Clinical Presentation and Percutaneous Treatment of Hemolytic Anemia Associated With Paravalvular Leak After TAVR.

Paravalvular leak (PVL) following transcatheter aortic valve replacement (TAVR) is an established complication, albeit rarely associated with hemolytic anemia. This report details 3 cases of significant hemolytic anemia attributed to TAVR-induced PVL, each with distinct clinical presentations and manifestations. These cases underscore the diverse and occasionally subtle clinical presentation of aortic PVL-associated hemolytic anemia.

Severe influenza pneumonitis in children with inherited TLR3 deficiency.

Autosomal recessive IRF7 and IRF9 deficiencies impair type I and III IFN immunity and underlie severe influenza pneumonitis. We report three unrelated children with influenza A virus (IAV) infection manifesting as acute respiratory distress syndrome (IAV-ARDS), heterozygous for rare variants (P554S in two patients and P680L in the third) causing autosomal dominant (AD) TLR3 deficiency. AD TLR3 deficiency can underlie herpes simplex virus-1 (HSV-1) encephalitis (HSE) by impairing cortical neuron-intrinsic type I IFN immunity to HSV-1.

Cardiac Paraganglioma Arising From the Right Atrioventricular Groove in a Paraganglioma-Pheochromocytoma Family Syndrome With Evidence of SDHB Gene Mutation: An Unusual Presentation.

Primary cardiac paragangliomas are extremely rare. Recently this neoplasm has been associated with a familiar syndrome as a result of mutation of genes that encode proteins in the mitochondrial complex II. We report a case of a 46-year-old woman having cases of vertebral paraganglioma in her family showing an unusual anatomic and clinical presentation of cardiac paraganglioma and expressing a genetic mutation never associated before with cardiac localization of this neoplasm.

Ventricular outputs, central blood flow distribution and flow pattern through the aortic isthmus of fetuses with simple transposition of the great arteries.

Introduction: Our objective was to determine the impact of simple transposition of the great arteries (TGA) on fetal left ventricular (LV) and right ventricular (RV) performances and central circulatory dynamics including the aortic isthmus.

Material And Methods: Ventricular stroke volumes were calculated as the product of the cross-sectional area of the corresponding semi-lunar valve and the flow velocity integral through these valves. Volume flow in ductus arteriosus (QDA ) was evaluated using the same technique.

Noninvasive electrocardiomapping facilitates previously failed ablation of right appendage diverticulum associated life-threatening accessory pathway.

Combination of structural (CT-scan) and functional (3D electrocardiomapping) imaging methods helped successfully accomplish ablation of a life-threatening manifest accessory pathway in association with a complex right atrial anomaly after previous unsuccessful attempts of endo-epicardial ablation guided by the invasive electroanatomic system in an adolescent female. Such a system has a potential to facilitate the ablation procedure and impact its outcome through accurate localization of the arrhythmogenic substrate.

Phase I of cardiac rehabilitation: A new challenge for evidence based physiotherapy.

Cardiac rehabilitation protocols applied during the in-hospital phase (phase I) are subjective and their results are contested when evaluated considering what should be the three basic principles of exercise prescription: specificity, overload and reversibility. In this review, we focus on the problems associated with the models of exercise prescription applied at this early stage in-hospital and adopted today, especially the lack of clinical studies demonstrating its effectiveness. Moreover, we present the concept of "periodization" as a useful tool in the search for better results.

Permanent junctional reciprocating tachycardia in children: a multicentre study on clinical profile and outcome.

Objectives: To investigate the clinical profile, natural history, and optimal management of persistent or permanent junctional reciprocating tachycardia (PJRT) in children.

Methods And Results: 85 patients meeting the ECG criteria for PJRT were enrolled in a retrospective multicentre study. Age at diagnosis varied from birth to 20 years (median 3 months).

[Aortic valve papillary fibroelastoma; report of a case].

Papillary fibroelastoma is a rare cardiac tumor. We report a case of surgical treatment for aortic valve papillary fibroelastoma. The patient was a 64-year-old female.

Extraction of epileptogenic foci from PET and SPECT images by fuzzy modeling and data fusion.

