RESUMEN
The most common clinical scenario in which RV is systemic, is dextroposition transposition of the great arteries (dTGA). This is the second most common cyanotic congenital heart disease [2]. Before the arterial switch operation was introduced in 1975 [3], patients with dTGA were treated with the Mustard or Senning procedure, also referred to as the atrial switch operation, where the systemic veins are routed to the subpulmonary morphological left ventricle and the pulmonary veins are routed to the systemic right ventricle [4].The atrial switch operation leaves the RV in a systemic position, leading to gradual RV dysfunction and RV failure [5-8]. In this context, sRV functional reserve and its ability to augment performance with exercise performance is not well studied.
RESUMEN
Background and aims of the study A functionally single ventricle (FSV) refers to a group of congenital heart defects that are not amenable for biventricular correction. The Fontan operation is utilized as surgical treatment for most of FSV patients. The evaluation of FSV function is extremely difficult due to its unique pathophysiology. This study aimed to explore the efficacy of speckle tracking echocardiography (STE) parameters measured at rest and during exercise for comprehensive assessment of univentricular heart. METHODS: We enrolled 37 patients with a functionally single ventricle after the Fontan operation, hospitalized in the Department of Congenital Heart Defects between years 2019 and 2021.The echocardiographic stress tests were performed in the Echocardiography Laboratory of the Congenital Heart Defects Department. The study was conducted on a bicycle ergometer in a semi-recumbent position. The parameters obtained by speckle tracking echocardiography (STE): the longitudinal strain of the FSV free wall (Æ) and the longitudinal strain of myocardial layers: subendocardial, medial and subepicardial were analyzed. A transmural longitudinal strain gradient (TG) was calculated as the difference between longitudinal deformation of the subendocardial and subepicardial layers. Current results of cardiac magnetic resonance imaging (CMR) and cardio-pulmonary test (CPET) were also incorporated. Demographic data, past interventions, pharmacological treatment and comorbidities were extracted from medical records. RESULTS: Æ at rest and during exercise were not related to the parameters of physical capacity obtained on CPET nor to the CMR results. The transmural strain gradient was dependent on physical performance parameter- peak oxygen uptake- and related to the FSV ejection fraction calculated by magnetic resonance imaging. CONCLUSIONS: The transmural strain gradient and FSV free wall strain are readily measurable and suitable for evaluating single ventricle function. The TG is positively correlated with peak oxygen uptake during the cardiopulmonary test and with the ejection fraction derived from cardiac magnetic resonance imaging. The applicability of these findings in patients undergoing the Fontan procedure warrants further exploration.
Asunto(s)
Ecocardiografía , Procedimiento de Fontan , Humanos , Procedimiento de Fontan/métodos , Masculino , Femenino , Ecocardiografía/métodos , Adolescente , Ventrículos Cardíacos/diagnóstico por imagen , Ventrículos Cardíacos/fisiopatología , Ventrículos Cardíacos/anomalías , Adulto , Corazón Univentricular/cirugía , Corazón Univentricular/diagnóstico por imagen , Cardiopatías Congénitas/cirugía , Cardiopatías Congénitas/diagnóstico por imagen , Niño , Adulto Joven , Prueba de Esfuerzo/métodos , Cuidados Paliativos/métodosRESUMEN
BACKGROUND: Chronic heart failure (CHF) is a major healthcare problem. However, there are no epidemiological studies assessing the prevalence of CHF in the general population with diagnosis based on algorithms recommended for clinical practice. AIM: The aim of the HF-Pomorskie survey was to assess the prevalence of three basic components of the 2016 ESC diagnostic algorithm for CHF (symptoms, N-terminal pro B-type natriuretic peptide [NT-proBNP], and abnormalities on echocardiography) and to determine whether this algorithm may be applicable to studies in general population samples. METHODS: The study was performed in a representative sample of 313 adults (170 women and 143 men) aged between 20 and 90 years (mean 55.2 years [15.3]) in Northern Poland. A questionnaire to determine New York Heart Association [NYHA] class, laboratory tests including NT-proBNP, as well as transthoracic echocardiography and spirometry examinations were performed in all subjects. RESULTS: Dyspnea (NYHA class II-IV) was reported by 13.7% of recruited participants. Dyspnea and elevated levels of NT-proBNP (>125 pg/ml) were found in 7.7% of all examined subjects, while dyspnea, elevated NT-proBNP levels accompanied by systolic or diastolic abnormalities on echocardiography occurred in 4.8%. In the group without dyspnea (86.3% of all examined subjects), every sixth subject had an elevated level of NT-proBNP. On the other hand, 5.8% of studied subjects reported a previous diagnosis of CHF, which was confirmed using the current ESC algorithm in 78% of them. CONCLUSIONS: The prevalence of CHF assessed by the 2016 ESC diagnostic algorithm in the representative sample of adults was equal to 4.8%. The clinical algorithm for the diagnosis of CHF is fully applicable to the representative surveys in the general population. However, due to logistic and economic factors, echocardiography examination and NT-proBNP determination can be limited to patients reporting dyspnea or previous diagnosis of CHF.
