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1.  Ruptured Aneurysm of the Right Sinus of Valsalva Associated with a Ventricular Septal Defect and an Anomalous Coronary Artery 
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.
PMCID: PMC1351829  PMID: 16429902
Aortic aneurysm/congenital; aortic rupture/surgery; heart defects; congenital; heart septal defects; ventricular; coronary vessel anomalies/surgery; sinus of Valsalva/abnormalities/surgery
2.  Endovascular Repair of Abdominal Aortic Aneurysm 
EXECUTIVE SUMMARY
The Medical Advisory Secretariat conducted a systematic review of the evidence on the effectiveness and cost-effectiveness of endovascular repair of abdominal aortic aneurysm in comparison to open surgical repair. An abdominal aortic aneurysm [AAA] is the enlargement and weakening of the aorta (major blood artery) that may rupture and result in stroke and death. Endovascular abdominal aortic aneurysm repair [EVAR] is a procedure for repairing abdominal aortic aneurysms from within the blood vessel without open surgery. In this procedure, an aneurysm is excluded from blood circulation by an endograft (a device) delivered to the site of the aneurysm via a catheter inserted into an artery in the groin. The Medical Advisory Secretariat conducted a review of the evidence on the effectiveness and cost-effectiveness of this technology. The review included 44 eligible articles out of 489 citations identified through a systematic literature search. Most of the research evidence is based on non-randomized comparative studies and case series. In the short-term, EVAR appears to be safe and comparable to open surgical repair in terms of survival. It is associated with less severe hemodynamic changes, less blood transfusion and shorter stay in the intensive care and hospital. However, there is concern about a high incidence of endoleak, requiring secondary interventions, and in some cases, conversion to open surgical repair. Current evidence does not support the use of EVAR in all patients. EVAR might benefit individuals who are not fit for surgical repair of abdominal aortic aneurysm and whose risk of rupture of the aneurysm outweighs the risk of death from EVAR. The long-term effectiveness and cost-effectiveness of EVAR cannot be determined at this time. Further evaluation of this technology is required.
OBJECTIVE
The objective of this health technology policy assessment was to determine the effectiveness and cost-effectiveness of endovascular repair of abdominal aortic aneurysms (EVAR) in comparison to open surgical repair (OSR).
BACKGROUND
Clinical Need
An abdominal aortic aneurysm (AAA) is a localized, abnormal dilatation of the aorta greater than 3 cm or 50% of the aortic diameter at the diaphragm. (1) A true AAA involves all 3 layers of the vessel wall. If left untreated, the continuing extension and thinning of the vessel wall may eventually result in rupture of the AAA. The risk of death from ruptured AAA is 80% to 90%. (61) Heller et al. (44) analyzed information from a national hospital database in the United States. They found no significant change in the incidence rate of elective AAA repair or ruptured AAA presented to the nation’s hospitals. The investigators concluded that technologic and treatment advances over the past 19 years have not affected the outcomes of patients with AAAs, and the ability to identify and to treat patients with AAAs has not improved.
Classification of Abdominal Aortic Aneurysms
At least 90% of the AAAs are affected by atherosclerosis, and most of these aneurysms are below the level of the renal arteries.(1)
An abdominal aortic aneurysm may be symptomatic or asymptomatic. An AAA may be classified according to their sizes:(7)
Small aneurysms: less than 5 cm in diameter.
Medium aneurysms: 5-7cm.
Large aneurysms: more than 7 cm in diameter.
Small aneurysms account for approximately 50% of all clinically recognized aneurysms.(7)
Aortic aneurysms may be classified according to their gross appearance as follows (1):
Fusiform aneurysms affect the entire circumference of a vessel, resulting in a diffusely dilated lesion
Saccular aneurysms involve only a portion of the circumference, resulting in an outpouching (protrusion) in the vessel wall.
Prevalence of Abdominal Aortic Aneurysms
In community surveys, the prevalence of AAA is reported to be between 1% and 5.4%. (61) The prevalence is related to age and vascular risk factors. It is more common in men and in those with a positive family history.
In Canada, Abdominal aortic aneurysms are the 10th leading cause of death in men 65 years of age or older. (60) Naylor (60) reported that the rate of AAA repair in Ontario has increased from 38 per 100,000 population in 1981/1982 to 54 per 100,000 population in 1991/1992. For the period of 1989/90 to 1991/92, the rate of AAA repair in Ontarians age 45 years and over was 53 per 100,000. (60) In the United States, about 200,000 new cases are diagnosed each year, and 50,000 to 60,000 surgical AAA repairs are performed. (2) Ruptured AAAs are responsible for about 15,000 deaths in the United States annually. One in 10 men older than 80 years has some aneurysmal change in his aorta. (2)
Symptoms of Abdominal Aortic Aneurysms
AAAs usually do not produce symptoms. However, as they expand, they may become painful. Compression or erosion of adjacent tissue by aneurysms also may cause symptoms. The formation of mural thrombi, a type of blood clots, within the aneurysm may predispose people to peripheral embolization, where blood vessels become blocked. Occasionally, an aneurysm may leak into the vessel wall and the periadventitial area, causing pain and local tenderness. More often, acute rupture occurs without any prior warning, causing acute pain and hypotension. This complication is always life-threatening and requires an emergency operation.
Diagnosis of Abdominal Aortic Aneurysms
An AAA is usually detected on routine examination as a palpable, pulsatile, and non-tender mass. (1)
Abdominal radiography may show the calcified outline of the aneurysms; however, about 25% of aneurysms are not calcified and cannot be visualized by plain x-ray. (1) An abdominal ultrasound provides more accurate detection, can delineate the traverse and longitudinal dimensions of the aneurysm, and is useful for serial documentation of aneurysm size. Computed tomography and magnetic resonance have also been used for follow-up of aortic aneurysms. These technologies, particularly contrast-enhanced computer tomography, provide higher resolution than ultrasound.
Abdominal aortography remains the gold standard to evaluate patients with aneurysms for surgery. This technique helps document the extent of the aneurysms, especially their upper and lower limits. It also helps show the extent of associated athereosclerotic vascular disease. However, the procedure carries a small risk of complications, such as bleeding, allergic reactions, and atheroembolism. (1)
Prognosis of Abdominal Aortic Aneurysms
The risk of rupture of an untreated AAA is a continuous function of aneurysm size as represented by the maximal diameter of the AAA. The annual rupture rate is near zero for aneurysms less than 4 cm in diameter. The risk is about 1% per year for aneurysms 4 to 4.9 cm, 11% per year for aneurysms 5 to 5.9 cm, and 25% per year or more for aneurysms greater than 6 cm. (7)
The 1-year mortality rate of patients with AAAs who do not undergo surgical treatment is about 25% if the aneurysms are 4 to 6 cm in diameter. This increases to 50% for aneurysms exceeding 6 cm. Other major causes of mortality for people with AAAs include coronary heart disease and stroke.
