The aim of our study was to investigate the reliability of automated oscillometric blood pressure (BP) monitoring in the presence and absence of atrial fibrillation (AF) in hypertensive patients. BP was measured and compared in 71 randomly selected patients with AF and arterial hypertension diagnosis, 4 times each by auscultatory and oscillometric (Microlife BP A6 PC with AF detection system) methods. Study included 71 patients: 36 males (mean age 67.4 years) and 35 females (70.2 years). At the time of BP measuring procedure, 36 patients were in sinus rhythm (SR) and 35 in AF. In SR patients mean systolic blood pressure (SBP) was 132 ± 17.9 mmHg with auscultatory method (AM), 137.4 ± 19.4 mmHg with oscillometric method (OM); mean diastolic BP was 77.1 ± 10.9 mmHg (AM), 78.5 ± 12.2 mmHg (OM), in AF patients mean SBP was 127.5 ± 15.1 mmHg (AM), 133.6 ± 17.4 mmHg (OM); mean diastolic BP was 81.4 ± 9.9 mmHg (AM), 83.5 ± 11.8 mmHg (OM), p = 0.037. The averages of differences for SBP and DBP in sinus rhythm group were (−5.3 mmHg (95% limits of agreement −27.2 – 16.6)) and (−1.4 mmHg (95% limits of agreement −12.8 – 10.0)), respectively. In patients with AF the averages of differences for SBP and DBP were (−6.1 mmHg (95% limits of agreement −23.9 – 11.7)) and (−2.1 mmHg (95% limits of agreement −12.9 – 8.7)), respectively. The oscillometric device validated for patients with AF on average gives 5.3 mmHg higher systolic BP values for patients with SR and 6.3 mmHg higher BP values for patients with AF. However, the limits of agreement between two methods reveal wide range of random error rates which is a questionable topic in clinical practice, as it could possibly affect the treatment of arterial hypertension in patients with AF.

To evaluate a diagnostic value of transoesophageal echocardiogram (TEE) in appropriately anticoagulated patients with a non-valvular atrial fibrillation (AF) and to establish possible additional indications for TEE; to evaluate the incidence of left atrial (LA) thrombi in appropriately anticoagulated patients in daily clinical practice. This retrospective study analyses data of 432 patients who had been anticoagulated by means of oral anticoagulants (OACs) prior to planned cardioversion during the period from 2012 to 2015. Thromboembolic (TE) and bleeding risks were assessed using CHA2DS2-VASc and HAS-BLED scores. Transthoracic and transoesophageal echocardiograms were evaluated. TE complications during 30 days after discharge were assessed. 432 patients were selected, aged from 22 to 89 years (mean 65.0 ±11.5), 277 (64.1%) males and 155 (35.9%) females, 306 (70.8%) on warfarin and 126 (29.2%) on non-vitamin K antagonist oral anticoagulants (NOAC). Mean CHA2DS2-VASc score was 3.5 ±1.5. TEE was performed for 120 (27.8%) patients, more frequently for patients on NOACs and for ones with III° LA enlargement. TEE revealed LA thrombi in seven (5.8%) of the patients. In warfarin and NOACs groups thrombi were revealed in five (7.0%) and two (4.1%) patients, respectively. TEE did not reveal any thrombi in patients with normal left ventricular (LV) function; however, thrombi were found in two (6.1%) patients with slightly decreased LV function, and in five (17.9%) patients with markedly decreased LV function. In patients with decreased left ventricular ejection fraction (LVEF) thrombi in LA were found more frequently than in patients with normal and slightly decreased LVEF (17.9% vs 2.2%, p=0.008). CHA2DS2-VASc score of all 7 patients was ≥5. None of the patients after cardioversion had TE complications 30 days after discharge. The risk of LA thrombi in patients prepared for scheduled cardioversion in line with the guidelines is low. Higher risk of thrombi was present in patients with decreased LVEF (≤40%), CHA2DS2-VASc ≥5. In order to assess more accurately indications to perform TEE for appropriately anticoagulated patients prior to scheduled cardioversion a study with larger number of patients is required.

Background and Objectives: Infective endocarditis in the pulmonary position is a rare disease. Isolated pulmonary valve endocarditis is extremely rare. The aim of our study was to assess patients who were treated surgically for pulmonary endocarditis at our institution from January 2003 to December 2017. Materials and Methods: We analyze eight cases of infectious endocarditis in pulmonary position out of 293 patients who were operated for infective endocarditis (2.7%, 8/293). Only two of these eight patients were not related to congenital heart malformation. They were followed for early and late mortality, long-term survival, postoperative morbidity and reoperations. Results: Among six patients suffering from congenital heart disease, four patients underwent corrections of pulmonary valve malformation previously, and their infected grafts were replaced by two allografts and two xenografts. The two other patients had replaced their infected pulmonary valves with allografts. Two non-congenital patients with pulmonary valve endocarditis underwent valve replacement with biological prosthesis. All patients survived the early postoperative course. The mean follow-up time was 9.1 (interquartile range (IQR), 5.3–12.6) years. The long-term follow-up included seven patients. One patient (12.5%, 1/8) died more than 4 years after the surgery due to sepsis. Pulmonary endocarditis was the rarest endocarditis treated surgically (p < 0.001). Conclusion: Surgery for infective endocarditis in the pulmonary position (IEPP) is an effective method of treatment with excellent early outcome and good late results despite a very uncommon pathology and few operations being performed. Surgery performed earlier may make the procedure less radical.

