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PURPOSE: The purpose of this study was to evaluate the performance of four-dimensional (4D) flow cardiac MRI in quantifying aortic flow in patients with bicuspid aortic valve (BAV). MATERIALS AND METHODS: Patients with BAV who underwent transthoracic echocardiography (TTE) and 4D flow cardiac MRI were prospectively included. Aortic flow was quantified using two-dimensional phase contrast velocimetry at the sinotubular junction and in the ascending aorta and using 4D flow in the regurgitant jet, in the left ventricular outflow tract, at the aortic annulus, the sinotubular junction, and the ascending aorta, with or without anatomical tracking. Flow quantification was compared with ventricular volumes, pulmonary flow using Pearson correlation test, bias and limits of agreement (LOA) using Bland Altman method, and with multiparametric transthoracic echocardiography quantification using weighted kappa test. RESULTS: Eighty-eight patients (63 men, 25 women) with a mean age of 50.5 ± 14.8 (standard deviation) years (age range: 20.8-78.3) were included. Changes in flow with or without tracking were modest (< 5 mL). The best correlation was obtained at the aortic annulus for forward volume (r = 0.84; LOA [-28.4; 25.3] mL) and at the regurgitant jet and sinotubular junction for regurgitant volume (r = 0.68; LOA [-27.8; 33.8] and r = 0.69; LOA [-28.6; 24.2] mL). A combined approach for regurgitant fraction and net volume calculations using forward volume measured at ANN and regurgitant volume at sinotubular junction performed better than each level taken separately (r = 0.90; LOA [-20.7; 10.0] mL and r = 0.48, LOA [-33.8; 33.4] %). The agreement between transthoracic echocardiography and 4D flow cardiac MRI for aortic regurgitation grading was poor (kappa, 0.13 to 0.42). CONCLUSION: In patients with BAV, aortic flow quantification by 4D flow cardiac MRI is the most accurate at the annulus for the forward volume, and at the sinotubular junction or directly in the jet for the regurgitant volume.
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Aortopathy encompasses a spectrum of conditions predisposing to dilation, aneurysm, dissection, or rupture of the aorta and other blood vessels. Aortopathy is diagnosed commonly in children, from infancy through adolescence, primarily affecting the thoracic aorta, with variable involvement of the peripheral vasculature. Pathogeneses include connective tissue disorders, smooth muscle contraction disorders, and congenital heart disease, including bicuspid aortic valve, among others. The American Heart Association has published guidelines for diagnosis and management of thoracic aortic disease. However, these guidelines are predominantly focused on adults and cannot be applied adeptly to growing children with emerging features, growth and developmental changes, including puberty, and different risk profiles compared with adults. Management to reduce risk of progressive aortic dilation and dissection or rupture in children is complex and involves genetic testing, cardiovascular imaging, medical therapy, lifestyle modifications, and surgical guidance that differ in many ways from adult management. Pediatric practice varies widely, likely because aortopathy is pathogenically heterogeneous, including genetic and nongenetic conditions, and there is limited published evidence to guide care in children. To optimize care and reduce variation in management, experts in pediatric aortopathy convened to generate this scientific statement regarding the cardiovascular care of children with aortopathy. Available evidence and expert consensus were combined to create this scientific statement. The most common causes of pediatric aortopathy are reviewed. This document provides a general framework for cardiovascular management of aortopathy in children, while allowing for modification based on the personal and familial characteristics of each child and family.
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American Heart Association , Enfermedades de la Aorta , Humanos , Niño , Estados Unidos , Enfermedades de la Aorta/terapia , Enfermedades de la Aorta/diagnóstico , Adolescente , Manejo de la Enfermedad , Lactante , PreescolarRESUMEN
Infective endocarditis (IE) is a serious cardiovascular condition with the potential to lead to severe valvular regurgitation. We present a case of a 65-year-old male who presented with a fever and was diagnosed with IE through point-of-care ultrasound (POCUS). The patient's condition subsequently led to severe aortic regurgitation. Timely diagnosis facilitated by POCUS played a crucial role in the management of this case. The patient underwent successful timely surgical intervention to prevent further infective embolism and heart failure due to severe acute aortic regurgitation. This case underscores the pivotal role of POCUS in the early diagnosis and multidisciplinary management of cardiology diseases, highlighting its importance in delivering optimal patient care.
