Spontaneous angular dichorionic diamniotic twin pregnancy: a case report.

OBJECTIVE: To report a peculiar case of ultrasound diagnosis of spontaneous angular twin pregnancy. In literature, the terms "angular," "interstitial," and "cornual" pregnancies are often used inappropriately. Confusion in terminology may have contributed to difficulties in developing diagnostic ultrasound criteria to differentiate these ectopic pregnancies. DESIGN: Case report. SETTING: Obstetrics and Gynecology, Community Hospital "S. Maria delle Croci." PATIENT: A 28-year-old patient with a previous cesarean delivery was admitted to our hospital for management of a presumed angular ectopic pregnancy. Transvaginal ultrasound confirmed an ectopic dichorionic diamniotic twin pregnancy eccentrically located in the right superior angle of the uterine cavity: the first gestational sac appeared to have right angular implantation, whereas the second gestational sac seemed to deepen inside the myometrium, with a thin myometrial margin of only 3 mm. INTERVENTIONS: After discussing the risks, the patient requested to proceed with termination. A single intramuscular injection of 75 mg of methotrexate was administered, followed by ultrasound-guided hysterosuction after 19 days due to severe vaginal bleeding. MAIN OUTCOME MEASURES: An early and accurate ultrasound diagnosis of a high-risk condition allowed for conservative medical treatment. RESULTS: The serum beta-human chorionic gonadotropin levels progressively decreased. After 1 month, a clinical and ultrasound examination showed a regular endometrial line with a regular reappearance of menstrual bleeding. CONCLUSIONS: Although there are remarkable advances in ultrasound techniques, angular pregnancy remains a condition of difficult diagnosis and management; it is potentially dangerous and may lead to severe complications. An early and accurate diagnosis of this condition is necessary to avoid complications and individualize the subsequent management.

Residual vein thrombosis and onset of post-thrombotic syndrome: influence of the 4G/5G polymorphism of plasminogen activator inhibitor-1 gene.

BACKGROUND: Plasminogen activator inhibitor-1 (PAI-1) is the most important inhibitor of plasminogen activator. The functional 4G/5G polymorphism of the gene coding for PAI-1 may affect PAI-1 plasmatic activity, influencing the imbalance between coagulation and fibrinolysis cascades. In this prospective cohort analytic study, we investigated the role of this single nucleotide polymorphism in the persistence of thrombotic lesion and the occurrence of post-thrombotic syndrome. PATIENTS/METHODS: In a group of 168 patients with post-surgical deep vein thrombosis of the legs, we analyzed the 4G/5G polymorphism in the promoter of PAI-1 gene and plasmatic PAI-1 activity. Enrolled patients were divided in two groups: patients with 4G/5G polymorphism and increased PAI-1 activity (n=85) and patients without 4G/5G polymorphism and normal PAI-1 activity (n=83). All patients were treated according to current protocols and re-examined after 3, 12 and 36 months in order to evaluate the persistence of thrombotic lesion and the occurrence of post-thrombotic syndrome. RESULTS: We found a significantly increased PAI activity in carrier of the 4G allele, who experienced much more frequently a persistence of thrombosis after 3, 12 and 36 months and/or the development of post-thrombosis syndrome, in spite of the anticoagulant treatment. CONCLUSIONS: These data not only confirm the role played by PAI-1 activity and by the 4G/5G SNP of the PAI-1 gene, but also suggest that current therapeutic protocols, recommending the administration of low weight molecular heparin and oral anticoagulant for the treatment of deep vein thrombosis, could be non sufficient for patients genetically predisposed to a less efficient clot lysis.

The association between the 4G/5G polymorphism in the promoter of the plasminogen activator inhibitor-1 gene and extension of postsurgical calf vein thrombosis.

