Section I: Sinus of valsalva aneurysms
Definition
Congenital sinus of Valsalva sinus of Valsalva aneurysms are thin-walled saccular or tubular outpouchings located between the aortic valve anulus and the sinutubular junction.
They usually involve the right sinus or adjacent half of the noncoronary sinus. They develop secondary to a weakness in the elastic lamina at the junction of the aortic media and the anulus fibrosus. A single sinus is usually affected, and it generally has an intracardiac protrusion. When rupture occurs, it often happens into the right heart chambers forming an aorto-cardiac fistula, which could be a potentially fatal complication requiring urgent treatment. Associated congenital cardiac anomalies are common.
Classification
In 1962, Sakakibara and Konno proposed a comprehensive classification for congenital sinus of Valsalva aneurysms. This was based on the origin of the aneurysm from the aortic sinus, the cardiac chamber into which the aneurysm protrudes, and whether a ventricular septal defect (VSD) was present or not. Type I, II, and III arise from different parts of the right coronary sinus, and Type IV arises from the noncoronary sinus. For example, if there a Type IV with an atrial protrusion, it is Type IVa, or with a ventricular protrusion, it is Type IVv. If there is a Type III aneurysm with a VSD, then it is Type III VSD.
Historical note
The syndrome of acute rupture of a congenital sinus of Valsalva aneurysm was apparently first described by Hope in 1839. A year later, Thurman published the first important paper on the subject. He discussed Hope’s case and added five of his own, none of which had ruptured. Eighty years later, Abbott reviewed the clinical features of acute rupture from eight previous cases and reported another case. At that time, and even as late as 1937, most ruptured and unruptured sinus of Valsalva aneurysms were considered syphilitic. Smith stated in 1914 that “the lesion, which is usually syphilitic, is not so rare as to be altogether devoid of clinical interest, but the diagnosis, perforating or otherwise, presents almost insurmountable difficulties.” Jones and Langley reviewed congenital and acquired aneurysms in 1949. They accepted 25 cases as being of congenital origin and elucidated most of the important features of the condition. In 1951, Venning may have been the first to diagnose acute rupture during life, although Oram and East claimed this distinction in 1955, as did Brown and colleagues. In Oram and East’s patients, cardiac catheterization confirmed the presence of a left-to-right shunt, although angiography was not performed. The earliest report of using aortography to diagnose an unruptured aneurysm was that of Falholt and Thomsen in 1953.
The first successful surgical repairs of sinus of Valsalva aneurysms were performed in 1956 at the Mayo Clinic and the University of Minnesota, using cardiopulmonary bypass (CPB). Spencer, Blake, and Bahnson and Morris and colleagues also reported early successful cases. In 1957, both Morrow and colleagues and Bigelow and Barnes successfully closed a ruptured congenital sinus of Valsalva aneurysm using mild hypothermia with inflow stasis, , but this technique was not subsequently employed. Numerous reports followed of one or two patients treated successfully using CPB. Dubost and colleagues reported eight cases in 1962, and Besterman and colleagues reported six cases in 1963. In a 1960 collective review, Kieffer and Winchell reported 78 surgical and nonsurgical patients, 59 of whom had rupture of the aneurysm into a cardiac chamber. Sakakibara and Konno noted the prevalence of this lesion in Japan and its association with VSD and aortic regurgitation (AR), and were among the first to provide a comprehensive classification. , , Their first patient underwent aneurysm repair in 1960.
Morphology
The essential lesion of congenital sinus of Valsalva aneurysms is separation of the aortic media of the sinus from the media adjacent to the hinge line of the aortic valve cusp, as emphasized by Edwards and Burchell in 1957 ( Fig. 37.1 ). This defect may result from absence of normal aortic elastic tissue and media in this region. , The congenitally weak area gradually enlarges under aortic pressure to form an aneurysm, although the age at which this occurs is uncertain. Viewed from the aorta, the aneurysm appears as an excavation of the sinus of Valsalva that protrudes into the underlying cardiac chamber ( Fig. 37.2 ).
Unruptured right sinus of Valsalva aneurysm in a non-Asian patient. Low-power photomicrograph of a longitudinal section through the central portion of right aortic sinus shows separation of aortic media of the sinus from media adjacent to the hinge line of right aortic cusp. Aneurysm is walled by atrophic muscular tissue of right ventricular outflow tract. LV, Left ventricle; RAL, right aortic leaflet (cusp); RV, right ventricle.
