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1.
Medicine (Baltimore) ; 98(36): e17044, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31490396

RESUMO

RATIONALE: A single atrium is a rare congenital heart disease (CHD) involving zero atrial septal traces and preserved intact ventricular septum and atrioventricular valves, requiring careful surgical intervention. However, developing to Eisenmenger syndrome (ES) makes the surgery complicated. Based on bidirectional cardiac shunting, vegetation easily develops in case of bacterial infection. PATIENT CONCERN AND DIAGNOSES: We reported a 35-year-old woman with a single atrium, patent ductus arteriosus, pulmonary hypertension, and ES who developed infective endocarditis on her left ventricular outflow tract and complicated cerebral abscess and who underwent challenged medical treatment. INTERVENTION: Infection was successfully controlled after 4-time change in antibiotics over 4 months. However, surgery is complicated for her. OUTCOMES: The patient presented a relatively good outcome during follow-up for >6 months. LESSONS: This case report suggests that patients with complex CHD should accept surgery therapy earlier before developing ES. It is imperative to avoid invasive interventions to prevent infectious endocarditis.


Assuntos
Abscesso Encefálico/complicações , Permeabilidade do Canal Arterial/complicações , Complexo de Eisenmenger/complicações , Endocardite/complicações , Átrios do Coração/anormalidades , Adulto , Antibacterianos/uso terapêutico , Abscesso Encefálico/diagnóstico por imagem , Abscesso Encefálico/tratamento farmacológico , Endocardite/diagnóstico por imagem , Endocardite/tratamento farmacológico , Feminino , Humanos
2.
World Neurosurg ; 130: 133-137, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-31295595

RESUMO

BACKGROUND: Eisenmenger syndrome is a rare sequela of uncorrected congenital heart disease complicated by pulmonary hypertension, from which reversal of the pathologic left-to-right cardiovascular shunt and cyanosis follow. Right-to-left shunting can lead to paradoxical cerebral emboli-increasing the risk of spontaneous or iatrogenic stroke and cerebral abscess. CASE DESCRIPTION: A 38-year-old man presented with new focal seizures due to a brain abscess. Ventricular septal defect and pulmonary hypertension were identified. Despite dexamethasone and broad-spectrum antibiotics, he developed hemiparesis and altered mental status and required emergent stereotactic abscess drainage. Despite the anesthetic hazards of Eisenmenger syndrome, the procedure was successful and the patient recovered completely. CONCLUSIONS: Noncardiac perioperative mortality in Eisenmenger syndrome is historically reported up to 19%, and risks are further increased with prolonged case duration or hypotension, mandating vigilant attention to volume status. Correspondingly, shorter- or lower-risk procedures such as stereotactic drainage are recommended. Procedures should be performed only at centers with expertise in management of Eisenmenger syndrome and cardiac-specialized anesthesiologists whenever possible. Although a conservative approach with early, aggressive medical management is preferred, operative intervention may be required in the setting of progressive deterioration and excellent postoperative outcomes are achievable.


Assuntos
Complexo de Eisenmenger/cirurgia , Cardiopatias Congênitas/cirurgia , Hipertensão Pulmonar/cirurgia , Hipotensão/cirurgia , Adulto , Abscesso Encefálico/complicações , Abscesso Encefálico/cirurgia , Complexo de Eisenmenger/complicações , Cardiopatias Congênitas/complicações , Comunicação Interventricular/complicações , Comunicação Interventricular/cirurgia , Humanos , Hipertensão Pulmonar/diagnóstico , Hipotensão/complicações , Hipotensão/diagnóstico , Masculino , Procedimentos Neurocirúrgicos
3.
Taiwan J Obstet Gynecol ; 58(2): 183-187, 2019 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-30910135

