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.

Tratamiento de la compresión del tronco de la arteria coronaria izquierda en pacientes con hipertensión pulmonar / Treatment of compression of the left main coronary artery in patients with pulmonary hypertension

La angina de pecho es un síntoma frecuente en pacientes con hipertensión pulmonar (HP) de cualquier etiología. Aunque su fisiopatología no está aclarada, las causas propuestas son: la isquemia subendocárdica por aumento del estrés parietal del ventrículo derecho, la dilatación de la arteria pulmonar por incrementos transitorios de la presión pulmonar y la compresión extrínseca del tronco de la arteria coronaria izquierda (TCI) por la arteria pulmonar (AP) dilatada. Se presentan tres casos que muestran la relación entre la angina de pecho y la compresión del TCI en pacientes con HP asociada a cardiopatías congénitas, tratados mediante implante de stent coronario.

Chest pain is a frequent symptom in patients with pulmonary hypertension of any etiology. Its pathophysiology has not been clearly established, the proposed causes are ischemia due to increased right ventricle wall stress, transient increased pulmonary hypertension resulting in acute pulmonary artery dilatation and external compression of the left main coronary artery (LMCA) by a dilated pulmonary artery. We report and discuss here three cases where the association between chest pain and compression of the LMCA by a dilated pulmonary artery could be shown, and they were treated with coronary stenting.

Hipertensão pulmonar nas cardiopatias congênitas: implicações terapêuticas e prognósticas / Pulmonary hypertension in congenital cardiopathy therapeutic and prognostic implications

A Hipertensão arterial pulmonar (HAP) é uma importante complicação na evolução das cardiopatias congênitas de hiperfluxo pulmonar. A síndrome de Eisenmenger (SE) é o espectro máximo desta condição e se caracteriza por inversão do shunt com cianose e irreversibilidade até o presente momento. Nos últimos anos, novas opções terapêuticas se expandiram e a terapia avançada (TA), com o uso de vasodilatadores pulmonares, tornou-se uma opção atraente para estes pacientes. Estudos recentes têm demonstrado melhora da qualidade de vida e capacidade física desta população em uso de TA. Entretanto, a melhor forma de tratamento ainda é a prevenção pela correção cirúrgica da cardiopatia na época adequada. A presente revisão tem como objetivo abordar o prognóstico e tratamento na HAP associada a cardiopatias congênitas na atualidade e discutir sobre futuras abordagens terapêuticas, como a possibilidade de posterior correção cirúrgica de pacientes em TA, antes considerados inoperáveis.

Hemi-central retinal artery occlusion in young adults.

Amongst the clinical presentations of retinal artery occlusion, hemi-central retinal artery occlusion (Hemi-CRAO) is rarely described. This case series of four adults aged between 22 and 36 years attempts to describe the clinical profile, etiology and management of Hemi-CRAO. Case 1 had an artificial mitral valve implant. Polycythemia and malignant hypertension were noted in Case 2. The third patient had Leiden mutation while the fourth patient had Eisenmenger’s syndrome. Clinical examination and fundus fluorescein angiography revealed a bifurcated central retinal artery at emergence from the optic nerve head, in all cases. Color Doppler examination of the central retinal artery confirmed branching of the artery behind the lamina cribrosa. It is hypothesized that bifurcation of central retinal artery behind the lamina cribrosa may predispose these hemi-trunks to develop an acute occlusion if associated with underlying risk factors. The prognosis depends upon arterial recanalisation and etiology of the thromboembolic event.

Abortamento terapêutico em gestante com comunicação interatrial e hipertensão arterial pulmonar grave complicadas com síndrome de Eisenmenger / Therapeutic abortion in pregnant woman with atrial septal defect and pulmonary arterial hypertension complicated with Eisenmenger syndrome

Este relato descreve a evolução de gestante, de 25 anos de idade, com síndrome de Eisenmenger, há 15 anos com hipertensão arterial pulmonar grave. A gravidez associou-se com agravamento de insuficiência cardíaca (IC). O abortamento terapêutico foi indicado após consentimento unânime da equipe multidisciplinar do Hospital das Clínicas da Universidade Federal de Minas Gerais (UFMG), com aspiração manual intrauterina (AMIU) na oitava semana gestacional. A paciente recebeu alta com prescrição contraceptiva e esclarecimentos a respeito da necessidade da salpingotripsia bilateral.

This article reports a case of a pregnant 25-year-old woman diagnosed with atrial septal defect (ASD) and severe pulmonary arterial hypertension since the age of 8, complicated in the course of the disease with Eisenmenger syndrome. After getting pregnant, the patient developed clinical decompensation. It was indicated and performed, under unanimous consent of the multidisciplinary team at the Hospital das Clínicas of UFMG, therapeutic abortion by the technique of manual vacuum aspiration (MVA) in the eighth week of gestation. The patient was discharged with a prescription of injectable contraceptive and clarified about the need of tubal sterilization.

