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Introduction: The risk factors for aortic stenosis have been shown to be similar to those for atherosclerosis. Thus, coronary disease is often found simultaneously in patients with aortic stenosis. Our work aims to determine the frequency of coronary disease in a Moroccan population with aortic stenosis while recalling the causes and the prognostic and therapeutic impacts of this association. Materials and Methods: This is a retrospective study of 148 patients hospitalized at the cardiology center of the military hospital of Rabat over a period of 24 months, during which we analyzed clinical, electrocardiographic, echocardiographic and coronarographic data of the patients in order to evaluate the coronary involvement during aortic stenosis. Results: The mean age of the population was 65 [57, 74] years, the sex ratio was 1.21. Smoking reported in 38.5% of patients was the main modifiable cardiovascular risk factor, followed by hypertension in 35.8% of patients. Dyspnea on exertion was the most frequent reason for consultation at 81%, 64% of which were at least NYHA functional class III, followed by angina, which represented 33% of the series. The aortic stenosis was tight in the majority (mean SAo: 0.8 cm²) and the left ventricular ejection fraction was preserved overall. Coronary artery disease was associated with aortic stenosis in 24% of cases, with predominantly monotruncal involvement (53%) followed by tritruncal involvement (30%). 21.6% of these patients underwent coronary artery bypass grafting concomitantly with surgical replacement of the aortic valve. Conclusion: The incidence of coronary artery disease associated with aortic stenosis is variable according to age. It is higher in European series because of aging. In our relatively younger population, it is lower but not negligible.

Multiple congenital heart disease in the small preterm newborn such as severe narrowing of aortic valve and patent ductus arteriosus (PDA) is a therapeutic challenge. We report successful transcatheter antegrade balloon dilatation of the aortic valve and device closure of the PDA in a 1700-gram preterm newborn. Meticulous planning and team work aids in such transcatheter intervention. Learning objective Preterm newborn babies rarely have multiple major structural cardiac lesions complicating the care. Transcatheter intervention for multiple congenital cardiac lesions such as severe valvular aortic stenosis and large patent ductus arteriosus in a small preterm baby is challenging but feasible. Precise anatomical diagnosis and meticulous planning is essential for successful intervention with minimum complications.

BACKGROUND The aim of our study was to characterize echocardiographic changes during pregnancy in women with known LVOT obstruction or AS compared to the healthy pregnancy controls, and to assess the relationship with pregnancy outcomes. METHODS We retrospectively studied 34 pregnant patients with congenital LVOT obstruction or AS with healthy age-matched pregnant controls. Patients with other significant valvular lesions, structural heart disease (LVEF

Graphical abstract, Highlights • Adults with SM have slower hemodynamic progression and more rapid AS progression. • Changes in LVOT velocities are minimal over time. • Presence of SM with AS poses challenges in determining hemodynamic significance. • In general, surgery should be recommended when AS is severe and symptoms develop.

Familial homozygous hypercholesterolemia is a rare disease with diverse clinical presentations ranging from premature ischemic heart disease to aortic root stenosis but rarely presents with anginal symptoms due to supravalvular and valvular aortic stenosis. We report a 19-year-old male patient with familial homozygous hypercholesterolemia with progressive supravalvular and valvular aortic stenosis that ultimately required aortic root enlargement and aortic valve replacement using a mechanical prosthesis, despite aggressive medical therapy. Surgical importance of this rare condition is highlighted.

Purpose of review Degenerative aortic stenosis (AS) is one of the most prevalent heart valve diseases in the adult population. The understanding of AS pathophysiology and involved risk factors have recently undergone a great advance, with low-density lipoprotein cholesterol (LDL-C), lipoprotein (a) [Lp(a)] and other clinical conditions taking on a relevant role. Although little is known about the prevention of AS, we can progressively find more evidence of the possible use of drugs to control risk factors as tools that may delay the progression to severe AS and aortic valve replacement. Recent findings Several factors have shown to be solid predictors of the development of AS. Mendelian randomization and observational studies on risk factors specifically lipid factors, such as hypercholesterolemia, Lp(a), proprotein convertase subtilisin/kexin type 9 and hypertension have provided meaningful new information. The SAFEHEART study has significantly contributed to define the role of LDL-C and Lp(a) in AS. In this review we discuss the interrelationship of dyslipidemia, especially hypercholesterolemia and Lp(a) in the development and prognosis of valvular AS. New imaging tools may contribute to its early detection. Future studies with proprotein convertase subtilisin/kexin type 9 inhibitors and specific therapies to lower Lp(a) might contribute to delay AS development.

