Pregnancy with Eisenmenger Syndrome: A Challenge to Obstetrician

Eisenmenger syndrome is defined as the development of pulmonary hypertension in response to a left-to-right cardiac shunt with consequent bidirectional or reversal (right-to-left) of shunt flow. Maternal mortality in the presence of Eisenmenger syndrome is reported to be 30-50%. If the patient continues her pregnancy against advice, a well-coordinated multidisciplinary team approach is advocated. Here, we report a case of pregnancy with Eisenmenger syndrome and its successful outcome.

Disfunción ventricular derecha en paciente adulto con comunicación interventricular posinfarto / Right ventricular dysfunction in adult patient with post-infarction ventricular septal defect

RESUMEN Introducción: La disfunción ventricular derecha es la alteración aguda o crónica de la morfología y función de dicha estructura, de origen multifactorial. En los últimos años existe consenso acerca de su función como determinante del estado funcional y factor pronóstico en diversas enfermedades. Objetivo: Destacar la importancia del manejo multidisciplinario y algunas de las opciones terapéuticas que se les pueden brindar a estos pacientes. Presentación del caso: Paciente de 58 años de edad, con antecedentes de salud aparente, que acudió al Servicio de emergencias, donde se le diagnosticó un infarto agudo de miocardio de topografía inferior. Se trasladó a la unidad de cuidados intensivos quirúrgicos del Servicio de Cirugía Cardiovascular debido a una complicación mecánica por comunicación interventricular. Se recibió con elementos clínicos de insuficiencia cardíaca aguda clase funcional IV. Progresivamente presentó deterioro del estado hemodinámico por lo que requirió el uso de apoyo vasoactivo con norepinefrina; hipoxia, hipercapnia, presiones venosas centrales entre 15 y 18 cmH2O y elementos clínicos de disfunción de ventrículo derecho; se decidió instrumentar vía aérea, administrar sedación y relajación por asincronías a paciente ventilado. Sin otra opción que el tratamiento quirúrgico se llevó a discusión donde se decidió esperar a la mejoría del estado hemodinámico con tratamiento médico. Conclusiones: Las enfermedades cardiovasculares no dejan de aparecer en escenarios cada vez más complejos con elevada morbimortalidad. Se evidencia la importancia del consenso clínico-quirúrgico en el momento de la toma de decisiones.

ABSTRACT Introduction: Right ventricular dysfunction is the acute or chronic alteration of the morphology and function of this structure, of multifactorial origin. In recent years there is consensus about its role as a determinant of functional status and prognostic factor in various diseases. Objective: Highlight the importance of multidisciplinary management and some of the therapeutic options that can be provided to these patients. Case Presentation: A 58-year-old patient with an apparent health history went to the Emergency Service, where he was diagnosed with an acute myocardial infarction of lower topography. He was transferred to the surgical intensive care unit of the Cardiovascular Surgery Service due to a mechanical complication from ventricular septal defect. He was received presenting clinical elements of acute heart failure functional class IV. He progressively presented deterioration of the hemodynamic state, so it required the use of vasoactive support with norepinephrine; hypoxia, hypercapnia, central venous pressures between 15 and 18 cmH2O and clinical elements of right ventricular dysfunction; it was decided to implement airway, administer sedation and relaxation by asynchrony to ventilated patient. With no other option than surgical treatment, it was brought to discussion and it was decided to wait for the improvement of the hemodynamic state with medical treatment. Conclusions: Cardiovascular diseases do not cease to appear in increasingly complex scenarios with high morbidity and mortality. The importance of clinical-surgical consensus at the time of decision-making is evident.

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.