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Insuficiencia Cardíaca , Masculino , Adulto , Humanos , Femenino , Adulto Joven , Persona de Mediana Edad , Anciano , Anciano de 80 o más Años , Prevalencia , Insuficiencia Cardíaca/diagnóstico , Insuficiencia Cardíaca/epidemiología , Enfermedad Crónica , Algoritmos , Disnea/diagnóstico , Disnea/epidemiologíaRESUMEN
INTRODUCTION: With advancing age, adults with congenital heart disease (ACHD) are at a higher risk of developing atherosclerotic coronary artery disease (CAD). OBJECTIVES: We aimed to determine the prevalence of CAD, its risk factors, and use of guidelinedirected pharmacotherapy among older patients with ACHD. Patients and methods: We studied all ACHD patients aged 60 years or older hospitalized in our department between the years 2013 and 2020. CAD was defined as a history of acute coronary syndrome or coronary revascularization, or more than 50% diameter stenosis on coronary angiography. Data regarding the underlying heart defect, prevalence of cardiovascular risk factors, and drug prescriptions were collected. RESULTS: A total of 198 patients with known coronary artery status (mean [SD] age, 66.2 [5.3] years; 43.3% men) were included in the analysis. Of them, 54 (27.3%) had CAD. The individuals with CAD were more often men, and they were more likely to have a mild heart defect, dyslipidemia, and a history of hypertension and tobacco use. Multivariable analysis showed that male sex (P = 0.001), dyslipidemia (P = 0.003), and hypertension (P = 0.04) were positive independent predictors of CAD, whereas overweight / obesity was identified as a negative independent predictor (P = 0.04). The proportion of CAD patients on antiplatelet and / or anticoagulant drugs was 92.6%. ßBlockers were prescribed to 87% of the patients, and a lipidlowering agent to 96% of the study population. CONCLUSIONS: CAD is common in older patients with ACHD. Our results underline the importance of identification and treatment of modifiable CAD risk factors in individuals with ACHD. The obesity paradox might also play a role in this population. The rate of guidelinerecommended pharmacotherapy implementation seems to be satisfactory.
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Enfermedad de la Arteria Coronaria , Dislipidemias , Cardiopatías Congénitas , Hipertensión , Humanos , Masculino , Anciano , Femenino , Enfermedad de la Arteria Coronaria/tratamiento farmacológico , Enfermedad de la Arteria Coronaria/epidemiología , Enfermedad de la Arteria Coronaria/etiología , Factores de Riesgo , Cardiopatías Congénitas/complicaciones , Cardiopatías Congénitas/epidemiología , Hipertensión/complicaciones , Dislipidemias/complicacionesAsunto(s)
Endocarditis Bacteriana , Cardiopatías Congénitas , Infecciones Estreptocócicas , Humanos , Streptococcus sanguis , Infecciones Estreptocócicas/complicaciones , Infecciones Estreptocócicas/tratamiento farmacológico , Endocarditis Bacteriana/complicaciones , Endocarditis Bacteriana/diagnóstico por imagen , Endocarditis Bacteriana/tratamiento farmacológico , Corazón , Cardiopatías Congénitas/complicacionesRESUMEN
BACKGROUND: Coronary artery fistulas (CAFs) are usually congenital coronary artery anomalies of termination. AIMS: This study aimed to assess the prevalence, anatomic characteristics, and clinical significance of CAFs detected by computed tomography (CT) in an adult population. METHODS: We performed 45 817 CT examinations in 39 066 subjects between 2008 and 2020. The electronic database was manually checked using specific keywords to identify patients with CAFs. The CT characteristics of CAFs were evaluated. CAF was defined as clinically significant if it was the most plausible cause of myocardial infarction, infective endocarditis, heart failure, death during follow-up, hospitalization, or if it required either percutaneous or surgical intervention. RESULTS: Of 39 066 patients, 56 CAFs were detected in 42 subjects (20 men, 47.6%) with a prevalence of 0.11%. Most CAFs originated from the right coronary artery (RCA) (48.2%) and drained into the pulmonary artery (PA) (58.9%). CAFs terminating in the PA were more frequently multiple (P <0.001) and tortuous (P <0.001) as compared to CAFs without PA drainage. Clinically significant CAFs, identified in 7 of 42 patients, were more common in younger (P = 0.03) and male (P = 0.04) subjects and had larger lumen area and diameter at the site of origin (P = 0.03, P = 0.03, respectively). CONCLUSIONS: In the unselected adult population undergoing coronary CT angiography, the RCA and the PA are the most common sites of origin and termination of CAFs, respectively. CAFs draining into the PA are more often multiple and tortuous. Clinically meaningful CAFs are larger and most frequently detected in younger and male patients.
