Hematologic abnormalities in cyanotic congenital heart disease patients

Introduction: Patients with cyanotic heart disease may have an acceptable quality of life. However, they are invariably prone to several complications. The aim of this study is search about hematologic abnormalities in cyanotic congenital heart disease patients

Materials and Methods: In this cross-sectional study, every cyanotic congenital heart disease patients who was referred to the adult congenital heart disease clinic was selected and asked of any possible hyperviscosity symptoms, gingival bleeding, epistaxis, hemoptysis, hypermenorrhagia, and gouty arthritis irrespective of their age, gender and primary diagnosis in a six-month period. In this regard, 02 saturation was obtained via pulse oximetry, an abdominal ultrasound was done in order to discover any gallstones and laboratory tests including CBC, coagulation parameters [bleeding time[BT],clotting time[CT], prothrombin time[PT],international ratio[ INR], Ferritin, blood urea nitrogen [BUN] and creatinine [Cr] were provided as well

Results: A total of 69 patients were enrolled in the present study. The mean age of the patients was 22.44 +/- 5.72. Twenty two [34.4%] of them were female and 45[65.6%] were male. In our research, 23% of the patients had serum levels of uric acid more than or equal to 8 mg/dl. 59%of patients presented with depleted iron storage. Mean hemoglobin [Hg], level was [16.9 +/- 2.5 mg/dl], mean hematocrit [HCT] level was [55.47 +/- 9 mg/dl], mean Ferritin level was [32.5 +/- 0.4]. Our collected data also revealed that plasma creatinine concentration was normal [0.9 +/- 0.2 mg/dl]. Furthermore, thrombocytopenia of less than 160000 was found in 30% of the patients. Hyperviscosity symptoms also found in 1.3% of patients

Conclusion: Our patients had less hyperuricemia, there is no correlation between hyperviscosity symptoms and haematocrit level and an inverse correlation between the Ferritin level and hyperviscosity symptoms were seen

Assessment of subclinical left ventricular dysfunction in patients with chronic mitral regurgitation using torsional parameters described by tissue doppler imaging

Left ventricular [LV] twist is due to oppositely directed apical and basal rotation and has been proposed as a sensitive marker of LV function. We sought to assess the impact of chronic pure mitral regurgitation [MR] on the torsional mechanics of the left human ventricle using tissue Doppler imaging. Nineteen severe MR patients with a normal LV ejection fraction and 16 non-MR controls underwent conventional echocardiography and apical and basal short-axis color Doppler myocardial imaging [CDMI]. LV rotation at the apical and basal short-axis levels was calculated from the averaged tangential velocities of the septal and lateral regions, corrected for the LV radius over time. LV twist was defined as the difference in LV rotation between the two levels, and the LV twist and twisting/untwisting rate profiles were analyzed throughout the cardiac cycle. LV twist and LV torsion were significantly lower in the MR group than in the non-MR group [10.38° +/- 4.04° vs. 13.95° +/- 4.27°; p value = 0.020; and 1.29 +/- 0.54 °/cm vs. 1.76 +/- 0.56 °/cm; p value = 0.021, respectively], both suggesting incipient LV dysfunction in the MR group. Similarly, the untwisting rate was lower in the MR group [-79.74 +/- 35.97 °/s vs.-110.96 +/- 34.65 °/s; p value = 0.020], but there was statistically no significant difference in the LV twist rate. The evaluation of LV torsional parameters in MR patients with a normal LV ejection fraction suggests the potential role of these sensitive variables in assessing the early signs of ventricular dysfunction in asymptomatic patients

Silent pulmonary artery dissection in a patient with old pulmonary balloon valvuloplasty

Percutaneous pulmonary balloon valvuloplasty [PBV] remains the treatment of choice for pulmonary stenosis [PS]. This procedure is effective, safe and gives excellent results. Pulmonary artery [PA] dissection is a rare complication of PBV. This report is a case of an asymptomatic 17-year-old male with a history of PBV due to severe PS dating back to fifteen years ago. During recent echocardiography, an intimal flap was detected in the main PA and entry site was clearly seen by contrast study

rare case of congenital heart disease with bifid cardiac apex: a unique finding in left ventricle