Treatment of Abdominal Aortic Aneurysms
Treatment of an aneurysm is indicated under any one of the following conditions:
The AAA is greater than 6 cm in diameter.
The patient is symptomatic.
The AAA is rapidly expanding irrespective of the absolute diameter.
Open surgical repair of AAA is still the gold standard. It is a major operation involving the excision of dilated area and placement of a sutured woven graft. The surgery may be performed under emergent situation following the rupture of an AAA, or it may be performed electively.
Elective OSR is generally considered appropriate for healthy patients with aneurysms 5 to 6 cm in diameter. (7) Coronary artery disease is the major underlying illness contributing to morbidity and mortality in OSR. Other medical comorbidities, such as chronic renal failure, chronic lung disease, and liver cirrhosis with portal hypertension, may double or triple the usual risk of OSR.
Serial noninvasive follow-up of small aneurysms (less than 5 cm) is an alternative to immediate surgery.
Endovascular repair of AAA is the third treatment option and is the topic of this review.
PMCID: PMC3387737  PMID: 23074438
3.  Eosinophilic myocarditis mimicking acute coronary syndrome secondary to idiopathic hypereosinophilic syndrome: a case report 
Introduction
Eosinophilic myocarditis is a rare form of myocarditis. It is characterized pathologically by diffuse or focal myocardial inflammation with eosinophilic infiltration, often in association with peripheral blood eosinophilia. We report a case of eosinophilic myocarditis secondary to hypereosinophilic syndrome.
Case presentation
A 74-year-old Caucasian woman with a history of asthma, paroxysmal atrial fibrillation, stroke and coronary artery disease presented to the emergency department of our hospital with chest pain. Evaluations revealed that she had peripheral blood eosinophilia and elevated cardiac enzymes. Electrocardiographic findings were nonspecific. Her electrocardiographic finding and elevated cardiac enzymes pointed to a non-ST-elevated myocardial infarction. Echocardiogram showed a severe decrease in the left ventricular systolic function. Coronary angiogram showed nonobstructive coronary artery disease. She then underwent cardiac magnetic resonance imaging, which showed neither infiltrative myocardial diseases nor any evidence of infarction. This was followed by an endomyocardial biopsy which was consistent with eosinophilic myocarditis. Hematologic workup regarding her eosinophilia was consistent with hypereosinophilic syndrome. After being started on steroid therapy, her peripheral eosinophilia resolved and her symptoms improved. Her left ventricular ejection fraction, however, did not improve.
Conclusion
Eosinophilic myocarditis can present like an acute myocardial infarction and should be considered in the differential diagnosis of acute coronary syndrome in patients with a history of allergy, asthma or acute reduction of the left ventricular function with or without peripheral eosinophilia.
doi:10.1186/1752-1947-4-40
PMCID: PMC2830978  PMID: 20181108
4.  Chronic periaortitis (retroperitoneal fibrosis) concurrent with giant cell arteritis: a case report 
Introduction
Giant cell arteritis is the most common form of large-vessel vasculitides. However, it is probable that extracranial involvement is underdiagnosed in patients with classical giant cell arteritis. In the recent literature most cases of giant cell arteritis have been described in conjunction with aortic aneurysms or dissections. Nonetheless the coexistence of giant cell arteritis and retroperitoneal fibrosis is extremely rare. Here, we describe a case of giant cell arteritis at a very early clinical stage, in a woman with coexistence of retroperitoneal fibrosis.
Case presentation
We report a case of giant cell arteritis at a very early clinical stage, in a 47-year-old Greek woman with coexistence of retroperitoneal fibrosis who was admitted to our hospital with a history of high-grade fever and mild right periumbilical abdominal pain for the past 30 days. In the context of fever of unknown origin, an abdomen computed tomography was ordered. A temporal artery biopsy was also performed because during hospitalization she complained of a headache. Examination of eosin and hematoxylin slides from biopsy specimens of her temporal artery, showed lesions consisting of predominantly lymphocytes, few plasma cells and occasional polymorphonuclear leucocytes. In addition no giant cells were detected in examining biopsies at multiple levels. This was consistent with giant cell arteritis according to the American college of Rheumatology criteria. An abdomen computed tomography revealed the presence of a retroperitoneal soft-tissue mass located anteriorly to the upper infrarenal aorta at the site of the scintigraphic uptake. The computed tomography and magnetic resonance imaging characteristics of the mass were consistent with retroperitoneal fibrosis, and its morphology suggestive of benignity. Our patient started oral prednisolone and was afebrile from day one.
Conclusions
In our experience this is the first case of retroperitoneal fibrosis due to giant cell arteritis occurring at the same time. Involvement of the aorta (aortitis) and its branches has been also observed in a subset of patients with giant cell arteritis. In addition, giant cell arteritis has been associated with a markedly increased risk of aortic aneurysm particularly thoracic aortic aneurysm.
doi:10.1186/1752-1947-8-167
PMCID: PMC4070639  PMID: 24885445
Giant cell arteritis; Periaortitis; Retroperitoneal fibrosis
5.  Unruptured Aneurysm of the Left Sinus of Valsalva Causing Coronary Insufficiency 
Texas Heart Institute Journal  2002;29(1):40-44.
Aneurysm of the left sinus of Valsalva is extremely rare. Compression of the left coronary artery by such an aneurysm is an unusual complication of this condition and can cause coronary insufficiency.
We describe the case of a 75-year-old woman who had an isolated unruptured aneurysm of the left coronary sinus with intraluminal thrombus, which caused coronary artery compression. We performed successful surgical correction by closing the mouth of the aneurysm without aortic valve replacement or coronary artery bypass grafting. A review of the world medical literature revealed 19 cases of sinus of Valsalva aneurysms that hindered the coronary arterial flow. The previously published reports of this rare condition and its treatment are discussed herein. (Tex Heart Inst J 2002;29:40–4)
PMCID: PMC101268  PMID: 11995849
Angina pectoris/etiology; aortic aneurysm/complications/diagnosis/surgery; coronary arteries; heart defects, congenital; myocardial ischemia/etiology; sinus of Valsalva
6.  Aortic Valve Regurgitation that Resolved after a Ruptured Coronary Sinus Aneurysm Was Patched 
Texas Heart Institute Journal  2013;40(4):489-492.