Síndrome de Eisenmenger en una paciente embarazada de 21 años / Eisenmenger syndrome in 21-year pregnant patient

Resumen ANTECEDENTES El síndrome de Eisenmenger es la cardiopatía congénita con mayor riesgo de mortalidad de las embarazadas. CASO CLÍNICO: Paciente primigesta de 21 años, con síndrome de Eisenmenger e insuficiencia cardiaca grado III NYHA e hipertensión arterial sistémica. El embarazo se interrumpió mediante cesárea a las 30 semanas, con recién nacido vivo de 1000 g, Apgar 3-7, 32 semanas de gestación por Capurro. Seis días después de la cesárea la paciente falleció debido a choque cardiogénico. El neonato falleció de insuficiencia respiratoria el décimo segundo día de vida extrauterina. CONCLUSIONES El caso aquí descrito permite reflexionar acerca de las oportunidades de mejora en la atención de las pacientes con síndrome de Eisenmenger. En el primer nivel de atención es importante centrarse en la educación y consejería específicas que permitan comprender a la paciente y su pareja los riesgos implícitos de su afección, pues la alternativa más segura hubiera sido evitar el embarazo. En el segundo nivel de atención ha de favorecerse siempre la práctica basada en evidencia y correcta coordinación en los equipos de salud que, en este caso particular, hubiera permitido que la paciente desde la semana 14 hubiera sido referida para atención altamente especializada que hubiese podido incrementar las probabilidades de supervivencia de ella y el neonato.

Abstract BACKGROUND Eisenmenger syndrome is congenital heart disease with a higher risk of mortality for pregnant women. CLINICAL CASE 21-years women with Eisenmenger syndrome, whose pregnancy was accompanied by class III NYHA heart failure and systemic arterial hypertension. Pregnancy was interrupted at 30 weeks by caesarean section. Six days after the caesarean section, the patient died due to cardiogenic shock. CONCLUSIONS The case described here allows us to reflect on the opportunities for improvement in the care of patients with Eisenmenger syndrome. At the first level of care, it is important to focus on specific education and counseling that allows the patient and her partner to understand the implicit risks of their condition, since the safest alternative would have been to avoid pregnancy. At the second level of care, the practice based on evidence and correct coordination in the health teams should always be favored, which in this particular case would have allowed the patient, since week 14, to have been referred for highly specialized care could increase the chances of survival of her and the neonate.

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)

Serum HMGB1 Levels and Its Clinical Significance in Patients with Idiopathic Pulmonary Arterial Hypertension / 中山大学学报(医学科学版)

[Objective]To estimate the clinical value of serum high mobility group box 1(HMGB1) as a biomarker of idiopathic pulmonary arterial hypertension (IPAH).[Methods]This study included 33 patients with IPAH that were confirmed by right heart catheter in the Second Xiangya Hospital, Central South University from May 2011 to April 2015. 8 patients with IPAH were followed up for 6 months during treating with PAH-specific pharmacotherapies. All the subjects ' clinical data were collected,HMGB1 levels were determined by enzyme linked immunosorbent assay(ELISA).[Results]Serum HMGB1 levels (ng/mL) were significantly increased in patients with IPAH compared with the control group(14.8 ± 2.4 vs. 3.8 ± 1.2, P<0.001);The serum HMGB1 levels were significant?ly positive correlation with mean pulmonary arterial pressure(MPAP) and pulmonary vascular resistauce (PVR) ( r=0.864, P<0.001; r=0.460,P=0.002) in the patients with IPAH. After treating with PAH-specific pharmacotherapies for 6 month, HMGB1 levels(ng/mL) were significantly decreased(15.9±5.3 vs 11.1±2.5,P=0.021)along with the patients'MPAP(62.3±9.7 vs 54.0±8.7,mmHg)and 6-min walk distances(m)improved(368±69 vs 401±55,P<0.001).[Conclusions]Our study suggested that serum HMGB1 may be used as a biomarker of treatment response to targeted therapy, and it will be used as a biomarker in the follow-up evaluation of patients with IPAH.

