Better postoperative management of patients who have undergone single ventricle (SV) Fontan procedure could potentially reduce long-term complications and improve the quality of life for patients. The present study determined the effect of tadalafil on myocardial and endothelial function and exercise performance after modified Fontan operation. Patients who had undergone SV modified Fontan operation were enrolled in this clinical trial. The demographic characteristics of the patients were recorded. Before administration of tadalafil and after the trial, ventricular function (MPI, EF, FS, E/A, VTI), exercise performance, and endothelial function were evaluated for sonographic and biochemical markers (FMD, IMT, ICAM, VCAM, NO) using echocardiography, exercise testing, vascular ultrasonography, and biochemical measurements, respectively. A single dose of tadalafil of 1 mg/kg was administered daily for 6 weeks, and the functional class of the patients before and after tadalafil was determined. A total of 15 patients completed this clinical trial. Tadalafil was shown to have a significant effect on myocardial function, exercise performance, and improvement in NYHA functional class (p < 0.05) of study population. It had no significant effect on the biochemical variables and endothelial function except for IMT (p > 0.05), which decreased significantly after tadalafil administration (p < 0.05). The findings indicate that tadalafil is a safe, well-tolerated agent for the use after modified Fontan operation to improve myocardial function and exercise performance and possibly reduce long-term morbidity and mortality of patients. More conclusive results could be obtained from further study with a larger sample size and long-term follow-up.
Many nonepileptic disorders may mimic epilepsy by history or clinical presentation. Neurally mediated syncope is one of the most important conditions that might be difficult to differentiate from epilepsy on clinical grounds. We investigated the value of the head-up tilt test (HUT) to diagnose syncope in epileptic children. We studied 40 patients (18 girls and 22 boys) between 5 and 20 years old (mean, 11.5 +/- 3.5) who had a previous diagnosis of epilepsy. All patients underwent a HUT test. The HUT test was positive in 26 patients (65%). No statistical difference was observed between the tilt positive and negative groups in sex, age, provocating factors, associated symptoms, family history of syncope and heart disease, findings in physical examination, and electroencephalogram result. There was a history in favor of true syncope in 58% of tilt positive patients compared to 14% of tilt negative patients (p < 0.05). Also, family history of seizure was more frequent in tilt positive patients (p < 0.05). After 18 +/- 6 months of follow-up, 18 of 26 patients with a positive tilt test were completely asymptomatic. Inadequate history taking and overemphasis on positive family history for seizures were important causes of misdiagnosis of epilepsy in our study. The HUT test is a simple, noninvasive diagnostic tool for distinguishing syncope and epilepsy in children and should be considered early in the diagnostic plan and for determining management of selected patients with a history of drop attack and loss of consciousness.
OBJECTIVES The prevention of pulmonary insufficiency (PI) is a crucial part of the tetralogy of Fallot repair. Many techniques have been introduced to construct valves from different materials for the right ventricular outflow tract, including the most commonly constructed monocusp valves. We are introducing a new bicuspid valve made intraoperatively using the autologous right atrial appendage (RAA) to prevent PI in these patients. METHODS The RAA valve was constructed and used in 21 patients with tetralogy of Fallot. The effective preservation of the native valve was impossible in all patients because of either a severe valve deformity or a small annulus. The RAA valve was created after ventricular septal defect closure and right ventricular outflow tract myectomy and was covered with a bovine transannular pericardial patch. The perioperative data were evaluated, and the echocardiography results were assessed immediately after operations and in follow-up with a median of 10.5 months. The data were retrospectively compared with 10 other patients with similar demographic data but with only transannular patches. RESULTS The mean age of the patients was 13.3 months. No mortality or related morbidity occurred after repair using the RAA valve. The PI severity early after the operation was trivial or no PI in 18 patients, and mild PI was observed in 3 patients, which progressed to moderate PI in one of them in the mean 12-month follow-up period. Fifteen patients had mild or no pulmonary stenosis, while moderate pulmonary stenosis was observed in 6 others. Compared with the other 10 patients with only transannular patches, the RAA valve patients had prolonged operative and clamping times, but no difference in postoperative course and shorter hospital stays. The degree of PI was, of course, significantly less in the RAA valve patients, but pulmonary stenosis was the same. CONCLUSIONS The RAA valve construction is a safe and effective technique to prevent PI after the tetralogy of Fallot repair, at least in terms of short- and mid-term results. A longer follow-up period is needed to confirm if this new valve can eliminate or significantly delay the need for pulmonary valve replacement in these patients.
Background:Dental health is one of the most important health burdens of children health. The association between dental health and endocarditis has been already demonstrated, but there is controversy about different frequency of dental caries, periodontitis, and saliva microorganism in comparison to healthy population and children with congenital heart diseases (CHDs). In this study, we evaluated these differences.Methods:Seventy-six healthy children and 68 CHD patients were enrolled in the present case–control study. Dental decay, periodontitis, oral microorganisms, serum calcium, phosphorus, and frequency of carbohydrate and protein consumption of all participants were evaluated by standards method.Results:CHD patients experienced more periodontitis, but the difference was not significant (0.12 vs. 0.09, P = 0.2). In healthy children, the mean saliva colony counts of Streptococcus mutans were more significant (50639 ± 3324 vs. 35285 ± 27226, P = 0.03), which was diminished by adjusting the carbohydrate consumption. The mean colony count of Lactobacilli in children with CHD was nonsignificant higher than healthy children (P = 0.3).Conclusions:Pediatric patients with CHD experience insignificantly higher dental decay, periodontitis, and saliva Lactobacilli colony counts. The frequency of decayed tooth and gingival diseases in healthy children is high, and hence, more dental care attention in our health system is needed for healthy children.
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