Sinus of Valsalva aneurysms appear to be rare. They occur most frequently in the right sinus of Valsalva (52%) and the noncoronary sinus (33%). More of these aneurysms originate from the right coronary cusp than from the noncoronary cusp. Surgical intervention is usually recommended when symptoms become evident.
We report the case of a 34-year-old woman who presented with a congenital, ruptured sinus of Valsalva aneurysm that originated from the noncoronary cusp. Moderate aortic regurgitation was associated with this lesion. Simple, direct patch closure of the ruptured aneurysm resolved the patient's left-to-right shunt and was associated with decreased aortic regurgitation to a degree that valve replacement was not necessary. Only trace residual aortic regurgitation was evident after 3 months, and the patient remained free of symptoms after 6 months.
Our observations support the idea that substantial runoff blood flow in the immediate supra-annular region can be responsible for aortic regurgitation in the absence of a notable structural defect in the aortic valve, and that restoring physiologic flow in this region and equalizing aortic-cusp closure pressure can largely or completely resolve aortic insufficiency. Accordingly, valve replacement may not be necessary in all cases of ruptured sinus of Valsalva aneurysms with associated aortic valve regurgitation.
WEBSITE FEATURE
PMCID: PMC3783149  PMID: 24082388
Aortic aneurysm/congenital/etiology/radiography/surgery; aortic valve insufficiency; cardiac surgical procedures/methods; differential diagnosis; sinus of Valsalva/pathology/radiography/surgery; treatment outcome
7.  Depiction of ruptured sinus of Valsalva aneurysms by cardiac computed tomography angiography 
Ruptured sinus of Valsalva aneurysm is an uncommon condition with variable manifestation that results in aortocardiac shunt. The presentation may range from an asymptomatic murmur to cardiogenic shock. The initial diagnosis is established or suspected by two-dimensional echocardiography and colour flow Doppler. Transesophageal echocardiography is especially helpful in delineating the anatomy of the aneurysm and its connections to other chambers. The gold standard diagnostic method for ruptured sinus of Valsalva aneurysm is cardiac catheterization and aortography. Recent reports have suggested a potential role of cardiac computed tomography in establishing diagnosis in such cases. The high spatial resolution of cardiac computed tomography provides anatomical details of the ruptured aneurysm by depicting a jet of contrast materials extending from the aneurysm and adjacent cardiac chamber. In addition, cardiac computed tomography provides a comprehensive cardiac evaluation including coronary artery anatomy, and the presence of other associated cardiac or vascular anomalies.
PMCID: PMC3628434  PMID: 23620709
Cardiac computed tomography; Cardiac imaging; Congenital heart disease; Ruptured sinus of Valsalva aneurysm
8.  Pulmonary valve endocarditis caused by right ventricular outflow obstruction in association with sinus of valsalva aneurysm: a case report 
Background
Right-sided infective endocarditis is uncommon. This is primarily seen in patients with intravenous drug use, pacemaker or central venous lines, or congenital heart disease. The vast majority of cases involve the tricuspid valve. Isolated pulmonary valve endocarditis is extremely rare. We report the first case of a pulmonary valve nonbacterial thrombotic endocarditis caused by right ventricular outlflow tract (RVOT) obstruction in association with a large sinus of Valsalva aneurysm.
Case presentation
A 60-year-old man with a six-week history of fever, initially treated as pneumonia and sinusitis with levofloxacin, was admitted to the hospital with a new onset of a heart murmur. An echocardiogram showed thickening of the pulmonary valve suggestive of valve vegetation. A dilated aortic root and sinus of Valsalva aneurysm measuring at least 6.4 cm were also identified. The patient was empirically treated for infective endocarditis with vancomycin and gentamycin for 28 days. Four months later, the patient underwent resection of a large aortic root aneurysm and exploration of the pulmonary valve. During the surgery, vegetation of the pulmonary valve was confirmed. Microscopic pathological examination revealed fibrinous debris with acute inflammation and organizing fibrosis with chronic inflammation, compatible with a vegetation. Special stains were negative for bacteria and fungi.
Conclusion
This is the first case report of a pulmonary valve nonbacterial endocarditis caused by RVOT obstruction in association with a sinus of Valsalva aneurysm. We speculate that jets created by the RVOT obstruction and large sinus of Valsalva aneurysm hitting against endothelium of the pulmonary valve is the etiology of this rare nonbacterial thrombotic endocarditis.
doi:10.1186/1749-8090-3-46
PMCID: PMC2491616  PMID: 18627635
9.  Coil Embolization for Intracranial Aneurysms 
Executive Summary
Objective
To determine the effectiveness and cost-effectiveness of coil embolization compared with surgical clipping to treat intracranial aneurysms.
The Technology
Endovascular coil embolization is a percutaneous approach to treat an intracranial aneurysm from within the blood vessel without the need of a craniotomy. In this procedure, a microcatheter is inserted into the femoral artery near the groin and navigated to the site of the aneurysm. Small helical platinum coils are deployed through the microcatheter to fill the aneurysm, and prevent it from further expansion and rupture. Health Canada has approved numerous types of coils and coil delivery systems to treat intracranial aneurysms. The most favoured are controlled detachable coils. Coil embolization may be used with other adjunct endovascular devices such as stents and balloons.
Background
Intracranial Aneurysms
Intracranial aneurysms are the dilation or ballooning of part of a blood vessel in the brain. Intracranial aneurysms range in size from small (<12 mm in diameter) to large (12–25 mm), and to giant (>25 mm). There are 3 main types of aneurysms. Fusiform aneurysms involve the entire circumference of the artery; saccular aneurysms have outpouchings; and dissecting aneurysms have tears in the arterial wall. Berry aneurysms are saccular aneurysms with well-defined necks.
Intracranial aneurysms may occur in any blood vessel of the brain; however, they are most commonly found at the branch points of large arteries that form the circle of Willis at the base of the brain. In 85% to 95% of patients, they are found in the anterior circulation. Aneurysms in the posterior circulation are less frequent, and are more difficult to treat surgically due to inaccessibility.
Most intracranial aneurysms are small and asymptomatic. Large aneurysms may have a mass effect, causing compression on the brain and cranial nerves and neurological deficits. When an intracranial aneurysm ruptures and bleeds, resulting in a subarachnoid hemorrhage (SAH), the mortality rate can be 40% to 50%, with severe morbidity of 10% to 20%. The reported overall risk of rupture is 1.9% per year and is higher for women, cigarette smokers, and cocaine users, and in aneurysms that are symptomatic, greater than 10 mm in diameter, or located in the posterior circulation. If left untreated, there is a considerable risk of repeat hemorrhage in a ruptured aneurysm that results in increased mortality.
