Schwannomy a paragangliomy jsou příkladem neurogenních tumorů, které jsou v případě povrchového uložení dostupné pro vyšetření ultrazvukem. Ultrazvukový obraz může být rozmanitý vzhledem k variabilní echogenitě i vaskularizaci těchto lézí. V některých případech, zejména při multicentrickém výskytu nebo při nemožnosti zobrazit návaznost na nerv, mohou být zaměněny za patologické uzliny. Kazuistická sdělení předkládají ultrazvukový obraz těchto tumorů včetně obrazu kontrastního vyšetření s použitím mikrobublinové kontrastní látky SonoVue (Bracco Imaging S.p.A, Itálie) u třech pacientů, kteří byli došetřováni pro hmatnou rezistenci v oblasti krku a axily. U všech pacientů byla provedena diagnostická exstirpace přítomných ložisek. Ložiska byla histologicky vyhodnocena jako schwannom krku a axily a paragangliom krku.

Schwannomas and paragangliomas, when localized superficially, are examples of tumors that are well accessible with sonography. Their appearance on ultrasound can be diverse due to variable echogenicity and vascularization. In some cases, especially in the event of multicentricity or an inability to demostrate contiguity with a nerve of origin, they can be mistaken for pathologic lymph nodes. The appearance of these tumors on conventional ultrasound and on contrast-enhanced ultrasound using the microbubble contrast agent SonoVue (Bracco Imaging, Italy) is presented in case reports of three patients undergoing investigation of palpable masses in the neck or axillary region. All patient underwent excisional biopsy of the lesions, which were histologically determined to be schwannoma of the neck and axila, and paraganglioma of the neck.

Clinical outcomes of catheter ablation for atrial fibrillation (AF) are suboptimal due, in part, to challenges in achieving durable lesions. Although focal point-by-point ablation allows for the creation of any required lesion set, this strategy necessitates the generation of contiguous lesions without gaps. A large-tip catheter, capable of creating wide-footprint ablation lesions, may increase ablation effectiveness and efficiency. In a randomized, single-blind, non-inferiority trial, 420 patients with persistent AF underwent ablation using a large-tip catheter with dual pulsed field and radiofrequency energies versus ablation using a conventional radiofrequency ablation system. The primary composite effectiveness endpoint was evaluated through 1 year and included freedom from acute procedural failure and repeat ablation at any time, plus arrhythmia recurrence, drug initiation or escalation or cardioversion after a 3-month blanking period. The primary safety endpoint was freedom from a composite of serious procedure-related or device-related adverse events. The primary effectiveness endpoint was observed for 73.8% and 65.8% of patients in the investigational and control arms, respectively (P < 0.0001 for non-inferiority). Major procedural or device-related complications occurred in three patients in the investigational arm and in two patients in the control arm (P < 0.0001 for non-inferiority). In a secondary analysis, procedural times were shorter in the investigational arm as compared to the control arm (P < 0.0001). These results demonstrate non-inferior safety and effectiveness of the dual-energy catheter for the treatment of persistent AF. Future large-scale studies are needed to gather real-world evidence on the impact of the focal dual-energy lattice catheter on the broader population of patients with AF. ClinicalTrials.gov identifier: NCT05120193 .

• MeSH
• Atrial Fibrillation * surgery   therapy   MeSH
• Single-Blind Method MeSH
• Catheter Ablation * methods   MeSH
• Middle Aged MeSH
• Humans MeSH
• Recurrence MeSH
• Aged MeSH
• Treatment Outcome MeSH
• Check Tag
• Middle Aged MeSH
• Humans MeSH
• Male MeSH
• Aged MeSH
• Female MeSH
• Publication type
• Journal Article MeSH
• Randomized Controlled Trial MeSH

Ultra-low temperature cryoablation (ULTC) using near-critical nitrogen (-196oC) has been shown to produce durable, contiguous, transmural lesions in ventricles of animal models. This report summarizes acute experience with ULTC in the first-ever 13 patients with recurrent monomorphic ventricular tachycardias (VTs) of both ischemic cardiomyopathy and nonischemic etiologies enrolled in the CryoCure-VT (Cryoablation for Monomorphic Ventricular Tachycardia; NCT04893317) clinical trial. After an average of 9.6 ± 4.6 endocardial ULTC lesions per patient, no clinical ventricular tachycardias were inducible in 91% of patients. Two procedure-related serious adverse events recorded in 2 patients resolved post-procedurally without clinical sequelae. Further investigation of both acute and chronic outcomes is warranted and ongoing.

