Prognostic assessment and management of a patient with Carmi syndrome. A case report.

Carmi syndrome is a rare and severe disease defined by pyloric atresia and junctional epidermolysis bullosa. There are no clear recommendations when to consider a curative therapy, including surgical repair of pyloric atresia and when to transition to palliative care. We report the case of a female preterm infant suffering from Carmi syndrome. After definitive diagnosis and appropriate ethical counselling, we decided for surgical repair of the pyloric atresia. Nonetheless, there was no clinical improvement and our patient died after 35 days. Reviewing the literature, we found immunofluorescence microscopy to be most decisive examination to determine the prognosis of this severe disease.

Laparoscopic sleeve gastrectomy reduces the predicted coronary heart disease risk and the vascular age in obese subjects.

BACKGROUND: Obesity is associated with high prevalence of coronary heart disease (CHD) and long term increased cardiovascular morbi-mortality. There are no data regarding the effect of laparoscopic sleeve gastrectomy (LSG) on long-term CHD - risk. It is known that "a man is as old as his arteries" and this concept is illustrated by Framingham coronary risk score, which can predict vascular age. PURPOSE: To assess the 10-year CHD risk in patients with obesity, preoperatively, and 6 and 12 months after LSG. METHODS: 47 consecutive obese subjects (44.7% males, mean age 39.8 years) scheduled for LSG were prospectively studied before and 6 and 12 months after surgery. The 10 years CHD risk and corresponding vascular age were calculated using Framingham risk score. RESULTS: The body mass index (BMI) decreased from 44.6 ± 10.6 kg m2 preoperatively to 32.2 ± 6.9 kg m2 and to 29.4 ± 5.4 kg m2 at 6 and 12 months follow-up (both p 0.05). Mean excessive weight loss (EWL) was 67.3 ± 23.7% and 78.3 ± 23.4% at 6 and 12 months postoperatively. At 6 and 12 months after LSG, there was a marked improvment of lipid profile(decrease of total cholesterol, LDL-cholesterol, triglycerides and increase of HDL-cholesterol) and a significant decrease in prevalence of diabetes mellitus, systemic hypertension and smoking. The 10-year CHD risk reduced from 10.1% preoperatively to 3.5% and to 2.2% at 6 and 12 months after surgery (both p 0.05). Patients' mean vascular age was 65.6 years preoperatively and decreased to 45.8 years 6 month spostoperatively (p 0.05) and to 40.7 years one year after LSG (p 0.05 vs. 6 months postoperatively, p=NS vs.chronological age). CONCLUSIONS: In obese subjects, CHD risk is significantly reduced early, beginning with 6 months after LSG and is diminished with 80% one year postoperatively. Despite the fact that not all patients had achieved the ideal weight yet,mean vascular age is similar to their chronological age one year after surgery.

Fibromuscular dysplasia--a rare cause of renovascular hypertension. Case study and overview of the literature data.

Renal artery stenosis (RAS) is associated with increased cardiovascular mortality and morbidity and may constitute a treatable cause of secondary hypertension. Fibromuscular dysplasia is frequently affecting children as the main cause of RAS, but is very rare in adults. We present the case of a 19-year-old overweight patient, with no known pathological conditions in her medical history or family background, admitted for severe, pulsing headaches during the past 3 months and increased blood pressure (BP) values for about a month (maximum BP 220/140 mmHg). The initial clinical exam and first-line imagistic methods did not provide a high suspicion for RAS. However, the invasive methods established the diagnosis of right renal artery medial dysplasia. Balloon angioplasty was the treatment of choice.

Aortic regurgitation due to quadricuspid aortic valve--case report and literature review.

Quadricuspid aortic valve is a rare variant of aortic semilunar valve, often being an unexpected discovery during cardiac surgery. We present the case of a 59 years old patient, who was admitted for dyspnea on light exertion and palpitations. The transthoracic echocardiography revealed severe aortic valve regurgitation due to a quadricuspid aortic valve, the result being confirmed by the transesophageal echo examination. The patient had a first class indication for aortic valve replacement and the surgical intervention was uneventful. The operative technique and case particularities are discussed in view of the literature published so far regarding this uncommon condition.

Coarctation of the aorta in adults: what is the best treatment? Case report and literature review.

Coarctation of the aorta is a congenital cardiac malformation that can go undiagnosed until old age with only hypertension as a marker of its presence because clinical signs can be subtle and overlooked if a complete physical exam is not performed. We report the case of a 45 year-old women, diagnosed with severe coarctation of the aorta just distal to the left subclavian artery, with poststenotic dilatation of the descending aorta and difficult control of blood pressure values. The patient was successfully treated interventionally, by balloon angioplasty with deployment of a covered stent. We review here the different methods employed for the treatment of coarctation of the aorta in adults, including surgical or percutaneous balloon angioplasty with or without stent placement, underlying their complications and the factors that influence the choice of the best coarctation repair method.

