Disclosure: The authors report no conflicts of interest regarding the content herein.

Donna Gagne, RCIS, can be contacted at dgagne3@maine.rr.com.

Case

The patient is a 73-year-old male with a history of congestive heart failure, chronic kidney disease, atrial fibrillation, coronary artery disease status post coronary artery bypass graft (CABG) x 3 in 2009, and percutaneous coronary intervention (PCI) x 2 in 2012. The patient presented to his outpatient clinic with progressive dyspnea on exertion, despite an increase in his diuretic regimen. 

The patient was admitted to the Maine Medical Center cardiology department and scheduled for a right heart catheterization with a diagnosis of cardiorenal syndrome. A right heart catheterization was performed via the right cephalic vein using a 6 French (Fr) Berman catheter (AngioCare BV). The findings of the right heart catheterization were as follows: 

• Oxygen saturations: aortic root (AO) 99%, right atrium (RA) high 51%, RA mid 69.4%, RA low 51%, right ventricle (RV) 72.9%, pulmonary artery (PA) 76.5%, IVC 66%, SVC 46%, pulmonary vein (PV) 99% (assumed); 
• Right heart pressures: AO 123/61/107, RA 25/32/25, RV 62/8/24, PA 59/27/41, pulmonary capillary wedge (PCW) 35/45/28;
• Calculated Fick cardiac output (CO) and cardiac index (CI) were 7.75L/min and 3.4L/min/m², respectively. 

A transthoracic echocardiogram was performed with findings of a ruptured sinus of Valsalva aneurysm of the right coronary sinus that had fistulized into the right atrium and was associated with a left-to-right shunt of approximately 2:1 (Figure 1). The patient was scheduled for a coronary angiogram to evaluate native coronaries and bypass grafts. A cardiothoracic surgeon was consulted and the patient underwent a redo sternotomy, division of aorta to right atrial fistula with patch closure of right atrium using glutaraldehyde bovine pericardial patch, and aortic root replacement with a 27mm Medtronic Freestyle porcine root. The patient was subsequently discharged and followed up in the outpatient clinic.

Discussion

The three bulges of the aortic wall are named the sinuses of Valsalva, after the Italian anatomist Antonio Valsalva (1740). Two of the three sinuses host the origin of the coronary arteries and the coronaries are named accordingly. The precise function of the sinuses of Valsava is unclear. There is evidence that the vortices created in the sinuses lead to stress reduction on the aortic leaflets and support coronary flow.¹ The primary cause of a sinus of Valsalva aneurysm are congenital. Associated structural defects in congenital sinus of Valsava aneurysms include ventricular septal defect (30-60%), bicuspid aortic valve (15-20%), and aortic regurgitation (44-50%).² Secondary causes include atherosclerosis, syphilis, Marfan syndrome, blunt or penetrating chest injury, and endocarditis. Sixty-five percent to 85% of sinus of Valsava aneurysms originate in the right sinus.³ Rupture of the dilated sinus may lead to intra-cardiac shunting when a communication is established with the right atrium or right ventricle. Rupture of the left coronary sinus into the left ventricle can masquerade as severe aortic insufficiency. Cardiac tamponade may occur if the rupture involves the pericardial space. With corrective surgery, there is a 94% survival rate at 10 years.⁴

A ruptured sinus of Valsalva is a rare cardiac anomaly. We are happy to report that this patient made a full recovery from his sinus of Valsalva aneurysm rupture. This patient’s diagnosis was made by recognizing abnormal oxygen saturations during a right heart catheterization shunt run and follow-up transthoracic echocardiogram using color Doppler. This case highlights the importance of measuring a superior vena cava (SVC) (or SVC + inferior vena cava) saturation during a right heart catheterization, as measurement of only the mid RA (69.4%) and PA (76.5) might have come close to missing the shunt.

Special thanks to Dr. Louis Coyle and Dr. Samip Vasaiwala for their assistance in writing this article.

References

1. Charitos EI, Sievers HH. Anatomy of the aortic root: implications for valve-sparing surgery. Ann Cardiothorac Surg. 2013 Jan; 2(1): 53-56. doi: 10.3978/j.issn.2225-319X.2012.11.18.
2. Tunuguntla A. Sinus of Valsalva Aneurysm. Available online at  https://emedicine.medscape.com/article/158160-overview. Accessed October 27, 2014.
3. Goldberg N, Krasnow N. Sinus of Valsalva aneurysms. Clin Cardiol. 1990 Dec; (12): 831-836.
4. Yan F, Huo Q, Qiao J, Murat V, Ma SF. Surgery for sinus of valsalva aneurysm: 27-year experience with 100 patients. Asian Cardiovasc Thorac Ann. 2008 Oct; 16(5): 361-365.