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 170,190 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Spontaneous aortic dissection is a rare, life-threatening cause of chest pain, and has a higher prevalence when traditional risks such as age, hypertension, dyslipidemia, or connective tissue disorders are present. However, even in the absence of risk factors, non-traumatic rupture of an aortic dissection may occur. Most are found in patients over 40 years of age. Younger victims of this disease often also suffer from other conditions such as cystic medial necrosis, connective tissue disorders such as Marfan's syndrome, or vasculitis. We present the case of an 18-year-old, previously healthy woman who was country line dancing when she began to complain of severe, cramping chest and back pain. She was hemodynamically stable on initial presentation but experienced two seizures while in the emergency department and was intubated. Subsequently, her blood pressure dropped and she developed cardiac arrest, and despite vigorous resuscitation that included blood products and emergency department thoracotomy, she was refractory to all attempts. At autopsy she was found to have a spontaneous, non-traumatic rupture of an aortic dissection. This patient had no discernable risk factors for aortic dissection or discoverable cause on necropsy. We present this case to raise awareness among physicians and review other reported cases in the literature of aortic dissection in patients under age 40 years.
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PMID:Thoracic aortic dissection in an 18-year-old woman with no risk factors. 1829 8

Two unrelated children with Marfan syndrome presented with recurrent intracranial hypertension. Both children complained of headache, nausea, and vomiting and one of them had papilledema. Both had increased cerebrospinal fluid pressure, and their complaints disappeared after lumbar puncture. Although severe headache has been reported in Marfan syndrome due to intracranial hypotension, this is to our knowledge the first report of intracranial hypertension in Marfan patients.
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PMID:Intracranial hypertension in 2 children with marfan syndrome. 1835 49

The 2006 practice guidelines from the American College of Cardiology and the American Heart Association recommend prophylactic aortic replacement for even an asymptomatic patient with a bicuspid aortic valve (BAV) when the aortic dimensions exceed arbitrary ranges based on Marfan syndrome, without comparing risk estimates of aortic dissection with operative risks. In the International Registry of Acute Aortic Dissection, which includes >1,000 autopsied subjects, the average age is 63 years; BAVs are found in only 3%, compared with histories of hypertension in 72%. The risk for valve-sparing aortic replacement is 4% and that for late mortality is 10%, on the basis of 5 publications. The aortic dimensions are from guidelines for Marfan syndrome, with a proved genetic weakness of connective tissue, whereas no culprit genes have been demonstrated in BAV. Although cystic medial necrosis is seen in dilated aortas associated with Marfan syndrome and BAV, it is also seen in dilated aortas with other causes. There is no convincing proof that cystic medial necrosis causes dissection or is simply an effect of dilatation. BAV is not associated with dilatation of the pulmonary arteries, in contrast to Marfan syndrome. Hemodynamic explanations for dilatation of the ascending aorta have been largely ignored because of a belief that it requires severe aortic stenosis or regurgitation. In conclusion, vascular dilatation without a genetic weakness is caused by coarse periodic vibrations from even trivial valve disorders, demonstrated experimentally. There is a natural history of progressive deterioration of the BAV, including the valve left in a valve-sparing aortic replacement, that makes the operation ill advised, as opposed to valve replacement with aortic reinforcement.
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PMID:A critical review of the American College of Cardiology/American Heart Association practice guidelines on bicuspid aortic valve with dilated ascending aorta. 1857 46

An increasing number of women with cardiac disease come up with the idea of becoming pregnant. Immigrants are frequently affected by valvular heart disease. Recently, arrhythmias, high blood pressure and ischemic heart disease affect many less-young pregnant women. Finally, grown-up patients with congenital heart disease have now entered their fertility period (particularly following the Fontan operation). Pregnancy is legitimate and frequently cannot be restrained; it calls for a careful evaluation of all its treatment aspects by a multidisciplinary team and the cardiologist role is of the utmost importance. A few conditions make pregnancy a hazard to both mother and fetus: pulmonary hypertension, left ventricular failure, left-heart obstruction, Marfan syndrome with aortic dilatation, and cyanosis. Most remaining cases are compatible with an (almost) normal course whereby mothers can be reassured. However, all items covered by guidelines should be met, according to the quality imperative.
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PMID:[How to manage pregnancy in cardiac patients]. 1868 89

Prolonged survival of patients with Marfan syndrome after aortic root replacement has led to an increased number of patients with aortic complications beyond the root. Elective replacement of the aortic root removes the most important predilection site for aneurysms, but the distal aorta remains at risk. Predictors for aortic growth and adverse events in the distal aorta include aortic diameter, aortic distensiblity, previous aortic root replacement, hypertension and aortic regurgitation. After aortic dissection, the initial false lumen diameter is an independent predictor for late aneurysm formation. Although there are a few reports of short-term success after endovascular stent grafting of the descending thoracic aorta, stent grafting in patients with Marfan syndrome is not recommended unless intervention is clearly indicated and the risk of conventional open surgical repair is deemed prohibitive. Optimal long-term outcome demands lifelong radiographic follow-up and medical treatment with beta-blocker therapy. After aortic dissection rigorous antihypertensive medication is of utmost importance. Losartan, an angiotensin II type I receptor antagonist, might offer the first potential for primary prevention of clinical manifestations in Marfan syndrome, but the results of clinical trials have to be awaited. (Neth Heart J 2008;16:382-6.).
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PMID:The distal aorta in the Marfan syndrome. 1906 77

