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The optimal management of heart valve disease (HVD) is still debated and many studies are underway to identify the best time to refer patients for the most appropriate treatment strategy (either conservative, surgical or transcatheter interventions). Exercise pulmonary hypertension (PH) can be detected during exercise stress echocardiography (ESE) and has been demonstrated to have an important prognostic role in HVD, by predicting symptoms and mortality. This review article aims to provide an overview on the prognostic role of exercise PH in valvulopathies, and its possible role in the diagnostic-therapeutic algorithm for the management of HVD.
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"Athlete's heart" is a spectrum of morphological, functional, and regulatory changes that occur in people who practice regular and long-term intense physical activity. The morphological characteristics of the athlete's heart may overlap with some structural and electrical cardiac diseases that may predispose to sudden cardiac death, including inherited and acquired cardiomyopathies, aortopathies and channelopathies. Overdiagnosis should be avoided, while an early identification of underlying cardiac life-threatening disorders is essential to reduce the potential for sudden cardiac death. A step-by-step multimodality approach, including a first-line evaluation with personal and family history, clinical evaluation, 12-lead resting electrocardiography (ECG), followed by second and third-line investigations, as appropriate, including exercise testing, resting and exercise echocardiography, 24-hour ECG Holter monitoring, cardiac magnetic resonance, computed tomography, nuclear scintigraphy, or genetic testing, can be determinant to differentiate between extreme physiology adaptations and cardiac pathology. In this context, cardiovascular imaging plays a key role in detecting structural abnormalities in athletes who fall into the grey zone between physiological adaptations and a covert or early phenotype of cardiovascular disease.
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OBJECTIVE: To describe the trends in management and outcomes of patients with acute type B aortic dissection in the International Registry of Acute Aortic Dissection. METHODS: From 1996 - 2022, 3 908 patients were divided into similar sized quartiles (T1, T2, T3, and T4). In hospital outcomes were analysed for each quartile. Survival rates following admission were compared using Kaplan-Meier analyses with Mantel-Cox Log rank tests. RESULTS: Endovascular treatment increased from 19.1% in T1 to 37.2% in T4 (ptrend < .001). Correspondingly, medical therapy decreased from 65.7% in T1 to 54.0% in T4 (ptrend < .001), and open surgery from 14.8% in T1 to 7.0% in T4 (ptrend < .001). In hospital mortality decreased in the overall cohort from 10.7% in T1 to 6.1% in T4 (ptrend < .001), as well as in medically, endovascularly and surgically treated patients (ptrend = .017, .033, and .011, respectively). Overall post-admission survival at three years increased (T1: 74.8% vs. T4: 77.3%; p = .006). CONCLUSION: Considerable changes in the management of acute type B aortic dissection were observed over time, with a significant increase in the use of endovascular treatment and a corresponding reduction in open surgery and medical management. These changes were associated with a decreased overall in hospital and three year post-admission mortality rate among quartiles.
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The pathogenesis of complex diseases such as pulmonary arterial hypertension (PAH) is entirely rooted in changes in the expression of some vasoactive factors. These play a significant role in the onset and progression of the disease. Indeed, PAH has been associated with pathophysiologic alterations in vascular function. These are often dictated by increased oxidative stress and impaired modulation of the nitric oxide (NO) pathway. NO reduces the uncontrolled proliferation of vascular smooth muscle cells that leads to occlusion of vessels and an increase in pulmonary vascular resistances, which is the mainstay of PAH development. To date, two classes of NO-pathway modulating drugs are approved for the treatment of PAH: the phosphodiesterase-5 inhibitors (PD5i), sildenafil and tadalafil, and the soluble guanylate cyclase activator (sGC), riociguat. Both drugs provide considerable improvement in exercise capacity and pulmonary hemodynamics. PD5i are the recommended drugs for first-line PAH treatment, whereas sGCs are also the only drug approved for the treatment of resistant or inoperable chronic thromboembolic pulmonary hypertension. In this review, we will focus on the current information regarding the nitric oxide pathway and its modulation in PAH.
