Surgery for congenital diseases of the aorta.

Congenital diseases of the aorta tend to be obstructive when they present early in life, and aneurysmal when they present later in life. The latter group also tends to be associated with connective tissue disorders and with repaired conotruncal lesions. The indications for intervention in the aneurysm group are still in evolution but are clearly age- and lesion-dependant. Disorders such as Loeys-Dietz syndrome and Turner syndrome may deserve aggressive prophylactic surgery, as well as Marfan syndrome to a lesser extent. The natural history of the dilated aorta after repair of congenital heart lesions is probably more benign than de novo aneurysms and therefore should be treated conservatively.

Multiple ruptures of left sinus of Valsalva aneurysm into main pulmonary artery.

Sinus of Valsalva aneurysm is a rare congenital glitch. We report an extremely rare case of left sinus of Valsalva aneurysm compressing and rupturing into the main pulmonary artery with 2 communications. The 34-year-old man also had severe aortic regurgitation and severe tricuspid regurgitation. He underwent successful polytetrafluoroethylene patch repair and aortic valve replacement. The rarity of this case highlights the need for a precise preoperative diagnosis, the roles of transthoracic echocardiography, transesophageal echocardiography, and 3-dimensional echocardiography, and the importance of prompt surgical management.

Quadricuspid aortic valve with sinus of Valsalva rupture.

A 22-year-old female with no medical history presented to the emergency room with 2 weeks of rapidly worsening dyspnea on exertion, orthopnea, and cough. On cardiac auscultation, she was noted to have to-and-fro murmurs and a continuous murmur with signs of right heart failure. Echocardiographic images obtained showed moderate to severe aortic regurgitation, severe tricuspid regurgitation, and a "windsock" originating in the right coronary sinus of Valsalva and terminating in the right atrium. The aortic valve had four leaflets, with the right leaflet function compromised by the ruptured sinus, causing aortic regurgitation. The patient underwent resection of the sinus aneurysm and aortic valve replacement with a bioprosthetic valve. Quadricuspid aortic valves are uncommon and are rarely associated with sinus of Valsalva aneurysm. The prevalence in the general population, clinical progression, and prognosis of this unique congenital abnormality are reviewed.

Ruptured congenital sinus of valsalva aneurysms.

BACKGROUND AND AIM OF THE STUDY: Rupture of the sinus of Valsalva is rare, and there is a higher incidence of such rupture in Oriental countries than elsewhere. The objective of this study is to present the pathologic features and the clinical outcome after surgery in such patients. METHODS: Between 1980 and 2001, a total of 17 patients (15 males and 2 females) with ruptured aneurysm of sinus of Valsalva underwent surgical intervention at the Tri-Service General Hospital, Taiwan. Their age ranged from 22 to 59 years with a mean of 33.5 years. These surgical operations made up 0.51% of the total cardiac operations (3305) performed during this period. The medical records were retrospectively reviewed. RESULTS: The origin of the ruptured aneurysm of the sinus of Valsalva was the right coronary sinus in 12 patients, the noncoronary sinus in 4, and the left coronary sinus in one patient. The aneurysms ruptured into the right ventricle in 12 patients, into the right atrium in 3 patients, and into the left ventricle in 2 patients. Associated cardiac anomalies were aortic regurgitation in eight patients, ventricular septal defect in seven, and coronary artery fistula in one patient. There was no early postoperative death and one patient underwent a second operation after aneurysm of the sinus of Valsalva (ASV) relapse. CONCLUSIONS: The operation for a ruptured aneurysm of sinus of Valsalva carries a low operative risk and results in excellent long-term survival after surgical treatment.

[Congenital Valsalva sinus aneurysm opening into the right ventricle. Two operated cases]. / L'anévrisme congénital du sinus de Valsalva rompu dans le ventricule droit. A propos de deux cas opérés.

Congenital sinus of valsalva aneurysm is a rare cardiac lesion. Once the aneurysm has ruptured, a large left-to-right shunt is created. Prognosis is grave unless operative intervention is undertaken. The authors present 2 cases (2 male) of congenital aneurysm of the right sinus of valsalva ruptured into the right ventricle. The first patient (52 years) have a history of strenuous exertion followed by acute precordial pain and dyspnea. The second patient (25 years) was asymptomatic. The diagnostic was made by 2D and color doppler echocardiography. The defect was closed with a patch with good postoperative course. The authors emphasise the possible rupture of the congenital aneurysm of the sinus of valsalva after 50 years of age. Echocardiography is certainly the best method for diagnosing the condition and for following up these patients. The prognosis after surgical repair has usually been satisfactory.