Bifid cardiac apex is a rare anomaly of human hearts. We report of the case of a 34-year-old man with a previous history of ventricular septal defect [VSD] and subvalvular pulmonary stenosis. He had undergone pulmonary commissurotomy and VSD closure 22 years before he was referred to our center for evaluation of progressive dyspnea. Transthoracic echocardiography revealed atrial septal defect [ASD], multiple VSDs, severe pulmonary regurgitation, and a bifid cardiac apex. The patient was referred for re-do surgery for ASD and VSD closure along with pulmonary valve replacement, but he refused the surgery

Noninvasive localization of accessory pathways in patients with Wolff-Parkinson-White syndrome: a strain imaging study

Noninvasive techniques for the localization of the accessory pathways [APs] might help guide mapping procedures and ablation techniques. We sought to examine the diagnostic accuracy of strain imaging for the localization of the APs in Wolff-Parkinson-White syndrome. We prospectively studied 25 patients [mean age = 32 +/- 17 years, 58.3% men] with evidence of pre-excitation on electrocardiography [ECG]. Electromechanical interval was defined as the time difference between the onset of delta wave and the onset of regional myocardial contraction. Time differences between the onset of delta wave [delta] and the onset of regional myocardial contraction [delta-So], peak systolic motion [delta-Sm], regional strain [delta-epsilon], peak strain [delta-epsilonp], and peak strain rate [delta-SRp] were measured. There was a significant difference between time to onset of delta wave to onset of peak systolic motion [mean +/- SD] in the AP location [A] and normal segments [B] versus that in the normal volunteers [C] [A: [57.08 +/- 23.88 msec] vs. B: [75.20 +/- 14.75] vs. C: [72.9 0 +/- 11.16]; p value [A vs. B] = 0.004 and p value [A vs. C] = 0.18] and [A: [49.17 +/- 35.79] vs. B: [67.60 +/- 14.51] vs. C: [67.40 +/- 6.06 msec]; p value [A vs. B] < 0.001 and p value [A vs. C] = 0.12, respectively]. Our study showed that strain imaging parameters [[delta-So] and [delta-Strain]] are superior to the ECG in the localization of the APs [84% vs. 76%]

role of echocardiography in coronary artery disease and acute myocardial infarction

Echocardiography is a non-invasive diagnostic technique which provides information regarding cardiac function and hemodynamics. It is the most frequently used cardiovascular diagnostic test after electrocardiography and chest X-ray. However, in a patient with acute chest pain, Transthoracic Echocardiography is essential both for diagnosing acute coronary syndrome, zeroing on the evaluation of ventricular function and the presence of regional wall motion abnormalities, and for ruling out other etiologies of acute chest pain or dyspnea, including aortic dissection and pericardia! effusion. Echocardiography is a versatile imaging modality for the management of patients with chest pain and assessment of left ventricular systolic function, diastolic function, and even myocardial and coronary per fusion and is, therefore, useful in the diagnosis and triage of patients with acute chest pain or dyspnea. This review has focused on the current applications of echocardiography in patients with coronary artery disease and myocardial infarction

Development and validation of the first Iranian questionnaire to assess quality of life in patients with heart failure: IHF-QoL