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Valvular heart disease is a common cause of morbidity and mortality worldwide and has no effective medical therapy. Severe disease is managed with valve replacement procedures, which entail high health care-related costs and postprocedural morbidity and mortality. Robust ongoing research programs have elucidated many important molecular pathways contributing to primary valvular heart disease. However, there remain several key challenges inherent in translating research on valvular heart disease to viable molecular targets that can progress through the clinical trials pathway and effectively prevent or modify the course of these common conditions. In this scientific statement, we review the basic cellular structures of the human heart valves and discuss how these structures change in primary valvular heart disease. We focus on the most common primary valvular heart diseases, including calcific aortic stenosis, bicuspid aortic valves, mitral valve prolapse, and rheumatic heart disease, and outline the fundamental molecular discoveries contributing to each. We further outline potential therapeutic molecular targets for primary valvular heart disease and discuss key knowledge gaps that might serve as future research priorities.
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American Heart Association , Enfermedades de las Válvulas Cardíacas , Humanos , Enfermedades de las Válvulas Cardíacas/tratamiento farmacológico , Enfermedades de las Válvulas Cardíacas/metabolismo , Estados Unidos , AnimalesRESUMEN
We present a case report of a patient with infective endocarditis. He came to the emergency room with respiratory failure due to severe pneumonia and pulmonary edema. On 2D transesophageal echocardiography, vegetations were seen in both mitral and aortic valves, with mitral valve perforation and severe regurgitation. His clinical presentation and severity of the disease made him suitable for urgent valve repair. He was submitted to mitral valvuloplasty with closure of the valve perforation and insertion of a bioprosthetic aortic valve. Despite significant clinical improvement, a post-surgical complication was noted with new-onset lung injury after cardiopulmonary bypass. This is an interesting case of a patient with suspected retrograde valve involvement, affecting the aortic valve, the mitral-aortic intervalvular fibrosa, and the mitral valve, ending with mitral valve abscess with leaflet perforation and valvular regurgitation.
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BACKGROUND: Ascending aorta dilation and aortic valve degeneration are common complications in patients with bicuspid aortic valve. Several retrospective studies have suggested the benefit of statins in reducing these complications. This study aimed to determine whether atorvastatin treatment is effective in reducing the growth of aortic diameters in bicuspid aortic valve and if it slows the progression of valve calcification. METHODS: In a randomized clinical trial, 220 patients with bicuspid aortic valve (43 women; 46±13 years of age) were included and treated with either 20 mg of atorvastatin per day or placebo for 3 years. Inclusion criteria were ≥18 years of age, nonsevere valvular dysfunction, nonsevere valve calcification, and ascending aorta diameter ≤50 mm. Computed tomography and echocardiography studies were performed at baseline and after 3 years of treatment. RESULTS: During follow-up, 28 patients (12.7%) discontinued medical treatment (15 on atorvastatin and 13 taking placebo). Thus, 192 patients completed the 36 months of treatment. Low-density lipoprotein cholesterol levels decreased significantly in the atorvastatin group (median [interquartile range], -30 mg/dL [-51.65 to -1.75 mg/dL] versus 6 mg/dL [-4, 22.5 mg/dL]; P<0.001). The maximum ascending aorta diameter increased with no differences between groups: 0.65 mm (95% CI, 0.45-0.85) in the atorvastatin group and 0.74 mm (95% CI, 0.45-1.04) in the placebo group (P=0.613). Similarly, no significant differences were found for the progression of the aortic valve calcium score (P=0.167) or valvular dysfunction. CONCLUSIONS: Among patients with bicuspid aortic valve without severe valvular dysfunction, atorvastatin treatment was not effective in reducing the progression of ascending aorta dilation and aortic valve calcification during 3 years of treatment despite a significant reduction in low-density lipoprotein cholesterol levels. REGISTRATION: URL: https://www.clinicaltrialsregister.eu; Unique identifier: 2015-001808-57. URL: https://www.clinicaltrials.gov; Unique identifier: NCT02679261.
