167: AN ADOLESCENT MALE WITH CONGESTIVE HEART FAILURE FROM A RARE CAUSE

Abstract

Introduction: A sinus of Valsalva aneurysm is an abnormal dilatation of the aortic root located between the aortic valve annulus and the sinotubular junction. It can either be congenital or acquired. The right coronary sinus is most commonly involved, followed by the noncoronary sinus. Rupture of the sinus of Valsalva aneurysm is extremely rare in children. Description: A 16-year-old previously healthy male was admitted with 1 month of worsening fatigue, bilateral leg swelling and orthopnea. He was hypertensive and tachycardic with a grade 3 holosystolic murmur over the precordium. The rest of his physical examination was normal. A chest x-ray showed cardiomegaly and an EKG showed ventricular bigeminy and inverted T waves. His pro-BNP was elevated to 1880 pg/mL but troponin and inflammatory markers were normal. A transthoracic echocardiogram (TTE) showed a ruptured aortic noncoronary sinus of Valsalva aneurysm into the right atrium with left to right shunting, aortic root dilatation and aortic valve regurgitation. A cardiac CT confirmed the TTE findings. He underwent surgical repair. Intraoperatively, a large fenestrated aneurysmal tissue was noted and was excised, with the defect repaired using two layers of autologous pericardial patches. A transesophageal echocardiogram (TEE) showed no residual defect and no aortic insufficiency. He was extubated the next day, but postoperative course was complicated by an accelerated junctional rhythm with ventricular bigeminy that was successfully treated with an esmolol infusion and later transitioned to atenolol. Discussion: Sinus of Valsalva aneurysm is present only in 0.09% of the general population. The mean age of diagnosis is between 30-45 years, and it is usually asymptomatic unless it ruptures. The right ventricle followed by the right atrium are the most common locations of the rupture. Cardiac catheterization and angiography is the gold standard for diagnosis but a TTE is the first line. Cardiac CT and MRI are supplemental tests. Ruptured aneurysms or unruptured symptomatic aneurysms indicate surgical repair. With the onset of heart failure symptoms, arrhythmias and aortic insufficiency diagnosed on a TTE, a surgical repair with patch closure was opted for our patient with a good postoperative outcome.