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.
Aortic aneurysm/congenital; aortic rupture/surgery; heart defects; congenital; heart septal defects; ventricular; coronary vessel anomalies/surgery; sinus of Valsalva/abnormalities/surgery
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.
Cardiac computed tomography; Cardiac imaging; Congenital heart disease; Ruptured sinus of Valsalva aneurysm
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.
left sinus of Valsalva; aneurysm
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)
Angina pectoris/etiology; aortic aneurysm/complications/diagnosis/surgery; coronary arteries; heart defects, congenital; myocardial ischemia/etiology; sinus of Valsalva
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.
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.
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.
Coronary aneurysms represent anomalies identified in 0.15%–4.9% of patients undergoing coronary angiography. At present, there is no uniform definition of this pathology. Aneurysms of the left main coronary artery (LMCA) are extremely uncommon, with an incidence of 0.1%. It has been demonstrated that atherosclerosis is the main cause of these anomalies in adults, and Kawasaki disease in children and adolescents. Other causes include connective tissue disorders, trauma, vasculitis, congenital, mycotic, and idiopathic. These dilated sections of the coronary artery are not benign pathology because they are subject to spasm, thrombosis, and subsequent distal embolism, spontaneous dissection and rupture. Treatment options include anticoagulation, custom-made covered stents, reconstruction, resection, and exclusion with bypass. Our report on an old case illustrates the giant saccular LMCA aneurysm leading to myocardial ischemia due to coronary steal phenomenon.
Coronary aneurysm; Coronary artery disease; Coronary angiography
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.
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.
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.
Behcet's syndrome; vasculitis; coronary aneurysm
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.
Ankylosing spondylitis/complications; aortic aneurysm/pathology; aortic root dilatation; aortic valve insufficiency; coronary aneurysm; HLA-B27 antigen/blood; sinus of Valsalva/pathology
A 46-year-old female with a giant aneurysm of the non-coronary sinus of Valsalva, associated with moderate aortic valve regurgitation, is described. The aneurysm was detected by echocardiography in a patient who was complaining of paroxysmal palpitations. The patient was accepted for aneurysmectomy. In this case report the possible aetiologies and clinical aspects of the aneurysm are reviewed. Treatment and the role of multi-sliced computed tomography (MSCT) in heart disease are discussed.
sinus of Valsalva aneurysm; echocardiography and MSCT
Behçet's disease is an inflammatory disorder of unknown origin, with mucocutaneous, ocular, articular, vascular, gastrointestinal and central nervous system manifestations. Although cardiac involvement is not an uncommon manifestation of Behçet's disease, coronary aneurysm has rarely been reported.
A 36-year-old Iranian man was admitted to our emergency department for retrosternal pain of two and a half hours duration. His detailed medical history revealed that he had no risk factors for coronary artery disease, however, Behçet's disease had been diagnosed about 10 years earlier. His electrocardiogram showed inferior myocardial infarction. He underwent coronary angiography that showed multiple giant aneurysms in his coronary arteries. Two months later, he experienced another episode of unstable angina. This was followed by two episodes of anterior myocardial infarction 2 and 5 months afterwards.
This case highlights the importance of careful diagnostic work-up in the evaluation of myocardial infarction in patients. In our patient, Behçet's disease proved to be the cause of recurrent myocardial infarction.
A coronary artery aneurysm is an uncommon disorder and is seen as a characteristic dilatation of a localized portion of the coronary artery. Clinical manifestation of a coronary artery aneurysm varies from an asymptomatic presentation to sudden death of a patient. Although coronary aneurysms are typically diagnosed by the use of coronary angiography, a new generation of coronary 64-slice multidetector computed tomography (64-MDCT) scanners have successfully been used for evaluating this abnormality in a noninvasive manner. In the present case, we performed coronary 64-MDCT scanning preoperatively and postoperatively on a patient with multiple giant coronary aneurysms. The use of coronary 64-MDCT may provide an evaluation technique not only for diagnosis but also for follow-up after surgery for this condition.
Coronary aneurysm; computed tomography
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.
Aortic aneurysm/congenital/etiology/radiography/surgery; aortic valve insufficiency; cardiac surgical procedures/methods; differential diagnosis; sinus of Valsalva/pathology/radiography/surgery; treatment outcome
A 37 year old man presenting with acute heart failure, hypotension, and acute renal failure was diagnosed by cardiac catheterisation and angiography to have the rare combination of congenital aneurysm of the non-coronary sinus of Valsalva rupturing into the right ventricle, and an anomalous origin of the right coronary artery from the main pulmonary artery. The diagnosis could not be confirmed by transthoracic echocardiography in this patient. This combination of defects, confirmed at cardiac surgery, has not been reported before, and this case report highlights the importance of preoperative definition of congenital defects associated with an aneurysm of the sinus of Valsalva.