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Hipertensión Arterial Pulmonar , Humanos , Hipertensión Arterial Pulmonar/tratamiento farmacológico , Óxido Nítrico , Hipertensión Pulmonar Primaria Familiar , Inhibidores de Fosfodiesterasa 5/farmacología , Inhibidores de Fosfodiesterasa 5/uso terapéutico , Citrato de Sildenafil/farmacología , Citrato de Sildenafil/uso terapéutico , Guanilil Ciclasa SolubleRESUMEN
CHARGE syndrome (CS) is a rare genetic disease that affects many areas of the body. The aim of the present systematic review was to evaluate the prevalence and types of congenital heart diseases (CHDs) in CS and their impact on clinical outcome. A systematic review from 1981 to September 2022 was conducted. Clinical studies that reported the association between CS and CHDs were identified, including a case report of a rare congenital anomaly of the aortic arch (AA) with persistent fifth aortic arch (PFAA). Demographic, clinical and outcome data were extracted and analyzed. Sixty-eight studies (44 case reports and 24 case series; n=943 CS patients) were included. The prevalence of CHDs was 76.6%, patent ductus arteriosus (PDA) 26%, ventricular (VSD) 21%, atrial septal defects (ASD) 18%, tetralogy of Fallot 11%, aortic abnormalities 24%. PFAA has not been previously reported in CS. Cardiac surgery was performed in more than half of CS patients (150/242, 62%). In-hospital mortality rate was about 9.5% (n=86/900) in case series studies and 12% (n=5/43) in case reports, including cardiovascular (CV) and non-CV causes. CHDs and feeding disorders associated with CS may have a substantial impact on prognosis. CHDs were usually associated with CS and represent important causes of morbidity and mortality. PFAA, although rare, may also be present. The prognosis is highly dependent on the presence of cardiac and non-cardiac developmental abnormalities. Further studies are needed to better identify the main causes of the long-term outcome of CS patients.
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Anabolic deficiencies play a pivotal role in left-sided heart failure. Little is known about their impact on idiopathic pulmonary arterial hypertension (iPAH). Therefore, the aim of this study was to assess the impact of multiple hormone-metabolic deficiencies on clinical features and outcomes in idiopathic pulmonary arterial hypertension. We have demonstrated that the assessment of anabolic hormone levels in patients with iPAH allows the identification of a subpopulation with worse exercise capacity, pulmonary hemodynamics, right ventricular size, and function generating the hypothesis about the potential role of hormonal replacement therapy. These data should be confirmed by larger studies.
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Insuficiencia Cardíaca , Humanos , Hipertensión Pulmonar Primaria Familiar , HemodinámicaRESUMEN
Pulmonary embolism (PE) is a very common clinical entity with clinical symptoms that range from no symptom to complete hemodynamic collapse, sometimes with similar-appearing clot burden on computed tomographic pulmonary angiogram. Given highly variable clinical presentation, the authors wanted to investigate if there is clinical correlation based on the age of a clot with microscopic examination to clinical presentation. Thirteen thrombectomy aspirates from patients with an acute PE were microscopically analyzed. The goal was to age the thrombus based on histologic features and correlate it to clinical course.
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Embolia Pulmonar , Trombosis , Humanos , Embolia Pulmonar/diagnóstico por imagen , Embolia Pulmonar/cirugía , Embolectomía , Trombectomía/métodos , Trombosis/diagnóstico por imagen , Enfermedad AgudaRESUMEN
During the sixth World Symposium on Pulmonary Hypertension, the threshold of mean pulmonary arterial pressure (mPAP) for the definition of pulmonary hypertension (PH) has been lowered to a value of greater than 20 mmHg, measured by means of right heart catheterization at rest. In this review, we aim at describing the impact of the new definition of PH, analyzing the available data from the latest scientific literature concerning subjects with mPAP between 21 and 24 mmHg (defined as "mildly elevated PH"), discussing the impact of the new threshold for mPAP in the clinical practice, and highlighting the new perspectives in this field.