Analysis of pregnancy outcome of pregnant women with Eisenmenger syndrome / 中国医师进修杂志

Objective To explore the maternal and fetal outcomes and treatment strategies of pregnant women with Eisenmenger syndrome. Methods Datas of pregnant women with Eisenmenger syndrome from September 2009 to March 2017 were analyzed retrospectively. Results Three patients did not accept regular prenatal care. Two patients suffered from eclampsism. Two patients suffered from arrhythmia. One patient suffered from pulmonary infection. Four patients suffered from congestive heart failure. One patient had no complications. One patient died from pulmonary hypertension crisis within 72 h of postpartum. Four of the seven perinatal infants died from premature complications. Conclusions Patients with Eisenmenger syndrome have tendency to become heart failure and so are unsuitable for pregnancy. Once paitents have pregnancy, high mortality exists. So progestation counseling and management should be strengthened. Early pregnancy should be terminated. In mid or late trimester of pregnancy, regular prenatal care should be needed to help mothers and fetuses get better outcomes.

Analysis of pregnancy outcome of pregnant women with Eisenmenger syndrome / 中国医师进修杂志

Objective To explore the maternal and fetal outcomes and treatment strategies of pregnant women with Eisenmenger syndrome. Methods Datas of pregnant women with Eisenmenger syndrome from September 2009 to March 2017 were analyzed retrospectively. Results Three patients did not accept regular prenatal care. Two patients suffered from eclampsism. Two patients suffered from arrhythmia. One patient suffered from pulmonary infection. Four patients suffered from congestive heart failure. One patient had no complications. One patient died from pulmonary hypertension crisis within 72 h of postpartum. Four of the seven perinatal infants died from premature complications. Conclusions Patients with Eisenmenger syndrome have tendency to become heart failure and so are unsuitable for pregnancy. Once paitents have pregnancy, high mortality exists. So progestation counseling and management should be strengthened. Early pregnancy should be terminated. In mid or late trimester of pregnancy, regular prenatal care should be needed to help mothers and fetuses get better outcomes.

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.

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.

Eisenmenger syndrome in a patient with ventricular septal defect: a case report.

Eisenmenger Syndrome (ES) represents Pulmonary Arterial Hypertension (PAH) associated with Congenital Heart Defects (CHD). Although patients survive until their third or fourth decades of life, the symptoms include dyspnea, cyanosis, fatigue, dizziness, and syncope. In addition, cardiac arrhythmias, a late complication are causing sudden death in patients with ES. Treatment options have been limited; however, recent successes have been achieved with the use of therapies targeted against the pathophysiological pathways that underlie PAH. The dual endothelin receptor antagonist and prostacyclins demonstrated to improve hemodynamics of the patients. This is the case of a 16 year old young female with ventricular septal defect that was admitted with increasing shortness of breath and cyanosis with clubbing which are clinical features of Eisenmenger syndrome. She was medicated with Furosemide, Sildenafil which improved her functional status.

A Rare Case of Heterotaxy Syndrome: Eisenmenger Syndrome with Dextrocardia in an Adult.

Aims: Heterotaxy syndrome is a rare, complex, and confusing presentation in the realm of situs anomalies. The concurrence of heterotaxy syndrome with dextrocardia and Eisenmenger syndrome has never been reported in an adult. We described, for the first time, an adult patient who had heterotaxy syndrome with dextrocardia, complicated by Eisenmenger syndrome with large ventricular septal defect (VSD). Presentation of Case: A 43 year-old female presented to the emergency room with worsening exertional dyspnea. She carried past medical history of cyanotic congenital heart disease since birth. Her physical examination findings were significant for hypoxia and clubbing with cyanotic fingers and toes, dextrocardia, and a 3/6-holosystolic murmur over the right lower sternal border. Laboratory investigation showed polycythemia andher chest x-ray showed dextrocardia. Echocardiography showed dextrocardia with a dilated left ventricle and a large VSD with left-to-right shunting. Computed tomography (CT) angiogram of the chest showed bilateral bi-lobed lungs with both main stem hyparterial bronchi, right-sided polysplenia, right-sided stomach with left-sided dominant liver. Discussion and Conclusion: Heterotaxy syndrome has a complex variation in clinic presentation. We illustrate the occurrence of heterotaxy syndrome, polysplenia type with Eisenmenger syndrome and dextrocardia. Recognition of this rare anomaly is important for establishing a diagnosis in a patient with multiple organ displacement.

