The association of bicuspid aortic valve with aortic stenosis, aortic regurgitation, and infective endocarditis has been known for almost 150 years and with dissection of the aorta for 75 years.1These complications are mentioned only briefly in cardiology textbooks3 and a standard work on medical insurance underwriting4 implies that it is usually a benign lesion: when diagnosed at routine medical examination and if the valve is functionally normal, insurance is offered at ordinary rates. The authors also state “If there is no stenosis by age 20 years (defined as a peak gradient of less than 40 mm Hg) and only trivial insufficiency, there is an excellent chance that significant haemodynamic change will not develop before age 70.” However, on the basis of published reports by this age the majority of patients will have died or developed serious symptoms requiring surgical intervention. This review summarises the major studies that provide information on the incidence, pathology, and natural history of the bicuspid aortic valve. Clinical diagnosis of this condition was unsatisfactory before the widespread use of cross sectional echocardiography approximately 15 years ago. Most of the information in this paper therefore comes from large necropsy series and from reports on patients who have undergone aortic valve replacement. It has to be accepted that conclusions based on these reports may be inaccurate, because of non-uniformity of diagnostic criteria and varying age range, source/selection of patients studied, and study objectives.

A small percentage of malfunctioning congenitally abnormal aortic valves are unicuspid or unicommissural,5 but as most reports do not distinguish this from bicuspid aortic valve the abbreviation “bicuspid aortic valve” will used to include both types of valve unless stated otherwise.

Early pathology studies1 6 documented three characteristics of bicuspid aortic valve: inequality of cusp size, the presence of …

https://heart.bmj.com/content/heartjnl/83/1/81.full.pdf

Clinical significance of the bicuspid aortic valve

Acute aortic dissection is the most common fatal condition that involves the aorta; nevertheless, despite major advances in noninvasive diagnosis, the correct antemortem diagnosis is made in less than half the cases. To promote continued improvement in the prompt recognition of aortic dissection, we present a review of the Mayo Clinic experience with 235 patients who had 236 substantiated aortic dissections. At the time of initial assessment, 158 patients (67%) had acute and 78 patients (33%) had chronic aortic dissection. Hypertension was the most common predisposing factor (78% of patients overall). The acute onset of severe chest pain was the most common initial complaint (74%), but 33 patients (15%) had painless aortic dissection and abnormal chest roentgenographic findings. Less common manifestations included congestive heart failure, syncope, cerebrovascular accident, shock, paraplegia, and lower extremity ischemia. The initial clinical impression was aortic dissection in 62% of patients overall. In 17 patients (28%), the correct diagnosis was not made before postmortem examination. Although the clinical features of aortic dissection have gained wider appreciation, the diagnosis still remains unsuspected in a substantial number of patients. In a patient who has a catastrophic illness and unexplained symptoms that could be of vascular origin, especially in the presence of chest pain, aortic dissection should always be included in the differential diagnosis.

https://www.mayoclinicproceedings.org/article/S0025-6196(12)60599-0/pdf

Clinical Features and Differential Diagnosis of Aortic Dissection: Experience With 236 Cases (1980 Through 1990)

Pathogenesis of dissecting aneurysm of the aorta.

A review of the history, etiology, pathophysiology, classification, clinical history, physical findings, and laboratory, electrocardiographic and roentogenographic criteria for aortic dissections and dissecting aneurysms is presented. The indications and modes of therapy are discussed as they are being developed and currently practiced. At present, continuation of drug therapy is favored in patients with uncomplicated type III acute or chronic dissections and in those with severe general contraindications to surgery. However, urgent operative treatment is now reemerging as the procedure of choice for patients with type I acute dissections or complicated type III dissections, as well as for patients who are normotensive. Stricter criteria for continuation of drug therapy and for surgery have been developed on the basis of recent reports and our own experience.

Aortic dissections and dissecting aneurysms.

Background Turner syndrome (gonadal dysgenesis with sex chromosome abnormalities) is recognized to be a disorder in which cardiovascular malformations are common. The prevalence and natural history of these findings, the risk for aortic dissection, and the occurrence of cardiovascular disease have all been the subject of debate, as have been the American Academy of Pediatrics recommendations for cardiac screening of patients with Turner syndrome. Objective To evaluate a large population of patients both cross-sectionally and longitudinally to determine the prevalence of cardiovascular malformations, the risk for dissection of the aorta, to determine whether there are phenotype:karyotype correlations that can allow for specific recommendations, and to devise an appropriate screening protocol. Design and methods Data have been collected for patients with Turner syndrome. These individuals have been seen in an ongoing clinic established for the study of the natural history of Turner syndrome. Data from physical examinations, evaluations by cardiologists, echocardiography results, medical and surgical complications, medical records, and causes of death were analyzed. A total of 244 of 462 individuals in this population with karyotype-proven Turner syndrome could be evaluated because echocardiograms had been obtained. In addition, the medical literature was reviewed for occurrences of aortic dissection in patients with Turner syndrome. Results A total of 136 (56%) of 244 of these patients had cardiovascular abnormalities, 96 (71%) were structural, 40 (29%) were functional, including hypertension (HBP), mitral valve prolapse and conduction defects. Coarctation of the aorta and bicuspid aortic valve, alone or in combination, comprised >50% of the cardiac malformations. Bicuspid valve was often not detected by examination, but only by echocardiography. Aortic dissection occurred in three of the patients. In one, it was traumatic; in a second, it occurred at the site of coarctation repair. The third patient had long-standing HBP with malignant obesity. In the literature, there have been 42 case reports of aortic dissection in Turner syndrome. In all except 5, predisposing risk factors of coarctation, bicuspid aortic valve, and/or HBP were present. Of these 5, sufficient information regarding predisposing risk factors was provided for only 2. No phenotype:karyotype correlations could be drawn with any certainty. Conclusions When the diagnosis of Turner syndrome is made, a screening echocardiogram should be obtained. Referral to a cardiologist first may be appropriate, but physical examination does not substitute for visualization. Individuals with and without evidence of structural cardiac malformations should be monitored for HBP on a lifelong basis. In the absence of structural cardiac malformations or HBP, the risk for aortic dissection appears small, and repeated echocardiography or magnetic resonance imaging to follow aortic root diameters does not appear to be warranted based on data currently available. Protocols for following patients with structural malformations need to be individualized, and wholesale recommendations have little merit. A longitudinal study using magnetic resonance imaging or cardiac echocardiography to establish normal parameters for aortic root diameters and to follow aortic root changes is needed.

https://pediatrics.aappublications.org/content/pediatrics/101/1/e11.full.pdf

Cardiovascular Malformations and Complications in Turner Syndrome

Dissecting aneurysm of the aorta; pathological aspects; an analysis of eighty-five fatal cases.

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Dissecting aneurysm of the aorta; pathological aspects; an analysis of eighty-five fatal cases.