Introduction: Valvular aortic stenosis (AS) is common with high morbidity and mortality in the absence of surgical intervention, but no current medical therapies are known to prevent or slow disease progression. Previous genetic studies have identified several genetic loci associated with prevalent AS, including LPA and PALMD , although most evidence is limited to populations of European ancestry. Methods: We performed a trans-ethnic genome-wide association study (GWAS) of prevalent AS in the Veterans Administration Million Veteran Program (MVP). Cases were identified by a combination of diagnostic billing and surgical codes and validated by association to the known LPA variant (rs10455872). GWAS was run separately for White, Black, and Hispanic individuals, controlling for age, sex, and six principal components, and combined using fixed effects meta-analysis. Results were limited to variants with a minor allele frequency greater than 1% in the trans-ancestry analysis. Lead independent genome wide significant loci were annotated by nearest gene. Results: 300,182 White, 80,744 Black, and 32,069 Hispanic participants were available for analysis. Of these, there were 12,385 (4.1%) White, 1,444 (1.8%) Black, and 611 (1.9%) Hispanic AS cases. Trans-ethnic analyses identified 10 independent genome wide significant (GWS, p≤5x10 -8 ) loci, replicating 6 known AS genetic loci ( ALPL, PALMD, TEX41, LPA, IL6, FADS1 ), and identifying 4 novel genetic loci ( CEP85L, CELSR2, NCK1, SLMAP ), of which 2 were present at nominal significance in Hispanic ( CELS2R ) or Black ( SLMAP ) individuals. Ethnicity-specific analyses additionally identified 9 novel GWS loci in White individuals, and 3 novel GWS loci in Hispanic individuals. Newly identified loci supported known biological pathways in AS including lipid/metabolic, inflammatory, and calcification, but also implicated new pathways such as those pertaining to QT interval ( SLC35F1 ) and the Brugada Syndrome ( SLMAP ). Conclusions: In this large trans-ethnic GWAS for AS we replicate previously identified genetic loci for AS, and identified several novel loci both in trans-ethnic and in ethnic-specific analyses. These loci implicate known and novel biological mechanisms for future prevention and treatment of AS.

Previous research in children and adolescents with congenital heart defects presents contradictory findings concerning their physical activity (PA) level, due to methodological limitations in the PA assessment. The aim of the present cross-sectional study was to compare PA in children and adolescents treated for valvular aortic stenosis with healthy controls using an improved accelerometer method. Seven-day accelerometer data were collected from the hip in a national Swedish sample of 46 patients 6–18 years old treated for valvular aortic stenosis and 44 healthy controls matched for age, gender, geography, and measurement period. Sports participation was self-reported. Accelerometer data were processed with the new improved Frequency Extended Method and with the traditional ActiGraph method for comparison. A high-resolution PA intensity spectrum was investigated as well as traditional crude PA intensity categories. Children treated for aortic stenosis had a pattern of less PA in the highest intensity spectra and had more sedentary time, while the adolescent patients tended to be less physically active in higher intensities overall and with less sedentary time, compared to the controls. These patterns were evident using the Frequency Extended Method with the detailed PA intensity spectrum, but not to the same degree using the ActiGraph method and traditional crude PA intensity categories. Patients reported less sports participation than their controls in both age-groups. Specific differences in PA patterns were revealed using the Frequency Extended Method with the high-resolution PA intensity spectrum in Swedish children and adolescents treated for valvular aortic stenosis.

