Prognostic Value of Early Risk Stratification in Pediatric Pulmonary Arterial Hypertension.

Background: Pulmonary arterial hypertension (PAH) is a life-threatening disease with risk stratification-based treatment strategy in adults. Although the risk factors have been studied individually in children, effective risk stratification is still lacking. We have tested the prognostic accuracy of pediatric PAH risk factors in our patient group.

Differences in characteristics, treatments and outcomes in patients with non-ST-elevation myocardial infarction: novel insights from four national European continuous real-world registries.

Aims: To study baseline characteristics, in-hospital managements and mortality of non-ST-elevation myocardial infarction (NSTEMI) patients in different European countries.

Methods And Results: NSTEMI patients enrolled in the national myocardial infarction (MI) registries [EMIR; n = 5817 (Estonia), HUMIR; n = 30 787 (Hungary), NORMI; n = 33 054 (Norway), and SWEDEHEART; n = 49 533 (Sweden)] from 2014 to 2017 were included and presented as aggregated data. The median age at admission ranged from 70 to 75 years.

Impaired cytoplasmic domain interactions cause co-assembly defect and loss of function in the p.Glu293Lys KNCJ2 variant isolated from an Andersen-Tawil syndrome patient.

Aims: Subunit interactions at the cytoplasmic domain interface (CD-I) have recently been shown to control gating in inward rectifier potassium channels. Here we report the novel KCNJ2 variant p.Glu293Lys that has been found in a patient with Andersen-Tawil syndrome type 1 (ATS1), causing amino acid substitution at the CD-I of the inward rectifier potassium channel subunit Kir2.

A Summary of Transplantation Activity in Hungary.

The first kidney transplantation was performed in Hungary by András Németh in 1962. It was a living donor procedure. After many years of silence, organized cadaveric programs were established in Budapest (1973), Szeged (1979), Debrecen (1991), and Pécs (1993).

Pregnant intravenous drug user tricuspid valve infective endocarditis treated with a successful simultaneous valve replacement and Cesarean section.

A 30-year-old female patient known to be an intravenous drug user (IVDU) was admitted to Bajcsy-Zsilinszky Hospital Cardiology Intensive Care Unit at 29-week gestation with severe sepsis and right heart failure. She had methicillin-sensitive Staphylococcus aureus on blood culture. Echocardiography confirmed the diagnosis of tricuspid valve infective endocarditis (IE).

Use of subcutaneous treprostinil in pediatric pulmonary arterial hypertension-Bridge-to-transplant or long-term treatment?

PAH is a progressive life-threatening disease in children. While parenteral prostacyclin therapy improves survival in patients with severe PAH, central line-related complications are common. Our aim was to assess the efficacy, safety, and tolerability of subcutaneous treprostinil treatment in pediatric PAH patients.

Are vital exhaustion and depression independent risk factors for cardiovascular disease morbidity?

Objective: The aim of this study was to examine the concurrent effects of vital exhaustion and depression on the development of cardiovascular disease (CVD) morbidity.

Method: The sample of this representative, 4-year longitudinal study comprised 2,725 participants (43.56% male, Mage = 58.

Timothy syndrome 1 genotype without syndactyly and major extracardiac manifestations.

Timothy syndrome 1 (TS1) is a rare genetic disorder characterized by multisystem abnormalities including QT prolongation, congenital heart defects, facial dysmorphism, episodic hypoglycemia, and neurological symptoms. A morphological hallmark of TS1 is syndactyly, present in all cases. TS1 is caused by the canonical p.

Long-term cerebral thromboembolic complications of transapical endocardial resynchronization therapy.

Purpose: Cardiac resynchronization therapy (CRT) is an established therapeutic option in selected heart failure patients (pts). However, the transvenous left ventricular (LV) lead implantation remains ineffectual in a considerable number of pts. Transapical LV (TALV) lead implantation is an alternative minimally invasive, surgical, endocardial implantation technique.

Two-Year Follow-Up after Contact Force Sensing Radiofrequency Catheter and Second-Generation Cryoballoon Ablation for Paroxysmal Atrial Fibrillation: A Comparative Single Centre Study.

Background. There are little comparative data on catheter ablation of paroxysmal atrial fibrillation (AF) using the contact force radiofrequency (CF-RF) catheter versus the second-generation cryoballoon (CB2). Methods and results.

