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Although congenital aneurysm in sinus of Valsalva is uncommon, accounting for only 0.31%-3.56% in congenital heart diseases (CHD),［1］ once an aneurysm in the sinus of valsalva ruptures, it will produce serious and immediate effects on cardiac function and is always followed by acute congestive heart failure, and even death,［2］ therefore, the diagnosis of ruptured aneurysm in sinus of Valsalva (RASV) should be made immediately and correctly, with surgical intervention employed as early as possible, if the general condition of the patient permits,［3］ from September 1981 to April 2001, 37 patients with RASV were diagnosed and underwent surgical intervention in our department, accounting for 4.1% of the cardiac operations performed during that period. In the present study, we summarized and discussed the diagnostic and therapeutic principles of RASV.
METHODS
General clinical information
Thirty-seven patients with RASV were recruited into the study, including 28 men and 9 women ranging from 12 to 57 years (mean: 29.6 years). Case histories ranged from 5 hours to 12 years (mean: 1.4 years).
Main symptoms and signs
Twenty-five patients presented acute dyspnea and chest pain after heavy activities. Ten noted gradual onset with progressive development; while the remaining 2 were asymptomatic at the time of diagnosis, requiring further investigation after discovery of a typical heart murmur revealed during routine physical examination.
Palpitation and dyspnea upon exertion occurred in 37 patients, angina pectoris in 19 and occasional unconsciousness in 3. A typical continuous murmur localized in the 3rd and/or 4th intercostal space left to the sternum was documented in 30 RASV patients, and a systolic murmur in 7 others, which was audible occurred extensively in the precordium. Twenty-three patients exhibited continuous cardiac thrill and systolic trill in 6. Peripheral vascular signs were observed in 33 patients with pulse pressure ranging from 30 to 140 mmHg . Two patients were categorized as having class Ⅰ cardiac function, 16 with class Ⅱ, 14 with class Ⅲ, and 5 with class Ⅳ. Of them, six patients suffered from leg edema.
Color echocardiographic imaging and intraoperative pathological detection
The diagnosis of RASV was determined by preoperative color echocardiographic imaging and intraoperative pathological detection. Twenty-six patients with RASV occurred in the right sinus of Valsalva, 18 of which had bulging into the right ventricular outflow tract, one into the right ventricle and 7 into the right atrium. The remaining eleven patients with RASV originated from the non-coronary cusp and produced protrusions into the right atrium. Most sinus aneurysms were observed as white, tapering windsocks with smooth and thin walls; thick walls were rarely observed. The terminal ostium of RASV ranged in size from 0.6 to 2.8 cm. Perforations were localized at the apex of the sac or in the waist exhibiting only one fistula in 27 cases, 2 fistulas in 9 cases and 3 fistulas in one. Twenty-one patients with RASV were associated with VSD at different locations. Other associated cardiac problems included overt-moderate aortic valvular insufficiency (8 cases), mild aortic insufficiency (4), atrial septal defects (8), stenotic right ventricular outflow tract (3), tricuspid insufficiency (6), mitral insufficiency (5) and patent ductus arteriosus (3).
Chest X-ray and electrocardiogram (ECG) detection
Chest X-ray showed pulmonary congestion in most cases, and hilar dance in some cases. Cardiac X-ray images were generally larger than normal with heart-to-thoracic ratios over 0.5 in 30 patients. Electrocardiograms indicated left-ventricular hypertrophy in 14 patients, right-ventricular hypertrophy in 10, double-ventricular hypertrophy in 5, complete or partial right bundle branch block in 9, myocardial injury changes in 20, and paroxysmal ventricular premature beats
in 7.
Operation
All subjects accepted open-heart surgery under general anesthesia and hypothermal extracorporeal circulation. Extracardiac investigations were performed after opened the pericardium and exposed the heart. If necessary, intracardiac detection was made with a finger through a purse-sutured hole at the apex of the right atrium.
Selection of the method used for cardioplegia-inducing solution infusion depended on the following factors. If the aortic valve was normal or mildly insufficient, the solution could be infused directly into the aortic root; but it was necessary to open the terminal chamber to close the fistula with fingers to keep an effective perfusion. If overt-moderate aortic insufficiency occurred, coronary ostial infusion or retrograde infusion was performed. A Fullet's urinary catheter could be used as an effective and convenient alternative to retrograde infusion. Otherwise, if the operation was complex or the cross-clamping time prolonged, especially when an aortic incision was necessary for valvuloplasty or replacement, retrograde coronary sinus infusion was conducted because it was essential to protect myocardium and save time.
Selection of the approach was another important factor. If the recipient chamber was the right atrium (clear of other cardiac defects), a single incision into the right atrium was enough. If upper VSD was present, a pulmonary artery incision might be necessary. If inferior VSD or stenotic right ventricular outflow tract occurred, an incision through the outflow tract was most appropriate. However, if aortic valvular detection, valvuloplasty or replacement were also going to be done, an aortic root incision was conducted depending on the situation.
In general, the approach was determined by the terminal location and actual complications. The overall goal was to expose the diseased area sufficiently, correct the defects precisely and minimize myocardial damage.
Method of repair was another factor. Once the aneurysm sac was cut symmetrically to expose the terminal opening edge of the fistula, we resected the sac wall 3-4 mm away from the edge. In 30 cases, Dacro patches were used to close the fistula with a mattress or running suture using 3-0 double-headed non-invasion silk or prolene silk. Twenty-one individuals underwent transaortic repair at the proximal orifice, some of whom required additional repair in the recipient chamber. If immediate upper-positioned VSD was identified, we utilized a single patch shaped like table tennis paddle to close the fistula and the VSD at the same time. After the repair was completed, the residual aneurysm wall could be used as an additional layer to consolidate the repair using a continuous or interrupted suture. If the VSD was distant from the RASV, we repaired both separately. The remaining seven patients underwent direct closure of RASV without patches.