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Hipertensión Pulmonar , Humanos , Hipertensión Pulmonar/diagnóstico , Cateterismo CardíacoRESUMEN
In recent years, several observations reported that intolerance of physical exertion and other cardinal symptoms in heart failure (HF) are closely related to the functionality of the right ventricular (RV), regardless of left heart. It has been demonstrated that the RV dysfunction complicates the course, aggravates the quality of life, and increases the mortality of HF patients. The present review is aimed to report tips physicians about the current therapeutic management of right HF during acute stage and chronic phase, shedding light on the RV and its failure and providing physicians with essential information for everyday clinical practice.
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Insuficiencia Cardíaca , Disfunción Ventricular Derecha , Humanos , Calidad de Vida , Insuficiencia Cardíaca/complicaciones , Insuficiencia Cardíaca/terapia , Disfunción Ventricular Derecha/terapia , Disfunción Ventricular Derecha/complicaciones , Ventrículos CardíacosRESUMEN
BACKGROUND: Findings from the T.O.S.CA. Registry recently reported that patients with concomitant chronic heart failure (CHF) and impairment of insulin axis (either insulin resistance-IR or diabetes mellitus-T2D) display increased morbidity and mortality. However, little information is available on the relative impact of IR and T2D on cardiac structure and function, cardiopulmonary performance, and their longitudinal changes in CHF. METHODS: Patients enrolled in the T.O.S.CA. Registry performed echocardiography and cardiopulmonary exercise test at baseline and at a patient-average follow-up of 36 months. Patients were divided into three groups based on the degree of insulin impairment: euglycemic without IR (EU), euglycemic with IR (IR), and T2D. RESULTS: Compared with EU and IR, T2D was associated with increased filling pressures (E/e'ratio: 15.9 ± 8.9, 12.0 ± 6.5, and 14.5 ± 8.1 respectively, p < 0.01) and worse right ventricular(RV)-arterial uncoupling (RVAUC) (TAPSE/PASP ratio 0.52 ± 0.2, 0.6 ± 0.3, and 0.6 ± 0.3 in T2D, EU and IR, respectively, p < 0.05). Likewise, impairment in peak oxygen consumption (peak VO2) in TD2 vs EU and IR patients was recorded (respectively, 15.8 ± 3.8 ml/Kg/min, 18.4 ± 4.3 ml/Kg/min and 16.5 ± 4.3 ml/Kg/min, p < 0.003). Longitudinal data demonstrated higher deterioration of RVAUC, RV dimension, and peak VO2 in the T2D group (+ 13% increase in RV dimension, - 21% decline in TAPSE/PAPS ratio and - 20% decrease in peak VO2). CONCLUSION: The higher risk of death and CV hospitalizations exhibited by HF-T2D patients in the T.O.S.CA. Registry is associated with progressive RV ventricular dysfunction and exercise impairment when compared to euglycemic CHF patients, supporting the pivotal importance of hyperglycaemia and right chambers in HF prognosis. Trial registration ClinicalTrials.gov identifier: NCT023358017.
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Diabetes Mellitus Tipo 2 , Insuficiencia Cardíaca , Insulinas , Disfunción Ventricular Derecha , Diabetes Mellitus Tipo 2/complicaciones , Prueba de Esfuerzo/métodos , Humanos , Sistema de Registros , Volumen Sistólico , Disfunción Ventricular Derecha/diagnóstico por imagen , Disfunción Ventricular Derecha/etiología , Función Ventricular DerechaRESUMEN
Mitral valve prolapse (MVP) is the most frequent valvulopathy with a prevalence of 1.2%-2.4% in general population and it is characterized by a benign course. Although it can be associated with some complications, ventricular arrhythmias (VA) and sudden cardiac death (SCD) as ultimate expressions, are the most worrying. The estimated risk of SCD in MVP is between 0.2% and 1.9% per year including both MVP patients with left ventricular (LV) dysfunction due to severe MR and MVP patients without significant MR. The latter ones constitute a particular phenotype called "malignant MVP" characterized by bileaflet myxomatous prolapse, ECG repolarization abnormalities and complex VAs (c-VAs) with polymorphic/right bundle branch block morphology (RBBB) and LV fibrosis of the papillary muscles (PMs) and inferobasal wall secondary to mechanical stretching visualized as late gadolinium enhancement (LGE) areas by cardiac magnetic resonance (CMR). In MVP, the first diagnostic approach is transthoracic echocardiography (TTE) that defines the presence of mitral annular disjunction (MAD) which seems to be associated with "arrhythmic MVP" (AMVP). From an ECG point of view, AMVP is characterized by frequent premature ventricular contractions (PVCs) arising from one or both PMs, fascicular tissue, and outflow tract, as well as by T-wave inversion in the inferolateral leads. The aim of the present paper is to describe TTE red flags that could identify MVP patients at high risk to develop complex arrhythmias as supported by the corresponding findings of LGE-CMR and anatomy studies. TTE could be a co-partner in phenotyping high-risk arrhythmic MVP patients.