In Ontario, intracranial aneurysms occur in about 1% to 4% of the population, and the annual incidence of SAH is about 10 cases per 100,000 people. In 2004-2005, about 660 intracranial aneurysm repairs were performed in Ontario.
Treatment of Intracranial Aneurysms
Treatment of an unruptured aneurysm attempts to prevent the aneurysm from rupturing. The treatment of a ruptured intracranial aneurysm aims to prevent further hemorrhage. There are 3 approaches to treating an intracranial aneurysm.
Small, asymptomatic aneurysms less than 10 mm in diameter may be monitored without any intervention other than treatment for underlying risk factors such as hypertension.
Open surgical clipping, involves craniotomy, brain retraction, and placement of a silver clip across the neck of the aneurysm while a patient is under general anesthesia. This procedure is associated with surgical risks and neurological deficits.
Endovascular coil embolization, introduced in the 1990s, is the health technology under review.
Literature Review
Methods
The Medical Advisory Secretariat searched the International Health Technology Assessment (INAHTA) Database and the Cochrane Database of Systematic Reviews to identify relevant systematic reviews. OVID Medline, Medline In-Process and Other Non-Indexed Citations, and Embase were searched for English-language journal articles that reported primary data on the effectiveness or cost-effectiveness of treatments for intracranial aneurysms, obtained in a clinical setting or analyses of primary data maintained in registers or institutional databases. Internet searches of Medscape and manufacturers’ databases were conducted to identify product information and recent reports on trials that were unpublished but that were presented at international conferences. Four systematic reviews, 3 reports on 2 randomized controlled trials comparing coil embolization with surgical clipping of ruptured aneurysms, 30 observational studies, and 3 economic analysis reports were included in this review.
Results
Safety and Effectiveness
Coil embolization appears to be a safe procedure. Complications associated with coil embolization ranged from 8.6% to 18.6% with a median of about 10.6%. Observational studies showed that coil embolization is associated with lower complication rates than surgical clipping (permanent complication 3-7% versus 10.9%; overall 23% versus 46% respectively, p=0.009). Common complications of coil embolization are thrombo-embolic events (2.5%–14.5%), perforation of aneurysm (2.3%–4.7%), parent artery obstruction (2%–3%), collapsed coils (8%), coil malposition (14.6%), and coil migration (0.5%–3%).
Randomized controlled trials showed that for ruptured intracranial aneurysms with SAH, suitable for both coil embolization and surgical clipping (mostly saccular aneurysms <10 mm in diameter located in the anterior circulation) in people with good clinical condition:Coil embolization resulted in a statistically significant 23.9% relative risk reduction and 7% absolute risk reduction in the composite rate of death and dependency compared to surgical clipping (modified Rankin score 3–6) at 1-year.
The advantage of coil embolization over surgical clipping varies widely with aneurysm location, but endovascular treatment seems beneficial for all sites.
There were less deaths in the first 7 years following coil embolization compared to surgical clipping (10.8% vs 13.7%). This survival benefit seemed to be consistent over time, and was statistically significant (log-rank p= 0.03).
Coil embolization is associated with less frequent MRI-detected superficial brain deficits and ischemic lesions at 1-year.
The 1- year rebleeding rate was 2.4% after coil embolization and 1% for surgical clipping. Confirmed rebleeding from the repaired aneurysm after the first year and up to year eight was low and not significantly different between coil embolization and surgical clipping (7 patients for coil embolization vs 2 patients for surgical clipping, log-rank p=0.22).
Observational studies showed that patients with SAH and good clinical grade had better 6-month outcomes and lower risk of symptomatic cerebral vasospasm after coil embolization compared to surgical clipping.
For unruptured intracranial aneurysms, there were no randomized controlled trials that compared coil embolization to surgical clipping. Large observational studies showed that:
The risk of rupture in unruptured aneurysms less than 10 mm in diameter is about 0.05% per year for patients with no pervious history of SAH from another aneurysm. The risk of rupture increases with history of SAH and as the diameter of the aneurysm reaches 10 mm or more.
Coil embolization reduced the composite rate of in hospital deaths and discharge to long-term or short-term care facilities compared to surgical clipping (Odds Ratio 2.2, 95% CI 1.6–3.1, p<0.001). The improvement in discharge disposition was highest in people older than 65 years.
In-hospital mortality rate following treatment of intracranial aneurysm ranged from 0.5% to 1.7% for coil embolization and from 2.1% to 3.5% for surgical clipping. The overall 1-year mortality rate was 3.1% for coil embolization and 2.3% for surgical clipping. One-year morbidity rate was 6.4% for coil embolization and 9.8% for surgical clipping. It is not clear whether these differences were statistically significant.
Coil embolization is associated with shorter hospital stay compared to surgical clipping.
For both ruptured and unruptured aneurysms, the outcome of coil embolization does not appear to be dependent on age, whereas surgical clipping has been shown to yield worse outcome for patients older than 64 years.
Angiographic Efficiency and Recurrences
The main drawback of coil embolization is its low angiographic efficiency. The percentage of complete aneurysm occlusion after coil embolization (27%–79%, median 55%) remains lower than that achieved with surgical clipping (82%–100%). However, about 90% of coiled aneurysms achieve near total occlusion or better. Incompletely coiled aneurysms have been shown to have higher aneurysm recurrence rates ranging from 7% to 39% for coil embolization compared to 2.9% for surgical clipping. Recurrence is defined as refilling of the neck, sac, or dome of a successfully treated aneurysm as shown on an angiogram. The long-term clinical significance of incomplete occlusion following coil embolization is unknown, but in one case series, 20% of patients had major recurrences, and 50% of these required further treatment.
Long-Term Outcomes
A large international randomized trial reported that the survival benefit from coil embolization was sustained for at least 7 years. The rebleeding rate between year 2 and year 8 following coil embolization was low and not significantly different from that of surgical clipping. However, high quality long-term angiographic evidence is lacking. Accordingly, there is uncertainty about long-term occlusion status, coil durability, and recurrence rates. While surgical clipping is associated with higher immediate procedural risks, its long-term effectiveness has been established.
Indications and Contraindications
Coil embolization offers treatment for people at increased risk for craniotomy, such as those over 65 years of age, with poor clinical status, or with comorbid conditions. The technology also makes it possible to treat surgical high-risk aneurysms.
