BACKGROUND: Patients with transposition of the great arteries (TGA) and systemic right ventricle often confront significant adverse cardiac events. The prognostic significance of invasive hemodynamic parameters in this context remains uncertain. Our hypothesis is that the aortic pulsatility index and hemodynamic profiling utilizing invasive measures provide prognostic insights for patients with TGA and a systemic right ventricle. METHODS: This retrospective multicenter cohort study encompasses adults with TGA and a systemic right ventricle who underwent cardiac catheterization. Data collection, spanning from 1994 to 2020, encompasses clinical and hemodynamic parameters, including measured and calculated values such as pulmonary capillary wedge pressure, aortic pulsatility index, and cardiac index. Pulmonary capillary wedge pressure and cardiac index values were used to establish 4 distinct hemodynamic profiles. A pulmonary capillary wedge pressure of ≥15 mm Hg indicated congestion, termed wet, while a cardiac index <2.2 L/min per m2 signified inadequate perfusion, labeled cold. The primary outcome comprised a composite of all-cause death, heart transplantation, or the requirement for mechanical circulatory support. RESULTS: Of 1721 patients with TGA, 242 individuals with available invasive hemodynamic data were included. The median follow-up duration after cardiac catheterization was 11.4 (interquartile range, 7.5-15.9) years, with a mean age of 38.5±10.8 years at the time of cardiac catheterization. Among hemodynamic parameters, an aortic pulsatility index <1.5 emerged as a robust predictor of the primary outcome, with adjusted hazard ratios of 5.90 (95% CI, 3.01-11.62; P<0.001). Among the identified 4 hemodynamic profiles, the cold/wet profile was associated with the highest risk for the primary outcome, with an adjusted hazard ratio of 3.83 (95% CI, 1.63-9.02; P<0.001). CONCLUSIONS: A low aortic pulsatility index (<1.5) and the cold/wet hemodynamic profile are linked with an elevated risk of adverse long-term cardiac outcomes in patients with TGA and systemic right ventricle.

• Klíčová slova
• arterial pressure, cardiac catheterization, cardiac output, congenitally corrected transposition of the great arteries, heart ventricles, hemodynamics, pulmonary wedge pressure, stroke volume,
• MeSH
• dospělí MeSH
• funkce pravé komory srdeční fyziologie   MeSH
• hemodynamika * fyziologie   MeSH
• lidé středního věku MeSH
• lidé MeSH
• plicní tlak v zaklínění fyziologie   MeSH
• prognóza MeSH
• retrospektivní studie MeSH
• srdeční katetrizace * MeSH
• srdeční komory * patofyziologie   diagnostické zobrazování   MeSH
• transpozice velkých cév * patofyziologie   chirurgie   MeSH
• Check Tag
• dospělí MeSH
• lidé středního věku MeSH
• lidé MeSH
• mužské pohlaví MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• multicentrická studie MeSH

PURPOSE OF REVIEW: Cardiogenic shock is a clinical syndrome with different causes and a complex pathophysiology. Recent evidence from clinical trials evokes the urgent need for redefining clinical diagnostic criteria to be compliant with the definition of cardiogenic shock and current diagnostic methods. RECENT FINDINGS: Conflicting results from randomized clinical trials investigating mechanical circulatory support in patients with cardiogenic shock have elicited several extremely important questions. At minimum, it is questionable whether survivors of cardiac arrest should be included in trials focused on cardiogenic shock. Moreover, considering the wide availability of ultrasound and hemodynamic monitors capable of arterial pressure analysis, the current clinical diagnostic criteria based on the presence of hypotension and hypoperfusion have become insufficient. As such, new clinical criteria for the diagnosis of cardiogenic shock should include evidence of low cardiac output and appropriate ventricular filling pressure. SUMMARY: Clinical diagnostic criteria for cardiogenic shock should be revised to better define cardiac pump failure as a primary cause of hemodynamic compromise.