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Prolapso de la Válvula Mitral , Arritmias Cardíacas/complicaciones , Arritmias Cardíacas/diagnóstico por imagen , Bloqueo de Rama/complicaciones , Medios de Contraste , Muerte Súbita Cardíaca/epidemiología , Muerte Súbita Cardíaca/etiología , Muerte Súbita Cardíaca/patología , Ecocardiografía , Gadolinio , Humanos , Prolapso de la Válvula Mitral/complicaciones , Prolapso de la Válvula Mitral/diagnóstico por imagen , Músculos Papilares/diagnóstico por imagen , Músculos Papilares/patología , FenotipoRESUMEN
AIMS: This prospective, randomized, controlled, multicentre study aimed to evaluate efficacy and safety of exercise training in patients with pulmonary arterial (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH). METHODS AND RESULTS: For the first time a specialized PAH/CTEPH rehabilitation programme was implemented in 11 centres across 10 European countries. Out of 129 enrolled patients, 116 patients (58 vs. 58 randomized into a training or usual care control group) on disease-targeted medication completed the study [85 female; mean age 53.6 ± 12.5 years; mean pulmonary arterial pressure 46.6 ± 15.1 mmHg; World Health Organization (WHO) functional class II 53%, III 46%; PAH n = 98; CTEPH n = 18]. Patients of the training group performed a standardized in-hospital rehabilitation with mean duration of 25 days [95% confidence interval (CI) 17-33 days], which was continued at home. The primary endpoint, change of 6-min walking distance, significantly improved by 34.1 ± 8.3 m in the training compared with the control group (95% CI, 18-51 m; P < 0.0001). Exercise training was feasible, safe, and well-tolerated. Secondary endpoints showed improvements in quality of life (short-form health survey 36 mental health 7.3 ± 2.5, P = 0.004), WHO-functional class (training vs. control: improvement 9:1, worsening 4:3; χ2P = 0.027) and peak oxygen consumption (0.9 ± 0.5 mL/min/kg, P = 0.048) compared with the control group. CONCLUSION: This is the first multicentre and so far the largest randomized, controlled study on feasibility, safety, and efficacy of exercise training as add-on to medical therapy in PAH and CTEPH. Within this study, a standardized specialized training programme with in-hospital start was successfully established in 10 European countries.
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Hipertensión Pulmonar , Adulto , Anciano , Enfermedad Crónica , Europa (Continente) , Ejercicio Físico , Tolerancia al Ejercicio , Femenino , Humanos , Hipertensión Pulmonar/terapia , Masculino , Persona de Mediana Edad , Estudios Prospectivos , Calidad de VidaRESUMEN
The interaction between the implanter team and the imager team is critical to the success of transcatheter native mitral valve replacement (TMVR), a novel interventional procedure in the therapeutic arsenal for mitral regurgitation. This imaging scenario necessitates the addition of a new dedicated professional figure, dubbed "the interventional imager," with specific expertise in structural heart disease procedures. As its clinical application grows, knowledge of the various imaging modalities used in the TMVR procedure is required for the interventional imager and beneficial for the interventional implanter team. The purpose of this review is to describe the key steps of the procedural imaging pathway in TMVR using the Tendyne mitral valve system, with an emphasis on echocardiography. Pre-procedure cardiac multi-modality imaging screening and planning for TMVR can determine patient eligibility based on anatomic features and measurements, provide measurements for appropriate valve sizing, plan/simulate the access site, catheter/sheath trajectory, and pros- thesis positioning/orientation for correct deployment and predict the risks of potential procedural complications and their likelihood of success. Step-by-step echocardiographic TMVR intraoperative guidance includes: apical access assessment; support for catheter/sheath localization, trajectory and positioning, valve positioning and clocking; post deployment: correct clocking; hemodynamic assessment; detection of perivalvular leakage; obstruction of the left ventricular outlet tract; complications. Knowledge of the multimodality imaging pathway is essential for interventional imagers and critical to the procedure's success.
