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Background: Three-dimensional echocardiography (3DE) is comparable to cardiac magnetic resonance imaging for estimating ventricular volume in congenital heart diseases. However, there are limited data on estimation of ventricular volumes by 3DE in univentricular heart and change in ventricular volumes after surgical creation of cavopulmonary connection. We sought to quantify the unloading of the single ventricle of left ventricular (LV) morphology by 3DE after superior cavopulmonary anastomosis (SCPA) or Fontan operation over a period of 3 months and thereby derive a preliminary 3DE data set on this patient subset. Patients and Methods: Eighteen patients with functional single ventricle of LV morphology, who underwent SCPA or completion of Fontan circulation, were included in the study. Volume of the ventricle was estimated by 3DE before surgery and after surgery (in the early postoperative phase and 3 months after surgery), and indexed end-diastolic volume (EDV), end-systolic volume (ESV), and ejection fraction (EF) were derived. Results: Twelve patients underwent SCPA and six patients underwent staged completion of Fontan circulation. Before surgery, EDV was similar in both groups. There was a significant fall in EDV immediately after SCPA (from 48.3 ± 14.9 ml/m² to 39.5 ± 12.3 ml/m²). However, EDV increased at 3 months' follow-up to 41.3 ± 10.5 ml/m². There was no significant fall in EDV immediately after Fontan operation (47.2 ± 10.1 ml/m²-46.6 ± 14.2 ml/m²), but EDV continued to fall at 3 months of follow-up (44.7 ± 10 ml/m²). There was no significant change in ESV in either group, but EF fell significantly after SCPA. Conclusions: We provide preliminary information on 3DE volume data of single ventricle of LV morphology and the pattern of unloading after SCPA and Fontan operation. Immediate significant volume unloading occurred after SCPA which tended to catch-up after 3 months, whereas continued fall in ventricular volume with time was noted after Fontan. [ABSTRACT FROM AUTHOR]

Hypertension is common in children with chronic kidney disease (CKD) and is a major determinant of CKD progression. Ambulatory blood pressure monitoring (ABPM) has been proposed to be better in detecting hypertension as compared to casual blood pressure (CBP). This study aims to study the usefulness of ABPM in detecting masked hypertension, evaluating the adequacy of blood pressure (BP) control and predicting left ventricular hypertrophy (LVH) amongst children with CKD. A prospective cross-sectional study of 46 children with stage 3-5 CKD was conducted at the Pediatric Nephrology department of a tertiary hospital in South India. All children underwent CBP, ABPM and an echocardiography. Results were categorized as normal BP; confirmed hypertension; masked hypertension and white coat hypertension. Out of 46 children studied, 11 were undergoing dialysis. While 39.1 % children had stage 3 and 4 CKD each, 21.7% had stage 5 CKD. Masked hypertension was detected in 19.6% and 21.7% had confirmed hypertension. Thirty-four (73.9%) children were already receiving antihypertensive medication. In these, CBP was elevated in 23.5% and ABP in 47%. Among children with hypertension as defined by ABPM, LVH was detected in 32.2%. We found that higher the number of abnormal ABPM indices (assessed by BP Index, nocturnal dipping and BP Load) higher the likelihood of LVH (P = 0.046). ABPM is better in detecting hypertension and monitoring adequacy of treatment in children with CKD. The high prevalence of masked hypertension and its association with LVH supports early echocardiography and ambulatory BP monitoring to evaluate cardiovascular risks in this population. [ABSTRACT FROM AUTHOR]

Weight gain after kidney transplant is common, and may be related to graft dysfunction and high cardiovascular risk. We investigated the prevalence of obesity and evaluated the relationship between obesity and graft dysfunction in kidney transplant recipients (KTRs). All patients who received kidney transplant at the Charlotte Maxeke Johannesburg Academic Hospital (CMJAH) between January 2005 and December 2009 were recruited. Information on demographics, clinical characteristics and post-transplant care were documented. All patients underwent transthoracic echocardiography and carotid Doppler ultrasound for the assessment of cardiac status and carotid intima-media thickness (cIMT), respectively. Inferential and modelling statistics were applied. One hundred KTRs were recruited, of which 63 were males. The mean age was 42.2 ± 12.42 years with a range of 19-70 years. The mean body mass index and waist circumference of the recipients were 26.4 ± 4.81 kg/m² and 90.73 ± 14.76 cm, respectively. Twenty-nine patients (29%) were obese; of these, 24 (82.8%) had moderate obesity, 4 (13.8%) had severe obesity, and 1 (3.4%) had morbid obesity. Graft dysfunction was present in 52%. Obese patients were older (P< 0.0001), had graft dysfunction (P = 0.03), higher mean arterial blood pressure (P = 0.022), total cholesterol (P= 0.019), triglycerides (P< 0.0001), left ventricular mass index (P = 0.035) and cIMT(P = 0.036). Logistic regression showed obesity to be independently associated with graft dysfunction (P = 0.033). Obesity after kidney transplantation is common and is associated with graft dysfunction and markers of atherosclerosis. [ABSTRACT FROM AUTHOR]