(From Edwards JE, Burchell HB. Specimen exhibiting the essential lesion in aneurysm of the aortic sinus. Mayo Clin Proc . 1956;31:407.)
Unruptured right sinus of Valsalva aneurysm. (A) Viewed from the aorta, aneurysm has a well-defined origin, or neck, presenting as an almost circular ostium in the base of the sinus of Valsalva just above the hinge line of the aortic cusp. (B) Viewed from right ventricle, aneurysm protrudes into right ventricular outflow tract just below pulmonary valve. (C) Anatomic depiction of a specimen with coexisting juxta-aortic ventricular septal defect (VSD) in right ventricular outlet portion of ventricular septum. The narrow band of aortic wall and septum separating aneurysm from VSD is located behind the aneurysm. An, Aneurysm of right aortic sinus of Valsalva; L, left aortic valve cusp; P, posterior (noncoronary) aortic valve cusp; PT, pulmonary trunk; R, right aortic valve cusp; RCA, right coronary artery; RV, right ventricle; TV, tricuspid valve; VSD, ventricular septal defect.
(From Edwards JE, Burchell HB. Specimen exhibiting the essential lesion in aneurysm of the aortic sinus. Mayo Clin Proc . 1956;31:407.)
Precise location of this basic congenital abnormality, which may be accompanied by an adjacent separation of the ventricular septum from the aorta to form a VSD, tends to be different in Asians and non-Asians. In Asians, the basic abnormality is located leftward and toward the commissural area between the right and left coronary cusps, so compared with non-Asians, rupture occurs more often into the right ventricle than right atrium (94% vs. 77%, P [χ 2 ] =.0001).
The coexisting VSD in Asian patients is usually leftward and juxta-arterial, whereas in non-Asians it is usually rightward and only juxta-aortic (see Chapter 33 for definitions). The leftward tendency in Asians is also manifested by fewer aneurysms of the more rightward noncoronary sinus than in non-Asians (11% vs. 32%, P <.0001). Left sinus of Valsalva aneurysms are uncommon in both Asians (2%) and non-Asians (5%) ( P [χ 2 ] for difference =.11). Acquired sinus of Valsalva aneurysms caused by medionecrosis, syphilis, arteriosclerosis, endocarditis, Behçet disease, , or penetrating injuries are usually readily distinguishable from congenital forms. They are more diffuse, involving more of the sinus or multiple sinuses and often the ascending aorta, and therefore project into the pericardium outside the heart. A congenital aneurysm is frequently diagnosed by exclusion of other etiologies as well as by presence of associated congenital cardiac defects. Difficulties arise in establishing a diagnosis of mycotic aneurysms, , because endocarditis complicates about 5% to 10% of congenital aneurysms. Similarly, difficulty exists in diagnosing the presence of medionecrosis (cystic medial degeneration), because it and Marfan syndrome are both present in some patients with congenital sinus of Valsalva aneurysms. ,
Rupture
In some patients, the aneurysm gradually develops a localized windsock, which ultimately ruptures into an adjacent low pressure cardiac chamber ( Fig. 37.3 ). The thin-walled, ruptured aneurysm characteristically has an intracardiac fistulous portion and a nipple-like projection into the cardiac chamber, with one or more points of rupture at its apex ( Fig. 37.4 ). Rarely, it projects outside the aortic root or heart. When the aneurysm coexists with a VSD, the windsock usually projects into the right ventricle through a thinned area of myocardium just downstream from the VSD; the aneurysm is separated from the VSD by the hinge line of the aortic valve cusp, at the septal portion of the left ventriculoaortic junction ( Fig. 37.5 ; see also Fig. 37.2 C).
Cineangiograms in right anterior oblique projection of a right sinus of Valsalva aneurysm ruptured into right ventricle in systole (A) and diastole (B). Noncoronary and left coronary sinuses and cusps are normal. Right coronary sinus is enlarged, and there is an aneurysm (windsock) protruding into right ventricular infundibulum. Arrows indicate contrast medium shunting through holes in aneurysm and filling right ventricular infundibulum in diastole and pulmonary trunk in systole (when the aneurysm almost prolapses through the pulmonary valve). There is no aortic regurgitation, but the ruptured aneurysm is associated with a large conoventricular juxta-aortic ventricular septal defect. A, Aneurysm; L, left coronary sinus; N, noncoronary sinus; PT, pulmonary trunk; R, right coronary sinus; RV, right ventricular infundibulum.
