Ruptured Sinus of Valsalva Aneurysm in a Pregnant Woman

Abstract

A ruptured sinus of Valsalva aneurysm is a rare cardiac anomaly, usually of a congenital nature. There are few documented cases of this condition during pregnancy, which renders unclear the therapeutic options. We report the case of a 35-year-old pregnant woman who presented at the hospital for cardiac evaluation due to the presence of a heart murmur. The patient was asymptomatic and in her 4th month of pregnancy. Transthoracic echocardiography revealed a ruptured sinus of Valsalva aneurysm and a fistula to the right ventricle with evidence of a continuous left-to-right shunt.

Asinus of Valsalva aneurysm is a rare cardiac anomaly thought to be caused by a deficiency of fusion of the media of the aorta with the annulus fibrosis of the aortic valve, or by actual detachment. Although most of these aneurysms are congenital in origin, they can also be acquired as the result of certain connective tissue diseases, such as Marfan syndrome.¹ These aneurysms usually originate from the right coronary sinus.² They can rupture, most commonly into the right atrium or ventricle, having potentially adverse consequences. This condition often goes unnoticed unless a rupture occurs, usually in the 3rd or 4th decade of life. These patients frequently present with chest pain, palpitations, syncope, or a heart murmur.³

There are few documented cases of a ruptured sinus of Valsalva aneurysm in a pregnant patient.^4,5 We report the case of a 35-year-old woman who was referred during her 4th month of pregnancy for cardiac evaluation due to the presence of a heart murmur. She was found to have a ruptured sinus of Valsalva aneurysm with left-to-right shunting into the right ventricle.

Case Report

In November 2002, a 35-year-old pregnant woman presented for cardiac evaluation due to the presence of a heart murmur. She was asymptomatic and in her 4th month of pregnancy, which had been uncomplicated. The patient was born in Korea and had been told that she had a “defect in her heart,” first detected as a murmur during childhood. She reported that she had undergone several echocardiograms throughout her life and knew that she had a congenital defect, but she did not recall the specific condition.

Upon evaluation at our institution, the patient felt well and did not complain of any shortness of breath, chest pain, palpitations, dizziness, or syncope. There was no history of peripheral edema, orthopnea, or paroxysmal nocturnal dyspnea. The patient's medical history was significant only for 2 uncomplicated pregnancies. Physical examination revealed a well-appearing woman in no distress, with a regular heart rate of 90 beats/min and a blood pressure of 126/70 mmHg. Palpation of the precordium revealed the presence of a prominent systolic and diastolic thrill. Auscultation of the heart was notable for the presence of a loud, harsh grade 4/6 continuous murmur that was audible throughout the precordium. The remainder of the examination was unremarkable. A 12-lead electrocardiogram showed normal sinus rhythm at a rate of 90 beats/min with no evidence of right-heart strain.

Transthoracic echocardiography revealed a thinned, slightly aneurysmal dilatation of the right coronary sinus of Valsalva of the aortic root. Color Doppler interrogation revealed a fistula between the aortic root and the right ventricle with evidence of prominent, continuous flow from the aortic root to the right ventricle (Figs. 1–3). Of note, the right ventricular size and function were normal. The main pulmonary artery was dilated, measuring 2.9 cm in diameter. Despite the slight dilatation of the right coronary sinus of Valsalva, the aortic root was normal in caliber. The left ventricular systolic function was normal. There was mild tricuspid, mitral, and aortic regurgitation. In addition, there was evidence of mild pulmonary hypertension, with an estimated pulmonary artery systolic pressure of 45 mmHg. An attempt was made to quantify the magnitude of the left-to-right shunt; however, this was likely overestimated due to the large diameter of the right ventricular outflow tract, which could have been the result of normal dilation of the pulmonary artery during pregnancy. Subsequent echocardiograms performed during the patient's 6th and 9th months of pregnancy showed similar findings. Although her echocardiographic findings indicated the presence of a significant shunt, and surgical options for repair were advised, the patient declined surgical intervention. In addition to undergoing serial echocardiograms at 6 and 9 months, the patient was monitored closely at our cardiology clinic throughout the remainder of her pregnancy and had an uncomplicated vaginal delivery at full term.