Not all aneurysms are suitable for coil embolization. Suitability depends on the size, anatomy, and location of the aneurysm. Aneurysms more than 10 mm in diameter or with an aneurysm neck greater than or equal to 4 mm are less likely to achieve total occlusion. They are also more prone to aneurysm recurrences and to complications such as coil compaction or parent vessel occlusion. Aneurysms with a dome to neck ratio of less than 1 have been shown to have lower obliteration rates and poorer outcome following coil embolization. Furthermore, aneurysms in the middle cerebral artery bifurcation are less suitable for coil embolization. For some aneurysms, treatment may require the use of both coil embolization and surgical clipping or adjunctive technologies, such as stents and balloons, to obtain optimal results.
Diffusion
Information from 3 countries indicates that coil embolization is a rapidly diffusing technology. For example, it accounted for about 40% of aneurysm treatments in the United Kingdom.
In Ontario, coil embolization is an insured health service, with the same fee code and fee schedule as open surgical repair requiring craniotomy. Other costs associated with coil embolization are covered under hospitals’ global budgets. Utilization data showed that in 2004-2005, coil embolization accounted for about 38% (251 cases) of all intracranial aneurysm repairs in the province. With the 2005 publication of the positive long-term survival data from the International Subarachnoid Aneursym Trial, the pressure for diffusion will likely increase.
Economic Analysis
Recent economic studies show that treatment of unruptured intracranial aneurysms smaller than 10 mm in diameter in people with no previous history of SAH, either by coil embolization or surgical clipping, would not be effective or cost-effective. However, in patients with aneurysms that are greater than or equal to 10 mm or symptomatic, or in patients with a history of SAH, treatment appears to be cost-effective.
In Ontario, the average device cost of coil embolization per case was estimated to be about $7,500 higher than surgical clipping. Assuming that the total number of intracranial aneurysm repairs in Ontario increases to 750 in the fiscal year of 2007, and assuming that up to 60% (450 cases) of these will be repaired by coil embolization, the difference in device costs for the 450 cases (including a 15% recurrence rate) would be approximately $3.8 million. This figure does not include capital costs (e.g. $3 million for an angiosuite), additional human resources required, or costs of follow-up. The increase in expenditures associated with coil embolization may be offset partially, by shorter operating room times and hospitalization stays for endovascular repair of unruptured aneurysms; however, the impact of these cost savings is probably not likely to be greater than 25% of the total outlay since the majority of cases involve ruptured aneurysms. Furthermore, the recent growth in aneurysm repair has predominantly been in the area of coil embolization presumably for patients for whom surgical clipping would not be advised; therefore, no offset of surgical clipping costs could be applied in such cases. For ruptured aneurysms, downstream cost savings from endovascular repair are likely to be minimal even though the savings for individual cases may be substantial due to lower perioperative complications for endovascular aneurysm repair.
Guidelines
The two Guidance documents issued by the National Institute of Clinical Excellence (UK) in 2005 support the use of coil embolization for both unruptured and ruptured (SAH) intracranial aneurysms, provided that procedures are in place for informed consent, audit, and clinical governance, and that the procedure is performed in specialist units with expertise in the endovascular treatment of intracranial aneurysms.
Conclusion
For people in good clinical condition following subarachnoid hemorrhage from an acute ruptured intracranial aneurysm suitable for either surgical clipping or endovascular repair, coil embolization results in improved independent survival in the first year and improved survival for up to seven years compared to surgical clipping. The rebleeding rate is low and not significantly different between the two procedures after the first year. However, there is uncertainty regarding the long-term occlusion status, durability of the stent graft, and long-term complications.
For people with unruptured aneurysms, level 4 evidence suggests that coil embolization may be associated with comparable or less mortality and morbidity, shorter hospital stay, and less need for discharge to short-term rehabilitation facilities. The greatest benefit was observed in people over 65 years of age. In these patients, the decision regarding treatment needs to be based on the assessment of the risk of rupture against the risk of the procedure, as well as the morphology of the aneurysm.
In people who require treatment for intracranial aneurysm, but for whom surgical clipping is too risky or not feasible, coil embolization provides survival benefits over surgical clipping, even though the outcomes may not be as favourable as in people in good clinical condition and with small aneurysms. The procedure may be considered under the following circumstances provided that the aneurysm is suitable for coil embolization:
Patients in poor/unstable clinical or neurological state
Patients at high risk for surgical repair (e.g. people>age 65 or with comorbidity), or
Aneurysm(s) with poor accessibility or visibility for surgical treatment due to their location (e.g. ophthalmic or basilar tip aneurysms)
Compared to small aneurysms with a narrow neck in the anterior circulation, large aneurysms (> 10 mm in diameter), aneurysms with a wide neck (>4mm in diameter), and aneurysms in the posterior circulation have lower occlusion rates and higher rate of hemorrhage when treated with coil embolization.
The extent of aneurysm obliteration after coil embolization remains lower than that achieved with surgical clipping. Aneurysm recurrences after successful coiling may require repeat treatment with endovascular or surgical procedures. Experts caution that long-term angiographic outcomes of coil embolization are unknown at this time. Informed consent for and long-term follow-up after coil embolization are recommended.
The decision to treat an intracranial aneurysm with surgical clipping or coil embolization needs to be made jointly by the neurosurgeon and neuro-intervention specialist, based on the clinical status of the patient, the size and morphology of the aneurysm, and the preference of the patient.
The performance of endovascular coil embolization should take place in centres with expertise in both neurosurgery and endovascular neuro-interventions, with adequate treatment volumes to maintain good outcomes. Distribution of the technology should also take into account that patients with SAH should be treated as soon as possible with minimal disruption.
PMCID: PMC3379525  PMID: 23074479
10.  Ruptured Aneurysm of the Noncoronary Sinus of Valsalva 
Sinus of Valsalva aneurysm is a rare disorder. It is usually congenital, but other origins have been described. It may be asymptomatic, or it may present as angina or with symptoms of valvular insufficiency or outflow obstruction. Once ruptured, it often produces hemodynamic instability. Diagnosis should be pursued with transesophageal echocardiography or catheterization.
A 50-year-old man was admitted with symptoms of congestive heart failure. His functional capacity had declined over 3 years, worsening over the last 3 weeks. His medical history was significant only for gunshot to the right hemithorax, at age 25. He was found to be in distress, with signs of heart failure, S3 and S4 gallop, and systolic and diastolic murmur at the right sternal border. Two-dimensional echocardiography revealed an ejection fraction of 0.25, dilated left and right atria, and a sinus of Valsalva aneurysm with an aortic–right atrial shunt. Left and right heart catheterization revealed metallic fragments in the anterior chest wall, normal coronary arteries, and a step-up in the oxygen saturation from the inferior vena cava to the pulmonary artery. Aortography revealed that the aneurysm had ruptured into the right atrium. Surgical repair was performed.
