RESUMEN
We present the case of a 26 year old polytrauma patient with a left intra-articular distal radial fracture, with an uncorrected late univentricular heart with severe pulmonary hypertension, subsequent Eisenmenger syndrome and supraventricular tachycardia managed with metoprolol. We find this case particularly interesting given the need for osteosynthesis with the obstacle of a non-palliated congenital heart disease in an adult; whose anesthetic implications required particular considerations in order to avoid fatal outcomes in the perioperative setting. Ultimately the patient underwent regional anesthesia with careful hemodynamics monitoring, leading to a satisfactory outcome.
Presentamos el caso de un paciente politraumatizado de 26 años con fractura de radio distal intraarticular izquierda, corazón univentricular tardío no corregido con hipertensión pulmonar severa, posterior síndrome de Eisenmenger y taquicardia supraventricular manejado con metoprolol. Este caso nos parece especialmente interesante dada la necesidad de osteosíntesis con el obstáculo de una cardiopatía congénita no paliada en un adulto; cuyas implicaciones anestésicas requirieron consideraciones particulares para evitar desenlaces fatales en el perioperatorio. Finalmente, el paciente se sometió a anestesia regional con un control hemodinámico cuidadoso, lo que llevó a un resultado satisfactorio.
Asunto(s)
Humanos , Masculino , Adulto , Traumatismo Múltiple/cirugía , Complejo de Eisenmenger/complicaciones , Corazón Univentricular/complicaciones , Anestesia/métodos , Anestésicos/administración & dosificación , Monitorización HemodinámicaRESUMEN
Interventricular septal defect (IVSD) is a congenital heart disease characterized by communication or non-closure of the interventricular septum in the embryonic or postnatal period, causing a failure in the separation between the systemic and pulmonary circulation. The severity of the hemodynamic consequences depends on the extent of the defect and the magnitude of the flow through it. Blood flow commonly occurs from left to right, because systemic vascular resistance is greater than pulmonary resistance. This report describes the echocardiographic findings in a 4-year-old male Poodle treated at the Veterinary Hospital with a history of exercise intolerance, cyanosis and episodes of syncope. On echocardiography, perimembranous IVSD was diagnosed with atrial and right ventricular dilatation, discontinuity of the interventricular septum in the subaortic region measuring approximately 5 mm. In the Doppler evaluation, reverse turbulent flow was evidenced, characterizing shunt from right to left (Eisenmenger syndrome). Despite preserved systolic function, abnormal relaxation was observed. Doppler echocardiography was a fundamental complementary imaging test for the diagnosis of this congenital heart disease since its suspicion is not commonly raised in adult patients. Doppler echocardiography allowed us to identify the defect, define its size and extension, determine the direction of blood flow as well as its hemodynamic consequences.(AU)
O defeito do septo interventricular (DSIV) é uma cardiopatia congênita caracterizada pela comunicação ou não fechamento do septo interventricular no período embrionário ou pós-natal, causando falha na separação entre a circulação sis-têmica e pulmonar. A gravidade das consequências hemodinâmicas depende da extensão do defeito e da magnitude do fluxo através dele. O fluxo sanguíneo comumente ocorre da esquerda para a direita, porque a resistência vascular sistêmica é maior do que a pulmonar. Este relato descreve os achados ecocardiográficos em um Poodle, macho, de 4 anos atendido no Hospital Veterinário com história de intolerância ao exercício, cianose e episódios de síncope. Ao ecocardiograma foi diagnosticado DSIV do tipo perimembranoso com dilatação atrial e ventricular direita, descontinuidade do septo interventricular em região subaórtica medindo aproximadamente 5 mm. Na avaliação Doppler, evidenciou-se fluxo turbulento reverso, caracterizando shunt da direita para a esquerda (síndrome de Eisenmenger). Apesar da função sistólica preservada, foi observado relaxamento anormal. A ecocardiografia Doppler foi um exame de imagem complementar fundamental para o diagnóstico dessa cardio-patia congênita, uma vez que sua suspeita não é comumente levantada em pacientes adultos. A ecocardiografia Doppler per-mitiu identificar o defeito, definir seu tamanho e extensão, determinar a direção do fluxo sanguíneo, bem como suas conse-quências hemodinâmicas.(AU)