RESUMO

OBJECTIVE: The goal of the study was to clarify the risk factors for pregnancy complicated with Eisenmenger syndrome (ES). MATERIALS AND METHODS: A retrospective study was performed in 15 patients with ES who were managed throughout pregnancy at one institution from 1982 to 2013. Cases associated with congenital heart diseases other than atrial septal defect (ASD), ventricular septal defect (VSD), and patent ductus arteriosus (PDA) were excluded. RESULTS: The congenital heart diseases in ES included ASD (n = 3), VSD (n = 9), and PDA (n = 3). Ten women chose termination and 5 continued with their pregnancies. In the 5 continuation cases (PDA 1, VSD 4), worsening of cyanosis, exertional fatigue and dyspnea appeared between 25 and 30 weeks gestation and cesarean section was performed at 30 (28-33) weeks. LVEF, PaO2, and SpO2 decreased and heart rate increased significantly from before pregnancy to 25-30 weeks gestation. From before to during the pregnancy, there were no significant changes in mean PABP or pulmonary vascular resistance (PVR) in four cases with data (582-592, 885 to 868, 1280 to 1291, 1476-1522 dyn × s/cm2). PVR at conception had a negative relationship with delivery weeks. NYHA classes before, during and 1 year after pregnancy were II, III and II. In one recent case, epoprostenol and tadalafil were administered during pregnancy. CONCLUSIONS: Pregnancy with ES has a high risk due to hypooxygenation, cyanosis, and cardiac failure, which can appear as common complications as early as the 2nd trimester. Early interventions with meticulous care are required for these complications during pregnancy and delivery.


Assuntos
Complexo de Eisenmenger/terapia , Insuficiência Cardíaca/terapia , Complicações Cardiovasculares na Gravidez/terapia , Gravidez de Alto Risco , Aborto Espontâneo , Aborto Terapêutico , Adulto , Cateterismo Cardíaco , Cesárea , Permeabilidade do Canal Arterial/complicações , Ecocardiografia , Complexo de Eisenmenger/complicações , Feminino , Insuficiência Cardíaca/complicações , Comunicação Interatrial/complicações , Comunicação Interventricular/complicações , Humanos , Gravidez , Estudos Retrospectivos , Fatores de Risco
5.
Circulation ; 139(1): 51-63, 2019 01 02.
Artigo em Inglês | MEDLINE | ID: mdl-30586694

RESUMO

BACKGROUND: Eisenmenger syndrome describes congenital heart disease-associated severe pulmonary hypertension accompanied by right-to-left shunting. The multicenter, double-blind, randomized, placebo-controlled, 16-week, phase III MAESTRO study (Macitentan in Eisenmenger Syndrome to Restore Exercise Capacity) evaluated the efficacy and safety of the endothelin receptor antagonist macitentan in patients with Eisenmenger syndrome. METHODS: Patients with Eisenmenger syndrome aged ≥12 years and in World Health Organization functional class II-III were randomized 1:1 to placebo or macitentan 10 mg once daily for 16 weeks. Patients with complex cardiac defects, Down syndrome and background PAH therapy were eligible. The primary end point was change from baseline to week 16 in 6-minute walk distance. Secondary end points included change from baseline to week 16 in World Health Organization functional class. Exploratory end points included NT-proBNP (N-terminal pro-B-type natriuretic peptide) at end of treatment expressed as a percentage of baseline. In a hemodynamic substudy, exploratory end points included pulmonary vascular resistance index (PVRi) at week 16 as a percentage of baseline. RESULTS: Two hundred twenty six patients (macitentan n=114; placebo n=112) were randomized. At baseline, 60% of patients were in World Health Organization functional class II and 27% were receiving phosphodiesterase type-5 inhibitors. At week 16, the mean change from baseline in 6-minute walk distance was 18.3 m and 19.7 m in the macitentan and placebo groups (least-squares mean difference, -4.7 m; 95% confidence limit (CL), -22.8, 13.5; P=0.612). World Health Organization functional class improved from baseline to week 16 in 8.8% and 14.3% of patients in the macitentan and placebo groups (odds ratio, 0.53; 95% CL, 0.23, 1.24). NT-proBNP levels decreased with macitentan versus placebo (ratio of geometric means, 0.80; 95% CL, 0.68, 0.94). In the hemodynamic substudy (n=39 patients), macitentan decreased PVRi compared with placebo (ratio of geometric means, 0.87; 95% CL, 0.73, 1.03). The most common adverse events with macitentan versus placebo were headache (11.4 versus 4.5%) and upper respiratory tract infection (9.6 versus 6.3%); a hemoglobin decrease from baseline of ≥2 g/dL occurred in 36.0% versus 8.9% of patients. Five patients (3 macitentan; 2 placebo) prematurely discontinued treatment and 1 patient died (macitentan group). CONCLUSIONS: Macitentan did not show superiority over placebo on the primary end point of change from baseline to week 16 in exercise capacity in patients with Eisenmenger syndrome. CLINICAL TRIAL REGISTRATION: URL: https://www.clinicaltrials.gov . Unique identifier: NCT01743001.


