Aorta of young and middle-aged heterozygous familial hypercholesterolemia patients shows no functional or morphological impairment assessed by MRI.

In familial hypercholesterolemia (FH) the level of LDL cholesterol is 2-3 times that of the normal population and leads to accelerated atherosclerosis. Improved care for risk factors has decreased cardiovascular mortality of these patients. We studied subclinical atherosclerotic changes with morphologic and functional aortic magnetic resonance imaging (MRI) in FH patients under the age of 50.39 DNA test-verified heterozygous FH-North Karelia patients, aged 6-48, 28 of them treated with statins, and 25 healthy controls, aged 12 to 50, underwent aortic MRI, carotid ultrasound (US), and risk-factor assessment. No differences in any of the morphologic or functional aortic parameters appeared between patients and controls. Age and gender were independent predictors of the majority of the morphologic and functional measures. Carotid intima-media thickness assessed by US was greater in patients (0.57 mm +/- 0.13 vs. 0.48 +/- 0.13 mm, p = 0.005) as was cholesterol-years score (243 +/- 122 vs. 137 +/- 74, p < 0.001). Patients had thicker intima-media of the common carotid artery and higher cholesterol burden as indicated by their cholesterol-years score. Despite this, no differences existed in morphologic or functional aortic parameters assessed with MRI. The improved care of cardiovascular risk factors, especially statin treatment, may protect the aorta of FH patients. However, larger confirmatory studies are needed.

Compliance of the aorta in two diseases affecting vascular elasticity, familial hypercholesterolemia and diabetes: a MRI study.

Arterial elasticity changes in familial hypercholesterolemia (FH) and diabetes mellitus (DM) with different but overlapping mechanisms. We compared aortic elasticity between 19 FH patients with the same mutation, 18 type 2 DM patients, and 30 controls, all aged 48 to 64. They underwent aortic magnetic resonance imaging, risk-factor assessment, and carotid and femoral ultrasound measurements. All patients were on adequate cardiovascular medication including statins and had established coronary heart disease (CHD). FH patients had longer-duration CHD (13.3 +/- 7.7 years) than did DM patients (5.0 +/- 3.1). Aortic compliance in the descending thoracic (DM 0.38 +/- 0.14 vs control 0.53 +/- 0.19, P = 0.032) and abdominal aorta (DM 0.45 +/- 0.20 vs control 0.66 +/- 0.25, P = 0.011) was lower in DM patients than in controls, whereas no significant difference existed between FH patients and controls. Carotid and femoral intima-media thickness was greater in FH and DM patients than in controls with no difference between patient groups. Carotid or femoral plaques appeared in 15 (79%) FH and in 10 (56%) DM patients. One control had a femoral plaque. Five FH patients showed stenosis, occlusion or both in carotid arteries. In our opinion, DM patients' lower compliance reflect mainly arterial media affecting arteriosclerosis, while FH patients' plaque status and longer duration of CHD suggest more advanced atherosclerosis. The FH patients may therefore be at increased risk for atherothrombotic events. However, due to small patient material, larger confirmatory studies are needed.

Familial hypercholesterolemia patients treated with statins at no increased risk for intracranial vascular lesions despite increased cholesterol burden and extracranial atherosclerosis.

BACKGROUND AND PURPOSE: To correlate known vascular disease risk factors and the signs of extracranial and intracranial changes of vascular origin in young patients with heterozygous familial hypercholesterolemia (FH). METHODS: 39 DNA test-verified heterozygous FH North Karelian patients (FH-NK), aged 6 to 48, 28 of them treated with statins, and 25 healthy controls underwent brain magnetic resonance imaging (MRI) and carotid ultrasound. RESULTS: Common carotid intima-media thickness was significantly greater in the patients (P=0.005). MR angiography showed no pathological changes, other than 1 incidental aneurysm. The number and size of white matter hyperintensities on T2-weighted MR images, considered as markers of microvascular alterations, did not differ between patients and controls. CONCLUSIONS: FH-NK patients treated with statins seem to be at no increased risk for brain infarcts or other brain lesions of vascular origin when younger than age 50.

Magnetocardiographic indices of left ventricular hypertrophy.

