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Aortic stiffening is as much an important risk factor in cardiovascular morbidity and mortality, as it serves as reliable surrogate marker for clinical endpoints like myocardial and cerebrovascular incidents. Elevated aortic stiffness induces high systolic blood pressure, augmented pulse pressure with increased ventricular afterload, reduced subendocardial blood flow and augmented pulsatile stress in the peripheral arteries. Factors with relevant impact on the epidemiology of arterial stiffness are widely spread. 3 major groups of parameters influencing the stiffness of the aorta and the large arteries have been studied and described up to now: (i) physiological properties like age, gender, body height, pressure, hormonal state, genetic factors; (ii) environmental factors like nutrition (fish-, salt-, garlic consumption), smoking, performance of sports and aerobic capacity; (iii) diseases like hypertension, hypercholesterolemia, diabetes, coronary heart disease, cerebrovascular disease, renal failure, Marfan-syndrome, growth hormone deficiency. Close association between several of these factors impedes analyzing them independently from each other. Age and blood pressure were found to be the most prominent predictors of arterial stiffness in normal as well as in disease populations. Physiological and environmental factors can modulate these effects of aging, diseases generally seem to amplify them.
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PMID:Epidemiology of the arterial stiffness. 1047 71

Osteogenesis imperfecta (OI) is an inherited connective tissue disorder, a group that includes Ehlers-Danlos syndrome, Marfan's syndrome and pseudoxanthoma elasticum. OI is a heterogeneous disease of collagen I biosynthesis characterized by variable clinical phenotypes, including skeletal and cardiovascular manifestations. A 65-year-old man with OI who had extensive prior successful cardiac valve surgeries is described. He survived for 18 years after his initial valve surgery, but died of multiorgan failure and sepsis after repair of a spontaneous type A aortic dissection. This is the fourth reported case of aortic dissection secondary to OI and illustrates the extensive cardiovascular pathology associated with OI. Aggressive management of arterial dissection risk factors, such as systemic arterial hypertension, is advocated for patients with OI.
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PMID:Aortic dissection: a rare complication of osteogenesis imperfecta. 1052 81

Maternal adaptation to pregnancy includes reproductive hormone interaction plasma, volume changes with an increase in total body water, vascular alterations with a decrease in systemic resistance and modifications associated with hypercoagulability. These explain, in part, the appearance of signs and symptoms, even in a normal pregnant woman, that are difficult to distinguish from those occurring in heart disease and why some cardiac abnormalities are not well tolerated during pregnancy. Cardiovascular abnormalities are considered the first non-obstetric cause of morbidity and mortality during pregnancy. Rheumatic and congenital heart diseases are currently the most frequent cardiopathy found in women of childbearing age, followed by hypertension, coronary artery disease and arrhythmia. Although pregnancy is well tolerated by most women with heart disease, there are some cardiovascular abnormalities which place the mother and the infant at extremely high risk: patients with congestive heart failure and severe cardiac dysfunction, pulmonary hypertension, cyanotic congenital heart disease, Marfan's syndrome, severe obstructive lesions of the left side of the heart, patients with prosthetic cardiac valves and antecedents of peripartum cardiomyopathy should be encouraged to avoid pregnancy and the interruption of pregnancy may be advisable in cases with great risk of disability or death. The most severe cardiopathies significantly increase the risk of fetal loss and the presence of a congenital cardiac abnormality in either parent increases the risk of congenital cardiac disease in the fetus. Medical care must be initiated early, prior to conception and women with cardiopathy should be informed of the possible risks of pregnancy to both the mother and fetus.
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PMID:[Practice Guidelines of the Spanish Society of Cardiology for the management of cardiac disease in pregnancy]. 1108 6