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Válvula Aórtica , Atorvastatina , Enfermedad de la Válvula Aórtica Bicúspide , Calcinosis , Progresión de la Enfermedad , Enfermedades de las Válvulas Cardíacas , Inhibidores de Hidroximetilglutaril-CoA Reductasas , Humanos , Atorvastatina/uso terapéutico , Femenino , Masculino , Persona de Mediana Edad , Válvula Aórtica/diagnóstico por imagen , Válvula Aórtica/patología , Válvula Aórtica/anomalías , Válvula Aórtica/efectos de los fármacos , Calcinosis/tratamiento farmacológico , Calcinosis/diagnóstico por imagen , Calcinosis/patología , Enfermedad de la Válvula Aórtica Bicúspide/diagnóstico por imagen , Enfermedad de la Válvula Aórtica Bicúspide/tratamiento farmacológico , Enfermedades de las Válvulas Cardíacas/tratamiento farmacológico , Enfermedades de las Válvulas Cardíacas/diagnóstico por imagen , Enfermedades de las Válvulas Cardíacas/patología , Adulto , Inhibidores de Hidroximetilglutaril-CoA Reductasas/uso terapéutico , Dilatación Patológica/tratamiento farmacológico , Estudios de Seguimiento , Método Doble Ciego , Resultado del Tratamiento , Aorta/diagnóstico por imagen , Aorta/patología , Aorta/efectos de los fármacos , Enfermedad de la Válvula Aórtica/tratamiento farmacológico , Estenosis de la Válvula AórticaRESUMEN
Mouse models of congenital aortic valve malformations are useful for studying disease pathobiology, but most models have incomplete penetrance [e.g., â¼2 to 77% prevalence of bicuspid aortic valves (BAVs) across multiple models]. For longitudinal studies of pathologies associated with BAVs and other congenital valve malformations, which manifest over months in mice, it is operationally inefficient, economically burdensome, and ethically challenging to enroll large numbers of mice in studies without first identifying those with valvular abnormalities. To address this need, we established and validated a novel in vivo high-frequency (30 MHz) ultrasound imaging protocol capable of detecting aortic valvular malformations in juvenile mice. Fifty natriuretic peptide receptor 2 heterozygous mice on a low-density lipoprotein receptor-deficient background (Npr2+/-;Ldlr-/-; 32 males and 18 females) were imaged at 4 and 8 wk of age. Fourteen percent of the Npr2+/-;Ldlr-/- mice exhibited features associated with aortic valve malformations, including 1) abnormal transaortic flow patterns on color Doppler (recirculation and regurgitation), 2) peak systolic flow velocities distal to the aortic valves reaching or surpassing â¼1,250 mm/s by pulsed-wave Doppler, and 3) putative fusion of cusps along commissures and abnormal movement elucidated by two-dimensional (2-D) imaging with ultrahigh temporal resolution. Valves with these features were confirmed by ex vivo gross anatomy and histological visualization to have thickened cusps, partial fusions, or Sievers type-0 bicuspid valves. This ultrasound imaging protocol will enable efficient, cost effective, and humane implementation of studies of congenital aortic valvular abnormalities and associated pathologies in a wide range of mouse models.NEW & NOTEWORTHY We developed a high-frequency ultrasound imaging protocol for diagnosing congenital aortic valve structural abnormalities in 4-wk-old mice. Our protocol defines specific criteria to distinguish mice with abnormal aortic valves from those with normal tricuspid valves using color Doppler, pulsed-wave Doppler, and two-dimensional (2-D) imaging with ultrahigh temporal resolution. This approach enables early identification of valvular abnormalities for efficient and ethical experimental design of longitudinal studies of congenital valve diseases and associated pathologies in mice.
