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1.
Cardiol Young ; 33(11): 2185-2189, 2023 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-36601954

RESUMEN

BACKGROUND: Despite advances in medical care, we still come across pregnancy in Eisenmenger syndrome. Eisenmenger syndrome represents the severe end of the spectrum for disease in pulmonary artery hypertension associated with CHD. Due to very high maternal and perinatal morbidity and mortality, pregnancy is contraindicated among these women. Current guidelines also recommend that the women who become pregnant should opt for early termination of pregnancy. Here, we present a case series of 11 women of Eisenmenger syndrome and their pregnancy outcome. METHODS: It was a retrospective analysis of 12 pregnancies among 11 women with Eisenmenger syndrome who were managed in a tertiary care referral centre of Northern India. RESULTS: The mean age of these women was 28 ± 4 years (range 22 to 36 years). Almost 80% of them (9/11) were diagnosed with Eisenmenger syndrome during pregnancy. The commonest cardiac lesion was Ventricular Septal defect (54.5%) followed by Atrial Septal defect (27.3%) and Patent Ductus arteriosus (9.1%). Only three women opted for medical termination of pregnancy, rest eight continued the pregnancy or presented late. Pregnancy complications found include pre-eclampsia (50%), abruption (22%), and fetal growth retardation (62.5%). There were three maternal deaths (mortality rate 27%) in postpartum period. CONCLUSION: This case series highlights the delay in diagnosis and treatment of CHD despite improvement in medical care. Women with Eisenmenger syndrome require effective contraception, preconceptional counselling, early termination of pregnancy, and multidisciplinary care.


Asunto(s)
Complejo de Eisenmenger , Defectos del Tabique Interventricular , Embarazo , Humanos , Femenino , Adulto Joven , Adulto , Complejo de Eisenmenger/complicaciones , Complejo de Eisenmenger/epidemiología , Complejo de Eisenmenger/diagnóstico , Estudios Retrospectivos , Centros de Atención Terciaria , Defectos del Tabique Interventricular/complicaciones , Resultado del Embarazo
2.
Int J Cardiol ; 374: 35-41, 2023 03 01.
Artículo en Inglés | MEDLINE | ID: mdl-36526021

RESUMEN

BACKGROUND: To explore the outcomes of mothers with Eisenmenger syndrome (ES) and their offspring. METHODS: Pregnant women with ES admitted to the Beijing Anzhen Hospital between 2010 and 2019 were retrospectively analyzed and followed up. RESULTS: Forty-two parturient women with ES were recruited, with an average age of 26.7 years (standard deviation [SD], ±4.0 years). The average gestational age was 33.7 weeks (SD, ±2.5 weeks). The average percutaneous oxygen saturation was 84.1 (±9.2), and 40 (95.2%) had caesarean delivery. The average pulmonary artery systolic pressure was 107.5 mmHg (SD, ±20.3 mmHg). Twelve (28.6%) women experienced pulmonary hypertensive crisis; 11 (26.2%) of these women died. Regarding the offspring, the average fetal weight was 1778.1 g (SD, ±555.3 g), six (14.3%) died, and congenital heart disease was diagnosed in three (7.1%). There were significant differences in age, gestational age, percutaneous oxygen saturation, Apgar score, and heart failure between the maternal death and non-death groups (P < 0.05). Death was mainly related to pulmonary hypertensive crisis and heart failure. CONCLUSIONS: We recommend pregnancy termination if ES occurs during early pregnancy; however, patients should be informed of the risks if it occurs during late pregnancy. Multidisciplinary cooperation should be strengthened to improve the prognosis of the mothers and their offspring.


Asunto(s)
Complejo de Eisenmenger , Insuficiencia Cardíaca , Hipertensión Pulmonar , Embarazo , Femenino , Humanos , Adulto , Lactante , Masculino , Resultado del Embarazo/epidemiología , Complejo de Eisenmenger/diagnóstico , Complejo de Eisenmenger/epidemiología , Estudios Retrospectivos , Hipertensión Pulmonar/diagnóstico
3.
Pediatr Cardiol ; 43(8): 1848-1856, 2022 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-35522268