Cardiac magnetic resonance imaging in ischemic heart disease: a clinical review.

Cardiac magnetic resonance imaging (MRI) has emerged as a prime player in the clinical and preclinical detection of ischemic heart disease (IHD) as well in the prognosis assessment by offering a comprehensive approach for all spectrums of coronary artery disease (CAD) patients. The aim of this review is to provide the reader a state-of-the art on how the newest cardiac MRI techniques can be used to study IHD patients. In patients with suspected/stable CAD, functional and perfusion imaging both at rest and during vasodilatatory stress (adenosine, dypiridamole)/dobutamine stress can accurately depict ischemic myocardium secondary to significant coronary artery stenosis. In patients with acute MI, MRI is a robust tool for differentiating and sizing the jeopardized and the infarcted myocardium by using a combination of functional, edema, perfusion and Gd contrast imaging. Moreover, important prognostic factors like myocardial salvage, the presence of microvascular obstruction (MVO), post reperfusion myocardial hemorrhage, RV involvement and infarct related complications can be assessed in the same examination. In patients with chronic ischemic cardiomyopathy, the role of the MRI extends from diagnosis by means of Gadolinium contrast scar imaging to therapy and prognosis by functional assessment and viability testing with rest and dobutamine stress imaging. In all the circumstances mentioned, MRI derived information has been proven valuable in every day clinical decision making and prognosis assessment. Thus, MRI is becoming more and more an accepted alternative to other imaging modalities both in the acute and chronic setting.

Left atrial remodelling early after mitral valve repair for degenerative mitral regurgitation.

OBJECTIVE: Left atrial (LA) size is an important predictor of outcome after mitral valve replacement in patients with symptomatic chronic mitral regurgitation (MR). Data on LA remodelling after mitral valve repair (MVr) for chronic non-ischaemic MR are scarce. The aim of this study was to assess changes in LA size early after MVr for chronic severe degenerative MR and to identify clinical and echocardiographic correlates of those changes. METHODS: The study analysed 225 consecutive patients who underwent MVr and were echocardiographically evaluated in our hospital within 1 month before and 1-6 months after surgery. Patients with MR aetiology other than degenerative, associated aortic valve replacement, or congenital heart disease were excluded. The remaining 79 patients (aged 60 (SD 12) years, 55 men) with isolated chronic severe degenerative MR formed the study group. LA reverse remodelling was defined as a decrease in LA volume index (LAVi) > or = 15%. RESULTS: LA dimensions significantly decreased after MVr (p<0.001). Mean LAVi reduction was 29% (SD 18%). LA reverse remodelling was observed in 63 patients (80%). Correlates of LAVi reduction were preoperative LAVi (p = 0.008), systolic and diastolic blood pressure (p = 0.032, p = 0.009), postoperative transmitral mean pressure gradient (p = 0.001) and residual MR (p = 0.043). LAVi reduction was lower in patients > 45 years (p = 0.008) and in hypertensive patients (p = 0.031). CONCLUSION: LA reverse remodelling is common early after MVr for chronic severe degenerative MR. Preoperative LAVi, blood pressure, postoperative transmitral mean pressure gradient, residual MR and age > 45 are related to LAVi reduction. The prognostic value of LA reduction in this setting needs further study.

Assessment of hypertension levels control and management (hypertension "AUDIT" project). Study in a population of workers.

The hypertension AUDIT project (WHO) was used for the study of large populations of workers in two Romanian industrial centers, Slatina and Sibiu, constituted into two main groups. The objects of the study were: the detection of new cases of arterial hypertension (AH) and of their proportion as compared with older cases, the assessment of the quality of diagnosis control and treatment methodology as well as the estimation of the patient's attitude regarding the conditions of treatment and of the physician's knowledge and attitude regarding AH. Group I (Slatina) included 22,839 workers and the program was applied in 15,740 randomly chosen subjects. Group II (Sibiu) included 14,874 workers of whom 2,838 were randomly chosen for study. From a total of 606 (6.20%) subjects aged 35 to 64 years with AH in both groups, 494 (81.51%) were older cases and 112 (18.48%) were newly detected. The prevalence of AH was found to increase with age and to be higher in women aged 55 to 64 years. The treatment in older cases from both groups was considered effective in 232 cases (46.96%) (of which 26 (11.2%) with overtreatment) and insufficient in 262 (53%). The reasons alleged by the patients for the late detection of AH were the absence of symptoms and a casual interest for their state of health. The risk factors were systematically checked. Smoking was found in 30.36% of the subjects in group I and in 31% of those in group II. The use of diagnostic laboratory procedures was corresponding to the present recommendations. The nonpharmaceutic therapy was frequently recommended, especially reduction of salt consumption. Besides that, pharmaceutic treatment was indicated in most of the patients (63% in group I and 90% in group II). Diuretics (41.26% in group I and 75% in group II), and beta blocking drugs (35.3% in group I and 70% in group II) were the most frequently administered and in a lesser proportion vasodilators with central or peripheral action, calcium blockers, Rauwolfia and angiotensin conversion enzyme inhibitors. The consumer inquiry showed satisfactory compliance of patients, most of them took their tablets regularly, were satisfied with the medical assistance and preferred to consult the same physician. The data of the physician inquiry regarding diagnosis treatment of AH and professional training also proved satisfactory. The use of the AUDIT project has allowed a complex estimation of AH in the groups studied and will prove useful for a more efficient control of AH in the whole population.