We tested the hypothesis that higher levels of plasma triglycerides and remnant cholesterol are observationally and genetically associated with increased risk of aortic valve stenosis.We included 108 559 individuals from the Copenhagen General Population Study. Plasma triglycerides, remnant cholesterol (total cholesterol minus low-density lipoprotein and high-density lipoprotein cholesterol), and 16 genetic variants causing such increased or decreased levels were determined. Incident aortic valve stenosis occurred in 1593 individuals. Observationally compared to individuals with triglycerides1 mmol/L (89 mg/dL), the multifactorially adjusted hazard ratio for aortic valve stenosis was 1.02 [95% confidence interval (CI) 0.87-1.19] for individuals with triglycerides of 1.0-1.9 mmol/L (89-176 mg/dL), 1.22 (1.02-1.46) for 2.0-2.9 mmol/L (177-265 mg/dL), 1.40 (1.11-1.77) for 3.0-3.9 mmol/L (266-353 mg/dL), 1.29 (0.88-1.90) for 4.0-4.9 mmol/L (354-442 mg/dL), and 1.52 (1.02-2.27) for individuals with triglycerides ≥5 mmol/L (≥443 mg/dL). By age 85, the cumulative incidence of aortic valve stenosis was 5.1% for individuals with plasma triglycerides2.0 mmol/L (77 mg/dL), 6.5% at 2.0-4.9 mmol/L (177-442 mg/dL), and 8.2% for individuals with plasma triglycerides ≥5.0 mmol/L (443 mg/dL). The corresponding values for remnant cholesterol categories were 4.8% for0.5 mmol/L (19 mg/dL), 5.6% for 0.5-1.4 mmol/L (19-57 mg/dL), and 7.4% for ≥1.5 mmol/L (58 mg/dL). Genetically, compared to individuals with allele score 13-16, odds ratios for aortic valve stenosis were 1.30 (95% CI 1.20-1.42; Δtriglycerides +12%; Δremnant cholesterol +11%) for allele score 17-18, 1.41 (1.31-1.52; +25%; +22%) for allele score 19-20, and 1.51 (1.22-1.86; +51%; +44%) for individuals with allele score 21-23.Higher triglycerides and remnant cholesterol were observationally and genetically associated with increased risk of aortic valve stenosis.

Background: The aim was to evaluate the Nt-proBNP discriminatory role between symptomatic and asymptomatic patients with severe aortic stenosis. Methods: 187 patients with severe valvular aortic stenosis, with normal EF > 50%, were included, 61 asymptomatic and 126 symptomatic. We used clinical, laboratory (Nt-proBNP) and echocardiographic parameters. Endpoints of monitoring (occurrence of event) were: the onset of symptoms in asymptomatic patients and death in both groups. Results: The symptomatic group with severe AS had a significantly higher means of Nt-proBNP, in comparison with the asymptomatic group. Nt-proBNP was a significant predictor for the risk of event occurrence (HR 1.4). In the group of severe AS without CAD (n = 101), the subgroup with Nt-proBNP above the cut-off value, took significantly higher percentage of patients with chest pain, fatigue and syncope. In the group with Nt-proBNP above the cut-off value, we had a significantly higher percentage of patients with severe AS without CAD, compared to those with CAD (n = 142). Nt-proBNP was negatively correlated with AVA and LVEF, whether the positive correlation was expressed for: LVEDd, LVEDs, IVSd, AV_Vmax, AV_MaxGrad, LVM and LA. Patients with Nt-proBNP above the cut-off, had a significantly lower event free survival, compared to patients with Nt-proBNP below the cut-off (n = 187; n = 101). Conclusion: The Nt-proBNP cut-off> 460 pg/ml was confirmed as a useful tool in the determination of event free survival in patients with severe AS. Nt-proBNP not only had relevance in the assessment of the severity of the disease, but also was a significant predictor for the risk of event occurrence.

With next-generation valves such as the ALLEGRA valve from NewValve Technologies (NVT), Hechingen Germany, there is a very likely treatment expansion for patients with aortic valve disease. Besides treatment of native valvular aortic stenosis with the ALLEGRA valve, the special implant mechanism seems to be an appealing concept for patients with degenerated surgical bioprostheses. We report the first case of a transfemoral implantation in small degenerated surgical bioprosthesis of a 76-year-old woman.

Sub valvular aortic stenosis (SAS) is the second most common type of aortic stenosis, representing 14% of the obstruction in the left ventricular outflow tract (LVOT), with valvular aortic stenosis being the most common cause (70%).