Ruptured sinus of Valsalva aneurysm demands prompt diagnosis and treatment. Patients are often men, in the 3rd or 4th decade of life when rupture occurs. In our patient, the position of the bullet and its fragments, leaflet perforation, and lack of a predisposing infection suggest a causal association between the gunshot wound and the aneurysm.
PMCID: PMC1351838  PMID: 16429911
Aortic aneurysm; aortic rupture; aortic valve/surgery; sinus of Valsalva/surgery; wounds; gunshot
11.  Giant aneurysm of the left main coronary artery in Takayasu aortitis 
Heart  1999;81(2):214-217.
Takayasu aortitis is a chronic inflammatory vasculitis characterised by stenosis or obliteration of large and medium sized arteries. Although coronary arteries are affected in approximately 10% of cases, most of the lesions are luminal narrowing, and coronary aneurysm formation is extremely rare. A case is described of giant aneurysm of the left main coronary artery complicated with Takayasu aortitis in a 46 year old Japanese woman who was followed until her death at age 71. Pronounced intimal proliferation and adventitial fibrous thickening of the involved arterial wall usually induce constriction or occlusion at the orifice of the main branch of the aorta in Takayasu aortitis. However, systemic hypertension, which resulted from renovascular stenoses in this case, is likely to have enlarged the vessel lumen before replacement of medial and adventitial fibrosis after extensive destruction of medial elastic fibres in the left main coronary artery. Moreover, associations such as autoimmune hepatitis, chronic thyroiditis, and Sjögren syndrome strongly suggests that Takayasu aortitis may be an autoimmune disease.

 Keywords: giant coronary aneurysm; Takayasu aortitis; renovascular hypertension; autoimmune disorders
PMCID: PMC1728945  PMID: 9922363
12.  Aorta-Right Atrial Tunnel 
Texas Heart Institute Journal  2010;37(4):480-482.
Aorta–right atrial tunnel is a vascular channel that originates from one of the sinuses of Valsalva and terminates in either the superior vena cava or the right atrium. The tunnel is classified as anterior or posterior, depending upon its course in relation to the ascending aorta. An origin above the sinotubular ridge differentiates the tunnel from an aneurysm of the sinus of Valsalva, and the absence of myocardial branches differentiates it from a coronary–cameral fistula. Clinical presentation ranges from an asymptomatic precordial murmur to congestive heart failure. The embryologic background and pathogenesis of this lesion are attributable either to an aneurysmal dilation of the sinus nodal artery or to a congenital weakness of the aortic media. In either circumstance, progressive enlargement of the tunnel and ultimate rupture into the low-pressure right atrium could occur under the influence of the systemic pressure.
The lesion is diagnosed by use of 2-dimensional echocardiography and cardiac catheterization. Computed tomographic angiography is an additional noninvasive diagnostic tool. The possibility of complications necessitates early therapy, even in asymptomatic patients or those with a hemodynamically insignificant shunt. Available treatments are catheter-based intervention, external ligation under controlled hypotension, or surgical closure with the patient under cardiopulmonary bypass.
Herein, we discuss the cases of 2 patients who had this unusual anomaly. We highlight the outcome on follow-up imaging (patient 1) and the identification and safe reimplantation of the coronary artery (patient 2).
PMCID: PMC2929865  PMID: 20844628
Aorta/abnormalities/surgery; coronary vessel anomalies/classification/diagnosis; diagnosis, differential; heart atria/abnormalities/surgery; sinus of Valsalva; tomography, X-ray computed; treatment outcome; vena cava, superior/abnormalities
13.  Sinus of Valsalva Fistula to the Right Ventricle along with Coronary Artery Fistula to the Pulmonary Artery in a Young Native American Female 
Case Reports in Cardiology  2013;2013:674608.
Sinus of Valsalva aneurysm is a rare condition and associated with a high rate of mortality if rupture occurs. The aneurysms are rarely diagnosed until rupture occurs. This case describes a young Native American female whose only symptom was intermittent chest pain prior to the detection of the aneurysm along with a small ventricular septal defect. The patient was also found to have a coexisting coronary artery fistula, and it is rare phenomenon to have these coexisting anomalies. The anomalies were demonstrated on both cardiac computed tomography and coronary angiography. The patient underwent surgical closure of both anomalies, which is the recommended treatment to avoid future complications.
doi:10.1155/2013/674608
PMCID: PMC4008390  PMID: 24804114
14.  Asymptomatic giant coronary aneurysm in an adolescent with Behcet's syndrome 
Objective
Behcet's is an idiopathic multi-organ syndrome, which may have onset during childhood. Vascular involvement is uncommon, with rarely reported coronary aneurysm formation. We present a case report of a teenager girl who developed recalcitrant life-threatening Behcet's vasculitis, involving both small and large venous and arterial systems including a giant coronary aneurysm.
Case report
De-identified data were collected retrospectively in case report format. Although our sixteen year old female with Behcet's vasculitis had resolution of many arterial aneurysms, she had persistent venous thrombosis of large vessels, as well as persistent, giant arterial aneurysms requiring intra-arterial coiling of a lumbar artery and coronary bypass grafting despite intensive immunosuppression including glucocorticoids, cyclophosphamide, infliximab, methotrexate, azathioprine and intravenous immunoglobulin.
Conclusions
Vascular manifestations may be seen in Behcet's syndrome, including asymptomatic coronary aneurysm, which may be refractory to immunosuppression and ultimately require surgical intervention. Increased awareness is essential for prompt diagnosis and management.
doi:10.1186/1546-0096-10-2
PMCID: PMC3275526  PMID: 22226364
Behcet's syndrome; vasculitis; coronary aneurysm
15.  Anomaly of the Left Anterior Descending Coronary Artery Arising from the Right Sinus of Valsalva and Ventricular Septal Defect in Adult: A Rare Case 
Yonsei Medical Journal  2005;46(5):729-732.
Anomaly of the left anterior descending (LAD) coronary artery arising from the right sinus of valsalva is frequently seen with tetralogy of Fallot (TOF). The association of the LAD coronary artery with ventricular septal defect (VSD) is uncommon. We described an anomalous origin of the LAD coronary artery from the right sinus of valsalva with ventricular septal defect in a 38-year-old male patient suffering from atypical angina. The LAD coronary artery arose from the right sinus of valsalva, just next to the right coronary artery. There was a single opening in the membranous part of the interventricular septum. From this case, we suggest that angiography is useful for both documenting anomalies of the LAD coronary artery associated with VSD and for determining the safest surgical procedures.
doi:10.3349/ymj.2005.46.5.729
PMCID: PMC2810584  PMID: 16259076
Coronary vessels; ventricular septal defects; congenital abnormalities
16.  Association of aortic and main left coronary aneurysms with severe aortic insufficiency in Takayasu’s arteritis 
Clinics and Practice  2011;1(2):e26.