The objective of this study was to evaluate whether the presence of a plasminogen activator inhibitor type 1 (PAI-1) promoter polymorphism 4G/5G could significantly influence the proximal extension of vein thrombosis in spite of anticoagulant treatment in patients with calf vein thrombosis (CVT) following orthopaedic, urological and abdominal surgery. We studied 168 patients with CVT, who had undergone orthopaedic, urological and abdominal surgery, subdivided as follows: first, 50 patients with thrombosis progression; second, 118 patients without thrombosis progression. The 4G/5G polymorphism of the plasminogen activator inhibitor 1 was evaluated in all patients and in 70 healthy matched controls. We also studied PAI-1 activity in plasma. The presence of 4G/5G genotype was significantly increased in the group of patients with the extension of thrombotic lesions and was associated with an increase in CVT extension risk (odds ratio adjusted for sex 2.692; 95% confidence interval 1.302-4.702). Moreover, we observed a significant increase of PAI-1 plasma activity in patients with extension of thrombotic lesion vs. patients without extension (P=0.0001). Patients with 4G/5G genotype in the promoter of the plasminogen activator inhibitor - 1 gene present a higher risk of extension of thrombotic lesions.

[Cardiorenal syndrome: the role of new biochemical markers]. / Sindrome cardiorenale: il ruolo dei nuovi marker biochimici.

Cardiorenal syndrome is a pathophysiological heart and kidney disorder, in which acute or chronic dysfunction of one organ induces a damage in the other. It's a syndrome more and more often encountered in clinical practice and this implies the need to recognize the syndrome through biochemical markers with a good sensitivity and specificity, since its earliest stages in order to optimize therapy. In addition to widely validated biomarkers, such as BNP, pro BNP, creatinine, GFR and cystatin C, other promising molecules are available, like NGAL (neutrophil gelatinase-associated lipocalin, KIM-1 (kidney injury molecule-1), MCP-1 (monocyte chemotactic peptide), Netrin-1, interleuchin 18 and NAG (N-acetyl-ß-glucosa-minidase). The role of these emerging biomarkers is still not completely clarified: hence the need of new clinical trials.

[Metabolic syndrome in children and in adults: is it an autonomous nosological entity?]. / Sindrome metabolica nel bambino e nell'adulto: è ancora un'entità nosologica autonoma?

The term metabolic syndrome defines a cluster of risk factors for cardiovascular diseases and diabetes. This syndrome is very frequent in general population. However, despite the efforts to define the diagnostic criteria, nowadays its clinical utility is object of a lively debate.

Diagnosis and definition of biventricular non-compaction associated to Ebstein's anomaly.

BACKGROUND: Non-compaction of ventricular myocardium is a rare congenital cardiomyopathy characterized by the presence of an extremely thickened endocardial layer with prominent trabeculations and deep recesses in communication with ventricular chamber and determining the typical spongeous aspect. The diagnosis of non-compaction of ventricular myocardium is possible through the identification of morphological alterations by echocardiographic evaluation. Ebstein's anomaly is a rare congenital cardiac disease, defined as the significant apical displacement of the part of the tricuspid valve causing significant tricuspid regurgitation and reduction of the functional right ventricle, right atrial and right ventricular dilatation and atrial and ventricular arrhythmias. CASE REPORT: We present a case of biventricular non-compaction and Ebstein's anomaly in a 29-year-old Italian man that was referred for chest pain. Diagnosis of Ebstein's anomaly was made during a medical control for military service through an echocardiographic evaluation which left the suspicion of myocardium non-compaction. We present the cardiac image of the 2D and 3D eco, RMN, scintigraphy and ventriculaography.

Protrhombotic effects of contraceptives.

The use of oral contraceptives first became widespread some 40 years ago, and reports of an excess risk of cardiovascular disease among women who used these agents soon followed. Few drugs have been the object of such intensive epidemiological research, the outcome of which has provided clinicians with detailed information about risks not only of specific thrombotic diseases but also important non-contraceptive benefits from the pill. Recently, oral contraceptives have been classified by some according to "generation" (first, second, third, and most recently, fourth generation): first-generation formulations containing lynestrenol or norethindrone, second-generation formulations containing levonorgestrel, third-generation formulations containing desogestrel or gestodene, and oral contraceptives containing an estrogen and other progestagens (cyproterone or norgestimate) or a progestagen alone. The results of several study was that the use of the older high-dose oral contraceptives increased the risk of cardiovascular disease by modifying the Low-density lipoprotein and High-Density lipoprotein cholesterol level, increasing triglyceride serum level, reducing glucose tolerance, raising blood pressure, and promoting clotting mechanisms. In this review we investigate the mechanism of the oral contraceptives and performed a risk assessment of every generation.

