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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. [ABSTRACT FROM AUTHOR]

Background: Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart disease. Most studies have focused on Right Ventricular (RV) dysfunction, while the left ventricle has received less attention in patients with TOF. Objectives: This study aimed to investigate the Left Ventricular (LV) function after surgical Pulmonary Valve Replacement (sPVR) in patients with repaired TOF (rTOF) by Speckle Tracking Echocardiography (STE). Methods: This single-center, observational, cross-sectional study was conducted on 58 volunteers (age: 15 - 31 years) divided into three groups as follows: 22 PVR patients (mean age: 18.96 ± 7 year), 16 patients with rTOF, and 20 healthy controls who were matched regarding the PVR age range. 2D echocardiography (including Doppler and M-Mode indices of the right and left ventricles) and Speckle Tracking Echocardiography (STE) (Global Longitudinal Strains (GLS) and 18 segment analyses) were performed for all patients. All analyses were done using the SPSS software and P < 0.05 was considered to be statistically significant. Results: 2D echocardiography showed normal LV Ejection Fraction (LVEF) in all study groups (64% in sPVR, 60% in rTOF (P = 0.127), and 62.5% in the control group). However, the mean GLS of the left ventricle significantly reduced in both sPVR (-17.5 ± 2.5%) and rTOF (-17.1 ± 4.7%) patients in comparison to the control group (-20.2 ± 0.7%) (P = 0.003). Yet, no significant difference was observed between the rTOF and sPVR groups regarding the GLS (P = 0.9). Segmental analysis of the Longitudinal Strain (LS) indicated a significant decrease in the sPVR and rTOF groups in basal anterior, basal septal, basal anterolateral, mid-anterior, and anterolateral segments. Except for the lower LS in the apical-anteroseptal segment, this level was mostly spared in both sPVR and rTOF patients. Conclusion: LVEF was within the normal range among the sPVR patients, but the pattern of impaired segmental LS and GLS did not change compared to the rTOF group. In conclusion, sPVR might not have a significant effect on the improvement of LV function assessed by STE in patients with rTOF. LV damage occurring during the surgical correction of TOF might have a permanent deteriorating effect on LV function. [ABSTRACT FROM AUTHOR]

Background: Type I Diabetes Mellitus (T1DM) is one of the most common endocrine diseases in the world. It is also the dominant type of diabetes mellitus among children and adolescents that, if left uncontrolled, becomes a major risk factor for cardiovascular disorders and may cause clinical and subclinical cardiac dysfunction. Objective: This study aimed to compare uncontrolled pediatric and adolescent patients and similar healthy people regarding cardiac function according to echocardiography and B-type Natriuretic Peptide (BNP). Methods: This case-control study was carried out in the Outpatient Department of Nemazi tertiary hospital in Shiraz, Iran between June and September 2016. The study included 29 consecutive outpatient cases aged 5 - 18 years who had been diagnosed with T1DM for more than three years and had normal ejection fraction in M-mode echocardiography. The control group consisted of 29 age- and gender-matched healthy non-diabetic volunteers. Both cases and controls were selected by simple random sampling. The participants underwent clinical evaluation, including BNP assay and echocardiographic examination composed of two-dimensional echocardiography, M-mode echocardiography, pulsed Doppler imaging, and Tissue Doppler Imaging (TDI). P-value ≤ 0.05 was considered to be statistically significant. Results: The results revealed a decline in cardiac function in TDI, which was related to the HbA1C level and duration of the disease. A significant difference was found between the cases and the controls regarding the left ventricular posterior wall, interventricular septal diameters in diastole, and E/A ratio of tricuspid valve (P = 0.047, P = 0.001, and P = 0.021, respectively). A significant difference was also observed between the two groups in terms of septal S' and E' parameters (P = 0.004 and P = 0.002, respectively). Moreover, diminished E/A ratio of tricuspid valve annulus was related to the increased duration of the disease. Even though left ventricular hypertrophy occurred in the patients, no significant increase was observed in BNP values. Conclusion: The results indicated that M-mode, two-dimensional, pulsed Doppler, and TDI echocardiography might efficiently evaluate cardiac function in patients with preserved ejection fraction. However, BNP measurement might not be useful for screening of cardiac function. [ABSTRACT FROM AUTHOR]