• MeSH
• Endocardium MeSH
• Tachycardia, Ventricular * MeSH
• Cryosurgery * adverse effects   MeSH
• Humans MeSH
• Heart Ventricles MeSH
• Temperature MeSH
• Animals MeSH
• Check Tag
• Humans MeSH
• Animals MeSH
• Publication type
• Journal Article MeSH
• Research Support, Non-U.S. Gov't MeSH
• Review MeSH

PURPOSE: Durable pulmonary vein (PV) isolation (PVI) determines the clinical success of catheter ablation for atrial fibrillation. In this randomized study, we investigated whether the temporally discontiguous deployment of ablation lesions adversely affected the acute efficacy of PVI. METHODS: Thirty-six consecutive patients with drug-refractory paroxysmal atrial fibrillation (aged 59 ± 11, 58% males) were randomized 1:1 to either discontiguous (D-PVI) or contiguous (C-PVI) encircling radiofrequency (RF) lesions around ipsilateral PVs. A contact force-sensing catheter was used targeting a final interlesion distance < 6 mm and the ablation index of 400-450 (anterior wall) and 300-350 (posterior wall). The study endpoint was defined as failure of first-pass PVI or acute PV reconnection during a waiting time (> 30 min) followed by adenosine challenge. RESULTS: The total RF time, number of RF lesions, and mean interlesion distance were comparable in both groups. Total endpoint rates were 1/36 (3%) in the D-PVI vs 4/36 (11%) in the C-PVI groups; P = 0.34 for superiority, P = 0.008 for non-inferiority. Adenosine-induced reconnection of right PVs was the only endpoint in the D-PVI group. In the C-PVI group, first-pass PVI failed in 2 right PVs and spontaneous reconnection occurred in 2 other circles (left and right PVs). CONCLUSION: Temporally discontiguous deployment of RF lesions is not associated with lower procedural PVI efficacy when strict criteria for interlesion distance and ablation index are applied. The development of local edema around each ablation site does not prevent effective RF lesion formation at adjacent positions. TRIAL REGISTRATION: clinicaltrials.gov (NCT03332862).

• MeSH
• Adenosine MeSH
• Atrial Fibrillation * surgery   MeSH
• Catheter Ablation * MeSH
• Humans MeSH
• Recurrence MeSH
• Pulmonary Veins * surgery   MeSH
• Treatment Outcome MeSH
• Check Tag
• Humans MeSH
• Male MeSH
• Female MeSH
• Publication type
• Journal Article MeSH
• Randomized Controlled Trial MeSH

Atrial fibrillation is the most common sustained cardiac arrhythmia and is associated with considerable morbidity and mortality. Electrically isolating the pulmonary veins from the left atrium by catheter ablation is superior to antiarrhythmic drug therapy for maintaining sinus rhythm, but its success varies depending on multiple factors, including arrhythmic burden. Although procedural outcomes have improved over the years, further gains are limited by a seemingly zero-sum relationship between effectiveness and safety, which is largely a product of the available technologies. Current energies used to create contiguous, transmural, and durable atrial lesions can result in serious complications if they reach the esophagus or phrenic nerve, for instance-structures that can be adjacent to the atrial myocardium, often within millimeters of the energy source. Consequently, high rates of pulmonary vein-left atrium reconnections are consistently seen in clinical studies and in clinical practice as operators appropriately forgo ablation effectiveness to protect patients from harm. However, as ablative technologies evolve to circumvent this stalemate, safer, and more effective pulmonary vein isolation seems increasingly realistic. Furthermore, the innovative nature of these technologies raises the prospect of markedly improved procedural efficiency, which could increase patient comfort, reduce operator occupational injuries, and enhance the use of health resources-all of which are increasingly important considerations particularly as the demand for catheter ablation for atrial fibrillation continues to rise. We herein review 3 promising candidate ablation technologies with the potential to revolutionize the management of patients with atrial fibrillation: electroporation (pulsed-field ablation), expandable lattice-tip radiofrequency ablation/electroporation, and ultra-low temperature cryoablation.