Assuntos
Anti-Hipertensivos/uso terapêutico , Complexo de Eisenmenger/complicações , Antagonistas dos Receptores de Endotelina/uso terapêutico , Tolerância ao Exercício/efeitos dos fármacos , Hemodinâmica/efeitos dos fármacos , Hipertensão Pulmonar/tratamento farmacológico , Artéria Pulmonar/efeitos dos fármacos , Pirimidinas/uso terapêutico , Sulfonamidas/uso terapêutico , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Anti-Hipertensivos/efeitos adversos , Biomarcadores/sangue , Criança , Método Duplo-Cego , Síndrome de Down/complicações , Complexo de Eisenmenger/diagnóstico por imagem , Complexo de Eisenmenger/fisiopatologia , Antagonistas dos Receptores de Endotelina/efeitos adversos , Feminino , Humanos , Hipertensão Pulmonar/diagnóstico por imagem , Hipertensão Pulmonar/etiologia , Hipertensão Pulmonar/fisiopatologia , Masculino , Pessoa de Meia-Idade , Peptídeo Natriurético Encefálico/sangue , Fragmentos de Peptídeos/sangue , Artéria Pulmonar/fisiopatologia , Pirimidinas/efeitos adversos , Recuperação de Função Fisiológica , Sulfonamidas/efeitos adversos , Fatores de Tempo , Resultado do Tratamento , Teste de Caminhada , Adulto Jovem
7.
Eur Arch Paediatr Dent ; 19(6): 449-453, 2018 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-30196521

RESUMO

BACKGROUND: Eisenmenger syndrome (ES) is a heart cyanotic condition characterised by elevated pulmonary vascular resistance and an intra-cardiac right-to-left shunting of blood through a systemic-to-pulmonary circulation connection. Affected children usually exhibit severe hypoxia, clubbing of fingers/toes, haemoptysis, anaemia, and organ damage. CASE REPORT: During autumn 2015, the patient and her parents arrived at the paediatric dentistry clinic. The patient presented with the main complaint of generalised inflamed gingival tissues, severely protruded upper incisors, and evident abnormal mouth breathing. TREATMENT: This was performed under local analgesia, rubber-dam isolation, and antimicrobial prophylaxis with amoxicillin (50 mg/kg). The patient's parents agreed to the treatment plan through a signed informed consent. This treatment consisted of the placement of pit and fissure sealants on the four permanent first molars (which included enamel preparation with fissurotomy burs), in-depth gingiva/dental frequent cleanings, local fluoride varnish applications, and an exhaustive programme of at-home oral hygiene (brushing, flossing, and chlorhexidine mouth rinses), including adequate nutrition. Gingivoplasty surgery to remove residual enlarged tissues was indicated for the near future. FOLLOW-UP: The child did not return to the clinic. When contacted, the parents reported that their daughter's systemic condition worsened significantly. She was confined to a bed at home under palliative care, with a life-span expectation of only a few months. CONCLUSION: Comprehensive dental care of children with ES requires careful consideration of their medical condition, and dental care delivery should be coordinated with the paediatric cardiologist. General analgesia should be considered only in strictly selected cases, due to the high peri-operative mortality reported.