RESUMEN

Eisenmenger syndrome is a life-threatening complication of congenital heart defects (CHD). Since Eisenmenger syndrome among children of repaired CHD is rare, very few studies have had the necessary data to investigate its distribution in children. The current study used data collected in rural China to investigate the prevalence of Eisenmenger syndrome in children with unrepaired CHD. Data were from the 2006 to 2016 patient medical records of China California Heart Watch, which is a traveling cardiology clinic in Yunnan Province, China. Patients were included if they (1) aged 18 or below, (2) had CHD(s), and (3) the defect was not repaired by the time of the clinic visit. The prevalence of Eisenmenger syndrome was calculated in each age and defect group. Using logistic regression models, we tested whether oxygen saturation, Down syndrome, sex, and age were significantly associated with Eisenmenger syndrome. Of the 1301 study participants, ventricular septum defect (VSD), atrial septal defect (ASD), and patent ductus arteriosus (PDA) were the most common CHD. About one-sixth of the patients had pulmonary hypertension and 1.5% had Eisenmenger syndrome. The percentages of Eisenmenger syndrome were 1.8% in VSD patients, 0 in ASD patients, and 0.9% in PDA patients. Patients in the age group between 15 and 18 years had the highest percentages of Eisenmenger syndrome (11.5%). Age and presence of Down syndrome were significantly associated with the presence of Eisenmenger syndrome. Our finding highlights the importance of early detection and correction of CHD.


Asunto(s)
Síndrome de Down , Conducto Arterioso Permeable , Complejo de Eisenmenger , Cardiopatías Congénitas , Defectos del Tabique Interatrial , Defectos del Tabique Interventricular , Niño , Humanos , Complejo de Eisenmenger/complicaciones , Complejo de Eisenmenger/epidemiología , Síndrome de Down/complicaciones , China/epidemiología , Cardiopatías Congénitas/complicaciones , Cardiopatías Congénitas/epidemiología , Defectos del Tabique Interventricular/cirugía , Defectos del Tabique Interatrial/complicaciones , Conducto Arterioso Permeable/complicaciones
4.
J Pregnancy ; 2021: 3248850, 2021.
Artículo en Inglés | MEDLINE | ID: mdl-34616573

RESUMEN

OBJECTIVES: This study is aimed at evaluating the maternal and perinatal characteristics and pregnancy outcomes of ES. Material and Methods. This is a retrospective cohort study of pregnancy with Eisenmenger syndrome (ES) in Dr. Soetomo Hospital from January 2018 to December 2019. Total sampling size was obtained. We collected all baseline maternal-perinatal characteristic data, cardiac status, and pregnancy outcomes as primary outcomes. The maternal death cases were also evaluated, and we compared characteristics based on defect size (< or >3 cm). RESULTS: During study periods, we collected 18 cases with ES from a total of 152 pregnancies with heart disease. The underlying heart disease type includes atrial septal defect (ASD), ventricle septal defect (VSD), and patent ductus arteriosus (PDA). All cases suffered pulmonary hypertension (PH), 3 cases moderate, and 15 cases as severe. 94% of cases fall into heart failure (DC FC NYHA III-IV) during treatment. The majority of cases are delivered by cesarean section (88.9%). Pregnancy complications found include preterm birth (78%), low birthweight (94%), intrauterine growth restriction (55%), oligohydramnios (16%), severe preeclampsia (33%), and placenta previa (5.5%). Large defect group has an older maternal ages (30.18 ± 4.60 vs. 24.15 ± 2.75; p = 0.002), higher clinical sign (100 vs. 40%, p = 0.003), and higher preterm delivery rate (100% vs. 69%, p = 0.047) compared to small defect groups. The R to L or bidirectional shunt is significantly higher at the large defect group (13 vs. 5 cases, p = 0.006, 95% confidence interval: -1.156 to -0.228). There were seven maternal death cases caused by shock cardiogenic. CONCLUSIONS: Pregnancy with ES is still associated with very high maternal neonatal mortality and morbidity. The larger defect size is correlated with clinical performances and pregnancy outcomes. Effective preconception counseling is the best strategy to reduce the risk of maternal and neonatal death in ES women.


Asunto(s)
Complejo de Eisenmenger , Nacimiento Prematuro , Cesárea , Complejo de Eisenmenger/epidemiología , Femenino , Humanos , Mortalidad Infantil , Recién Nacido , Morbilidad , Embarazo , Resultado del Embarazo/epidemiología , Nacimiento Prematuro/epidemiología , Estudios Retrospectivos
5.
Medicina (Kaunas) ; 57(10)2021 Sep 28.
Artículo en Inglés | MEDLINE | ID: mdl-34684068

RESUMEN

Mirror syndrome (MS) or Ballantyne's syndrome is a rare maternal condition that can be life-threatening for both mother and fetus. The condition is characterized by maternal signs and symptoms similar to those seen in preeclampsia in the setting of fetal hydrops. Despite recent advances in the field of maternal-fetal medicine, the etiopathogenesis of MS remains elusive. For patients and doctors, the COVID-19 pandemic has become an extra hurdle to overcome. The following case illustrates how patients' non-compliance associated with mirror syndrome and SARS-CoV-2 infection led to the tragic end of a 19-year-old patient. Therefore, knowledge of the signs and symptoms of mirror syndrome should always be part of the armamentarium of every obstetrician.