In its Constitution of 1948, WHO defined health as "a state of complete physical, mental, and social well-being, and not merely the absence of disease and infirmity". In 1994, the Agency for Health Care Policy and Research published clinical practice guidelines recommending providers to routinely evaluate patients' HRQoL [Health Related Quality of Life] and use their assessment to modify and guide patient care. To create a valid, sensitive, disease-specific Persian health status quality of life questionnaire for patients with chronic heart failure in Iran. Considering the existing relevant inventories and scientific literature, the authors designed the first draft of questionnaire which was modified and validated, using expert opinions and finalized in a session of expert panel. The questionnaire was processed among 130 patients with heart failure. Construct validity evaluated by principle component factor analysis, and promax method was used for factor rotation. MacNew quality of life questionnaire was selected to assess convergence validity, and the agreements were measured in 60 patients. Discriminant validity was also assessed. Thirty patients were followed for 3 months and responsiveness of questionnaire was measured. Cronbach's alpha, item analysis, and Intra-class correlation coefficients [ICCs] were used to investigate reliability of questionnaire. SPSS 15 for Windows was applied for statistical analysis. Principle component factor analysis revealed 4 main components. Sub-group analysis suggested that IHF-QoL questionnaire demonstrated an acceptable discriminant validity. High conformity between this inventory and MacNew questionnaire revealed an appropriate convergence validity. Cronbach's alpha [alpha] for the overall questionnaire was equal to 0.922. Intra-class correlation coeffcients [ICCs] for all components were significant [from. 708 to. 883; all P values < 0.001]. Patients fallow-up revealed an acceptable responsiveness of our questionnaire. IHF-QoL questionnaire is a valid and reliable inventory. It can be applied in daily clinical practice and in the clinical research context

Liver function tests and uric acid serum levels in relation to hemodynamic profile: a comparison between heart failure and pulmonary arterial hypertension patients

Systemic venous congestion has been considered the main cause of liver dysfunction in heart failure patients. In this study, we assessed the relation of liver function tests to hemodynamic profile in patients with systolic heart failure [SHF] and primary pulmonary arterial hypertension [PAH]. Fifty patients with left ventricular ejection fraction < 35% and 27 patients with mean PAH> 25 mmHg were enrolled. Hemodynamic indices, including PAP, right atrial and ventricular pressures [RAP and RVP], pulmonary capillary wedge pressure [PCWP], and cardiac simultaneously. Fifty age- and sex-matched normal controls were also studied. CO was significantly lower in the HF group [P= 0.006]. Multivariate analysis showed a significant correlation between total bilirubin level and mean PAP [R=0.04, P=0.004], RAP [R=0.33, P<0.03], RVP [R=0.43, P<0.004], PCWP [R=0.36,P<0.01], and direct bilirubin with mean PAP [R=0.01, P=0.02], RVP[R=0.33, P<0.03], PCWP[R=0.32, P<0.03], and CI [R=0.33, P<0.01]. In the PAH group, such correlations were mostly absent. Systemic venous congestion, more prominent in PAH, might not play the main role in deteriorating liver function. Further studies are needed to determine whether hepatic blood flow, which is significantly decreased in SHF, is a more important factor

Tissue doppler imaging and doppler studies in patients with transmural and non-transmural myocardial infarction

Myocardial longitudinal shortening play an important role in cardiac contraction [1,2]. Tissue velocity imaging [TVI] is an ultrasonographic technique that measure myocardial motion and providing a quantitative agreement of left ventricular regional myocardial function in different modalities [3]. The present review discusses the most recent development in the application of TDI in coronary artery disease. Seventy patients with myocardial infarction [transmural and nontransmural] were included in the study. These subjects were diagnosed with recent myocardial infarction wall [septal side of mitral annulus] and basal segment of base of RV free wall were examined for tissue Doppler study with complete transthoracic echocardiography study. Mean age in group of inferior MI, anterior MI and non Q wave MI are as follows: 61.87 +/- 10.7, 57.04 +/- 10.7, 58.45 +/- 9.2. Sm was significantly reduced in anterior MI groups than non Q wave MI [PV=0.01]. In patients with inferior myocardial infarction 88% of patients had left ventricular ejection fraction [LVEF]>45% and in patients with anterior MI 18.2% patients had EF>45%. In non Q wave MI groups 60% patients had LVEF>45%. Except for Sm, other TDI parameters had no significant difference between two groups [transmural and nontransmural infarction] but it has significant changes in reduced left ventricle function and could be of determinants for prognosis

evolutionary development of echocardiography

Echocardiography is a non-invasive diagnostic technique which provides information on cardiac morphology, function, and hemodynamics. It is the most frequently used cardiovascular diagnostic test only after electrocardiography. In less than five decades, the evolution in this technique has made it the basic part of cardiovascular medicine. Herein, the evolution of various forms of echocardiography is briefly described