Asunto(s)
Animales , Perros/anomalías , Complejo de Eisenmenger/veterinaria , Tabique Interventricular , EcocardiografíaAsunto(s)
Humanos , Complejo de Eisenmenger/clasificación , Complejo de Eisenmenger/tratamiento farmacológico , Complejo de Eisenmenger/diagnóstico por imagen , Broncoscopía/métodos , Cateterismo Cardíaco/métodos , Electrocardiografía , Inhibidores de Fosfodiesterasa 5/administración & dosificación , Bosentán/administración & dosificación , Cardiopatías CongénitasRESUMEN
BACKGROUND: Inflammation may be an important factor contributing to the progression of Eisenmenger syndrome (ES). The purpose of the current study was to: characterize the inflammatory profile in ES patients and compare measures to reference values for congenital heart disease and pulmonary arterial hypertension (CHD-PAH); and investigate whether inflammatory markers are associated with other clinical markers in ES. METHODS: Twenty-seven ES patients were prospectively selected and screened for systemic inflammatory markers, including interleukin (IL)-1ß, tumor necrosis factor-alpha (TNF-α) and IL-10. Clinical data and echocardiographic parameters were obtained, with concomitant analysis of ventricular function. Functional capacity was assessed using the 6-min walk test (6MWT). Renal function and blood homeostasis were evaluated by the level of blood urea nitrogen (BUN), creatinine, and plasma electrolytes. RESULTS: Patients with ES expressed higher IL-10, IL-1ß and TNF-α compared to reference values of patients with CHD-PAH. IL-10 was negatively associated with BUN (r = -0.39,p = 0.07), creatinine (r = -0.35, p = 0.002), sodium (r = -0.45, p = 0.03), and potassium (r = -0.68, p = 0.003). IL-10 was positively associated with bicarbonate (r = 0.45, p = 0.02) and trended toward a positive association with right ventricular fractional area change (RVFAC) (r = 0.35, p = 0.059). IL-1ß was negatively associated with potassium (r = -0.5, p = 0.01). TNF-α demonstrated positive association with creatinine (r = 0.4,p = 0.006), BUN (r = 0.63,p = 0.003), sodium (r = 0.44, p = 0.04), potassium (r = 0.41, p = 0.04), and was negatively associated with RVFAC (r = -0.38,p = 0.03) and 6MWT distance (r = -0.54, p = 0.004). CONCLUSION: ES patients exhibit a more severe inflammatory profile compared to reference values for CHD-PAH. Furthermore, inflammatory markers are related to renal dysfunction, right ventricular impairment and poorer functional capacity.
Asunto(s)
Complejo de Eisenmenger , Hipertensión Pulmonar , Biomarcadores , Estudios Transversales , Complejo de Eisenmenger/diagnóstico por imagen , Hipertensión Pulmonar Primaria Familiar , HumanosRESUMEN
A Síndrome de Eisenmenger (SE) é uma doença rara que exige tratamento interdisciplinar, caracterizada por hipertensão pulmonar associada a alterações cardiovasculares de forma congênita. A literatura tem pouca informação sobre a abordagem interdisciplinar entre odontologia e cardiologia no suporte a indivíduos com SE. Portanto, o objetivo deste artigo é apresentar, através de uma revisão integrativa, a abordagem odontológica em indivíduos com SE. Realizou-se pesquisa estruturada com os seguintes termos: "((dentistry) OR (Dental) OR (dental management) OR (dental care) AND (Eisenmenger syndrome))", utilizando para a busca as bases de dados PUBMED e SCOPUS. A busca gerou 32 artigos, dos quais após aplicados os critérios de inclusão e exclusão, foram analisados 7 que mencionavam algum tipo de atendimento odontológico em indivíduos com SE. Os resultados mostraram somente relatos de casos clínicos, que trouxeram informações sobre o atendimento odontológico sempre com decisão conjunta entre cirurgião-dentista e o médico cardiologista. Foram apresentadas as alterações bucais e os riscos cardiológicos associados e o manejo clínico interdisciplinar nesse grupo de pacientes. Concluiu-se que a ação interdisciplinar no atendimento e suporte clínico aos pacientes com SE exigem conhecimentos específicos sobre a doença e a abordagem especial que proporcionará o manejo o mais seguro possível além de qualidade de vida e da condição bucal.