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Implantación de Prótesis de Válvulas Cardíacas , Prótesis Valvulares Cardíacas , Insuficiencia de la Válvula Mitral , Humanos , Válvula Mitral/diagnóstico por imagen , Válvula Mitral/cirugía , Implantación de Prótesis de Válvulas Cardíacas/métodos , Cateterismo Cardíaco/métodos , Resultado del Tratamiento , Insuficiencia de la Válvula Mitral/diagnóstico por imagen , Insuficiencia de la Válvula Mitral/cirugía , EcocardiografíaRESUMEN
Takotsubo syndrome is an acute reversible heart failure syndrome, most frequently seen in postmenopausal women and precipitated generally by significant emotional stress or physical illness. A sudden sympathetic activation seems to play a key role in the pathophysiology, but growing evidence is emerging about the role of inflammation in the subacute and chronic phases. An incidence of life-threatening complications occurring in the acute phase and at long-term follow-up has been demonstrated, comparable with the acute coronary syndrome. Multimodality imaging could be useful to stratify in-hospital and long-term prognosis. The efficacy of specific medical treatments in long-term follow-up should be investigated.
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Cardiomiopatía de Takotsubo , Femenino , Humanos , Pronóstico , Síndrome , Cardiomiopatía de Takotsubo/diagnóstico , Cardiomiopatía de Takotsubo/epidemiología , Cardiomiopatía de Takotsubo/etiologíaRESUMEN
Fabry disease (FD, OMIM 301500) is an X-linked lysosomal storage disease caused by pathogenic variants in the GLA gene. Cardiac involvement is common in FD and is responsible for impaired quality of life and premature death. The classic cardiac involvement is a nonobstructive form of hypertrophic cardiomyopathy, usually manifesting as concentric left ventricular hypertrophy, with subsequent arrhythmogenic intramural fibrosis. Treatment of patients with FD should be directed to prevent the disease progression to irreversible organ damage and organ failure. The aim of this review is to describe the current state of knowledge regarding cardiovascular involvement in FD, focusing on clinical and instrumental features, cardiovascular management, and targeted therapy.
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Cardiomiopatía Hipertrófica , Enfermedad de Fabry , Terapia de Reemplazo Enzimático , Enfermedad de Fabry/complicaciones , Enfermedad de Fabry/diagnóstico , Enfermedad de Fabry/genética , Humanos , Hipertrofia Ventricular Izquierda , Calidad de VidaRESUMEN
Transthyretin cardiac amyloidosis (ATTR-CA) is a systemic disorder resulting from the extracellular deposition of amyloid fibrils of misfolded transthyretin protein in the heart. ATTR-CA is a life-threatening disease, which can be caused by progressive deposition of wild type transthyretin (wtATTR) or by aggregation of an inherited mutated variant of transthyretin (mATTR). mATTR Is a rare condition transmitted in an autosomal dominant manner with incomplete penetrance, causing heterogenous phenotypes which can range from predominant neuropathic involvement, predominant cardiomyopathy, or mixed. Diagnosis of ATTR-CA is complex and requires integration of different imaging tools (echocardiography, bone scintigraphy, magnetic resonance) with genetics, clinical signs, laboratory tests, and histology. In recent years, new therapeutic agents have shown good efficacy and impact on survival and quality of life in this subset of patients, nevertheless patients affected by ATTR-CA may still carry an unfavorable prognosis, thus highlighting the need for new therapies. This review aims to assess cardiovascular involvement, diagnosis, and management of patients affected by ATTR-CA.
