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We report the cases of 2 patients whose tricuspid aortic valves were found to have partial commissural fusion. Both patients experienced complications that were probably related to this abnormality: bacterial endocarditis in 1 instance and a lacunar stroke in the other. In order to illustrate the similarity of physical findings, we also describe the case of a 3rd patient, who had a typical bicuspid aortic valve. Tricuspid aortic valve with partial commissural fusion has been described in autopsy series and has been predicted to cause an ejection sound, but we could find no previously published description of this lesion in living patients. We wish to alert others to the possible presence of aortic commissural fusion, to its potential for serious and likely preventable sequelae, and to the ability of carefully performed transthoracic high-resolution digital echocardiography to demonstrate this condition when its characteristics are found on physical examination.
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PMID:Tricuspid aortic valve with partial commissural fusion: prelminary report of an apparently common cause of aortic ejection sounds with potential for serious sequelae, illustrated by two cases. 1092 9

Turner syndrome is a condition usually associated with reduced final height, gonadal dysgenesis, and thus insufficient circulating levels of female sex steroids, and infertility. A number of other signs and symptoms are seen more frequently with the syndrome. With respect to cardiac function, congenital malformations of the heart and the great vessels, hypertension and ischemic heart disease, and increased risk of aortic dissection are all conditions that the pediatrician or the physician caring for females with Turner syndrome should keep in mind. Many girls and adolescents with Turner syndrome receive growth hormone (GH) treatment, which has so far been an effective and well-tolerated therapy. Nevertheless, because of the experience from acromegaly, the physician should monitor blood pressure and perform echocardiography, together with clinical examinations by a cardiologist at regular intervals. During adulthood most women with Turner syndrome are faced with premature menopause and the need for female hormone replacement therapy (HRT). During clinical evaluation of girls and women with Turner syndrome, these conditions and complications should be kept under surveillance. Here the cardiovascular complications of Turner syndrome are reviewed. The risk of congenital heart defects such as bicuspid aortic valves, aortic coarctation, other valve abnormalities, and septal defect is increased. Likewise, the risk of aortic dissection at a young age is increased, as is the risk of hypertension, ischemic heart disease, and stroke. GH therapy does not seem to adversely affect the heart, although longer-term follow-up studies are needed. In short-term studies, HRT lowers blood pressure, while any effect on the risk of ischemic heart disease has not been evaluated. Treatment with GH and HRT are discussed in relation to the heart and great vessels. Presently, the pathophysiology of the congenital cardiovascular malformation in Turner syndrome is unexplained, although different theories exist. Recommendations for clinical practice are given, including life-long surveillance of cardiac function, aortic diameter and blood pressure.
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PMID:Turner syndrome and the heart: cardiovascular complications and treatment strategies. 1472 55

Whilst the pulmonary valve is a tricuspid valve, very few reports exist of bicuspid pulmonary valves, the majority of which are associated with congenital heart disease. Isolated bicuspid valves not associated with congenital abnormalities are even more rare. Herein, the case is described of a 65-year-old man who died from a stroke, and in whom a bicuspid pulmonary valve was found during post-mortem dissection for tissue donation.
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PMID:Isolated bicuspid pulmonary valve: an unusual finding. 1522 1

Spontaneous calcific cerebral embolus is a rare complication of calcific vascular disease such as calcified aortic plaques, and calcific aortic and mitral stenosis. Fewer than 10 case reports are described in the literature. Cerebral ischaemic symptoms may provide the indication for surgical replacement, even in the absence of valvular insufficiency or stenotic symptoms. We report a case of stroke in a young man with a known bicuspid calcific aortic valve.
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PMID:Spontaneous calcific cerebral embolus. 1584 55

The population of patients with adult congenital heart disease is approximately 800,000 in the U.S. Those with prior cardiac surgery often consider themselves "cured," although the majority faces a lifetime of problems including arrhythmias, ventricular dysfunction, and one or more re-operations. Even patients with repaired "simple" lesions such as an atrial septal defect may not have normal survival if they are repaired in adulthood. Patients with repaired coarctation may have premature cardiovascular complications including sudden cardiac death, myocardial infarction, and stroke. They also have aortic complications such as aneurysm and dissection, which result from a diffuse arteriopathy and continued hypertension that may be caused by underlying endothelial dysfunction. In addition, bicuspid aortic valve occurs in more than one-half of the patients with coarctation, so continued surveillance for significant aortic valvular heart disease is necessary. More complex lesions also pose problems after "total correction." Patients with repaired tetralogy of Fallot often have pulmonary regurgitation, which is frequently overlooked on clinical exam and echocardiography. Pulmonary valve replacement should be performed before the development of irreversible right ventricular dysfunction and an increased risk of ventricular tachycardia or sudden cardiac death. Because they are vulnerable to deterioration of systemic ventricular function, those with congenitally corrected transposition require special vigilance, usually with concomitant atrio-ventricular valve regurgitation. Late referral is common with a deleterious effect on long-term survival. These patients need lifelong follow-up and the residua and sequelae of their complex anomalies must be understood in order to provide optimum care.
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PMID:The adult with congenital heart disease: born to be bad? 1599 27

