Background
Eisenmenger syndrome is defined as pulmonary vascular obstructive disease which develops as a consequence of preexisting large left-to-right shunt such that pulmonary artery pressures approach systemic values and the direction of the flow becomes bidirectional or right-to-left. Congenital heart defects that can lead to Eisenmenger syndrome include “simple” defects like ASD, VSD, PDA or “complex” like AVSD, truncus arteriosus, aortopulmonary window and univentricular heart. Elevated pulmonary vascular resistance usually occurs in infancy, by the age of 2 years. Clinical symptoms (exercise intolerance, dyspnea) usually occur in second or third decade. After third decade deterioration of the functional capacity is often reported. Survival rate in patients with Eisenmenger syndrome is 77% – 15 years and 42% – 25 years. Most common modes of death are sudden cardiac death, congestive heart failure, haemoptysis, pregnancy, non-cardiac surgery and infectious causes (brain abscess and endocardidtis).
Case presentation
We present a case of a 41-year-old woman with ventricular septal defect and Eisenmenger syndrome. Congenital heart defect and Eisenmenger syndrome were diagnosed in early childhood. First time she was diagnosed in Department of Cardiac and Vascular Diseases in July 2005; then VSD and severe pulmonary arterial hypertension were confirmed in right heart catheterization (mean pulmonary artery pressure 90 mmHg, pulmonary vascular resistance 40 Wood units). At that time she was WHO functional class III; BNP level was 115 pg/ml and distance in 6 minute walking test was 310 m. She was given sitaxentan in dose of 100 mg daily in clinical trial STRIDE III. In December 2010 sitaxentan therapy was stopped because of the withdrawal of the drug from the market due to its hepatotoxicity. Finally in January 2011 she was administered bosentan in standard dose (2×125 mg). Since July 2005 to January 2011 she was stable, WHO functional class II. Also NT-proBNP level (ca 246 pg/ml) and distance in 6 minute walking test (ca 420 m) were stable. Since January 2011 she started to complain of abdominal pain, vaginal bleeding, dyspnoea and weakness. In lab tests iron-deficiency anemia was diagnosed (RBC – 7 150 000, Hgb – 13,6 g/dl, HcT 46%, MCV – 65 fL, MCH – 19 pg, MCHC – 29 g/dl, Fe 4,5 ug/dl). Iron supplementation was ordered. She was consulted by gynecologist, on the basis of physical examination and ultrasound of uterus, final diagnosis of the myoma of the uterus was established.
In year 2012 bleedings from uterus myomatosus and iron-deficiency anemia became more severe. Consulting gynecologist considered hysterectomy. But because of the presence of congenital heart defect and Eisenmenger syndrome surgical treatment is associated with very high risk of complications. The patient’s medical history was presented on the Meeting of the Center for Rare Cardiovascular Diseases where she was qualified to the surgical treatment – hysterectomy in epidural anesthesia. Before surgery biopsy of endometrium must be done to exclude malignant lesions.
Current guidelines
According to current guidelines surgery in patients with Eisenmenger syndrome is associated with high risk of complications (6 – 23%). In each case risk and benefit ratio must be carefully considered before final decision about surgery and require expertise. Iron-deficiency anaemia is also a very bad prognostic factor. Iron supplementation should be performed in the presence of iron deficiency (MCV ,80 fL) and carefully followed (rebound effect).
References
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Expert’s comments:(Written authorization required from each expert)
1. Prof. Andrzej Rudziński, MD, PhD
Surgery should be done soon, because the patient is in quite good and stable condition.
Surgery is associated with high risk, but anemia is a very bad prognostic factor in Eisenmenger syndrome.
2. Prof. Krzysztof Rytlewski MD, PhD
Surgery should be done soon, because the patient is in quite good and stable condition. Surgery is associated with high risk, but anemia is a very bad prognostic factor in Eisenmenger syndrome.
Authors:
Magdalena Kaźnica-Wiatr MD1, Magdalena Nowacka MD1, Grzegorz Kopeć MD, PhD1, Maria Olszowska MD, PhD1, Prof. Piotr Podolec MD, PhD1
Experts:
Prof. Andrzej Rudziński MD, PhD2, Prof. Krzysztof Rytlewski MD, PhD3
1Department of Cardiac and Vascular Disease in John Paul II Hospital, Institute of Cardiology, Faculty of Medicine, Jagiellonian University, Krakow, Poland.
2Department of Pediatric Cardiology, Children’s Hospital, Krakow, Poland.
3Department of Obstetrics and Perinatology, University Hospital, Krakow, Poland There is a shortage of experience in adult clinic.