Intracranial aneurysmal hemorrhage is a common but devastating condition associated with significant morbidity and mortality. Epidemiologic studies have identified risk factors associated with this condition. Genetic factors involve family history and the presence of certain heritable connective tissue disorders such as Ehlers-Danlos syndrome, Marfan's syndrome, neurofibromatosis, and polycystic kidney disease. Acquired factors include traumatic brain injury, sepsis, smoking, and hypertension. Management of these patients consists of prevention, patient screening, and prophylactic aneurysm repair.
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PMID:Epidemiology of intracranial aneurysm and subarachnoid hemorrhage. 1123 60

Aortic dissection is a life-threatening cardiovascular emergency requiring immediate diagnosis and treatment. It is mainly associated with hypertension and the Marfan syndrome. Diagnosis has been revolutionized by the use of transesophageal echocardiography (TEE), which allows for rapid and safe assessment of this condition. Echocardiographic hallmarks of aortic dissection ar the presence of a dissection membrane separating a true from a false lumen, rupture sites in the membrane with to-and-from flow, aortic regurgitation, and pericardial effusion. Dissection of the ascending aorta, which has an extremely high mortality and necessitates emergency surgery, is diagnosed quickly and accurately by TEE.
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PMID:Aortic dissection. 1123 67

This report describes a case of a Stanford Type B aortic dissection (originating distal to the left subclavian artery and extending to the aortic bifurcation and proximal left iliac artery) in a 31-year-old primigravid woman who was at 39 weeks gestation and had Marfan's syndrome. The dissection was managed conservatively. Caesarean section was performed under epidural anaesthesia with aggressive control of hypertension. Postoperatively, there was no extension of the dissection and no aneurysm formation. She was discharged from hospital two weeks after delivery and remained asymptomatic at six months. There are no plans for surgical intervention.
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PMID:Anaesthetic management of a caesarean section in a patient with Marfan's syndrome and aortic dissection. 1126 16

The physiological infrarenal aortic diameter varies between 12.4 mm in women an 27.6 mm in men. As defined, an aneurysmatic dilatation begins with 29 mm. According to that, 9% of all people above the age of 65 are affected by an abdominal aortic aneurysm (AAA). Compared with the female sex, the male sex predominates at a rate of about 5:1. The disease is predominant in men of the white race. In black men, black and white women the incidence of AAA is identical. 38 to 50 percent of the AAA patients (patients) suffer from hypertension, 33 to 60% from coronary, 28% from cerebrovascular and 25% from peripheral occlusive disease. The AAA expansion rate varies between 0.2 and 0.8 cm per year and is exponential from a diameter of 5 cm on. In autopsy studies, the rupture rates with AAA diameters of < 5 cm, between 5.1 and 6.9 cm, and of > 7 cm were below 5%, 39% and 65%, respectively. 70% of the AAA patients do not die of a rupture, but of a cardiac disease. Serum markers, such as metalloproteinases and procollagen peptides are significantly increased in AAA patients. Thoraco-abdominal aneurysms (TAA) make up only 2 to 5% of all degenerative aneurysms. 20 to 30% of the TAA patients are also affected by an AAA. 80% of the TAA are degenerative, 15 to 20% are a consequence of the chronic dissection--including 5% of Marfan patients--, 2% occur in case of infections and 1 to 2% in case of aortitis. The TAA incidence in 100,000 person-years is 5.9% during a monitoring period of 30 years. In case of TAA, an operation is indicated with a maximum diameter of 5.5 to 6 cm and more and, in case of a Marfan's syndrome (incidence of 1:10,000), with a maximum diameter of 5.5 cm and more. With regard to aorto-iliac occlusive diseases, there are defined 3 types of distribution. Type I refers to the region of the bifurcation itself. Type II defines the diffuse aorto-iliac spread of the disease. Type III designates multiple-level occlusions also beyond the inguinal ligament. Type I patients in most cases are female and more frequently suffer from hyperlipidaemia, while Type II patients are affected by hypertension and diabetes. Compared to Type II patients, Type I patients have a life expectancy that is 10 years higher. Type I and Type II patients often suffer from a pelvic claudication and, unlike Type III patients, are more frequently affected by arterio-arterial embolisms.
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PMID:[Epidemiology of aortic disease: aneurysm, dissection, occlusion]. 1155 76