[Infectious endocarditis in mitral valve prolapse]. / Endocardita infectioasa pe prolaps de valva mitrala.

The paper reports on 13 cases of infectious endocarditis in the patients with prolapse of the mitral valve admitted for a period of 10 years (1979-1989) into the Clinic of Cardiology of the Fundeni Hospital. These cases stand for 3.6% of the cases with prolapse of the mitral valve admitted during that period, and 5% of the patients with infectious endocarditis. Our study dealt only with the cases of the prolapse of the mitral valve, clinically and echographically documented before the appearance of the septic graft. The hemocultures were positive in all the patients (viridans streptococci in 84.61% cases). The symptomatology, the clinical objective data and the paraclinical results (phonocardiographic, echocardiographic, electrocardiographic, radiologic, investigations with isotopes), the response to the treatment (medical, surgical) and the evolution in time were analyzed. An increase was found during endocarditis in the number of patients with holosystolic murmurs (30.7% cases) versus those with click-telesystolic murmur, the appearance in 41.15% of the cases of valvular vegetations at the Echo examination, and in 15.38% cases of ruptures of cordages. Mitral insufficiency secondary to endocarditis became worse, in 30.76% cases. The treatment with antibiotics resulted in the healing of the infection in all the cases. The surgery was not necessary in any patient during the evolution of endocarditis. The surgery (valvular prosthesis) was made in 23.07% cases, which presented, after curing the septic graft, important mitral regurgitation with cardiac insufficiency refractory to the medical treatment. Prophylaxis of the infectious endocarditis in the prolapse of mitral valve with mitral regurgitation is necessary.

[Arrhythmias in patients with mitral valve prolapse]. / Aritmiile la bolnavii cu prolaps al valvei mitrale.

The object of the present investigation was the discovery of the arrhythmias in the patients with prolapse of the mitral valve (PMV), the types of arrhythmias, the factors favouring them, the therapy used. In a group of 126 patients suffering from PMV, 25 had mitral insufficiency, and 48% of the cases had arrhythmias too. The ventricular arrhythmias existed in 18 patients, in the most of them as ventricular extrasystoles. Only in 2 cases, ventricular paroxysmal tachycardia was noticed, and only one case of ventricular fibrillation was recorded. The following conclusions were drawn from the study: the increase of arrhythmias is low, their appearance is correlated with mitral insufficiency, ventricular arrhythmias are predominant, the majority are benign, they are more frequent than in the patients with neurovegetative dystonia (if considering the prolapse associated with systolic murmur), necessity of periodic control for discovering the cases with high risk of ventricular arrhythmias with malignant potential.

Changes of human platelets studied by scanning electron microscopy in acute myocardial infarction.

The formation of platelet aggregates has been suggested to be the initial step in coronary occlusion and the subsequent myocardial infarction (AMI). By scanning electron microscopy we followed: the platelet morphology and aggregation, the macrophage (M) morphology and the lymphocyte (T, B) count and structure in 11 patients with AMI and in 18 patients with unstable angina (UA). Generally, in the patients with AMI, most of the platelets presented relatively frequent (42.5%) or very frequent pseudopodia (35.27%) and the network of surface extensions was associated with several huge platelet aggregates in 6 patients. The mean lymphocyte count was: T = 40.36 +/- 23.95%; B = 28.09 +/- 7.38%; M = 31.54 +/- 21.25%. In the patients with unstable angina the proportion of platelets with pseudopodia was more reduced, namely, that of platelets with relatively frequent pseudopodia was 33.8% and with very frequent pseudopodia was 27.8%. The mean lymphocyte count was: T = 40.30 +/- 20.24%; B = 34.6 +/- 14.39%; M = 25 +/- 10.50%. These data indicate that platelet changes and the formation of aggregates can be an important factor in the occurrence of AMI. The change of immunocompetent cell count in both groups of coronary patients suggests the association of an immunologic process in coronary atherosclerosis.