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Válvula Aórtica , Modelos Animales de Enfermedad , Receptores del Factor Natriurético Atrial , Animales , Válvula Aórtica/anomalías , Válvula Aórtica/diagnóstico por imagen , Válvula Aórtica/patología , Femenino , Masculino , Receptores del Factor Natriurético Atrial/genética , Receptores del Factor Natriurético Atrial/deficiencia , Receptores del Factor Natriurético Atrial/metabolismo , Ratones , Ratones Noqueados , Receptores de LDL/genética , Receptores de LDL/deficiencia , Ratones Endogámicos C57BL , Enfermedad de la Válvula Aórtica Bicúspide/diagnóstico por imagenRESUMEN
Patients with bicuspid aortic valve (BAV) commonly have associated aortic stenosis and aortopathy. The geometry of the aortic arch and BAV is not well defined quantitatively, which makes clinical classifications subjective or reliant on limited 2D measurements. The goal of this study was to characterize the 3D geometry of the aortic arch and BAV using objective and quantitative techniques. Pre-TAVR computed tomography angiogram (CTA) in patients with BAV and aortic stenosis (AS) were analyzed (n = 59) by assessing valve commissural angle, presence of a fused region, percent of fusion, and calcium volume. The ascending aorta and aortic arch were reconstructed from patient-specific imaging segmentation to generate a centerline and calculate maximum curvature and maximum area change for the ascending aorta and the descending aorta. Aortic valve commissural angle signified a bimodal distribution suggesting tricuspid-like (≤ 150°, 52.5% of patients) and bicuspid-like (> 150°, 47.5%) morphologies. Tricuspid like was further classified by partial (10.2%) or full (42.4%) fusion, and bicuspid like was further classified into valves with fused region (27.1%) or no fused region (20.3%). Qualitatively, the aortic arch was found to have complex patient-specific variations in its 3D shape with some showing extreme diameter changes and kinks. Quantitatively, subgroups were established using maximum curvature threshold of 0.04 and maximum area change of 30% independently for the ascending and descending aorta. These findings provide insight into the geometric structure of the aortic valve and aortic arch in patients presenting with BAV and AS where 3D characterization allows for quantitative classification of these complex anatomic structures.
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Aorta Torácica , Válvula Aórtica , Enfermedad de la Válvula Aórtica Bicúspide , Imagenología Tridimensional , Humanos , Enfermedad de la Válvula Aórtica Bicúspide/diagnóstico por imagen , Válvula Aórtica/anomalías , Válvula Aórtica/diagnóstico por imagen , Válvula Aórtica/patología , Aorta Torácica/diagnóstico por imagen , Masculino , Femenino , Anciano , Enfermedades de las Válvulas Cardíacas/diagnóstico por imagen , Persona de Mediana Edad , Estenosis de la Válvula Aórtica/diagnóstico por imagen , Estenosis de la Válvula Aórtica/fisiopatología , Anciano de 80 o más Años , Angiografía por Tomografía ComputarizadaRESUMEN
Trichinella spiralisis an uncommon parasitic disease contracted through the consumption of undercooked pork. We report the case of a 59-year-old man with a history of bicuspid aortic valve with recent travel to the Philippines and consumption of raw pork presenting with progressive myalgia and hypereosinophilia (nadir 12,940/uL) in profound cardiogenic shock in the setting of critical aortic stenosis. He underwent emergent balloon valvuloplasty, which was complicated by aortic insufficiency. This necessitated a transcatheter aortic valve replacement. However, despite hemodynamic stabilization, he developed catastrophic eosinophilic myocarditis, complicated by cardiac arrest from ventricular tachycardia. A rectus femoris muscle biopsy confirmed the diagnosis, showing a T. spiralis parasite and significant eosinophilic infiltration. Empiric treatment with albendazole, ivermectin, and methylprednisolone resulted in the significant resolution of symptoms and the liberalization of critical illness. This case highlights the challenges of diagnosing the underlying etiologies of hypereosinophilia and/or eosinophilic myocarditis, underscoring the importance of considering parasitic etiologies, particularly in endemic regions or in patients who have a significant travel history to such areas. Prompt diagnosis and treatment are essential to prevent morbidity and mortality.