Cineangiogram in left anterior oblique projection of an aneurysm and fistula arising from the noncoronary sinus of Valsalva and rupturing into right atrium. Aneurysm fills from nadir of noncoronary sinus. There is shunting of contrast medium to the right atrium, through the tricuspid valve, and to the right ventricle. Right and left coronary sinuses appear normal. There is no aortic regurgitation and no ventricular septal defect. A, Aneurysm; L, left coronary sinus; N, noncoronary sinus; R, right coronary sinus; RA, right atrium; RV, right ventricle.
Anatomic depiction of a ruptured aneurysm of the rightward portion of right sinus of Valsalva associated with a conoventricular juxta-aortic ventricular septal defect (VSD). Rupture has occurred at apex of the windsock. Narrow band of aortic wall and septum separates aneurysm from VSD. R, Right coronary cusp; RV, right ventricle; TV, tricuspid valve.
About one-fourth of patients have no windsock or other suggestion of aneurysm formation, but rather have a direct fistulous communication between the aortic sinus and the heart. , This defect has been recognized in a few patients at or soon after birth. , , , Windsock deformity is typical in lesions originating from the right sinus and communicating with the right ventricle; a direct fistula is typical in those from the noncoronary sinus to the right atrium, , and an extracardiac aneurysm is typical , , in the rare cases of left sinus origin.
Although the prevalence of aneurysms in various locations is different among Asians and non-Asians, in both groups the sinus of origin is the main determinant of the direction of protrusion and rupture of the aneurysm, and thus of the chamber into which it ruptures ( Fig. 37.6 ). Also in both populations, but with differing prevalence, aneurysms of the right aortic sinus are most common. , , , , , , The aneurysm may arise from the leftward portion of this sinus, with the windsock projecting into the adjacent right ventricular outflow tract just below the pulmonary valve, termed Type I by Sakakibara and Konno. It may also originate more centrally (Type II Sakakibara and Konno) and project through the substance of the outlet portion of the right ventricular aspect of the ventricular septum ( Fig. 37.7 ), or from the rightward portion of the sinus (Type III Sakakibara and Konno), entering the right ventricle beneath the parietal band (parietal extension of the infundibular septum) in the region of the membranous septum. Rarely the aneurysm may project into the pulmonary trunk.
Diagrammatic representation of structures depicted in right anterior oblique view of heart. Arrows indicate common sites of rupture of sinus of Valsalva aneurysms. A, Aorta; C, conal (infundibular) septum; IVC, inferior vena cava; M, membranous septum; NC, noncoronary sinus; P, pulmonary trunk; R, right coronary sinus; S, septal band; SVC, superior vena cava; V, atrioventricular septum.
Cineangiogram in diastole (lateral projection) of an aneurysm of right coronary sinus that protrudes into right ventricular infundibulum, filled by contrast medium shunting through ruptured aneurysm. Pulmonary valve is still closed. Left coronary and noncoronary sinuses appear normal. There is no aortic regurgitation. At operation, aneurysm arose from center of right sinus, with a prominent windsock in the infundibular septum; immediately beneath it was a moderate-sized ventricular septal defect. L, Left coronary sinus; N, noncoronary sinus; P, pulmonary valve; R, right coronary sinus; RV, right ventricular infundibulum.
Aneurysms from the noncoronary sinus (Type IV Sakakibara and Konno) usually originate from its anterior portion and project into the right atrium ( Fig. 37.8 ), but in rare cases, they project and rupture into the right ventricle. Rarely, rupture can occur simultaneously into the right ventricle and right atrium or into the muscular ventricular septum. Aneurysms arising from the posterior portion of the noncoronary sinus may rupture into the pericardium. Another rare occurrence is a right sinus or noncoronary sinus aneurysm that ruptures into the left ventricle. Rarity of rupture into the left ventricle may be related to the relatively thick wall and high pressure in that chamber. Aneurysms arising from the left coronary sinus may rupture into the left atrium, left ventricle, or rarely the pulmonary trunk or pericardium. ,
Cineangiogram in right anterior oblique projection (diastole) of a large aneurysmal connection of noncoronary sinus (N) to right atrium (RA). Contrast flow (arrows) was observed to enter RA close to tricuspid anulus (dashed line) before passing through tricuspid valve to right ventricle (RV). Right (R) and left (L) coronary sinuses appear normal. There is no aortic regurgitation and no ventricular septal defect. At operation, a 15-mm-long windsock aneurysm was projecting into the right atrium adjacent to anteroseptal commissure of tricuspid valve.
