Aneurysmal dilation of sinus of Valsalva in a patient with undiagnosed acromegaly.

Key Clinical Message: In patients presenting with aortic ectasia and myxomatous valve diseases at young ages, possible underlying acromegaly should be in mind. Abstract: Acromegaly is a chronic systemic disease mainly caused by the benign pituitary adenoma secreting growth hormone (GH) in excess. Acromegaly is associated with various complications such as cardiovascular diseases. In this regard, timely diagnosis, and management of these patients could be life-saving. Herein, a case of aneurysmal dilation of the sinus of Valsalva with severe aortic and mitral regurgitation in a patient with undiagnosed acromegaly is presented.

Preparation and characterization of tamoxifen loaded silica and NH2 functionalized mesoporous silica nanoparticles as delivery systems against MCF-7 breast cancer cells.

Objectives: Controlled drug delivery using nanotechnology enhances drug targeting at the site of interest and prevents drug dispersal throughout the body. This study focused on loading a poorly water-soluble drug tamoxifen (TMX) into silica nanoparticles (SNPs) and amine-functionalized mesoporous silica nanoparticles (NH2-SBA-15). Materials and Methods: SNPs were prepared according to the Stöber method and functionalized with an amine group using 3-aminopropyl triethoxysilane (APTES) through a one-pot synthesis method to produce amine-functionalized mesoporous silica nanoparticles (NH2-SBA-15). Characterization of both nanoparticles was performed using FT-IR, FE-SEM, CHN analysis, porosity tests (BET), and dynamic light scattering (DLS). Results: The results showed an average particle size of 103.7 nm for SNPs and 225.9 nm for NH2-SBA-15. Based on the BET results, the pore size of NH2-SBA-15 was about 5.4 nm. In both silica nanoparticles, drug release at pH=5.7 was greater than that of pH=7.4. However, Tamoxifen-loaded NH2-SBA-15 (TMX@NH2-SBA-15) indicated the highest drug release in the acidic medium among TMX-loaded SNPs (TMX@SNPs), perhaps due to the high columbic repulsion in the functionalized NH2-SBA-15 nanoparticles. Regarding cytotoxicity results against MCF-7 breast cancer cell lines, both TMX@SNPs and TMX@NH2-SBA-15 nanoparticles exhibited greater cytotoxicity compared to the free TMX as a positive control. Although TMX@SNPs had a small size and high loading capacity, the cytotoxic effects were higher than those of TMX@NH2-SBA-15. Conclusion: Amine functionalization of SNPs can improve the potential activity of these nanoparticles for target therapy.

The Long-Term Response to Treatment with Calcium Channel Blockers in Patients with Idiopathic Pulmonary Arterial Hypertension.

Background: Long-term outcomes in patients with idiopathic pulmonary arterial hypertension (IPAH) treated with calcium channel blockers (CCBs) are not well documented. Therefore, this study aimed to determine the long-term response to treatment with CCBs in patients with IPAH. Methods: This retrospective cohort study was performed on 81 patients with IPAH admitted to our center. Vasoreactivity testing with adenosine was performed in all patients. Twenty-five patients showed a positive response to vasoreactivity testing and were included in the analysis. Results: Of 24 patients, 20 (83.3%) were female, and the mean age of the patients was 45.90±10.42 years. Fifteen patients improved after 1 year on CCB therapy (the long-term CCB responders group), and 9 showed no improvement (the CCB failure group). The CCB responders group had a greater proportion of patients in New York Heart Association (NYHA) functional class I or II (93.3%), a longer distance walked, and less severe hemodynamic parameters. At the 1-year evaluation, the long-term CCB responders had more improvements in the mean 6-minute walk test result (437.43±125.32 vs 268.17±130.06; P=0.040), the mixed venous oxygen saturation level (71.84±9.87 vs 59.03±9.95; P=0.041), and the cardiac index (4.76±1.12 vs 3.15±0.90; P=0.012). Additionally, mPAP was lower in the long-term CCB responders group (47.35±12.70 vs 67.23±14.08; P=0.034). Finally, all the CCB responders were in NYHA functional class I or II (P=0.001). Conclusion: Our study illustrated that long-term treatment with oral CCBs was effective in 60% of acute responders and 18.5% of the entire study population.