Assuntos
Assistência Odontológica para Crianças/métodos , Complexo de Eisenmenger , Gengivite/terapia , Anestesia Local , Criança , Cárie Dentária/complicações , Cárie Dentária/terapia , Complexo de Eisenmenger/complicações , Feminino , Gengivite/complicações , Humanos , Respiração Bucal/complicações , Sobremordida
10.
Congenit Heart Dis ; 13(2): 210-216, 2018 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-29071783

RESUMO

OBJECTIVE: Congenital cardiac malformations with high pulmonary blood flow and pressure due to left-to-right shunts are usually repaired in early infancy for both the benefits of early relief of heart failure and the fear that the concomitant pulmonary hypertension may become irreversible unless these defects are corrected at an early age. Age, however, has been a poor predictor of irreversibility of pulmonary hypertension in our experience, which is presented here. DESIGN: A retrospective observational study. We defined "late" as age ≥2 years. We examined clinical, echocardiographic, and hemodynamic data from all patients aged ≥2 years with such malformations referred to us from 2004 untill 2015. SETTING: Department of Pediatric Cardiology and Cardiac Surgery, University Hospital of Vaud, Lausanne, Switzerland. PATIENTS: There were 39 patients, aged 2-35 years (median: 5 years), without chromosomal abnormalities. All had malformations amenable to biventricular repair, and all had high systolic right ventricular pressures by echocardiography prior to referral. INTERVENTIONS: All patients underwent catheterization for assessment of pulmonary hypertension. If this was reversible, surgical correction was offered. OUTCOME MEASURES: (1) Operability based on reversibility of pulmonary hypertension. (2) When surgery was offered, mortality and evidence of persisting postoperative pulmonary hypertension were examined. RESULTS: Eighteen patients had no pulmonary hypertension, 5 of variable ages were inoperable due to irreversible pulmonary hypertension, and 16 had reversible pulmonary hypertension. Therefore, 34 patients underwent corrective surgery, with no immediate or late mortality. Pulmonary arterial and right ventricular pressures decreased noticeably in all operated patients. This is sustained to date; they are all asymptomatic with no echocardiographic evidence of pulmonary hypertension at a median follow-up of 7 years (range 2-13 years). CONCLUSIONS: Pulmonary hypertension may still be reversible in many surprisingly old patients with left-to-right shunt lesions, who may therefore still be operable.


Assuntos
Complexo de Eisenmenger/complicações , Hemodinâmica/fisiologia , Hipertensão Pulmonar/fisiopatologia , Artéria Pulmonar/fisiopatologia , Adolescente , Adulto , Fatores Etários , Cateterismo Cardíaco , Procedimentos Cirúrgicos Cardíacos , Criança , Pré-Escolar , Progressão da Doença , Complexo de Eisenmenger/diagnóstico , Complexo de Eisenmenger/cirurgia , Feminino , Seguimentos , Humanos , Hipertensão Pulmonar/epidemiologia , Hipertensão Pulmonar/etiologia , Incidência , Masculino , Prognóstico , Artéria Pulmonar/diagnóstico por imagem , Estudos Retrospectivos , Fatores de Risco , Taxa de Sobrevida/tendências , Suíça/epidemiologia , Adulto Jovem
11.
Heart ; 104(1): 45-50, 2018 01.
Artigo em Inglês | MEDLINE | ID: mdl-28663364

RESUMO

OBJECTIVES: Although a significant proportion of patients with cyanotic congenital heart disease are thrombocytopaenic, its prevalence and clinical significance in adults with Eisenmenger syndrome (ES) is not well studied. Accordingly, we examined the relationship of thrombocytopaenia and mean platelet volume (MPV) to bleeding or thrombotic complications and survival in a contemporary cohort of patients with ES, including patients with Down syndrome. METHODS: Demographics, laboratory and clinical data were analysed from 226 patients with ES under active follow-up over 11 years. RESULTS: Age at baseline was 34.6±11.4 years and 34.1% were men. Mean platelet count and MPV were 152.6±73.3×109/L and 9.6±1.2 fL, respectively. A strong inverse correlation was found between platelet count and haemoglobin concentration and MPV. During the study, there were 39 deaths, and 21 thrombotic and 43 bleeding events. On univariate Cox regression analysis, patients with a platelet count <100×109/L had a twofold increased mortality (HR 2.10, 95% CI 1.10 to 4.01, p=0.024). Platelet count was not associated with an increased risk of thrombosis. However, there was a threefold increased thrombotic risk with MPV >9.5 fL (HR 3.50, 95% CI 1.28 to 9.54, p=0.015). Patients with either severe secondary erythrocytosis (>220g/L) or anaemia (<130g/L) were at higher risk of thrombotic events (HR 3.93, 95% CI 1.60 to 9.67, p=0.003; and HR 4.75, 95% CI 1.03 to 21.84, p=0.045, respectively). CONCLUSIONS: Thrombocytopaenia significantly increased the risk of mortality in ES. Furthermore, raised MPV, severe secondary erythrocytosis and anaemia, but not platelet count, were associated with an increased risk of thrombotic events in our adult cohort.