• MeSH
• Atrial Fibrillation surgery   MeSH
• Catheter Ablation methods   MeSH
• Humans MeSH
• Pulmonary Veins surgery   MeSH
• Animals MeSH
• Check Tag
• Humans MeSH
• Animals MeSH
• Publication type
• Journal Article MeSH
• Research Support, Non-U.S. Gov't MeSH
• Review MeSH

OBJECTIVES: This study was designed to evaluate lesion durability on invasive electrophysiologic remapping. BACKGROUND: The lattice-tip catheter generates a large thermal footprint during temperature-controlled irrigated radiofrequency ablation. In a first-in-human study, this catheter performed rapid point-by-point pulmonary vein isolation (PVI) and other linear atrial ablations. METHODS: In a prospective 3-center single-arm study, paroxysmal or persistent atrial fibrillation patients underwent PVI and, as needed, linear ablation at the cavotricuspid isthmus (CTI), mitral isthmus (MI), and/or left atrial roof; no other atrial substrate was ablated. Using the lattice catheter and a custom electroanatomic mapping system, temperature-controlled (Tmax 73° to 80°C; 2 to 7 s) point-by-point ablation was performed. Patients were followed for 12 months. RESULTS: A total of 65 patients (61.5% paroxysmal/38.5% persistent) underwent ablation: PVI in 65, MI in 22, left atrial roof in 24, and CTI in 48 patients. At a median of 108 days after the index procedure, protocol-mandated remapping was performed in 27 patients. The pulmonary veins (PVs) remained durably isolated in all but 1 reconnected PV-translating to durable isolation in 99.1% of PVs, or 96.3% of patients with all PVs isolated. Of 47 linear atrial lesions initially placed during the index procedure, durability was observed in 10 of 11 (90.9%) MI lines, all 11 (100%) roof lines, and all 25 (100%) CTI lines. After a median follow-up of 270 days, the 12-month Kaplan-Meier estimate for freedom from atrial arrhythmias was 94.4 ± 3.2%. CONCLUSIONS: Temperature-controlled lattice-tip point-by-point ablation showed not only highly durable PVI lesion sets, but also durable contiguity of linear atrial lesions.

• MeSH
• Catheter Ablation * MeSH
• Catheters MeSH
• Humans MeSH
• Prospective Studies MeSH
• Temperature MeSH
• Pulmonary Veins * surgery   MeSH
• Check Tag
• Humans MeSH
• Publication type
• Journal Article MeSH
• Research Support, Non-U.S. Gov't MeSH

Tuberózní skleróza je onemocnění s autosomálně dominantním typem dědičnosti, pro které je charakteristický vývoj benigních tumorů v mnoha tkáních a orgánech. Klinické příznaky jsou extrémně variabilní. Příčinou jsou mutace v genu TSC1 nebo genu TSC2. Komplex vytvořený produkty těchto TSC genů reguluje buněčný růst a proliferaci inhibicí mTORC1 signalizace. Včasně stanovená diagnóza TSC je velmi důležitá pro naplánování příslušné perinatální péče. Použitím ultrazvuku a případně MRI je možné v prenatálním období zachytit následující majoritní znaky tuberózní sklerózy: srdeční rhabdomyomy, subependymální noduly, kortikální tubery a renální angiomyolipomy. V souvislosti se syndromem přilehlých genů TSC2/PKD1 lze u plodu detekovat i renální cysty. Často tyto TSC asociované léze představují náhodný nález během rutinního ultrazvuku. V období od 20. týdne gravidity je nejčastějším markerem vyvolávajícím podezření na tuberózní sklerózu srdeční rhabdomyom(y). V případě, kdy jeden z rodičů je nositelem již identifikované mutace v TSC genu, je možné provést cílené genetické testování vzorku DNA izolovaného z buněk choriových klků, amniocytů, případně z tkáně potracených plodů. Výrazně časově náročnější je provedení molekulární analýzy TSC genů u plodů se suspektní tuberózní sklerózou bez výskytu onemocnění v rodině. Po nálezu příčinné mutace a její konfirmace je možné nabídnout genetické testování dalších osob v riziku, prenatální (eventuálně preimplantační) diagnostiku pro další těhotenství. Rovněž je nutné zvážit možnost výskytu gonadálního mozaicismu.