• MeSH
• hemodynamika fyziologie   MeSH
• kardiogenní šok * diagnóza   patofyziologie   terapie   etiologie   MeSH
• lidé MeSH
• srdeční zástava terapie   diagnóza   MeSH
• Check Tag
• lidé MeSH
• Publikační typ
• časopisecké články MeSH
• přehledy MeSH

BACKGROUND: The effect of left ventricular septal myocardial pacing (LVSP) and left bundle branch pacing (LBBP) on ventricular synchrony and left ventricular (LV) hemodynamic status is poorly understood. OBJECTIVES: The aim of this study was to investigate the impact of LVSP and LBBP vs biventricular pacing (BVP) on ventricular electrical synchrony and hemodynamic status in cardiac resynchronization therapy patients. METHODS: In cardiac resynchronization therapy candidates with LV conduction disease, ventricular synchrony was assessed by measuring QRS duration (QRSd) and using ultra-high-frequency electrocardiography. LV electrical dyssynchrony was assessed as the difference between the first activation in leads V1 to V8 to the last from leads V4 to V8. LV hemodynamic status was estimated using invasive systolic blood pressure measurement during multiple transitions between LBBP, LVSP, and BVP. RESULTS: A total of 35 patients with a mean LV ejection fraction of 29% and a mean QRSd of 168 ± 24 ms were included. Thirteen had ischemic cardiomyopathy. QRSd during BVP, LVSP, and LBBP was the same, but LBBP provided shorter LV electrical dyssynchrony than BVP (-10 ms; 95% CI: -16 to -4 ms; P = 0.001); the difference between LVSP and BVP was not significant (-5 ms; 95% CI: -12 to 1 ms; P = 0.10). LBBP was associated with higher systolic blood pressure than BVP (4%; 95% CI: 2%-5%; P < 0.001), whereas LVSP was not (1%; 95% CI: 0%-2%; P = 0.10). Hemodynamic differences during LBBP and LVSP vs BVP were more pronounced in nonischemic than ischemic patients. CONCLUSIONS: Ultra-high-frequency electrocardiography allowed the documentation of differences in LV synchrony between LBBP, LVSP, and BVP, which were not observed by measuring QRSd. LVSP provided the same LV synchrony and hemodynamic status as BVP, while LBBP was better than BVP in both.

• Klíčová slova
• BVP, LBBP, LVSP, UHF-ECG, cardiac resynchronization therapy, dyssynchrony,
• MeSH
• dysfunkce levé srdeční komory patofyziologie   terapie   MeSH
• elektrokardiografie * MeSH
• funkce levé komory srdeční fyziologie   MeSH
• hemodynamika * fyziologie   MeSH
• lidé středního věku MeSH
• lidé MeSH
• mezikomorová přepážka patofyziologie   MeSH
• senioři MeSH
• srdeční resynchronizační terapie * metody   MeSH
• Check Tag
• lidé středního věku MeSH
• lidé MeSH
• mužské pohlaví MeSH
• senioři MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• srovnávací studie MeSH

Hemodynamic derangements are defining features of cardiogenic shock. Randomized clinical trials have examined the efficacy of various therapeutic interventions, from percutaneous coronary intervention to inotropes and mechanical circulatory support (MCS). However, hemodynamic management in cardiogenic shock has not been well-studied. This State-of-the-Art review will provide a framework for hemodynamic management in cardiogenic shock, including a description of the 4 therapeutic phases from initial 'Rescue' to 'Optimization', 'Stabilization' and 'de-Escalation or Exit therapy' (R-O-S-E), phenotyping and phenotype-guided tailoring of pharmacological and MCS support, to achieve hemodynamic and therapeutic goals. Finally, the premises that form the basis for clinical management and the hypotheses for randomized controlled trials will be discussed, with a view to the future direction of cardiogenic shock.