Asunto(s)
COVID-19 , Complejo de Eisenmenger , Adulto , Complejo de Eisenmenger/complicaciones , Complejo de Eisenmenger/epidemiología , Femenino , Humanos , Hidropesía Fetal , Pandemias , Embarazo , SARS-CoV-2 , Adulto Joven
6.
Open Heart ; 8(1)2021 04.
Artículo en Inglés | MEDLINE | ID: mdl-33883228

RESUMEN

OBJECTIVE: Adults with congenital heart disease (ACHD) may be at a higher risk of a fatal outcome in case of COVID-19. Current risk stratification among these patients relies on personal experience and extrapolation from patients with acquired heart disease. We aimed to provide an expert view on risk stratification while awaiting results from observational studies. METHODS: This study was an initiative of the EPOCH (European Collaboration for Prospective Outcome Research in Congenital Heart disease). Among nine European countries (Austria, Belgium, Denmark, France, Germany, Italy, the Netherlands, Spain and Switzerland), 24 experts from 23 tertiary ACHD centres participated in the survey. ACHD experts were asked to identify ACHD-specific COVID-19 risk factors from a list of potential outcome predictors and to estimate the risk of adverse COVID-19 outcomes in seven commonly seen patient scenarios. RESULTS: 82% of participants did not consider all ACHD patients at risk of COVID-19 related complications. There was a consensus on pulmonary arterial hypertension, Fontan physiology and cyanotic heart disease as risk factors for adverse outcomes. Among different ACHD scenarios, a patient with Eisenmenger syndrome was considered to be at the highest risk. There was a marked variability in risk estimation among the other potential outcome predictors and ACHD scenarios. CONCLUSIONS: Pulmonary arterial hypertension, Fontan palliation and cyanotic heart disease were widely considered as risk factors for poor outcome in COVID-19. However, there was a marked disparity in risk estimation for other clinical scenarios. We are in urgent need of outcome studies in ACHD suffering from COVID-19.


Asunto(s)
COVID-19 , Complejo de Eisenmenger/epidemiología , Procedimiento de Fontan/estadística & datos numéricos , Cardiopatías Congénitas , Hipertensión Arterial Pulmonar/epidemiología , Medición de Riesgo/métodos , COVID-19/diagnóstico , COVID-19/epidemiología , Europa (Continente)/epidemiología , Carga Global de Enfermedades , Cardiopatías Congénitas/clasificación , Cardiopatías Congénitas/epidemiología , Humanos , Evaluación de Resultado en la Atención de Salud/métodos , Pronóstico , Factores de Riesgo , SARS-CoV-2 , Sociedades Médicas , Encuestas y Cuestionarios
7.
Chest ; 158(5): 2097-2106, 2020 11.
Artículo en Inglés | MEDLINE | ID: mdl-32565271

RESUMEN

BACKGROUND: Thoracic transplantation is considered for patients with Eisenmenger syndrome (ES) who have refractory right ventricular failure despite optimal therapy for pulmonary arterial hypertension. This study compared the outcomes of bilateral lung transplantation (BLT) with cardiac defect repair vs combined heart-lung transplantation (HLT). RESEARCH QUESTION: This study presents an updated analysis using a US national registry to evaluate the outcomes of patients diagnosed with ES who underwent HLT or BLT with repair of cardiac defects. STUDY DESIGN AND METHODS: This study identified patients with ES who underwent thoracic transplantation from 1987 to 2018 from the United Network for Organ Sharing database. Survival curves were estimated by using the Kaplan-Meier method and were compared by using the log-rank test. RESULTS: During the study period, 442 adults with ES underwent thoracic transplantation (316 HLTs and 126 BLTs). Following BLT, overall survival 1, 5, and 10 years' posttransplant was 63.1%, 38.5%, and 30.2%, respectively. Following HLT, overall survival 1, 5, and 10 years' posttransplant was 68.0%, 47.3%, and 30.5% (P = .6). When survival analysis was stratified according to type of defect, patients with an atrial septal defect had better survival following BLT than following HLT (88.3% vs 63.2% 1 year posttransplant, P < .01; 71.1% vs 49.8% 3 years' posttransplant, P < .01; and 37.4% vs 29.9% 10 years' posttransplant, P = .08). Patients with a ventricular septal defect (VSD) exhibited better survival following HLT than following BLT (78.2% vs 49.6% 1 year posttransplant, P < .01; 55.6% vs 34.3% 5 years' posttransplant, P < .01; and 35.7% vs 26.5% 10 years' posttransplant, P = .03). The most common cause of mortality in patients with VSD undergoing BLT was cardiac ventricular failure. INTERPRETATION: This study suggests that the best transplant option for patients with VSD remains HLT, which prevents subsequent development of ventricular failure. BLT with cardiac defect repair should be considered as the first-line treatment option in patients with ES due to an uncorrected atrial septal defect. These patients can be considered to have isolated and reversible right ventricular failure akin to patients with advanced pulmonary arterial hypertension.