OBJECTIVE: We tested the hypothesis that multichannel magnetocardiographic (MCG) mapping can detect and quantify the degree of left ventricular hypertrophy (LVH). DESIGN: A cross-sectional study. SETTING: Helsinki University Central Hospital, a tertiary referral center. PARTICIPANTS: Forty-two patients with pressure overload induced LVH by gender-specific echocardiographic criteria (LVH group), and 12 healthy middle-aged controls. MAIN OUTCOME MEASURES: MCG QRS-T area integrals and QRS-T angle in magnetic field maps in relation to echocardiographic LVH as well as left ventricular (LV) mass and structure. Conventional 12-lead electrocardiographic (ECG) LVH indices (Sokolow-Lyon voltage, Cornell voltage, Cornell voltage duration product) were assessed for comparison. RESULTS: MCG QRS- and T-wave integrals provided complementary information of echocardiographic LV mass. Their combination, the QRS-T integral, and the QRS-T angle were increased in patients with LVH and, in those patients, correlated significantly with LV mass indexed to body surface area (r = 0.455;P = 0.002 and r= 0.379; P= 0.013, respectively). A QRS-T integral 16000 fT.s had identical sensitivity of 62% at 92% specificity as the gender-adjusted Cornell voltage duration product of 240 micro V.s for the detection of LVH. CONCLUSIONS: The MCG method can detect patients with LVH and also quantify the degree of LVH in patients with increased LV mass.

Treatment of bleeding pseudoaneurysms in patients with chronic pancreatitis.

BACKGROUND: In patients with chronic pancreatitis, an actively bleeding pseudoaneurysm can be life-threatening. Angioembolization is an attractive alternative to often complex operative management, and its feasibility was assessed in a retrospective analysis. METHODS: During 1993-2005, 33 patients (27 males, median age 51 years) with bleeding pancreatic pseudoaneurysms underwent urgent angiographic evaluation followed by angioembolization if possible. Angioembolization was performed in 23 patients, whereas 10 patients required hemostatic surgery, including 6 distal pancreatectomies and 3 vessel ligations. RESULTS: Between 1993 and 2005 33 out of 745 patients (4.4%) admitted for chronic pancreatitis had bleeding pancreatic pseudoaneurysms. The proportion of bleeders out of the total number of hospital admissions for chronic pancreatitis was 33 out of 1,892 (1.7%). The overall success rate of angioembolization was 22 out of 33 (67%) including 3 patients requiring re-embolization for recurrent bleeding. The success rate was 16 out of 20 (80%) when the pseudocyst was in the head of the pancreas, and only 50% when the splenic artery was the source of bleeding. Four of the 5 cases with free bleeding into the peritoneal cavity required operative intervention. The overall mortality and morbidity rates were 2 out of 33 (6%) and 7 out of 33 (21%) respectively, with no significant differences between embolized and operated patients. Angioembolization was associated with a significantly lower need for total blood transfusions and length of hospital stay. During the years 2000-2005, the overall success rate of angioembolization was 95%. CONCLUSIONS: All hemodynamically stable patients with chronic pancreatitis and bleeding pseudoaneurysms should undergo prompt initial angiographic evaluation and embolization if possible. Repeated angioembolization is feasible in patients with recurrent bleeding, whether initially embolized or operated. Patients with unsuccessful embolization should undergo emergency hemostatic surgery with ligation of the bleeding vessel in the head of the pancreas and distal resection in patients bleeding from the splenic artery or its branch. The combination of angioembolization and later endoscopic drainage of the pseudocyst via endoscopic retrograde cholangiopancreatography (ERCP) is effective in the majority of the cases of pseudoaneurysms in chronic pancreatitis.

Characterization of familial and sporadic arrhythmogenic right ventricular cardiomyopathy in Finland.

