Digital thermography and vascular involvement in ß-thalassemia intermedia.

Beta-thalassemia intermedia (ß-TI) is associated with vascular dysfunction. We used digital thermal monitoring (DTM), a non-invasive tool that evaluates vascular function based on changes in fingertip temperature during and after cuff occlusion on ß-TI patients. Thirty-three patients (18 years and older) were recruited in this study and divided into 3 groups: thalassemia, anemic controls, and healthy controls. Exclusion criteria included factors that are known to be associated with vascular damage. Patients underwent DTM and results were extracted as vascular reactivity index (VRI), a measure of how well the circulatory system responds to stimuli that require adjustments of blood flow. One-way analysis of variance (ANOVA) was used to test the mean difference in VRI between the 3 groups. A multiple linear regression was also carried out with VRI as the outcome of interest and a function of covariates that were thought to be of clinical relevance to VRI. The frequency, mean VRI ± standard error (SE) for the thalassemic group were (N = 16), mean = 2.243 ± 0.111; for anemic controls (N = 9), mean = 2.374 ± 0.162; and for the controls (N = 8), mean = 2.338 ± 0.092. ANOVA test indicated a non-significant difference in mean VRI between the three groups (P value = 0.731). Multiple linear regression couldn't detect any significant association between VRI and any of the predictors including the groups. Our study did not show a significant difference in VRI between the 3 study groups. Prospective studies of larger sample size are warranted to establish DTM as a possible non-invasive tool used to evaluate vascular function in ß-TI patients.

Central and Peripheral Hemodynamics in Young Adults Who Use Water Pipes and the Acute Effects of Water-Pipe Use.

BACKGROUND: Tobacco use via water pipe (commonly referred to as water-pipe smoking [WPS]) is popular among young adults globally and exposes those who smoke to toxicants. RESEARCH QUESTION: Is WPS associated with impaired measures of arterial function and does WPS acutely impair these measures in young adults? STUDY DESIGN AND METHODS: We assessed heart rate (HR), brachial and aortic BP, HR-adjusted augmentation index (AI), and carotid-femoral pulse wave velocity (CFPWV) in 62 individuals who use water pipes and 34 individuals who have never used a water pipe recruited from the community (mean age, 22.5 ± 3.0 years; 48% female). Measurements were obtained before and after an outdoor session of WPS among participants who use water pipes and among the control group of participants who have never used a water pipe. Measurements were compared after vs before exposure and between those who use and those who do not use water pipes, adjusting for possible confounders using linear regression. RESULTS: Participants who use water pipes and control participants had similar demographic characteristics. BP and HR increased acutely after WPS (brachial systolic BP by 4.13 mm Hg [95% CI, 1.91-6.36 mm Hg]; aortic systolic BP by 2.31 mm Hg [95% CI, 0.28-4.33 mm Hg]; brachial diastolic BP by 3.69 mm Hg [95% CI, 1.62-5.77 mm Hg]; aortic diastolic BP by 3.03 mm Hg [95% CI, 0.74-5.33 mm Hg]; and HR by 7.75 beats/min [95% CI, 5.46-10.04 beats/min]), but not in the control group. AI was significantly higher in participants who use water pipes compared with those who do not (9.02% vs 3.06%; P = .03), including after adjusting for BMI and family history of cardiovascular disease (ß = 6.12; 95% CI, 0.55-11.69; P = .03) and when assessing habitual tobacco use via water-pipe extent (water pipes used/day × water-pipe use duration) in water-pipe-years (ß = 2.51/water-pipe-year; 95% CI, 0.10-4.92/water-pipe-year; P = .04). However, CFPWV was similar in those who use water pipes and those who do not, and AI and CFPWV did not change acutely after WPS. INTERPRETATION: In apparently healthy young individuals from the community, habitual WPS was associated with increased AI, a predictor of cardiovascular risk, and one WPS session acutely increased HR and brachial and aortic BP.

The role of selective imidazoline receptor agonists in modern hypertension management: an international real-world survey (STRAIGHT).

