eisenmenger syndrome: a clinical review

European Journal of Cardiovascular Nursing 8 (2009) 237–245www.elsevier.com/locate/ejcnurse

Review article

Eisenmenger syndrome: A clinical review

Philip Moons a,b,⁎, Mary M. Canobbio c,d, Werner Budts b

a Centre for Health Services and Nursing Research, Katholieke Universiteit, Leuven, Kapucijnenvoer 35 post box 7001, B-3000 Leuven, Belgiumb Division of Congenital and Structural Cardiology, University Hospitals of Leuven, Belgium

c Ahmanson UCLA Center for Adult Congenital Heart Disease, Los Angeles, CA, United States of Americad UCLA School of Nursing, Los Angeles, CA, United States of America

Received 31 March 2009; received in revised form 18 May 2009; accepted 25 May 2009Available online 21 June 2009

Abstract

Eisenmenger syndrome is a medical condition, characterized by elevated pulmonary vascular resistance and right-to-left shunting of bloodthrough a systemic-to-pulmonary circulation connection. Patients with Eisenmenger syndrome are not very prevalent in cardiovascularnurses' practice. However, nurses need to have some basic knowledge about the syndrome, in case they have to care for such a patient in theirclinical practice. In this article, we describe the epidemiology, outcome, pathophysiology, clinical presentation, medical management andcounseling issues of this condition.© 2009 European Society of Cardiology. Published by Elsevier B.V. All rights reserved.

Keywords: Congenital heart disease; Eisenmenger; Pulmonary arterial hypertension; Cardiovascular; Nursing

Contents

1. Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2382. Epidemiology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2383. Outcome and Long-term Survival . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2384. Pathophysiology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2395. Clinical presentation: signs and symptoms . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 239

5.1. Physical Signs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2395.1.1. Inspection. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2395.1.2. Palpation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2395.1.3. Auscultation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 239

5.2. Symptoms . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2396. Secondary erythrocytosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2397. Medical management . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 241

7.1. Conservative treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2417.1.1. Phlebotomy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2417.1.2. Iron deficiency . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2427.1.3. Drug treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2427.1.4. Oxygen . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 242

⁎ Corresponding author. Centre for Health Services and Nursing Research, Katholieke Universiteit, Leuven, Kapucijnenvoer 35 post box 7001, B-3000Leuven, Belgium. Tel.: +32 16 336984; fax: +32 16 336970.

E-mail address: [email protected] (P. Moons).

1474-5151/$ - see front matter © 2009 European Society of Cardiology. Published by Elsevier B.V. All rights reserved.doi:10.1016/j.ejcnurse.2009.05.004

mailto:[email protected]

http://dx.doi.org/10.1016/j.ejcnurse.2009.05.004

238 P. Moons et al. / European Journal of Cardiovascular Nursing 8 (2009) 237–245

7.1.5. Vaccination . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2427.1.6. Endocarditis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 242

7.2. Pulmonary vasodilating agents . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2427.3. Transplantation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 243

8. Counseling and Education . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2438.1. Quality of life and perceived health status . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2438.2. Gynecologic issues, Contraception and Pregnancy Management . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 243

8.2.1. Gynecologic Issues. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2438.2.2. Contraception . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2438.2.3. Pregnancy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 243

8.3. Physical Activity . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2448.4. Travel . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2448.5. Smoking. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 244

9. Conclusion . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 244References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 244

1. Introduction

Viktor Eisenmenger, an Austrian physician (1864–1932)described in 1897 a case of a man with cyanosis and exerciseintolerance since infancy who died from massive hemoptysisat the age of 32 years. Postmortem examination showed alarge ventricular septal defect with overriding aorta andsevere pulmonary vascular disease [1]. In the decadesthereafter, efforts were made to both describe and refine theinitial description of Eisenmenger. It wasn't until 1958 thatDr. Paul Wood broadened the anatomic concept of theEisenmenger complex and redefined to describe the under-lying pathophysiology as “pulmonary hypertension atsystemic level, due to a high pulmonary vascular resistance(N800 dynes-sec/cm5), with reversed or bidirectional shuntthrough a large ventricular septal defect (1.5–3 cm across)”[2]. Since then, the term Eisenmenger syndrome has beenused to describe a variety of cardiac lesions which arehemodynamically similar because of the presence ofpulmonary vascular disease and cyanosis resulting fromany systemic-to-pulmonary circulation communication witha left-to-right shunts, such as atrial septal defect, ventricularseptal defect, atrioventricular septal defect, patent ductusarteriosus, truncus arteriosus and single ventricle anomalies[3]. Eisenmenger physiology may also occur in patients withlarge surgically created extracardiac left-to-right shuntswhich initially cause increased pulmonary blood flow.

