Comparison of echocardiographic aortic stiffness index measurements and pulse wave velocity measurements in obese and overweight children.

BACKGROUND: Aortic/arterial stiffness is a reliable, independent predictor and a risk factor for cardiovascular mortality. Arterial stiffness is assessed by pulse wave velocity and echocardiography. The purpose of this study is to analyse aortic/arterial stiffness in patients using echocardiographic and pulse wave velocity techniques. MATERIALS AND METHODS: The participants of this study consisted of 62 patients who presented to the Gazi University Pediatric Endocrinology and Pediatric Cardiology outpatient clinics, including 21 obese, 20 overweight, and 21 normal-weight patients. Echocardiography was performed on all patients, and echocardiographic measurements were compared to pulse wave velocity measurements. RESULTS: The mean (min-max) arterial strain measurements were 0.146 ± 0.0 (0.06-0.3) in the obese group and 0.106 ± 0.0 (0.05-0.18) in the overweight group. In comparison to the overweight group, the obese group had greater arterial strain measurements. The pulse wave velocity measurements in the obese and overweight groups were greater than those in the normal weight group (p > 0.05). Elastic modulus and aortic stiffness ß index values were shown to be positively correlated with pulse wave velocity measurements in the obese group (r = 0.56, r = 0.53, respectively; p = 0.008, p = 0.01, respectively). Systolic and diastolic blood pressure measurements were correlated with pulse wave velocity measurements in the obese group (r = 0.98, p = 0.0001, respectively). CONCLUSION: In our study, echocardiographic aortic measurements showing the vessel wall were correlated with pulse wave velocity measurements. Echocardiographic evaluation should be included in the routine follow-up of patients because pulse wave velocity measurement devices are not available in all centres, echocardiography is available in many centres, it is easily applicable, and it facilitates the follow-up of patients.

Evaluation of handgrip strength in children with pulmonary hypertension.

BACKGROUND: Handgrip strength is a crucial indicator of upper extremity muscular strength and is vital for monitoring disorders like cardiac diseases that restrict a patient's physical activity and result in muscle atrophy. The aim of our study was to evaluate whether muscle strength loss is present in patients with pulmonary hypertension and whether this test can be an alternative to 6-minute walk test. MATERIALS AND METHODS: The study included 39 healthy children who were admitted to the outpatient clinic and 16 children with a diagnosis of pulmonary hypertension who were being followed in our centre. We assessed the differences in upper extremity handgrip strength using the Jamar Hydraulic Hand Dynamometer device among both healthy children and those diagnosed with pulmonary hypertension. Moreover, we compared the handgrip strength of pulmonary hypertension patients with significant prognostic indicators such as NYHA class, 6-minute walk test, and pro-brain natriuretic peptide. RESULTS: The mean dominant handgrip strength was 20.8 ± 12 kg in the patient group and 21.6 ± 12.4 kg in the control group (p = 0.970). Handgrip strength was shown to be negatively connected with pro-brain natriuretic peptide (r = -0.565, p = 0.023) and positively correlated with 6-minute walk test (r = 0.586, p = 0.022) during the patient group evaluation. CONCLUSION: Six-minute walk test needs a customised physical area (30 m of a straight hallway) and trained personnel for applying the test. The handgrip strength test, a different muscle strength indicator, can be used to more clearly and simply indicate the decline in patients' ability for effort. Additionally, it was found in our study that handgrip strength decreased as pro-brain natriuretic peptide levels rose, a crucial measure in the monitoring of pulmonary hypertension.

Coexistence of transposition of the great arteries, coarctation of the aorta, and bilateral pulmonary artery hypoplasia.

Transposition of the great arteries is the most common cyanotic CHD in newborns. This CHD, in which the aorta arises from the right ventricle and the pulmonary artery from the left ventricle, is often accompanied by one or several defects such as atrial septal defect or patent foramen ovale, ventricular septal defect and patent ductus arteriosus, which allow the transition between both parallel circulations. Rarely, the disease may be accompanied by left ventricular outflow tract obstruction (subpulmonary obstruction) and coarctation of the aorta.We present a highly complicated and unusual transposition of the great arteries patient with critical aortic coarctation and hypoplastic pulmonary arteries with abnormal outflow and course.

