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Pulmonary Vascular Disease |

Unraveling the Mystery of Pulmonary Hypertension in a Young Female FREE TO VIEW

Hayas Haseer Koya, MD; Dona Varghese, MD; Dinesh Ananthan, MD; Dinesh John, MD; Tanya George, MD
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SUNY Upstate Medical University, Syracuse, NY


Chest. 2014;146(4_MeetingAbstracts):892A. doi:10.1378/chest.1968180
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Abstract

SESSION TITLE: Pulmonary Vascular Disease Student/Resident Case Report Posters II

SESSION TYPE: Medical Student/Resident Case Report

PRESENTED ON: Tuesday, October 28, 2014 at 01:30 PM - 02:30 PM

INTRODUCTION: Sinus of Valsalva aneurysm(SVA) is an extremely rare cardiac anomaly (dilatation of aortic wall located between aortic valve and sinotubular junction due to lack of continuity of middle layer of aortic wall and aortic valve). Rupture of sinus of Valsalva(RSOV) is a dreaded complication which has varying presentations. We present a case of rupture of sinus of Valsalva to right atrium

CASE PRESENTATION: A 37 year old female with past medical history of velocardiofacial syndrome(autosomal dominant disorder due to deletion in chromosome 22q11 and characterized by conotruncal heart defects, velopharyngeal insufficiency, immune defects, and hypoparathyroidism with hypocalcemia)presented with exertional shortness of breath and occasional retrosternal chest pain of one month duration. On evaluation she had hypoxia, tachypnea and a continuous murmur with maximal intensity over left lower sternal border. EKG revealed normal sinus rhythm and mild left atrial enlargement.Transthoracic echocardiogram revealed ejection fraction(EF) of 50-55%, severe tricuspid regurgitation and severe pulmonary hypertension. Cardiac catheterization with aortogram revealed SVA at the right aortic cusp and RSOV into right atrium. A diagnosis of pulmonary hypertension due to RSOV to right atrium was made and patient underwent successful surgical repair of RSOV.

DISCUSSION: SVA is a very rare cardiac anomaly with a prevalence of less than 1% and can be congenital(most common) or acquired(due to infective endocarditis, trauma, connective tissue disorders,atherosclerosis). In cases with congenital etiology,there is deficiency of elastic and muscular tissue at the junction of aortic media and annulus fibrosus of the aortic valve. Common complications of SVA include rupture, local compression, aortic regurgitation. RSOV occurs more commonly in males(mean age of 34 years). Majority of SVA’s arise from right coronary sinus and rupture into right ventricle, or rarely to right atrium. Common presentations of SVA include acute heart failure due to RSOV to cardiac chamber, acute coronary syndrome, cardiac tamponade, heart block and aortic regurgitation. The most common diagnostic tool is echocardiogram and the gold standard for diagnosis is cardiac catheterization with aortography. Additional imaging tools include MRI and CT angiography. Treatment of RSOV is by surgical repair- simple suture or patch closure and even percutanoeus closure in anatomically suitable cases. RSOV if left untreated has a median survival of one to two years and after surgical repair 10 year survival rate is 90%.

CONCLUSIONS: We reiterate the need for considering RSOV among the differential diagnoses in young and middle aged patients who present with symptoms of pulmonary hypertension, acute heart failure or new murmurs.

Reference #1: Arıkan E, Karagöz A, Bayata S, Yilik L, Unlüer EE. A rare cause of dyspnea: sudden rupture of aortic valsalva sinus aneurysm. Case Rep Med. 2013;2013:909302. doi: 10.1155/2013/909302. .

DISCLOSURE: The following authors have nothing to disclose: Hayas Haseer Koya, Dona Varghese, Dinesh Ananthan, Dinesh John, Tanya George

No Product/Research Disclosure Information


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