Pathogenetic Significance of Myxomatous Degeneration in Fenestration-Related Massive Aortic Regurgitation

Abstract

enestration of the aortic valve is not an uncommon malformation, and appears as an oval hole immediately below the free edge of the cusp near its commissural attachment. Fenestration of the aortic cusp is not considered to have any pathological significance, and is rarely associated with aortic regurgitation, but we are operating on an increasing number of cases of massive aortic regurgitation in which large fenestrations play an important role in the pathogenesis of regurgitation. We present 6 cases in which the clinicopathological findings were analyzed. Methods PatientsDuring the past 9 years at Iwaki Kyoritsu General Hospital, more than 200 aortic valves have been replaced because of aortic regurgitation caused by idiopathic annular dilatation, infected endocarditis, rheumatic valvular disease or congenital bicuspid valve. Of these patients, 6 cases with large aortic valve fenestrations (Fig 1), which played an important role in producing massive aortic regurgitation, were evaluated from the clinical and pathological viewpoints. None of them showed systemic connective tissue disease or annuloaortic ectasia. The interval since onset was defined as the time span to surgical treatment from the appearance of new aortic regurgitant murmur, the Circulation Journal Vol.68, May 2004first experience of cardiac symptoms or echocardiographic detection of aortic regurgitation. Left ventricular hypertrophy was defined electrocardiographically by the QRS voltage criteria including RI + SIII ≥2.5 mV, R in aVf >2.0 mV, S in V1 ≥2.4 mV, R in V5 or V6 >2.6 mV, and R in V5 or V6 + S in V1 >3.5 mV. The aortic annular diameter (AAD), left ventricular diastolic dimension (LVDd), and left ventricular systolic dimension (LVDs) were measured by 2-dimensional echocardiography in the parasternal long-axis view. The severity of the aortic regurgitation was graded as trivial (I), mild (II), moderate (III) or severe (IV) using color flow mapping in the apical view of transthorac-

Acute Valvular Regurgitation

“…5 Another less common cause is rupture of a fenestration in the aortic leaflet. 6 Acute mitral regurgitation may result from either "organic" or "functional" causes. Organic causes are those that result in permanent structural disruption of the valve, such as leaflet perforation from endocarditis, chordal rupture in myxomatous valve disease, or papillary muscle rupture due to myocardial infarction.…”

Aortic Regurgitation Due to Fibrous Strand Rupture in the Fenestrated Left Coronary Cusp of the Tricuspid Aortic Valve

“…1) These reports have mostly been from Japan and other Asian countries 1,3,4) suggesting that some form of heredity involvement may exist with regard to fibrous strand formation. 4) In the present case, fibrous strands supported the aortic valve and maintained coaptation of the valve (Figure 3).…”

“…4) Transthoracic echocardiography and transesophageal echocardiography are reported to be useful for the detection of fibrous strand rupture. 1) In the absence of any other obvious cause, physicians must consider the possibility of a fenestrated aortic valve in patients with chronic AR or sudden deterioration of regurgitation.…”

“…Fibrous strand rupture causes acute aortic regurgitation (AR) and has been reported to occur in the congenital bicuspid valve or in the right coronary cusp of the tricuspid valves. 1) Here we report the case of a patient with a fenestrated tricuspid aortic valve with a fibrous strand. The patient had a history of hypertension and suffered from acute AR due to fibrous strand rupture in the left coronary cusp.…”

Estudo biomolecular de produtos de Chlamydophila pneumoniae, Mycoplasma pneumoniae e Borrelia burgdorferi na etiopatogenia da degeneração mixomatosa da valva mitral

“…(estágio II, moderado) do grupo 2 (p = 0,013) [69]. e participando, portanto, do processo inflamatório desencadeado pela degeneração mixomatosa.…”