Background: Improvement of endothelial dysfunction could prevent or delay the occurrence of the atherosclerosis process in patients with Kawasaki disease (KD). It is suggested that Vitamin C could improve endothelial dysfunction. In this study, we investigated whether administration of Vitamin C as a water-soluble antioxidant could be effective in this regard among patients with KD. Materials and Methods: In this case control analytic-experimental study, children aged 3-18 years with KD, and a group of healthy children evaluated. Vitamin C (250 mg/daily) administrated for the two studied groups for 1 month. Endothelial function evaluated by flow-mediated dilatation (FMD) and intima-media thickness (IMT) measurement using vascular Doppler ultrasonography, before and after trial. Results: In this study, 16 patients with KD and 19 normal children were studied. At baseline mean of IMT and FMD was not different in the two studied groups (P > 0.05). After Vitamin C administration IMT decreased significantly in two studied groups (from 27.06 ± 6.27 to 21.56 ± 3.77 in KD group and from 27.66 ± 5.66 to 23.33 ± 3.66 in control group [P < 0.05]). FMD increased in two studied groups, but the difference was significant in the control group (from 6.84 ± 2.51 to 7.03 ± 2.87 in KD group and from 6.53 ± 2.36 to 7.82 ± 2.14 in the control group). Conclusion: Vitamin C might improve the endothelial function of patients with KD. [ABSTRACT FROM AUTHOR]

AIMS: The main objective of this study is to describe patients' clinical characteristics and physiological and hemodynamic parameters at the time of diagnosis in a pulmonary hypertension center in Saudi Arabia. MATERIALS AND METHODS: This study reports the results from a single pulmonary hypertension specialized center in Riyadh, Saudi Arabia, namely Prince Sultan Medical Military City/Cardiac Center (PSMMC & CC). Both newly diagnosed (incidence) and referred (prevalence) cases of pulmonary arterial hypertension are included. All characteristics, including clinical, physiological, and hemodynamic parameters at the time of diagnosis are described. RESULTS: A total of 107 patients were identified as having pulmonary arterial hypertension as diagnosed by right heart catheterization. The mean age at diagnosis was 36 (± 9) years, and there was a female preponderance of 62.6%. The mean duration between symptom onset and diagnosis was 27.8 (± 9.0) months. At the time of enrollment, 56.1% of patients were in functional class III and 16.8% were in functional class IV. Fifty five patients (51.4%) were diagnosed as idiopathic pulmonary arterial hypertension, 29 patients (27.1%) as congenital heart disease associated with pulmonary arterial hypertension, 16 patients (15.0%) as connective tissue diseases associated with pulmonary arterial hypertension, 4 patients (3.7%) as heritable pulmonary arterial hypertension, and 3 patients (2.8%) as portopulmonary hypertension. CONCLUSION: This data highlights the current situation of pulmonary arterial hypertension in Saudi Arabia. Our patients are much younger than patients described in other international registries but still detected as late in the course of the disease. A majority of patients displays severe functional and hemodynamic compromise. [ABSTRACT FROM AUTHOR]

Background: Echocardiography is one of the diagnostic tools that can be applied at the bedside, along with avoiding transporting critically ill patients. This prospective observational study was designed to assess the clinical applicability of the transthoracic echocardiography (TTE) device by noncardiologist intensivists. Materials and Methods: Intensivists performed a limited TTE examination on critically ill patients admitted to the surgical intensive care unit (ICU). After initial cardiac clinical assessment in 85 critically ill adult patients, a limited TTE was performed by an intensivist to assess left ventricular (LV) function and LV volume status as well as valvular function and qualitative factors. Data were analyzed and presented in proportions using descriptive statistics. The setting was in surgical ICU of an academic medical center. Results: Valvular abnormalities (44.8%) as well as qualitative indices (68.3%) were the most frequently detected abnormalities. The ejection fraction was the only alteration, which was affected by the risk factors (P = 0.05, mean = 55.57). Conclusion: Transthoracic approach can provide useful information on cardiac anatomy and function in most ICU patients along with detecting severe previously unknown conditions in some patients. [ABSTRACT FROM AUTHOR]

