Abstract:Introduction: Behçet's disease (BD) is a chronic relapsing inflammatory disease. 40-70% of BD patients display rheumatic features with a 9% incidence as an initial presentation. However, there are no pathognomonic laboratory tests in BD and the diagnosis depends on the occurrence and grouping of sufficient clinical manifestations to allow the physician to either suspect, or definitely diagnose the condition. Our aim was to assess the utility of Tc-99m methylene diphosphonate (Tc-99m MDP) bone scintigraphy for the detection of articular involvement in BD patients and its impact on disease activity & severity scoring.Methods: 25 BD patients diagnosed according to the international study group criteria for BD diagnosis (ISG) [18 , 7 , mean age 32.44±7.78, disease duration 6.98±4.61year] were included and subjected to selected joint x-ray and bone scintigraphy.Results: 25/25 was scintigraphically positive for arthritis [100%, 64%, 44%, 40%, 36%& 20% in wrist, Metacarpophalangeal, proximal interphalangeal (PIPs), distal interphalangeal (DIPs), Knees & ankles respectively]. 13/25 were clinically negative while scintigraphically positive for arthritis. 11/25 (44%) and 8/25 (32%) turned from inactive to active BD and from mild to moderate disease severity, based on their positive hand scintigraphy with treatment modification. 4/25 (16%) had recurrent oral ulcers with normal laboratory data and only positive hand scintigraphy; with follow-up they met ISG criteria.Conclusion: skeletal scintigraphy is a simple diagnostic modality with a low radiation burden, when compared to conventional radiology. Hence, when used appropriately in the diagnostic algorithm of BD, bone scintigraphy can be used for detection of early joint involvement, assessment of the extent and activity status with guidance for the selected therapy.
Background Pulmonary arterial hypertension (PAH) is a disease of the pulmonary circulation with resultant effects on the right heart. Conceptually, left heart anatomy and function should be normal in patients with PAH. However, the RV and left ventricle (LV) do not function in isolation, sharing a common pericardial sac and inter-ventricular septum which in turn refers to the significance of assessment of both ventricles which has implications for patient prognosis with PAH. Aim Study the correlation between echocardiographic hemodynamic parameters and functional capacity in patients with pulmonary hypertension due to congenital heart disease and detection which parameter is the most independent predictor of functional capacity in patients with PAH-CHD. Methodology The study was conducted in the period from November 2016 to September 2017 including 34 patients who had been diagnosed with pulmonary arterial hypertension associated with congenital heart diseases (PAH-CHD). All of them were recruited from the congenital heart diseases unit at Ain Shams University hospital. The studied group was divided into two groups in a cross-sectional study: Group I: This included 21 patients with functional capacity according the 6MWT less than 330 m as an average for all subjects. Group II: This included 13 patients with functional capacity more than 330 m. Patients’ participation was voluntary and the subject had the right to withdraw from the study at any time without affecting his/her further medical care. Results The whole study population was divided according to the 6MW distance into two groups less than 330 and more than or equal 330 m. Comparing the 6MWD of each patient with the different Echocardiographic variables of systolic & diastolic dysfunction revealed that the group of shorter 6MW distance had higher RVSP, MPAP, E/e’ ratio, LVMPI and RVMPI, while they had lower LV e’ TDI, LV GRS, RV FAC and RV GLS (less negative value). Multivariate analysis of these Echocardiographic variables revealed that MPAP is independent predictor of 6MWD & thus an independent predictor of functional capacity in PAH-CHD patients. Conclusion Assessment of RV and LV function in PAH-CHD patients is important. Strain measurement by STE provides valuable information on the quantitative RV and LV functional assessment in the setting of volume and pressure overload. The MPAP and saturation were found to be the most independent predictors affecting the functional capacity of the patients with PAH-CHD. Recommendations Assessment of functional capacity in patients with PH using the 6MWT is recommended as a routine investigation from diagnosis and during follow-up and correlated with the echocardiographic variables, it has been shown to be practical and feasible and provides an easy and not expensive tool for clinical and prognostic assessment.
