Ruptured Aneurysm of the Right Sinus of Valsalva Associated with a Ventricular Septal Defect and an Anomalous Coronary Artery

Abstract

Aneurysms of the sinus of Valsalva are extremely rare. Ruptured aneurysms of the sinus of Valsalva are frequently associated with other congenital defects, particularly with ventricular septal defect, aortic valve regurgitation, and bicuspid aortic valve. We describe the case of a 26-year-old man who had a ruptured aneurysm of the right coronary sinus, a ventricular septal defect, and an anomalous origin of the right coronary artery. Successful surgical correction of the aneurysm and ventricular septal defect was performed with patch repair and aortic valve replacement. A review of the English-language medical literature revealed only 1 other case of a sinus of Valsalva aneurysm associated with a ventricular septal defect and an anomalous coronary artery. Previously published reports of the coexistence of a single coronary artery with a sinus of Valsalva aneurysm or with a ventricular septal defect, and their management, are discussed herein.

Sinus of Valsalva aneurysms are extremely rare cardiac anomalies that may be acquired or congenital. The acquired aneurysm is caused by conditions affecting the aortic wall, such as infections (syphilis, bacterial endocarditis, and tuberculosis), trauma, and degenerative disease (connective tissue disorders, Marfan syndrome, and cystic medial necrosis).¹ The congenital aneurysm is more common and is most often caused by weakness at the juncture of the aortic media and the annulus fibrosus.² Aneurysms may originate in the right coronary sinus (65%–85%), the noncoronary sinus (10%–30%), and, rarely, the left coronary sinus (1%–5%).³ Aneurysms usually remain asymptomatic unless they are complicated by rupture. Ruptured sinus of Valsalva aneurysms are frequently associated with ventricular septal defects; this is particularly true of aneurysms that rupture into the right ventricle.⁴ We report herein the successful treatment of a patient who had a rare combination of a ruptured aneurysm of the right sinus of Valsalva, a ventricular septal defect, and an anomalous origin of the right coronary artery.

Case Report

In December 2004, a 26-year-old man was admitted to our hospital because of a 2-day history of fatigue, substernal chest pressure, palpitations, and “pounding” headache. His symptoms remained severe for 1 day, then slowly subsided, leaving him with mild residual chest discomfort. He had a history of cardiac murmur (since birth) and was diagnosed at that time with a small, perimembranous ventricular septal defect (VSD) by transthoracic echocardiography. He was followed closely with echocardiograms every 1 to 2 years and remained asymptomatic until the current presentation.

The patient was a thin white man in no acute distress. His height was 182 cm, and his weight was 63 kg. His heart rate was 110 beats/min and regular, his blood pressure was 115/45 mmHg, and his oxygen saturation was 98% while he was breathing ambient air. He had bilateral carotid bruits and a hyperdynamic left ventricular impulse with a thrill over the left sternal border. A loud, harsh, continuous grade 5/6 murmur, loudest in systole, was best heard near the left lower sternal border. There were rales at both lung bases. Water-hammer femoral and dorsalis pedis pulses with a bisferious contour were present.

Results of urinalysis, complete blood count, and blood chemistry tests were normal. An electrocardiogram showed sinus tachycardia at a rate of 100 beats/min and met the voltage criteria for left ventricular hypertrophy. Chest radiography revealed a normal cardiac silhouette with mild congestion of the pulmonary vasculature. Telemetry documented multiple episodes of nonsustained ventricular tachycardia, which were not associated with symptoms.

Transthoracic echocardiography showed normal left and right ventricular dimensions; left ventricular systolic function was hyperdynamic. Color-flow Doppler echocardiography detected abnormal flow consistent with rupture of a right coronary sinus of Valsalva aneurysm into the right ventricular outflow tract (Fig. 1). Transesophageal echocardiography confirmed the presence of a ruptured right coronary sinus of Valsalva aneurysm with high-velocity flow (4 m/sec) into the right ventricular cavity during both systole and diastole (Fig. 2).

