Vascular Wall Changes and Arterial Functions in Children with Surgically Repaired Aortic Coarctation.

OBJECTIVE: We investigated arterial functions by measuring carotid-intima-media thickness, flow-mediated dilatation of the brachial artery, and distensibility and stiffness of the abdominal aorta as early indicators of cardiovascular risk in children followed up after coarctation repair. MATERIALS AND METHODS: Twenty patients with successful repair of coarctation and 27 healthy children were investigated. Two-dimensional echocardiographic images, and carotid and brachial ultrasound studies were performed. RESULTS: The ages of the study group ranged from 5.3 to 22 years, and those of the control group from 7 to 17 years. The age at time of surgery was between 0.23 and 257 months. Average follow-up duration after repair was between 11 and 257 months. The stiffness index of the abdominal aorta was significantly higher in patients with coarctation repair than controls (mean = 0.625 ± 0.41, mean = 0.11 ± 0.73; P = .007). Flow-mediated dilatation of the brachial artery in the first minute decreased significantly among the patients (mean = 4.5 ± 2.7, mean = 6.9 ± 4.5; P = .005). Age of the patients had a negative correlation with distensibility of the abdominal aorta (r = -0.572; P = .008) and a positive correlation with stiffness of abdominal aorta (r = 0.566, P = .009). CONCLUSION: This study suggested that vascular wall changes in children and adolescents can be seen even after successful coarctation repair and may progress toward overt atherosclerosis at older ages.

Paediatric and adult congenital cardiology education and training in Europe.

BACKGROUND: Limited data exist on training of European paediatric and adult congenital cardiologists. METHODS: A structured and approved questionnaire was circulated to national delegates of Association for European Paediatric and Congenital Cardiology in 33 European countries. RESULTS: Delegates from 30 countries (91%) responded. Paediatric cardiology was not recognised as a distinct speciality by the respective ministry of Health in seven countries (23%). Twenty countries (67%) have formally accredited paediatric cardiology training programmes, seven (23%) have substantial informal (not accredited or certified) training, and three (10%) have very limited or no programme. Twenty-two countries have a curriculum. Twelve countries have a national training director. There was one paediatric cardiology centre per 2.66 million population (range 0.87-9.64 million), one cardiac surgical centre per 4.73 million population (range 1.63-10.72 million), and one training centre per 4.29 million population (range 1.63-10.72 million population). The median number of paediatric cardiology fellows per training programme was 4 (range 1-17), and duration of training was 3 years (range 2-5 years). An exit examination in paediatric cardiology was conducted in 16 countries (53%) and certification provided by 20 countries (67%). Paediatric cardiologist number is affected by gross domestic product (R2 = 0.41). CONCLUSION: Training varies markedly across European countries. Although formal fellowship programmes exist in many countries, several countries have informal training or no training. Only a minority of countries provide both exit examination and certification. Harmonisation of training and standardisation of exit examination and certification could reduce variation in training thereby promoting high-quality care by European congenital cardiologists.

Subclavian flap aortoplasty for infant coarctation of the aorta

Aortic resection with an extended end-to-end anastomosis is the surgical gold standard treatment for infant aortic coarctation and has excellent early and long-term outcomes.  Subclavian flap aortoplasty is an alternative surgical technique that offers some advantages because there is no need to do extensive dissection and mobilization of the aortic arch and descending aorta as required in an extended end-to-end anastomosis.  This video tutorial illustrates the technical aspects of subclavian flap aortoplasty in an infant.

How safe are children with COVID-19 from cardiac risks? Pediatric risk assesment; insights from echocardiography and electrocardiography

Background/aim: Approximately 40 million individuals worldwide have been infected with SARS-CoV-2, the virus responsible for the novel coronavirus disease-2019 (COVID-19). Despite the current literature about the cardiac effects of COVID-19 in children, more information is required. We aimed to determine both cardiovascular and arrhythmia assessment via electrocardiographic and echocardiographic parameters. Materials and methods: We evaluated seventy children who were hospitalized with COVID-19 infections and seventy children as normal control group through laboratory findings, electrocardiography (ECG), and transthoracic echocardiography (TTE). Results: We observed significantly increased levels of Tp-Te, Tp-Te/QT, and Tp-Te/QTc compared with the control group. Twenty-five of 70 (35.7%) patients had fragmented QRS (fQRS) without increased troponin levels. On the other hand, none of the patients had pathologic corrected QT(QTc) prolongation during the illness or its treatment. On TTE, 20 patients had mild mitral insufficiency, among whom only five had systolic dysfunction (ejection fraction < 55%). There was no significant difference between the patient and control groups, except for isovolumic relaxation time (IVRT) in terms of mean systolic and diastolic function parameters. IVRT of COVID patients was significantly lower than that of control group. Conclusion: Despite all the adult studies, the effects of COVID­19 on myocardial function are not well established in children. The thought that children are less affected by the illness may be a misconception.