BACKGROUND: Multiple pharmacologic strategies are currently available to lower blood pressure (BP). Renin-angiotensin system (RAS)-inhibitors, calcium channel blockers and diuretics are widely recommended as first line therapies. Sympathetic activation is an important contributor to BP elevation but remains unopposed or is even increased by some of these drug classes. Selective imidazoline receptor agonists (SIRAs) reduce increased central sympathetic outflow and are considered as add-on therapy in most guidelines. We conducted an international survey to evaluate contemporary hypertension management strategies in countries with high prescription rates of SIRAs to better understand the rationale and practical indications for their use in a real-world setting. METHODS: Physicians from seven countries (India, Jordan, Lebanon, Russia, Saudi Arabia, South Africa, United Arab Emirates) were asked to complete a web-based questionnaire and comment on clinical case scenarios to provide information on their current practice regarding antihypertension strategies, underlying rationale for their choices, and adherence to relevant guidelines. RESULTS: 281 physicians completed the questionnaire including mainly cardiologists (35%) and general practitioners (32%). 96% reported using European (60%) or local (56%) guidelines in their daily practices. The majority of responding physicians (83%) had knowledge of SIRAs and 70% prescribed SIRAs regularly typically as a third line antihypertensive strategy (63%). The preferred combination partners for SIRAs were RAS-inhibitors (72%). CONCLUSIONS: Contemporary hypertension management varies between countries and therapeutic approaches in a real-world setting are not always in line with recommendations from available guidelines. In the countries selected for this survey prescription of SIRAs was common and appeared to be guided predominantly by considerations relating to the underlying pathophysiologic mechanism of sympathetic inhibition.

Diabetes without Manifest Cardiovascular Disease: A Novel Approach in Risk Stratification and Treatment Selection.

BACKGROUND: Cardiovascular disease (CVD), the main macro vascular complication of type 2 diabetes (T2D), increases the risk of death significantly in patients with T2D. INTRODUCTION: Most of the patients with T2D do not have obvious CVD symptoms. Due to the paucity of data, CVD screening in asymptomatic patients with T2D remains highly controversial. METHODS: This has driven a panel of experts to establish a novel consensus on how to approach patients with T2D at high CVD risk. The panel formulated a stepwise algorithm by which patients with T2D undergo initial risk stratification into low, intermediate and high risk using the ASCVD calculator. In patients with intermediate risk, coronary artery calcium measurement is used to further stratify those patients into new low and high-risk categories. RESULTS AND CONCLUSION: The panel recommends using standard diabetes care in low risk patients and using SGLT2 inhibitors and GLP1 agonists with cardio protective effect, on top of standard care, in high risk individuals.

Founder Mutation in N Terminus of Cardiac Troponin I Causes Malignant Hypertrophic Cardiomyopathy.

BACKGROUND: Cardiac troponin I (TNNI3) gene mutations account for 3% of hypertrophic cardiomyopathy and carriers have a heterogeneous phenotype, with increased risk of sudden cardiac death (SCD). Only one mutation (p.Arg21Cys) has been reported in the N terminus of the protein. In model organisms, it impairs PKA (protein kinase A) phosphorylation, increases calcium sensitivity, and causes diastolic dysfunction. The phenotype of this unique mutation in patients with hypertrophic cardiomyopathy remains unknown. METHODS: We sequenced 29 families with hypertrophic cardiomyopathy enriched for pediatric-onset disease and identified 5 families with the TNNI3 p.Arg21Cys mutation. Using cascade screening, we studied the clinical phenotype of 57 individuals from the 5 families with TNNI3 p.Arg21Cys-related cardiomyopathy. We performed survival analysis investigating the age at first SCD in carriers of the mutation. RESULTS: All 5 families with TNNI3 p.Arg21Cys were from South Lebanon. TNNI3 p.Arg21Cys-related cardiomyopathy manifested a malignant phenotype-SCD occurred in 30 (53%) of 57 affected individuals at a median age of 22.5 years. In select carriers without left ventricular hypertrophy on echocardiogram, SCD occurred, myocyte disarray was found on autopsy heart, and tissue Doppler and cardiac magnetic resonance imaging identified subclinical disease features such as diastolic dysfunction and late gadolinium enhancement. CONCLUSIONS: The TNNI3 p.Arg21Cys mutation has a founder effect in South Lebanon and causes malignant hypertrophic cardiomyopathy with early SCD even in the absence of hypertrophy. Genetic diagnosis with this mutation may be sufficient for risk stratification for SCD.