Advances in the treatment of Eisenmenger syndrome / 临床儿科杂志

Eisenmenger syndrome (ES) was considered as a surgical contraindication in the past time. Only a few patients got lung transplantations or heart-lung transplantations due to lack of donors. Most of the patients had to choose conservative treatment to prolong their survival time. Recently, some clinical trials indicated targeted therapies in pulmonary arterial hypertension (PAH) were effective for the treatment of ES. This article reviewed the recent advances in this area.

Eisenmenger Syndrome - a Case Report.

Eisenmenger syndrome (ES), the most advanced form of pulmonary arterial hypertension associated with congenital heart disease, is a devastating condition that has a considerable impact on patient's life. Patients who develop ES typically exhibit one or more of a range of cardiac defects including ventricular septal defects (VSD), atrial septal defects (ASD) and patent ductus arteriosus (PDA). The nature of the congenital defect underlying ES is important because it has prognostic implications. Early diagnosis & treatment of cardiac defect before development of pulmonary hypertension is the key to definite management, otherwise only supportive management can be offered. Our patient Mr. Abdul Khaleque, 55 years old, businessman, non diabetic, non hypertensive admitted in DAMCH, cardiac unit with the complaints of shortness of breath on exertion since childhood, bluish discoloration of nails and toes for 10 years, headache and vertigo for 2 weeks. Initially it was during severe exertion and relieved by rest. Gradually the severity of breathlessness has increased. Now he has developed NYHA class III. He had history of repeated attacks of cough with expectoration of mucoid sputum with occasional scanty amount of blood which did not foul smell and did not change with posture. We diagnose him as Eisenmenger syndrome secondary to ASD and treat him with only medical measure.

Hipertensión arterial pulmonar (síndrome de Eisenmenger) en el embarazo: Presentación de caso / Pulmonary arterial hypertension (Eisenmenger´s syndrome) in pregnancy: Case report

El síndrome o complejo Eisenmenger (hipertensión arterial pulmonar asociada a cardiopatía congénita) es una entidad poco frecuente en la población y en la mujer gestante, describiéndo se una alta mortalidad materno fetal (20 al 40%) y complicaciones obstétricas relacionadas con la entidad: amenaza de parto pretérmino, restricción del crecimiento fetal, insuficiencia placentaria, entre otras. Este artículo describe el caso de una mujer en segundo trimestre de embarazo con síndrome de Eisenmenger, diagnosticada y manejada en un hospital de cuarto nivel colombiano, con el fin de revisar la literatura al respecto, pero, de especial interés, con el fin de compartir la experiencia en el manejo médico de esta situación tan compleja y de baja prevalencia. Este ejemplo puede resultar útil a nivel local y nacional para otros profesionales de la salud que se vean enfrentados a manejar una mujer en estado de gravidez con síndrome de Eisenmenger.

Eisenmenger complex (pulmonary arterial hypertension associated with congenital heart disease) is a rare entity in the individual population and in pregnant women, describing a high maternal and fetal mortality (20 to 40%) and it’s well known obstetric complications related entity: preterm delivery, intrauterine growth restriction, placental insufficiency, etc. We describe the case of a pregnant woman in the second trimester with Eisenmenger´s syndrome who was monitored, diagnosed and managed by high complex hospital, in order to review scientific literature and illustrate the experience of medical management of Eisenmenger´s Syndrome during the pregnancy.

