Familial unruptured sinus of Valsalva aneurysm obstructing the right ventricular outflow tract

Abstract

A neurysm of the coronary sinus of Valsalva (ASV) is rare. It may be congenital, sometimes in association with other cardiac defects (most commonly ventricular septal defect or coarctation), or it may be acquired. The first manifestation of ASV is most often related to rupture, whereas unruptured ASVs are rare. Although unruptured aneurysms are frequently asymptomatic without physical findings, they can be associated with complications and can compress adjacent structures. We present a case of a familial unruptured right ASV causing obstruction of the right ventricular outflow tract (RVOT) and subsequent evolution following repair. CASE PRESENTATIONA 77-year-old woman presented with stable dyspnea on exertion (New York Heart Association [NYHA] class III), which she had been experiencing for the past year. Her medical history revealed only hypothyroidism and hysterectomy with bilateral salpingooophorectomy. A transthoracic echocardiography performed five years earlier was normal except for a mild increase in peak transvalvular pulmonary velocities (1.3 m/s). Examination revealed normal blood pressure and a systolic heart murmur (grade 3/6) best heard along the left sternal border. Further examination showed distension of the jugular veins and mild ankle edema. Recorded electrocardiogram alterations were a mild Q wave with inversion of the T wave in inferior leads. Computed tomography angiography of the chest showed no pulmonary embolism but revealed moderate proximal thoracic aorta ectasia. Pulmonary function tests were normal. Controlled transthoracic echocardiography (Figure 1) showed a right ASV and RVOT obstruction with a subvalvular pulmonary systolic gradient of 45 mmHg. Left ventricular contractility was normal on echocardiography with moderate aortic regurgitation (grade 3/4). Aortic angiography (Figure 2) confirmed a right ASV while cardiac catheterization confirmed RVOT obstruction with no shunt. Coronary angiography was normal. The patient was unable to undergo magnetic resonance imaging due to claustrophobia.The decision was made to repair the aneurysm because the patient's symptoms were likely related to RVOT obstruction and there was evidence of aneurysm progression on serial echocardiography. It was also known from the pathology report that the patient's younger brother had died 24 years earlier at 48 years of age from a ruptured ASV. The ASV was repaired with a pericardial patch. The aortic valve was also replaced with a Carpentier-Edwards porcine case report ©2009 Pulsus Group Inc. All rights reserved A case demonstrating compression of the right ventricular outflow tract by an unruptured coronary sinus of Valsalva aneurysm in which repair resulted in symptomatic improvement is presented. The pathology report revealed that the patient's younger brother had died from a ruptured aneurysm of the coronary sinus of Valsalva. The present report is the first to describe a familial unruptured coronary sinus of Valsalva aneurysm raising questions regarding the screening of relatives of patients with sinus...

Unruptured Left Coronary Sinus of Valsalva Aneurysm Causing Right Ventricular Outflow Tract Obstruction Following Coronary Re-Implantation Surgery: Case Report

“…The diagnosis of aneurysm of the sinus of Valsalva with RVOT obstruction was suspected only during a routine follow up echocardiography for the previous ALCAPA repair. There are multiple reports from the adult patient population describing aneurysm of sinus of Valsalva causing RVOT obstruction (8,9,11).As our case exemplifies, RVOT obstruction may be one of the manifestations of unruptured sinus of Valsalva aneurysm.…”

“…The clinical manifestations of these aneurysms are related to their intrinsic mass effect on the adjacent cardiac structures. The clinical consequences of the mass effect in the nearby structures may be in the form of right ventricular outflow tract obstruction, heart block and coronary artery occlusion resulting in myocardial ischemia (4,(6)(7)(8)(9)(10)(11).In adults, unruptured sinus of Valsalva aneurysms may present with chest pain and dyspnea on exertion (8,9). Interestingly, Sundraum, et al from southern India, reported unruptured sinus of Valsalva aneurysm in a patient with inflammatory aortitis (12).…”

Pulmonary valve endocarditis associated with a ruptured aneurysm of the right sinus of Valsalva

“…Nishida et al [2] reported a case of a pulmonary valve endocarditis caused by the RVOT obstruction in association with aneurysm of the sinus of Valsalva. Aneurysm of the right sinus of Valsalva can protrude into the RVOT and cause obstruction [2][3][4]. The high-velocity jet created by the RVOT obstruction is usually responsible for the endothelial damage of the pulmonary valve and predisposes to infective endocarditis [2].…”

Sinus of Valsalva Aneurysm in Females

“…[2][3][4] However, the genetic predisposition of SVA is yet to be elucidated, as familial SVA is extremely rare. 5) Recently, an association with aortic aneurysm and SVA has been revealed in periventricular nodular heterotopia (PVNH) patients with loss-of-function Filamin A (FLNA) mutations, which were located on chromosome X and almost exclusively affect females. 6) Therefore, the present report reviewed an electronic case database of patients hospitalized for aortic surgery with aortic root diameter over 4.0 cm who have been referred for genetic testing in our institution.…”