Ruptured sinus of valsalva aneurysm: early recurrence and fate of the aortic valve.

BACKGROUND: We reviewed our experience with congenital ruptured sinus of Valsalva aneurysms (RSVA) to determine patterns of early recurrence and the fate of the aortic valve (AV). METHODS: Over a 28-year period, RSVA was identified in 34 patients, (mean age 31.6 years). Primary closure of the RSVA was performed in 10 patients, and a patch employed in 24. Aortic insufficiency was present in 24 patients. AV replacement (AVR) was performed in 5 patients; AV repair in 6. RESULTS: Follow-up of 9.2 +/- 8.3 years (6 months to 24 years) was complete in all but 2 patients. Five early fistula recurrences (in 4 patients) correlated with primary rather than patch closure (p < 0.03). Kaplan-Meier survival at 10 years is 90 +/- 7%. Freedom from reoperative AVR at 10 years is 83 +/- 9%. Late AVR was performed in 6 patients for progressive aortic insufficiency due to bicuspid valve (n = 3), cusp disease of affected sinus (n = 2), or aortic root dilatation (n = 2). CONCLUSIONS: Patch closure of the RSVA should be routinely employed. A bicuspid valve may be associated with the late need for AVR.

[Rupture of a congenital right coronary sinus of Valsalva aneurysm as etiology of acute cardiac decompensation]. / Die Ruptur eines kongenitalen rechtskoronaren Sinus-Valsalva-Aneurysmas als Ursache einer akuten kardialen Dekompensation.

A 54 year old woman was admitted to a hospital because of acute thoracal pain, dyspnea, and clinical signs of overt right heart failure. Transthoracic and multiplane echocardiography established the diagnosis of a ruptured aneurysm of the right coronary sinus of valsalva into the right atrium which could be confirmed by cardiac catheterization. During heart surgery, which was done without complications, the defect was closed with a suture. Six months later echocardiography showed a normal right ventricle; the function of the aortic valve was regular. Acute heart failure may be due to a congenital structural cardiac anomaly in spite of the age of 54. In such conditions transthoracic and especially transesophageal echocardiography serve as noninvasive excellent tools to make an exact diagnosis.

Surgical repair in ruptured congenital sinus of Valsalva aneurysms: a 13-year experience.

BACKGROUND AND AIM OF THE STUDY: Rupture of congenital sinus of Valsalva aneurysm is a rare cardiac malformation that usually causes reduced cardiac performance. METHODS: Twenty patients (mean age 28.3 +/- 10.7 years; range: 14 to 55 years) with rupture of congenital sinus of Valsalva aneurysm were operated on at our institution between January 1985 and March 1999. The origin of the ruptured aneurysms was the right coronary sinus in 18 patients (90%) and the non-coronary sinus in two (10%). No ruptures were observed originating from the left coronary sinus. The aneurysms ruptured into the right ventricle in 14 patients (70%), into the right atrium in five (25%), and into the left ventricle in one patient (5%). Subarterial ventricular septal defect (VSD) was the most common associated defect (30%), and aortic insufficiency the second (20%). No correlation was found between subarterial VSDs and aortic insufficiency (p > 0.05). To achieve repair, the aorta and cavity into which the aneurysm had ruptured were opened. The aneurysmal sac was excised and the defect closed with a patch in 18 patients, and without patch in two. RESULTS: One patient died in hospital (mortality rate 5%); no late mortality was observed. Surviving patients were followed up for 4.4 +/- 3.3 years (range: 1 to 13 years). The actuarial survival rate was 95% at 12.35 years. Recurrence of fistula was observed in one patient (5%) in whom the ruptured aneurysm had been closed by primary suture without the use of a patch. At 12.35 years, the actuarial freedom from recurrence of fistula was 94.74% for all survivors, and 100% for patients with patch closure. No late complications have been observed. CONCLUSIONS: Following diagnosis of ruptured sinus of Valsalva aneurysm, surgical repair is the treatment of choice. Surgery performed as rapidly as possible after diagnosis in general leads to an excellent outcome.

[Congenital aneurysm of the sinus of Valsalva ruptured to right ventricle with a ventricular septal defect and aortic regurgitation]. / Aneurisma congénito del seno de Valsalva roto a ventrículo derecho, comunicación interventricular e insuficiencia aórtica.

We report the case of a patient with a congenital aneurysm of the sinus of Valsalva ruptured in the right ventricle, aortic valvular regurgitation and ventricular septal defect that remained asymptomatic for many years and presented clinically with severe volume overload of the heart and congestive heart failure. We describe the anatomic, echocardiographic and angiographic findings of this unusual entity with a review of the literature.