This work presents a developed zero-dimensional cardiovascular (CV) system model, based on an electrical analogy, with a detailed compartmental description of the heart and the main vascular circulation which is able to simulate normal and diseased conditions of CV system, especially the stenosis valvular aortic. To know the effect of each parameter on hemodynamics, the number of parameters is increased by adding more segments. The developed model consists of 14 compartments. The results show that the severity of aortic stenosis (AS) effect varies with the effective orifice area and the mean pressure gradient for the case of no AS; the effective orifice area is 4[Formula: see text]cm2 and the mean pressure gradient is 0[Formula: see text]mmHg, while for the case of mild AS, the effective orifice area is 1.5[Formula: see text]cm2 and the mean pressure gradient is 27.24[Formula: see text]mmHg. For the case of moderate AS, the effective orifice area is 1.0[Formula: see text]cm2 and the mean pressure gradient is 44.68[Formula: see text]mmHg. For the case of the severe AS, the effective orifice area is 0.61[Formula: see text]cm2 and the mean pressure gradient is 77.51[Formula: see text]mmHg. It is found that the developed model can estimate an accurate value of the effective orifice area for any value of mean pressure gradient in AS. The results obtained for the CV system under normal and diseased conditions show a good agreement compared to published results.

Supravalvular aortic stenosis is the rarest form of congenital left ventricular outflow tract obstruction. It can be associated with Williams syndrome or it may be an isolated anomaly. Although infective endocarditis is common in valvular aortic stenosis, in supravalvular aortic stenosis infective endocarditis/endarteritis is rarely reported. We report a case of infective endarteritis in a case of supravalvular aortic stenosis due to resistant enterococci, causing prolonged fever. Blood culture-directed antibiotic therapy cured the infection.

OBJECTIVES This study presents short- and long-term follow-up after treatment for isolated valvular aortic stenosis in children with surgical valvotomy as the preferred 1st intervention. METHODS All patients aged 0-18 years treated between 1994 and 2013 at our centre were reviewed regarding the mode of first treatment, mortality, reinterventions and the need for aortic valve replacement. RESULTS A total of 113 patients were identified in local registries. There were 44 neonates, 31 infants and 38 children. The mean follow-up period was 11 years (range 2-22 years). No early deaths and only 2 late deaths were reported. Of the 113 patients, 92 patients had open surgical valvotomy as the 1st intervention. Freedom from reintervention was 80%, 69%, 61%, 57% and 56% at 1, 5, 10, 15 and 20 years, respectively. The main indication for reintervention was valvular stenosis. Freedom from aortic valve replacement was 67%. CONCLUSIONS Surgical valvotomy of aortic stenosis in this long-term follow-up study resulted in no 30-day mortality and

Aortic valve area is one of the main criteria used by echocardiography to determine the degree of valvular aortic stenosis, and it is calculated using the continuity equation which assumes that the flow volume of blood is equal at two points, the aortic valve area and the left ventricular outflow tract (LVOT). The main fallacy of this equation is the assumption that the LVOT area which is used to calculate the flow volume at the LVOT level is circular, where it is often an ellipse and sometimes irregular. The aim of this review is to explain the physiology of the continuity equation, the different sources of errors, the added benefits of using three-dimensional imaging modalities to measure LVOT area, the latest recommendations related to valvular aortic stenosis, and to introduce future perspectives. A literature review of studies comparing aortic valve area and LVOT area, after using three-dimensional data, has shown underestimation of both measurements when using the continuity equation. This has more impact on patients with discordant echocardiographic measurements when aortic valve area is disproportionate to haemodynamic measurements in assessing the degree of aortic stenosis. Although fusion imaging modalities of LVOT area can help in certain group of patients to address the issue of aortic valve area underestimation, further research on introducing a correction factor to the conventional continuity equation might be more rewarding, saving patients additional tests and potential radiation, with no clear evidence of cost-effectiveness.