Successful treatment of two cases of acute myocarditis with colchicine.

Colchicine is an FDA-approved medicine that has been used for many years to prevent and treat gout flares as well as familial mediterranean fever. It is also used off-label to treat pericarditis, calcium pyrophosphate illness, and Behçet's syndrome. There are additional studies on the use of colchicine, which is accepted as the standard treatment for pericarditis in adults, post-pericardiotomy syndrome, post-operative and post-ablation atrial fibrillation, coronary artery disorders, prior to percutaneous coronary procedures, and myocarditis. Colchicine appears to be a promising oral cardiovascular treatment targeting the inflammatory axis, owing to its low cost and moderate side-effect profile. Our aim is to emphasise that colchicine treatment, which has a strong and effective anti-inflammatory effect profile, should be kept in mind in addition to conventional treatment in childhood myocarditis.

Evaluation of quality of life and parental attitudes in patients who underwent atrial septal defect closure in childhood.

OBJECTIVE: In this study, we aimed to evaluate quality of life and parental attitudes in children who underwent an atrial septal defect closure procedure with a transcatheter or surgical method in childhood and whether they continued their lives with similar activities to their healthy peers by comparing the two groups. METHODS: Patient forms to define sociodemographic and clinical features, the Questionnaire for Measuring Health-Related Quality of Life in Children and Adolescents (KINDL) to measure the quality of life of children, and the Parent-Child Relationship Test (Parental Attitude Research Instrument) to measure parental attitudes were used. RESULTS: The groups were similar in terms of age and sex. The mean quality of life scale scores were high in all groups, and there was no statistically significant difference between the scores. Parents of the patients who underwent closure received higher scores from the demographic attitudes and recognition of quality subdimension compared to the parents of the healthy group. CONCLUSIONS: The quality of life of children with atrial septal defect closure was found to be similar to their healthy peers. Additionally, the effects of surgical or percutaneous closure of atrial septal defect on quality of life are similar. Children with atrial septal defect closure perceive their health status as well as their healthy peers, and this perception does not cause any difference in the attitudes and behaviours of families.

Effectiveness of using 2D atlas and 3D PDF as a teaching tool in anatomy lectures in initial learners: a randomized controlled trial in a medical school.

BACKGROUND: Anatomy is a crucial part of medical education, and there have been attempts to improve this field by utilizing various methods. With the advancement of technology, three-dimensional (3D) materials have gained popularity and become a matter of debate about their effectiveness compared to two-dimensional (2D) sources. This research aims to analyze the effectiveness of 3D PDFs compared to 2D atlases. METHODS: This study is a randomized controlled trial involving 87 Year-1 and Year-2 medical students at Gazi University Faculty of Medicine, Turkey. The study was conducted in two steps. In Step-1, students were randomized to watch lecture videos on liver anatomy and male genitalia anatomy supplemented with either a 3D PDF (intervention group) or 2D atlas (control group) images. Following the video lectures, a test (immediate test) was administered. In Step-2, the same test (delayed test) was administered 10 days after the immediate test. The test scores were compared between the intervention and control groups. In addition to the descriptive analyses, Chi-square and Mann-Whitney U tests were performed. RESULTS: In the immediate test, while there was no significant difference between the groups for the liver test (p > 0.05), 3D PDF group's scores (Median = 24.50) was significantly higher than the 2D atlas group's in the genitalia test (Median = 21.00), (p = 0.017). The effect size (Cohen's d) was 0.57. In the delayed test, there was no significant difference between the groups in the liver and genitalia tests (p > 0.05). However, the effect size in the immediate genitalia test was 0.40. Year-1 students' immediate test of genitalia performances were significantly higher in the 3D PDF group (Median = 24.00) than the 2D atlas group (Median = 19.00), (p = 0.016). The effect size was 0.76. Also, Year-1 students' 3D PDF group (Median = 20.50) presented with significantly higher performance than the 2D atlas group (Median = 12.00), (p = 0.044) in the delayed test of genitalia, with the 0.63 effect size. CONCLUSION: 3D PDF is more effective than 2D atlases in teaching anatomy, especially to initial learners. It is particularly useful for teaching complex anatomical structures, such as male genitalia, compared to the liver. Hence, it may be a valuable tool for medical teachers to utilize during lectures.