Tuberous sclerosis is a disease with an autosomal dominant pattern of inheritance which is characterized by the development of benign tumours in many tissues and organs. Clinical signs are extremely variable, causing mutations in the gene TSC1 or TSC2. Complex formed by the products of the TSC genes regulates cell growth and proliferation by inhibition of mTORC1 signalling. Early diagnosis of TSC is very important to plan appropriate perinatal care. Using ultrasound and eventually MRI it is possible in the prenatal period to capture the following major features of tuberous sclerosis: cardiac rhabdomyo­ma, subependymal nodules, cortical tubers and renal angiomyolipomas. In connection with the syndrome of contiguous genes TSC2 / PKD1 can also be detect foetal renal cysts. Often these TSC-associated lesions represent an incidental finding during a routine ultrasound. In the period from the 20th week of pregnancy it is most often found cardiac rhabdomyoma/s as the first marker suggestive of tuberous sclerosis. In the case, where one of the parents is a carrier of already identified mutation in the TSC gene, it is possible to carry out targeted genetic testing of a sample of DNA isolated from cells of chorionic villi, amniocytes or tissue from aborted foe­tuses. Significantly more time consuming is to perform molecular analysis of the TSC genes in foetuses with suspected tuberous sclerosis without the occurrence of illness in the family. After finding a causal mutation and its confirmation, it is possible to offer genetic testing for other persons at risk, prenatal (eventually preimplantation) diagnosis for future pregnancies. It is also necessary to consider the possibility of gonadal mosaicism.

• MeSH
• Point Mutation genetics   MeSH
• Genetic Testing MeSH
• Pregnancy Complications MeSH
• Humans MeSH
• Mosaicism MeSH
• Prenatal Diagnosis * methods   MeSH
• Prenatal Care MeSH
• Sequence Analysis, DNA MeSH
• Practice Guidelines as Topic MeSH
• Pregnancy MeSH
• Tuberous Sclerosis * diagnosis   genetics   MeSH
• Check Tag
• Humans MeSH
• Pregnancy MeSH
• Female MeSH
• Publication type
• Review MeSH

AIMS: A challenge of pulmonary vein isolation (PVI) in catheter ablation for paroxysmal atrial fibrillation (PAF) is electrical reconnection of the PV. EFFICAS I showed correlation between contact force (CF) parameters and PV durable isolation but no prospective evaluation was made. EFFICAS II was a multicentre study to prospectively assess the impact of CF guidance for an effective reduction of PVI gaps. METHODS AND RESULTS: Pulmonary vein isolation using a radiofrequency (RF) ablation catheter with an integrated force sensor (TactiCathTM) was performed in patients with PAF. Operators were provided EFFICAS I-based CF guidelines [target 20 g, range 10-30 g, minimum 400 g s force-time integral (FTI)]. Conduction gaps were assessed by remapping of PVs after 3 months, and gap rate was compared with EFFICAS I outcome. At follow up, 24 patients had 85% of PVs remaining isolated, compared with 72% in EFFICAS I (P = 0.037) in which CF guidelines were not used. The remaining 15% of gaps correlated to the number of catheter moves at creating the PVI line, quantified as Continuity Index. For PV lines with contiguous lesions and low catheter moves, durable isolation was 81% in EFFICAS I and 98% in EFFICAS II (P = 0.005). At index procedure, the number of lesions was reduced by 15% in EFFICAS II vs. EFFICAS I. CONCLUSION: The use of CF with the above guidelines and contiguous deployment of RF lesions in EFFICAS II study resulted in more durable PVI in catheter ablation of PAF.