• Klíčová slova
• cardiogenic shock, heart transplantation, hemodynamics, inotropes, left ventricular assist devices, mechanical circulatory support,
• MeSH
• hemodynamika * fyziologie   MeSH
• jednotky intenzivní péče * MeSH
• kardiogenní šok * terapie   patofyziologie   MeSH
• lidé MeSH
• podpůrné srdeční systémy MeSH
• Check Tag
• lidé MeSH
• Publikační typ
• časopisecké články MeSH
• přehledy MeSH

A well-developed heart is essential for embryonic survival. There are constant interactions between cardiac tissue motion and blood flow, which determine the heart shape itself. Hemodynamic forces are a powerful stimulus for cardiac growth and differentiation. Therefore, it is particularly interesting to investigate how the blood flows through the heart and how hemodynamics is linked to a particular species and its development, including human. The appropriate patterns and magnitude of hemodynamic stresses are necessary for the proper formation of cardiac structures, and hemodynamic perturbations have been found to cause malformations via identifiable mechanobiological molecular pathways. There are significant differences in cardiac hemodynamics among vertebrate species, which go hand in hand with the presence of specific anatomical structures. However, strong similarities during development suggest a common pattern for cardiac hemodynamics in human adults. In the human fetal heart, hemodynamic abnormalities during gestation are known to progress to congenital heart malformations by birth. In this chapter, we discuss the current state of the knowledge of the prenatal cardiac hemodynamics, as discovered through small and large animal models, as well as from clinical investigations, with parallels gathered from the poikilotherm vertebrates that emulate some hemodynamically significant human congenital heart diseases.

• Klíčová slova
• Axolotl, Chick embryo, DORV, Developing myocardium, ET1, Embryogenesis, Endothelin 1, Fetal heart, Guinea pig, HLHS, Hemodynamics, Hyperplasia, Hypertrophy, Hypoplastic left heart syndrome, KLF2, Krüppel-like factor 2, Lamb, Mouse, NOS3, Nitric oxide synthase 3, Pressure overload, Rat, Reptile, VSD, Volume overload, Zebrafish,
• MeSH
• hemodynamika * fyziologie   MeSH
• lidé MeSH
• srdce * růst a vývoj   fyziologie   MeSH
• vrozené srdeční vady patofyziologie   MeSH
• zvířata MeSH
• Check Tag
• lidé MeSH
• zvířata MeSH
• Publikační typ
• časopisecké články MeSH
• přehledy MeSH

• MeSH
• arteriovenózní píštěl patofyziologie   diagnóza   komplikace   MeSH
• arteriovenózní zkrat metody   MeSH
• dialýza ledvin MeSH
• hemodynamika * fyziologie   MeSH
• lidé středního věku MeSH
• lidé MeSH
• senioři MeSH
• srdeční selhání * patofyziologie   diagnóza   MeSH
• vysoký srdeční výdej patofyziologie   etiologie   diagnóza   MeSH
• Check Tag
• lidé středního věku MeSH
• lidé MeSH
• mužské pohlaví MeSH
• senioři MeSH
• ženské pohlaví MeSH
• Publikační typ
• dopisy MeSH

BACKGROUND AND OBJECTIVES: Age-related arterial stiffening increases peripheral resistance and decreases arterial distensibility, thus contributing to hypertension, an important risk factor of atherosclerosis. It causes abnormal blood flow, endothelial dysfunction, higher pulse wave velocity, and consequently elevated pressure wave amplitude. METHODS: This paper presents the influence of these changes via multiscale 3D-0D transient computational fluid dynamics simulations of blood flow in five patient-specific geometries of human carotid bifurcation using archetypal flow waveforms for young and old subjects. RESULTS: The proposed model shows a significant decrease in the time-averaged wall shear stress (TAWSS) for the old archetypal flow waveform. This is in good agreement with clinical data on a straight segment of common carotid arteries available for young and old subjects. Moreover, our study showed that the decrease of area-averaged TAWSS related to the old flow waveform is much more pronounced (2.5 ÷ 4.5 times higher) at risk areas (areas showing TAWSS below its threshold value of 0.48 Pa) than in straight segments commonly considered in clinical studies. CONCLUSIONS: Since arterial stiffness can be lowered through long-term usage of any of the five basic groups of antihypertensives, possible benefits of such medical therapy could be not only lowering blood pressure and peripheral resistance but also in increasing the TAWSS and thus attenuating an important mechanism of the atherosclerotic process.