Variations in the referral patterns to stress nuclear imaging and stress echocardiography scans.

BACKGROUND: Stress myocardial perfusion imaging (MPI) and stress echocardiography (Echo) are commonly used for the noninvasive evaluation of patients with suspected coronary artery disease (CAD). Very few studies have compared the referral patterns to these imaging modalities in terms of the clinical profile of patients, reasons for referral, and type of referring physicians. METHODS AND RESULTS: This was a prospective study of 1,020 consecutive patients who were referred for stress MPI (429 patients) or stress Echo (591 patients) at the American University of Beirut Medical Center in the year of 2008. Patients referred to MPI were older and had a higher prevalence of diabetes, hypertension, hypercholesterolemia, smoking, and previous myocardial infarction, coronary angioplasty, or bypass surgery. There were more abnormal scans in the stress MPI group (24% vs 15%, P < 0.001), as well as a higher prevalence of ischemia (15% vs 7.6%, P < 0.001) and impaired left ventricular function with an ejection fraction <50% (11% vs 1.7%, P < 0.001). A higher percentage of stress Echo studies were self-referred by physicians who themselves interpret the scans (31% vs 19%, P < 0.001). CONCLUSION: Patients referred for stress MPI are at a higher risk than those referred for stress Echo having more CAD risk factors, more prior history of coronary events, and an older age. These findings have important implications in the interpretation of studies that compare the diagnostic and prognostic power of these two imaging modalities.

International variations in the ischemic burden post-acute myocardial infarction: prognostic implications.

OBJECTIVES: To determine if there is a variation in the ischemic burden post-acute myocardial infarction (AMI), as assessed by myocardial perfusion imaging (MPI), between different populations in different geographic locations and to see if this variation is associated with different clinical outcomes. METHODS AND RESULTS: We characterized the MPI findings in 104 stable patients who were hospitalized with AMI at the American University of Beirut Medical Center (AUBMC), a tertiary referral hospital in an East Mediterranean country and we compared them to 126 patients who were enrolled according to a similar protocol in a previous study done at Baylor College of Medicine (BCM), Houston, Texas. There were no differences between the two populations with respect to prevalence of diabetes, hypertension, smoking, the use of thrombolysis, percentage of anterior MIs, Q-wave MIs, and multivessel disease on coronary angiography. However, the quantified ischemic defect size in the BCM population was double that in the AUBMC population (12 +/- 12% vs 6 +/- 8%, P < .01). This was associated with almost doubling of the 1 year event rate of death/myocardial infarction (18.3% vs 10.6%, P = .02) in the BCM population. CONCLUSION: Our study suggests that the ischemic burden post-AMI, as assessed by MPI, might vary between different populations in different geographic locations. This variation carries important prognostic implications and is associated with different patient outcomes.

Non-familial cardiomyopathies in Lebanon: exome sequencing results for five idiopathic cases.

BACKGROUND: Cardiomyopathies affect more than 0.5% of the general population. They are associated with high risk of sudden cardiac death, which can result from either heart failure or electrical abnormalities. Although different mechanisms underlie the various types of cardiomyopathies, a principal pathology is common to all and is usually at the level of the cardiac muscle. With a relatively high incidence rate in most countries, and a subsequent major health burden on both the families and governments, cardiomyopathies are gaining more attention by researchers and pharmaceutical companies as well as health government bodies. In Lebanon, there is no official data about the spectrum of the diseases in terms of their respective prevalence, clinical, or genetic profiles. METHODS: We used exome sequencing to unravel the genetic basis of idiopathic cases of cardiomyopathies in Lebanon, a relatively small country with high rates of consanguineous marriages. RESULTS: Five cases were diagnosed with different forms of cardiomyopathies, and exome sequencing revealed the presence of already documented or novel mutations in known genes in three cases: LMNA for an Emery Dreifuss Muscular Dystrophy case, PKP2 for an arrhythmogenic right ventricle dysplasia case, and MYPN for a dilated cardiomyopathy case. Interestingly two brothers with hypertrophic cardiomyopathy have a novel missense variation in NPR1, the gene encoding the natriuretic peptides receptor type I, not reported previously to be causing cardiomyopathies. CONCLUSION: Our results unravel novel mutations in known genes implicated in cardiomyopathies in Lebanon. Changes in clinical management however, require genetic profiling of a larger cohort of patients.