Fig. 1 Transthoracic echocardiography (parasternal short-axis view) with Doppler interrogation. The ruptured right sinus of Valsalva aneurysm is shown (arrow).

Ao = aorta; LA = left atrium; RA = right atrium; RV = right ventricle

Fig. 2 Transesophageal Doppler echocardiography in A) long-axis view, B) short-axis view, and C) short-axis view with Doppler interrogation. The ruptured sinus of Valsalva aneurysm is shown (arrows).

Ao = aorta; LV = left ventricle; RV = right ventricle

Right heart catheterization revealed a right atrial pressure of 3 mmHg, a right ventricular pressure of 43/4 mmHg, a pulmonary artery pressure of 36/6 mmHg, and a pulmonary wedge pressure of 19 mmHg. There was a 15% step-up in oxygen saturation between the right atrium and the right ventricle. The ratio of pulmonary to systolic flow (Qp/Qs) was calculated to be 1.5.

Left heart catheterization revealed a right coronary sinus of Valsalva aneurysm with rupture into the right ventricular outflow tract (Fig. 3). A pigtail catheter passed via the femoral artery entered the right ventricular cavity through the ruptured sinus of Valsalva (Fig. 4). Coronary angiography revealed an anomalous origin of the right coronary artery from the left main trunk, traversing anteriorly between the aorta and the pulmonary artery (Fig. 5).

Fig. 3 Left ventriculogram (left anterior oblique cranial projection). Contrast dye can be seen in the right ventricular cavity (arrows).

Fig. 4 Catheter is passed via the arterial circulation through the ruptured right sinus of Valsalva aneurysm into the right ventricular outflow tract.

Fig. 5 Coronary angiography (right anterior oblique caudal projection). The right coronary artery originates from the left main artery (arrow).

The patient underwent surgical repair on cardiopulmonary bypass. We found a right sinus of Valsalva aneurysm that had ruptured into the right ventricle through a 4.6-cm defect, and we repaired it with valve sutures. A perimembranous VSD was closed with a dime-size patch. The noncoronary cusp of the aortic valve was fenestrated, and the valve was replaced with a St. Jude prosthesis (St. Jude Medical, Inc.; St. Paul, Minn). Pathologic evaluation of the aneurysmal tissue revealed marked myxoid degeneration within fibrous tissue (Fig. 6). The patient made an uneventful recovery, had no further episodes of ventricular arrhythmia, and was discharged from the hospital 5 days after surgery.

Fig. 6 Sinus of Valsalva aneurysm tissue shows marked myxoid degeneration.

Discussion

Sinus of Valsalva aneurysms are very uncommon, with an incidence ranging from 0.1% to 3.5% of all congenital heart defects.⁵ Sinus of Valsalva aneurysms have a marked male preponderance (4:1), and their incidence is highest in Asian populations. Most sinus of Valsalva aneurysms arise from the right or the noncoronary sinuses, and they commonly protrude and rupture into the right ventricle or right atrium. Aneurysms may also rupture into the left ventricle, pulmonary artery, left atrium, or pericardial cavity, but these entry sites are exceptional.^6,7

The typical congenital aneurysm begins as a blind pouch that originates from a localized site in one of the sinuses. Edwards and Burchell² described the structural defect of the aneurysm as a lack of continuity between the aortic media and the aortic annulus, which sets the stage for subsequent avulsion and aneurysm formation. Ventricular septal defects, as well as aortic valve regurgitation and bicuspid aortic valve, are frequently associated with ruptured sinus of Valsalva aneurysms—particularly with aneurysms that rupture into the right ventricle.^4,8 The association between the right and noncoronary sinus of Valsalva aneurysms and VSDs is probably not accidental. It has been proposed that the defect results from incomplete fusion between the right and left distal bulbous septum in the fetus, the base of which forms the right and the noncoronary sinuses of Valsalva. The resulting congenital weakness along the aortic annulus might be closely related to defects in the membranous ventricular septum.^9,10

The principal VSD associated with a ruptured sinus of Valsalva aneurysm is the supracristal type.¹¹ The supracristal VSD (type I) is the predominant type seen in Asian countries, whereas the perimembranous VSD (type II), such as was present in our patient, is often seen in Western countries.^12,13 The incidence of associated aortic sinus aneurysms and ventricular septal defects is higher in Asian countries as well.