• MeSH
• Equipment Failure Analysis MeSH
• Surgery, Computer-Assisted instrumentation   methods   MeSH
• Equipment Design MeSH
• Adult MeSH
• Atrial Fibrillation diagnosis   surgery   MeSH
• Catheter Ablation instrumentation   methods   MeSH
• Humans MeSH
• Body Surface Potential Mapping instrumentation   methods   MeSH
• Stress, Mechanical MeSH
• Transducers, Pressure MeSH
• Heart Conduction System surgery   MeSH
• Pulmonary Veins surgery   MeSH
• Treatment Outcome MeSH
• Check Tag
• Adult MeSH
• Humans MeSH
• Male MeSH
• Female MeSH
• Publication type
• Journal Article MeSH
• Controlled Clinical Trial MeSH
• Multicenter Study MeSH
• Research Support, Non-U.S. Gov't MeSH
• Geographicals
• Europe MeSH

In this case report we describe the case of a 24 year-old female with a fulminant demyelinating disease of white matter. Disease progression was most probably consistent with the Marburg variant (malignant form) of multiple sclerosis with rapid deterioration of the patient's clinical condition, including bulbar symptoms and epileptic paroxysms and ending with persistent coma and tetraparesis, over the course of 6 months from first symptoms. Repeated Magnetic Resonance Imaging (MRI) examination showed progression of multiple demyelinating lesions culminating in a contiguous focal disorder of the white matter extending both supratentorially and infratentorially. The serial MRI changes closely mapped the deterioration in the patients clinical status. Our patient showed no response to repeated pulse corticotherapy, administration of intravenous immunoglobulins, serial plasmapheresis, and combined high-dose pulse immunosuppression (specify what was used here) and mitoxantrone.

• MeSH
• Acute Disease MeSH
• Demyelinating Diseases pathology   physiopathology   therapy   MeSH
• Humans MeSH
• Magnetic Resonance Imaging MeSH
• Young Adult MeSH
• Treatment Failure MeSH
• Multiple Sclerosis pathology   physiopathology   therapy   MeSH
• Severity of Illness Index MeSH
• Check Tag
• Humans MeSH
• Young Adult MeSH
• Female MeSH
• Publication type
• Journal Article MeSH
• Case Reports MeSH

We present six cases (five females, one male; aged 26-81 years) of dacryops, also known as lacrimal gland cyst, all of which occurred at the outer canthal area below the upper eyelid. All presented clinically as a painless cystic lesion that was white to blue in color. Microscopically, in addition to typical features of dacryops, which is characterized by a partially cystic proliferation that includes a double layer of columnar to cuboidal epithelial cells associated with lobules of lacrimal gland tissue, we identified evidence of apocrine secretion (i.e. apical snouts projecting into the lumen), either in the cystic component of the proliferation or in contiguous lacrimal duct, in all cases. One example was unusual. It manifested, in addition to typical cyst formation, with areas of ductal and probably acinar hyperplasia. We conclude that apocrine secretion in dacryops is a common and underrecognized phenomenon. Dacryops should be distinguished from apocrine hidrocystoma, a lesion commonly encountered in the periorbital area in the practice of dermatopathology.

• MeSH
• Cysts pathology   secretion   MeSH
• Adult MeSH
• Mucus secretion   MeSH
• Middle Aged MeSH
• Humans MeSH
• Lacrimal Apparatus Diseases metabolism   pathology   MeSH
• Goblet Cells pathology   secretion   MeSH
• Aged, 80 and over MeSH
• Aged MeSH
• Lacrimal Apparatus pathology   secretion   MeSH
• Check Tag
• Adult MeSH
• Middle Aged MeSH
• Humans MeSH
• Male MeSH
• Aged, 80 and over MeSH
• Aged MeSH
• Female MeSH
• Publication type
• Journal Article MeSH
• Case Reports MeSH