[Epidemiology of cardiac tumors in adults in Lebanon]. / Epidémiologie des tumeurs cardiaques des adultes au Liban.

Primary cardiac tumors are rare but potentially fatal. No studies have discussed this issue yet on the national level. We describe the epidemiology of cardiac tumors in adults in Lebanon. The data were taken from the hospitals, the patients and/or their doctors by means of a questionnaire. Fifty-seven cases of cardiac tumors were found, mainly myxomas in the left atrium. Dyspnea was the most frequent symptom. Transthoracic echocardiography was the main diagnostic tool. Transesophageal echocardiography was inconstantly made, scanner and MRI rarely while contrast echocardiography has never been used. Almost half of the patients had a follow-up and none had a familial screening. The collected data urged us to propose a unique and homogenous strategy for diagnosis, treatment and follow-up of cardiac tumors in Lebanon.

The management of acute myocardial infarction in developing countries.

Developing countries contribute a major share to the global burden of cardiovascular disease. Acute myocardial infarction (AMI) in particular remains one of the leading causes of death in the developing world as well as in the developed world. While the risk factors, management and outcome of AMI have been extensively studied in the developed world, limited data is available on this subject from developing countries. The current review looks at the prevalence of the classical coronary artery disease risk factors in developing countries and their association with myocardial infarction, as well as the management and outcome of AMI patients in these countries.

Transesophageal echocardiography improves risk assessment of thrombolysis of prosthetic valve thrombosis: results of the international PRO-TEE registry.

OBJECTIVES: The goal of this study was to evaluate whether quantitation of thrombus burden with transesophageal echocardiography (TEE) can help risk-stratify patients undergoing thrombolysis of prosthetic valve thrombosis (PVT). BACKGROUND: Thrombolytic therapy of PVT has an unpredictable risk of embolization and complications. METHODS: An international registry of patients with suspected PVT undergoing two-dimensional/Doppler and TEE before thrombolysis was established. All TEE studies were reviewed and quantitated by a single observer blinded to all data. RESULTS: From 1985 to 2001, 107 patients (71 females; age 24 to 86 years) from 14 centers (6 in the U.S.) were identified. The majority of cases involved the mitral valve (79 mitral, 13 aortic, and 15 tricuspid). Hemodynamic success rate was achieved in 85% and was similar across valves. Overall complications were observed in 17.8%, and death in 5.6%. Predictors of complications were: New York Heart Association (NYHA) functional class, presence of shock, sinus tachycardia, hypotension, previous history of stroke, thrombus extension beyond the valve ring, and thrombus area. Multivariate analysis demonstrated that two variables were independent predictors of complications: thrombus area by TEE (odds ratio [OR] 2.41 per 1 cm2 increment, 95% confidence interval [CI] 1.12 to 5.19) and prior history of stroke (OR 4.55, 95% CI 1.35 to 15.38). A thrombus area <0.8 cm2 identified patients at lower risk for complications from thrombolysis, irrespective of NYHA functional class. CONCLUSIONS: In PVT, the thrombus size imaged with TEE is a significant independent predictor of outcome. Transesophageal echocardiography can identify low-risk groups for thrombolysis irrespective of symptom severity and is therefore recommended in the management of prosthetic valve thrombosis.

Efficacy and safety of lercanidipine/enalapril fixed combination in Lebanon: a prospective observational study.