Asunto(s)
Complejo de Eisenmenger/cirugía , Trasplante de Corazón-Pulmón/métodos , Trasplante de Pulmón/métodos , Sistema de Registros , Receptores de Trasplantes , Adulto , Complejo de Eisenmenger/epidemiología , Femenino , Estudios de Seguimiento , Humanos , Incidencia , Masculino , Pronóstico , Estudios Retrospectivos , Tasa de Supervivencia/tendencias , Estados Unidos/epidemiología
8.
Nucl Med Commun ; 41(3): 206-211, 2020 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-31923047

RESUMEN

BACKGROUND: Compared to primary pulmonary hypertension (PPH), the right ventricular (RV) contractile function is preserved for a long time in patients with Eisenmenger syndrome and is likely the most important determinant of relatively higher survival. The differences in myocardial perfusion have been purported to explain this discrepancy. The exact prevalence of myocardial perfusion abnormalities in Eisenmenger syndrome is not known. We sought to examine the prevalence of myocardial perfusion abnormalities in patients with Eisenmenger syndrome. METHODS: In this prospective study, 20 consecutive adult patients with Eisenmenger syndrome were subjected to clinical assessment, six-minute walk test and echocardiography. Myocardial perfusion was assessed using one day stress-rest Gated Technetium-99 m Sestamibi single-photon emission computed tomography. RESULTS: Nineteen (95%) patients were in New York Heart Association functional class I or II. All patients had RV hypertrophy. Five (25%) patients had RV systolic dysfunction. Left ventricular systolic function was normal in all except in three patients. Two (10%) patients had perfusion defects in the RV and 4 (20%) patients had perfusion defects in the left ventricle (LV). CONCLUSION: Myocardial perfusion defects, both in RV and LV, occur even in asymptomatic or mildly symptomatic patients with Eisenmenger syndrome.


Asunto(s)
Circulación Coronaria , Complejo de Eisenmenger/diagnóstico por imagen , Complejo de Eisenmenger/fisiopatología , Adolescente , Adulto , Ecocardiografía , Complejo de Eisenmenger/epidemiología , Femenino , Humanos , Procesamiento de Imagen Asistido por Computador , Masculino , Prevalencia , Estudios Prospectivos , Radiofármacos , Tecnecio Tc 99m Sestamibi , Tomografía Computarizada de Emisión de Fotón Único , Función Ventricular Izquierda , Prueba de Paso , Adulto Joven
9.
Can J Cardiol ; 35(12): 1664-1674, 2019 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-31813503

RESUMEN

Eisenmenger syndrome is the most severe and extreme phenotype of pulmonary arterial hypertension associated with congenital heart disease. A large nonrestrictive systemic left-to-right shunt triggers the development of pulmonary vascular disease, progressive pulmonary arterial hypertension, and increasing pulmonary vascular resistance at the systemic level, which ultimately results in shunt reversal. Herein, we review the changing epidemiological patterns and pathophysiology of Eisenmenger syndrome. Multiorgan disease is an integral manifestation of Eisenmenger syndrome and includes involvement of the cardiac, hematological, neurological, respiratory, gastrointestinal, urinary, immunological, musculoskeletal, and endocrinological systems. Standardized practical guidelines for the assessment, management, risk stratification, and follow-up of this very fragile and vulnerable population are discussed. Multidisciplinary care is the best clinical practice. An approach to the prevention and management of a broad spectrum of complications is provided. Relevant therapeutic questions are discussed, including anticoagulation, noncardiac surgery, physical activity, transplantation, and advanced-care planning (palliative care). Advanced pulmonary arterial hypertension therapies are indicated in patients with Eisenmenger syndrome and World Health Organization functional class II or higher symptoms to improve functional capacity, quality of life, and-less well documented-survival. Specific recommendations regarding monotherapy or combination therapy are provided according to functional class and clinical response. The ultimate challenge for all care providers remains early detection and management of intracardiac and extracardiac shunts, considering that Eisenmenger syndrome is a preventable condition.