Patent foramen ovale and thromboembolic complications.

The foramen ovale, an atrial septal defect which is essential in the fetal circulation, remains patent through adulthood in approximately 25% of the general population and so it represents the most common persistent abnormality of fetal origin. Patent foramen ovale (PFO) allows interatrial right-to-left blood shunting during those periods of the cardiac cycle in which the right atrial pressure exceeds the left one. An increasing number of pathological manifestations of PFO has been recently identified; among these, paradoxical systemic embolism, refractory hypoxemia in patients with right ventricular myocardium infarction or severe pulmonary disease, orthostatic oxygen desaturation in the rare platypnea-orthodeoxia syndrome, neurological decompression illness in divers, high altitude pilots and astronauts, and finally, migraine headache with aura. Nowadays many techniques allow to detect a PFO. In this study we investigated each of them, assessing their potential diagnostic role even in comparison with the main features of the other methods.

An update on the role of markers of inflammation in atherosclerosis.

In recent years, several studies have used the measurement of carotid intima-media thickness (IMT) as a marker of early atherosclerosis: IMT has been shown to correlate significantly with the presence of coronary artery disease (CAD) and to predict fatal and not fatal cerebro- and cardio-vascular events. These findings highlight the importance of recognizing and managing early stages of atherosclerosis for effective cardiovascular prevention. Beyond traditional established cardiovascular risk factors, inflammation has been shown to be crucial throughout atherosclerosis from endothelial dysfunction to plaque rupture and thrombosis. Several studies have shown the existence of a strong relation between CAD and fibrinogen or highly sensitive C-reactive protein (hs-CRP) levels and their predictive role has been examined through stratification or multivariable statistical analyses: levels of these markers of inflammation have been independently associated with the incidence of coronary events after adjusting for traditional cardiovascular risk factors. Recent studies have further addressed the importance of therapeutical modulation of hs-CRP levels in high-risk patients for the prevention of vascular events. The strong relationship between hs-CRP and IMT may potentially account for the complex role of hs-CRP and IMT in the pathogenesis of cardiovascular events. However, beyond the utility of measuring markers of inflammation to assess patients with subclinical carotid atherosclerosis at higher risk of vascular events, further studies are needed to evaluate the therapeutic implications in this category of patients.

Ventricular dysfunction and number of non compacted segments in non compaction: non-independent predictors.

BACKGROUND: Isolated ventricular noncompaction (IVNC) is characterized by multiple prominent trabeculations and deep intertrabecular recesses. Some reports prove that the chronic heart failure may occur in approximately half of the patients. In this report we investigate the correlation between the number of non compacted segments and entity of systolic dysfunction from the registry and subregistries of the SIEC. METHOD: To identify the correlation between ventricular dysfunction and number of segments involved in non compaction we evaluated a consecutive series of 238 patients affected by non compaction, from the SIEC (Società Italiana di Ecografia Cardiovascolare) registry. The average age of patients was 41.5 years (range: 1-92 years), 137 were males and 101 females. In 122 cases we found ventricular systolic dysfunctions with an EF average of 34.6%. The number of affected segments by non-compactation and diastolic dysfunction were found to be non-independent predictors of LV systolic dysfunction. CONCLUSION: From the analyses we carried out, it seems that ventricular dysfunction seems to be completely independent from the segment numbers of non compacted segments.

Magnetic resonance in isolated noncompaction of the ventricular myocardium.