Sinus of Valsalva aneurysms rupturing into areas adjacent to the tricuspid valve are also adjacent to the atrioventricular (AV) node and His bundle and may be a cause of heart block, bundle branch block, and ventricular fibrillation. Table 37.1 shows the overall distribution of the various sites of rupture, based on analysis by Chu and colleagues of 361 cases in the literature, including 57 from their own institution.
TABLE 37.1
Prevalence of Sites of Rupture for Sinus of Valsalva Aneurysms
Data from Chu and colleagues.
| Site of Rupture | Asian (% of 195) | Non-Asian (% of 166) | Total (% of 361) |
|---|---|---|---|
| Right atrium | 13 | 35 | 23 |
| Right ventricle | 84 | 57 | 72 |
| Right ventricle + right atrium | <1 | 1 | <1 |
| Left atrium | <1 | 1 | <1 |
| Left ventricle | <1 | 2 | |
| Right atrium + left atrium + left ventricle | <1 | <1 | |
| Ventricular septum | 1 | 1 | <1 |
| Pulmonary trunk | <1 | <1 | <1 |
| Right ventricle + pulmonary trunk | <1 | <1 | <1 |
| Pericardium | <1 | 2 | <1 |
Associated cardiac anomalies
Ventricular septal defect.
A VSD is the most common coexisting cardiac anomaly and may arise from the same congenital anomaly that produced the aneurysm. VSDs occur in 30% to 50% of patients, , , , , but prevalence is higher when the aneurysm arises from the right sinus. , When the aneurysm arises from the left third of the right aortic sinus, the VSD is juxta-arterial, with its upper margin formed by the confluent aortic and pulmonary valves. When the aneurysm arises from the central third of the right sinus, the VSD may be juxta-aortic or may lie within the muscle of the outlet portion of the septum. When the aneurysm arises from the right third of the right sinus (or rarely, the anterior portion of the noncoronary sinus), , the VSD is usually conoventricular and may be perimembranous as well (see Chapter 33 for definitions). Rarely, a conoventricular VSD occurs in association with an aneurysm arising from the central or leftward third of the right sinus. Sakakibara and Konno considered this a coincidental association between two independent malformations rather than a combined developmental anomaly.
Aortic valve abnormalities and aortic regurgitation.
Aortic valve abnormalities and AR are common in patients with sinus of Valsalva aneurysms. , , , When a VSD is present, AR usually results from a prolapsed aortic cusp, similar to the finding in the syndrome of VSD and AR (see Section II of Chapter 33 ). When a VSD is not present, AR usually arises from other aortic valve abnormalities, including a bicuspid valve. As in VSD and AR, when prolapse of the aortic cusp into a VSD is the cause, severity of AR progressively worsens. If the fibrous hinge line remains intact at the base of a prolapsed cusp, the aneurysm projects toward the ventricle superior to the hinge line, and the cusp projects through the VSD inferior to it. When the hinge line does not retain its integrity, however, as in long-standing cases, both structures form a single sac. , , Taguchi and colleagues noted that prolonged AR produces a fixed fibrous deformity of the prolapsed cusp.
The frequency of aortic cusp prolapse in sinus of Valsalva aneurysms was undoubtedly underestimated in earlier reports, particularly when no aortograms or echocardiograms were obtained and the aorta was not opened at operation. Aortic cusp prolapse is also less common if only ruptured sinus aneurysm is considered. Thus, in the series of Taguchi and colleagues, which included unruptured cases, AR (although usually mild) was present in 75% of patients, whereas in the series of Okada and colleagues from Japan, which included only ruptured cases, the prevalence was 17%.
A complicating problem is the difficulty of determining what constitutes a true (unruptured) sinus aneurysm with combined VSD and AR. Aneurysmal enlargement of the aortic sinus is common in this setting, and the distinction from unruptured sinus aneurysm is difficult to delineate by aortography and even at operation or autopsy. However, 7 (15%) of 48 surgical patients with VSD and AR operated on at Green Lane Hospital from 1960 to 1982 had a distinct but unruptured sinus of Valsalva aneurysm.