Fig. 1 Transthoracic echocardiogram in parasternal long-axis view with color-flow Doppler shows flow from the aortic root into the right ventricular outflow tract through the ruptured sinus of Valsalva aneurysm (arrow). Ao = aortic root; LVOT = left ventricular outflow tract; RVOT = right ventricular outflow tract

Fig. 2 Transthoracic echocardiogram in apical 5-chamber view reveals the aneurysmal right sinus of Valsalva (arrow).AoV = aortic valve; LV = left ventricle; RV = right ventricle

Fig. 3 Transthoracic echocardiogram in apical 3-chamber view shows the aneurysmal right sinus of Valsalva (arrow). LA = left atrium; LV = left ventricle

Discussion

Sinus of Valsalva aneurysm makes up less than 1% of all congenital cardiac anomalies according to some reports, but it occurs at higher rates in patients of Asian origin.^6–8 The disease occurs more frequently in males and has a wide clinical spectrum.³ The aneurysm can remain intact or can rupture, thereby causing symptoms that can be associated with detrimental effects. Even unruptured, the aneurysm can occasionally obstruct cardiac outflow or cause heart block. Although there are not many data on the natural progression of this disease, it is clear that aneurysmal rupture can result in sudden death as a consequence of the acute onset of congestive heart failure, arrhythmia, cardiac tamponade, or ischemia. Endocarditis is also common.3,6,9,10 In addition, there have been several reports of a sinus of Valsalva aneurysm as the source of an embolic stroke.¹¹ A recent report indicated that echocardiography is a very accurate and reliable tool in diagnosing a sinus of Valsalva aneurysm when correlated with surgical findings.¹² In addition, several reports have shown the usefulness of computed tomographic angiography and magnetic resonance imaging in the diagnosis of sinus of Valsalva aneurysm.^13,14

Our patient presented us with a complex management situation. There is little discussion in the literature regarding this abnormality in a pregnant patient, and the physiologic changes that occur with pregnancy (Table I)^15,16 increase the risk in patients with an anatomic cardiac defect. One case has been reported in which a rupture of the sinus of Valsalva occurred at 37 weeks' gestation and was successfully repaired 1 week postpartum.⁵ Although it is not clear when the aneurysm ruptured in our patient, the possibility exists that the patient had multiple, successful pregnancies in the context of a left-to-right shunt, given her reported history of a congenital cardiac defect since childhood. Pregnancy is a state that places hemodynamic strain on the cardiovascular system and can be risky in women with underlying cardiac disease. It is well established that women with significant pulmonary hypertension are at great risk during pregnancy, when there is normally a large increase in total blood volume and a significant increase in cardiac output.¹⁷ Such increases could place excessive burden on a patient with a left-to-right shunt, such as in our patient.

Table I. Hemodynamic Changes During Pregnancy that May Contribute to Worsening of Cardiac Status in Patients with Anatomic Cardiac Defects^15,16

It is not clear when surgery should be performed in an asymptomatic patient with a ruptured sinus of Valsalva aneurysm. The mortality rate is presumably high in patients after rupture when surgical correction is not achieved. Several recent studies have shown an excellent prognosis in patients after surgical repair via the open-heart method, using aortotomy or incision into the cardiac chamber where the fistula is present.^7,8,18 However, the vast majority of patients in these studies were symptomatic. Because our patient was asymptomatic, surgery was not undertaken during pregnancy, and the patient was observed closely without complication. Medical management in such patients is usually focused on treating the heart failure, endocarditis, or arrthythmia.¹ Although our patient's pregnancy was unremarkable, this ruptured sinus of Valsalva aneurysm could have had serious adverse consequences. In addition, cardiac surgery during pregnancy has been found to result in increased fetal loss.¹⁹

Recent reports have examined transcatheter clo-sure of a ruptured sinus of Valsalva.^15,20 Arora and colleagues²⁰ have reported their experience with 8 patients in whom the defect was repaired by crossing retrogradely from the aortic side, over an arteriovenous wire loop after balloon sizing. Devices were successfully deployed via the antegrade venous approach across the defect. These reports highlight the fact that transcatheter closure is safe and effective, and has the advantage of avoiding open-heart surgery.

Conclusion

This case illustrates the diagnosis of a rare cardiac condition during pregnancy. It raises the issue of whether to perform cardiac surgery during early pregnancy or to pursue medical management. If indeed the patient had this condition for many years, hers would be the first documented case of multiple, successful pregnan-cies in a patient with a previously ruptured sinus of Valsalva aneurysm.