Síndrome de Eisenmenger secundario a comunicación interatrial tipo ostium secundum en mujer adulta / Eisenmenger Syndrome Associated with an ostium secundum Atrial Septal Defect in an Adult Woman

El síndrome de Eisenmenger es la forma más severa de presentación de hipertensión arterial pulmonar secundaria a defectos cardíacos congénitos no reparados, aunque su prevalencia es baja, continúa siendo un reto para los sistemas de salud de los países en vías de desarrollo por su complejidad en el manejo. Presentación del caso. Paciente femenina sin antecedentes médicos conocidos quien consulta por disnea relacionada a los esfuerzos y policitemia. Intervención terapéutica. Se realiza ecocardiograma transesofágico que arroja la presencia de defecto interatrial tipo ostium secundum e hipertensión arterial pulmonar severa, con cortocircuito de derecha a izquierda, se inicia oxigenoterapia y terapia farmacológica. Evolución clínica. Paciente permaneció ingresada presentando notable mejora a la disnea, se le dio de alta con referencia a la clínica de cardiopatías congénitas del adulto en Hospital Nacional Rosales.

Eisenmenger syndrome is the most severe form of pulmonary arterial hypertension secondary to an unrepaired congenital heart disease. Despite the low prevalence, it remains a challenge for the public health service of developing countries due to the complexity of the treatment. Case presentation. A female patient without known medical history, who consults with dyspnea on exertion and polycythemia. Treatment. A transesophageal echocardiogram was performed, showing an ostium secundum atrial septal defect and severe pulmonary arterial hypertension with a right-left shunt. Supplemental oxygen was administrated and pharmacological treatment was started. Outcome. The patient presented remarkable clinical improvement to dyspnea, she was discharged with medical reference to the Adult Congenital Heart Disease clinic at Rosales National Hospital.

Endodontic treatment as a Reduction in the Risk of infective Endocarditis in a Male Patient with Eisenmenger Syndrome

This case report aimed to describe the importance of endodontic treatment in reducing infectious foci in patients with Eisenmenger syndrome (ES) and describe the characteristics of ES, so that the endodontist can safely treat these patients. A 57-year-old male with ES sought dental care complaining of dental pain. Irreversible pulpitis was diagnosed in tooth 37 and pulp necrosis in teeth 36, 34 and 31. Tests of prothrombin time (PT), activated partial thromboblastin time (APTT) and international normalized ratio (INR) were prescribed to evaluate the profile of coagulation using Marevan and antibiotic prophylaxis with amoxicillin. The endodontic treatments were performed. At the end, the patient reported no pain or discomfort in the teeth and improved masticatory function. The removal of oral infectious foci in patients with ES is important to reduce the risk of IE, which could seriously compromise the health and overall prognosis of the patient.

Este reporte de caso tuvo como objetivo describir la importancia del tratamiento endodóntico en la reducción de focos infecciosos en pacientes con síndrome de Eisenmenger (SE) y describir las características del SE, para que el endodoncista pueda tratar con seguridad a estos pacientes. Un hombre de 57 años con ES buscó atención dental quejándose de dolor dental. Se diagnosticó pulpitis irreversible en el diente 37 y necrosis pulpar en los dientes 36, 34 y 31. Se solicitaron pruebas de tiempo de protrombina (PT), tiempo de tromboblastina parcial activada (TTPA) y índice internacional normalizado (INR) para evaluar el perfil de coagulación utilizando Marevan y profilaxis antibiótica con amoxicilina. Se realizaron los tratamientos de endodoncia. Al final, el paciente no refirió dolor ni molestias en los dientes y mejoró la función masticatoria. La eliminación de focos infecciosos orales en pacientes con ES es importante para reducir el riesgo de EI, que podría comprometer gravemente la salud y el pronóstico general del paciente.

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.

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.

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.

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.

Therapeutic effect and safety of ambrisentan on Eisenmenger syndrome / 心血管康复医学杂志

Objective:To observe therapeutic effect of ambrisentan on Eisenmenger syndrome (ES) .Methods:A total of 79 ES patients ,who hospitalized in Wuhan Asia Heart Hospital from Apr 2011 to Mar 2014 ,were divided into ambrisentan group (n=38 ,received digoxin combined ambrisentan treatment) and routine treatment group (n=41 , received digoxin treatment) ,and both groups were treated for 12 weeks .The 6min walking distance (6MWD) , WHO functional class (WHO FC) of pulmonary hypertension etc .were observed in both groups before and 12-week after treatment .Changes of hemodynamic parameters ,including mean pulmonary artery pressure (mPAP) ,total pulmonary resistance (TPR) and ratio of pulmonary circulating flow to system circulating flow (Qp/Qs) etc .,were observed in ambrisentan group before and after treatment . Results:Compared with before treatment , after 12 weeks ,6MWD significantly rose ,TPR significantly reduced ,Qp/Qs significantly increased and WHO FC signifi-cantly improved in ambrisentan group ,P<0.05 or <0.01. Compared with routine treatment group after 12 weeks , there was significant rise in 6MWD [ (356.8 ± 74.1) m vs .(393.7 ± 69.2) m] ,significant reduction in BNP level [(832.5 ± 422.2) ng/L vs .(646.6 ± 297.1) ng/L] and significant improvement in WHO FC (class I/II ,67.5% vs . 86.8% ) in ambrisentan group ,P<0.05 or <0.01. No patient died ,the adverse reactions were mild in ambrisentan group ;one case died in routine treatment group .Conclusion:Oral ambrisentan can significantly improve heart func-tion and exercise capacity in patients with Eisenmenger syndrome ,and it possesses good safety and tolerance .