Physician-related factors affecting cardiac rehabilitation referral

Despite the positive impact of cardiac rehabilitation [CR] on quality of life and mortality, the majority of people who could benefit from this program fail to participate in it. The lack of referral from the physician is a common reason that patients give for not seeking CR. The objective of this study was to compare factors affecting CR referral by cardiologists. A cross-sectional survey of 122 cardiologists, including 89 general cardiac specialists and 33 fellows in cardiology from 11 major cardiology training centers in Iran, was done in 2010. They responded to the 14- item investigatorgenerated survey, examining the physician's attitudinal and knowledge factors affecting CR referral. 47.9% of the subjects reported having available CR centers but only 6.6% reported continuous medical education on the topic. 90.7% of the physicians reported that less than 15% of patients are referred to CR centers. The main factor affecting the low referral rate was limited general knowledge about CR programs [79.5%] such as program attributes and benefits, methods of reimbursement. Lack of insurance coverage, unavailability of CR centers in the community and low physicians' fee were other factors reported by the physicians. Cardiologists' inadequate general knowledge of and attitude toward CR programs seem to be a potential threat for cardiac prevention and rehabilitation in some societies

Factors associated with failure to complete phase ii cardiac rehabilitation: survey registry in rajaie cardiovascular medical and research center

A significant proportion of patients who begin CR [cardiac rehabilitation] do not complete the program. The purpose of this study was to determine the predicting factors that interfere with adherence and completion of an outpatient CR program. A cross-sectional survey was conducted with all 128 patients who entered the CR program at the Rajaie Cardiovascular Medical and Research Center in Tehran, IR Iran, from March 2009 to March 2010. The demographic variables included age, sex, education, employment status, insurance status, and return to work. These variables were compared in patients who completed and did not complete phase II CR. The reason for CRincompletion was asked in follow-up phone interviews. The most frequent clinical diagnosis among the patients enrolled in the CR program was coronary artery disease. 83.6% of patients who participated had a CABG or PCI procedure during the last year. CR participation increased when cardiac revasculariza-tion procedures were performed during the first hospitalization. 88 of the 128 patients dropped out, yielding a dropout rate of 68.7%, which was significantly [P < 0.01] higher than the same study in other countries. Sex and age did not predict the completion rate. As education increased, cardiac rehabilitation utilization and completion increased. Unemployed patients were less likely than employed patients to complete the program. Our data indicate a low rate of CR completion, with lower rates among unemployed, uninsured, and less educated patients

Pseudoaneurysm of ascending aorta with fistulization to left atrium after coronary artery bypass graft surgery

A 53-year-old man with a history of coronary artery bypass graft surgery 4 years previously was admitted to our hospital with dyspnea on exertion [New York Heart Association class II] of three months' duration, lower extremities edema of two weeks' duration, and pulmonary edema of two weeks' duration. Transthoracic and transesophageal echocardiographic examinations revealed pseudoaneurysm of the ascending aorta with fistulization to the left atrium. He was, therefore, scheduled for surgery, during which repair of the ascending aorta with a pericardial patch in conjunction with repair of the aortic valve and removal of the fistulization between the left atrium and ascending aorta was performed. The patient was discharged ten days after admission in very good physical condition. Postoperative echocardiography demonstrated only mild aortic regurgitation and no residual connection between the left atrium and ascending aorta, with the latter having a normal size

Proteinuria in congenital heart disease: is it a real problem?