Pulmonary stenosis.
Important pulmonary stenosis is uncommon in patients with congenital sinus of Valsalva aneurysms, but small gradients are common. The stenosis may be valvar but is usually caused by either a projection of the windsock in front of the infundibular septum or a developmental anomaly of the right ventricular outflow tract similar to that present in tetralogy of Fallot and VSD-AR syndrome.
Other anomalies.
Infrequently, other congenital cardiac anomalies coexist with sinus of Valsalva aneurysms, including aortic coarctation, patent ductus arteriosus, atrial septal defect, subaortic stenosis, and tetralogy of Fallot. , , ,
Clinical features and diagnostic criteria
Unruptured congenital sinus of Valsalva aneurysms are usually silent lesions; their diagnosis depends on echocardiograms or aortograms usually obtained to demonstrate associated symptomatic lesions such as VSD or AR. Diagnosis can be made incidentally during echocardiography or coronary angiography. Rarely, unruptured aneurysms produce tricuspid valve dysfunction or right ventricular outflow obstruction, bringing the patient to medical attention. , These aneurysms may also produce severe myocardial ischemia by compressing the right or left main coronary artery. , Embolization from unruptured sinus of Valsalva aneurysms and complete heart block have also been reported. Presence of this anomaly should be considered in men, who represent 80% of patients with sinus of Valsalva aneurysms. ,
Acute symptoms occur in about 35% of patients with rupture of the aneurysm. , , In 45% of patients, surprisingly, rupture is associated only with gradual onset of effort dyspnea, and in 20%, no symptoms develop. Acute symptoms consist of sudden breathlessness and pain. The pain is usually precordial and may also be epigastric, probably because of acute hepatic congestion. Precordial pain may mimic myocardial infarction, although radiation of the pain beyond the substernal area is unusual. In a few patients, death occurs within days of rupture from right-sided heart failure, but most patients improve during the latent period, which may last for weeks, months, or years. This improvement may occur without specific medical therapy. The latent period is usually followed by recurrence of dyspnea and signs of right-sided heart failure. Characteristic features at this final stage are aortic and tricuspid regurgitation, an unusual combination. ,
The infrequency of severe symptoms at rupture may be due to the initially small size of the rupture in many patients. Studies by Sawyers and colleagues in dogs indicate that symptoms are severe when the fistula is greater than 5 mm in diameter. However, in humans, Taguchi and colleagues found little correlation between size of the fistulous opening at operation and a history of acute symptoms. Acute symptoms at rupture may occur less often with a VSD , and more often with severe AR. Acute symptomatic ruptures may be precipitated by heavy exertion, but they also occur after serious automobile accidents and at cardiac catheterization. Rarely, an episode of infective endocarditis may be the precipitating factor. Marfan syndrome may also predispose the aneurysm to rupture. Rupture is heralded not only by pain and dyspnea but also by a characteristic murmur that is loud, harsh, superficial, and accompanied by a coarse thrill. The murmur is usually continuous with either systolic or diastolic accentuation, but it may be to and fro, similar to that present in the VSD-AR syndrome. In the past, this murmur has been mistaken for that of patent ductus arteriosus, but it is maximal at a lower site, usually the left second, third, or fourth intercostal space. With rupture into the sinus portion of the right ventricle or right atrium, the murmur tends to be maximal at a low level over the sternum or to the right of the lower sternum. , , , Rarely the murmur is systolic only, possibly because the communication is small. Alternatively, the murmur may be confined to diastole in those few cases when rupture occurs into the high-pressure left ventricle , or when right ventricular pressure is at systemic level, as in the neonate. When the murmur is continuous, its timing and accentuation are a function of several factors including degree of associated AR, degree of aortic systolic murmur, functional size of the VSD, and size of the fistula. Morch and Greenwood assessed the various causes of murmurs that were believed to be continuous and associated with signs of rapid aortic runoff in their adult patients and found that ruptured sinus aneurysm (8 cases) was the second most common cause after patent ductus arteriosus (33 cases), followed by VSD and AR (3 cases), aortopulmonary window (3 cases), coronary arteriovenous fistula (1 case), and pulmonary arteriovenous fistula (1 case). Other physical