Takayasu’s arteritis is a granulomatous vasculitis of unknown etiology that affects mainly the aorta and its branches. As a result of intimal fibroproliferation, segmental stenosis, occlusion, dilatation, and aneurysmal formation of the involved vessels may develop. It is an uncommon disease and usually affects young Asian female patients during the second and third decades of life. Coronary arteries are exceptionally affected and coronary aneurysm formation is a very rare finding. We describe a case of a previously healthy 26-year-old Caucasian female whose Takayasu’s arteritis presented as a previously undescribed association of aortic and main left coronary aneurysms with severe aortic insufficiency.
doi:10.4081/cp.2011.e26
PMCID: PMC3981242  PMID: 24765288
Takayasu’s arteritis; vasculitis; aneurysm; coronary artery; aortitis.
17.  Acquired Pseudoaneurysm of the Sinus of Valsalva 
Texas Heart Institute Journal  2008;35(2):186-188.
Localized aneurysms of the sinus of Valsalva are uncommon. Repair is tailored to the defective anatomy of the sinus, the aortic valve, and the coronary artery.
Herein, we report the successful surgical treatment of 2 patients who had unruptured pseudoaneurysms of the sinus of Valsalva. An evident fissure in the intima of the sinus of Valsalva was seen in both patients. Patient 1 was a 57-year-old man with annuloaortic ectasia who was diagnosed with pseudoaneurysm of the right sinus of Valsalva. A thrombus that had formed in the pseudoaneurysm subsequently migrated into the right coronary artery. Aortic root remodeling was performed. Patient 2 was a 23-year-old man with a history of blunt chest trauma. He developed a pseudoaneurysm in the right sinus of Valsalva and tears on the aortic cusps. He underwent aortic valve replacement and repair of the Valsalva wall.
PMCID: PMC2435462  PMID: 18612456
Aorta/injuries; aortic aneurysm/complications/diagnosis/epidemiology/pathology/surgery; heart valve prosthesis implantation; sinus of Valsalva/abnormalities/pathology/radiography/surgery; thrombosis/complications/surgery; vascular fistula
18.  Endovascular GDC Treatment of an Idiopathic Carotid-Cavernous Fistula Caused by Aneurysmal Rupture of the Intra-Cavernous Carotid Artery 
Interventional Neuroradiology  2006;12(Suppl 1):174-177.
Summary
Aneurysmal rupture of the intra-cavernous carotid artery may cause idiopathic carotid-cavernous fistula (CCF), and the treatment choice for occluding shunting fistula in this type of CCF is an endovascular approach using detachable balloons. However, little has been reported on treating such lesions with the intra-aneurysmal embolization using Guglielmi detachable coils (GDCs). To our knowledge, ours is the first reported case of successful treatment by selective intra-fistula and intra-aneurysmal embolization with GDCs. A 74-year-old woman exhibited proptosis and chemosis of her left eye over a period of one month. Symptoms of double vision in conversion and pulsatile murmur in her left eye were also noted. Angiography revealed an intra-cavernous aneurysm of the left internal carotid artery (ICA) with a shunting fistula, which drained into the dilated cavernous sinus, superior orbital vein (SOV), superior petrosal sinus, inferior petrosal sinus, and pterygoid plexus. We thought the fistula would occlude by intra-aneurysmal embolization, but we had no confidence of tight packing of the aneurysm since the aneurismal neck was relatively wide. So, we embolized the venous side of the shunting fistula and then the dome of the aneurysm with GDCs. Immediately after the operation, her symptoms and signs were ameliorated, and complete occlusion of the CCF was observed on long-term follow-up. We suggest selective intrafistula and intra-aneurysmal embolization with GDCs as an alternative method of treatment of idiopathic CCF originating from aneurysmal rupture of the intra-cavernous carotid artery.
PMCID: PMC3387948  PMID: 20569626
idiopathic CCF; aneurysm; GDC
19.  Hypereosinophilic syndrome masquerading as a myocardial infarction causing decompensated heart failure 
Background
An 81 year old female patient diagnosed with a chronic low grade hypereosinophilic syndrome presented with angina and dyspnoea.
Case presentation
She was managed for a non-ST elevated myocardial infarction since her troponin levels were elevated. On day 5, she suffered an acute clinical deterioration with type I respiratory failure and cardiogenic shock, accompanied by deterioration in left ventricular systolic function demonstrated on echocardiography, and this coincided with a marked rise in eosinophil count. Secondary causes of eosinophilia were excluded permitting a diagnosis of Hypereosinophilic Syndrome (HES) to be made. Coronary angiography revealed unobstructed arteries. Supportive treatment for heart failure included diuretic and inotropes but she dramatically improved both clinically and echocardiographically upon commencement of high dose steroids and hydroxycarbamide. Cardiac magnetic resonance imaging (CMR) demonstrated diffuse, shallow endomyocardial enhancement with late gadolinium, consistent with a diagnosis of eosinophilic myocarditis.
Conclusion
Hypereosinophilic Syndrome can masquerade as a myocardial infarction causing decompensated heart failure. Early recognition and treatment with steroids can improve outcome.
doi:10.1186/1471-2261-13-75
PMCID: PMC3849331  PMID: 24053402
20.  Unruptured congenital aneurysm of the left sinus of Valsalva in an adult with complex left heart malformations 
Heart  2003;89(1):e3.
A 26 year old man who presented with the first signs of right heart failure was found to have a large congenital aneurysm of the aortic sinus of Valsalva and of the left coronary sinus. These were combined with left heart anomalies in the form of a bicuspid aortic valve, a rare variant of a persistent left superior vena cava with blood flow from the left atrium through the brachiocephalic vein into the superior vena cava and a kink in the aortic arch. An aortic coarctation had been corrected with a patch 12 years earlier. Although the aneurysm was not perforated and there were no clinical signs of infarction, the aneurysm was resected prophylactically and the left coronary artery was reinserted through a bypass with a Gore-Tex conduit. The case is noteworthy because this diagnosis is very rare and its early treatment may prevent several complications. The clinical features, treatment, and outcome are discussed.
PMCID: PMC1767492  PMID: 12482813
left sinus of Valsalva; aneurysm
21.  Sinus of Valsalva Aneurysm Rupture: An Unusual Presentation of Chromosome 22q11.2 Deletion: A Case Report 
Case Reports in Pediatrics  2012;2012:387075.