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.

Defecto de septación auricular: un desafío para el cardiólogo de adultos / Atrial septal defect: a challenge for the adult's cardiologist

Fundamento: el defecto de septación auricular, también conocido con el término de comunicación interauricular es la cardiopatía congénita más frecuente en la edad adulta con predominio en el sexo femenino sobre el masculino. Muchos de los niños y jóvenes con comunicación interauricular se encuentran asintomáticos y los hallazgos físicos son insignificantes, por lo que la supervivencia hasta la edad adulta es la norma. Objetivo: profundizar en los diversos aspectos anatómicos, fisiopatológicos y clínicos de los pacientes adultos con defecto de septación auricular, con vistas a facilitar un diagnóstico precoz y un manejo adecuado por sus médicos de asistencia. Desarrollo: se realizó una revisión bibliográfica acerca del tema en las que se utilizaron las bases de datos de Medline e Hinari. La sobrecarga volumétrica crónica de las cavidades derechas puede tener efectos deletéreos, tales como arritmias auriculares, enfermedad vascular pulmonar irreversible y, eventualmente insuficiencia cardiaca y embolismos paradójicos. La ecocardiografía transesofágica constituye la técnica de elección para evaluar la localización anatómica de la comunicación interauricular y su relación con las estructuras vecinas. Conclusiones: el diagnóstico de una comunicación interauricular se realiza a partir de criterios clínicos, ecocardiográficos y angiográficos. El cierre de una comunicación interauricular ostium primum y seno venoso se realiza exclusivamente a través de la vía quirúrgica. La vía de elección para el cierre de una comunicación interauricular ostium secundum, siempre y cuando cumpla con determinados criterios, es la percutánea donde el Amplatzer septal occluder el dispositivo más utilizado. El cierre de un defecto septal auricular esta contraindicado en pacientes con hipertensión pulmonar secundaria también denominada fisiología de Eisenmenger.

Background: atrial septal defect also known as atrial communication is the most frequent congenital cardiopathy in adults with marked prevalence in women. Many of children and young people with atrial septal defects are asymptomatic and physical findings are insignificant, that´s why survival until the adulthood is the norm. Objective: to deepen knowledge on the diverse anatomical, psychopathological, clinical and diagnostic aspects of the adult’s patients with atrial septal defects with a view to provide an early diagnosis and an appropriate management by their primary physicians. Development: a bibliographic review on the subject was performed. Medline and HINARI databases were used. The chronic volumetric overdrive of the right cardiac cavities may cause deleterious effects such as atrial arrhythmias, irreversible lung vascular disease and, possibly heart failure and paradoxical embolisms. The transesophageal echocardiography constitutes the election technique to evaluate the anatomical localization of the atrial septal defect and its relationship with neighbour’s structures. Conclusions: the diagnosis of an atrial septal defect is carried out starting from clinical, echocardiography and angiography criteria. The closure of an atrial septal defect ostium primum and sinus venosus types is carried out exclusively through the surgical way. The closure of an atrial septal defect ostium secundum provided it meets certain criteria, is the percutaneous one, being the Amplatzer septal occluder device the most commonly used. The closure of atrial septal defect is contraindicated in patients with secondary pulmonary hypertension also called Eisenmenger physiology.

Clinical analysis of Eisenmenger syndrome during pregnancy / 中华围产医学杂志

Objective To investigate the outcome and the management of pregnant women with Eisenmenger syndrome during perinatal period to reduce the maternal fatality rate.Methods The clinical data of 23 pregnant women with Eisenmenger syndrome admitted into Beijing Anzhen Hospital Capital Medical University from January 1st,2001 to December 31st,2009 were retrospectively analyzed.Gestational week,primary disease of the heart,pulmonary hypertension,oxygen saturation and heart function of the patients were compared with Chi-square test or Fisher's exact test.Results (1) The gestational week of 23 patients with Eisenmenger syndrome were between 15 to 38 weeks,among which 11 cases in the midterm pregnancy and 12 in the last term pregnancy.The severity of pulmonary hypertension,heart function and pregnant outcomes have no difference in patients with differeut gestational weeks (P＞0.05).(2) Ventricular septal defect (VSD) were the most common disease (65.2％,15/23) among the four primary heart diseases of the patients.The heart function of the patients with VSD decreased most severely after pregnancy,and the fatality rate of the heart function Ⅳ was up to 73.3％ (11/15).The incidence of severe pulmonary hypertension was 20.0％ (3/15).The decreasing of the heart function of the patients with different primary heart diseases were different (P＜0.05).(3) Among the pulmonary hypertension patients,there were 7 mild cases (30.4％),12 moderate cases (52.2％) and 4 severe cases (17.4％).The severity of the pulmonary hypertension of the patients correlated to the incidence of postpartum heart failure and death (P＜ 0.05).The outcomes of four patients with severe pulmonary hypertension were the worst and all were reported to be dead (4/4).(4) Oxygen saturation related to the mortality of perinatal infants,incidence of premature birth and spontaneous abortion (P＜0.05).As oxygen saturation decreased to the lowest level,the fatality rate of perinatal infants increased to the highest (5.3 ％,1/19),so did the incidence of the premature birth (84.2％,16/19) and spontaneous abortion ( 10.5％,2/19).Conclusions The key point to increase the safety of patients with Eisenmenger syndrome during perinatal period was to strengthen monitoring,decrease the pulmonary hypertension and terminate pregnancy timely.