Assuntos
Plaquetas/fisiologia , Complexo de Eisenmenger/sangue , Previsões , Trombocitopenia/sangue , Adulto , Complexo de Eisenmenger/complicações , Complexo de Eisenmenger/mortalidade , Feminino , Seguimentos , Humanos , Masculino , Volume Plaquetário Médio , Contagem de Plaquetas , Estudos Retrospectivos , Fatores de Risco , Taxa de Sobrevida/tendências , Trombocitopenia/complicações , Reino Unido/epidemiologia
12.
ASAIO J ; 64(1): e8-e10, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-28195885

RESUMO

Pregnancy is typically contraindicated in Eisenmenger syndrome because of its association with excessively high maternal and fetal morbidity and mortality. Here, we report on our novel approach to successfully managing a 29 year old pregnant woman with an unrepaired patent ductus arteriosus (PDA) with Eisenmenger syndrome through delivery and recovery. Venovenous extracorporeal membrane oxygenation (ECMO) was utilized during induction of labor to stabilize maternal hemodynamics and optimize fetal oxygenation by creating an oxygenated right-to-left shunt through the PDA. Delivery ultimately required cesarean section on ECMO support, with her hospital course complicated by intraabdominal hemorrhage and, later, recannulation with venoarterial ECMO for postoperative respiratory failure with decompensated pulmonary hypertension. Ultimately, both the mother and baby were successfully discharged to home. This case highlights not only the potential for ECMO in the peripartum setting for patients with severe pulmonary hypertension, but also the complexity of management and the need for a highly experienced, multidisciplinary team.


Assuntos
Complexo de Eisenmenger/complicações , Oxigenação por Membrana Extracorpórea/métodos , Complicações Cardiovasculares na Gravidez/terapia , Adulto , Feminino , Humanos , Trabalho de Parto/fisiologia , Gravidez
13.
Heart Lung Circ ; 27(3): 394-396, 2018 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-28882496

RESUMO

BACKGROUND AND OBJECTIVES: Pericardial effusion (PE) confers a poor prognosis in non-congenital pulmonary arterial hypertension (PAH). The clinical significance and prognostic implications of PE in Eisenmenger syndrome (ES) is much less well characterised. Accordingly, we aimed to define the frequency, echocardiographic characteristics, natural history and clinical sequelae of PE in those with ES. PATIENTS AND METHODS: We retrospectively studied 55 consecutive individuals presenting since 2000, age >18 years, with ES. Analysis of clinical notes, transthoracic echocardiograms and vital status was undertaken via local records and the National Death Index. RESULTS: Mean age was 38 years (11.4SD), 22% had Down syndrome and the mean follow-up time was 14.7 years (13.4SD). Nine patients (16%) had a PE during their follow-up period with the majority being small (56%). No PE exhibited clinical or echocardiographic signs of tamponade and none required pericardiocentesis. At the time of last review, 42% of individuals had died. The presence of PE was not associated with mortality (p=0.83). CONCLUSIONS: Pericardial effusion occurs relatively frequently in ES but is usually small and with no evidence of haemodynamic compromise. Pericardial effusion in this setting is not related to mortality.