Background Functional single ventricle (FSV) is a spectrum of severe congenital heart disease, with multiple anatomic variations but similar surgical treatment strategies. FSV patients are living longer into adulthood compared to two or three decades ago, and they are more frequently undergoing imaging to assist in clinical and surgical management. Objective To determine impact of ventricular morphology on myocardial deformation in patients with single ventricle. Material and Methods From November 2017 to July 2018, 41 patients with single ventricle morphology or Physiology who were referred to Ain-Shams University hospitals for follow up Echocardiography or diagnostic cardiac catheterization were enrolled in the study.All patients subjected to history, clinical examination Echocardiography (conventional 2D and speckle tracking “global longitudinal strain and strain rate”.Dominant ventricular strain was analyzed using conventional two-dimensional echocardiographic. The region of interest was obtained by tracing the dominant ventricle endocardial borders at end-systole. An automated software program was used to calculate the frame-to-frame displacements of speckle pattern within the region of interest throughout the cardiac cycle. Results There is statiscially significance between the basic anatomy of the single ventricle and the ejection fraction (p value 0.001) and global longitudinal strain pattern by speckle tracking echocardiography with (p value 0.031) with no statistically effect of the side of dominance. Conclusion: Regarding the side of dominant ventricle there is no significant relation between the side of dominant ventricle on myocardial deformation (by ejection fraction, global strain and strain rate by speckle tracking echocardiography) but there is significant relation between the ventricular morphology and the myocardial deformation that was assessed by by the ejection fraction and global longitudinal strain (by speckle tracking echocardiography) but with no significant effect on the strain rate (by speckle tracking echocardiography)
Background: Pulmonary hypertension (PH) with congenital heart disease (CHD) affects the functional capacity (FC), quality of life, and survival. However, the importance of different echocardiographic parameters and their correlation with FC is unclear. Methods and Results: A custom-made sheet for 34 consecutive patients with PH due to CHD was made to include patient's demographic data, underlying cardiac disorder, and FC by 6-min walk test (6MWT). The patients were subdivided into Group 1 with 6MWT < 330 m and Group 2 with 6MWT > 330 m. A cutoff value of 330 m was selected because it reflected the survival and outcome of patients in many studies before. Left ventricle global radial strain, baseline saturation, and saturation after 6MWT showed a significant strong positive correlation with 6MWT ( r = 0.755, 0.714, and 0.721, P = 0.001, 0.000, and 0.000, respectively). Multiple regression analysis using a multivariate model showed that the mean pulmonary artery pressure (MPAP) and baseline saturation are the most independent predictors of the FC ( P = 0.028 and 0.049, respectively), with a cutoff point for MPAP > 30 mmHg (area under the curve [AUC]: 0.85) with a sensitivity and specificity of 69.23% and 95.24%, respectively, and cutoff point for saturation < 94% (AUC: 0.852) with a sensitivity and specificity of 92.31% and 76.19%, respectively. Conclusion: The MPAP and the baseline oxygen saturation were the most independent predictors of impaired FC. They can be used for risk stratification and as surrogate predictors of outcome in this group of patients.
Aim and objectives The aim of this study was To correlate between 3D echocardiographic assessment of RV volumes and functions with invasive RV quantification by angiography in children with mainly valvular pulmonary stenosis undergoing balloon pulmonary valvuloplasty pre & post balloon dilation, to evaluate the immediate impact of relieving obstruction on RV volumes. Patients and Methods The study included 25 patients in the pediatric age group (up to 18 years old) with Pulmonary valve stenosis referred to our congenital and structural heart disease unit cardiology department in Ain shams university hospital for balloon pulmonary valvuloplasty. All patients were subjected to history taking, clinical examination and ECG recording. Then echocardiographic assessment by 2D and 3D was done to all patients pre and post BPV. All patients were subjected to have pre and post RV angiography for assessment of RV volumes and functions angiographically. Results The study included 25 patients,65% of patients were males and 44% were females, mean of age was 22.79 with range from 2 to 144 months There was statistically significant increase in 3D Echo RV quantification post BPV in comparison to 3D Echo RV quantification pre BPV with RV/EDV(p-value:0.001), RV/ESV(p-value:0.034), RV/SV(p-value:0.002), FAC %(p-value:0.014), TAPSE(p-value:0.001). Also,there was statistically significant increase in angiographic RV quantification post BPV in comparison to angiographic RV quantification pre BPV with EDV(p-value:<0.001), ESV(p-value:<0.001), SV(pvalue:<0.001) and EF %(p-value:<0.001). Conclusion 3DE RV volumes and function shows good correlation and agreement with invasive RV quantification by angiography in children with valvular pulmonary stenosis undergoing balloon pulmonary valvuloplasty.
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