Case Presentation: We describe a 59-year-old male treated for a ruptured sinus of Valsalva aneurysm (SVA) causing multiorgan failure. The patient presented to the emergency department (ED) with acute chest pain, dyspnea, palpitations, nausea, weakness, and diaphoresis after lifting heavy boxes a day prior. The ED evaluation revealed sinus tachycardia, tachypnea, elevated troponin, acute kidney injury (AKI), mild lactic acidosis, leukocytosis, normal liver function test, low D-dimer, and unremarkable chest X-ray. Acute coronary syndrome was ruled out by cardiology, and the patient was admitted to hospital medicine for infection evaluation. On day 1, the patient was noted to have a holosystolic murmur, an a transthoracic echocardiogram (TTE) showed non-specific mild aortic root dilation. On day 3, the patient had persistent sinus tachycardia and worsening lactic acidosis and AKI despite IV fluids, so thyroid and adrenal workups were sent. On day 4, the patient developed acute liver failure, worsening renal failure, severe lactic acidosis, disseminated intravascular coagulopathy, thrombocytopenia, hyponatremia and was transferred to the medical ICU, where he had further hepatobiliary, infectious, autoimmune, and hematologic workups with no clear etiology. On day 5, the patient was transferred to another institution for liver transplant evaluation. A repeat TTE showed new right ventricle (RV) failure and possible ventricular septal defect (VSD). A transesophageal echocardiogram (TEE) revealed a ruptured SVA in the right atrium causing a left-to-right shunt. The patient underwent urgent surgical repair with a Dacron patch, and he improved clinically.
Discussion: A SVA is a rare cardiac malformation caused by connective tissue weakness leading to dilation of the aortic root around the aortic valve, and it can be due to congenital conditions, vasculitides, connective tissue diseases, infections or mechanical (trauma or surgery). SVA are usually found incidentally upon evaluation of other conditions. Strenuous activities that increase intrathoracic pressure such as heavy lifting or physical sports increase the risk for a SVA to rupture. A ruptured SVA requires urgent surgical repair or transcatheter closure. However, a ruptured SVA has a poor prognosis and high mortality since it usually goes unrecognized. The patient’s presentation of chest pain, dyspnea, tachycardia is typical of SVA rupture, but it is non-specific. In addition, this clinical presentation is more typical for admission to general medicine rather than a surgical admission. We stipulate this patient had an asymptomatic congenital SVA, and the patient’s occupation of lifting heavy boxes led to the SVA rupture due to an increase in intrathoracic pressure. Although the admitting hospitalist noted a holosystolic murmur (which is a sign of SVA rupture), the patient’s initial TTE only showed a non-specific mild aortic root dilation. Thus, the ruptured SVA was difficult to diagnose initially, and it led to rapid multiorgan failure without a clear etiology. This patient’s outcome turned favorably once a repeat TTE showed progression of RV failure and possible VSD and the TEE showed a ruptured SVA that was urgently repaired.
Conclusions: This case highlights how rare and elusive a ruptured SVA is for clinicians to diagnose. This case highlights the importance for hospitalists and general practitioners to be educated and considered ruptured SVA when patients present similarly to this patient. Otherwise, a ruptured SVA will continue to be fatal.