Asunto(s)
Complejo de Eisenmenger/epidemiología , Cardiopatías Congénitas/epidemiología , Cardiopatías Congénitas/terapia , Hipertensión Pulmonar/epidemiología , Guías de Práctica Clínica como Asunto , Adulto , Terapia Combinada , Comorbilidad , Complejo de Eisenmenger/diagnóstico , Complejo de Eisenmenger/terapia , Femenino , Cardiopatías Congénitas/diagnóstico , Humanos , Hipertensión Pulmonar/diagnóstico , Hipertensión Pulmonar/terapia , Comunicación Interdisciplinaria , Masculino , Enfermedades Raras , Medición de Riesgo , Análisis de Supervivencia , Poblaciones Vulnerables
10.
Clin Respir J ; 13(11): 693-699, 2019 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-31419027

RESUMEN

INTRODUCTION: Current guidelines emphasize that accurate risk stratification is important for patients with pulmonary arterial hypertension (PAH), however, few suggestions have been specified for PAH associated with congenital heart disease (PAH-CHD). OBJECTIVES: The aim of this study was to propose an accurate and simple system based on current guidelines for risk stratification in PAH-CHD patients during 12-month follow-up. METHODS: We reviewed 288 Chinese PAH-CHD patients between January 2014 and December 2016 in this retrospective cohort study. The low-risk criteria according to 2015 European Society of Cardiology guidelines and the adverse events (AEs) during follow-up were collected. The association between low-risk criteria and AEs was assessed with Cox regression, and a simplified risk stratification system was proposed. RESULTS: There were 105 PAH-CHD patients included in the final analysis. Twenty-nine patients had AEs defined as death, initiation of new or combined medication treatment, or re-hospitalisation because of the PAH worsening. Among the low-risk criteria, WHO/NYHA functional class, 6-minute walking distance (6MWD), NT-proBNP and SvO2 were significantly different between AE and AE-free groups. However, 6MWD (HR = 0.08, 95% CI: 0.03-0.19, P < 0.001) and NT-proBNP (HR = 0.35, 95% CI: 0.16-0.78, P = 0.01) were the only independent predictors of AEs in multivariable model. When taking them into a simplified system for risk stratification, the number of low-risk criteria at diagnosis discriminated the risk of AEs (P < 0.001). CONCLUSIONS: Among the low-risk criteria proposed by current guidelines, 6MWD and NT-proBNP predicted AEs independently for PAH-CHD patients. Simplified risk stratification system by taking these two parameters numerically provides accurate prognostic information in PAH-CHD patients.


Asunto(s)
Implementación de Plan de Salud/métodos , Cardiopatías Congénitas/epidemiología , Hipertensión Arterial Pulmonar/epidemiología , Medición de Riesgo/métodos , Adulto , China/epidemiología , Complejo de Eisenmenger/diagnóstico , Complejo de Eisenmenger/epidemiología , Femenino , Cardiopatías Congénitas/complicaciones , Cardiopatías Congénitas/metabolismo , Cardiopatías Congénitas/mortalidad , Humanos , Incidencia , Lactante , Masculino , Persona de Mediana Edad , Péptido Natriurético Encefálico/sangre , Oxígeno/sangre , Fragmentos de Péptidos/sangre , Guías de Práctica Clínica como Asunto , Valor Predictivo de las Pruebas , Pronóstico , Hipertensión Arterial Pulmonar/etiología , Hipertensión Arterial Pulmonar/mortalidad , Hipertensión Arterial Pulmonar/fisiopatología , Estudios Retrospectivos , Prueba de Paso/métodos
13.
Congenit Heart Dis ; 12(4): 512-519, 2017 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-28523857

RESUMEN

BACKGROUND: Patients with Eisenmenger syndrome have a shorter lifespan than the general population. A significant proportion develop arrhythmia and some, sudden death. OBJECTIVE: The aims of this study were to characterize the frequency, type and effects of arrhythmias in adult patients with Eisenmenger's syndrome and to identify risk factors for arrhythmias. METHODS: This retrospective study included patients aged ≥ 18 years of age with Eisenmenger's syndrome from three institutions. Arrhythmias were noted from electrocardiograms and Holter study reviews. RESULTS: A total of 167 patients, 96 females, 63 males (gender not available in 9 patients) were included in this study. The mean age was 38 ± 9 years (range: 18-63 years) with a majority in NYHA functional class II or III (57% and 32% respectively). Twenty-eight patients (17%) had significant tachyarrhythmia: paroxysmal supraventricular tachycardia (8 patients, 29%), atrial fibrillation (6 patients, 21%), atrial fibrillation and flutter (2 patients, 7%), nonsustained ventricular tachycardia (6 patients, 21%) and sustained ventricular tachycardia (6 patients, 21%). Among the entire study group, 26 patients (16%) were currently on antiarrhythmic therapy and 77 patients (49%) were on advanced therapies for pulmonary hypertension. Down syndrome was present in 78 patients (46%). There were 21 (13%) documented deaths, of which 8 (5%) were sudden death. Patients with arrhythmia were older [P = .01] and were more likely to have atrioventricular valvar regurgitation [Odds ratio: 4.33]. Advanced pulmonary hypertension therapy was associated with decreased all-cause mortality in logistic regression analysis [odds ratio: 0.31], while antiarrhythmic therapy was associated with sudden death [odds ratio: 6.24]. CONCLUSIONS: Arrhythmias are common among patients with Eisenmenger syndrome occurring in around 1 in 5 individuals and are associated with all-cause mortality and sudden death.