Asunto(s)
Complejo de Eisenmenger , Grupo de Atención al Paciente , Cardiología , Atención Odontológica , Odontología Basada en la EvidenciaRESUMEN
RESUMEN El síndrome de Eisenmenger es una patología del sistema vascular pulmonar, que se caracteriza por un incremento de las resistencias en los vasos sanguíneos pulmonares, en pacientes con antecedentes de cardiopatía con comunicación entre la circulación pulmonar y circulación sistémica. Este síndrome en la mujer embarazada representa un alto índice de morbilidad y mortalidad tanto para la madre como para el feto; los síntomas que presentan surgen como consecuencia de los cambios fisiológicos en el sistema cardiovascular y hematopoyético. Es un desafío para el obstetra el manejo de estas pacientes idealmente debe ofrecerse asesoría preconcepcional, y valoración por cardiología; si no es posible, se realizará evaluación del riesgo desde el primer control prenatal. El mejor tratamiento consiste en oxigenoterapia, medicamentos vasodilatadores, y anticoagulantes, logrando disminuir el porcentaje de pobres desenlaces materno-fetales. Es de vital importancia definir el momento del nacimiento con el fin de tomar las medidas terapéuticas necesarias para evitar complicaciones, además se requiere de un manejo multidisciplinario, incluido el apoyo por una unidad de cuidado intensivo dado la alta probabilidad de complicaciones asociadas a esta patología.
ABSTRACT Eisenmenger syndrome is a pathology of the pulmonary vascular system, which is characterized by an increase in resistance of the pulmonary blood vessels in patients with a history of heart disease with communication between the pulmonary circulation and the systemic circulation. This syndrome in pregnant women represents a high morbidity and mortality rate for both the mother and the fetus. The symptoms that arise are a consequence of the physiological changes in the cardiovascular and hematopoietic system. The management of these patients is a challenge for the obstetrician; ideally, preconception counseling and cardiology assessment should be offered and if this is not possible, risk assessment should be carried out from the first prenatal control. The best treatment consists of oxygen therapy, vasodilating agents, and anticoagulant medications with the goal of reducing the percentage of poor maternal-fetal outcomes. It is vitally important to define the time of birth in order to take the necessary therapeutic measures to avoid complications. In addition, multidisciplinary management and an intensive care unit are required due to the high probability of complications associated with this pathology.
Asunto(s)
Humanos , Femenino , Embarazo , Adulto , Complicaciones Cardiovasculares del Embarazo/etiología , Complejo de Eisenmenger/complicaciones , Complicaciones Cardiovasculares del Embarazo/terapia , Complejo de Eisenmenger/terapia , Hipertensión Pulmonar/etiologíaRESUMEN
El Síndrome de Eisenmenger es un desorden multisistémico caracterizado por hipertensión pulmonar con inversión o bidireccionalidad del flujo a través de una comunicación intracardiaca o aortopulmonar. Se presenta el caso de un paciente masculino de 48 años de edad con antece- dentes de Hipertensión Pulmonar e Insuficiencia Cardíaca en tratamiento, quien acude por cuadro de melenas, decaimiento e inestabilidad hemodinámica. Al examen físico se ausculta soplo sistólico ascendente en foco pulmonar, abdomen distendido con presencia de onda ascíti- ca. En los exámenes complementarios; la radiografía convencional de tórax mostró aumento de la trama vascular pulmonar, además de encontrarse alteraciones electrocardiográficas. El paciente mostró una evolución desfavorable produciéndose falla cardíaca, que dio lugar al posterior fallecimiento debido a un paro cardiorrespiratorio.