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Enfermedad de la Arteria Coronaria , Anomalías de los Vasos Coronarios , Fístula , Adulto , Humanos , Masculino , Angiografía Coronaria/métodos , Enfermedad de la Arteria Coronaria/diagnóstico por imagen , Enfermedad de la Arteria Coronaria/epidemiología , Tomografía Computarizada por Rayos X/métodos , Anomalías de los Vasos Coronarios/diagnóstico por imagen , Anomalías de los Vasos Coronarios/epidemiologíaRESUMEN
BACKGROUND: The growing number of adult patients after the Fontan operation requires regular surveillance tests in specialized centers. AIMS: Our study aimed to evaluate the current practice of care for Fontan patients in Poland using a multicenter survey. METHODS: Eight centers were included in the study including 5 adult congenital heart disease (ACHD) and 3 pediatric centers for adolescents. To compare the centers and facilitate interpretation of results, the Fontan Surveillance Score (FSS) was developed. The higher score is consistent with better care, with a maximum of 19 points. RESULTS: We included in the study 398 Fontan patients (243 adults and 155 adolescents [aged 14-18 years]). The median FSS was 13 points with variability between centers (interquartile range 7-14 points). Centers providing continuous care from the pediatric period until 18 years of age achieved a higher FSS compared to ACHD centers (median: 14 points vs. 12 points; P <0.001). Most of the patients, both in the ACHD (82.3%) and pediatric centers (89%), were seen annually and had a physical examination, electrocardiogram, and echocardiogram performed at each visit. However, we observed unsatisfactory utilization of tests identifying early stages of Fontan circulation failure (cardiopulmonary exercise tests, cardiac magnetic resonance, liver biochemistry and imaging, detection of protein-losing enteropathy). CONCLUSIONS: Our results showed that there is no unified surveillance approach for Fontan patients in Poland. The practice of care for adults differs from that of adolescents.
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Procedimiento de Fontan , Cardiopatías Congénitas , Niño , Humanos , Adulto , Adolescente , Cardiopatías Congénitas/cirugía , Cardiopatías Congénitas/diagnóstico , Polonia , Prueba de Esfuerzo , Electrocardiografía , Estudios RetrospectivosAsunto(s)
Implantación de Prótesis de Válvulas Cardíacas , Prótesis Valvulares Cardíacas , Insuficiencia de la Válvula Pulmonar , Válvula Pulmonar , Reemplazo de la Válvula Aórtica Transcatéter , Humanos , Cateterismo Cardíaco , Polonia , Diseño de Prótesis , Válvula Pulmonar/diagnóstico por imagen , Válvula Pulmonar/cirugía , Insuficiencia de la Válvula Pulmonar/cirugía , Resultado del TratamientoRESUMEN
Pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) patients have a more severe COVID-19 course than the general population. Many patients report different persistent symptoms after SARS-CoV-2 infection. The aim of our study is to analyze the prevalence of long COVID-19 symptoms and assess if COVID-19 affects pulmonary hypertension (PH) prognosis. PAH/CTEPH patients who survived COVID-19 for at least 3 months before visiting the PH centers were included in the study. The patients were assessed for symptoms in acute phase of SARS-CoV-2 infection and persisting in follow-up visit, WHO functional class, 6-min walk distance, NT-proBNP concentration. The COMPERA 2.0 model was used to calculate 1-year risk of death due to PH at baseline and at follow-up. Sixty-nine patients-54 (77.3%) with PAH and 15 (21.7%) with CTEPH, 68% women, with a median age of 47.5 years (IQR 37-68)-were enrolled in the study. About 17.1% of patients were hospitalized due to COVID-19 but none in an ICU. At follow-up (median: 155 days after onset of SARS-CoV-2 symptoms), 62% of patients reported at least 1 COVID-19-related symptom and 20% at least 5 symptoms. The most frequently reported symptoms were: fatigue (30%), joint pain (23%), muscle pain (17%), nasal congestion (17%), anosmia (13%), insomnia (13%), and dyspnea (12%). Seventy-two percent of PH patients had a low or intermediate-low risk of 1-year death due to PH at baseline, and 68% after COVID-19 at follow-up. Over 60% of PAH/CTEPH patients who survived COVID-19 suffered from long COVID-19 syndrome, but the calculated 1-year risk of death due to PH did not change significantly after surviving mild or moderate COVID-19.