During the presurgical evaluation of medically intractable epilepsy, isotopic functional imagery provides an increasing amount of data concerning the potential location of the focus. The aim of this study is to facilitate the surgical decision by presenting an image fusion method able to extract epileptogenic foci from periictal single photon emission computed tomography (SPECT), interictal SPECT, fluoro-desoxy-glucose (FDG) position emission tomography (PET), and flumazenil PET. After spatial coregistration, the images are converted into fuzzy maps whose membership functions indicate the pathological degree of each voxel, according to each modality.

Automated detection of local normalization areas for ictal-interictal subtraction brain SPECT.

Unlabelled: Whole-brain activity is often chosen to quantitatively normalize peri-ictal and interictal SPECT scans before their subtraction. This use is not justified, because significant and extended modification of the cerebral blood flow can occur during a seizure. We validated and compared 2 automatic methods able to determine the optimal reference region, using simulation and clinical data.

Large experience with a factor VIII binding assay of plasma von Willebrand factor using commercial reagents.

The present diagnostic assay for type 2N von Willebrand disease (VWD) is based on the in vitro measurement of the capacity of plasma von Willebrand factor (VWF) to bind exogeneous factor VIII (VWF:FVIIIB). We report a method using only commercially available reagents that is easy to perform. This method has been validated in a cohort of 144 patients with FVIII/VWF ratios < 0.

The short- and mid-term results of bidirectional cavopulmonary shunt with additional source of pulmonary blood flow as definitive palliation for the functional single ventricular heart.

Objective: The purpose of this study was to demonstrate the early and late outcomes of bidirectional cavopulmonary shunt (BCPS) as a definitive procedure for the functional single ventricular heart.

Method: From September 1991 to December 1997, 34 patients underwent a BCPS procedure without a routine conversion to Fontan circulation. The additional source of pulmonary blood flow was left in all patients.

Theoretical model for myocardial functional characterization: application to a group of patients evaluated before and after surgical revascularization.

Background: The functional improvements resulting from coronary revascularization (CABG) in patients with depressed ventricular function may be described by the use of a model combining global or local quantification of myocardial perfusion, viability, and contraction. An illustration of this model, with data provided by conventional radionuclide studies as they are performed routinely in many centers, is presented and the limitations of this approach for predicting the results of CABG are discussed.

Methods And Results: The model is based on three independent variables, which can be approximated in this preliminary study by parameters derived from standard stress and redistribution/reinjection thallium-201 single-photon emission computed tomography (SPECT) acquisitions with quantification of the tracer uptake defects and from a planar gated blood pool left ventricular ejection fraction (LVEF) measurement: Perfusion is assumed to correspond to 100-stress defect (in percentage), viability is 100-redistribution/reinjection defect, and contraction is 100(LVEF/70), assuming that a normal 70% LVEF corresponds to 100% contraction.

A prospective study of helical computed tomography angiography versus angiography for the detection of renal artery stenoses in hypertensive patients.

Objective: To compare helical computed tomography angiography with arterial digital subtraction angiography in the diagnosis of renal artery stenoses.

Methods: Fifty hypertensives (24 men; mean age 53 years) were prospectively studied with computed tomography (Somaton Plus S, Siemens) and digital angiography (double-blind evaluation). Computed tomography was performed both in the sequential (the length of the abdomen) and in the helical (6 cm around renal arteries) modes during injection of 120 cm3 contrast medium.

Discordant results of visual and quantitative estimates of stenosis severity before and after coronary angioplasty.

The ability to accurately estimate the severity of epicardial coronary stenoses is critical in the assessment of the immediate and long-term results of percutaneous transluminal coronary angioplasty (PTCA). We prospectively compared visual estimates, performed by experienced interventional cardiologists, with computerized quantitative angiographic measurements of stenosis severity in a group of patients (n = 305) before, immediately after and 6 months after PTCA. Before PTCA the visual estimate of the mean (+/- SD) percentage stenosis severity, 80.

The benefit of graded physical exercise in chronic heart failure.

A new program of rehabilitation is less demanding on cardiac output than standard programs. Twenty-five patients with chronic heart failure (ejection fraction [EF]: 0.26 +/- 0.