Eisenmenger Syndrome is a multisystemic disorder identified by pulmonary hypertension with reversal or bidirectional flow through intracardiac or aortopulmonary communication. The case of a 48-year-old male patient with a history of Pulmonary Hypertension and Heart Failure in treatment is presented, who comes due to mane of mane, decay, and hemodynamic instability. The physical examination auscultated systolic murmur ascending in pulmonary focus, distended abdomen with presence of ascites wave. In the complementary exams, conventional chest x-ray, increased pulmonary vascular weft, in addition to finding electrocardiographic abnormalities. The patient seemed an unfavorable evolution, producing a heart failure, which resulted in subse- quent death due to cardiorespiratory arrest.
Asunto(s)
Humanos , Masculino , Persona de Mediana Edad , Síndrome , Complejo de Eisenmenger , Hipertensión Pulmonar , Pacientes , Tórax , CorazónRESUMEN
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/epidemiologíaRESUMEN
El síndrome de Eisenmenger es un trastorno multisistémico caracterizado por hipertensión pulmonar con inversión o bidireccionalidad del flujo a través de una comunicación intracardiaca o aortopulmonar. El propósito de este estudio es dar a conocer las características generales del síndrome de Eisenmenger. Se presenta el caso de un paciente masculino de 48 años de edad con diagnóstico de lupus eritematoso sistémico de 6 años de evolución con hipertensión pulmonar e insuficiencia cardiaca en tratamiento, que acude por cuadro de melenas, decaimiento e inestabilidad hemodinámica. Al examen físico destaca la presencia de un abdomen distendido y edemas de los miembros inferiores. Se realizaron exámenes complementarios hasta llegar al diagnóstico de síndrome de Eisenmenger. A pesar de presentar múltiples complicaciones en el curso del lupus eritematoso sistémico no se encontraron reportes que asociaran al lupus con el síndrome de Eisenmenger. El diagnóstico en la infancia de las cardiopatías congénitas cianóticas y la realización de la cirugía correctora constituyen la principal medida preventiva de aparición del síndrome de Eisenmeger(AU)
Eisenmenger syndrome is a multisystemic disorder characterized by pulmonary hypertension with inversion or bidirectional flow through intracardiac or aortopulmonary communication. To publicize the general characteristics of Eisenmenger syndrome. The case of a 48-year-old male patient with a diagnosis of 6-year-old systemic lupus erythematosus with pulmonary hypertension and cardiac insufficiency in treatment is presented, which is due to mane of mane, decay and hemodynamic instability. The physical examination highlights the presence of a distended abdomen and edema of the lower limbs. Complementary examinations were made to reach the diagnosis of Eisenmenger syndrome. Despite presenting multiple complications in the course of systemic lupus erythematosus, no reports were found that associated lupus with Eisenmenger syndrome. Diagnosis of cyanotic congenital heart disease in childhood and corrective surgery are the main preventive measures for the appearance of Eisenmenger syndrome(AU)
Asunto(s)
Humanos , Masculino , Persona de Mediana Edad , Examen Físico , Complejo de Eisenmenger , Cardiopatías Congénitas , Insuficiencia Cardíaca , Lupus Eritematoso SistémicoRESUMEN
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.