signs of ruptured aneurysm include widened aortic pulse pressure, suggesting mild to severe AR. An elevated jugular venous pressure with a prominent v wave, suggesting tricuspid regurgitation, may be caused by direct entrance of a fistula into the right atrium, but in most cases this sign is absent until onset of right-sided heart failure, when liver enlargement and pulsation also occur. The chest radiograph does not show enlargement of the aortic root. Plethora may be present, although the left-to-right shunt through both the fistula and any associated VSD is usually small. The electrocardiogram shows either left ventricle or biventricular hypertrophy. Right bundle branch block may occur and may be more common in aneurysms with an intracardiac course close to the AV node and bundle of His. Complete heart block can also occur. , Although the diagnosis is virtually certain on clinical grounds in patients with acute symptoms and sudden appearance of a continuous murmur, two-dimensional Doppler color flow echocardiography is used for verification ( Fig. 37.9 ). , Cardiac catheterization and angiography are generally performed to study the site of origin and termination of the fistula and the presence of associated anomalies, particularly VSD, AR, and pulmonary stenosis (see Figs. 37.3 , 37.4 , 37.7 , and 37.8 ). The true size of the VSD cannot be estimated angiographically when the right aortic cusp is prolapsed into the VSD. Degree of left-to-right shunting through the fistulous communication with the VSD is calculated, as is pulmonary vascular resistance (see “ Cardiac Catheterization ” under Clinical Features and Diagnostic Criteria in Section I of Chapter 33 ). Magnetic resonance imaging may establish the diagnosis of aneurysm in certain circumstances.
Transesophageal echocardiogram (short-axis view) of sinus of Valsalva aneurysm (arrow) that has ruptured into right ventricular outflow tract. L, Left aortic sinus; LA, left atrium; N, noncoronary aortic sinus; PT, pulmonary trunk; R, right coronary sinus; RA, right atrium; RVO, right ventricular outflow tract.
(From van Son and colleagues. )
Natural history
Our current understanding of the natural history of this lesion is based on numerous case reports and series that include a variety of clinical presentations and management strategies. An unruptured congenital sinus of Valsalva aneurysm usually remains asymptomatic and undetected, affecting our ability to understand the time-related probability of size progression, aneurysm rupture, and development of aortic valve insufficiency. An early report documented no changes in an asymptomatic patient with a left sinus of Valsalva aneurysm followed for over 19 years. In contrast, an asymptomatic patient with a noncoronary sinus of Valsalva aneurysm required surgical intervention four years after diagnosis, due to developing severe aortic insufficiency.
The presentation of symptomatic unruptured sinus of Valsalva aneurysms depends on the cardiac chamber or intracardiac structure affected. For example, protrusion into the right atrium can cause tricuspid stenosis and/or regurgitation ; extension into the right ventricle can create right ventricular outflow obstruction, , , and complete heart block or ventricular tachycardia may result from the sheer mass of a large and strategically located aneurysm. , , Sinus of Valsalva aneurysms may be complicated by infective endocarditis in approximately 10% of cases.
In a recent review of the literature that included 223 case reports of ruptured sinus of Valsalva aneurysms, the most common presentation was a right aneurysm with a fistulous connection into the right chambers (73% of cases). Only 9% were asymptomatic. Aneurysmal rupture diagnosis tends to occur in the third or fourth decade of life. Patients tend to be younger when a ruptured sinus of Valsalva aneurysm is diagnosed in an asymptomatic phase. Once symptoms develop and heart failure worsens, most patients die within 1 year without surgical treatment. Occasionally, a small fistulous communication might be present at birth, which it is generally well tolerated and is not a cause of early death in pediatric patients. , ,
Approximately 20% of patients with a ruptured sinus of Valsalva aneurysm present with a VSD and AR. The natural history then becomes similar to that of VSD and AR (see Section II of Chapter 33 ). The AR becomes progressively more severe, as does prolapse of the right aortic cusp and aneurysmal sac. This process gradually reduces the size of the VSD until even an anatomically large defect becomes functionally small.
Pulmonary arterial hypertension and increased pulmonary vascular resistance therefore are rare. By the time most patients with this combination of anomalies reach age 15 to 20 years, a fixed fibrous deformity of the prolapsed cusp has developed.
