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.
Female ; Humans ; Pregnancy ; Cesarean Section ; Eisenmenger Complex/therapy* ; Hypertension, Pulmonary/therapy* ; Maternal Mortality ; Pregnancy Complications, Cardiovascular/therapy* ; Pregnancy Outcome

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.
Arrhythmias, Cardiac ; Critical Care ; Eisenmenger Complex ; Female ; Fetal Mortality ; Fetus ; Heart ; Heart Arrest ; Hemorrhage ; Humans ; Hypertension ; Hypertension, Pulmonary ; Maternal Age ; Mothers ; Obstetric Labor, Premature ; Peripartum Period ; Pneumonia ; Postpartum Period ; Pregnancy ; Pregnant Women ; Sildenafil Citrate ; Urinary Tract Infections

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.
Adult ; Aneurysm ; Autopsy ; Cardiac Tamponade ; Death, Sudden ; Eisenmenger Complex ; Humans ; Hypertension ; Hypertension, Pulmonary ; Pulmonary Artery ; Rupture

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.
Anesthesia ; Anoxia ; Arterial Pressure ; Arthroplasty ; Arthroplasty, Replacement, Hip* ; Dobutamine ; Dopamine ; Eisenmenger Complex* ; Etomidate ; Female ; Femoral Neck Fractures ; Heart Arrest ; Heart Septal Defects, Atrial ; Humans ; Hypertension, Pulmonary ; Hypotension ; Intensive Care Units ; Middle Aged ; Norepinephrine ; Respiration ; Vascular Resistance

No abstract available.
Eisenmenger Complex* ; Humans ; Pulmonary Artery*

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.
Anesthesia, General ; Anesthetics, Local ; Anxiety ; Cyanosis ; Death, Sudden ; Deep Sedation* ; Down Syndrome* ; Dyspnea ; Eisenmenger Complex* ; Epinephrine ; Fatigue ; Hemoptysis ; Humans ; Hypertension ; Propofol

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.
Adult ; Apgar Score ; Atrioventricular Block* ; Cardiopulmonary Resuscitation ; Cesarean Section* ; Defibrillators, Implantable ; Eisenmenger Complex* ; Emergencies ; Extracorporeal Membrane Oxygenation ; Female ; Heart Arrest* ; Humans ; Obstetric Labor, Premature ; Pregnancy ; Shock ; Tachycardia, Ventricular ; Ventricular Fibrillation

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.
Adult* ; Aging ; Arrhythmias, Cardiac ; Communicable Diseases ; Eisenmenger Complex ; Fontan Procedure ; Heart Defects, Congenital* ; Heart Diseases ; Heart Failure ; Hemorrhage ; Hospitalization* ; Humans ; Japan ; Male ; Medical Records ; Physiology ; Prognosis ; Retrospective Studies ; Survival Rate ; Tetralogy of Fallot ; Thrombosis

No abstract available.
Anesthesia, General* ; Cesarean Section* ; Eisenmenger Complex* ; Emergencies* ; Female ; Humans ; Pregnancy ; Pregnant Women*

No abstract available.
Anesthesia, General* ; Cesarean Section* ; Eisenmenger Complex* ; Emergencies* ; Female ; Humans ; Pregnancy ; Pregnant Women*