Perioperative Management of a Parturient with Eisenmenger's Syndrome Undergoing Cesarean Section :A case report

The Eisenmenger's syndrome is presented with an abnormal communication between the systemic and pulmonary circulations and a predominant right to left shunt caused by increased vascular resistance in the pulmonary circulation. The maternal mortality and morbidity rate associated with cesarean section in the presence of Eisenmenger's syndrome is very high. We report a successful anesthetic management of a patient with the Eisenmenger's syndrome who underwent emergency cesarean section under general anesthesia, which is the first case in Korea of using nitric oxide in an attempt to improve pulmonary hypertension and arterial oxygenation. In addition to the basic monitoring devices, arterial and pulmonary catheters were inserted before the anesthesia. A fall in blood pressure was immediately counteracted by the administration of norepinephrine, and loss of blood by transfusion and fluid. After the operation, patient was closely monitored at coronary care unit for 15 days and discharged 18 days after the delivery without complication.

Heart-lung transplantation for 4 patients with Eisenmenger’s syndrome / 中华器官移植杂志

Objective To summarize the experience of heart-lung transplantation.Methods Four patients with Eisenmenger’s syndrome underwent heart-lung homoplastic transplantation. All patients were complicated with severe pulmonary hypertension in New York Heart Association ( NYHA ) functional class IV. Cannulation for cardiopulmonary bypass consisted of a cannula in the high ascending aorta and separate vena caval cannulas. The heart-lung graft was moved into the chest, beginning with passage of the lung before the phrenic nerve pedicle. The bronchus was trimmed, leaving two cartilaginous rings proximal to the orifice of the upper lobe. The tracheal anastomosis was performed with a continuous 4-0 polypropylene suture, with the posterior portion continuously and anterior interrupted. The lungs were then ventilated (

Bilateral Sequential Single Lung Transplantation for Eisenmenger's Syndrome / 대한흉부외과학회지

Eisenmenger's syndrome is the disease of right to left shunt developing from the increased pulmonary vascular resistance caused by excessive pulmonary blood flow in patients with abnormal connections of systemic to pulmonary blood passage. The heart-lung transplantation was the only curative method in early transplantation period, but good results after bilateral lung transplantation have been reported as the fact that right heart function improved by only lung transplantation. We successfully carried out bilateral sequential single lung transplantation in a 34-year-old female patient with Eisenmenger's syndrome with large PDA. We report this case with a brief review of the literature.

Bilateral Sequential Single Lung Transplantation for Eisenmenger's Syndrome / 대한흉부외과학회지

Eisenmenger's syndrome is the disease of right to left shunt developing from the increased pulmonary vascular resistance caused by excessive pulmonary blood flow in patients with abnormal connections of systemic to pulmonary blood passage. The heart-lung transplantation was the only curative method in early transplantation period, but good results after bilateral lung transplantation have been reported as the fact that right heart function improved by only lung transplantation. We successfully carried out bilateral sequential single lung transplantation in a 34-year-old female patient with Eisenmenger's syndrome with large PDA. We report this case with a brief review of the literature.

Diagnosis and treatment of Eisenmenger's syndrome during pregnancy / 中华围产医学杂志

Objective To investigate clinical presentation, diagnosis,gestational outcome and treatment of Eisenmenger’s syndrome during pregnancy. Methods Two patients with Eisenmenger’s syndrome during pregnancy were reported retrospectively. Results The first case was admitted in labor at 34 weeks and 4 days in pregnancy,She had dysphoria,tachypnea and dyspnea right after vaginal delivery and died immediately The diagnosis of pulmonary embolism was highly suspected clinically. The second case was admitted because of fetal growth restriction Cesarean section was performed after cyanosis worsened at 35 weeks and 4 days She was discharged from the hospital two weeks after delivery. Conclusion Eisenmenger’s syndrome during pregnancy was associated with extremely poor maternal and fetal outcome Pregnancy is contraindicated in such patients Contraception is necessary after marriage Pregnancy should be terminated as early as possible in such women. In cases who intends to continue the pregnancy,closely observation must be provided to ensure the safety for both mother and the fetus.