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Acute High-Output Failure from an Aortoventricular Fistula Due to a Ruptured Sinus of Valsalva Aneurysm After Blunt Chest Trauma

Muehlschlegel, Jochen D. MD*; Alomar-Melero, Estibaliz MD*; Staples, Edward D. MD; Janelle, Gregory M. MD*

doi: 10.1213/01.ane.0000242526.04908.2e
Cardiovascular Anesthesia: Echo Didactics & Rounds
Video 1

From the *Departments of Anesthesiology and †Surgery, University of Florida College of Medicine, Gainesville, Florida.

Accepted for publication August 8, 2006.

Presented in poster format at the 28th Annual Society of Cardiovascular Anesthesia Meeting and Workshops, April 29–May 3, 2006, San Diego, CA.

Address correspondence to Jochen D. Muehlschlegel, MD, Department of Anesthesiology, University of Florida College of Medicine, PO Box 100254, 1600 SW Archer Road, Gainesville, FL 32610-0254. Address e-mail to danny@muehlsch.de. Reprints will not be available from the author.

This article has supplementary material on the Web site: www.anesthesia-analgesia.org.

A previously healthy, 18-yr-old Caucasian male without a known medical history of connective tissue disease was transferred to our institution from an outside hospital with fever and heart failure 3 wk after being involved in an all-terrain vehicle accident. A transesophageal echocardiogram (TEE) revealed a small subpulmonic ventricular septal defect (VSD), as well as a small atrial septal defect (ASD) (please see video loop available at www.anesthesia-analgesia.org), a ruptured right sinus of Valsalva aneurysm with aortic regurgitation into the right ventricle (Figs. 1a and 1b; also please see video loop available at www.anesthesia-analgesia.org), and normal biventricular systolic function. When questioned, the patient's family remembered having been told that, as a child, the patient had an unimportant “extra hole” in his heart. Right and left heart catheterization showed a 3:1 pulmonary-to-systemic shunt ratio. The patient was scheduled for emergency repair. Intraoperative TEE confirmed the preoperative findings. Continuous wave Doppler across the VSD demonstrated a measured gradient of 80 mm Hg between the aorta and the right ventricle during systole. The VSD and aortopulmonary connection were explored and revealed a thinning of the right coronary sinus, creating a windsock into a subpulmonic VSD. The end of this windsock was opened and ruptured (Fig. 2). It was thus confirmed that this patient had a preexisting subpulmonic VSD which was partially obstructed by the windsock from a right coronary sinus of Valsalva aneurysm. His systolic pulmonary artery pressures decreased from 33 mm Hg before bypass to 23 mm Hg after bypass. On follow-up, transthoracic echocardiography demonstrated the absence of a VSD. The patient had no further events and was discharged on postoperative day 20.

Figure 1

Figure 1

Figure 2

Figure 2

A weakness in the aortic intima at the junction of the annulus fibrosis is thought to be the etiology of congenital sinus of Valsalva aneurysms. Congenital sinus of Valsalva aneurysms are more prevalent in Middle Eastern countries, with a male predominance of 4:1. These aneurysms occur at 70% in the right coronary sinus and are associated with other cardiac anomalies such as a VSD, ASD, bicuspid aortic valve, or persistent ductus arteriosus. Ruptured aneurysms cause acute symptoms in only one-third of patients. One-third gradually develop symptoms of right ven-tricular volume overload, and one-third remain asymptomatic (1). The right coronary sinus usually ruptures into the right ventricle, whereas a left coronary sinus aneurysm tends to rupture into the right atrium (2). Structures of interest that need to be examined in detail by TEE are the aortic root with the sinuses of Valsalva, the left ventricular outflow tract, the ascending aorta, and the atrial and ventricular septum. Useful views include the midesophageal aortic valve short axis, and long axis, the transgastric long axis, and the deep transgastric long axis. Continuous wave Doppler can delineate the difference between high-velocity gradients between chambers and lower velocity gradients from valvular regurgitation. Injection of agitated saline may demonstrate a negative contrast effect in the regurgitant chamber.

There are several reports of sinus of Valsalva aneurysms and VSDs after blunt trauma (3), but no reports of blunt traumatic sinus of Valsalva aneurysms rupturing into the right ventricle and causing acute right ventricular overload are there. It is conceivable that in our patient, the windsock aneurysm of the sinus of Valsalva aneurysm eroded into the right ventricular septum, causing a VSD; however, in light of the patient's associated anomaly of an ASD, we hypothesize that a preexisting VSD predisposed the sinus of Valsalva aneurysm to rupture into the right ventricle, causing a high-flow fistula, all of which led to the patient's pulmonary edema and heart failure symptoms.

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REFERENCES

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2. Shah RP, Ding ZP, Ng AS, Quek SS. A ten-year review of ruptured sinus of valsalva: clinico-pathological and echo-Doppler features. Singapore Med J 2001;42:473–6.
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