Assuntos
Ecocardiografia/métodos , Complexo de Eisenmenger/complicações , Previsões , Derrame Pericárdico/diagnóstico , Adulto , Complexo de Eisenmenger/diagnóstico , Feminino , Seguimentos , Humanos , Masculino , Derrame Pericárdico/etiologia , Estudos Retrospectivos
14.
Intern Med ; 56(24): 3299-3304, 2017 Dec 15.
Artigo em Inglês | MEDLINE | ID: mdl-29021475

RESUMO

The patient was a 19-year-old woman who was diagnosed with patent ductus arteriosus complicating Eisenmenger syndrome at a previous medical institution. She was referred to our hospital and arranged for lung transplantation. She developed hemoptysis after the introduction of i.v. epoprostenol, which was administered as a bridging treatment while the patient awaited lung transplantation. She continued to suffer from recurrent hemoptysis, even after switching from i.v. epoprostenol to i.v. treprostinil. Angiography of the systemic and pulmonary arteries revealed the vessel responsible for the recurrent hemoptysis and pulmonary artery embolization was successfully performed. It is essential to identify the culprit vessel and physicians must not hesitate in performing embolization when patients develop lethal hemoptysis.


Assuntos
Permeabilidade do Canal Arterial/complicações , Complexo de Eisenmenger/complicações , Embolização Terapêutica/métodos , Hemoptise/cirurgia , Artéria Pulmonar , Angiografia , Anti-Hipertensivos/efeitos adversos , Epoprostenol/efeitos adversos , Feminino , Hemoptise/induzido quimicamente , Humanos , Adulto Jovem
15.
Cardiovasc Res ; 113(12): 1423-1432, 2017 Oct 01.
Artigo em Inglês | MEDLINE | ID: mdl-28957530

RESUMO

In pulmonary arterial hypertension (PAH), right ventricular (RV) adaptation is essential to overcome the chronic increases in RV pressure overload. Ultimately, RV compensatory mechanisms are not sufficient and patients succumb to RV failure. The processes underlying the transition of RV adaptation to RV failure are not well understood. In this review, we propose that important insights in RV adaptation processes can be obtained by comparing different etiologies of PAH, namely patients with PAH secondary to Eisenmenger syndrome, patients with PAH secondary to systemic sclerosis and patients where no cause is identified: idiopathic PAH. Although the amount of RV afterload does not differ between these patient groups, their prognosis is distinctly different. We will show that an adaptive RV phenotype, as is observed in Eisenmenger patients, coincides with RV hypertrophy, increased RV contractility, low RV fibrosis and low RV diastolic stiffness. Whereas a phenotype of RV failure, as is observed in patients with PAH-secondary to systemic sclerosis, is characterized by impaired contractile reserve, RV fibrosis and RV diastolic stiffness.


Assuntos
Pressão Arterial , Complexo de Eisenmenger/complicações , Insuficiência Cardíaca/etiologia , Hipertensão Pulmonar/etiologia , Hipertrofia Ventricular Direita/etiologia , Artéria Pulmonar/fisiopatologia , Escleroderma Sistêmico/complicações , Disfunção Ventricular Direita/etiologia , Função Ventricular Direita , Adaptação Fisiológica , Animais , Progressão da Doença , Complexo de Eisenmenger/diagnóstico , Complexo de Eisenmenger/fisiopatologia , Hipertensão Pulmonar Primária Familiar/etiologia , Hipertensão Pulmonar Primária Familiar/fisiopatologia , Fibrose , Insuficiência Cardíaca/diagnóstico , Insuficiência Cardíaca/fisiopatologia , Humanos , Hipertensão Pulmonar/diagnóstico , Hipertensão Pulmonar/fisiopatologia , Hipertrofia Ventricular Direita/diagnóstico , Hipertrofia Ventricular Direita/fisiopatologia , Fatores de Risco , Escleroderma Sistêmico/diagnóstico , Escleroderma Sistêmico/fisiopatologia , Disfunção Ventricular Direita/diagnóstico , Disfunção Ventricular Direita/fisiopatologia , Remodelação Ventricular
17.
Medicine (Baltimore) ; 96(24): e7105, 2017 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-28614229

RESUMO

RATIONALE: Few studies had reported syndromes that include patent ductus arteriosus (PDA) with Eisenmenger syndrome and congenital deafness clustered in male siblings without facial, skeletal, or mental abnormalities. PATIENT CONCERNS: Two brothers, who were deaf and had PDA with Eisenmenger complex, were first seen at our Cardiology clinic at the ages of 25 and 41, respectively. They presented with progressive dyspnea on exertion. Upon physical examination, both brothers had clubbing and/or cyanotic toes, normal fingers, and without facial, skeletal, ophthalmological, or mental abnormalities. DIAGNOSES AND INTERVENTIONS: Echocardiography and multidetector computed tomography revealed large PDAs in both brothers. Cardiac catheterization showed bidirectional shunting via the PDA. OUTCOMES AND LESSONS: Familial clustering of Eisenmenger PDA and congenital deafness is rare. Further studies are warranted to define possible genetic links.