• Klíčová slova
• Antihypertensives, Atherosclerosis, Carotid artery, Multiscale flow simulations, Parameter optimization, Patient-specific model,
• MeSH
• analýza pulzové vlny MeSH
• antihypertenziva * farmakologie   MeSH
• arteriae carotides MeSH
• ateroskleróza * farmakoterapie   MeSH
• hemodynamika fyziologie   MeSH
• lidé MeSH
• modely kardiovaskulární MeSH
• počítačová simulace MeSH
• rychlost toku krve MeSH
• Check Tag
• lidé MeSH
• Publikační typ
• časopisecké články MeSH
• Názvy látek
• antihypertenziva * MeSH

OBJECTIVES: Fabry disease (FD) is a rare X-linked lysosomal storage disorder with variable phenotypes, including neurological symptoms. These can be influenced by vascular impairment. Extracranial and transcranial vascular sonography is an effective and noninvasive method for measuring arterial structures and blood flow. The study aims to investigate cerebrovascular phenotype characteristics in FD patients compared to controls using neurosonology. METHODS: This is a single-center, cross-sectional study of 130 subjects-65 patients (38 females), with genetically confirmed FD, and 65 sex- and age-matched controls. Using ultrasonography, we measured structural and hemodynamic parameters, including distal common carotid artery intima-media thickness, inner vertebral artery diameter, resting blood flow velocity, pulsatility index, and cerebral vasoreactivity (CVR) in the middle cerebral artery. To assess differences between FD and controls and to identify factors influencing investigated outcomes, unadjusted and adjusted regression analyses were performed. RESULTS: In comparison to sex- and age-matched controls, FD patients displayed significantly increased carotid artery intima-media thickness (observed FD 0.69 ± 0.13 mm versus controls 0.63 ± 0.12 mm; Padj = .0014), vertebral artery diameter (observed FD 3.59 ± 0.35 mm versus controls 3.38 ± 0.33 mm; Padj = .0002), middle cerebral artery pulsatility index (observed FD 0.98 ± 0.19 versus controls 0.87 ± 0.11; Padj < .0001), and significantly decreased CVR (observed FD 1.21 ± 0.49 versus controls 1.35 ± 0.38; Padj = .0409), when adjusted by age, BMI, and sex. Additionally, FD patients had significantly more variable CVR (0.48 ± 0.25 versus 0.21 ± 0.14; Padj < .0001). CONCLUSIONS: Our results suggest the presence of multiple vascular abnormalities and changes in hemodynamic parameters of cerebral arteries in patients with FD.

• Klíčová slova
• Fabry disease, breath-holding index, cerebral blood flow, cerebral vasoreactivity, intima-media thickness, pulsatility index,
• MeSH
• Fabryho nemoc * diagnostické zobrazování   MeSH
• hemodynamika fyziologie   MeSH
• intimomediální šíře tepenné stěny MeSH
• lidé MeSH
• mozkový krevní oběh fyziologie   MeSH
• průřezové studie MeSH
• rychlost toku krve fyziologie   MeSH
• ultrasonografie dopplerovská transkraniální metody   MeSH
• ultrasonografie MeSH
• Check Tag
• lidé MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH

BACKGROUND: A biomechanical model of the heart can be used to incorporate multiple data sources (electrocardiography, imaging, invasive hemodynamics). The purpose of this study was to use this approach in a cohort of patients with tetralogy of Fallot after complete repair (rTOF) to assess comparative influences of residual right ventricular outflow tract obstruction (RVOTO) and pulmonary regurgitation on ventricular health. METHODS: Twenty patients with rTOF who underwent percutaneous pulmonary valve replacement (PVR) and cardiovascular magnetic resonance imaging were included in this retrospective study. Biomechanical models specific to individual patient and physiology (before and after PVR) were created and used to estimate the RV myocardial contractility. The ability of models to capture post-PVR changes of right ventricular (RV) end-diastolic volume (EDV) and effective flow in the pulmonary artery (Qeff) was also compared with expected values. RESULTS: RV contractility before PVR (mean 66 ± 16 kPa, mean ± standard deviation) was increased in patients with rTOF compared with normal RV (38-48 kPa) (P < 0.05). The contractility decreased significantly in all patients after PVR (P < 0.05). Patients with predominantly RVOTO demonstrated greater reduction in contractility (median decrease 35%) after PVR than those with predominant pulmonary regurgitation (median decrease 11%). The model simulated post-PVR decreased EDV for the majority and suggested an increase of Qeff-both in line with published data. CONCLUSIONS: This study used a biomechanical model to synthesize multiple clinical inputs and give an insight into RV health. Individualized modeling allows us to predict the RV response to PVR. Initial data suggest that residual RVOTO imposes greater ventricular work than isolated pulmonary regurgitation.