Giant cell myocarditis following COVID-19 successfully treated by immunosuppressive therapy.

It has been shown that severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), by coronavirus disease 2019 (COVID-19), can lead to multi-organ impairment including cardiac involvement and immunological problems. Acute myocarditis is one of serious and fatal complications of COVID-19. In this case report, we present a 46-year-old lady with a history of lichen planus dermatitis who has developed a rapidly progressive heart failure after an episode of COVID-19. The pathologic examination of her endomyocardial biopsy specimens was compatible with GCM, and she was successfully treated with a combined immunosuppressive therapy regimen.

Prevalence of Structural Heart Diseases Detected by Handheld Echocardiographic Device in School-Age Children in Iran: The SHED LIGHT Study.

Background: Structural heart disease (SHD) has great impacts on healthcare systems, creating further public health concerns. Proper data are scant regarding the magnitude of the affected population by SHD. Objectives: This study aimed to determine the prevalence of SHD among children and adolescents in an Iranian population. Methods: In this population-based study, a multistage cluster-random sampling was used to choose schools from the Tehran urban area. All students were examined using a handheld Vscan device by echocardiographer, and the results were concurrently supervised and interpreted by cardiologists. All the major findings were reevaluated in hospital clinics. Results: Of 15,130 students (6-18 years, 52.2% boys) who were examined, the prevalence of individuals with congenital heart disease (CHD) and cardiomyopathy was 152 (10.046 per 1,000 persons) and 9 (0.595 per 1,000 persons), respectively. The prevalence of definite and borderline rheumatic heart disease (RHD) was 30 (2 per 1,000 persons) and 113 (7.5 per 1,000 persons), correspondingly. Non-rheumatic valvular heart disease (VHD) was also detected in 465 (30.7 per 1,000 persons) students. Of all the pathologies, only 39 (25.6%) cases with CHD and 1 (0.007%) cases with RHD had already been diagnosed. Parental consanguinity was the strongest predictor of CHD and SHD (odds ratio [OR]: 1.907, 95% CI, 1.358 to 2.680; P < 0.001 and OR, 1.855, 95% CI, 1.334 to 2.579; P < 0.001, respectively). The female sex (OR, 1.262, 95% CI, 1.013 to 1.573; P = 0.038) and fathers' low literacy (OR, 1.872, 95% CI, 1.068 to 3.281; P = 0.029) were the strongest predictors of non-rheumatic VHD and RHD, correspondingly. Conclusions: The implementation of echocardiographic examinations for detecting SHD among young population is feasible which detected SHD prevalence in our population comparable to previous reports. Further studies are required to delineate its economic aspects for community-based screening.

Targeted treatment in viral-associated inflammatory cardiomyopathy.

Detection of viruses like HHV-6 in endomyocardial biopsy or serum serology of patients with myocarditis or heart failure features unresponsive to conventional heart failure therapies could be a potential targeted treatment especially in refractory cases.

Saw tooth cardiomyopathy: a case report.

Saw tooth cardiomyopathy is an unusual and rare type of left ventricular dysplasia that is characterized by multiple projections of compacted myocardium that makes the appearance of 'saw tooth' in noninvasive imaging. We present a young man with signs and symptoms of heart failure and reduced left ventricular function in echocardiography who showed distinctive left ventricle features of saw tooth cardiomyopathy (saw tooth appearance of myocardium in basal inferolateral and basal to mid lateral segments) in cardiac magnetic resonance imaging.

Muscle wasting in young patients with dilated cardiomyopathy.