Anestesia general para cirugía no cardiaca en un paciente con síndrome de Eisenmenger / General anesthesia for noncardiac surgery in patients with Eisenmenger syndrome

El Síndrome de Eisenmenger es un cuadro clínico caracterizado por hipertensión pulmonar y cortocircuito de derecha a izquierda, generalmente secundario a cardiopatías congénitas no solucionadas oportunamente. La mortalidad perioperatoria para cirugía no cardiaca en la edad adulta de este tipo de pacientes puede alcanzar valores cercanos a 20%. Se presenta el caso de una paciente adulta, portadora de síndrome de Eisenmenger sometida a cirugía radical de oído bajo anestesia general. Se revisa la literatura y se discuten algunas implicancias para el anestesiólogo.

The Eisenmenger's Syndrome can be defined as a clinical condition with pulmonary hypertension, and right to left shunt, due to unsolved congenital heart diseases. Perioperative mortality if undergo non cardiac surgery in the adulthood, can be as high as 20%. We report a case of an adult patient with Eisenmenger's syndrome who underwent ear surgery under general anesthesia. A review of the literature is presented. Specific anesthesia procedures are suggested.

Síndrome de Eisenmenger na gravidez / Eisenmenger syndrome in pregnancy

A síndrome de Eisenmenger consiste em hipertensão pulmonar com shunt reverso ou bidirecional ao nível atrioventricular ou aortopulmonar. Na gestação, essa síndrome está associada com altas taxas de mortalidade (30 a 50 por cento). Normalmente, a hipertensão pulmonar é agravada durante a gestação, levando a um resultado desfavorável. Neste artigo, relatamos um caso de gestante portadora de síndrome de Eisenmenger com boa evolução materna e do recém-nascido.

Eisenmenger's syndrome consists of pulmonary hypertension with a reversed or bidirectional shunt at the atrioventricular, or aortopulmonary level. Eisenmerger's syndrome in pregnancy is usually associated with high mortality rates (nearly 30-50 percent). Unfortunately, pulmonary hypertension is aggravated during pregnancy and often leads to an unfavorable outcome. Here, we report a successful pregnancy in a woman with Eisenmenger syndrome.

Hipertensão arterial pulmonar associada a cardiopatias congênitas / Pulmonary hypertension in congenital heart disease

A hipertensão arterial pulmonar é frequentemente encontrada nas cardiopatias congênitas associadas a hiperfluxo pulmonar secundário a grandes shunts da esquerda para a direita. A hipertensão pulmonar é um fator complicador importante no tratamento desses pacientes, podendo impedir a correção cirúrgica da cardiopatia naqueles com doença vascular pulmonar já estabelecida. O diagnóstico precoce e o conhecimento da história natural do defeito cardíaco usualmente levam à prevenção da doença vascular pulmonar, por permitir seu fechamento em tempo adequado. Nos pacientes com doença vascular pulmonar irreversível, o tratamento clínico convencional continua indicado, embora novos medicamentos demonstrem seus efeitos potenciais sobre variáveis hemodinâmicas e sobrevida. Após a falência ao tratamento clínico otimizado, existe a opção de transplante pulmonar.

General Anaesthesia for emergency caesarian section in a patient with Eisenmenger's Syndrome and Pre-eclampsia

Pregnancy is poorly tolerated in patients with Eisenmenger syndrome [ES] with maternal mortality of 30-50%. Physiological changes of pregnancy decreases systemic vascular resistance that further aggravates the bi-directional or right to left shunt associated with ES. When it occurs with eclampsia, the morbidity and mortality are even higher. We report a case of 30 weeks pregnant woman with ES, who underwent emergency caesarian section because of pre-eclampsia. The intra-operative course was uneventful but she died on the second post-operative day. Post-operatively she was managed by the cardiologist in the coronary care unit. The probable cause being that she was over transfused, as the fluid status was not assessed by any invasive monitoring [like CVP]. It was concluded that patients should be monitored closely in the post-operative period in the intensive care unit with complete invasive monitoring for up to a week to prevent factors resulting in worsening of the shunt [such as fluid balance] and thromboembolic phenomenon

Large thrombus in the pulmonary arteries in a case of atrial septal defect-Eisenmenger syndrome.

A 41-year-old man with atrial septal defect-Eisenmenger syndrome presented with progressively worsening breathlessness. The echocardiogram, computerized tomographic scan and magnetic resonance imaging of the thorax showed dilated pulmonary arteries and large thrombi in the right and left pulmonary arteries. Contrast-enhanced computerized tomographic scan was better than magnetic resonance imaging in picking up the thrombus. The possibility of in situ thrombus formation was considered more likely than thromboembolism, as there were none of the acute symptoms expected with the embolization of such large thrombi.