Asunto(s)
Arritmias Cardíacas/epidemiología , Muerte Súbita Cardíaca/epidemiología , Complejo de Eisenmenger/complicaciones , Medición de Riesgo/métodos , Adolescente , Adulto , Arritmias Cardíacas/diagnóstico , Arritmias Cardíacas/etiología , Complejo de Eisenmenger/epidemiología , Electrocardiografía Ambulatoria , Femenino , Estudios de Seguimiento , Humanos , Incidencia , Masculino , Persona de Mediana Edad , Estudios Retrospectivos , Factores de Riesgo , Tasa de Supervivencia/tendencias , Reino Unido/epidemiología , Adulto Joven
14.
Heart ; 103(17): 1353-1358, 2017 09.
Artículo en Inglés | MEDLINE | ID: mdl-28450553

RESUMEN

OBJECTIVE: Improved diagnostic tools, timely closure of the shunt and a better understanding of the complexity of Eisenmenger syndrome (ES) have led to improved care and treatment in tertiary centres. These may have decreased the incidence of ES and improved survival of patients with ES, although evidence is still lacking. The aim of this study was to investigate temporal changes in incidence, prevalence and mortality in patients with ES for 35 years in the Nordic region. METHODS: This was a retrospective population-based study including 714 patients with ES. Survival analysis was performed based on all-cause mortality and accounting for immortal time bias. RESULTS: The incidence of ES decreased from 2.5/million inhabitants/year in 1977 to 0.2/million inhabitants/year in 2012. Correspondingly, prevalence decreased from 24.6 to 11.9/million inhabitants. The median survival was 38.4 years, with 20-year, 40-year and 60-year survival of 72.5%, 48.4%, and 21.3%, respectively. Complex lesions and Down syndrome were independently associated with worse survival (HR 2.2, p<0.001 and HR 1.8, p<0.001, respectively). Age at death increased from 27.7 years in the period from 1977 to 1992, to 46.3 years from July 2006 to 2012 (p<0.001). CONCLUSIONS: The incidence and prevalence of ES in the Nordic region have decreased markedly during the last decades. Furthermore, the median age at death increased throughout the study period, indicating prolonged life expectancy in the ES population. However, increasing age represents decreased incidence, rather than improved survival. Nonetheless, longevity with ES is still shorter than in the background population.


Asunto(s)
Complejo de Eisenmenger/epidemiología , Predicción , Vigilancia de la Población/métodos , Sistema de Registros , Medición de Riesgo/métodos , Adulto , Causas de Muerte/tendencias , Femenino , Estudios de Seguimiento , Humanos , Incidencia , Masculino , Persona de Mediana Edad , Prevalencia , Estudios Retrospectivos , Países Escandinavos y Nórdicos/epidemiología , Tasa de Supervivencia/tendencias
15.
Ann Cardiol Angeiol (Paris) ; 65(6): 440-445, 2016 Dec.
Artículo en Francés | MEDLINE | ID: mdl-27816173

RESUMEN

Gender influences the clinical presentation and the management of some acquired cardiovascular diseases, such as coronary artery disease, resulting in different outcomes. Differences between women and men are also noticed in congenital heart disease. They are mainly related to the prevalence and severity of some congenital heart defects at birth, and in adulthood to the prognosis, incidence of Eisenmenger syndrome and risks of pregnancy. The role of gender on the risk of operative mortality of congenital heart surgery remains debated.


Asunto(s)
Cardiopatías Congénitas/epidemiología , Caracteres Sexuales , Adulto , Estudios Transversales , Complejo de Eisenmenger/diagnóstico , Complejo de Eisenmenger/epidemiología , Complejo de Eisenmenger/cirugía , Femenino , Cardiopatías Congénitas/diagnóstico , Cardiopatías Congénitas/cirugía , Humanos , Incidencia , Recién Nacido , Masculino , Embarazo , Complicaciones Cardiovasculares del Embarazo/diagnóstico , Complicaciones Cardiovasculares del Embarazo/epidemiología , Pronóstico , Tasa de Supervivencia
17.
Cardiol Young ; 26(7): 1250-9, 2016 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-26980152