OBJECTIVE: The DUAL study evaluated the effectiveness and safety of the fixed-dose combination of lercanidipine and enalapril in a real-practice scenario; the effects of this combination on a number of markers of cardiovascular risk have been also investigated. RESEARCH DESIGN AND METHODS: This was a 2 month, phase IV, open-label, single-group, prospective observational study. Adult patients with untreated or uncontrolled hypertension (blood pressure [BP] >140/90 mmHg) were eligible for this study. All patients received lercanidipine/enalapril, in a once-daily fixed combination (10 mg/10 mg). MAIN OUTCOME MEASURES: The patients were evaluated at baseline, at 1 month and at 2 months. The following parameters were evaluated at all time points: systolic BP (SBP) and diastolic BP (DBP); heart rate (HR). A number of laboratory parameters were measured at baseline and at 2 months. Safety considerations were performed. RESULTS: In total, 188 patients were enrolled (104 males; mean age 58 ± 12 years). At baseline, mean SBP was 159 ± 10 mmHg and mean DBP was 94 ± 7 mmHg. Treatment with lercanidipine/enalapril in fixed combination was associated with a reduction in both SBP and DBP already at 1 month; this reduction was sustained until month 2 (SBP: 131 ± 7 mmHg; DBP: 79 ± 5 mmHg; p < 0.05 vs baseline). At baseline HR was 78 ± 10 bpm; a significant reduction in this parameter was observed at month 2 (75 ± 7 bpm; p < 0.05 vs baseline). A significant decrease in total cholesterol, low-density lipoprotein cholesterol, triglycerides, and fasting glucose, and a significant increase in K(+), and Ca(2+) was observed at month 2 compared with baseline values. In total, two patients (1%) experienced dry cough. No other adverse effects were reported. CONCLUSIONS: Even with all the limitations of any observational study, these data show that a 2 month treatment with a fixed dose of lercanidipine/enalapril is associated with significant reductions in SBP and DBP, HR, and improvement in a number of laboratory parameters.

Sodium intake reduction efforts in Lebanon.

Sodium intake reduction efforts in Lebanon are quite recent and have just started to take effect on the national level. Starting out from an academic institution, the Lebanese Action on Sodium and Health (LASH) campaign was established to counter the increasing prevalence of hypertension and associated adverse health effects. The campaign's strategy was based on four pillars: research, health communication, advocacy, and monitoring. The LASH group set out with determining: baseline sodium intake of the population, main sources of sodium intake, and the knowledge, attitudes, and behaviors (KAB) of the population as a situation analysis that prompts for action. This gave LASH tangible evidence of the magnitude of the problem and the need for the government, the food industry, and the consumers, to be mobilized to take part in devising a solution. Currently, Lebanon is at a stage of technically working to reduce the sodium content in the major sources of sodium, namely local bread and bread-like products. The next steps will include implementation of a plan for monitoring industry compliance, while studying other food targets, including dairy products and processed meat. Meanwhile, the health communication plan is ongoing and the Salt Awareness Week is celebrated every year with media appearances of LASH researchers to raise the issue to the public eye.

Patterns of congenital heart disease in unoperated adults: a 20-year experience in a developing country.

BACKGROUND: The number of patients with congenital cardiac disease reaching adulthood is increasing steadily. Many adults with such disease face both medical and surgical difficulties. HYPOTHESIS: This retrospective study was undertaken to assess the frequency and outcome of congenital heart disease (CHD) in unoperated adults. METHODS: The charts of all patients with unoperated CHD, who were admitted to a tertiary care center in Beirut, Lebanon, between 1980 and 2000 were reviewed. Of these, 206 patients (52% men, age at admittance 18-71 years [32.8 +/- 13.3 years]) with a diagnosis of CHD were evaluated. Atrial septal defect (ASD) was the most common cardiac malformation with a relative frequency of 53%, followed by ventricular septal defects (11%), tetralogy of Fallot (11%), aortic anomalies (7%), pulmonary stenosis (6%), and Ebstein anomaly (4%). Most patients were symptomatic upon presentation, with dyspnea on exertion being the most common presenting symptom. Twenty-seven patients (13%) had cyanotic CHD. Of 179 acyanotic patients, 113 (63%), and 17 of 27 cyanotic patients (63%) underwent surgical intervention. In-hospital surgical complications for the acyanotic group included cerebrovascular accident (2%) and heart block (1%). Total surgical mortality was 4 of 130 (3%). One patient with tetralogy of Fallot presented with endocarditis and died. CONCLUSION: Atrial septal defect is the most common defect reported in our experience; however, it occurs more frequently than that reported in the literature. Although most patients were symptomatic on presentation, their functional status was stable. Accordingly, their hospital course, whether managed medically or surgically, held a relatively low complication rate. This could be attributed to the uncomplicated nature of pathologies in our series. The surgical mortality and in-hospital complications were slighter higher than those reported for similar lesions if repaired during childhood. This study reflects the relative frequency of various cardiac malformations in selected patients with "grown up" congenital heart disease (GUCH) and their natural survival pattern.