CONTEXTE :: Une modélisation biomécanique du cœur peut être utilisée pour intégrer des sources de données multiples (électrocardiographie, imagerie, hémodynamique invasive). Le but de cette étude était d’utiliser cette approche pour une cohorte de patients atteints de tétralogie de Fallot aprèsr réparation complète (TdFr) pour évaluer, au niveau du ventricule, les influences comparatives de la sténose résiduelle de la voie d’éjection du ventricule droit (SVEVD) et de la régurgitation pulmonaire. MÉTHODES :: Vingt patients atteints de TdFr ayant subi un remplacement percutane de la valve pulmonaire (RVP) et une imagerie par résonance magnétique cardiovasculaire ont été inclus dans cette étude rétrospective. Des modèles biomécaniques adaptés à chaque patient et à sa physiologie (avant et après le RVP) ont été créés et utilisés pour estimer la contractilité myocardique du ventricule droit (VD). La capacité des modèles à capturer les changements post-RVP du volume télédiastolique (VTD) du VD et du débit effectif dans l’artère pulmonaire (Qeff) a également été comparée aux valeurs attendues. RÉSULTATS :: La contractilité du VD avant le RVP (moyenne 66 ± 16 kPa, moyenne ± déviation standard)) était plus élevée chez les patients atteints de TdFr par rapport au VD normal (38–48 kPa) (P < 0,05). La contractilité a diminué de manière significative chez tous les patients après le RVP (P < 0,05). Les patients présentant une SVEVD prédominante ont montré une plus grande réduction de la contractilité (diminution médiane de 35 %) après le RVP que ceux présentant une régurgitation pulmonaire prédominante (diminution médiane de 11 %). Le modèle a simulé une diminution du VTD-VD après le RVP pour la majorité des patients et a suggéré une augmentation du Qeff, ce qui est conforme aux données publiées. CONCLUSIONS :: Cette étude a utilisé un modèle biomécanique pour synthétiser de multiples données cliniques et donner un aperçu de l’état de santé du VD. La modélisation individualisée nous permet de prédire la réponse du VD au RVP. Les premières données suggèrent que la SVEVD résiduelle impose un travail ventriculaire plus important que la régurgitation pulmonaire isolée.

• MeSH
• biologické modely * MeSH
• chirurgická náhrada chlopně metody   MeSH
• dospělí MeSH
• Fallotova tetralogie chirurgie   MeSH
• hemodynamika fyziologie   MeSH
• insuficience plicnice vrozené   diagnóza   chirurgie   MeSH
• kardiochirurgické výkony metody   MeSH
• lidé MeSH
• magnetická rezonance kinematografická MeSH
• mnohočetné abnormality * MeSH
• následné studie MeSH
• plicní chlopeň abnormality   diagnostické zobrazování   chirurgie   MeSH
• reoperace MeSH
• retrospektivní studie MeSH
• srdeční komory diagnostické zobrazování   patofyziologie   MeSH
• Check Tag
• dospělí MeSH
• lidé MeSH
• mužské pohlaví MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• pozorovací studie MeSH
• práce podpořená grantem MeSH