RESUMEN
INTRODUCTION: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a progressive disease leading to ventricular arrhythmias and heart failure. Determining optimal time for heart transplantation (HTx) is challenging; therefore, it is necessary to identify risk factors for disease progression. OBJECTIVES: The study aimed to identify predictors of endstage heart failure and to evaluate the role of biomarkers in predicting adverse outcomes in ARVC. PATIENTS AND METHODS: A total of 91 individuals with ARVC (59 men; mean [SD] age, 47 [16] years) were included. In all patients, information on medical history was collected, electrocardiography and echocardiography were performed, and serum levels of selected biomarkers (soluble form of the ST2 protein [sST2], galectin3 [Gal3], extracellular matrix metalloproteinases [MMP2 and MMP9], Nterminal pro-Btype natriuretic peptide [NTproBNP], and highsensitivity troponin T [hsTnT]) were measured. Thereafter, the participants were followed for the primary end point of death or HTx, as well as the secondary end point of major arrhythmic events (MAEs), defined as sudden cardiac death, ventricular fibrillation, sustained ventricular tachycardia, or appropriate implantable cardioverterdefibrillator intervention. RESULTS: During the median (interquartile range) followup of 36.4 (29.8-41.2) months, 13 patients (14%) reached the primary end point of death or HTx, and 27 (30%) experienced MAEs. The patients who achieved the primary end point had higher levels of sST2, MMP2, NTproBNP, and hsTnT, but not of Gal-3 and MMP-9. Three factors turned out to be independent predictors of death or HTx: higher NTproBNP concentration (≥890.3 pg/ml), greater right ventricular enddiastolic area (≥39 cm2), and a history of atrial tachycardia. None of the biomarkers predicted MAEs. CONCLUSIONS: An NTproBNP concentration greater than or equal to 890.3 pg/ml, right ventricular end-diastolic area of 39 cm2 or greater, and a history of atrial tachycardia were identified as risk factors for death or HTx in ARVC. Higher levels of sST2, MMP2, NTproBNP, and hsTnT were associated with reaching the primary end point of death or HTx. The biomarkers had no value in predicting ventricular arrhythmias.
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Arritmias Cardíacas , Displasia Ventricular Derecha Arritmogénica , Insuficiencia Cardíaca , Adulto , Anciano , Femenino , Humanos , Masculino , Persona de Mediana Edad , Arritmias Cardíacas/sangre , Arritmias Cardíacas/diagnóstico , Arritmias Cardíacas/etiología , Displasia Ventricular Derecha Arritmogénica/sangre , Displasia Ventricular Derecha Arritmogénica/complicaciones , Displasia Ventricular Derecha Arritmogénica/cirugía , Biomarcadores/sangre , Electrocardiografía , Insuficiencia Cardíaca/sangre , Insuficiencia Cardíaca/diagnóstico , Insuficiencia Cardíaca/etiología , Trasplante de Corazón , Péptido Natriurético Encefálico/sangre , Fragmentos de Péptidos/sangre , Factores de RiesgoRESUMEN
INTRODUCTION: Pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) lead to progressive right heart failure. The mortality rates in PAH and CTEPH patients due to COVID19 are high, and vaccination against COVID19 is recommended in this group. OBJECTIVES: We analyzed the incidence and outcomes of COVID19in the PAH/CTEPH patients for 2 years of the pandemic, as well as the predictors of worse outcomes of COVID19 in this group. PATIENTS AND METHODS: PAH/CTEPH patient data for this observational, cohort study were obtained from 3 pulmonary hypertension centers between March 11, 2020 and March 11, 2022. RESULTS: A total of 364 consecutive patients with PAH/CTEPH (248/122; 232 women [64%]; median [interquartile range] age, 61 years [18-92]) were included in the study. All the patients had advanced pulmonary hypertension at baseline. Eightyfive patients (23%) suffered from COVID19. Seven of them (8%), all of whom were unvaccinated, died of COVID19. The unvaccinated patients suffered from COVID19 more often than the vaccinated ones (46% vs 9%; P <0.001). As many as 31% of the PAH/CTEPH patients with COVID19 needed hospitalization, in 8% of cases in the intensive care unit. Age equal to or above 65 years and severe pulmonary hypertension defined as a World Health Organization functional class 3 or 4 were associated with severe COVID19 in the PAH/CTEPH patients. CONCLUSIONS: The vaccinated PAH/CTEPH patients suffered from COVID19 less frequently than the unvaccinated ones. The mortality rate and hospitalization due to COVID19 were higher in the PAH/CTEPH patients than in the general population. All efforts should be made to convince the PAH/CTEPH patients to vaccinate against COVID19.