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.
Sinus of valsalva aneurysm is a rare condition arising from any of the three aortic sinuses. Among them, an aneurysm arising from the left coronary sinus is the rarest. Most of these cases were earlier diagnosed using echocardiography and conventional angiography. But with the availability of advanced imaging modalities like 64 slice cardiac CT and MR modalities, this condition can be accurately assessed noninvasively. We report a case of ruptured aneurysm originating from the left coronary sinus with a long windsock type of fistulous track between the aneurysm and right atrium evaluated by 64 slice cardiac CT imaging. This was later confirmed perioperatively.
Sinus of Valsalva; Aneurysm; Rupture; Fistula; CT imaging
This study sought to characterize the inflammatory infiltrate in ascending thoracic aortic aneurysm (TAAs) in patients with Marfan syndrome (MFS), familial TAA (FTAA), and non-familial TAA cases.
TAAs are associated with a pathologic lesion termed medial degeneration, which was described as a noninflammtory lesion. TAAs are a complication of MFS and also can be inherited in an autosomal dominant manner of FTAA.
Full aortic segments were collected from patients undergoing elective repair with MFS (n=5), FTAA (n=6) and TAAs (n=9), along with control aortas (n=5). Immunohistochemistry staining was performed using antibodies directed against markers of lymphocytes and macrophages. Real-time PCR analysis was performed to quantify the expression level of T cell receptor β chain variable region gene.
Immunohistochemisty of TAA aortas demonstrated that the media and adventitia from MFS, FTAA and sporadic cases had increased numbers of T lymphocytes and macrophages when compared with control aortas. The number of T cells and macrophages in the aortic media of the aneurysm correlated inversely with the patient’s age at the time of prophylactic surgical repair of the aorta. Surprisingly, T cell receptor profiling indicated a similar clonal nature of the T cells in the aortic wall in a majority of aneurysms, whether the patient had MFS, FTAA or sporadic disease.
These results indicate that infiltration of inflammatory cells contributes to the pathogenesis of TAAs. Superantigen-driven stimulation of T lymphocytes in the aortic tissues of the TAA patients may contribute to the initial immune response.
This study sought to investigate the infiltration of T-lymphocytes and macrophage in the aortas of patients with MFS, FTAA and sporadic TAAs. The results indicate that infiltration of inflammatory cells contributes to the pathogenesis of TAAs and superantigen-driven stimulation of T-lymphocytes may contribute to the initial immune response.
Sinus of Valsalva aneurysms rarely present until rupture occurs. We describe the case of a patient who presented with acute right heart failure and upon investigation was found to have an unruptured sinus of Valsalva aneurysm causing right ventricular outflow obstruction; there was an associated subaortic ventricular septal defect. To the best of our knowledge, only 1 other case with these features has been reported in the medical literature.
The diagnosis was made by transthoracic echocardiography and cardiac catheterization. Through an aortic and right ventricular approach, we successfully excised the aneurysmal right coronary sinus, closed the ventricular septal defect, and replaced the aortic valve. (Tex Heart Inst J 2003;30:152–4)
Aortic aneurysm/complications/diagnosis/pathology; aortic valve insufficiency/complications/diagnosis/pathology; heart septal defects, ventricular; heart valve diseases/diagnosis; ventricular outflow obstruction/etiology; sinus of Valsalva/surgery; ventricular septal defect
A 35-year-old menstruating woman presented with exertional angina, and was diagnosed with an unruptured left sinus of Valsalva and a noncoronary sinus of Valsalva aneurysm. Hypereosinophilia and coronary artery occlusion was also observed; this association is extremely rare.
Hypereosinophilia; Sinus of Valsalva aneurysm
Blau syndrome is a rare granulomatous disorder inherited in an autosomal dominant manner characterized by the early appearance of granulomatous arthritis, skin rash and anterior uveitis. There are very few data on the cardiovascular manifestations of Blau syndrome. Here we report the first case of sinus of valsava aneurysm in Blau syndrome. In isolated unruptured aneurysms of a sinus of Valsalva without compromise of the aortic valve and/or the coronary ostia, repair may be accomplished by simple placation of the aneurysm or excision of the aneurysm(s) and patch closure of the defect(s) between the aortic annulus and the sinu-vascular ridge. Because of the particular conditions in our case, the repair was performed with replacement of the aortic valve and root using a composite graft employing a modified Bentall's technique.
