, p=0.002) and fractional shortening (r=−0.395, p=0.021) in the children with pulmonary hypertension. Serum interleukin-6 concentrations also correlated negatively with Qs (r=−0.572, p=0.021), positively with Rs (r=0.774, p=0.001), and positively with pulmonary wedge pressure (r=0.796, p=0.006) in the pulmonary hypertension group. A cut-off value of 2.2 IU/L for C-reactive protein was able to predict pulmonary hypertension with 77.5% sensitivity and 77.5% specificity. When the cut-off point for interleukin-6 concentration was 57.5 pg/ml, pulmonary hypertension could be predicted with 80% sensitivity and 75% specificity.ConclusionInflammation is associated with the pathophysiology of pulmonary hypertension. The inflammatory markers C-reactive protein and interleukin-6 may have a role in the clinical evaluation of paediatric pulmonary hypertension related to CHDs.

AbstractObjectiveThe present study aims to evaluate the epidemiological characteristics of paediatric pulmonary hypertension within the entire Turkish population over a period of 5 years using the registry of the National Health Insurance System.MethodsAll individuals aged <18 years who were admitted to a Turkish hospital for the first time between 2009 and 2013 with a discharge diagnosis of idiopathic pulmonary hypertension and secondary pulmonary hypertension were identified.ResultsThe overall annual incidence of idiopathic pulmonary hypertension during childhood was 11.7 cases/million, whereas the overall annual incidence of secondary pulmonary hypertension during childhood was 9.5 cases/million. There was a gradual and significant increase in the annual incidence of idiopathic pulmonary hypertension and that of secondary pulmonary hypertension during the 5-year study period (p=0.001 for both). In the years 2012 and 2013, idiopathic pulmonary hypertension was significantly more frequent in children aged <2 years when compared with children aged above 2 years (p=0.002 for both). The male to female ratio was 1.2:1 for idiopathic pulmonary hypertension, whereas the female to male ratio was 1.1:1 for secondary pulmonary hypertension during childhood.ConclusionThe incidence of paediatric pulmonary hypertension in Turkey is higher than those reported for the Western populations. Moreover, no female dominance could be observed. These discrepancies may be attributed to the differences in the study design, study cohort, timing of the study, and the definitions adopted for pulmonary hypertension classification.

AbstractTetralogy of Fallot is the most common cyanotic congenital heart disease with decreased pulmonary blood flow. Right-to-left shunt and infundibular pulmonary stenosis in this disease lead to a decrease in arterial O2saturation. Hypoxia is a strong stimulus for angiogenesis; however, the reason for insufficiency in the pulmonary vascular growth in patients despite chronic arterial hypoxia is still not known. This study was planned considering that the impairment in vascular endothelial growth factor-receptor relationship or the vascular endothelial growth factor-receptor deficiency in the pulmonary vascular bed during development may cause insufficiency of pulmonary vascular growth. A total of 24 patients were grouped as cyanotic – including 13 patients with tetralogy of Fallot – and acyanotic – including 11 patients with left-to-right shunt lesions. During cardiac catheterisation, vascular endothelial growth factor measurements were performed; and oxygen saturations, pressures, and haemoglobin levels were measured. Perioperative lung biopsy for vascular endothelial growth factor receptors was performed in the cyanotic group. Vascular endothelial growth factor of the aorta was higher in the acyanotic group. There was a significant negative correlation between vascular endothelial growth factor levels and aortic O2saturation in the cyanotic group (p < 0.05). Vascular endothelial growth factor tissue staining was negative in 11 out of 13 (84.6%) patients. KDR/Flk-1 receptor was positive in four out of 13 (30.7%) patients; Flt-1 receptor was positive in six out of 13 (46.1%) patients. Vascular endothelial growth factor values were found to be lower than those of the acyanotic patients in this study. Low serum vascular endothelial growth factor levels of the cyanotic group, in spite of the hypoxia, demonstrated the importance of studying vascular endothelial growth factor tissue levels and vascular endothelial growth factor receptors in these patients.

Abstract: A 13-year-old boy with atrial septal defect and tricuspid valve abnormality was reported. He had crepitan ralles and signs of heart failure. He was treated with digital, diuretic and antimicrobial therapies. After clinical improvement he underwent surgery. The atrial septal defect was closed, and ringplasty was applied to the tricuspid valve. After the operation, he could not be extubated because of respiratory failure. On the seventh day following the surgery, he developed pneumothorax and hyportension and died. Postmortem examination showed bilateral diffuse pulmonary tuberculosis. The aim of this report is to emphasise the association of tuberculosis and congenital heart disease.