Assuntos
Cianose/complicações , Surdez/complicações , Permeabilidade do Canal Arterial/complicações , Complexo de Eisenmenger/complicações , Adulto , Cianose/diagnóstico , Cianose/tratamento farmacológico , Surdez/diagnóstico , Surdez/tratamento farmacológico , Diagnóstico Diferencial , Permeabilidade do Canal Arterial/diagnóstico , Permeabilidade do Canal Arterial/tratamento farmacológico , Complexo de Eisenmenger/diagnóstico , Complexo de Eisenmenger/tratamento farmacológico , Humanos , Irmãos
18.
Cardiol Young ; 27(9): 1845-1848, 2017 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-28651656

RESUMO

Patients with patent ductus arteriosus and significant left-to-right shunt develop Eisenmenger syndrome at an early age and are not operable after development of irreversible pulmonary artery hypertension. Patients with mitral stenosis, however, are treatable even with suprasystemic pulmonary artery pressures. A combination of these two lesions is rare. We document a patient with differential cyanosis who improved after corrective surgery of both the lesions. The importance of post-capillary pulmonary artery hypertension in shunt lesions needs to be better appreciated.


Assuntos
Permeabilidade do Canal Arterial , Hipertensão Pulmonar/complicações , Estenose da Valva Mitral , Cateterismo Cardíaco , Procedimentos Cirúrgicos Cardiovasculares/métodos , Criança , Cianose/complicações , Permeabilidade do Canal Arterial/complicações , Permeabilidade do Canal Arterial/diagnóstico por imagem , Permeabilidade do Canal Arterial/cirurgia , Complexo de Eisenmenger/complicações , Eletrocardiografia , Humanos , Masculino , Estenose da Valva Mitral/complicações , Estenose da Valva Mitral/diagnóstico por imagem , Estenose da Valva Mitral/cirurgia , Resultado do Tratamento
19.
Congenit Heart Dis ; 12(4): 512-519, 2017 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-28523857

RESUMO

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.


Assuntos
Arritmias Cardíacas/epidemiologia , Morte Súbita Cardíaca/epidemiologia , Complexo de Eisenmenger/complicações , Medição de Risco/métodos , Adolescente , Adulto , Arritmias Cardíacas/diagnóstico , Arritmias Cardíacas/etiologia , Complexo de Eisenmenger/epidemiologia , Eletrocardiografia Ambulatorial , Feminino , Seguimentos , Humanos , Incidência , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Fatores de Risco , Taxa de Sobrevida/tendências , Reino Unido/epidemiologia , Adulto Jovem
20.
Turk Kardiyol Dern Ars ; 45(2): 184-188, 2017 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-28424444

RESUMO

Presently described is successful cesarean delivery in a pregnancy superimposed on long-term bosentan treatment in an Eisenmenger syndrome patient with cor triatriatum sinistrum, double-orifice mitral valve, and large ventricular septal defect resulting in single functioning ventricle with double outlets. Cesarean delivery was performed at 27th week of gestation without maternal or fetal morbidity. The infant had no congenital cardiovascular abnormality or any probable teratogenic effect of bosentan treatment during pregnancy.


Assuntos
Cesárea , Coração Triatriado/complicações , Comunicação Interventricular/complicações , Lactente Extremamente Prematuro , Sulfonamidas/uso terapêutico , Adulto , Bosentana , Fármacos Cardiovasculares/uso terapêutico , Coração Triatriado/tratamento farmacológico , Complexo de Eisenmenger/complicações , Complexo de Eisenmenger/tratamento farmacológico , Feminino , Humanos , Recém-Nascido
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