RESUMEN

BACKGROUND: The aim of this study was to assess health-related quality of life in patients with pulmonary arterial hypertension associated with CHD and correlations with clinical status. METHODS: This prospective cross-sectional observational study included CHD patients with pulmonary arterial hypertension in 14 tertiary-care centres in France. We used two health-related quality of life questionnaires - SF-36 and Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR) - and one anxiety/depression Hospital Anxiety and Depression Scale (HADS) questionnaire. RESULTS: Clinical data were collected for the 208 included patients (mean age: 42.6 years, range from 15.1 to 85.8 years, 69.7% female). Most patients were in NYHA functional class II (48.1%) and III (37.5%). Patients' phenotype was classified as Eisenmenger syndrome (70.7%), pulmonary arterial hypertension associated with systemic-to-pulmonary shunts (12.0%), with small defects (3.4%), or after corrective cardiac surgery (13.9%). In total, 76.4% of the patients were receiving pulmonary arterial hypertension-specific treatments. SF-36 scores showed impairment compared with normalised data. Health-related quality of life scores were significantly lower in females than in males for most dimensions of both questionnaires and were independent of the patients' phenotype, even after gender adjustment - except for CAMPHOR functioning - but significantly depended on NYHA functional class. The Hospital Anxiety and Depression Scale (HADS) scores suggested anxiety and depression associated with increasing NYHA functional class but independent of patients' phenotype. NYHA functional class, 6-minute walk distance, HADS, gender, and recent stressful event significantly affected quality of life in the multivariate analysis. CONCLUSIONS: This study showed impairment of quality of life in a large cohort of patients with pulmonary arterial hypertension associated with CHD with both generic and specific questionnaires. NYHA functional class and HADS scores were predictive of most quality of life scores.


Asunto(s)
Ansiedad/epidemiología , Depresión/epidemiología , Complejo de Eisenmenger/complicaciones , Complejo de Eisenmenger/epidemiología , Hipertensión Pulmonar Primaria Familiar/epidemiología , Calidad de Vida , Adolescente , Adulto , Anciano , Anciano de 80 o más Años , Estudios Transversales , Femenino , Francia , Humanos , Masculino , Persona de Mediana Edad , Análisis Multivariante , Estudios Prospectivos , Escalas de Valoración Psiquiátrica , Análisis de Regresión , Encuestas y Cuestionarios , Adulto Joven
18.
Curr Opin Anaesthesiol ; 29(3): 273-81, 2016 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-26978591

RESUMEN

PURPOSE OF REVIEW: Purpose of review is to summarize and highlight recent advances in the management of pregnant patients with pulmonary hypertension. RECENT FINDINGS: Despite recent advances in the therapy of pulmonary hypertension, prognosis for pregnant patients with pulmonary hypertension remains poor with high maternal mortality. Pregnancy is still considered contraindicated in these patients. If pregnancy occurs, referral to a tertiary hospital and a multidisciplinary approach ensure the best possible outcome. All pregnant patients with pulmonary hypertension should be counseled for a termination of pregnancy. If the patient wants to continue the pregnancy despite strong recommendations for therapeutic interruption, specific pulmonary hypertension therapy has to be initiated, adjusted, and/or augmented. A close clinical follow-up of the mother throughout the entire pregnancy is of utmost importance. Elective caesarean section in week 34-36 is recommended as preferred mode of delivery, preferentially under epidural or low-dose combined spinal-epidural anesthesia. Because of an acute increase in pulmonary vascular resistance and delivery-associated acute volume overload, the immediate postpartum period carries the highest risk for acute right ventricular failure necessitating close monitoring and treatment on an ICU. SUMMARY: Anesthesiologists involved in the management of pregnant patients with pulmonary hypertension must have detailed knowledge of pathophysiological alterations in pregnancy and during birth, cardiac (patho)physiology, cardiovascular and obstetric pharmacology, hemodynamic monitoring, and echocardiography. Both regional and general anesthesia have typical adverse effects that can severely jeopardize the cardiovascular system in patients with pulmonary hypertension, and should therefore be anticipated/prevented/rapidly treated by the attending anesthesiologist.


Asunto(s)
Anestesia de Conducción/métodos , Cesárea/métodos , Complejo de Eisenmenger/fisiopatología , Hipertensión Pulmonar/fisiopatología , Complicaciones Cardiovasculares del Embarazo/fisiopatología , Anestesia de Conducción/efectos adversos , Anestésicos Generales/administración & dosificación , Anestésicos Generales/efectos adversos , Sistema Cardiovascular/efectos de los fármacos , Sistema Cardiovascular/fisiopatología , Cesárea/efectos adversos , Complejo de Eisenmenger/epidemiología , Complejo de Eisenmenger/cirugía , Femenino , Monitorización Hemodinámica/métodos , Humanos , Hipertensión Pulmonar/epidemiología , Hipertensión Pulmonar/cirugía , Trabajo de Parto/fisiología , Grupo de Atención al Paciente , Cuidados Posoperatorios/métodos , Embarazo , Complicaciones Cardiovasculares del Embarazo/epidemiología , Complicaciones Cardiovasculares del Embarazo/cirugía , Prevalencia , Centros de Atención Terciaria , Disfunción Ventricular Derecha/inducido químicamente , Disfunción Ventricular Derecha/fisiopatología
19.
Int J Cardiol ; 184: 717-723, 2015 Apr 01.
Artículo en Inglés | MEDLINE | ID: mdl-25781723