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Neuropatías Amiloides Familiares , Cardiomiopatías , Neuropatías Amiloides Familiares/genética , Cardiomiopatías/genética , Corazón , Humanos , Prealbúmina/genética , Calidad de VidaRESUMEN
Background andObjectives: Current guidelines on cardiac rehabilitation (CR) suggest moderate-intensity physical activity after acute coronary syndrome (ACS). Recent report have shown that high-intensity interval training (HIIT) could be more effective than moderate-intensity continuous training (MCT) in improving cardiac performance. Our aim was to analyze the effects of HIIT protocol after ACS on advanced echocardiographic parameters of myocardial function. Materials and Methods: In total, 75 patients with recent ACS, with or without ST segment elevation, were enrolled and compared with a control group of 50 age- and sex-comparable healthy subjects. Patients were randomized to perform a MCT training or HIIT-based rehabilitation program. A complete echocardiographic evaluation, including left ventricular (LV) and left atrial (LA) global longitudinal strain (GLS) and myocardial work (MW) through speckle-tracking analysis, was performed for all patients, before and after cardiac rehabilitation training. A cardiopulmonary exercise testing (CPET) was also performed at the end of the rehabilitation program. Results: Patients who followed the HIIT rehabilitation program showed improved LV diastolic function compared to the MCT group (E/e': 3.4 ± 3.1 vs. 6.4 ± 2.8, respectively, p < 0.01). Similarly, LV systolic function showed significant improvement in the group of patients performing HIIT (ejection fraction: 53.1 ± 6.4 vs. 52.3 ± 5.4%, p < 0.01; GLS: −17.8 ± 3.8 vs. −15.4 ± 4.3, p < 0.01). In addition, LA strain was improved. MW efficiency was also increased in the HIIT group (91.1 ± 3.3 vs. 87.4 ± 4.1%, p < 0.01), and was closely related to peak effort measurements expressed in peak VO2 by CPET. Conclusions: In patients with recent ACS, the HIIT rehabilitation program determined reverse cardiac remodeling, with the improvement of diastolic and systolic function, assessed by standard echocardiography. In addition, cardiac deformation index as GLS, LA strain and MW efficiency improved significantly after HIIT, and were associated with functional capacity during effort.
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Síndrome Coronario Agudo , Entrenamiento de Intervalos de Alta Intensidad , Ejercicio Físico , Atrios Cardíacos , Entrenamiento de Intervalos de Alta Intensidad/métodos , Humanos , Miocardio , Ensayos Clínicos Controlados Aleatorios como AsuntoRESUMEN
The 143 low- and middle-income countries (LMICs) of the world constitute 80% of the world's population or roughly 5.86 billion people with much variation in geography, culture, literacy, financial resources, access to health care, insurance penetration, and healthcare regulation. Unfortunately, their burden of cardiovascular disease in general and acute ST-segment-elevation myocardial infarction (STEMI) in particular is increasing at an unprecedented rate. Compounding the problem, outcomes remain suboptimal because of a lack of awareness and a severe paucity of resources. Guideline-based treatment has dramatically improved the outcomes of STEMI in high-income countries. However, no such focused recommendations exist for LMICs, and the unique challenges in LMICs make directly implementing Western guidelines unfeasible. Thus, structured solutions tailored to their individual, local needs, and resources are a vital need. With this in mind, a multicountry collaboration of investigators interested in LMIC STEMI care have tried to create a consensus document that extracts transferable elements from Western guidelines and couples them with local realities gathered from expert experience. It outlines general operating principles for LMICs focused best practices and is intended to create the broad outlines of implementable, resource-appropriate paradigms for management of STEMI in LMICs. Although this document is focused primarily on governments and organizations involved with improvement in STEMI care in LMICs, it also provides some specific targeted information for the frontline clinicians to allow standardized care pathways and improved outcomes.