An unusual case of ruptured aneurysm of sinus of Valsalva.

Ruptured congenital aneurysms of the noncoronary aortic sinus shunting into both the right atrium and right ventricle are extremely rare. We present here such an anomaly in a 40-year-old man, focusing on the diagnostic reliability of echocardiography and the unusual angiographic features of the aortic sinus aneurysm in this patient.

[Fatal outcome of a congenital aneurysm of the right sinus valsalvae ruptured into the right atrium]. / Letaler Verlauf eines in den rechten Vorhof rupturierten kongenitalen Aneurysmas des rechten Sinus Valsalvae.

In a 24-year-old female patient with sudden collapse an acute tricuspid insufficiency was presumed by echocardiography after the exclusion of pulmonary embolism. Aortography, however, revealed an aneurysm of the right sinus of Valsalva which had ruptured into the right atrium. Despite emergency surgery the patient died in protracted cardiac shock. Biopsy and autopsy showed pathognomonic findings of a congenital aneurysm (medial degeneration, reduction of elastic lamellae). Aneurysms of the sinuses of Valsalva are mainly caused by a rare connatal defect of the aortic wall; the right aortic sinus is involved most frequently, the sudden rupture uncovers the lesion. The characteristics of the reported case are compared with the literature under special consideration of etiology and pathologic anatomy of this unusual entity.

[Ruptured congenital aneurysm of the sinus of valsalva in the aged].

Ruptured aneurysm of the sinus of Valsalva is a rare cardiac lesion. A ruptured aneurysm of the sinus of Valsalva in the right ventricle of a 64-year-old man was successfully repaired. The patient was admitted to the hospital with high fever and chest oppression. Diagnosis was made by two dimensional echocardiography, cardiac catheterization, and cardiac angiography. An aortotomy, main pulmonary arteriotomy, and right ventriculotomy were performed. There was no VSD, and the aneurysm originated from the right coronary sinus, rupturing into the right ventricle inlet portion. The ruptured aneurysm of the sinus of Valsalva was closed with a Dacron patch from inside the aorta. He is doing well after surgery. There was no heart murmur. CTR decreased and pulmonary blood flow fell to a normal value. As far as we know, this patient is the second oldest patient in Japan with surgical repair.

[Congenital aneurysm of Valsalva's sinus rupturing into the left ventricle. Apropos of a case in a 3-year-old child]. / Anévrysme congénital du sinus de Valsalva rompu dans le ventricule gauche. A propos d'une observation chez un enfant de 3 ans.

The authors present the case of a 3 year old child with cardiac failure of recent onset, arterial hyperpulsatility and a systolo-diastolic murmur. Aortic angiographic showed an aneurysm of the sinus of Valsalva and valvular aortic regurgitation. At surgery the aneurysm was found to have ruptured into the left ventricle. The fistula was closed. The postoperative course has been satisfactory with a 20 month follow-up period, despite some residual aortic regurgitation. This is the first published pediatric case of a congenital sinus of Valsalva aneurysm rupturing into the left ventricle. The case is discussed and compared with 6 other cases in the literature concerning adults.

Echocardiographic diagnosis of congenital sinus of Valsalva aneurysm with dissection of the interventricular septum.

A case is reported in which a congenital aneurysm of the right coronary sinus of Valsalva ruptured and dissected into the interventricular septum. M-mode and cross-sectional echocardiographic examination allowed accurate preoperative assessment of the pathologic anatomy, which was confirmed by angiography, surgery and autopsy. Dissection of the interventricular septum by a congenital sinus of Valsalva aneurysm is a rare lesion, has a poor prognosis, and can be diagnosed noninvasively with echocardiography.

Ruptured congenital aneurysm of the right sinus of Valsalva into the right ventricle: with special reference to pathoanatomic and hemodynamic characteristics in symptomless cases.

Clinical features of 19 cases with congenital aneurysm of the right sinus of Valsalva rupturing into the right ventricular outflow region (Type 1) were analysed in relation to their pathoanatomic lesions and hemodynamic alterations. Sixteen cases were operated with one surgical death. All were catheterized together with ascending aortographic study. Rupture of the aneurysm in many cases was silent or symptomless and progressive heart failure was not quite common. Symptomatology of the patients did not seem to be related entirely to status of the pathoanatomical lesions or hemodynamic alterations. Time of the rupture, and inherent right ventricular characteristics, tolerating volume overload rather well, might be, in part, responsible for its better prognosis in some cases. However, all patients with ruptured aneurysm of the sinus of Valsalva should be treated surgically. Bacterial endocarditis is a serious complication leading to death.