Chronic kidney disease related-mineral bone disorder (CKD-MBD) has been poorly studied in pre-dialysis Indian CKD patients. We aimed to study the clinical, biochemical and extra skeletal manifestations of untreated CKD-MBD in pre-dialysis Stage 4 and 5 CKD patients attending nephrology out-patient clinic at a tertiary care hospital in South India. A hospital based cross-sectional survey including, demographic profile, history of CKD-MBD symptoms, measurement of serum calcium, phosphate, parathyroid hormone, 25 hydroxy vitamin D (25(OH) D) and alkaline phosphatase; lateral abdominal X-rays for abdominal aortic calcification (AAC) and echocardiography for valvular calcification (VC) was carried out. Of the 710 patients surveyed, 45% had no CKD-MBD related symptom. Prevalence of hypocalcemia, hyperphosphatemia, hyperparathyroidism (>150 pg/mL) and 25(OH) D levels <30 ng/mL was 66.3%, 59%, 89.3% and 74.7% respectively. Echocardiography was carried out in 471 patients; 96% of whom had VC (calcification score ≥1). Patients with VC were older and had lower 25(OH) D levels than those without. Lateral abdominal X-rays were obtained in 558 patients, 6.8% of whom were found to have AAC, which was associated with older age. Indian patients with incident CKD-MBD have a high prevalence of hypocalcemia, 25(OH) D deficiency and VC even prior to initiating dialysis while AAC does not appear to be common. The association between 25(OH) D deficiency and VC needs further exploration. [ABSTRACT FROM AUTHOR]

Background: The dilated aorta in adults with bicuspid aortic valve has been shown to have different shapes, but it is not known if this occurs in children. This observational study was performed to determine if there are different shapes of the dilated aorta in children with bicuspid aortic valve and their association with age, gender, hemodynamic alterations, and degree of aortic enlargement. Methods: One hundred and eighty-seven echocardiograms done on pediatric patients (0 . 18 years) for bicuspid aortic valve, during 2008, were reviewed. Aortic valve morphology, shape/ size of the aorta, and pertinent hemodynamic alterations were documented. Aortic dilation was felt to be present when at least one aortic segment had a z-score > 2.0; global aortic enlargement was determined by summing the aortic segment z-scores. The aortic shape was assessed by age, gender, valve morphology, and hemodynamic alterations. Results: Aortic dilation was present in 104/187 patients. The aorta had six different shapes designated from S1 through S6. There was no association between the aortic shape and gender, aortic valve morphology, or hemodynamic abnormalities. S3 was the most common after the age of six years and was associated with the most significant degree of global aortic enlargement. Conclusions: The shape of the dilated aorta in children with bicuspid aortic valve does not occur in a uniform manner and multiple shapes are seen. S2 and S3 are most commonly seen. As aortic dilation becomes more significant, a single shape (S3) becomes the dominant pattern. [ABSTRACT FROM AUTHOR]

CONTEXT: Several international studies have described the epidemiology of pulmonary hypertension (PH). However, information about the incidence and prevalence of PH in Saudi Arabia is unknown. AIMS: To report cases of PH and compare the demographic and clinical characteristics of PH due to various causes in a Saudi population. METHODS: Newly diagnosed cases of PH [defined as mean pulmonary artery pressure ≥25 mmHg at right heart cauterization (RHC)] were prospectively collected at a single tertiary care hospital from January 2009 and June 2012. Detailed demographic and clinical data were collected at the time of diagnosis, along with hemodynamic parameters. RESULTS: Of the total 264 patients who underwent RHC, 112 were identified as having PH. The mean age at diagnosis was 55.8 ± 15.8 years, and there was a female preponderance of 72.3%. About 88 (78.6%) of the PH patients were native Saudis and 24 (21.4%) had other origins. Twelve PH patients (10.7%) were classified in group 1 (pulmonary arterial hypertension), 7 (6.2%) in group 2 (PH due to left heart disease), 73 (65.2%) in group 3 (PH due to lung disease), 4 (3.6%) in group 4 (chronic thromboembolic PH), and 16 (14.3%) in group 5 (PH due to multifactorial mechanisms). PH associated with diastolic dysfunction was noted in 28.6% of group 2 patients, 31.5% of group 3 patients, and 25% of group 5 patients. CONCLUSIONS: These results offer the first report of incident cases of PH across five groups in Saudi Arabia. [ABSTRACT FROM AUTHOR]