BACKGROUND: Autosomal dominant inheritance is reported in arrhythmogenic right ventricular cardiomyopathy (ARVC) but the prevalence of the familial and sporadic forms in the general population is unknown. AIM: To evaluate the familial occurrence and clinical features of ARVC in the genetically homogenous Finnish population. METHODS: The study included 29 Finnish ARVC index patients and 135 relatives from 21 families evaluated. They underwent echocardiography, 24-hour electrocardiographic monitoring, signal-averaged electrocardiography, and exercise stress test. RESULTS: Twenty-two index patients had ventricular arrhythmias as first manifestation, and three developed arrhythmias later. The right ventricle (RV) was mildly affected in 22 and strongly dilated in 7 index patients. Patients with dilated RV manifested first symptoms at younger age (mean 28 years) than those without RV dilatation (mean 38 years). Of the 135 relatives, ARVC was present in 12 (9%) patients belonging to 5 of the 21 families studied, resulting in 24% familial involvement. In addition, 46 relatives (34%) had subtle cardiac abnormalities, suggesting subclinical presentation. CONCLUSIONS: The ARVC in Finland presents with distinct arrhythmic and RV dilative subtypes. The sporadic disease is similar to the familial one which may reflect low penetration in relatives. The proportion of familial manifestation of ARVC in Finland seems comparable to that elsewhere in Europe.

Overview of systematic reviews on invasive treatment of stable coronary artery disease.

OBJECTIVES: The aim of the study was to evaluate the validity of the systematic reviews as a source of best evidence and to present and interpret the evidence of the systematic reviews on effectiveness of surgery and percutaneous interventions for stable coronary artery disease. METHODS: Electronic databases were searched without language restriction from January 1966 to March 2004. The databases used included the Cochrane Database of Systematic Reviews, the Cochrane Central Register of Controlled Trials, DARE, the Health Technology Assessment Database, MEDLINE(R), MEDLINE(R) In-Process & Other Non-Indexed Citations. We included systematic reviews of randomized clinical trials on patients with stable coronary heart disease undergoing percutaneous coronary intervention or coronary artery bypass surgery in comparison with medical treatment or a comparison between invasive techniques. At least one of the following outcomes had to be reported: death, myocardial infarction, angina pectoris, revascularization. The methodological quality was assessed using a modified version of the scale devised by Oxman and Guyatt (1991). A standardized data-extraction form was used. The method used to evaluate clinical relevance was carried out with updated method guidelines from the Cochrane Back Research Group. Quantitative synthesis of the effectiveness data is presented. RESULTS: We found nineteen systematic reviews. The median score of validity was 13 points (range, 6-17 points), with a maximum of 18 points. Coronary artery bypass surgery gives better relief of angina, and the need for repeated procedures is reduced after bypass surgery compared with percutaneous interventions. There is inconsistent evidence as to whether bypass surgery improves survival compared with percutaneous intervention. A smaller need for repeated procedures exists after bare metal stent and even more so after drug-eluting stent placement than after percutaneous intervention without stent placement. However, according to the current evidence, these treatment alternatives do not differ in terms of mortality or myocardial infarction. CONCLUSIONS: We found some high-quality systematic reviews. There was evidence on the potential of invasive treatments to provide symptomatic relief. Surgery seems to provide a longer-lasting effect than percutaneous interventions with bare metal stents or without stents. Evidence in favor of drug-eluting stents so far is based on short-term follow-up and mostly on patients with single-vessel disease.

Surgical treatment of inflammatory abdominal aortic aneurysms: a long-term follow-up of 19 patients.

The prevalence of inflammatory abdominal aortic aneurysms (IAAA) in autopsy material ranges between 2.5 and 10% of all aneurysms. Clinical findings, the distinction between inflammatory and degenerative aneurysms, and epidemiological data are uncertain, and only a few long-term follow-up studies of patients after surgical treatment of IAAAs exist. In this study, 19 patients underwent either emergency or elective surgery for IAAA during the 10-year period between 1983 and 1993 at Helsinki University Central Hospital. Demographics, symptoms, and operative and follow-up data were collected retrospectively with emphasis on the long-term outcome of IAAA. Causes of late death were available from hospital records and the central statistical office of Finland. For survival analysis we compared ruptured versus nonruptured and emergency versus elective cases of IAAAs. Mean follow-up for the 18 surviving patients (1 hospital death) was 7.4 years. One patient (5%) died of a long-term complication of the aneurysmal disease. There was no statistically significant difference in survival rates for emergency versus elective surgery cases or ruptured versus nonruptured aneurysms. The most common cause of late death was myocardial infarction. The hospital stay mortality (5%) and morbidity (31%), and the survival rate of 26% at 5 years for ruptured and 65% and 43% for nonruptured IAAAs at 5 years and 10 years, respectively, are comparable to normal AAA survival rates. These findings show that surgery is recommended, especially as ruptures also occur in this subgroup of aneurysms.