UNLABELLED: Non-compaction of the ventricular myocardium (LCVM) is a rare disorder of myocardial morphogenesis usually diagnosed in paediatric age. The diagnosis was echocardiographically made on the basis of a reported spongeous/compacted ratio >2 in one or more segments of the left ventricle during the diastolic period. We aimed to test the diagnostic accuracy of cardiovascular magnetic resonance (CMR) imaging in distinguishing pathological left ventricular non-compaction. METHODS: We collected a consecutive series of 8 patients, 5 males and 3 females, with a mean age of 14.9 years with non-compaction of left ventricular myocardium. All patients were admitted in our divisions of cardiology. In all cases the diagnosis was performed by echocardiography. The diagnosis was obtained when the spongeous/compacted ratio was >2 in one or more segments of left ventricle, evaluated in systolic and diastolic period. In the end we completed the diagnosis by scanning with a Signa HD 1.5 T (GE, Milwaukee, USA) the same 8 patients affected by non compaction of ventricular myocardium. In all patients cardiac-gated T1 and T2 black-blood FSE images in short axis and in four-chamber horizontal long axis were obtained. Breath hold cine MR sequences (FIESTA) were performed, covering the whole left ventricle in short-axis plane and in four-chamber view. A segmented inversion-recovery fast gradient echo sequence (IR-FGE) was performed in the short-axis plane of the LV and in four-chamber-view after Gadolinium injection in 8 patients affected by non compaction of left ventricle. At the end of examination the spongeous/compacted ratio >2 was calculated in all involved segments of the left ventricle in diastole. RESULTS: In all cases we demonstrated by echocardiography an involvement of the ventricular apex. In 3 cases the structural alterations involved also lateral wall of left ventricle. Magnetic resonance evaluation showed that involvement demonstrated by the echocardiogram was the same: ventricular apex involved in every patient, lateral wall in 3 and all segments in 2. However the spongeous/compacted ratio was >>2 in all patients, with a mean value of 3,1. CONCLUSIONS: Although our data refer to a small population of patients and need further confirmation, they suggest that it seems reasonable increase the cut-off for spongeous/compacted ratio from a value of 2 to 2.5 for non-compaction diagnosis when high-resolution magnetic resonance is used.

Asymptomatic ventricular pre-excitation in children.

OBJECTIVE: This retrospective study was planned for a good risk assessment of asymptomatic patients affected by ventricular pre-excitation. METHODS: From 1985 to 2007, 124 patients with an atrioventricular pathway (electrocardiographic signs of ventricular pre-excitation) were admitted to our cardiology division. The average age was 7 years (range 1 month to 18 years). The mean follow-up period in the whole population of patients was 4.2 years (range 1-13 years). Four patients were lost during the follow-up. During this period, all patients remained in good health. In all of them, we performed a Holter evaluation every year. An intermittent pathway was detected in 18 patients (15%), and four of them (3.4%) showed a supraventricular tachycardia even though they were asymptomatic patients. An ergometric test was performed in 76 asymptomatic patients; 16 children (21%) showed a total abrupt vanishing of delta wave. A transoesophageal electrophysiological evaluation was performed in 14 patients. CONCLUSION: According to our data, asymptomatic Wolff-Parkinson-White syndrome in children has a good outcome during a short-term (4 years) follow-up. The usefulness of electrophysiological evaluation (in particular its predictive value) is uncertain.

Anticoagulant drugs in noncompaction: a mandatory therapy?

BACKGROUND: Noncompaction of left ventricular myocardium is a rare congenital cardiomyopathy resulting from an incomplete myocardial morphogenesis that leads to the persistence of the embryonic myocardium. This condition is characterized by a thin compacted epicardial and an extremely thickened endocardial layer with prominent trabeculations and deep intertrabecular recesses. It is not clear, in noncompaction of myocardium, whether intertrabecular recesses could be responsible for thrombi formation and thromboembolic complications. METHODS: The prevalence of stroke and echocardiographic finding of thrombus was evaluated in a continuous series of 229 patients (men and women) affected by noncompaction of the left ventricular myocardium, who were included in the SIEC registry. We excluded patients affected by atrial fibrillation. RESULTS: The mean age of the patients was 49.5 years. Fifty percent of the patients were affected by a ventricular systolic dysfunction. The mean period of follow-up was 7.3 years. Only four patients had a history of ischemic stroke. A large thrombus into the left ventricular chamber was observed in a 1-year-old child affected by Behcet's disease (high risk of thrombi formation). CONCLUSION: Noncompaction of the left ventricular myocardium, by itself, does not seem to be a risk factor for stroke or embolic results, so there is no indication for oral anticoagulant therapy.

