Multidisciplinary approach for the management of term pregnancy complicated by Eisenmenger syndrome / 浙江大学学报（英文版）（B辑：生物医学和生物技术）

Pregnancy in patients with Eisenmenger syndrome (ES) is associated with high maternal mortality rates of 30%‒50%, or even up to 65% in the case of a cesarean section (Yuan, 2016). Here, we report a case of term pregnancy complicated with ES and severe pulmonary artery hypertension (PAH), which was managed by a multidisciplinary team (MDT) and resulted in an uncomplicated delivery via elective cesarean section. The goal of this study is to emphasize the importance of multidisciplinary approach in the management of pregnancy with ES, which can profoundly improve maternal and infant outcomes.

Síndrome de Eisenmenger y embarazo - reporte de 2 casos / Eisenmenger's and pregnancy syndrome - 2 case report

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.

Paciente con síndrome de Eisenmenger complicado por sangrado digestivo alto / Patient with Eisenmenger syndrome complicated by upper digestive bleeding

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.

Sangrado digestivo alto como complicación en un paciente lúpico con síndrome de Eisenmenger / High digestive bleeding as a complication in a lupus patient with Eisenmenger syndrome

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)

Sudden Death due to Rupture of Pulmonary Trunk Aneurysm in a Patient with Eisenmenger Syndrome / 대한법의학회지

Proximal pulmonary artery aneurysms and dissection are rare and life-threatening conditions, which are usually detected only during autopsy examination in cases of sudden death. These pathological entities often occur as complications of chronic pulmonary hypertension and most commonly result from pulmonary arterial hypertension associated with various congenital cardiac lesions involving left-to-right shunting. This study describes an autopsy case of a 38-year-old man who was diagnosed with Eisenmenger syndrome 5 years prior to sudden death secondary to cardiac tamponade following a ruptured pulmonary trunk aneurysm.

Pulmonary Arterial Hypertension and Pregnancy: Single Center Experience in Current Era of Targeted Therapy

OBJECTIVES: To report our experiences in pregnant patients with pulmonary arterial hypertension (PAH) who were treated with targeted therapy. METHODS: From 2011 to 2017, women who decided to maintain pregnancies in our PAH clinic were included. Clinical data, management, and outcomes of the mothers and fetuses were reviewed. RESULTS: Nine women with PAH and 10 deliveries were reviewed. The median maternal age was 28 (26–32) years old. The functional status of each patient was New York Heart Association functional class II or III at first visit. Sildenafil was prescribed in advance in 9 cases of delivery. Multidiscipline team approach management and intensive care were performed during the peripartum period. There was no maternal or fetal mortality. Severe cardiac events occurred in 2 patients with Eisenmenger syndrome: cardiac arrest and uncontrolled arrhythmia. Non-cardiac events occurred in 3 cases: postpartum bleeding, urinary tract infection, and pneumonia. The median gestational period at delivery was about 34 (32–38) weeks. Three cases were emergent delivery because of unexpected preterm labor. Intrauterine growth restriction developed in 4 fetuses. CONCLUSIONS: Pregnancy could be maintained by the introduction of targeted therapy rather more safely than the previous era in the case of maintenance of pregnancy. Intensive care and a multidisciplinary team approach can possibly improve the outcomes of the pregnant women with PAH and their babies. However, pregnancy in patients with PAH is still strongly prohibited and it can be tried in expert center where there has sufficient multidisciplinary team approach in case of inevitability.

Síndrome de Eisenmenger em um gato / Eisenmenger syndrome in cat

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)

Anesthetic Management for Elective Total Hip Replacement Arthoroplasty in a Patient with Eisenmenger's Syndrome

Eisenmenger's syndrome describes the elevation of pulmonary arterial pressure to the systemic level caused by an increased pulmonary vascular resistance with reversed or bi-directional shunt through an intracardiac or aortopulmonary communication. We report the case of 48-year-old woman with an elective total hip replacement arthroplasty for right femur neck fracture with Eisenmenger's syndrome secondary to large atrial septal defect. Anesthesia was induced with etomidate and rocuronium, maintained with desflurane 5 vol% and O₂ 3 L/min. Mirinone and norepinephrine were infused continuously to decrease right to left shunt. The patient was extubated after spontaneous breathing recovery and transferred to the intensive care unit. She was treated with mirinone, norepinephrine, dopamine, and dobutamine for hypoxemia and hypotension. After 3 hours of admission to intensive care unit, the patient had a cardiac arrest and died 30 minutes later.