Aortic coarctation is often linked to bicuspid aortic valve and only rarely to congenital aneurysm of the circle of Willis. It is possible that a common pathogenetic substrate determines this association, but it has not yet been demonstrated. In this report we describe the case of a 32-year-old woman, affected by previous anamnestic functional cardiac murmur. She has suddenly presented a hemorrhagic stroke due to the breaking of the sylvian artery, angiographically demonstrated and surgically corrected. On the same occasion aortic angiography revealed aortic coarctation. Later, an echocardiographic exam demonstrated the coexistence of bicuspid aortic valve. Afterwards the patient underwent angioplasty in order to correct aortic coarctation. Any coexisting cardiac and vascular disease may influence survival in unoperated adults with aortic coarctation, which can even be lethal. In this case the simultaneous presence of these three malformations was particularly dangerous since the patient was not enough symptomatic and so an early diagnosis was not possible. Therefore, we strongly recommend to perform echocardiography in every young patient affected by hemorrhagic stroke and to take into consideration the possibility of performing cerebral angiography in all cases of aortic coarctation.
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PMID:[Aortic coarctation is correlated with bicuspid aortic valve and intracranial aneurysm: a case report and review of the literature]. 1653 27

Infective endocarditis is uncommon in children, and there is a paucity of literature concerning cases that involve unique or resistant organisms. Complications associated with infective endocarditis are distinctly rare and poorly characterized, especially unusual sequelae such as pseudoaneurysm of the abdominal mesentery. Our case involves an adolescent who presented with several weeks of fever and eventual cardiac murmur and was found to have vancomycin-resistant Enterococcus growing as a vegetation on a previously undiagnosed bicuspid aortic valve. He had a cerebral stroke presenting as Broca's aphasia before cardiac surgery, as well as a superior mesenteric artery pseudoaneurysm several days postoperatively. The case highlights some of the serious surgical complications that can occur in young persons with infective endocarditis, as well as many of the problems involved in managing a patient with highly resistant pathogens and a surgically challenging location of the aneurysm.
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PMID:Mesenteric pseudoaneurysm and cerebral stroke as sequelae of infective endocarditis in an adolescent. 1892 34

Aortic valve calcification is common in the elderly and in patients with congenital bicuspid aortic valve but unlike calcific mitral valve disease it is not a well recognized risk factor for stroke. Although autopsy studies have revealed evidence of systemic embolism in one-third of cases with calcific aortic valves, there is conflicting data from larger clinical studies examining the association between calcific aortic valve and stroke. There are only 8 reported cases of symptomatic stroke from spontaneous cerebral thromboembolism associated with calcific aortic valve in the literature. Computerized tomography (CT) angiography and CT without contrast are modalities of choice to diagnose calcific embolism, while MRI may be useful in delineating the extent of ischemia. Ideal management strategy, the role of antiplatelet therapy, anticoagulation or recommendations for valve replacements are poorly defined. We present a focused literature review on this topic.
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PMID:Calcific aortic valve and spontaneous embolic stroke: a review of literature. 1971 38

Pseudoaneurysm of the mitral-aortic intervalvular fibrosa is a rare complication of infective endocarditis of the aortic valve eventually resulting in coronary artery compression, stroke or rupture into the left atrium, aorta or pericardial space. A prompt diagnosis by either transthoracic or transesophageal echocardiography is mandatory to address the patient to cardiac surgery. We report the clinical case of a 25-year-old white man who was admitted to the emergency department for dyspnoea and fever. Echocardiographic examination showed a bicuspid aortic valve with a huge sessile vegetation and a pseudaneurysm of the mitral-aortic intervalvular fibrosa. In addition to conventional two-dimensional examination, three-dimensional echocardiography provided additional information of this complication, precisely delineating the lesions and addressing the cardiac surgeon in choosing the most appropriate operating strategy. Patient underwent then aortic root replacement and the pseudoaneurysm was closed by means of a bovine pericardial patch.
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PMID:3D echocardiographic delineation of mitral-aortic intervalular fibrosa pseudoaneurysm caused by bicuspid aortic valve endocarditis. 2058 51

We report on a case of endocarditis attributable to Cardiobacterium hominis in a 31-year-old man who presented with acute-onset, left-sided hemiparesthesia. Magnetic resonance imaging of the brain showed acute infarctions in 2 areas of the right cerebral cortex, and a transesophageal echocardiogram revealed vegetation in a previously unrecognized bicuspid aortic valve. The patient completed a 6-week course of ceftriaxone and aspirin, with resolution of the vegetation and neurological complications. Our literature review of C. hominis endocarditis suggests that aortic-valve involvement is associated with high stroke and valve-surgery rates. Favorable outcomes and treatment success are evident with either penicillin or ceftriaxone, in combination with (if indicated) valve surgery in patients with neurological complications.
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PMID:Cardiobacterium hominis endocarditis presenting as acute embolic stroke: a case report and review of the literature. 2059 74


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