Effect of vitamin D on myocardial remodeling and inflammatory status in children with congenital heart disease.

BACKGROUND: Vitamin D insufficiency is a common public health problem that is often unrecognized in children with congenital heart disease, and is not generally evaluated at congenital heart disease (CHD) follow-up. Recent studies have suggested that inadequate vitamin D status may have an adverse effect on cardiovascular health. This study investigates the relationship between vitamin D levels and hemodynamic parameters in children with CHD. METHODS: Included in the study 40 patients (25 females, 15 males) with CHD, who were evaluated for Ross heart failure score, vitamin D, parathyroid hormone (PTH), calcium, phosphorus, alkaline phosphatase (ALP), whole blood count (WBC) and echocardiographic measurements, and all measurements were repeated in the third month of the therapy. RESULTS: The mean vitamin D level was 16.4 ± 6.6 ug/L before and 27.5 ± 9.9 µg/L in the third month of therapy, while the mean PTH level was 53.3 ± 34.9 pg/mL before and 43.8 ± 21.4 pg/mL in the third month of therapy. The mean WBC was 8084 ± 2324/µL before and 7378±1893/µL in the third month of the therapy, and the mean platelet (PLT) count was 280,897 ± 80,119/µL before and 307,179 ± 60,202/µL in the third month of the therapy. The mean ejection fraction (EF) was 64% ± 7.2% before and 66.7% ± 6.2% in the third month of therapy, while the right ventricle (RV) myocardial performance index (MPI) was 32.1% ± 6.7% before and 28.9% ± 6.5% in the third month of the therapy. IL10 level was increased in four patients in the third month of therapy. A statistically significant decrease in PTH level and WBC, and an increase in PLT number and IL-10 level were detected by the therapy. Furthermore, echocardiographic findings revealed a statistically significant increase in EF and a decrease in RVMPI attributable to the therapy. DISCUSSION: The decreased levels of PTH, which is a proinflammatory marker, the increases in IL-10, which is an antiinflammatory cytokine, and the decreases in the number of WBC resulting from vitamin D treatment demonstrate the antiinflammatory effects of vitamin D. An improvement in EF means improvement in left ventricular contractility, while a decrease in RV MPI has been shown to improve the systolic and diastolic function of the right ventricle. These results suggest that vitamin D therapy has a positive effect on the heart, and so vitamin D levels should be evaluated during the routine follow-up of congenital heart disease.

High take-off right coronary artery in a patient with tetralogy of Fallot.

High 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.

Prognostic value of oxygen saturation and heart rate during a six-minute walk test in pediatric pulmonary hypertension

Background/aim: To evaluate the pre and post-treatment heart rate and oxygen (O2) saturation values measured before, during and after 6MWT in children who received PAH-specific treatment and to determine the effect of these variables on prognosis. Materials and methods: This retrospective study included 29 patients who were diagnosed as PAH and treated. The transcutaneous O2 saturation and heart rate levels were recorded before the test: baseline (Sat0, HR0), at the end of the test: exercise (Sat1, HR1) and 5 min after the test: recovery (Sat2, HR2). The increase in heart rate was defined as HR1-HR0 and the decrease in saturation as Sat0-Sat1. The results obtained before and after the PAH-specific treatment were analyzed and their effect on survival was assessed. Results: Fifteen of 29 patients were female (51.7%). The mean age was 127.2 ± 44.8 months and the median follow-up time was 60 (12­156) months. Only pre-treatment HR1 was found associated with survival. The mean HR1 value of survivors was 122.8 ± 18.4 and that of deceased 94.3 ± 19.1 (p = 0.03). Post-treatment 6MWT was associated with survival. It was 453.3 ± 96.5 m for survivors and 250 ± 135.2 m for deceased (p = 0.02). Conclusion: Post-treatment 6MWT, FC and proBNP had prognostic value in pediatric patients with PAH. The decrease in saturation and increase in heart rate were not found to have a prognostic value. Pre-treatment HR1 was associated with survival. Identification of these prognostic factors at the beginning and throughout the treatment may be a guide for detecting the severity of the disease and follow-up.