Éxitus materno tras cesárea en gestante con anomalía de Taussig-Bing y síndrome de Eisenmenger / Maternal Exitus after Cesarean Section in Pregnant Women with Taussig-Bing Anomaly and Eisenmenger Syndrome

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)

Pulmonary artery dissection in a patient with Eisenmenger's syndrome and successful conservative medical treatment

No abstract available.

Eisenmenger syndrome in pregnancy

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.

Deep sedation for dental treatment in a Down syndrome patient with Eisenmenger syndrome: A case report

Eisenmenger syndrome (ES) is characterized by pulmonary arterial hypertension and right-to-left shunting. The signs and symptoms of ES include cyanosis, shortness of breath, fatigue, hemoptysis, and sudden death. In patients with ES, it is important that the systemic and pulmonary circulations be properly distributed and maintained. General dental treatment is not known to be particularly dangerous. To control pain and anxiety, local anesthetics without epinephrine are usually recommended. However, in cases of difficulty of cooperation, general anesthesia for dental treatment makes the condition worse. In the present case, intravenous deep sedation with propofol and remifentanil was administered for behavioral management during dental treatment successfully.

Hipertensão pulmonar secundária a cardiopatias congênitas: onde estamos e onde podemos chegar / Pulmonary hypertension secondary to congenital heart disease: where we are and where we can go

A prevalência da hipertensão pulmonar associada às cardiopatias congênitas(HAP-CCG) é estimada em 1,6-12,5 casos por um milhão de adultos, com 25-50% destes pacientes exibindo a forma mais séria da doença, a síndrome de Eisenmenger. Mais de 90% dos pacientes portadores de cardiopatias congênitas com comunicações simples entre as circulações sistêmica e pulmonar são submetidos à correção cirúrgica ou percutânea dos defeitos ainda na infância. Entretanto 5 a 10% dos pacientes portadores destes defeitos não apresentam esse padrão, mesmo na presença de comunicações não restritivas, com risco maior de complicações graves no pós-operatório, incluindo falência ventricular direita e óbito. A avaliação e o manejo clínico dos pacientes com HAP-CCG devem ser individualizadas devido à grande multiplicidade de situações. Envolve uma ampla faixa etária, com formas de apresentação muito distintas e diferentes problemas a serem equacionados. Ainda hoje a literatura é escassa no que diz respeito ao manejo medicamentoso, principalmente dentro da faixa etária pediátrica. As perspectivas futuras são na direção de expansão do conhecimento fisiopatológico, incluindo aspectos genéticos, com o objetivo de melhora progressiva tanto na avaliação precoce quanto na conduta terapêutica para esses pacientes.

The prevalence of pulmonary hypertension associated with congenital heart disease(PHT-CHD) is estimated at 1.6-12.5 cases per million adults, with 25%-50% of these presenting the most serious form of the disease: Eisenmenger’s syndrome. More than 90% of patients with congenital heart disease with simple communications between the systemic and pulmonary circulations under go surgical or percutaneous correction of the defects while still in infancy. However, 5% to 10% of patients with these defects do not present this same pattern, even in the presence of non-restrictive communications, with a higher risk of severe postoperative complications, including right ventricular failure and death. The evaluation and clinical management of patients with PHT-CHD should be individualized , due to the great multiplicity of situations. It involves a wide age range, with very distinct forms of presentation and different problems to be resolved. The literature still lacks studieson drug management, particularly in pediatric patients. The future prospects are towards the expansion of physio-pathological knowledge, including genetic aspects, with the aim of gradually improving both early evaluation and therapeutic conduct for these patients.

Hipertensión pulmonar asociada a cardiopatías congénitas y síndrome de Eisenmenger / Pulmonary hypertension associated with congenital heart disease and Eisenmenger syndrome

La hipertensión arterial pulmonar es una complicación frecuente de las cardiopatías congénitas (CC). Es reconocido que las CC son las malformaciones más frecuentes al nacimiento con una prevalencia que va de 6 a 8 por 1,000 recién nacidos vivos. En nuestro país se calcula que cada año nacen de 12,000 a 16,000 niños con algún tipo de malformación cardiaca. En los pacientes no corregidos con cortocircuitos de izquierda a derecha el aumento de la presión pulmonar provoca incremento en las resistencias pulmonares y desencadena una disfunción endotelial y remodelación vascular, consecuencia de alteraciones en los mediadores vasoactivos que provocan vasoconstricción, inflamación, trombosis, proliferación y apoptosis celular así como fibrosis. Como consecuencia hay un incremento progresivo de las resistencias pulmonares y de la presión del ventrículo derecho. Finalmente ocurre que el flujo sanguíneo se invierte y se desarrolla el síndrome de Eisenmenger, la forma más avanzada de hipertensión arterial pulmonar consecutiva a CC. La frecuencia de hipertensión arterial pulmonar secundaria a CC ha disminuido en países desarrollados no así en países en vías de desarrollo tanto por un diagnóstico tardío como por falta de infraestructura hospitalaria o de recursos humanos para la atención de los pacientes portadores de CC. Con el advenimiento del tratamiento farmacológico para la hipertensión arterial pulmonar, se han vislumbrado nuevas oportunidades terapéuticas, siendo cada vez más cotidiano que se sumen al tratamiento intervencionista o quirúrgico en pacientes con hipertensión arterial pulmonar secundaria a CC. Se requiere conocer los factores fisiopatológicos involucrados así como llevar a cabo una cuidadosa evaluación para definir la mejor estrategia terapéutica.