The combination of a sinus of Valsalva aneurysm and a single coronary artery is quite rare. Chamsi-Pasha and colleagues¹⁴ reported the 1st case of a ruptured sinus of Valsalva coexisting with a single coronary artery in 1989. Goto and coworkers,¹⁵ in the same year, reported a case of an aneurysm of the right sinus of Valsalva that ruptured into the right ventricular outflow tract and coexisted with a single coronary artery originating from the left coronary sinus, wherein the right coronary artery branched from the left main trunk and ran dorsally to the aorta. Cabrera and associates,¹⁶ in 1990, reported a rare case of complex cardiac malformation with an aneurysm of the sinus of Valsalva, atrial appendage, and ascending aorta, associated with a ventricular septal defect, fibromuscular subaortic stenosis, and a single coronary artery. In 1991, Dazai and co-authors¹⁷ described a case of an infective aneurysm involving all 3 sinuses of Valsalva and complicated by single left coronary artery. In 1992, Sundar's group¹⁸ reported a case of a young man with a congenital aneurysm of the noncoronary sinus of Valsalva rupturing into the right ventricle, and an anomalous origin of the right coronary artery from the main pulmonary artery. More recently, Ishii and colleagues¹⁹ reported a case similar to ours, wherein a rare combination of a congenital ruptured aneurysm of the sinus of Valsalva coexisted with a ventricular septal defect and a single coronary artery.

Isolated single coronary artery is a rare congenital anomaly occurring in approximately 0.02% to 0.06% of the population and is frequently associated with other congenital cardiovascular abnormalities.^20,21 Absent right and left coronary ostia occur with similar prevalence, and no sex-related differences have been noted.²⁰ Only a few cases of VSD associated with single coronary artery have been reported.16,19,22–28 In 3 cases, the single coronary artery and the VSD were the only cardiac anomalies present;^22,27,28 in the remaining cases, they were associated with other cardiovascular defects, such as subaortic and subpulmonic stenosis,^16,24 tetralogy of Fallot,²⁶ and an aneurysm of the sinus of Valsalva.^16,19 Having a single coronary artery predisposes patients to severe complications, such as myocardial ischemia, heart failure, and sudden death.²¹ The risk of sudden death or myocardial infarction is considered to be negligible when the right coronary artery originates from the left coronary artery,²⁹ although several reports suggest otherwise.^30,31 The combination of a ruptured sinus of Valsalva aneurysm, a VSD, and a single coronary artery is very rare and has only been reported once before in the world medical literature.¹⁹

Rupture of a sinus of Valsalva aneurysm frequently occurs in previously healthy young adults who suddenly develop chest pain, dyspnea, and a loud continuous murmur. A substantial number of ruptures develop well after puberty and before age 40 years, with a mean age of 35.8 years and a range of 13 to 65 years.³² A smaller number of ruptures occur in infancy or childhood,³³ as well as in late adulthood.⁵ The physiologic consequences of a rupture depend mainly upon the rapidity of the rupture, the size of the ruptured aneurysm, and the chamber that receives the hemorrhagic discharge. Transthoracic and transesophageal echocardiography with color-flow imaging and spectral Doppler interrogation frequently establish the diagnosis, as was done in our case. Cardiac catheterization is often useful in establishing the diagnosis and evaluating the hemodynamic effects of the ruptured aneurysm. Early, aggressive intervention is recommended to prevent endocarditis or enlargement of the ruptured aneurysm; the long-term results are excellent after surgical repair.^5,32 Transcatheter closure of ruptured sinus of Valsalva aneurysm has been described and may become an alternative to surgical repair.^34,35