Endothelial dysfunction (ED), insulin resistance (IR), and inflammation are risk factors for increased cardiovascular morbidity and mortality in autosomal dominant polycystic kidney disease (ADPKD). ADPKD patients may have increased carotid intima-media thickness (CIMT) and decreased coronary flow velocity reserve (CFVR). The neutrophil-to-lymphocyte ratio (NLR) was introduced as a marker to determine inflammation in various disorders. We aimed to investigate the relationship between NLR and IR, CFVR, CIMT, and the left ventricular mass index (LVMI) in normotensive ADPKD patients. Twentynine ADPKD patients (age 38.8 ± 10.2 years; 8 men and 21 women) and 19 healthy controls (age 33.8 ± 7.4 years; 8 men and 11 women) were included in this crosssectional study. CFVR was calculated with echocardiography as the ratio of hyperemic to baseline diastolic peak coronary flow velocities. CIMT was measured in the distal common carotid artery by using a 10-MHz linear echocardiography probe. HOMA-IR was calculated NLR was calculated as the ratio of the neutrophil and lymphocyte counts. Age, sex, body mass index, and levels of glucose, creatinine, low-density lipoprotein (LDL)-cholesterol, high-density lipoprotein (HDL)-cholesterol, triglycerides, C-reactive protein (CRP), microalbuminuria, and creatinine clearance were similar between ADPKD patients and healthy subjects. NLR, CIMT, LVMI, and HOMA-IR were significantly higher and CFVR values were significantly lower in patients with ADPKD compared to that in healthy subjects. NLR showed positive correlation with CIMT, HOMA, insulin, glucose, and HDL cholesterol levels, while it was inversely correlated with CFVR and albumin level in all subjects. In patients with ADPKD, NLR showed positive correlation with HDL cholesterol level and inverse correlation with LVMI and albumin level. NLR that was found to be increased in patients with ADPKD may be a readily available marker of inflammation and ED. [ABSTRACT FROM AUTHOR]

Background : The objective of this study was to assess the results of the pulmonary artery (PA) banding in patients with congenital heart defects (CHD) and pulmonary hypertension (PH) in the current era. Methods : We analyzed data from 305 patients who underwent PA banding between April 2005 and April 2010 at our centre. All patients were approached through a left thoracotomy. Twenty percent of patients underwent PA banding based on Trusler's rule (Group 1), 55% of them underwent PA banding based on PA pressure measurement (Group 2), and the rest of them (25%) based on surgeon experience (Group 3). The follow-up period was 39 ± 20 month and 75% of patients (230 cases) had definitive repair at mean interval 23 ± 10 months. Results : The rate of anatomically and functionally effectiveness of PA banding in all groups was high (97% and 92%, respectively). There were no significant differences in anatomically and functionally efficacy rate between all groups (P=0.77, P=0.728, respectively). There was PA bifurcation stenosis in six cases (2%), and pulmonary valve injury in one case (0.3%). The mortality rate in PA banding was 2% and in definitive repair was 3%. Conclusions : We believe that PA banding still plays a role in management of patients with CHD, particularly for infants with medical problems such as sepsis, low body weight, intracranial hemorrhage and associated non cardiac anomalies. PA banding can be done safely with low morbidity and mortality. [ABSTRACT FROM AUTHOR]

Objective : To assess brain development and brain injury in neonates with cyanotic and acyanotic congenital heart disease (CHD). Methods : The study included 52 term infants with CHD who were divided into two groups: Cyanotic (n=21) and acyanotic (n=31). Fifteen healthy neonates of matched age and sex were enrolled in the study as controls. Three-dimensional proton magnetic resonance spectroscopic imaging and diffusion tensor imaging were used to assess brain development and injury. We calculated the ratio of N-acetylaspartate (NAA) to choline (which increases with maturation), average diffusivity (which decreases with maturation), fractional anisotropy of white matter (which increases with maturation), and the ratio of lactate to choline (which increases with brain injury). Results : As compared with control neonates, those with CHD had significant decrease in NAA/ choline ratio (P<0.001), significant increase in lactate/choline ratio (P<0.0001), significant increase in average diffusivity (P<0.0001), and significant decrease of white matter fractional anisotropy (P<0.001). Neonates with cyanotic CHD had significant less brain development and more brain injury than those with acyanotic CHD (P<0.05). Conclusions : Newborn infants with cyanotic and acyanotic CHD are at high risk of brain injury and impaired brain maturity. [ABSTRACT FROM AUTHOR]