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COVID-19 , Hipertensión Pulmonar , Hipertensión Arterial Pulmonar , Humanos , Femenino , Persona de Mediana Edad , Anciano , Hipertensión Pulmonar/etiología , SARS-CoV-2 , Estudios de Cohortes , COVID-19/complicacionesAsunto(s)
Displasia Ventricular Derecha Arritmogénica , Enfermedad de la Válvula Aórtica Bicúspide , Enfermedades de las Válvulas Cardíacas , Humanos , Displasia Ventricular Derecha Arritmogénica/complicaciones , Displasia Ventricular Derecha Arritmogénica/diagnóstico , Válvula Aórtica/diagnóstico por imagen , Válvula Aórtica/cirugía , Enfermedades de las Válvulas Cardíacas/complicaciones , Enfermedades de las Válvulas Cardíacas/diagnóstico por imagen , Enfermedades de las Válvulas Cardíacas/cirugíaRESUMEN
Resumo Desde a primeira descrição da tetralogia de Fallot (ToF) em 1671 por Niels Stensen e em 1888 por Étienne-Louis Arthur Fallot, vários trabalhos relataram essa anomalia juntamente com suas variantes e anomalias cardiovasculares concomitantes. A artéria subclávia direita aberrante (ASDA) é a anomalia do arco aórtico mais comum. Diferentemente da artéria subclávia esquerda aberrante, a ocorrência de ASDA em pacientes com ToF só foi relatada casuisticamente. Apresentamos dois pacientes de ToF com ASDA. É importante notar que o conhecimento da coexistência das duas anomalias tem pontos muito práticos durante correções endovasculares ou cirúrgicas de defeitos cardíacos congênitos (inclusive ToF).
Abstract Since the first description of Tetralogy of Fallot (ToF) in 1671 by Niels Stensen and in 1888 by Étienne-Louis Arthur Fallot, numerous papers have reported on this anomaly, along with its variants and concomitant cardiovascular anomalies. Aberrant right subclavian artery (ARSA) is the most common anomaly of the aortic arch. Different from the left aberrant subclavian artery, occurrence of ARSA in ToF-patients has only casuistically been reported so far. The present study reports on two ToF-patients with ARSA. It is important to note that knowledge of the coexistence of both anomalies has highly practical points during surgical or endovascular corrections of congenital heart defects (including ToF).
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Insuficiencia de la Válvula Aórtica , Enfermedad de la Válvula Aórtica Bicúspide , Tetralogía de Fallot , Humanos , Tetralogía de Fallot/complicaciones , Tetralogía de Fallot/diagnóstico por imagen , Tetralogía de Fallot/cirugía , Insuficiencia de la Válvula Aórtica/cirugía , Válvula Aórtica/diagnóstico por imagen , Válvula Aórtica/cirugíaRESUMEN
Since the first description of Tetralogy of Fallot (ToF) in 1671 by Niels Stensen and in 1888 by Étienne-Louis Arthur Fallot, numerous papers have reported on this anomaly, along with its variants and concomitant cardiovascular anomalies. Aberrant right subclavian artery (ARSA) is the most common anomaly of the aortic arch. Different from the left aberrant subclavian artery, occurrence of ARSA in ToF-patients has only casuistically been reported so far. The present study reports on two ToF-patients with ARSA. It is important to note that knowledge of the coexistence of both anomalies has highly practical points during surgical or endovascular corrections of congenital heart defects (including ToF).
Desde a primeira descrição da tetralogia de Fallot (ToF) em 1671 por Niels Stensen e em 1888 por Étienne-Louis Arthur Fallot, vários trabalhos relataram essa anomalia juntamente com suas variantes e anomalias cardiovasculares concomitantes. A artéria subclávia direita aberrante (ASDA) é a anomalia do arco aórtico mais comum. Diferentemente da artéria subclávia esquerda aberrante, a ocorrência de ASDA em pacientes com ToF só foi relatada casuisticamente. Apresentamos dois pacientes de ToF com ASDA. É importante notar que o conhecimento da coexistência das duas anomalias tem pontos muito práticos durante correções endovasculares ou cirúrgicas de defeitos cardíacos congênitos (inclusive ToF).