Spontaneous infrarenal abdominal aortic dissection is rare. We observed enlargement of a spontaneous infrarenal aortoiliac dissection in a 55-year-old hypertensive man. Open surgical repair with a bifurcated polyester graft was successful. A review of the English literature found 41 previously published cases. Mean age was 58 years, 74% of the patients were male, and 62% had hypertension. None had Marfan or Ehlers-Danlos syndrome. More than three fourths of the patients had symptoms, 6 patients (14%) presented with aortic rupture. Dissection was limited to the infrarenal aorta in 50% and extended into the iliac or femoral arteries in 50%. Three patients died before treatment, no death occurred after endovascular repair of after elective open aortic grafting. Mortality following rupture was 67%. Abdominal aortic dissection did not reoccur but 1 patient died at 14 month because of rupture of a thoracic aneurysm. Spontaneous infrarenal abdominal aortic dissections are rare, but usually symptomatic and 14% rupture. Rupture carries high mortality. Elective open repair is recommended, but endovascular repair is a new treatment option for suitable patients.
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PMID:Spontaneous dissection of the infrarenal abdominal aorta. 1206 Sep 3

We report here the management of labor for a 33-year-old woman with Marfan's syndrome. She was diagnosed as Marfan's syndrome at the age of 5 and experienced corrective surgery for abdominal aortic aneurysm at 28 years of age. As there was no progression of cardiovascular lesion, she was allowed to be pregnant. She was planned to proceed with vaginal delivery, since she was in trouble of circulation during her gestational period. In order to prevent catastrophe such as aortic dissection or aortic regurgitation elicited by hypertension related with labor pain, we performed continuous epidural anesthesia to control labor pain under the invasive blood pressure monitoring. Two epidural catheters were inserted into the epidural space via the L 2-3 and the L 5-S 1 intervertebral space, and mixed solutions containing both 0.125% bupivacaine and 0.0002% fentanyl were administered continuously. After 7 hours and 47 minutes from the start of her labor, she delivered her baby vaginally with the aid of forceps technique due to attenuated abdominal muscle activity. No cardiovascular mishaps occurred during her labor and she was discharged 6 days after the delivery. Thus, continuous epidural anesthesia with intensive monitoring of circulation may be useful for vaginal delivery in a patient with Marfan's syndrome by avoiding cardiovascular complications due to labor pain.
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PMID:[Continuous epidural analgesia with intensive monitoring of cardiovascular system for vaginal delivery in a patient with Marfan's syndrome]. 1222 48

The major diseases affecting the aorta are aortic aneurysms and dissections, which are classified based on anatomic location. Diseases affecting the ascending aorta, such as thoracic aortic aneurysms and type I and II dissections, are primarily associated with medial necrosis on pathologic examination. Medial necrosis is characterized by fragmentation and loss of elastic fibers, loss of smooth muscle cells, and interstitial collections of collagenous tissue and basophilic ground substance. Medial necrosis occurs as part of the normal aging of the aorta but is accelerated by other conditions, including hypertension and genetic alterations that predispose persons to these aortic diseases. The etiologies of many of the genetic syndromes, such as Marfan syndrome, that predispose persons to thoracic aortic aneurysms and dissections are understood. Studies are just beginning to elucidate the genes that predispose persons without known syndromes to these aortic diseases, and a major locus for this condition, termed the TAAD1 locus, has been mapped to 5q13-14. Future characterization of this gene and others will enhance the ability to determine persons at risk for aortic aneurysms and dissections and will define molecular mechanisms involved in the pathogenesis of this disorder.
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PMID:Genetic basis of thoracic aortic aneurysms and dissections. 1246 12


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