Cystic echinococcosis of the interventricular septum: a rare clinical presentation.

Cystic echinococcosis caused by infection with the larvae form of Echinococcus granulosus remains highly endemic and constitutes a public health concern in some regions of the world. In this case report, we present a rare children case of interventricular hydatid cyst with a size of approximately 5 cm and its successful treatment.

Decrease in Cerebral Oxygen Saturation During the 6-Minute Walk Test in Pediatric Pulmonary Arterial Hypertension.

This observational study aimed to investigate the relationship between regional cerebral oxygen saturation (rSO2) during the 6-minute walk test (6-MWT) and the demographic/clinical features of patients with pulmonary arterial hypertension (PAH). Cerebral oxygenation was evaluated during the 6-MWT in 20 pediatric patients with PAH aged ≥ 7 years [13 male, 7 female; median age 12.25 (range 7-18) years]. In all patients, regional cerebral oxygen saturation    (rSO2), arterial oxygen saturation (SpO2), and heart rate (HR) were measured using near-infrared spectroscopy (NIRS) for 2 min before the test, during the 6-MWT test, and 2 min after the test. The relationship between the changes in rSO2, heart rate, and SpO2 values and clinical and laboratory features was compared statistically. The mean pulmonary arterial pressure (mPAP) was 77 (range 25-126) mmHg, and the median 6-minute walk distance (6-MWD) was 427.5 (300-570) m. The changes in rSO2 measurements ranged between 3.41 and 21.8%, and 70% of the patients had a greater than 10% decrease in rSO2 during the test compared with baseline. Eight patients showed a decrease in rSO2 without SpO2 changes. The present study demonstrated a significant decrease in cerebral oxygenation in pediatric patients with PAH during the 6-MWT. We hypothesized that using a combination of the 6-MWT and regional cerebral oxygenation monitoring in pediatric patients with PAH in order to evaluate exercise capacity, as a reflection of reduced daily activities, would provide more precise predictive values than the 6-MWT alone.

The role of inflammatory biomarkers in CHD-associated pulmonary hypertension in children.

OBJECTIVE: The 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. METHODS: This is a prospective review of 37 children with CHD-related pulmonary hypertension, 21 children with congenital heart defects, and 22 healthy children. RESULTS: Serum 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. CONCLUSION: Inflammation 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.

Unknown vascular compression of the airway in patients with congenitalheart disease and persistent lower respiratory symptoms

Background/aim: Airway compression (AC) by vascular structures is an important complication of congenital heart disease (CHD) that often goes unrecognized. It is not easy to identify whether CHD patients require additional invasive examinations or not. Therefore, the present study aims to develop an AC diagnostic algorithm for CHD patients. Materials and methods: CHD patients with persistent respiratory symptoms that were treated between January 2007 and December 2015 were retrospectively reviewed. The following data were recorded for all CHD patients with AC: age, cardiac anomalies, the compressed structure, the airway diameter ratio (ADR), the compressing structure(s), treatment, and follow-up.Results: During the 8-year study period, 62 of 253 CHD patients had persistent respiratory symptoms, of which 11 cases were diagnosed as AC via bronchoscopy and/or thoracic computed tomography angiography. The most frequently affected structures were the left main bronchus and trachea, and the most common compressing structure was the right pulmonary artery. The ADR was near total compression in 3 patients and >0.50 in 3 patients. During follow-up, 5 of the 11 patients with AC underwent surgery, 2 died, and 4 were followed clinically. Patients with ADR of >0.50 did not require surgery and were followed clinically. Conclusion: CHD patients with persistent respiratory symptoms associated with lower respiratory airway obstruction should be evaluated via invasive examination. An AC diagnostic algorithm for pediatric CHD patients was developed.