This report describes the case of a 45-year-old Korean female who had suffered from Behçet's syndrome for two years with a huge, unruptured aneurysm originating from the left coronary sinus. The aneurysm had caused myocardial and aortic insufficiency by compressing the proximal left anterior descending coronary artery. The orifice of the aneurysm was at the left coronary sinus, about 5mm from the left main coronary ostium, and it was filled with organized thrombi. Surgical repair was performed by closing the entrance of the aneurysm with a Dacron patch and by implementing aortic valve repair and coronary artery bypass grafting. The patient's coronary flow was restored postoperatively, and all anginal symptoms disappeared.
Aneurysm; coronary sinus; myocardial infarction
A case of aneurysm of the main stem of the left coronary artery associated with aortic insufficiency and an aneurysm of the ascending aorta is reported. The importance of coronary angiography in diagnosing this condition is illustrated. Surgical repair included isolation of the coronary aneurysm and replacement of the ascending aorta and aortic valve, combined with triple aortocoronary saphenous vein bypass grafts. A review of the aetiology, clinical features, and surgical management of coronary artery aneurysms is presented.
Whilst the incidence of CAA has been reported as up to 5%, giant CAA (>2 cm) is rare.
PRESENTATION OF CASE
We present a rare case of 3 cm × 4 cm giant coronary artery aneurysm (CAA) in the context of aorto-iliac aneurysmal disease, treated by staged open surgical repair.
Abdominal aortic aneurysm (AAA) and CAA share risk factors and aetiological mechanisms, therefore should be considered, particularly when CAA is diagnosed in the first instance.
Surgical intervention for diagnosed giant CAA appears to be the treatment of choice in the reported literature, with the order of intervention when AAA co-exists remaining a point for debate.
Coronary aneurysm; Aortic aneurysm; Iliac aneurysm
Aneurysms of the sinuses of Valsalva, when detected before rupture, are repaired electively. When they are small, they are most frequently repaired by direct closure; when they are large, they are repaired with a prosthetic patch. When they are associated with prolapse of the corresponding aortic cusp and aortic insufficiency, valve replacement is usually performed concomitantly. We present the case of a patient in whom both abnormalities were corrected by implantation of a Freestyle® bioprosthesis, the wall of which completely excluded the aneurysm of the sinus of Valsalva. The patient remains asymptomatic 18 months after surgery. (Tex Heart Inst J 2003;30:202–4)
Aortic aneurysm/surgery; heart valve prosthesis implantation; sinus of Valsalva/surgery
Takayasu's Arteritis, formerly known as "pulseless disease", is a chronic idiopathic vasculitis which affects the large vessels in the body. First described in the 1800's, this rare condition is more commonly found in Asian women in their 40's. The aorta and its main branches are the primary vessels affected, with the most typical features reflected as ischemia or aneurysm formation. With Takayasu's Arteritis being a rare condition and its acute phase presentation often similar to other conditions, diagnosis is often difficult.
A 48 year old Irish Caucasian female, who presented as a typical history of an aortic dissection (chest pain radiating to her back in an interscapular region and a systolic blood pressure differential of 50 mmHg between her right and left upper limbs), was investigated with a number of imaging modalities and diagnosed with Takayasu's Arteritis, involving arteries affecting a number of organs. She was treated as per protocol for Takayasu's Arteritis. A diagnosis of cervical cancer quickly followed.
This case report highlights that a differential diagnosis should never be dispelled based upon a "typical" history. The importance of modern day imaging techniques such as CT, MRI and angiography, can often be paramount to confirming a diagnosis and the extent of the pathology.
A possible link between Takayasu's Arteritis and gynaecological malignancies may exist.
Takayasu aortoarteritis (TA) rarely affects the nervous system, but when it does, it usually manifests as cerebral ischemia or stroke. These strokes have mainly been attributed to stenotic extracranial vessels. Stenoses of intracranial vessels, although rare in TA, can occur due to either embolization into the vessel or because of the vasculitic process itself. Intracranial aneurysms are very rare in patients with TA. Bilateral cavernous internal carotid artery (ICA) aneurysms are rarer. They have been reported following radiation therapy and in association with fibromuscular dysplasia and juvenile Paget disease. Bilateral mycotic intracavernous aneurysms also occur. Bilateral giant cavernous ICA aneurysms with carotid-cavernous fistula (CCF) consequent to rupture into the cavernous sinus in a case of TA are extremely unusual. We report a case that fulfilled both American College of Rheumatology and European League against Rheumatology criteria for TA. The patient had bilateral cavernous sinus giant aneurysms and CCF because the right-sided aneurysm had ruptured and was leaking into the cavernous sinus.
Bilateral giant ICA aneurysms; carotid cavernous fistula; Takayasu arteritis