Bilateral percutaneous stent implantation in the pulmonary vasculature: a modality now available in Lebanon.

We report a child with Truncus Arteriosus who developed bilateral proximal branch pulmonary stenosis, following total surgical repair of her condition with construction of the right ventricular outflow tract utilizing a Contegra conduit (Medtronic Inc., Minneapolis, Minn.). The obstruction was relieved completely utilizing bilateral percutaneous stent implantation. To the best of our knowledge, this is the first reported case of bilateral stent implantation to relieve branch pulmonary artery stenosis in Lebanon. This technique could be applied to similar cases of obstruction in the pulmonary tree.

Antithrombotic prophylaxis in the middle East.

Several factors have been proposed to explain the persistence of a high incidence of venous thromboembolism worldwide with its associated morbidity and mortality. Underutilization of anticoagulants and failure of adherence to thromboprophylaxis guidelines are emerging global health concerns. We herein review this alarming observation with special emphasis on the Middle East region. We also discuss strategies that could help control this increasingly reported problem.

The interaction of gender and clinical risk profile in patients referred for exercise nuclear imaging versus exercise echocardiography.

BACKGROUND: Exercise myocardial perfusion imaging (E-MPI) and exercise echocardiography (E-Echo) are thought to be compatible for the non-invasive evaluation of patients with suspected coronary artery disease (CAD). The interaction of gender and clinical risk profile in the referral of patients to either of these two imaging modalities has not been well studied. METHODS AND RESULTS: This was a prospective study of 922 consecutive patients who were referred for either E-MPI (331 patients) or E-Echo (591 patients) at the American University of Beirut Medical Center in the year of 2008. Men undergoing E-MPI had a higher risk profile than those undergoing E-Echo. They were older (58±12 years versus 55±12 years, p=0.002) and had a higher prevalence of multiple (>2) CAD risk factors (55% versus 37%, p<0.001) as well as a higher prevalence of prior PCI (22% versus 15%, p=0.017) or CABG (12% versus 7%, p=0.016). Furthermore, they achieved lower METS in their exercise (9.4±2.0 versus 10.4±2.3, p<0.001) and had a higher incidence of ischemia and on their scans (16% versus 9.1%, p=0.008). In contrast, women undergoing E-MPI had a similar profile to those undergoing E-Echo in terms of prevalence of risk factors, prior history of coronary events and the prevalence of ischemia or impaired ejection fraction on their scans. CONCLUSION: There is an important interaction between gender and clinical risk profile in patients undergoing E-MPI versus E-Echo. In men, E-MPI seems to be the preferred test for the higher risk profile patients, whereas in women the two tests are interchangeable.

Prosthetic valve thrombosis with adequate anticoagulation.

We report the case of a 41-year-old woman who developed thrombosis of a CarboMedics prosthetic valve in the mitral position despite adequate anticoagulation. This report provides further supportive data to recently published studies suggesting an increased thrombogenicity of the CarboMedics prosthetic valve in the mitral position and it underscores the importance of conducting large prospective, randomized trials for further evaluation of the thromboembolic complications of this valve.

Contrast-induced encephalopathy following coronary angioplasty with iohexol.

We report a case of a patient who developed acute encephalopathy following coronary angioplasty with iohexol contrast. The patient's clinical condition was associated with slowing on the EEG. Studies did not reveal any other etiology or contributing cause for the encephalopathy. The patient recovered spontaneously in 24 hours with only supportive measures. This report suggests that such a drug reaction could occur with iohexol injected in the coronaries, but reassures that the encephalopathy is self limiting.