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BACKGROUND: The potential impact of exercise on valvular function and aortic diameters in patients with a bicuspid aortic valve remains unclear. Therefore, we assessed the association between lifelong exercise characteristics, valvular dysfunction, and aortic dilatation in patients with a bicuspid aortic valve. METHODS AND RESULTS: In this cross-sectional study, exercise volume (metabolic equivalent of task minutes per week), exercise intensity, and sport type were determined from the age of 12 years to participation using a validated questionnaire. Echocardiography was used to assess aortic stenosis or aortic regurgitation and to measure diameters at the sinuses of Valsalva and ascending aorta. Aortic dilatation was defined as a Z-score ≥2. Four hundred and seven patients (42±17 years, 60% men) were included, of which 133 were sedentary (<500 metabolic equivalent of task minutes per week), 94 active (500-1000 metabolic equivalent of task minutes per week), and 180 highly active (≥1000 metabolic equivalent of task minutes per week). Moderate-to-severe aortic stenosis or aortic regurgitation was present in 23.7% and 20.0%, respectively. Sinuses of Valsalva and ascending aorta diameters were 34.8±6.6 and 36.5±8.1 mm, whereas aortic dilatation was found in 21.6% and 53.4%, respectively. Exercise volume was not associated with valve dysfunction or aortic dilatation. Vigorous intensity and mixed sports were associated with a lower prevalence of aortic stenosis (adjusted odds ratios, 0.43 [0.20-0.94] and adjusted odds ratios, 0.47 [0.23-0.95]). Exercise intensity and sport type were not associated with aortic regurgitation and aortic dilatation. CONCLUSIONS: We found no deleterious associations between lifelong exercise characteristics, valvular dysfunction, and aortic dilatation in patients with a bicuspid aortic valve. Vigorous intensity and exercise in mixed sports were associated with a lower prevalence of moderate-to-severe aortic stenosis. These observations suggest that lifelong exercise does not appear to induce adverse cardiovascular effects in patients with a bicuspid aortic valve.
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Insuficiencia de la Válvula Aórtica , Estenosis de la Válvula Aórtica , Enfermedad de la Válvula Aórtica Bicúspide , Masculino , Humanos , Niño , Femenino , Insuficiencia de la Válvula Aórtica/diagnóstico por imagen , Insuficiencia de la Válvula Aórtica/epidemiología , Insuficiencia de la Válvula Aórtica/complicaciones , Válvula Aórtica/diagnóstico por imagen , Estudios Transversales , Estudios Retrospectivos , Estenosis de la Válvula Aórtica/diagnóstico por imagen , Estenosis de la Válvula Aórtica/epidemiología , Estenosis de la Válvula Aórtica/complicaciones , Dilatación PatológicaRESUMEN
Limited data are available about the impact of permanent pacemaker (PPM) implantation on long-term survival in patients with a bicuspid aortic valve (BAV) and severe aortic stenosis (AS) treated with transcatheter aortic valve replacement (TAVR). We aimed to evaluate the long-term clinical outcomes of patients with BAV with AS who underwent periprocedural PPM implantation after TAVR with a self-expandable prosthesis. Data from patients with BAV and severe AS who underwent TAVR between April 2009 and January 2022 and followed in the framework of the One Hospital ClinicalService-CoreValve Project were collected. Patients were categorized in 2 groups according to PPM implantation after TAVR ("PPM" group) or not ("no PPM" group). The coprimary end points were all-cause death and a composite of cardiac mortality, rehospitalization because of cardiac causes, stroke, and myocardial infarction. Overall, 106 patients were considered (74 in the "no PPM" group and 32 in the "PPM" group). No statistically significant difference was found between the groups in terms of follow-up and baseline characteristics. Patients in the PPM group were more likely to show baseline conduction abnormalities (p = 0.023). Patients in the PPM group were more often treated with older generation prosthesis than those in the no PPM group (28.1% vs 5.4%, respectively, p = 0.013). At 2 years of follow-up, all-cause death in the no PPM and PPM groups occurred in 20.0% and 10.0% of patients, respectively (hazard ratio 0.37, 95% confidence interval 0.08 to 1.67). Similarly, no difference was evident for the composite end point between the 2 groups (no PPM vs PPM: 8 [14.6%] vs 6 [19.3%], hazard ratio 1.67, 95% CI 0.58 to 4.81). In conclusion, patients with severe AS and BAV treated with TAVR complicated by PPM implantation are not exposed to an increased risk of major adverse events at 2 years of follow-up.
