Title: Ruptured aneurysm of the sinus of Valsalva protruding into the pulmonary artery
Abstract: Congenital sinus of Valsalva aneurysm is uncommon. The rupture results in a fistulous connection between the aorta and any cardiac chamber. This report describes a patient with a ruptured aneurysm of the right sinus of Valsalva that extended to the right ventricle, crossed over the pulmonary valve, and protruded into the main pulmonary artery. The aneurysm obstructed the right ventricular outflow tract (RVOT) and made a pressure gradient between the right ventricle and the pulmonary artery. The top of the aneurysmal sac had a ruptured hole opening in the pulmonary artery that caused an aortopulmonary shunt. To the best of our knowledge, no other cases with these features have been reported in the medical literature. A 46-year-old man presented with dyspnea on exertion, which he had experienced for the past several years. A heart murmur had been diagnosed in the patient at the age of 7 years. A ventricular septal defect was suspected, but the patient had not undergone a checkup because he had no symptoms. On presentation, he was in New York Heart Association class II. Transthoracic echocardiography revealed an aneurysm of the right sinus of Valsalva that extended into the RVOT. An aortogram showed a fistulous connection between the aneurysm and the pulmonary artery, which led to the diagnosis of a ruptured aneurysm of the sinus of Valsalva. There was a pressure gradient of 40 mm Hg between the right ventricle and the pulmonary artery. Magnetic resonance imaging angiography and computed tomography confirmed that the aneurysm extended into the RVOT, crossed over the pulmonary valve, and protruded into the pulmonary artery (Figure 1). The operation was performed through a median sternotomy. After aortic crossclamping, a transverse aortotomy was made. We searched the right sinus of Valsalva for the right coronary orifice to apply the cardioplegic solution. We found the neck of the aneurysm in the right coronary sinus. After cardioplegic arrest, the main pulmonary artery was opened transversely. A large aneurysm approximately 4 to 5 cm in diameter was found in the pulmonary artery arising from the right ventricle. The aneurysm had a ruptured hole on the top (∼7 mm in diameter) that was opening into the pulmonary artery (Figure 2). The wall from the right coronary sinus to the neck of the aneurysm was calcified. The aneurysm was removed through a pulmonary incision. Because of the calcified wall, we could not resect all of the aneurysm and left some of it in the right ventricle. We closed the defect of the right sinus of Valsalva with an expanded polytetrafluoroethylene patch through the pulmonary incision. The patient was easily weaned from cardiopulmonary bypass. The postoperative course was good. On postoperative echocardiography, no residual shunt was detected. Sinus of Valsalva aneurysms are rare. In most cases, the aneurysm arises from the right coronary sinus, which is normally adjacent to the RVOT. The aneurysm can rupture any cardiac chamber, most commonly into the right ventricle and occasionally into the right atrium. Rupture into the pulmonary artery is rare, and only 4 cases have been reported in the medical literature.1Heilman 3rd, K.J. Groves B.M. Campbell D. et al.Rupture of left sinus of Valsalva aneurysm into the pulmonary artery.J Am Coll Cardiol. 1985; 5: 1005-1007Abstract Full Text PDF PubMed Scopus (19) Google Scholar, 2Luckraz H. Naik M. Jenkins G. et al.Repair of a sinus of Valsalva aneurysm that had ruptured into the pulmonary artery.J Thorac Cardiovasc Surg. 2004; 127: 1823-1825Abstract Full Text Full Text PDF PubMed Scopus (13) Google Scholar, 3Kar A.K. Bhattacharya S. Ray D. et al.Rupture of the sinus of Valsalva into the pulmonary artery.Indian Heart J. 2002; 54: 415-417PubMed Google Scholar, 4Fazio G. Zito R. Dioco D.D. et al.Rupture of a left sinus of Valsalva aneurysm into the pulmonary artery.Eur J Echocardiogr. 2006; 7 (Epub 2006 Jan 6): 230-232Crossref PubMed Scopus (16) Google Scholar In addition, 1 additional case involved an unruptured aneurysm of the sinus of Valsalva into the pulmonary artery.5Shiraishi S. Watarida S. Katsuyama K. et al.Unruptured aneurysm of the sinus of Valsalva into the pulmonary artery.Ann Thorac Surg. 1998; 65: 1458-1459Abstract Full Text Full Text PDF PubMed Scopus (8) Google Scholar In the presented case, an aneurysm originated from the right sinus of Valsalva and protruded into the pulmonary artery. The aneurysm extended from the right ventricle passing through the pulmonary valve and caused a pressure gradient between the right ventricle and the pulmonary artery. Aortography showed an aortopulmonary fistula but did not correctly reveal that the aneurysm had passed through the pulmonary valve. Magnetic resonance imaging angiography and computed tomography confirmed the diagnosis. Other characteristics of this case are that the onset of heart failure was not sudden and that the patient did not have any other cardiac defects, such as a ventricular septal defect. These clinical features suggested that the patient's heart murmur from childhood was caused by a chronic fistula between the aneurysm and the pulmonary artery. In cases of ruptured sinus of Valsalva aneurysm, it is common to have a sudden onset of symptoms. In the presented case, his symptoms became insidiously worse. Preoperative aortic regurgitation was slight in this case and did not worsen postoperatively. However, because of the calcified deformity of the remains of the sinus of Valsalva, we are concerned about the progression of aortic regurgitation and the patient must be kept under close long-term observation.