The relationship between congenital heart disease and nephropathy has been known for a long time although its mechanism has not been understood thoroughly. Furthermore such studies have been performed in older populations. 74 children aged between two months to 168 months [20 normal as control group, 20 cyanotic and 34 acyanotic patients with congenital heart disease were investigated for their renal function and protein excretion. The data were analyzed using SPSS [version 16.1] independent t- test. Creatinine and glomerular filtration rate in cyanotic was lower than acyanotic group but these were not significant while both protein excretion incidence [65% vs 24%] and quantity [1.2 vs 0.2; measured as urine protein to creatinine ratio] were higher significantly in cyanotic group [P< 0. 001]. In cyanotic children with congenital heart disease proteinuria is more common and more severe compared with acyanotic patients; this is not related to age in children as it may occur in the same nephrotic range in infants with cyanotic congenital heart disease

Right ventricular dysfunction in thiamine-responsive megaloblastic anemia syndrome: a case report

Thiamine or Vitamin B[1] is an essential cofactor for many metabolic processes in numerous tissues. Thiamine-Responsive Megaloblastic Anemia Syndrome is a genetic disorder affecting thiamine transportation with consequent bone marrow, pancreatic, neurological, and cardiac functional and developmental anomalies. There are limited reports of the cardiac manifestations of this syndrome. Here, we present a case of this syndrome in a male patient with right ventricular dysfunction and atrial dysrhythmias

Prosthetic pulmonary valve malfunction

Prosthetic heart valve thrombosis is a serious complication in which systemic thrombolysis has emerged as a suitable alternative to surgery, especially for right-sided prosthetic valve thrombosis. We report the case of a thrombosed Carbomedics Medical prosthetic valve placed in the pulmonary position in a patient who had undergone total repair of tetralogy of Fallot, which was successfully treated with intravenous thrombolytic therapy with streptokinase

Post-surgical right ventricular pseudoaneurysm presenting as and epigastric pulsatile mass

We report a case of right ventricular pseudoaneurysm three weeks after open mitral valve commissurotomy. Transthoracic echocardiography showed a cavity of approximately 5 x 3 cm contiguous to the right ventricular inflow, communicating with the right ventricle by a small neck. Doppler study showed the presence of systolic and diastolic flow at the site of the rupture. Pseudoaneurysm is an infrequent surgical complication involving right ventriculotomy and often increases progressively in size. The association with open mitral valve commissurotomy has not been previously reported

Evaluation of longitudinal right ventricular mechanical dyssynchrony before and early after cardiac resynchronization therapy: a strain imaging study

The right ventricular [RV] dyssynchrony has not been extensively and the existing literature has established the effect of cardiac resynchronization therapy [CRT] on the left ventricular [LV] dyssynchrony, but there is a death of data on the effect of CRT on the forgotten ventricle. We sought to evaluate the presence of mechanical right ventricular dyssynchrony in patients with systolic heart failure, selected for CRT, and track the changes early afterward utilizing the longitudinal strain analysis. Thirty-six patients with severe left ventricular systolic dysfunction, candidated for CRT, were enrolled in this study. Mechanical dyssynchrony was assessed using tissue Doppler echocardiography. The time interval between the onset delay was calculated as the absolute value of the difference in the time-to-peak measurements between the RV and spetum. The RV dyssynchrony was defined as the calculated delay in strain imaging, which was +/- 2 SD above the mean value for the control subjects [20 cases]. The RV function was evaluated using the RV fractional area change [RVFAC], tricuspid annulus plane systolic excursion [TAPSE], and peak systolic strain values of the RV free wall. Four to 7 days after CRT implantation, echocardiographic reevaluations were done. The calculated cut-off value for the RV dyssynchrony was 41.5 msec, according to which the pre-CRT analysis specified two patient groups: Group 1 [16 cases] with RV dyssynchrony and Group 2 [20 patients] without RV dyssynchrony. Significant improvement in the RV dyssynchrony was noted in Group 1 after CRT [30 +/- 28.9 msec vs. 68.8 +/- 21 msec; p value <0.01 vs. 14 +/- 10 msec vs. 19 +/- 16.5 msec; p value = 0.18 respectively]. A significant correlation was found between the severity of the RV dyssynchrony and peak systolic strain in the RV free wall [r = -0.5; p value <0.05]. No significant relation was found between the RV dyssynchrony and right ventricle fractional area change [RVFAC], LV mechanical dyssynchrony, time-to-peak systolic strain in the RV free wall, QRS width, or morphology. In group I, the peak systolic strain increased insignificantly [p value = 0.15 for the basal segment; p value = 0.20 for the mid segment]. A moderately significant correlation was found between the RV mechanical delay before CRT vs. the post-CRT values [r = 0.4; p value = 0.01]. Early after CRT, the RV mechanical delay can improve and the significant improvement is seen in patients with baseline RV mechanical dyssynchrony