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Estenosis de la Válvula Aórtica , Enfermedad de la Válvula Aórtica Bicúspide , Marcapaso Artificial , Reemplazo de la Válvula Aórtica Transcatéter , Humanos , Reemplazo de la Válvula Aórtica Transcatéter/efectos adversos , Enfermedad de la Válvula Aórtica Bicúspide/cirugía , Resultado del Tratamiento , Válvula Aórtica/cirugía , Factores de RiesgoRESUMEN
Purpose: This study aimed to compare the diagnostic performance of cardiac CT and transthoracic echocardiogram (TTE) depending on the degree of valvular calcification and bicuspid aortic valve (BAV) subtype. Materials and Methods: This retrospective study included 266 consecutive patients (106 with BAV and 160 with tricuspid aortic valve) who underwent cardiac CT and TTE before aortic valve replacement. Cardiac CT was used to evaluate the morphology of the aortic valve, and a calcium scoring scan was used to quantify valve calcium. The aortic valves were classified into fused and two-sinus types. The diagnostic accuracy of cardiac CT and TTE was calculated using a reference standard for intraoperative inspection. Results: CT demonstrated significantly higher sensitivity, negative predictive value, and accuracy than TTE in detecting BAV (p < 0.001, p < 0.001, and p = 0.003, respectively). The TTE sensitivity tended to decrease as valvular calcification increased. The error rate of TTE for CT was 10.9% for the two-sinus type of BAV and 28.3% for the fused type (p = 0.044). Conclusion: Cardiac CT had a higher diagnostic performance in detecting BAV than TTE and may help diagnose BAV, particularly in patients with severe valvular calcification.
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BACKGROUND: Data concerning the outcomes of transcatheter aortic valve replacement in type 0 bicuspid aortic stenosis (AS) are scarce. The study aims to compare the outcomes of transcatheter aortic valve replacement for AS in patients with type 0 bicuspid, type 1 bicuspid, and tricuspid aortic valve anatomy. METHODS: We enrolled consecutive patients undergoing transcatheter aortic valve replacement for severe AS between 2012 and 2022 in this single-center retrospective cohort study. The primary outcome was mortality, while secondary outcomes included in-hospital complications such as stroke and pacemaker implantation and transcatheter heart valve hemodynamic performance. RESULTS: The number of patients with AS with type 0 bicuspid, type 1 bicuspid, and tricuspid aortic valve anatomy was 328, 302, and 642, respectively. Self-expanding transcatheter heart valves were used in the majority of patients (n=1160; 91.4%). In the matched population, differences in mortality (30 days: 4.2% versus 1.7% versus 1.7%, Poverall=0.522; 1 year: 10% versus 2.3% versus 6.2%, Poverall=0.099) and all stroke (30 days: 1.0% versus 0.9% versus 0.0%, Poverall=0.765; 1 year: 1.4% versus 1.6% versus 1.3%, Poverall=NS) were nonsignificant, and the incidence of overall in-hospital complications was comparable among groups. Ascending aortic diameter was the single predictor of 1-year mortality in type 0 bicuspid patients (hazard ratio, 1.59 [95% CI, 1.03-2.44]; P=0.035). The proportion of patients with a mean residual gradient ≥20 mm Hg was the highest in those with type 0 bicuspid anatomy, although the need for permanent pacemaker implantation was the lowest in this group. CONCLUSIONS: Major clinical outcomes of transcatheter aortic valve replacement for AS in patients with type 0 bicuspid, type 1 bicuspid, and tricuspid aortic valve anatomy are equivalent at short- and mid-term follow-up. These observations merit further exploration in prospective international registries and randomized controlled trials.