Technique of operation
The many types and variations of ruptured and unruptured sinus of Valsalva aneurysms, as well as the rarity of some, make detailed description of repair of each impractical. Instead, there are some basic surgical principles that we should keep in mind when repairing sinus of Valsalva aneurysms. These include: tension-free patch closure of the fistulous communication is preferable, the aneurysmal sac should be completely removed, an occult VSD should be ruled out, the aortic valve structure and function should be assessed, and sutures should always be placed in normal tissue. Unruptured sinus of Valsalva aneurysms are repaired using an identical method as in ruptured aneurysms. A recent literature review reported that patch closure of sinus of Valsalva aneurysms was the preferred technique in 60% of cases. In some reports, a single patch was used to repair both the fistula and the VSD. The rest of the cases underwent a direct suture closure. The major concern with primary closure is the distortion of the aortic sinus geometry, resulting in aortic valve incompetence. Liu and colleagues reported that patients who underwent direct suture closure of a ruptured sinus of Valsalva aneurysm had a significantly higher incidence of AR in the early postoperative period.
The group from the Fu Wai Hospital in China published a modification to the classic Sakakibara classification system for ruptured SVA to guide the decision-making on how to approach the surgical repair. In this classification, the etiology of the lesion is no longer emphasized. Instead, the modified classification is based on the anatomic location of the protrusion/rupture. Because most SVO aneurysms rupture into the right ventricle or atrium, there is a detailed description involving these two chambers.
Ruptured right sinus of valsalva aneurysm, with ventricular septal defect
Repair of a ruptured aneurysm at the midportion of the right sinus of Valsalva with coexisting juxta-aortic VSD is described first because the surgical principles are more easily appreciated in this setting. If the aneurysm is in the rightward portion of the right sinus, the VSD is probably conoventricular (perimembranous) and would be approached through the right atrium, often with detachment of the anterior and septal leaflets of the tricuspid valve. If the aneurysm is in the leftward portion of the right sinus of Valsalva, the associated VSD in the infundibular septum would be juxta-arterial, and the approach would be through the right ventricle or pulmonary trunk. In either case, operation is usually facilitated by a combined aortic and right ventricular, pulmonary trunk, or right atrial approach. , , , ,
Initial preparations follow the usual routine (see Section III of Chapter 2 ). After median sternotomy, the pericardium is opened, and complete external evaluation of the heart is made. The protruding nipple of the ruptured aneurysm may be palpated through the free wall of the right ventricle. It is important to note that no external evidence of the aneurysm itself is usually seen, and the aortic root appears to be normal on inspection. Intraoperative transesophageal echocardiography (TEE) is useful for defining the location of the aneurysm and the cardiac chamber into which it has ruptured (see Fig. 37.9 ), and for assessing completeness of the fistula and VSD repair and severity of AR before and after repair.
CPB is established after ascending aorta and direct caval cannulation, and body temperature is reduced. The aorta is clamped promptly, caval tapes are placed and secured, the right atrium is opened through a short oblique incision, and a sump suction catheter is placed across the foramen ovale. In most cases the aortic valve is at least mildly regurgitant. The aortic root is opened transversely ( Fig. 37.10 A), and cold cardioplegic solution is infused directly into the left and right coronary ostia or retrogradely through the coronary sinus, which is cannulated directly through the opened right atrium (see Chapter 3 ).
Repair of ruptured sinus of Valsalva aneurysm into right ventricle, with ventricular septal defect (VSD). (A) Initial incision is a transverse aortotomy. The orifice of the aneurysm in the right sinus is visualized. The right ventricle is opened through a transverse incision. Care must be taken to ensure that the aortic incision does not extend into right coronary artery. (B) Windsock of ruptured aneurysm is seen overlying VSD. The thinned-out portion of the windsock containing the perforation is excised, taking care not to damage the hinge line of the right aortic valve cusp. (C) Hinge line is now visible between orifice of the aneurysm and VSD. (D) Repair is performed using one patch and inserting it through right ventriculotomy. The patch is first sutured to inferior rim of VSD, incorporating septal leaflet of tricuspid valve and avoiding conduction system, using a continuous polypropylene suture. (E) Midportion of patch is sutured to hinge line of right aortic cusp using interrupted polypropylene mattress sutures. This step is accomplished before remainder of patch is sewn into place. (F) Superior aspect of patch is sewn into place over orifice of the aneurysm in the aortic sinus, completing the repair.