BACKGROUND: Muscle wasting can be accelerated by chronic diseases such as heart failure and is one of the major causes of disability, morbidity, and mortality in this population. We aimed to investigate the incidence of muscle wasting and its associated factors in dilated cardiomyopathy patients younger than 55 years of age. METHODS: Between April 2014 and December 2015, all symptomatic patients with a diagnosis of non-ischaemic dilated cardiomyopathy who were referred to heart failure clinic were included in our study. Dual energy X-ray absorptiometry was used to evaluate body composition and identify muscle wasting. Muscle mass was calculated as the ratio of an individual's total lean mass of legs and arms (also called appendicular skeletal muscle) to their squared height (kg/m2 ). The muscle mass values of less than 5.45 kg/m2 for women and 7.26 kg/m2 for men were considered low. RESULTS: A total of 55 patients (32 male) were included. The mean (standard deviation) of age was 37.3 (10.1) years, and the mean of left ventricular ejection fraction was 21.4%. Most of the patients were in the New York Heart Association classes of II and II-III. Twenty-six patients (47.3%) met criteria for muscle wasting. Patients with muscle wasting had lower left ventricular ejection fraction, lower 6-min walk distance, and higher New York Heart Association function class and hospitalization rate. CONCLUSIONS: We concluded that muscle wasting might be present in younger patients with heart failure, particularly in those who are in worse clinical condition.

Pulmonary Vascular Capacitance as a Predictor of Vasoreactivity in Idiopathic Pulmonary Arterial Hypertension Tested by Adenosine.

BACKGROUND: Acute pulmonary vasoreactivity testing has been recommended in the diagnostic work-up of patients with idiopathic pulmonary arterial hypertension (IPAH). Pulmonary arteriolar capacitance (Cp) approximated by stroke volume divided by pulmonary pulse pressure (SV/PP) is considered as an independent predictor of mortality in patients with IPAH. OBJECTIVES: We sought to evaluate any differences in baseline and adenosine Cp between vasoreactive and non-vasoreactive IPAH patients tested with adenosine. PATIENTS AND METHODS: Fourteen patients with IPAH and a vasoreactive adenosine vasoreactivity testing according to the ESC guidelines were compared with 24 IPAH patients with nonreactive adenosine test results. RESULTS: There were no statistical significant differences between the two groups regarding NYHA class, body surface area, heart rate, and systemic blood pressure during right heart catheterization. Hemodynamic study showed no statistical significant differences in cardiac output/Index, mean pulmonary artery pressure, pulmonary vascular resistance, and baseline Cp between the two groups. There was a statistical significant but weak increase in adenosine Cp in vasoreactive group compared to non-reactive group (P = 0.04). Multivariable analysis showed an association between Cp and vasoreactivity (Beta = 2, P = 0.04, OR = 0.05 (95%CI = 0.003 - 0.9). CONCLUSIONS: Cp could be considered as an index for the prediction of vasoreactivity in patients with IPAH. Prediction of long-term response to calcium channel blockers in patients with IPAH and a positive vasoreactive test by this index should be addressed in further studies.

Diagnostic Pitfalls and Challenges in Interpretation of Heart Transplantation Rejection in Endomyocardial Biopsies With Focus on our Experience.

BACKGROUND: The current trend of heart transplantation in recent years has taken a quantum leap forward. We decided to look back at our experience in this center. OBJECTIVES: Here, we focus on the diagnostic pitfalls and challenges in these biopsies. PATIENTS AND METHODS: Forty two patients based on the standard protocol of heart transplantation group, yielded 63 biopsy samples over a period of 33 months (April 2010 - December 2012). The mean age was 30.4 years (ranging from 16 to 58 years) with 51 males (81%) and 12 females (19%). All the patients were examined periodically and biopsy samples were taken from the right ventricular wall. RESULTS: Rarely fewer than three pieces of myocardial samples were procured. Scar, adipose tissues and blood clots may be seen instead. Quilty effect (nodular endocardial lesions composed of inflammatory cell infiltrates) was seen in 8 cases (12.7%). Other findings not directly related to rejection including early ischemic injury, Quilty effect and post-transplant lymphoproliferative disorders (PTLD) were not encountered. CONCLUSIONS: Specimen inadequacy was not a major problem in our center. It poses a great limitation, because suboptimal specimens sometimes mislead the pathologist. Other findings especially Quilty effect were within the range defined for this finding.