Peripheral atherosclerosis is associated with the occurrence of restenosis after percutaneous coronary intervention.

INTRODUCTION: The aim of our study was to evaluate, in patients with proven coronary artery disease (CAD) and treated with elective percutaneous coronary intervention (PCI), whether the coexistence of asymptomatic carotid and femoral atherosclerotic lesions would provide prognostic information in terms of occurrence of restenosis. METHODS: We studied 104 patients with CAD (M/F=77/27), mean age 60.5+/-9 years. All patients were treated with elective PCI. After PCI the suspicion of restenosis was confirmed by coronary angiography. All patients underwent ultrasound duplex scan of carotid and femoral-popliteal-tibial axis to detect atherosclerotic lesions. According to ultrasound results, patients were classified as normal, with increased intima-media thickness (IMT) or with asymptomatic plaque (AP). If carotid and femoral lesions coexisted (together with coronary ones) patients were considered to have multifocal atherosclerosis. RESULTS: About 90% of the patients had carotid lesions: 40% had carotid IMT and 50% AP. Femoral lesions were found in 72% of the population and in 41% there was an increased IMT and in 21% an increased AP. Prevalence of restenosis after PCI was 12.5%. Patients with restenosis had a significantly higher prevalence of asymptomatic carotid and/or femoral lesions than those without restenosis. The occurrence of restenosis was independently associated with the detection of carotid, femoral and multifocal atherosclerosis. CONCLUSION: The detection of carotid and/or peripheral atherosclerotic lesions in patients with CAD who underwent PCI may be a marker of increased risk. We believe that investigating these areas, by echo-Doppler duplex scanning, may be a cost-effective strategy in the work-up before elective PCI. It may allow identification of high-risk subgroups of patients, and enable the planning of patient-tailored therapeutic strategies and follow-up.

The treatment of venous leg ulcers: a new therapeutic use of iloprost.

BACKGROUND: We conducted a study using an intravenous (i.v.) infusion of iloprost in the treatment of venous ulcers to verify whether the association of i.v. iloprost + local therapy + elastic compression has a favorable effect when compared with traditional treatment with local therapy and elastic compression. STUDY DESIGN: We evaluated the effects of iloprost in 98 consecutive patients with noncomplicated venous ulcers of lower limbs subdivided into 2 groups: the first group (48 patients) received iloprost in saline solution for 3 weeks and the second group (50 patients) received a venous infusion of a saline solution. The patients were examined at baseline time 0 (first visit) and then after 15, 30, 45, 60, 75, 90, 105, 120, 135, and 150 days. RESULTS: In the first group, after 90 days, all the ulcers had healed, whereas in the second group only 50% of ulcers had healed after 105 days. At the end of the study, in the second group only 84.09% of ulcers had healed. The statistical analysis showed a significant difference between the first (iloprost group) and the second group (placebo group). Besides, in the first group the cicatrization of the ulcer happened in a shorter period (27.90% after 60 days; 41.86% after 75 days; and 100% after 100 days) whereas in the second group, at the end of the study, in 15.91% of patients the ulcers had not recovered. CONCLUSION: Iloprost can significantly reduce healing time for venous leg ulcers through several actions.

Optimal duration of treatment in surgical patients with calf venous thrombosis involving one or more veins.