BACKGROUND: Cardiac muscle hypercontractility is a key pathophysiological abnormality in hypertrophic cardiomyopathy, and a major determinant of dynamic left ventricular outflow tract (LVOT) obstruction. Available pharmacological options for hypertrophic cardiomyopathy are inadequate or poorly tolerated and are not disease-specific. We aimed to assess the efficacy and safety of mavacamten, a first-in-class cardiac myosin inhibitor, in symptomatic obstructive hypertrophic cardiomyopathy. METHODS: In this phase 3, randomised, double-blind, placebo-controlled trial (EXPLORER-HCM) in 68 clinical cardiovascular centres in 13 countries, patients with hypertrophic cardiomyopathy with an LVOT gradient of 50 mm Hg or greater and New York Heart Association (NYHA) class II-III symptoms were assigned (1:1) to receive mavacamten (starting at 5 mg) or placebo for 30 weeks. Visits for assessment of patient status occurred every 2-4 weeks. Serial evaluations included echocardiogram, electrocardiogram, and blood collection for laboratory tests and mavacamten plasma concentration. The primary endpoint was a 1·5 mL/kg per min or greater increase in peak oxygen consumption (pVO2) and at least one NYHA class reduction or a 3·0 mL/kg per min or greater pVO2 increase without NYHA class worsening. Secondary endpoints assessed changes in post-exercise LVOT gradient, pVO2, NYHA class, Kansas City Cardiomyopathy Questionnaire-Clinical Summary Score (KCCQ-CSS), and Hypertrophic Cardiomyopathy Symptom Questionnaire Shortness-of-Breath subscore (HCMSQ-SoB). This study is registered with ClinicalTrials.gov, NCT03470545. FINDINGS: Between May 30, 2018, and July 12, 2019, 429 adults were assessed for eligibility, of whom 251 (59%) were enrolled and randomly assigned to mavacamten (n=123 [49%]) or placebo (n=128 [51%]). 45 (37%) of 123 patients on mavacamten versus 22 (17%) of 128 on placebo met the primary endpoint (difference +19·4%, 95% CI 8·7 to 30·1; p=0·0005). Patients on mavacamten had greater reductions than those on placebo in post-exercise LVOT gradient (-36 mm Hg, 95% CI -43·2 to -28·1; p<0·0001), greater increase in pVO2 (+1·4 mL/kg per min, 0·6 to 2·1; p=0·0006), and improved symptom scores (KCCQ-CSS +9·1, 5·5 to 12·7; HCMSQ-SoB -1·8, -2·4 to -1·2; p<0·0001). 34% more patients in the mavacamten group improved by at least one NYHA class (80 of 123 patients in the mavacamten group vs 40 of 128 patients in the placebo group; 95% CI 22·2 to 45·4; p<0·0001). Safety and tolerability were similar to placebo. Treatment-emergent adverse events were generally mild. One patient died by sudden death in the placebo group. INTERPRETATION: Treatment with mavacamten improved exercise capacity, LVOT obstruction, NYHA functional class, and health status in patients with obstructive hypertrophic cardiomyopathy. The results of this pivotal trial highlight the benefits of disease-specific treatment for this condition. FUNDING: MyoKardia.

• MeSH
• benzylaminy škodlivé účinky   terapeutické užití   MeSH
• beta blokátory terapeutické užití   MeSH
• blokátory kalciových kanálů terapeutické užití   MeSH
• dvojitá slepá metoda MeSH
• hemodynamika fyziologie   MeSH
• hodnocení výsledků pacienta MeSH
• hypertrofická kardiomyopatie farmakoterapie   patofyziologie   MeSH
• kardiovaskulární látky terapeutické užití   MeSH
• lidé středního věku MeSH
• lidé MeSH
• senioři MeSH
• spotřeba kyslíku fyziologie   MeSH
• srdeční myosiny antagonisté a inhibitory   MeSH
• tolerance zátěže fyziologie   MeSH
• uracil škodlivé účinky   analogy a deriváty   terapeutické užití   MeSH
• Check Tag
• lidé středního věku MeSH
• lidé MeSH
• mužské pohlaví MeSH
• senioři MeSH
• ženské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• klinické zkoušky, fáze III MeSH
• multicentrická studie MeSH
• práce podpořená grantem MeSH
• randomizované kontrolované studie MeSH
• Názvy látek
• benzylaminy MeSH
• beta blokátory MeSH
• blokátory kalciových kanálů MeSH
• kardiovaskulární látky MeSH
• MYK-461 MeSH Prohlížeč
• srdeční myosiny MeSH
• uracil MeSH