Both the European Society of Cardiology (ESC) and American Heart Association/American College of Cardiology (AHA/ACC) guidelines for management of adults with valvular heart disease have been updated this year in response to publication of new evidence justifying revised or new recommendations.1 2 In particular, more detailed guidance is provided for choice of valve prosthesis type and implantation mode in patients with severe valvular aortic stenosis (AS). There are three distinct steps in decision making in adults with severe AS: Both guidelines acknowledge the complexity of AS assessment, emphasising that the current definition of severe AS includes: In patients with severe AS and a high velocity/gradient, valve area usually is 1.0 cm2 or less, but this is not required for diagnosis in either guideline as long as a reversible high flow state has been excluded. Low-flow in this context is defined as a stroke volume index 35 mL/m2 or less. When LV EF is low (

Valvular aortic stenosis (AS) is a progressive disease that affects 2% of the population aged 65 years or older. The major cause of valvular AS in adults is calcification and fibrosis of a previously normal tricuspid valve or a congenital bicuspid valve, with rheumatic AS being rare in the United States. Once established, the rate of progression of valvular AS is quite variable and impossible to predict for any particular patient. Symptoms of AS are generally insidious at onset, though development of any of the three cardinal symptoms of angina, syncope, or heart failure portends a poor prognosis. Management of symptomatic AS remains primarily surgical, though transcatheter aortic valve replacement (TAVR) is becoming an accepted alternative to surgical aortic valve replacement (SAVR) for patients at high or prohibitive operative risk.

Isolated congenital valvular stenosis of either aortic or pulmonary valve is commonly seen yet the presence of both these lesions in the same patient is rare. This combination presents unusual diagnostic as well as management problems. Apart from a few case reports, there is little in the literature on the combined stenosis of both semilunar valves. We present here a case report of a three and half year old boy diagnosed as a combined congenital severe valvular aortic stenosis with valvular pulmonary stenosis. The patient underwent successful balloon dilatation of both these valves in the same sitting.

Homozygous Familial Hypercholesterolaemia is a genetic disorder which usually presents with early cardiovascular disease ranging from premature ischaemic disease, including myocardial infarction to aortic root stenosis. A 21 year old Bangladeshi male presented with exertional chest pain and breathlessness. He was diagnosed as a case of Homozygous Familial Hypercholesterolaemia. His angina symptoms were due to underlying valvular aortic stenosis which is a rare presentation of Homozygous Familial Hypercholesterolaemia. DOI: http://dx.doi.org/10.3329/cardio.v6i2.18365 Cardiovasc. j. 2014; 6(2): 180-183

This paper presents calculation of the pressure gradient at the point of aortic stenosis in patients with valvular aortic stenosis. The pressure gradients obtained by calculation were compared with the pressure gradients measured using catheterization in 12 patients with valvular aortic stenosis. It has been found that the maximum separation factor influences the maximum blood flow velocity (p

Given that coronary artery disease (CAD) in octogenarians undergoing transcatheter aortic valve implantation (TAVI) often presents with more complex lesions and extensive calcification, rotational atherectomy (RA) may be needed in some cases before stenting. However, data regarding the feasibility and safety of simultaneous RA during TAVI using the Medtronic CoreValve (MCV; Medtronic, Minneapolis, MN, USA) system are lacking. Three out of 107 (2.8%) patients (2 females, average age 85.6 years, mean aortic valve area 0.5 cm2, mean left ventricular ejection fraction 39%, mean Logistic EuroScore 70%), with complex, heavily calcified coronary stenosis, and severe valvular aortic stenosis (AS) were treated with TAVI and RA due to high surgical risk. After balloon valvuloplasty, all coronary lesions were successfully treated with RA and stenting, immediately followed by transfemoral TAVI with a self-expandable MCV. Our data suggested that in the very elderly patients with severe and heavily calcified CAD and AS who were turned down for cardiac surgery, RA and stenting followed by TAVI may be performed successfully in a combined, single-stage procedure.

Twenty-six patients with valvular aortic stenosis were followed up for an average period of 9 years after the initial evaluation when the valvular disease had been considered too mild for surgical treatment. The valve area was 0.7-1.9 (mean 1.2) cm2 at the first study and 0.3-2.0 (mean 0.9) cm2 at the second. The mean annual decrease was about 0.1 cm2 in ten and less in the remaining patients. Advanced age and low physical working capacity at the first investigation were associated with rapid progression of the severity of the stenosis, but rapid progression was not predictable. At follow-up the combination of 1) calcifications of the valve on chest X-ray, 2) low physical working capacity and 3) negative/biphasic T wave in V6 after exercise was present in 100% of the severe stenoses (much less than 0.6 cm2) and in 10% of the mild (much greater than 1.0 cm2). The rate of progression of valvular aortic stenosis in adults is usually slow, but moderate stenoses may become severe within a few years.