Atrial fibrillation (AF) is the most common supraventricular arrhythmia, affecting 2–3% of the adult population, with an increasing prevalence due to demographic shifts; however, detection methods have also improved. This rhythm disorder is associated with significant morbidity, manifesting through symptoms that worsen the quality of life, as well as with adverse outcomes and increased mortality. The substantial AF burden on the healthcare system necessitates the development of effective and durable treatment strategies. While pharmacological management represents the first-line approach for AF, the limitations associated with this approach, including side effects and insufficient efficacy, have promoted the evolution of catheter ablation techniques that isolate pulmonary veins (PVs) and, thus, disrupt arrhythmia-causing impulses from the atria. Currently, three energy sources have gained U.S. Food and Drug Administration (FDA) and European regulatory approval (The Conformité Européene (CE) mark certification) for catheter ablation: radiofrequency ablation (RFA), cryoballoon ablation (CBA), and, more recently, pulsed-field ablation (PFA). RFA has subsequently become an effective treatment, demonstrating superior outcomes in randomized controlled trials compared to antiarrhythmic drug therapy. CBA has also proven to be a safe and effective alternative, particularly for patients with symptomatic paroxysmal AF, showing comparable efficacy to RFA and similar rates of complications. Meanwhile, PFA is emerging as a promising technique, offering non-inferior efficacy to conventional thermal methods while potentially minimizing the thermal damage to adjacent tissues associated with RFA and CBA. Despite higher equipment costs, the advantages of PFA in reducing complications highlight its potential role in AF management. However, considering the novelty of PFA, no data currently exist comparing this strategy with thermal techniques. Therefore, further research is needed to improve the management of AF and patient outcomes to reduce healthcare burdens.

Background: Fabry disease (FD) is a rare X-linked inherited lysosomal storage disorder caused by α-galactosidase A deficiency leading to intracellular glycosphingolipid accumulation. FD manifestation is multisystem, and can differ depending on disease-related genetic variants. Currently, more than 700 different FD-causing mutations have been identified in the human GLA gene. We identified a novel mutation in a Lithuanian family with classical manifestations of Fabry disease, revealing severe effects to the cardiovascular systems of heterozygous women. Case presentation: A 49-year-old woman underwent echocardiography due to progressive dyspnea that lasted seven years, reduced physical activity, and periodic cardiac arrhythmia. Echocardiography revealed left ventricular hypertrophy with normal diastolic function. The patient had experienced acroparesthesia in her upper limbs and abdominal pain since childhood, and in the last decade had experienced mild proteinuria without renal failure. Her renal biopsy was typical for Fabry disease. The patient’s brain magnetic resonance imaging (MRI) (T2 flair) showed white matter hyperintensities lesions. DNA sequencing of the proband, her mother and one of her sons showed a novel GLA gene exon 2 mutation, c.270C>G (p.Cys90Trp). All three patients had decreased α-galactosidase A activity and specific FD manifestations. Conclusions: A novel GLA mutation, c.270C>G (p.Cys90Trp), was found in a Lithuanian family with a classical form of Fabry disease in heterozygous women with predominant cardiac involvement. However, the exact manifestation of this mutation is still unclear as it is newly reported and further research must be done.

Background and Objectives: Hereditary transthyretin amyloidosis (ATTRv) is a rare disease caused by pathogenic variants in the transthyretin (TTR) gene. More than 140 different disease-causing variants in TTR have been reported. Only a few individuals with a rare TTR variant, c.302C>T, p.(Ala101Val) (historically known as p.(Ala81Val)), primarily associated with cardiac ATTRv, have been described. Therefore, our aim was to analyze the clinical characteristics of individuals with the identified c.302C>T TTR variant at our center. Materials and Methods: We analyzed data from individuals with ATTRv who were diagnosed and treated at Vilnius University Hospital Santaros Klinikos. ATTRv was confirmed by negative hematological analysis for monoclonal protein, positive tissue biopsy or bone scintigraphy and a pathogenic TTR variant. Results: During 2018-2021, the TTR NM_000371.3:c.302C>T, NP_000362.1:p.(Ala101Val) variant was found in one individual in a homozygous state and in three individuals in a heterozygous state. The age of onset of symptoms ranged from 44 to 74 years. The earliest onset of symptoms was in the individual with the homozygous variant. A history of carpal tunnel syndrome was identified in two individuals. On ECG, three individuals had low QRS voltage in limb leads. All individuals had elevated NT-proBNP and hsTroponine I levels on baseline laboratory tests and concentric left ventricular hypertrophy on transthoracic echocardiography. The individual with the homozygous c.302C>T TTR variant had the most pronounced polyneuropathy with tetraparesis. Other patients with the heterozygous variant had more significant amyloid cardiomyopathy. When screening family members, the c.302C>T TTR variant was identified in two phenotypically negative relatives at the ages of 33 and 47 years. Conclusions: c.302C>T is a rare TTR variant associated with ATTRv cardiomyopathy. The homozygous state of this variant was not reported before, and is associated with earlier disease onset and neurological involvement compared to the heterozygote state.