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Estenosis de la Válvula Aórtica , Enfermedades de las Válvulas Cardíacas , Accidente Cerebrovascular , Reemplazo de la Válvula Aórtica Transcatéter , Humanos , Válvula Aórtica/diagnóstico por imagen , Válvula Aórtica/cirugía , Reemplazo de la Válvula Aórtica Transcatéter/efectos adversos , Estudios Prospectivos , Estudios Retrospectivos , Enfermedades de las Válvulas Cardíacas/diagnóstico por imagen , Enfermedades de las Válvulas Cardíacas/cirugía , Resultado del Tratamiento , Estenosis de la Válvula Aórtica/diagnóstico por imagen , Estenosis de la Válvula Aórtica/cirugía , Estenosis de la Válvula Aórtica/etiología , Accidente Cerebrovascular/etiología , Accidente Cerebrovascular/cirugíaRESUMEN
Aims: Dilation of the ascending aorta (AA) is often asymptomatic until a life-threatening dissection or rupture occurs. An overall increase in the use of thoracic imaging has enabled early and sometimes incidental identification of AA dilation. Still, the prevalence and determinants of AA dilation remain to be clarified. The aim was to identify and characterize persons with AA dilation in a middle-aged Swedish population. Methods and results: We used the Swedish CardioPulmonary BioImage Study Linköping (n = 5058, age 50-65 years) to identify cases with AA diameter ≥ 40â mm on coronary computed tomography angiography (CCTA) or chest computed tomography. Age- and gender-matched individuals with AA diameter < 40â mm served as controls. Echocardiography, blood pressure (BP) measurements (office and home), pulse wave velocity (PWV), coronary artery calcification (CAC), CCTA-detected coronary atherosclerosis, and carotid ultrasound were used to characterize these subjects. We identified 70 cases (mean AA diameter 44â mm, 77% men) and matched these to 146 controls (mean AA diameter 34â mm). Bicuspid aortic valve and aortic valve dysfunction were more common in cases than in controls (8% vs. 0% and 39% vs. 11%, respectively). Both office and home BP levels were significantly higher among cases. Also, high PWV (>10â m/s) levels were more common in cases (33% vs. 17%). Neither CAC scores nor prevalence or burden of atherosclerosis in coronary and carotid arteries differed between groups. Conclusion: The prevalence of dilated AA was 1.4% and showed positive associations with male gender, aortic valve pathology, and diastolic BP, though not with subclinical atherosclerosis.
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Prosthetic valvular infolding during transcatheter aortic valve implantation (TAVI) is an under-recognized yet significant complication that can occur. Here, we describe the case of a 61-year-old male with a history of heart failure with reduced ejection fraction (HFrEF) and low-flow, low-gradient severe aortic valve stenosis of a bicuspid aortic valve who presented to undergo TAVI. During the procedure, repositioning of the valve resulted in prosthetic valvular infolding and resultant severe aortic regurgitation (AR), culminating in cardiac arrest. Swift balloon valvuloplasty corrected the valve geometry and eliminated any AR, allowing hemodynamic recovery and completion of the procedure. Our case and review highlight methods, both angiographic and echocardiographic, to recognize prosthetic valvular infolding the moment it presents, as well as strategies to correct the infolding with minimal detriment to the patient.
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At present, transcatheter aortic valve replacement (TAVR) is not only used in high-surgical-risk patients with aortic stenosis (AS), but its use has also been extended to low-risk patients, resulting in its increasing utilization in patients with bicuspid aortic valve (BAV). BAV however presents unique challenges for TAVR due to its distinct valvular anatomy, and surgical aortic valve replacement (SAVR) remains the primary recommended method of aortic valve replacement in patients with BAV. Nonetheless, observational data have been quickly accumulating regarding the successful use of TAVR in BAV. Here, we present a case of a 73-year-old female who presented with heart failure symptoms and was found to have severe AS and BAV with calcified raphe (Sievers 1a). Due to her age and complicated medical history, including coronary artery disease and chronic kidney disease, she was considered to be at intermediate surgical risk (Society of Thoracic Surgeons (STS) score 5.4%) and underwent TAVR with the successful deployment of a 29 mm Edwards SAPIEN valve (Edwards Lifesciences, California, USA). A post-procedure echocardiogram confirmed the appropriate placement of the prosthesis without any valvular or paravalvular regurgitation. This case, therefore, adds to the growing body of evidence regarding the use of TAVR in patients with BAV despite anatomical challenges.