Assessment of cardiac and vessel functions in childhood psoriasis

Background/aim: Psoriasis is a chronic inflammatory disease. The effect of psoriasis on the cardiovascular system has not been studied in children before. We studied ventricular strain and vascular functions to assess early cardiovascular effects of psoriasis during childhood. Materials and methods: The study population consisted of 20 psoriatic and 20 age- and sex-matched control subjects. Two-dimensional echocardiography images, longitudinal and global strain, and carotid and brachial ultrasound studies were performed. Results: The mean age of psoriatic children was 14 ± 0.89 years and that of the controls was 14.05 ± 0.88. There were significant increases in terms of interventricular septum diastolic and left ventricular posterior wall diastolic diameter and decreases in mitral E, mitral A, and E/A values between groups. Tissue Doppler imaging revealed significant differences between groups in terms of lateral annulus E', A', E'/A, isovolumetric contraction time, and ejection time. Aortic stiffness was significantly higher and global circumferential strain and longitudinal strain were significantly lower in the psoriasis group. Carotid intima media thickness and flow-mediated dilatation did not differ significantly between the groups. Conclusion: Cardiac left ventricular and arterial functions are affected in psoriatic children and may be an alarming sign of atherosclerotic heart disease in the long term. Early detection of these changes may be helpful for eliminating other risk factors.

Repair of Scimitar syndrome using an extracardiac conduit in pediatric patients: Report of two patients.

Scimitar syndrome (SS) can be repaired by different surgical techniques including direct implantation, intracardiac rerouting, and in situ pericardial channel to direct the Scimitar vein (SV) to the left atrium. The presence of several anatomical variations such as remote infradiaphragmatic drainage of the SV and abnormal situs makes the repair more challenging with conventional repair techniques. In this paper, we present our experience in using an extracardiac-ringed polytetrafluoroethylene conduit in two pediatric patients (14 months and 2 years old) with SS.

Aortic elasticity and carotid intima-media thickness in children with mitral valve prolapse.

Aim We aimed to study the dimensions, systolic and diastolic functions of the left ventricle; dimensions and elasticity of the aorta; and carotid intima-media thickness and flow-mediated dilatation of the brachial artery in mitral valve prolapse. METHODS: The study group consisted of 43 patients (mean age=13.3±3.9) and 42 healthy children (mean age=12.9±3.4). Left ventricular end-diastolic, end-systolic, left atrial diameters, interventricular septum, and left ventricular posterior wall thickness were measured. Ejection and shortening fractions were calculated by M-mode. Measurements were adjusted to the body surface area. Mitral annulus, and systolic and diastolic diameters of the aortic annulus and aorta at each level were obtained; z-scores, aortic strain, distensibility, stiffness index were calculated. Carotid intima-media thickness and flow-mediated dilatation were studied. Patients were classified as classical/non-classical mitral valve prolapse and younger/older patients. RESULTS: Left ventricular end-systolic, end-diastolic, and left atrial diameters (p=0.009, p=0.024, p=0.001) and aortic z-scores at annulus, sinus valsalva, and sinotubuler junction were larger (p=0.008, p=0.003, p=0.002, respectively) in the mitral valve prolapse group. Aortic strain and distensibility increased and stiffness decreased at the ascending aorta in the patient group (p=0.012, 0.020, p=0.019, respectively). Classical mitral valve prolapse had lower strain and distensibility and higher stiffness of the aorta at sinus valsalva level (p=0.010, 0.027, 0.004, respectively). Carotid intima-media thickness was thinner in the patient group, especially in the non-classical mitral valve prolapse group (p=0.037). Flow-mediated dilatation did not differ among the groups. CONCLUSION: Mitral valve prolapse is a systemic disease of the connective tissue causing enlarged cardiac chambers and increased elasticity of the aorta. Decreased carotid intima-media thickness in this group may indicate low atherosclerosis risk.

The association between obesity, hypertension and left ventricular mass in adolescents.

BACKGROUND: Obesity and hypertension (HT) are well known cardiac risk factors. Our goal was to show that even if arterial blood pressure (BP) measurements of obese adolescents are normal during clinical examination, ambulatory blood pressure monitoring (ABPM) can be high, may include cardiac involvement and can also detect left ventricular mass indices (LVMI) value for obese adolescents to diagnose left ventricular hypertrophy (LVH). METHODS: This study included 130 children (57 obese hypertensive, 36 obese normotensive, 14 normal weight hypertensive and 23 normal weight normotensive). Adolescents whose BP was measured during clinical examination, after 24-h BP was detected using ABPM, were examined with echocardiography for calculation of LVMI to determine cardiac risk factors for LVH. RESULTS: There was a significant difference between the LVMI of obese-normotensive and obese-hypertensive adolescents, which showed the effect of obesity on LVMI independent of HT. Twenty (35.7%) of 56 obese adolescents with HT detected with ABPM had normal BP measurements during clinical examination. Dipper and nondipper features of obese adolescents were significantly higher in ABPM than those with normal body mass index. When the cutoff LVMI value for LVH was set at ≥38 g/m2.7, 38.9% of obese-normotensive and 50.9% of obese-hypertensive subjects had LVH; however, when the cutoff value was set at ≥51 g/m2.7, the rates were 2.8% and 19.3%, respectively. CONCLUSIONS: Obesity is a risk factor for LVH independent of HT. To identify masked HT, 24-h ABPM and cardiac examination should be routinely performed in obese adolescents. Using a limit of LVMI ≥38 g/m2.7 in evaluating LVH secondary to HT in obese individuals may lead to an overestimated diagnosis rate of LVH.