Effects of whole blood viscosity and plasma NOx on cardiac function and cerebral blood flow in children with chronic kidney disease

Background/aim: The aim of the study was to investigate the effects of whole blood viscosity and plasma nitric oxide on cerebral and cardiovascular risks associated with chronic kidney disease. Materials and methods: The study group consisted of 40 pediatric patients and 21 healthy control subjects. Hematologic and biochemical variables, viscosity and plasma nitric oxide levels, echocardiographic findings, and middle cerebral artery blood flow velocity were examined. Results: Viscosity values of patients were significantly lower than those of the control group. Lower values of hematocrit, total protein, and albumin and higher values of ferritin in all patient groups resulted in significantly low viscosity levels. Plasma nitric oxide levels were higher in all patient groups than those in the controls. No statistically significant difference was present in middle cerebral artery blood flow velocity between the patient and control groups. Even when systolic functions were normal, the patient group had significant deterioration in diastolic functions, suggesting morbidity and mortality risks. Conclusions: Cerebral blood flow velocities were not affected by viscosity and nitric oxide levels, suggesting that cerebral circulation has the ability to make adaptive modulation. The metabolism of nitric oxide levels needs further investigation and studies in patients with chronic renal disease.

Short-Term Outcomes of Patent Ductus Arteriosus Closure With New Occlutech® Duct Occluder: A Multicenter Study.

AIM: Over the past 2 decades, transcatheter occlusion of patent ductus arteriosus (PDA) with coils and the duct occluders evolved to be the procedure of choice. A new device, the Occlutech PDA® occluder (ODO) device has been designed. Herein, we aimed to evaluate the characteristics and short-term results of patients who underwent transcatheter closure of PDA using the ODO. METHODS: We reviewed the clinical records of 60 patients from different centers in Turkey between December 2013 and January 2016. The medical records were reviewed for demographic characteristics and echocardiographic findings. Device size was selected on the narrowest diameter of PDA. RESULTS: The median patient age was 2.5 years (6 months-35 years), and median PDA diameter was 2.5 mm (1.2-11 mm). Fifty-eight of 60 patients (96.6%) had successful ODO implantation. The occlusion rates were 37/58 (63.7%) at the end of the procedure, 51/58 (87.9%) at 24-48 hours post-procedure, and 57/58 (98.2%) on echocardiography at a median follow-up of 7.6 months. CONCLUSION: Our results indicate that transcatheter closure of PDA using the ODO is effective. Larger studies and longer follow-up are required to assess whether its shape and longer length make it superior to other duct occluders in large, tubular, or window-type ducts. (J Interven Cardiol 2016;29:325-331).

Effect of plasma NOx values on cardiac function in obese hypertensive and normotensive pediatric patients.

BACKGROUND: Hypertension (HT) is a major comorbidity of obesity that is associated with an increased risk of cardiovascular disease and higher mortality. The aim of our study was to evaluate cardiac function in obese hypertensive (OHT) and obese normotensive (ONT) pediatric patients and determine the effects of plasma nitric oxide (NOx) values on cardiac function, while demonstrating the role of plasma NOx in HT in obese pediatric patients. METHODS: The study population consisted of 62 patients (27 boys, 35 girls), aged 13-18 years and 21 age-matched healthy controls. All subjects enrolled in the study underwent echocardiography (Echo) evaluation and ambulatory blood pressure monitoring for HT. Plasma NOx and biochemical values were studied in both patient groups separately. RESULTS: Plasma NOx levels were found to be lower in the OHT group than in the ONT and control groups (p < 0.001) and to be negatively correlated with left ventricular mass index values (p < 0.05). Both the OHT and ONT groups had concentric hypertrophy of the heart. CONCLUSIONS: Plasma NOx plays an essential role in obesity-induced HT. Concentric hypertrophy of the left ventricle was found in both the OHT and ONT groups, indicating structural deformation of the heart.