The aim of this study was to evaluate different durations of treatment in patients with calf venous thrombosis (CVT) involving 1 or more deep veins. The authors studied 2 groups of patients with postsurgical CVT diagnosed by echo-color Doppler. The first group consisted of 68 patients with CVT involving a single vein, and the second group consisted of 124 patients with CVT involving 2 or more veins. Immediately after diagnosis, all patients were treated with nadroparin calcium and sodium warfarin. Heparin treatment was withdrawn after 5-6 days of treatment, when the international normalized ratio (INR) was stabilized between 2 and 3. Each group was divided into 2 subgroups receiving anticoagulation treatment for 6 or 12 weeks, respectively. The endpoint was proximal extension of the thrombotic lesion, defined as the extension of the thrombus to the popliteal and/or femoral vein. In patients with single-vessel CVT there was no significant difference between the 2 subgroups, whereas in patients with CVT involving 2 or more vessels, a statistically significant difference was observed, the number of cases showing proximal extension of the thrombus being higher among patients treated for 6 weeks. Twelve weeks of anticoagulation treatment is better than 6 weeks only in patients with postsurgical CVT involving 2 or more veins.

Methylenetetrahydrofolate reductase mutation in subjects with abdominal aortic aneurysm subdivided for age.

The aim of the study was to verify the association between abdominal aortic aneurysms (AAA) and methylenetetrahydrofolate reductase (MTHFR) mutation, in relation to age. We studied the frequency of the MTHFR 677T allele in two groups of AAA patients, over and under 60 years. The first AAA group included 42 patients (30 men and 12 women) aged between 65 and 75 years; the second AAA group included 46 patients (32 men and 14 women) aged between 49 and 59 years; the control group included 44 healthy controls (29 men and 15 women) aged between 49 and 75 years. We examined MTHFR allele frequency and MTHFR genotype using Nuclear Laser Medicine. MTHFR allele frequency was significantly increased in AAA patients >60 compared to healthy controls and in AAA patients <60 compared to those >60. The genotype study showed a difference between controls and AAA patients and between AAA patients >60 and those <60. The frequency of MTHFR mutation was more elevated in both AAA groups vs controls, but it was more elevated in younger patients than in the older ones. This mutation might induce an early elastin degradation in the aortic wall.

A case of ovarian hyperstimulation syndrome associated with the methylenetetrahydrofolate reductase mutation gene.

OBJECTIVE: To report a case of ovarian hyperstimulation syndrome with methylenetetrahydrofolate reductase (MTHFR) gene 677T homozygosis mutation and A1298C gene heterozygosis mutation. DESIGN: Case report. SETTING: A pregnant woman in an academic hospital. PATIENT(S): A woman with ovarian hyperstimulation syndrome. INTERVENTION(S): Nadroparin was administered for 2 weeks at a dosage of 200 IU/kg twice per day and then once per day; also administered once per day were folates, 5 mg; B6 vitamin, 15 mg; and B12 vitamin, 1 mg. MAIN OUTCOME MEASURE(S): Clinical follow-up. RESULT(S): Delivery was regular within the set time limits, and the fetus was born alive and in good health. CONCLUSION(S): We believe that MTHFR mutation research could be executed in women before ovarian stimulation treatment, but other observations are necessary to support this recommendation.

Subclavian stenosis/occlusion in patients with subclavian steal and previous bypass of internal mammary interventricular anterior artery: medical or surgical treatment?

There are only a few published studies on the association between subclavian steal syndrome and ischemic heart disease. The objective of this report is to evaluate the efficacy of subclavian steno-occlusion treatment in patients with subclavian steal syndrome (SSS) and previous coronary bypass. Over the last 8A years we observed 207 patients who underwent left internal mammary artery-intraventricular artery (LIMA-IVA) bypass graft. Of these, 31 patients were affected by steno-occlusion of the homolateral subclavian artery. Ten patients (group 1) showed latent vertebral-SSS and were pharmacologically treated. Seven patients (group 2) had an intermittent vertebral-SSS; four patients were treated with angioplasty and stent application and three were pharmacologically treated. Fourteen patients (group 3) with complete vertebral-SSS were treated with angioplasty and stent application or carotid-subclavian bypass graft. All patients were followed up every 3 months for a period of 5A years after the diagnosis. The first group of patients showed no angina and no sign of subclavian restenosis. In the second group only two patients, who were affected by angina, showed subclavian restenosis at angiography. In the third group only one patient underwent further angioplasty for restenosis. The results of this study show that the SSS may be an adverse event in patients with a LIMA-IVA bypass graft. Identification of the steal is essential to choose an appropriate therapeutic approach.