Asunto(s)
Atención Dental para Niños/métodos , Complejo de Eisenmenger , Gingivitis/terapia , Anestesia Local , Niño , Caries Dental/complicaciones , Caries Dental/terapia , Complejo de Eisenmenger/complicaciones , Femenino , Gingivitis/complicaciones , Humanos , Respiración por la Boca/complicaciones , SobremordidaRESUMEN
A 6-month-old female, 1.0kg, uncastrated female Persian cat was brought to the Veterinary Hospital at State University of Ceara, with a history of dyspnea, prostration, hyporexia and progressive weight loss for a month. On physical examination, systolic cardiac murmur, cyanosis and dyspnea were detected. Unfortunately, the cat died during oxygen therapy. Necropsy examination revealed an increase in cardiac silhouette and ventricular septal defect of 2cm in diameter. Macroscopically the lungs were collapsed, with absent and diffusely reddish blackish crepitus, and the liver with blackish red coalescent multifocal areas, interspersed with lighter areas and lobular pattern with irregular brownish multifocal areas intercepted by brownish areas. Thus, the necropsy results together with the history and physical examination of the animal confirmed the diagnosis of Eisenmenger Syndrome, becoming the report of the first case, in a cat, in Brazil.(AU)
Asunto(s)
Animales , Femenino , Gatos , Complejo de Eisenmenger/clasificación , Gatos/anomalías , Complejo de Eisenmenger/diagnóstico , Defectos del Tabique Interventricular/veterinariaRESUMEN
A 6-month-old female, 1.0kg, uncastrated female Persian cat was brought to the Veterinary Hospital at State University of Ceara, with a history of dyspnea, prostration, hyporexia and progressive weight loss for a month. On physical examination, systolic cardiac murmur, cyanosis and dyspnea were detected. Unfortunately, the cat died during oxygen therapy. Necropsy examination revealed an increase in cardiac silhouette and ventricular septal defect of 2cm in diameter. Macroscopically the lungs were collapsed, with absent and diffusely reddish blackish crepitus, and the liver with blackish red coalescent multifocal areas, interspersed with lighter areas and lobular pattern with irregular brownish multifocal areas intercepted by brownish areas. Thus, the necropsy results together with the history and physical examination of the animal confirmed the diagnosis of Eisenmenger Syndrome, becoming the report of the first case, in a cat, in Brazil.(AU)
Asunto(s)
Animales , Femenino , Gatos , Gatos/anomalías , Complejo de Eisenmenger/clasificación , Complejo de Eisenmenger/diagnóstico , Defectos del Tabique Interventricular/veterinariaRESUMEN
La anomalía de Taussig-Bing es una cardiopatía congénita cianosante caracterizada por la dextrotransposición de grandes vasos. Esta produce una doble salida arterial desde el ventrículo derecho, asociado a una comunicación interventricular. Este cuadro puede generar una hipertensión pulmonar secundaria al aumento de las resistencias vasculares y un flujo reverso cardiaco, conocido como síndrome de Eisenmenger. Normalmente, se presenta antes de la pubertad, aunque en ocasiones, puede debutar en la vida adulta, progresando durante dicha etapa. Clínicamente, se caracteriza por rasgos crónicos, como las acropaquias, la disnea, la sensación de cansancio o la cianosis. El diagnóstico de este tipo de cardiopatías se basa en la clínica y en las pruebas de imagen, preferentemente en el estudio ecocardiográfico fetal o durante la edad pediátrica. El tratamiento de elección es la corrección quirúrgica de las malformaciones cardiacas, siendo preferente la rectificación de la salida de la aorta y el cierre de la comunicación interventricular. El pronóstico depende del grado de hipertensión pulmonar, del momento del diagnóstico y de la corrección quirúrgica precoz. A edades tempranas se obtiene un mejor resultado, aunque las tasas de mortalidad alcanzan 50 por ciento en algunos casos, incluso tras una corrección quirúrgica óptima. La gestación no está recomendada en pacientes que padecen dicha patología, la cual se ha contraindicado, según algunos estudios, en ausencia de tratamiento adecuado. Así pues, describimos un caso en el que una gestante con una anomalía Taussig-Bing sufre una atonía uterina y un posterior paro cardiorrespiratoria tras el parto, realizado mediante cesárea electiva, tras la que la paciente falleció(AU)