Evaluation of the relation between anterior mitral valve leaflet motion based on height-to-length ratio and the immediate outcome of percutaneous balloon mitral valvuloplasty

The purpose of this study was to investigate whether there is any relation between mitral leaflet motion based on height-to-length ratio of the anterior mitral valve leaflet doming in diastole and the immediate outcome of balloon mitral valvuloplasty,. The study population consisted of 49 patients [47 women, mean age: 43.7 +/- 13.35 years] with symptomatic rheumatic mitral stenosis who underwent balloon valvuloplasty. Complete transthoracic and transesophageal studies were performed in all the patients before valvuloplasty, and transthoracic study was repeated 24-48 hours after valvuloplasty. The severity of the restriction of the mitral valve leaflet motion was classified based on the heightto- length ratio of the anterior mitral valve leaflet doming. Mitral valve thickness, calcification, subvalvular thickening, and mobility were scored according to the Wilkins system. Optimal immediate outcome of balloon mitral valvuloplasty was defined as a valve area improvement of 50% or more or a final mitral valve area of >/= 1.5 cm[2] and mitral regurgitation Sellers' grade >/= 2. There was a significant relation between the total mitral valve score and its thickness with the optimal immediate post-balloon mitral valvuloplasty results [p value=0.03 and 0.04, respectively], but no relation was found between the Wilkins score and its components with the anterior mitral valve leaflet height-to-length ratio. There was no significant relationship between the amount of increase in the mitral valve area, decrease in trans-mitral pressure gradients, decrease in pulmonary artery pressure, and anterior mitral leaflet height-to-length ratio [all p values>0.05; all the correlation coefficients<0.2]. Our study showed that post-balloon mitral valvuloplasty results are mainly affected by valve thickness and the total Wilkins score. In addition, the severity of mitral leaflet motion restriction in terms of the height-to-length ratio of the anterior mitral valve leaflet has no significant relation with the immediate result of balloon mitral valvuloplasty

[ comparison of the cardiovascular diseases procedures of the classification systems, in selected countries with Iran]

Cardiovascular diseases are important factors at human death and factor destroys human resources. Health intervention is a service for developing of health and diagnoses or modifies of process diseases. It is necessary that every country regarding to its goals, laws national, interests and standards, considers establishing such a system. A comparison of cardiovascular disease classification systems in selected countries with that in Iran could assist health care policy makers in their efforts to battle cardiovascular diseases. This descriptive-comparative study conducted 2007-2008. The cardiovascular diseases of the classification systems in USA, Australia, England and Canada were reviewed in order to prepare an appropriate model for Iran. Data collecting was done through literature review, Internet and email. The cardiovascular disease classification systems of all the selected countries are national. The selected countries, with the exception of England, utilize a multiaxial classification model, especially designed to reflect the individual requirements of every single one of them. This model employs health care standards, e-learning, annual educational programs, and consultation with experts. Iran lacks such a national classification system for cardiovascular diseases. It seems essential that the cardiovascular disease classification system in health interventions in Iran be national if improvements are to be made in the fields of prevention and management of these diseases