Can PDA patient in early stage of Eisenmenger syndrome accept interventional surgery ? / 中国介入心脏病学杂志

Objective To explore whether the PDA patients in the early stage of Eisenmenger syndrome can accept interventional surgery. Methods Three patients were choose from the “National Multicenter Prospective Registration Study on Pulmonary Artery Hypertension”, Clinical assessment, as well as examinations and registrations associated with PAH, was conducted in accordance with relevant provisions of the registration study within 1 wk prior to surgery. The way of the interventional treatment were right heart catheterization and pulmonary vasodilator testing (a capsule of iloprost solution for inhalation), and measurement of the pulmonary arterial pressure, descending aortic pressure, ratio of pulmonary to systemic blood flow, ratio of pulmonary to systemic blood pressure, pulmonary capillary wedge pressure [mean≤15 mmHg(1 mmHg=0.133 kPa)], before and after testing. After clear the results of pulmonary vasodilator test all patients underwent transcatheter closure testing, if it was positive,the amplatzer was released if her family members’ approved;Conversely, the amplatzer was quickly withdrawn with negative results, and symptomatic treatment was conducted based on the patient’s clinical symptoms. Results The first cases pulmonary vasodilator test was negative, but positive in transcatheter closure testing. Satisfaction immediate effect of surgery, and the occluder was released with her family members’ approval. patients of Example 2 and Example 3 were negative in pulmonary vasodilator testing and transcatheter closure testing, can not released the occluder. Conclusions Some Patents Ductus Arteriosus (PDA) patients in early stage of Eisenmenger syndrome is feasible to accept interventional therapy , try plugging test is may be an important indicator of prognostic assessment.

Síndrome de Eisenmenger en una paciente de la tercera edad / Eisenmenger syndrome in an elderly patient

Se describe el caso clínico de una paciente de 62 años de edad con cardiopatía congénita cianótica, quien fue ingresada en el Servicio de Cardiología del Hospital Provincial Docente Clinicoquirúrgico "Saturnino Lora Torres" de Santiago de Cuba por presentar disnea. Al examen físico se constató cianosis, arritmia completa y soplo sistólico en mesocardio, con signos de insuficiencia cardíaca refractaria del lado derecho. Los exámenes complementarios efectuados confirmaron el diagnóstico de síndrome de Eisenmenger atribuible a comunicación interventricular. A pesar de la medicación que recibía evolucionó desfavorablemente hasta que falleció. Es poco frecuente que pacientes con esta complicación sobrevivan hasta la tercera edad.

A case report of a 62 year-old patient with cyanotic congenital heart disease, who was admitted to the Cardiology Department of "Saturnino Lora" Provincial Teaching Clinical Surgical Hospital of Santiago de Cuba to present with shortness of breath was described. On physical examination cyanosis, complete arrhythmia and systolic murmur in mesocardium with signs of refractory heart failure of the right side were found. Complementary tests confirmed the diagnosis of Eisenmenger syndrome attributable to ventricular septal defect. Despite receiving medication she made bad progress and died. It is rare for patients with this complication survive into old age.

A Case of Patient with Eisenmenger Syndrome Admitted to Intensive Care Unit Due to Septic Shock Complicated with Newlydiagnosed Down Syndrome and Moyamoya Disease / 대한구급학회지

Eisenmenger syndrome is a severe form of pulmonary arterial hypertension related to congenital cardiac defects. Many patients die at a young age from such complications. The treatment of primary pulmonary hypertension is being applied to Eisenmenger syndrome such as endothelin receptor antagonists, phosphodiesterase-5 blockers, and prostacyclin. We experienced a case of 29-year female with ventricular septal defect-related Eisenmenger syndrome complicated with Down syndrome and Moyamoya disease, who was admitted to intensive care unit due to enteritis-associated septic shock. After the combination treatment with iloprost and sildenafil within the intensive care unit, the patient was able to wean mechanical ventilation without further applications of invasive rescue therapy such as extracorporeal membrane oxygenator. She was later discharged with bosentan. She maintained bosentan therapy for 34 months continuously without aggravations of symptom but eventually died with intracranial hemorrhage, a complication of Moyamoya disease. To our knowledge, this is the first case report of Eisenmenger syndrome accompanied by mosaic Down syndrome and Moyamoya disease.