The Taussig-Bing anomaly is a congenital cyanosis characterized by the dextrotransposition of large vessels. It produces a double arterial exit from the right ventricle, associated with an interventricular communication. This may lead to pulmonary hypertension secondary to increased vascular resistance and a cardiac reverse flow, known as Eisenmenger syndrome. Generally, it occurs before puberty, although occasionally, it can debut in adults, progressing during that stage. It is clinically characterized by chronic features, such as acropachies, dyspnea, tiredness or cyanosis. The diagnosis of this type of heart disease is based on clinical exam and imaging tests, if at all possible in the fetal echocardiographic study or during the pediatric age. The surgical correction of cardiac malformations is the treatment of choice, modifying the aortic exit and closing of ventricular septal defect. The prognosis depends on the degree of pulmonary hypertension, the time of diagnosis, and the early surgical correction. Better outcome is obtained at early ages, although mortality rates reach 50 percent in some cases, even after optimal surgical correction. Pregnancy is not recommended in patients suffering from this disease. Some studies contraindicate pregnancy in absence of proper treatment. Thus, we describe a case of a pregnant woman with a Taussig-Bing anomaly, who suffered uterine atony and a subsequent cardiorespiratory arrest after delivery. Elective cesarean section was performed. This patient died(AU)
Asunto(s)
Humanos , Femenino , Embarazo , Adulto , Ventrículo Derecho con Doble Salida/complicaciones , Ventrículo Derecho con Doble Salida/mortalidad , Muerte Materna , Cesárea/mortalidad , Complejo de Eisenmenger/mortalidadRESUMEN
La anomalía de Taussig-Bing es una cardiopatía congénita cianosante caracterizada por la dextrotransposición de grandes vasos. Esta produce una doble salida arterial desde el ventrículo derecho, asociado a una comunicación interventricular. Este cuadro puede generar una hipertensión pulmonar secundaria al aumento de las resistencias vasculares y un flujo reverso cardiaco, conocido como síndrome de Eisenmenger. Normalmente, se presenta antes de la pubertad, aunque en ocasiones, puede debutar en la vida adulta, progresando durante dicha etapa. Clínicamente, se caracteriza por rasgos crónicos, como las acropaquias, la disnea, la sensación de cansancio o la cianosis. El diagnóstico de este tipo de cardiopatías se basa en la clínica y en las pruebas de imagen, preferentemente en el estudio ecocardiográfico fetal o durante la edad pediátrica. El tratamiento de elección es la corrección quirúrgica de las malformaciones cardiacas, siendo preferente la rectificación de la salida de la aorta y el cierre de la comunicación interventricular. El pronóstico depende del grado de hipertensión pulmonar, del momento del diagnóstico y de la corrección quirúrgica precoz. A edades tempranas se obtiene un mejor resultado, aunque las tasas de mortalidad alcanzan 50 % en algunos casos, incluso tras una corrección quirúrgica óptima. La gestación no está recomendada en pacientes que padecen dicha patología, la cual se ha contraindicado, según algunos estudios, en ausencia de tratamiento adecuado. Así pues, describimos un caso en el que una gestante con una anomalía Taussig-Bing sufre una atonía uterina y un posterior paro cardiorrespiratoria tras el parto, realizado mediante cesárea electiva, tras la que la paciente falleció(AU)
The Taussig-Bing anomaly is a congenital cyanosis characterized by the dextrotransposition of large vessels. It produces a double arterial exit from the right ventricle, associated with an interventricular communication. This may lead to pulmonary hypertension secondary to increased vascular resistance and a cardiac reverse flow, known as Eisenmenger syndrome. Generally, it occurs before puberty, although occasionally, it can debut in adults, progressing during that stage. It is clinically characterized by chronic features, such as acropachies, dyspnea, tiredness or cyanosis. The diagnosis of this type of heart disease is based on clinical exam and imaging tests, if at all possible in the fetal echocardiographic study or during the pediatric age. The surgical correction of cardiac malformations is the treatment of choice, modifying the aortic exit and closing of ventricular septal defect. The prognosis depends on the degree of pulmonary hypertension, the time of diagnosis, and the early surgical correction. Better outcome is obtained at early ages, although mortality rates reach 50% in some cases, even after optimal surgical correction. Pregnancy is not recommended in patients suffering from this disease. Some studies contraindicate pregnancy in absence of proper treatment. Thus, we describe a case of a pregnant woman with a Taussig-Bing anomaly, who suffered uterine atony and a subsequent cardiorespiratory arrest after delivery. Elective cesarean section was performed. This patient died(AU)
Asunto(s)