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Consenso , Países en Desarrollo/economía , Recursos en Salud/economía , Pobreza/economía , Infarto del Miocardio con Elevación del ST/economía , Infarto del Miocardio con Elevación del ST/epidemiología , Servicios Médicos de Urgencia/economía , Servicios Médicos de Urgencia/normas , Personal de Salud/economía , Personal de Salud/normas , Recursos en Salud/normas , Humanos , Intervención Coronaria Percutánea/economía , Intervención Coronaria Percutánea/normas , Guías de Práctica Clínica como Asunto/normas , Infarto del Miocardio con Elevación del ST/terapia , Terapia Trombolítica/economía , Terapia Trombolítica/normasRESUMEN
BACKGROUND: Iron deficiency affects up to 50% of patients with pulmonary arterial hypertension (PAH) but iron markers such as ferritin and serum iron are confounded by several non-disease related factors like acute inflammation and diet. The aim of this study was to identify a new marker for iron deficiency and clinical outcome in PAH patients. METHODS: In this single-center, retrospective study we assessed indicators of iron status and clinical parameters specifying the time to clinical worsening (TTCW) and survival in PAH patients at time of initial diagnosis and at 1-year follow-up using univariable and multivariable analysis. RESULTS: In total, 150 patients were included with an invasively confirmed PAH and complete data on iron metabolism. The proportion of hypochromic erythrocytes > 2% at initial diagnosis was identified as an independent predictor for a shorter TTCW (p = 0.0001) and worse survival (p = 0.002) at initial diagnosis as well as worse survival (p = 0.016) at 1-year follow-up. Only a subset of these patients (64%) suffered from iron deficiency. Low ferritin or low serum iron neither correlated with TTCW nor survival. Severe hemoglobin deficiency at baseline was significantly associated with a shorter TTCW (p = 0.001). CONCLUSIONS: The presence of hypochromic erythrocytes > 2% was a strong and independent predictor of mortality and shorter TTCW in this cohort of PAH patients. Thus, it can serve as a valuable indicator of iron homeostasis and prognosis even in patients without iron deficiency or anemia. Further studies are needed to confirm the results and to investigate therapeutic implications.
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Eritrocitos/patología , Hemoglobinas/metabolismo , Hipertensión Arterial Pulmonar/sangre , Biomarcadores/sangre , Eritrocitos/metabolismo , Femenino , Estudios de Seguimiento , Humanos , Masculino , Persona de Mediana Edad , Pronóstico , Hipertensión Arterial Pulmonar/diagnóstico , Estudios RetrospectivosRESUMEN
PURPOSE: This study was a quality-control study of resting and exercise Doppler echocardiography (EDE) variables measured by 19 echocardiography laboratories with proven experience participating in the RIGHT Heart International NETwork. METHODS: All participating investigators reported the requested variables from ten randomly selected exercise stress tests. Intraclass correlation coefficients (ICC) were calculated to evaluate the inter-observer agreement with the core laboratory. Inter-observer variability of resting and peak exercise tricuspid regurgitation velocity (TRV), right ventricular outflow tract acceleration time (RVOT Act), tricuspid annular plane systolic excursion (TAPSE), tissue Doppler tricuspid lateral annular systolic velocity (S'), right ventricular fractional area change (RV FAC), left ventricular outflow tract velocity time integral (LVOT VTI), mitral inflow pulsed wave Doppler velocity (E), diastolic mitral annular velocity by TDI (e') and left ventricular ejection fraction (LVEF) were measured. RESULTS: The accuracy of 19 investigators for all variables ranged from 99.7 to 100%. ICC was > 0.90 for all observers. Inter-observer variability for resting and exercise variables was for TRV = 3.8 to 2.4%, E = 5.7 to 8.3%, e' = 6 to 6.5%, RVOT Act = 9.7 to 12, LVOT VTI = 7.4 to 9.6%, S' = 2.9 to 2.9% and TAPSE = 5.3 to 8%. Moderate inter-observer variability was found for resting and peak exercise RV FAC (15 to 16%). LVEF revealed lower resting and peak exercise variability of 7.6 and 9%. CONCLUSIONS: When performed in expert centers EDE is a reproducible tool for the assessment of the right heart and the pulmonary circulation.