Review of Eisenmenger Syndrome

Arvanitaki A, Gatzoulis MA, Opotowsky AR, et al.
Eisenmenger Syndrome: JACC State-of-the-Art Review. J Am Coll Cardiol 2022;79:1183-1198.

The following are key points to remember from this state-of-the-art review of Eisenmenger syndrome:

  1. Eisenmenger syndrome (ES) is defined as pulmonary hypertension at a systemic level, caused by a high pulmonary vascular resistance (PVR), with reversed or bidirectional shunt at the aortopulmonary, ventricular, or atrial level.
  2. ES is characterized by chronic hypoxemia and multiorgan involvement, including secondary erythrocytosis and increased thrombotic and bleeding risk, arrhythmias, increased infection risk, and progressive heart failure.
  3. Iron deficiency is common in ES, even in the setting of normal or elevated hemoglobin levels. Iron deficiency is caused by high iron requirements, reduced iron absorption, and blood loss. Iron supplementation can be administered safely and improves exercise capacity and quality of life.
  4. Routine venesections (phlebotomy) are not indicated for patients with ES and can cause or worsen iron deficiency and reduce oxygen delivery. Indications for phlebotomy include severely elevated hemoglobin with symptoms of hyperviscosity.
  5. Routine anticoagulation is not indicated for patients with ES. Anticoagulation should be reserved for selected patients with atrial arrhythmias or known pulmonary artery thrombus or embolism.
  6. Independent prognosticators for poor outcomes in ES include Down syndrome and pre-tricuspid shunt, older age, lower oxygen saturations, World Health Organization (WHO) functional class ≥III, reduced exercise capacity, elevated B-type natriuretic peptide, and iron deficiency.
  7. Advanced pulmonary arterial hypertension therapies have an important role in the management of patients with ES. The authors propose a staged approach beginning with monotherapy with an endothelin receptor antagonist or phosphodiesterase type 5 inhibitor. The next steps would include combination therapy, then triple therapy, and then assessment for lung or heart-lung transplantation.
  8. Pregnancy in women with ES has historically been associated with maternal mortality as high as 50%. Recent registries have reported maternal mortality of 10%, although morbidity remains extremely high, with 55% of women experiencing heart failure during pregnancy or the post-partum period. For this reason, women with ES should continue to be counseled against pregnancy.
  9. Patients require expert care at the time of noncardiac surgery, with the rate of major complications ranging from 5-25%. Expert anesthetic care and patient optimization are important in the perioperative period.
  10. The authors emphasize the importance of incorporating the patient’s perspective to enable patients to manage their condition in their day-to-day life and define their needs in a holistic fashion.

Clinical Topics: Anticoagulation Management, Arrhythmias and Clinical EP, Cardiac Surgery, Congenital Heart Disease and Pediatric Cardiology, Diabetes and Cardiometabolic Disease, Heart Failure and Cardiomyopathies, Invasive Cardiovascular Angiography and Intervention, Prevention, Pulmonary Hypertension and Venous Thromboembolism, Vascular Medicine, Implantable Devices, SCD/Ventricular Arrhythmias, Atrial Fibrillation/Supraventricular Arrhythmias, Cardiac Surgery and Arrhythmias, Cardiac Surgery and CHD and Pediatrics, Cardiac Surgery and Heart Failure, Congenital Heart Disease, CHD and Pediatrics and Arrhythmias, CHD and Pediatrics and Interventions, CHD and Pediatrics and Prevention, Acute Heart Failure, Heart Failure and Cardiac Biomarkers, Heart Transplant, Pulmonary Hypertension, Interventions and Structural Heart Disease, Interventions and Vascular Medicine, Hypertension

Keywords: Anticoagulants, Arrhythmias, Cardiac, Eisenmenger Complex, Endothelin Receptor Antagonists, Exercise Tolerance, Heart Defects, Congenital, Heart Failure, Heart-Lung Transplantation, Hemoglobins, Hypertension, Pulmonary, Iron, Maternal Mortality, Natriuretic Peptide, Brain, Phlebotomy, Phosphodiesterase 5 Inhibitors, Postpartum Period, Pregnancy, Pulmonary Arterial Hypertension, Pulmonary Embolism, Quality of Life, Thrombosis, Vascular Resistance

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