AbstractObjectiveMitral valvar prolapse is the most common anomaly of the mitral valve apparatus throughout childhood. Fibrillin is one of the structural components of the elastin-associated microfibrils found in the mitral valve. A case-controlled study has performed to investigate the relationship between fibrillin 1gene intron 56 polymorphism and risk of mitral valvar prolapse in Turkish children.Patients and methodsA total of 77 patients with mitral valvar prolapse diagnosed by clinical evaluation and echocardiography and 89 normal children of same age and sex were studied. The fibrillin-1gene intron 56 polymorphism was identified by the polymerase chain reaction-based restriction analysis.ResultsThere was a significant difference in the distribution of fibrillin-1gene intron 56 genotypes (p = 0.0001) and allelic frequency (p = 0.0001) between the cases and the controls.ConclusionsPatients with mitral valvar prolapse have higher frequencies of fibrillin-1gene intron 56 GC genotypes. Healthy children have higher frequencies of fibrillin-1gene intron 56 CC genotypes. We speculate that the higher frequency of fibrillin-1gene intron 56 G-allele increases the risk of mitral valvar prolapse.

Pulmonary hypertension is a group of diseases, including pulmonary arterial hypertension associated with congenital heart disease (APAH-CHD), characterized by progressive deterioration in pulmonary hemodynamics associated with substantial morbidity and mortality risk. THALES is a national multicenter, prospective observational registry, providing data on patients with APAH-CHD. The study comprised APAH-CHD patients (>3 months of age) with confirmed diagnosis of right heart catheterization or echocardiographic findings. Initial and follow-up data were collected via regular hospital visits. Descriptive statistics are used for definitive purposes. Overall, 1034 patients aged 3 months–79 years (median 11.2 [Q1–Q3: 2.2–24.3] years) with APAH-CHD were enrolled at 61 centers, 50.3% being retrospectively enrolled. Most had either Eisenmenger's syndrome (49.2%) or systemic-to-pulmonary shunts (42.7%). Patients were mostly in functional class I–II at the time of diagnosis (46.6%). Mean 6-min walk distance (6MWD) was 369 ± 120 m. Mean pulmonary arterial pressure was 54.7 ± 22.2 mmHg for the whole group, and was highest in patients with Eisenmenger's syndrome. Targeted therapies were noted in 398 (38.5%) patients (monotherapy in 80.4%). Follow-up data were available in 506 patients. Survival at 140 months was 79% and was associated with baseline 6MWD >440 m (p= 0.009), brain natriuretic peptide level < 300 ng/L (p< 0.001). Follow-up 6MWD >165 m (p< 0.0001), brain natriuretic peptide level <300 ng/L (p= 0.031), and targeted therapies (p= 0.004) were also predictive of survival. THALES is the largest registry dedicated to APAH-CHD to date and provides important contributions on demographics, clinical characteristics, and gaps in disease management.

The actual incidence of central nervous system complications with varicella ranges from 0.1 to 0.7% in several series and the time interval between the rash and the type of neurological manifestation varies widely. Cerebellar ataxia and hypotonia are the most common neurological abnormalities associated with varicella. We present a report on a 6‐year‐old girl with an unusual course of disease finally presenting a probable diagnosis of acute disseminated encephalitis.

Patients with acute rheumatic fever sometimes present with atypical signs and symptoms. In these circumstances, the Jones criterions may not be sufficient to make a clinical diagnosis. We describe here two patients with unusual presentations, highlighting that, both in regions where the disease is endemic, or where it is seen only sporadically, physicians should be more alert and careful in making the diagnosis.

Turner Syndrome is a genetic disease with a frequency of 1 in every 1500-2500 live-born female babies. In this report, a 2 years 10 months old patient who had the genotype of 46,XOi (Xq) will be introduced, although her phenotype and cardiovascular system involvement are not seen in the classic Turner spectrum.

A 7-year-old boy developed a left ventricular pseudoaneurysm after being struck by a tractor. The pseudoaneurysm was diagnosed and successfully resected 5 years after the accident.

The purpose of the study was to report a patient with Seckel syndrome associated with high intraocular pressures despite intensive antiglaucoma treatment.