Humanos , Femenino , Embarazo , Adulto , Ventrículo Derecho con Doble Salida/complicaciones , Ventrículo Derecho con Doble Salida/mortalidad , Muerte Materna , Cesárea/mortalidad , Complejo de Eisenmenger/mortalidadRESUMEN
BACKGROUND: Eisenmenger syndrome (ES) precipitates the extreme manifestation of pulmonary hypertension, which leads to severe functional limitation and poor quality of life. The propose of the current study was: 1) examined the acute effects of 40% oxygen supplementation during the 6-minute walk test (6MWT); and 2) evaluate the relation between exercise capacity and clinical cardiac parameters in patients with ES. METHODS: Thirty subjects were prospectively included; all were submitted to a 6MWT with compressed air and with 40% of oxygen. Heart rate recovery at the first minute (HRR1) and perceived effort Borg scale for dyspnea and lower limb fatigue were recorded in both tests scenarios. RESULTS: The 6MWT distance was modestly, negatively associated with pulmonary vascular resistance (PVR) [r=0.46, p=0.02]. Patients improved 6MWT distance (p<0.001) and exhibited a faster HRR1 (p<0.001) with 40% supplemental oxygen compared to compressed air. With 40% supplemental oxygen, subjects revealed lower dyspnea and lower limb fatigue compared to 6MWT without oxygen supplementation (p<0.001). The amount of change in the 6MWT distance from air to oxygen was moderate, positively associated with tricuspid annular plane systolic excursion (TAPSE) and right ventricular fractional area change (RVFAC) [r=0.50, p=0.03; r=0.64, p<0.001, respectively]. CONCLUSION: Acute 40% oxygen supplementation in patients with Eisenmenger syndrome led to an improvement in 6MWT distance, faster HRR1 and lower dyspnea and lower limb fatigue perception. Moreover, functional capacity was positively associated with right ventricular parameters.
Asunto(s)
Complejo de Eisenmenger/terapia , Tolerancia al Ejercicio/fisiología , Frecuencia Cardíaca/fisiología , Terapia por Inhalación de Oxígeno/métodos , Recuperación de la Función/fisiología , Resistencia Vascular/fisiología , Función Ventricular Derecha/fisiología , Adulto , Ecocardiografía , Complejo de Eisenmenger/fisiopatología , Prueba de Esfuerzo , Femenino , Frecuencia Cardíaca/efectos de los fármacos , Humanos , Masculino , Estudios ProspectivosRESUMEN
Eisenmenger syndrome is very rare in pregnant women. Debates remain concerning the management of Eisenmenger syndrome in this patient population and the prognosis is unclear in terms of maternal and fetoneonatal outcomes. Epidural analgesia is preferred for Cesarean section as it alleviates perioperative pain and reduces the pulmonary and systemic vascular resistances. Maternal mortality in the presence of Eisenmenger syndrome is reported as 30-50% and even up to 65% in those with Cesarean section. The major causes of death could be hypovolemia, thromboembolism and preeclampsia. Pregnancy should ideally be avoided in a woman with Eisenmenger syndrome concerning the high maternal mortality rate and probable poor prognosis of the baby. A short labour and an atraumatic delivery under epidural block are preferred in the women with a strong desire of pregnancy. The purpose of this article is to discuss the debates of Eisenmenger syndrome in pregnancy and the possible resolutions.
Asunto(s)
Complejo de Eisenmenger/diagnóstico , Complicaciones Cardiovasculares del Embarazo/diagnóstico , Cesárea , Complejo de Eisenmenger/fisiopatología , Complejo de Eisenmenger/terapia , Femenino , Humanos , Embarazo , Complicaciones Cardiovasculares del Embarazo/fisiopatología , Complicaciones Cardiovasculares del Embarazo/terapia , Resultado del Embarazo , PronósticoRESUMEN
Abstract Eisenmenger syndrome is very rare in pregnant women. Debates remain concerning the management of Eisenmenger syndrome in this patient population and the prognosis is unclear in terms of maternal and fetoneonatal outcomes. Epidural analgesia is preferred for Cesarean section as it alleviates perioperative pain and reduces the pulmonary and systemic vascular resistances. Maternal mortality in the presence of Eisenmenger syndrome is reported as 30-50% and even up to 65% in those with Cesarean section. The major causes of death could be hypovolemia, thromboembolism and preeclampsia. Pregnancy should ideally be avoided in a woman with Eisenmenger syndrome concerning the high maternal mortality rate and probable poor prognosis of the baby. A short labour and an atraumatic delivery under epidural block are preferred in the women with a strong desire of pregnancy. The purpose of this article is to discuss the debates of Eisenmenger syndrome in pregnancy and the possible resolutions.