RESUMEN

BACKGROUND: Pulmonary arterial hypertension (PAH) is common in patients with congenital heart disease (CHD). Although Eisenmenger syndrome may be on decline, other types of PAH-CHD are increasing and little is known on long-term outcome of this population. We report the PAH-CHD population of Spain via a national registry with focus on long-term survival. METHODS AND RESULTS: A total of 240 consecutive patients (age 37.7 ± 14.1 years, 67.9% females) with PAH-CHD included in the REHAP registry were analysed. Patients were classified into 3 groups: 1) Eisenmenger syndrome, 2) postoperative-PAH and 3) PAH associated with small defects. Over a median follow-up time of 4.5[1.6-7.1]years, 50 patients (20.8%) died or underwent lung/heart-lung transplantation. Patients with Eisenmenger syndrome had better survival than postoperative-PAH (HR 0.1 95% CI: 0.2-0.9, p=0.048) but no advantage compared to small defects (HR 4.4, 95% CI 0.6-31.4, p=0.15). In the overall PAH-CHD population, patients in NYHA functional class III-IV had a 3-fold increased risk of death (HR 3.0, 95% CI: 1.5-5.9, p=0.001). Amongst patients with Eisenmenger syndrome, a pre-tricuspid shunt had a 2.6-fold increase risk of death (HR 2.6, 95% CI: 1.2-5.6, p=0.03). There was no significant difference in survival between patients with postoperative-PAH and patients with iPAH (HR 0.99 95% CI: 0.6-1.7, p=0.97). CONCLUSION: PAH-CHD is associated with mid to long-term mortality. Outcome relates closely to functional class, type of PAH-CHD and within the Eisenmenger cohort, with location of the shunt. Adults with postoperative-PAH have the worse prognosis in the PAH-CHD cohort, reinforcing the need for lifelong close follow-up of such patients.


Asunto(s)
Complejo de Eisenmenger/diagnóstico , Complejo de Eisenmenger/epidemiología , Hipertensión Pulmonar/diagnóstico , Hipertensión Pulmonar/epidemiología , Sistema de Registros , Adulto , Femenino , Estudios de Seguimiento , Cardiopatías Congénitas/diagnóstico , Cardiopatías Congénitas/epidemiología , Humanos , Masculino , Persona de Mediana Edad , España/epidemiología , Adulto Joven
20.
J Cardiol ; 63(4): 286-90, 2014 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-24145195

RESUMEN

BACKGROUND: The management of Eisenmenger syndrome (ES) has dramatically changed since the advent of disease-targeted therapy (DTT). However, guidelines for ES management, including DTT, have not been established. We aimed to clarify the current incidence, underlying disease, and management of ES in Japan, using a nationwide survey. METHODS: A written questionnaire was sent to members of the Japanese Society for Adult Congenital Heart Disease, through which information was obtained from 86 institutions. RESULTS: A total of 251 patients with ES (80.5% cases≥20 years of age) were followed as of February 2012; DTT was performed in 124 (49.4%) patients. Unrepaired simple anatomy was reported as an underlying condition in 165 patients (65.7%). Among patients with ES, 55 (21.9%), 128 (51%), 53 (21.1%), and 12 (4.8%) were classified into functional classes I, II, III, and IV, respectively. DTT was routinely performed at 52 (60.5%) institutions, but there were variations in the DTT therapeutic strategy at these institutions. Combined therapy was more often used than monotherapy; an endothelin receptor antagonist was the most frequently prescribed medication. There were institutional differences regarding heart failure treatment and indications for anticoagulation. Digitalis and angiotensin-converting enzyme inhibitors or angiotensin II receptor blockers were widely used, but beta-blockers were infrequently used to manage heart failure. CONCLUSIONS: This survey describes the current status, including prevalence and underlying disease, and variations in the practical management of ES in Japan. The results will help in the creation of future guidelines for ES management.


Asunto(s)
Complejo de Eisenmenger/tratamiento farmacológico , Adulto , Anciano , Antagonistas de Receptores de Angiotensina/uso terapéutico , Inhibidores de la Enzima Convertidora de Angiotensina/uso terapéutico , Anticoagulantes/uso terapéutico , Glicósidos Digitálicos/uso terapéutico , Quimioterapia Combinada , Complejo de Eisenmenger/clasificación , Complejo de Eisenmenger/epidemiología , Complejo de Eisenmenger/etiología , Antagonistas de los Receptores de Endotelina , Femenino , Humanos , Incidencia , Japón/epidemiología , Masculino , Persona de Mediana Edad , Terapia Molecular Dirigida , Guías de Práctica Clínica como Asunto , Prevalencia , Encuestas y Cuestionarios , Adulto Joven
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