Background: Conduction disturbances can occur early after total correction of Tetralogy of Fallot (TOF). Some of these arrhythmias and alterations like complete right bundle branch block are permanent, while some others like Junctional Ectopic Tachycardia (JET) are transient. Objective: This study aimed to evaluate the early post-operative arrhythmias and their risk factors among pediatric patients with TOF after surgical total correction. Methods: This retrospective study was conducted on pediatric patients aged six months to 15 years old (52.5% male and 47.5% female) with TOF who had consecutively underwent surgical total correction during 2015 - 2017. Patients with additional concomitant cardiac operations, previous arrhythmias before the surgery, or any co-existing cardiac anomalies as well as those with severe post-operative complications were excluded. All pertinent data and Electrocardiographic (ECG) documents were recorded several times right after the operation to detect arrhythmias and ECG changes and to find any association between the related risk factors and the occurrence of arrhythmias. Results: Out of the 118 TOF patients who underwent total correction, 27% were under the age of one year and 73% were above one year old. Among the patients, 78.5% developed permanent ECG changes, mostly complete right bundle branch block and left anterior or posterior hemiblock. Out of these patients, 8.5% had JET together with other permanent arrhythmias and 8.5% had transient JET solely. Therefore 17% of all patients developed JET and 21.5% had no permanent noticeable ECG changes. The most permanent arrhythmia was right bundle branch block followed by left anterior hemiblock and left posterior hemiblock. These arrhythmias mainly manifesed amongst the patients with lower age and those with severe subvalvar pulmonary stenosis. The results showed a significant relationship between the incidence of JET and use of milrinon after the operation. There was no myocardial ischemia or infarction from injury to aberrant coronary arteries passing over the right ventricular outflow tract. Conclusion: Early post-operative permanent arrhythmias after total TOF repair was probably more common amongst the patients who were below one year old with more severe subvalvar pulmonary stenosis. Nonetheless, the occurrence of transient JET was mostly observed in the patients who were given milrinone as a post-operative inotrope medicine. [ABSTRACT FROM AUTHOR]

Background: Congenital Aortic Stenosis (AS) is a common problem among congenital heart diseases and balloon valvuloplasty is known as the procedure of choice for treatment. Objectives: This study aimed at evaluation of the function and remodeling of the heart after a successful balloon AS valvuloplasty. Patients and Methods: From September 2012 to 2016, 33 patients with congenital AS who had successful balloon valvuloplasty were enrolled into this study. All patients were evaluated by 2D, M-mode, Doppler, and tissue Doppler echocardiography before and after the procedure. The data were analyzed using the SPSS statistical software. Results: The median duration of follow-up was 2 years (range = 6 months to 7.4 years). Besides, the mean of immediate peak-to-peak gradient reduction was 41.50 ± 17.36 mmHg. Moderate to severe aortic insufficiency occurred in less than 28% of the patients. Additionally, M-mode echocardiography showed residual left ventricular hypertrophy in 50% of the patients. Moreover, E/A ratio was less than -2 Z-scores in 73% of the patients. E/Ea ratio was also more than 2 Z-scores in 50% of the patients, which was an indicator of diastolic dysfunction. The most common associated lesion was bicuspid aortic valve in 48% of the cases. Conclusions: Balloon aortic valvuloplasty effectively reduced peak systolic pressure gradient across the aortic valve in patients with congenital AS although left ventricular hypertrophy and diastolic dysfunction persisted in a significant number of patients. [ABSTRACT FROM AUTHOR]