Nosology Spectrum of the Bicuspid Aortic Valve Condition: Complex-Presentation Valvulo-Aortopathy.
Nosology Spectrum of the Bicuspid Aortic Valve Condition: Complex-Presentation Valvulo-Aortopathy.
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DOI：
10.1161/circulationaha.120.046892
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发表时间：
2020-07-21
期刊：
影响因子：
37.8
通讯作者：
Prakash, Siddharth K.
中科院分区：
文献类型：
--
作者：
Michelena, Hector I.;Vallabhajosyula, Saarwaani;Prakash, Siddharth K.
Patient presentation: A 24-year-old man, who smoked 1 pack a day of cigarettes, presented to the emergency department with sudden onset of 7/10 chest pain radiating to the back. His medical history was significant for anxiety and depression and a bicuspid aortic valve (BAV) diagnosed at age 5 years because of an auscultatory click. Outside transthoracic echocardiogram 3 years previously had shown normal valve function and aortic sinuses (root) of 42 mm. His family history was notable for a mother and uncle with BAV and aortic dilatation, but there was no early sudden cardiac death or aortic dissection in the family. The patient denied illicit drug use. On examination, he was in mild distress from chest pain. His heart rate was 75 beats/minute, his blood pressure was 124/72 mm Hg and equal in both arms, and room air oxygen saturation was 96%. Physical examination was unremarkable with normal cardiac auscultation. The ECG showed an early repolarization pattern and troponins were undetectable. A triplerule-out computed tomography angiogram (CTA) showed no evidence of pulmonary embolus, coronary disease, or aortic dissection. The aortic root measured 48 mm with asymmetric noncoronary sinus dilatation (Figure 1) and no aortic coarctation. The remainder of the thoracic aorta was normal. After resolution of chest pain had occurred with analgesics administration, the patient was discharged and scheduled for outpatient cardiology follow-up in 2 weeks. The cause of his chest pain remained unknown.Dr Michelena: The congenital BAV condition affects 1% to 2% of the population and is fundamentally a valvulo-aortopathy characterized by substantial heterogeneity of its valvular and aortic phenotypic expressions, associated disorders, complications, and prognosis. 1 From a nosology perspective, the most common presentation of BAV is typical-presentation valvulo-aortopathy with progressive BAV dysfunction and/or aorta dilatation (the clinical manifestation of aortopathy). This typical-presentation valvulo-aortopathy commonly is diagnosed in young adults and adults, requires long-term surveillance, and commonly necessitates surgical treatment, particularly for aortic valve dysfunction (> 50% of patients need aortic valve replacement within 25 years after BAV diagnosis). 1 The 24-year-old patient seemed to present in a typical manner, with a known nondysfunctional BAV with predominance of aortopathy manifested as a root of 48 mm measured by ECG-gated CTA, the gold standard for measuring the diameter of the aorta. 2 There are 2 general BAV aortopathy phenotypes depending on which aortic segment is predominantly dilated: the ascending phenotype and the root phenotype, affecting≈ 70% and