We performed valvuloplasty by vavular folding in 2 patients and by vavular suspension in 4. Aortic valvular replacement was performed in 2 patients with severely diseased aortic valve which could not be treated by valvuloplasty.
RESULTS
The operations in this series were performed successfully with aortic clamping time ranging from 0.5 to 2.0 hours with a mean of 60.7±5.2 minutes. The duration of cardiopulmonary bypass ranged from 1.0 to 6.5 hours with a mean of 90.2±8.7 minutes. No perioperative mortality occurred. Acute renal failure affected one case, which was possibly caused by 2 occurrences of sudden cardiac arrest resulting in hypotension. After treatment with 4 days bedside blood dialysis, the patient experienced 4 days of oliguria and 3 days of anuria, but recovered subsequently. Residual shunting was revealed in 2 patients half a year after surgery with shunt orifice diameters of 0.2 and 0.3 cm, respectively. Three individuals developed mild aortic insufficiencies, and three developed wound infections. No sternum infection occurred. Follow-up was conducted for 1 month to 20 years in 24 patients. All patients followed up were asymptomatic and reported improved cardiac function, 13 of whom were reclassified as classⅠ, 6 as class Ⅱ and 5 as class Ⅲ. Most maintained normal lives with no recurrence during follow up.
DISCUSSION
Most aneurysms of sinus Vaisava (ASV) are congenital and rarely develop due to trauma, endocarditis, syphilis or necrosis of artery tissue. Congenital aneurysm in the sinus of valsalva occurred most frequently in the right coronary cusp (70.3%) probably because the cusp abuts the largest portion of septum. It occurs less frequently in the non-coronary cusp (29.7%) and extremely rarely in the left coronary cusp. The majority of the patients were adults with an average age of 29.4 years and a male-to-female ratio of 3.1 to 1.0. About 73.1% of the aneurysms occurring in the right sinus of Valsava ruptured into the right ventricle (including the right ventricular outflow tract), while 26.9% penetrated into the right atrium. A few patients were asymptomatic but were identified as having ASV during physical examination. About 56.7% ASV were associated with VSD and 32.4% were complicated by aortic insufficiency.［4,5］
A ruptured aneurysm in the Valsalva sinus usually produces serious hemodynamic changes, especially when the occurrence is sudden; this event is almost always followed by congestive cardiac dysfunction, cardiac shock or even sudden death.Therefore, diagnosis of RASV should be made immediately and correctly, followed by surgical treatment if the patient's condition permits. Identification of the following risk factors would provide valuable information for RASV diagnosis:［6］ ① adult male patients; ② physical activity, such as heavy-lifting, deep cough or alcohol consumption led to sudden chest pain and dyspnea that continued to worsen; ③ discovery of a newly-formed extendively present cardiac continuous murmur, usually accompanied by thrill; ④ congestive heart failure; ⑤ unexplained tachycardia or angina. When RASV is suspected, color echocardiographic detection should be used immediately to distinguish it from other similar diseases including pneumothorax, aortopulmonary window, coronary artery fistula, pulmonary arteriovenous fistula and patent ductus arteriosus.Cardiac catheterization examination and/or retrograde angiography of aorta is recommended when an echographic test fails.［3］
Once the diagnosis of RASV is confirmed, surgical correction should be performed immediately and, if not an emergent operation, necessary operation should not be delayed because of concerns about cardiac function improvement. The strategy of operation should be based on the location, size of the RASV fistula, presence or absence of VSD and aortic insufficiency, and other possible associations.［8］
How the cardioplegia solution should be infused will be based on the actual situations. Options include aortic root, coronary ostia or retrograde infusion. The cardiac incision should be small, but should produce under a clear operating field and be convenient for repair. Hamid believed that transaortic repair could close the VSD and detect possibly-damaged aortic valve simultaneously, and if necessary, the valve could be replaced or valvuloplasty performed at that time.［9］ We realized that a single paddle-shaped patch utilized for adjacent RASV and VSD could consolidate repair and meanwhile, treat mild and moderate aortic insufficiency by suspending the prolapsed aortic valve cusp.［10］
Some surgeons have recommended that the suture be parallel to the axis of the aorta in order to avoid the deformation of the aortic ring and to diminish the recurrence of ASV and injury to the valves.However, we believe that if there are no complications involving the aortic valve, the aortic root should not be opened and the defects should be closed from the right-side of the heart to minimize the aortic cross clamping time, and reduce the injury to the myocardium and improve heart function. The operation procedure should be safe, simple, and effective. If the diameter of the RASV fistula is less than 1.0 cm and the orifice edge is solid, it can be closed directly without a patch. If a patch is used, a mattress suture, and sometimes a running suture using prolene or non-invasive silk is also effective and does not cause deformation. Resection of the aneurysm sac should be small and intraoperative stretching must be gentle to avoid affecting the aortic ring or damaging the valve. If an unruptured aneurysm is encountered, it should be opened and close the terminal orifice completely.［11］
Based on the pathology of the aortic valves, we used different treatments, either valvuloplasty or replacement to gain long-term and valuable results.［12］
In general, we believe that an early and correct diagnosis of RASV leading to immediate surgical treatment based on the general condition of the patient is the key to successful treatment. All associated cardiac problems may be treated at the same time and should be carried out with meticulous attention to obtain safe and satisfactory results and to avoid the recurrence or residual shunting.
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