The aetiology of aortic aneurysms (AAs) is the subject of intense clinical investigation. One of the critical points in their pathogenesis is the disruption of the balance between vascular extracellular matrix deposition and degradation. AAs are common features in some genetically determined diseases of the connective tissue, such as Marfan and Loeys-Dietz Syndromes. Acquired factors determining an enhanced inflammatory state of the arterial wall also play a key role. Previous studies have determined the role of TGF-beta as the principal mediator of the pathogenesis of the alterations of the arterial wall homeostasis in aneurysms. The current medical management of any AA is mainly focused on the use of pharmacological agents that reduce hemodynamic stress of aortic wall, since hypertension is the major risk factor for the enlargement and rupture of the AAs. Thus, this approach is useful to reduce the risk of aneurysm rupture but is far from being a comprehensive pathophysiology-based therapeutic approach. Drugs with the potential of reducing the action of TGF-beta, which activation and expression has been reported to have a major role in the molecular pathogenesis of the aneurysms, improving matrix repair, decreasing the proteolytic pattern and inhibition of angiotensin converting enzyme as well as preventing angiotensin II-induced AT1R (angiotensin type 1 receptor) activation, can represent new options in the medical therapy of AAs. We propose that a combination of statins and PPAR-gamma agonist could be a useful adjunctive therapy in this condition. The new pathophysiology-based therapeutic approach, involving the pathological patterns and mechanisms leading to the rupture of the AAs, could represent an interesting additional tool in combination with the current established anti-hypertensive therapy.
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PMID:A combination of PPAR-gamma agonists and HMG CoA reductase inhibitors (statins) as a new therapy for the conservative treatment of AAS (aortic aneurysm syndromes). 1957 52

Aortic dissection (AD) is a disease characterized by tear of the aortic intimal layer and separation of the arterial wall. Some risk factor such as hypertension and Marfan syndrome is well known in AD. However, the role of genetic factors in AD is largely unknown. Insertion/deletion (I/D) polymorphism of the angiotensin-converting enzyme (ACE) gene is associated with cardiovascular diseases; patients with D allele have higher serum and tissue ACE levels. We investigated the relationship between the I/D polymorphism of the ACE gene and non-syndromic acute AD. Sixteen patients diagnosed with AD were included in the study (mean age: 60.1 +/- 6.2 years). The diagnosis was established by clinical evaluation and imaging techniques. The control group consisted of 22 age-matched patients without AD (60.9 +/- 7.3 years), who suffered from chest pain. Incidence of hypertension was similar in dissection and control groups (62% vs. 59%). The I/D polymorphism was investigated in both groups by PCR analysis. Dissection types according to the DeBakey classification were identified as type 1 (proximal + distal) in 7 patients (43%), type 2 (proximal) in 5 patients (31%), and type 3 (distal) in 4 patients (25%). The D/D and D/I polymorphisms are present in 13 and 3 AD patients, respectively. None of patients with AD have the II polymorphism. The frequencies of the D allele (DD + ID) are significantly higher in dissection group than control (100% vs. 68%, P < 0001). These results indicate that the D allele of ACE gene is a risk factor for AD.
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PMID:The deletion polymorphism of the angiotensin-converting enzyme gene is associated with acute aortic dissection. 1971 82

The case of 25 years old male patient with symptoms of hypertensive storm in the course of pheochromocytoma was presented. For some years he had been suffering from moderate increase in arterial blood pressure whose secondary cause was not suspected by physicians examining this patient. In presented case the course of pheochromocytoma has special characteristics, such as: clinical presentation in young age, the course alternate between periods of hypertension and phases of normal blood pressure, physical signs of Marfan's syndrome, increase of troponin level within hypertensive storm, and attributes of malignant hypertension presented as transient proteinuria and glycosuria within normoglycemia.
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PMID:[Pheochromocytoma--a disease which is worth to keep in mind]. 1982 37

Aortic aneurysm and dissection cause significant morbidity and mortality. There are several known single gene disorders that predispose to isolated aortic disease and eventually aneurysm and dissection. FBN1 mutations are associated with multiple clinical phenotypes, including Marfan syndrome (MFS), MASS phenotype, and familial ectopia lentis, but rarely with isolated aortic aneurysm and dissection. In this report, we describe three patients who presented with primary descending thoracic aortic dissection and who were found to have an FBN1 mutation. None of the patients fulfilled clinical criteria for the diagnosis of MFS, and all had few or none of the skeletal features typical of the condition. Two patients had a history of long-term hypertension, and such a history was suspected in the third patient. These observations suggest that some individuals with FBN1 mutations have significant aortic disease involvement of other systems that is typical of FBN1 mutation-related syndromes. Superimposed risk factors, such as hypertension, may weaken the aortic wall and eventually lead to aortic dissection. Given that the cost continues to decrease, we suggest that diagnostic DNA sequencing for FBN1 mutations in patients with thoracic aortic aneurysms and dissection may be a practical clinical step in evaluating such patients and at-risk family members.
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PMID:FBN1 mutations in patients with descending thoracic aortic dissections. 2008 64

Aortic dissection is a catastrophic illness that is a significant source of liability for hospitals if diagnosis and treatment are not done promptly. The diagnosis is often difficult to make because not all dissections have the typical presentation of sudden severe chest pain radiating to the back. Symptoms often include abdominal pain, flu-like complaints, vomiting and diarrhea, low back pain, stroke syndromes and syncope. Patients at risk include those with Marfan syndrome and other connective tissue diseases, familial aortic disease, age and hypertension. Aortic dissection is a different clinical entity than abdominal aortic aneurysm. Strategies to reduce risk and improve outcome include staff education on various presentations and risk factors, rapid availability of diagnostic testing modalities such as chest CT scan or transesophageal echocardiogram, and protocols to ensure prompt transfer for cardiothoracic surgery.
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PMID:Case studies in acute aortic dissection: strategies to avoid a catastrophic outcome. 2019 21


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