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Systemic hypertension (SH) in dogs typically occurs secondary to renal disease, diabetes mellitus, hyperadrenocorticism, malignant adrenal tumors, or various medications. Echocardiography performed on people with SH has shown asymmetric dilation of the sinuses at the level of the aortic valves, previously undescribed in canine patients. The objective of this retrospective case-control study was to determine if there was a difference in size and shape of the aortic cusps at the level of the sinus of Valsalva in dogs with SH compared to dogs with normal blood pressure. We reviewed echocardiographic findings in 74 dogs with SH and 37 dogs with normal blood pressure (NBP), defined as less than 150 mmHg. Systemic hypertension was classified as mild (150-159 mm Hg), moderate (160-179 mm Hg), or severe (greater than 180 mm Hg). There was a significant difference (P < .001) in the mean size of the aorta cusps at the level of the sinuses in 67 of 74 dogs with SH and none of 37 dogs with NBP. Of the 74 dogs with SH, 70 had left ventricular hypertrophy (18 asymmetric, 15 concentric, and 37 eccentric). Additionally, there was aortic root dilatation in 15 dogs, aortic insufficiency in 21, mitral regurgitation in 50, and left atrial enlargement in 71. The standard deviation of the three cusp measurements between the dogs with SH and dogs with NBP had a sensitivity of 92% and specificity of 97% for diagnosis of SH. Asymmetric size of the aortic cusps on echocardiography is therefore a reliable indicator of SH in dogs.

BACKGROUND Dilatation of the ascending aorta has an important role in the anatomical conformation of interatrial septum (IAS) especially when a patent foramen ovale (PFO) is present. The aim of the study was to investigate the relationship between ascending aortic dilation and PFO-related cryptogenic stroke in a cohort of cryptogenic strokes. METHODS It is a retrospective, single-center echocardiographic study assessing aortic root dilatation in 315 consecutive patients with cryptogenic stroke between January 2011 and January 2019. Aortic root dilatation was defined by a diameter of the Valsalva sinuses of the proximal aorta >40 mm. Predictive factors of PFO were assessed by a multivariate analysis. Propensity score matching was applied to account for clinical differences. RESULTS Of the 315 patients, 68 (22%) had an aortic root dilatation and 167 (53%) had a PFO. In the aortic root dilation group, PFO was more often diagnosed (n = 47/68 [69%], vs n = 120/247 [49%], P = .004). In the PFO group with aortic dilatation, IAS was more mobile (n = 37/47[79%] vs n = 69/120[57%], P

A case of severe early-onset Marfan syndrome (MFS) led us to ask the question: 'Should an ARB, particularly losartan [intervention], compared with beta blockade alone [comparison], be commenced in all paediatric MFS patients [patient] with aortic root dilatation [outcome]?' The current literature was critically appraised to address this question.

Aim: To assess the effect of beta-blockade therapy on progressive aortic dilatation and on clinical outcome in children and adolescents with Marfan’s syndrome (MFS). Methods: The meta-analysis was instituted, which included studies identified by a systematic review of MEDLINE of peer-reviewed publications. Echocardiogram measurements of the aortic root dimension and outcome measures of mortality and major morbidity were compared between patients who were treated and untreated with beta-blockade therapy. Results: Five studies were included. A total of 224 young patients treated with beta-blocker therapy and 168 patients did not accept medical management. Compared with non-beta-blockade treatment, beta-blockade therapy significantly decreased the rate of aortic dilatation (SMD = −1.30 with 95% CI −2.11 to −0.49). A tendency of clinical outcome beneficial was observed in the beta-blocker treatment group when compared with no beta-blocker treatment group (odds ratio = 0.87 with 95% CI 0.37–2.04). Conclusion: There is evidence that beta-blockade therapy can slow down the rate of dilatation of the aorta and has clinical benefits on children and adolescents with MFS.

Marfan syndrome (MFS) is a connective tissue disorder with major features in cardiovascular, ocular and skeletal systems. Recently, diagnostic criteria were revised where more weight was given to the aortic root dilatation. We applied the revised Marfan nosology in an established adult Marfan population to define practical repercussions of novel criteria for clinical practice and individual patients. Out of 180 MFS patients, in 91% (n = 164) the diagnosis of MFS remained. Out of 16 patients with rejected diagnosis, four patients were diagnosed as MASS (myopia, mitral valve prolapse, borderline non-progressive aortic root dilatation, skeletal findings and striae) phenotype, three as ectopia lentis syndrome and in nine patients no alternative diagnosis was established. In 13 patients, the diagnosis was rejected because the Z-score of the aortic root was

We report on a 22-year-old male carrying a presumptive clinical diagnosis of Dubowitz-like phenotype who has been followed-up by cardiology for bicuspid aortic valve with ascending aorta and aortic root dilatation. Cardiac magnetic resonance imaging (CMRI) confirmed these findings, along with an incidental finding of left ventricular non-compaction (LVNC). Genetic workup revealed the diagnosis of 22q11.2 distal deletion encompassing the BCR gene. This is the first time LVNC has been reported in a patient with 22q11.2 distal deletion. © 2010 Wiley-Liss, Inc.

Shprintzen-Goldberg syndrome is a rare and lethal association of craniosynostosis with features of Marfan syndrome. 1 It has been traced to a defect of chromosome 15. 2 Cardiac features include progressive aortic root dilatation. To our knowledge, there is no known association of this syndrome with congenital heart defects.

Background Aortic root dilatation may result from the embryologic maldevelopment that causes Tetralogy of Fallot (TET). It could also relate to haemodynamic factors since the aorta carries a larger fraction of the combined cardiac output. Methods Retrospective data analysis and review of echocardiographic, catheterisation and follow up data on children who underwent TET repair between 1985 and 1990. The tirst part of the study focused on impact of arteriopulmonary shunts on aortic root size. The second part of the study focused on the comparative aortic root sizes in children who had been repaired in infancy as against those who had been repaired postinfancy. Results In the first part of the study, there were 147 infants at ages 3.4 f 2.9 months with complete M-Mode echo studies. Their aortic annulus was significantly larger than expected for age. Catheterisation data was available in 54 patients in whom systemic saturation did not correlate with aortic root diameter on echo studies done within a 3month period. There were 46 infants who underwent palliative arteriopulmonary shunts with a further important increase in aortic annular dimension. The second part of the study compared aortic root size in those who had been repaired before 1 years of age (n = 43) and after 1 years of age (n = 385) with a follow up between 1 and 35 years. In cases that were corrected in infancy, the aortic annulus size approached upper limit of normal by 6 years of age whereas aortic annular dilatation persisted in those corrected post infancy. Conclusion The study was unable to differentiate the relative contributions of maldevelopment and haemodynamic factors in aortic root dilatation in TET. Aortic root dilatation persists in patients repaired beyond infancy, and seems to ‘regress’ in those operated in infancy