Disseminated Peripheral Mycotic Aneurysms and Septic Embolizations Related to an Infected Stent Deployed for Restenosis of Surgically Repaired Supravalvular Aortic Stenosis.

Percutaneous treatment of supravalvular aortic stenosis (SVAS) by means of balloon dilation and stent deployment has been rarely reported in the literature. In this report, we present the case of a patient with mycotic aneurysms, disseminated peripheral and cerebral septic embolizations, and infected vegetations associated with a stent that had previously been deployed to treat restenosis of surgically corrected SVAS in the infancy.

The efficacy and safety of naproxen in acute rheumatic fever: The comparative results of 11-year experience with acetylsalicylic acid and naproxen.

The objective was to compare the efficacy and safety of naproxen (NXN) to acetylsalicylic acid (ASA) in the treatment of acute rheumatic fever (ARF). The data of 338 children were retrospectively analyzed. The patients were grouped according to joint and valve involvement and also drug chosen [methyl prednisolone (mPSL), ASA or NXN]. The treatment results and adverse events in each group were compared. The mean age was 10.3 years and the median follow-up was 62 months. Median time for normalization of acute phase reactants was 1 week in patients given steroids and 2 weeks in patients given ASA or NXN. ASA was replaced with NXN in 18 patients (10.2%) due to hepatic toxicity. The rate of rebound, recurrence and the prevalence of rheumatic valve disease were not different in patients given NXN, ASA or mPSL. In conclusion, NXN is a safe and effective alternative to ASA in the treatment of ARF in children.

Embolization of a complex pulmonary arteriovenous fistula and coarctation treatment with covered stent at the same session.

Pulmonary arteriovenous fistula (PAVF) are rare malformations that may cause serious complications such as paradoxical embolism, stroke, pulmonary hemorrhage and hemoptysis. Accompanying cardiac malformations such as aortic coarctation were not reported previously. Here we present a case of complex PAVF associated with aortic coarctation. The patient was treated successfully by transcatheter embolization of PAVF with amplatzer vascular plug I and II and implantation of a covered CP stent for coarctation at the same session.

What is the outcome of rheumatic carditis in children with Sydenham's chorea?

We evaluated the echocardiographic features of 69 children diagnosed with Sydenham's chorea at the first attack of acute rheumatic fever. By echocardiography, carditis was detected in 71% of cases and silent carditis was shown in 28.9% of cases at initial presentation. Most patients had mild or moderate valvular regurgitation. Sixty-three cases were followed from 1-10 years. The improvement rate in valvulitis in cases with silent carditis (29.4%) was not different than in cases with clinical carditis (18.5%) (p > 0.05). Persistence of valvular pathologies occurred in 72.2% of cases with carditis in the long-term follow-up (> 2 years). Most patients (88.8%) complied with secondary prophylaxis, so relapse of carditis was exclusively prevented in our patients. Recurrence of chorea was identified in 20.6% of cases and was not associated with clinical or laboratory evidence for streptococcal reinfection. Patients with chorea usually had mild carditis, and carditis showed resolution. Relapse of carditis in our population was exclusively prevented with secondary prophylaxis. Recurrence of chorea was not rare, despite regular treatment with benzathine penicillin.

A large intracardiac fungus ball in a premature infant.

We present a premature infant with large intracardiac mass. She had a history of sepsis and umbilical venous catheterization in the neonatal period. Twenty-seven days after withdrawal of the catheter, a precordial murmur was noted. A large right atrial highly mobile mass suspected to be thrombus was detected by echocardiography. C-reactive protein was elevated. Three blood cultures were negative. Anticoagulation treatment was started. After one week, no resolution of the thrombus was observed. The mass was surgically resected and diagnosis of thrombus infected by fungi was made on histopathological examination. Early screening of cardiac chambers by echocardiography is recommended in all preterms with intravascular catheterization.

Second degree heart block associated with QT prolongation.

Pseudo 2:1 AV block when sinus intervals are shorter than the ventricular refractory period has been reported with long QT syndrome (LQTS). We report the characteristics and treatment of a patient suffering from congenital LQTS with episodes of true 2:1 AV block. The pseudo 2:1 AV block relates to the extreme prolongation of ventricular refractoriness. Several histologic studies have documented abnormalities within the conduction system, including apoptosis. Because of the rare occurrence and poor prognosis of the LQTS with impaired AV conduction, international guidelines for diagnosis and treatment are needed.