Pulmonary arterial hypertension is a common complication of congenital heart disease (CHD). Congenital cardiopathies are the most frequent congenital malformations. The prevalence in our country remains unknown, based on birthrate, it is calculated that 12,000 to 16,000 infants in our country have some cardiac malformation. In patients with an uncorrected left-to-right shunt, increased pulmonary pressure leads to vascular remodeling and endothelial dysfunction secondary to an imbalance in vasoactive mediators which promotes vasoconstriction, inflammation, thrombosis, cell proliferation, impaired apotosis and fibrosis. The progressive rise in pulmonary vascular resistance and increased pressures in the right heart provocated reversal of the shunt may arise with the development of Eisenmenger' syndrome the most advanced form de Pulmonary arterial hypertension associated with congenital heart disease. The prevalence of Pulmonary arterial hypertension associated with CHD has fallen in developed countries in recent years that is not yet achieved in developing countries therefore diagnosed late as lack of hospital infrastructure and human resources for the care of patients with CHD. With the development of targeted medical treatments for pulmonary arterial hypertension, the concept of a combined medical and interventional/surgical approach for patients with Pulmonary arterial hypertension associated with CHD is a reality. We need to know the pathophysiological factors involved as well as a careful evaluation to determine the best therapeutic strategy.

Unscheduled Hospitalization in Adults with Congenital Heart Disease

BACKGROUND AND OBJECTIVES: Little information is available regarding adult patients with congenital heart disease (CHD) who needed unscheduled hospitalization (USH). This paper aims to elucidate the clinical features of adult patients with CHD requiring USH. SUBJECTS AND METHODS: Study subjects included patients with CHD aged 18 years or older who were hospitalized at our facility during a 5-year study period. Medical records were retrospectively reviewed and data regarding USH were collected. Patient's background, underlying heart disease, cause of hospitalization, and prognosis (second USH regardless of cause or death) were examined. RESULTS: Overall, 959 CHD patients underwent a total of 1761 hospitalizations, including 145 patients who were unexpectedly hospitalized 239 times. The median age at USH was 27 years old. Of the 959 patients, 54% were male. Underlying heart diseases included repaired tetralogy of Fallot (21%), single ventricular physiology after Fontan operation (17%), and Eisenmenger syndrome (12%). The causes of USH included arrhythmia (40%), heart failure (20%), infectious disease (13%), and hemorrhage or thrombus (13%). A total of 48 patients required readmission. In total, 13 patients died, including four hospital deaths. The USH-free survival rate was 77% for 1 year and 58% for 3 years. CONCLUSION: The rate of USH was high for adults with complicated CHD. Common causes of USH included arrhythmia, heart failure, hemorrhage-related or thrombus-related conditions and infection. These data provide the current status of medical care for adult CHD patients in Japan and their therapeutic needs.

Management of cardiac arrest in a parturient with Eisenmenger's syndrome and complete atrioventricular block during Cesarean section: a case report / 대한마취과학회지

A 26-year-old parturient with Eisenmenger's syndrome and complete atrioventricular block was presented for emergency Cesarean section due to preterm labor. Ventricular tachycardia (VT), which progressed to ventricular fibrillation (VF), started immediately after the incision. Cardiopulmonary resuscitation with electric shocks was given by anesthesiologists while the obstetrician delivered the baby between the shocks. A cardiac surgeon was ready for extracorporeal membrane oxygenation institution in case of emergency but spontaneous circulation of the patient returned after the 3rd shock and the delivery of the baby. The newborn's Apgar score was 4 at 1 minute and 8 at 5 minutes. An implantable cardioverter-defibrillator was inserted before the discharge because the patient had recurrent episodes of VT and VF postoperatively.