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BACKGROUND: Thoracic aortic disease and bicuspid aortic valve (BAV) likely have a heritable component, but large population-based studies are lacking. This study characterizes familial associations of thoracic aortic disease and BAV, as well as cardiovascular and aortic-specific mortality, among relatives of these individuals in a large-population database. METHODS: In this observational case-control study of the Utah Population Database, we identified probands with a diagnosis of BAV, thoracic aortic aneurysm, or thoracic aortic dissection. Age- and sex-matched controls (10:1 ratio) were identified for each proband. First-degree relatives, second-degree relatives, and first cousins of probands and controls were identified through linked genealogical information. Cox proportional hazard models were used to quantify the familial associations for each diagnosis. We used a competing-risk model to determine the risk of cardiovascular-specific and aortic-specific mortality for relatives of probands. RESULTS: The study population included 3 812 588 unique individuals. Familial hazard risk of a concordant diagnosis was elevated in the following populations compared with controls: first-degree relatives of patients with BAV (hazard ratio [HR], 6.88 [95% CI, 5.62-8.43]); first-degree relatives of patients with thoracic aortic aneurysm (HR, 5.09 [95% CI, 3.80-6.82]); and first-degree relatives of patients with thoracic aortic dissection (HR, 4.15 [95% CI, 3.25-5.31]). In addition, the risk of aortic dissection was higher in first-degree relatives of patients with BAV (HR, 3.63 [95% CI, 2.68-4.91]) and in first-degree relatives of patients with thoracic aneurysm (HR, 3.89 [95% CI, 2.93-5.18]) compared with controls. Dissection risk was highest in first-degree relatives of patients who carried a diagnosis of both BAV and aneurysm (HR, 6.13 [95% CI, 2.82-13.33]). First-degree relatives of patients with BAV, thoracic aneurysm, or aortic dissection had a higher risk of aortic-specific mortality (HR, 2.83 [95% CI, 2.44-3.29]) compared with controls. CONCLUSIONS: Our results indicate that BAV and thoracic aortic disease carry a significant familial association for concordant disease and aortic dissection. The pattern of familiality is consistent with a genetic cause of disease. Furthermore, we observed higher risk of aortic-specific mortality in relatives of individuals with these diagnoses. This study provides supportive evidence for screening in relatives of patients with BAV, thoracic aneurysm, or dissection.
Asunto(s)
Aneurisma de la Aorta Torácica , Enfermedades de la Aorta , Disección Aórtica , Enfermedad de la Válvula Aórtica Bicúspide , Enfermedades de las Válvulas Cardíacas , Humanos , Válvula Aórtica , Enfermedades de las Válvulas Cardíacas/diagnóstico , Estudios de Casos y Controles , Prevalencia , Causas de Muerte , Aneurisma de la Aorta Torácica/genética , Disección Aórtica/genéticaRESUMEN
PURPOSE: The purpose of this study was to investigate the benefit of aortic volumes compared to diameters or cross-sectional areas on three-dimensional (3D) magnetic resonance imaging (MRI) in discriminating between patients with dilated aorta and matched controls. MATERIALS AND METHODS: Sixty-two patients (47 men and 15 women; median age, 66 years; age range: 33-86 years) with tricuspid aortic valve and ascending thoracic aorta aneurysm (TAV-ATAA) and 43 patients (35 men and 8 women; median age, 51 years; age range: 17-76 years) with bicuspid aortic valve and dilated ascending aorta (BAV) were studied. One group of 54 controls matched for age and sex to patients with TAV-ATAA (39 men and 15 women; median age, 68 years; age range: 33-81 years) and one group of 42 controls matched for age and sex to patients with BAV (34 men and 8 women; median age, 50 years; age range: 17-77 years) were identified. All participants underwent 3D MRI, used for 3D-segmentation for measuring aortic length, maximal diameter, maximal cross-sectional area (CSA) and volume for the ascending aorta. RESULTS: An increase in ascending aorta volume (TAV-ATAA: +107%; BAV: +171% vs. controls; P < 0.001) was found, which was three times greater than the increase in diameter (TAV-ATAA: +29%; BAV: +40% vs. controls; P < 0.001). In differentiating patients with TAV-ATAA from their controls, the indexed ascending aorta volume showed better performances (AUC, 0.935 [95% confidence interval (CI): 0.882-0.989]; accuracy, 88.7% [95% CI: 82.9-94.5]) than indexed ascending aorta length (P < 0.001), indexed ascending aorta maximal diameter (P = 0.003) and indexed ascending aorta maximal CSA (P = 0.03). In differentiating patients with BAV from matched controls, indexed ascending aorta volume showed significantly better performances performance (AUC, 0.908 [95% CI: 0.829-0.987]; accuracy, 88.0% [95% CI: 80.9-95.0]) than indexed ascending aorta length (P = 0.02) and not different from indexed ascending aorta maximal diameter (P = 0.07) or from indexed ascending aorta maximal CSA (P = 0.27) CONCLUSION: Aortic volume measured by 3D-MRI integrates both elongation and luminal dilatation, resulting in greater classification performance than maximal diameter and length in differentiating patients with dilated ascending aorta or aneurysm from controls.