Sinus of Valsalva aneurysm (SVA) is defined as a weakness in the aortic valve wall, immediately above the attachments of each of the aortic cusps. This weakness can rupture and create an aortocardiac fistula. There are many congenital heart defects associated with chromosome 22q11 deletion, especially involving the aortic arch and its branches. SVA is not an anomaly usually associated with chromosome 22 deletion. We report the case of a 19-year-old female who presented to our institution with SVA rupture. She was subsequently diagnosed with chromosome 22q11 deletion syndrome. Despite dysmorphic facial features and a learning disability, our patient had not been diagnosed with the chromosome abnormality. SVA is a rare congenital heart defect and has only once previously been reported in a child with a chromosome 22q11 deletion. We report the first case where aneurysm rupture preceded the chromosomal findings. Chromosome 22q11 deletion could be missed due to either the unfamiliarity of physicians with the syndrome or the variability and subtlety of the phenotype. This was demonstrated by our patient who, at age 19 after presenting with an SVA rupture, prompted physicians to find an explanation for her coexisting dysmorphic features and her learning disability.
doi:10.1155/2012/387075
PMCID: PMC3461629  PMID: 23050182
22.  Aortic Root Dilatation with Sinus of Valsalva and Coronary Artery Aneurysms Associated with Ankylosing Spondylitis 
Texas Heart Institute Journal  2006;33(1):70-73.
The HLA-B27-related spondyloarthopathies are associated with cardiovascular disease in 2% to 10% of cases. Inflammation and sclerosis of the aortic root and ventricular septum have been linked to the development of isolated aortic regurgitation and conduction abnormalities; however, aneurysms of the aortic sinuses and coronary arteries have not been previously described.
We report the case of a 58-year-old white man who presented for evaluation of dyspnea and was found to have aneurysms of the sinuses of Valsalva and the circumflex coronary artery. The patient underwent aortic root replacement. Approximately 3 months later, he presented with symptoms, radiographs, and laboratory data consistent with ankylosing spondylitis.
To our knowledge, these particular cardiovascular manifestations of HLA-B27-related disease have not been previously reported. This case expands the clinical spectrum of the disease and should prompt the clinician to consider the possibility of HLA-B27-associated cardiovascular disease in patients who have aortic and coronary aneurysms.
PMCID: PMC1413595  PMID: 16572876
Ankylosing spondylitis/complications; aortic aneurysm/pathology; aortic root dilatation; aortic valve insufficiency; coronary aneurysm; HLA-B27 antigen/blood; sinus of Valsalva/pathology
23.  Surgical correction of ruptured aneurysms of the sinus of Valsalva using on-pump beating-heart technique 
Background
Rupture of aneurysms of the sinus of Valsalva results in abrupt onset of congestive heart failure. On-pump beating-heart surgery may reduce cardiac impairment by maintaining coronary blood flow and avoiding cardioplegia. Herein, we report the operative correction of thirty-one patients of ruptured aneurysms of the sinus of Valsalva, using the on-pump beating-heart technique.
Methods
Thirty-one patients with ruptured aneurysms of the sinus of Valsalva underwent operative corrections using the on-pump beating-heart technique. In patients with fistula diameter less than 1 cm and no aortic regurgitation, the aorta was unclamped throughout cardiopulmonary bypass(CPB) while receiving antegrade heart perfusion. In remainder of patients, retrograde perfusion was used.
Results
After intracardiac manipulation was complete and the nasopharyngeal temperature was raised to 36-37°C, the patients were smoothly weaned off CPB. There were no early or late postoperative deaths. All patients were in New York Heart Association functional class I at follow-up (range, 0.5-1 year). Mild-to-moderate aortic valve regurgitation was observed in one patient. No recurrence of the left-to-right shunt from ruptured aneurysms of the sinus of Valsalva was observed.
Conclusions
Beating heart on pump allows adequate examination of the aortic lesion under near-physiologic conditions, allows decrease in ischemia-reperfusion injury and potentially decreases the risk of serious or fatal rhythm disturbances. On-pump beating-heart technique for repair of ruptured aneurysm of sinus of Valsalva is feasible and promising. Antegrade heart perfusion is suitable for patients with a fistula diameter <1 cm and no aortic regurgitation, and retrograde perfusion is suitable for the others.
doi:10.1186/1749-8090-5-37
PMCID: PMC2877041  PMID: 20470371
24.  Steal syndrome secondary to coronary artery fistulae associated with giant aneurysm 
World Journal of Cardiology  2014;6(3):112-114.
Giant coronary artery aneurysms and coronary artery fistulae are uncommon pathologies. We present the case of an elderly woman who was referred to cardiology for investigation of possible ischaemic heart disease prior to orthopaedic surgery. The patient had developed chest pain in the setting of a septic total knee replacement associated with changes on electrocardiography. Coronary angiography revealed multiple coronary arteriovenous fistulae associated with giant coronary artery aneurysm causing steal syndrome in the setting of haemodynamic stress.
doi:10.4330/wjc.v6.i3.112
PMCID: PMC3964188  PMID: 24669293
Coronary angiography; Coronary disease; Myocardial ischaemia; Coronary aneurysm; Vascular fistula; Chest pain
25.  Unruptured aneurysm of the left sinus of Valsalva extending into the left ventricular outflow tract: presentation and imaging 
Heart  1998;80(2):190-193.
The symptomatic presentation of an unruptured sinus of Valsalva aneurysm is rare. A 48 year old man with a history of treated hypothyroidism, and a five year history of ileocolonic Crohn's disease of chronic low grade activity presented with a profound left hemiplegia. He was in sinus rhythm and normotensive. Cardiac auscultation was repeatedly normal. Computed tomography of the head performed early in the course of the illness was reported as normal. Duplex Doppler examination of the carotid arteries performed six months later revealed no significant atheroma. There was complete resolution of the neurological deficit over a period of months. A year later he presented with chest pain suggestive of myocardial ischaemia. Computed tomography, magnetic resonance imaging, transthoracic and transoesophageal echocardiography, and cardiac catheterisation pointed to a sinus of Valsalva aneurysm protruding into the left ventricular outflow tract. In view of the previous neurological event and ongoing chest pain suggestive of myocardial ischaemia, the lesion was resected. The patient made a good recovery and postoperative transoesophageal echocardiography showed normal aortic valve function with no residual regurgitation. This is the first reported case of pure left ventricular outflow tract extension of an unruptured left sinus aneurysm. The presentation with ischaemic cardiac pain does not seem to be explained by conventional mechanisms.

 Keywords: sinus of Valsalva aneurysm;  non-invasive imaging;  transoesophageal echocardiography
PMCID: PMC1728773  PMID: 9813568

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