Aortic root dilatation and dilated cardiomyopathy in an adult with Tatton‐Brown‐Rahman syndrome

OBJECTIVES: Prevalence, clinical significance, and long-term consequences of aortic root (AoR) dilatation in competitive athletes are not yet investigated. Our aim was to assess the distribution and determinants of AoR size in a large population of competitive athletes. METHODS: AoR dimension were assessed by echocardiography in 2,361 athletes participating in 48 different sports. Of them, 43 were excluded because aortic structural abnormalities, such as bicuspid aortic valve, Marfan’s Syndrome, aortic prosthesis. The remaining 2,318, including 1,301 (56%) males and 1,017 (44%) females were the study population. Arbitrary cut-off of ≥ 40 mm, according to #36 th Bethesda Conference, was used as upper normal limits for AoR. RESULTS: AoR dimension was 32.2 ± 2.7 mm (23 to 44) in males, and 27.5 ± 2.6 mm (20 to 36) in females. AoR dimension exceeded accepted upper limits in only 18 male athletes (0.8%). Figure . Multivariate regression analysis showed left ventricular (LV) mass and body size explaining majority of AoR variability (R 2 = 0,59). Surprisingly, type of sport was not a determinant for AoR dimension. The 18 athletes with enlarged AoR were periodically followed for 7.0 ± 4.2 years. None developed cardiovascular events or symptoms. Two showed progression of AoR dilatation (from 40 to 48, and 43 to 46 mm, respectively), one had incident myocarditis and one developed moderate aortic regurgitation with enlarged LV cavity. CONCLUSIONS: Dilated aortic root is an uncommon finding in competitive athletes and do not represent expression of physiologic cardiac remodelling of the athlete’s heart. Therefore, athletes with dilated AoR deserves continued clinical surveillance.

Download Full-text

Abstract 5769: Correlation of Bicuspid Valve Morphology and Pattern of Aortic Root Dilatation: A Substudy of the Aortic Stenosis Progression Observation Measuring Effects of Rosuvastatin (ASTRONOMER) Study

Circulation ◽

10.1161/circ.118.suppl_18.s_998-b ◽

2008 ◽

Vol 118 (suppl_18) ◽

Author(s):

Kapil M Bhagirath ◽

Davinder S Jassal ◽

James W Tam ◽

Randy A Sochowski ◽

Jean G Dumesnil ◽

...

Keyword(s):

Aortic Stenosis ◽

Aortic Root ◽

Type A ◽

Left Ventricular ◽

Doppler Imaging ◽

Type B ◽

Root Parameters ◽

Standardized Protocol ◽

Aortic Root Dilatation ◽

Annular Calcification

Bicuspid aortic valve is the leading cause of aortic stenosis in patients younger than the age of 50. A classification scheme of bicuspid aortic valves (BAV) was recently proposed based upon leaflet orientation: Type A (fusion of right and left coronary cusps) and Type B (fusion of right and non-coronary cusps). The correlation between BAV leaflet orientation and aortic root pathology however remains ill defined. To describe a potential relationship between BAV leaflet morphology and aortic root measurements in the ASTRONOMER study, a multicentre study to assess the effect of Rosuvastatin on the progression of AS. Transthoracic echocardiography was performed with 2D and Doppler imaging following a standardized protocol. BAV morphology was classified as Type A or Type B orientation following review of the parasternal short-axis view. Echo measurements including left ventricular and aortic root dimensions were obtained according to the ASE recommendations. We identified 89 patients (56±11 years; 44 males). There were 63 patients with Type A and 26 with Type B BAV. Baseline demographic, hemodynamics, aortic root and left heart dimensions are listed in Table 1 . Patients with Type A BAV had larger aortic and ascending root dimensions than those patients with Type B BAV (p<0.05). Aortic valvular calcification and mitral annular calcification were similar between the two groups. All values are expressed as mean±SD. In patients with mild to moderate asymptomatic BAV, the presence of Type A valve orientation was associated with significantly greater aortic root parameters compared to Type B valve orientation. Whether the morphology of BAV may predict a subset of patients who will respond to statin therapy in preventing the progression of AS remains to be determined upon completion of the ASTRONOMER trial.

Download Full-text

Arterial hypertension and aortic root dilatation: an unsolved mystery

Italian Journal of Medicine ◽

10.4081/itjm.2011.6 ◽

2013 ◽

pp. 6-11

Author(s):

Alberto Milan ◽

Francesco Tosello ◽

Sara Abram ◽

Ambra Fabbri ◽

Alessandro Vairo ◽

...

Keyword(s):

Arterial Hypertension ◽

Thoracic Aorta ◽

Aortic Root ◽

Aortic Wall ◽

Aortic Aneurysms ◽

Published Data ◽

Thoracic Aortic Aneurysms ◽

Biological Variables ◽

Aortic Root Dilatation ◽

Active Debate

Introduction: Acute and chronic aortic syndromes are associated with substantial morbidity and mortality. Silent risk factors such as arterial hypertension and aortic root dilatation can increase the likelihood of aortic dissection or rupture. The relationship between arterial hypertension and the dimensions of the aortic root dimension is a topic of active debate. Materials and methods: We reviewed the literature on the physiopathology, diagnosis, natural history, and management of thoracic aortic aneurysms. Results: Biological variables influencing the size of the aorta include age, sex, body surface area, pressure values, and stroke volume. Pathologic enlargement of the thoracic aorta can be caused by genetic, degenerative, inflammatory, traumatic, or toxic factors. Studies investigating the correlation between aortic dimensions and arterial pressures (diastolic, systolic, or pulse) have produced discordant results. Discussion: Classically, emphasis has been placed on the importance of hypertension-related degeneration of the medial layer of the aortic wall, which leads to dilatation of the thoracic aorta, reduced aortic wall compliance, and increased pulse pressures. However, there are no published data that demonstrate unequivocally the existence of a pathogenetic correlation between arterial hypertension and aortic root dilatation. Furthermore, there is no evidence that antihypertensive therapy is effective in the management of nonsyndromic forms of aortic root dilatation. An interesting branch of research focuses on the importance of genetic predisposition in the pathogenesis of thoracic aortic aneurysms. Different genetic backgrounds could explain differences in the behaviour of aortic walls exposed to the same hemodynamic stress. Further study is needed to evaluate these focal physiopathological aspects.

Download Full-text