EMBRACE STEMI study: a Phase 2a trial to evaluate the safety, tolerability, and efficacy of intravenous MTP-131 on reperfusion injury in patients undergoing primary percutaneous coronary intervention.

Aims: Among patients with ST-elevation myocardial infarction (STEMI), reperfusion injury contributes to additional myocardial damage. MTP-131 is a cell-permeable peptide that preserves the integrity of cardiolipin, enhances mitochondrial energetics, and improves myocyte survival during reperfusion.

Methods And Results: EMBRACE STEMI is a multicentre, randomized, double-blind Phase 2a trial that evaluated the efficacy and safety of MTP-131 vs.

Identification and functional characterisation of a novel KCNJ2 mutation, Val302del, causing Andersen-Tawil syndrome.

Loss-of-function mutations of the KCNJ2 gene encoding for the inward rectifier potassium channel subunit Kir2.1 cause Andersen-Tawil Syndrome (ATS), a rare genetic disorder characterised by periodic paralysis, ventricular arrhythmias, and dysmorphic features. Clinical manifestations of the disease appear to vary greatly with the nature of mutation, therefore, functional characterisation of ATS-causing mutations is of clinical importance.

Preoperative and perioperative use of levosimendan in cardiac surgery: European expert opinion.

In cardiac surgery, postoperative low cardiac output has been shown to correlate with increased rates of organ failure and mortality. Catecholamines have been the standard therapy for many years, although they carry substantial risk for adverse cardiac and systemic effects, and have been reported to be associated with increased mortality. On the other hand, the calcium sensitiser and potassium channel opener levosimendan has been shown to improve cardiac function with no imbalance in oxygen consumption, and to have protective effects in other organs.

Hemodynamic effects of slow breathing: does the pattern matter beyond the rate?

Purpose: Patterned breathing allows standardized serial measurements of heart rate variability and baroreflex indices. The slow breathing augments these parameters, and regular exercises, including yoga breathing practices with even respiratory rates have long-term beneficial effects in cardiovascular diseases. The role of temporization of breathing phases, i.

The effects of acute blood loss on blood pressure recovery from the Valsalva maneuver.

Human baroreflex regulation plays an important role in stabilising blood pressure. Though we have several indices to quantify cardiovagal responses, sympathetic baroreflex gain remains difficult to assess. We investigated how the recently validated pressure recovery time (PRT) and sympathetic baroreflex gain (SBRS) derived from the Valsalva maneuver was influenced by acute blood loss.

Aprotinin and renal dysfunction after pediatric cardiac surgery.

Background: Aprotinin is a potent antifibrinolytic drug, which reduces postoperative bleeding and transfusion requirements. Recently, two observational studies reported increased incidence of renal dysfunction after aprotinin use in adults. Therefore, the aim of the study was to investigate the safety of aprotinin use in pediatric cardiac surgery patients.

Mid-term echocardiographic follow up of left ventricular function with permanent right ventricular pacing in pediatric patients with and without structural heart disease.

Background: Chronic right ventricular apical pacing may have detrimental effect on left ventricular function and may promote to heart failure in adult patients with left ventricular dysfunction.

Methods: A group of 99 pediatric patients with previously implanted pacemaker was studied retrospectively. Forty-three patients (21 males) had isolated congenital complete or advanced atrioventricular block.

Intraoperative and postoperative risk factors for prolonged mechanical ventilation after pediatric cardiac surgery.

Background: Early extubation after cardiac surgery in children is feasible; however, predictors of prolonged mechanical ventilation (MV) should be recognized as soon as possible.

Methods: At a tertiary pediatric cardiac center, prospective case series analyses were carried out with a total of 411 patients within 1 year of cardiac surgery. Perioperative factors were evaluated for strength of association with duration of MV > 61 h (medium, MMV) and > 7 days (long, LMV).

Metabolic energy metabolism in diabetes: therapeutic implications.

Diabetic alterations of myocardial metabolism result mainly from malfunctions of acetyl-coenzyme A carboxylase, carnitine-palmitoyl-transferase-I and pyruvate-dehydrogenase inducing an overshoot of fatty acid oxidation that inhibits glucose oxidation. Gene expression of pyruvate-dehydrogenase and glucose transporters and depression of the third step of the mitochondrial respiratory chain also contribute to the diabetic alterations of myocardial metabolism. Ischaemic cardiovascular alterations are common and treatment is rarely successful in cases of diabetes since fatty acid oxidation is the costliest metabolic pathway for oxygen.