An epidemiological study of paediatric pulmonary hypertension in Turkey.

OBJECTIVE: The 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. METHODS: All 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. RESULTS: The 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. CONCLUSION: The 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.

Assessment of pulmonary arterial hypertension and vascular resistance by measurements of the pulmonary arterial flow velocity curve in the absence of a measurable tricuspid regurgitant velocity in childhood congenital heart disease.

This study aimed to determine mean pulmonary arterial pressure (PAPmean) and pulmonary vascular resistance (PVR) using transthoracic echocardiography (TTE) measurements of the pulmonary artery flow velocity curve in children with pulmonary arterial hypertension (PAH) and congenital heart disease when the tricuspid regurgitant velocity (TRV) is not sufficient. This study enrolled 29 congenital heart disease cases with pulmonary arterial hypertension and 40 healthy subjects followed at our center. The mean age was 66.9 ± 77.9 months in the patient group and 76.3 ± 62.1 months in the control group. A positive correlation was found between TRV and systolic pulmonary arterial pressure (r = 0.394, p = 0.035, 95% confidence interval [CI] = 0.032-0.665), whereas a negative correlation was found between corrected acceleration time (AcTc) and PAPmean (r = -0.559, p = 0.002, 95% CI = -0.768 to -0.242). Furthermore, a negative correlation was found between parameters TRV and AcTc (r = -0.383, p = 0.001, 95% CI = -0.657 to -0.019). Based on the cutoff criterion of 124 ms for AcTc, sensitivity was found to be 79.3% and specificity to be 77.5% in distinguishing between the PAH patients and the healthy control patients (receiver operating characteristic [ROC] area under the curve [AUC] = 0.804, 95% CI = 0.691-0.890, p < 0.0001). The sensitivity and specificity of the concomitant use of AcTc and/or TRV were found to be 90 and 73%, respectively, in distinguishing between the PAH patients and the the healthy control patients. The data obtained by TTE also can be appropriate for measuring PAPmean, PVR, and the vasoreactivity test and for determining the priority of implementing cardiac catheterization even if there is no measurable TRV value.

[Bilateral pulmonary thromboendarterectomy for chronic thromboembolic pulmonary hypertension: the youngest case in our region]. / Kronik tromboembolik pulmoner hipertansiyon nedeniyle uygulanan iki tarafli pulmoner tromboendarterektomi: Bölgemizdeki en küçük olgu.

The insertion of ventriculoatrial (VA) shunts for the treatment of hydrocephalus is associated with the development of chronic thromboembolic pulmonary hypertension (CTEPH). Chronic thromboembolic pulmonary hypertension occurs in patients with recurrent or chronic pulmonary embolism, and is a rare but, potentially devastating disease in children. Pulmonary thromboendarterectomy (PTE) is an important curative therapy for patients with CTEPH. Herein, we present a case of a 14 year-old male patient with CTEPH that developed after a VA shunt procedure. After successful PTE, systolic pulmonary artery pressure was decreased from 75 mmHg to 30 mmHg. PTE is recommended in the pediatric CTEPH population.

Subcutaneous use of treprostinil in pediatric pulmonary hypertension patients: A report of three cases.

Treprostinil was approved by the United States Food and Drug Administration for use in the treatment of pulmonary arterial hypertension in 2002. Intravenous or subcutaneous treprostinil is used in pulmonary arterial hypertension patients in the functional classes of II-IV to alleviate exercise-related symptoms, or in cases where epoprostenol treatment should be reduced due to side effects. In this article, we describe three pediatric cases of pulmonary arterial hypertension in whom subcutaneous treprostinil was used.