The purpose of this article is to provide a review of theepidemiology; outcome and long-term survival; patho-physiology, clinical presentation, secondary erythrocytosis;medical management; counseling issues and patient educa-tion in this condition.

2. Epidemiology

Precise data on the incidence of Eisenmenger syndrome isunavailable. It is estimated that between 5–10% of patientswith congenital heart disease develop pulmonary arterialhypertension (PAH) [4–7], while 1% to 4% develop

Eisenmenger syndrome [7,8]. The likelihood of developingthe Eisenmenger syndrome depends on the size and locationof the intracardiac defect [6]. For example, 3% of patientswith small or moderate-sized ventricular septal defectswould develop Eisenmenger syndrome, whereas approxi-mately 50% of infants with large ventricular septal defectsdevelop Eisenmenger syndrome if the defect remainsunrepaired [9]. In patients with unrepaired atrial septaldefect, only 10% develop Eisenmenger syndrome [6,10].Patients with patent ductus arteriosus or ventricular septaldefects have an earlier onset of Eisenmenger syndrome (80%during infancy) than do patients with atrial septal defects(90% during adulthood) [6]. In this latter group thepulmonary vascular injury may begin in infancy andchildhood, but may go unrecognized until the second orthird decade of life. While the incidence of Eisenmengersyndrome may be declining in the Western world, because ofthe increased number of afflicted patients migrating fromdeveloping countries, the prevalence may remain constant.

3. Outcome and Long-term Survival

It is now estimated that more than 50% of the patientswith Eisenmenger's reaction can reach the fifth decade of life[11,12], and although rare, patients living into the seventhdecade have been reported [13,14]. These rates of survivalare much better than previously reported [9,15,16]. Prog-nosis seems, however, to be dependent on the age ofdiagnosis. When diagnosis is made during adulthood, theestimated 10 year survival of patients is 58% [17]. Predictorsof mortality include functional class, history of arrhythmias,right ventricular dysfunction or hypertrophy, early age atpresentation, creatinine and responsiveness to inhaled nitricoxide [5,11,16,18]. Survival is worse in patients withcomplex lesions [16]. Sudden death, presumably fromarrhythmias, is the usual cause of death. Other causes ofdeath include heart failure, pneumonia, pulmonary infarctionfrom arterial thrombosis, and complications of cerebralabscesses and stroke [19].

239P. Moons et al. / European Journal of Cardiovascular Nursing 8 (2009) 237–245

4. Pathophysiology

Physiologically, the process begins in defects with bloodflow from the systemic to the pulmonary circulation. Theshunting occurs as a result of a pressure gradient in which thepressure in the systemic circulation is higher than the pressure inthe pulmonary circulation (Box 1). Additionally, the pulmonaryvascular resistance (PVR) is normally lower as compared tosystemic vascular resistance (SVA) (Box 1). Over time, theincreased flow through the pulmonary circulation leads to(initially reversible) morphological changes in the pulmonarymicrovasculature. These morphological alterations result in anincrease in the PVR, which is associated with development ofpulmonary arterial hypertension (mean pulmonary arterialpressure at rest N25mmHg). As the pulmonary arterial pressureapproaches or exceeds the systemic arterial pressure, the left-to-right shunt reverses into a right-to-left shunt. Consequently,deoxygenated (desaturated) blood mixes with oxygenated(saturated) blood, the patient becomes cyanotic and a chronichypoxemic state occurs. Cyanosis and chronic tissue hypox-emia result in altered organ metabolism, which is expressed inrenal, liver and bone marrow dysfunction [5].

The hematological response to chronic hypoxemia is asecondary erythrocytosis. Due to tissue hypoxia, erythropoie-tin production by the kidneys is triggered, resulting in anincrease in the number of circulating red blood cells andexpansion of cell blood volume. The presence of more redblood cells increases the oxygen-carrying capacity of the bloodand maintains an adequate oxygen supply to metabolizingtissues. This hematological response, however, produces anumber of adverse multi-systemic physiologic effects includ-ing hyperviscosity, bleeding diatheses (because of the lowerproduction of platelets, the presence of dysfunctional platelets,insufficient production of coagulation proteins), iron defi-ciency anemia (because of frequent phlebotomy), and severalmetabolic complications (because of low tissue flow).

5. Clinical presentation: signs and symptoms

5.1. Physical Signs

5.1.1. InspectionSymmetric central cyanosis with digital clubbing of

fingers and toes varies from mild to moderate and increasesover time [5]. When systemic vascular resistance drops

Mean arterial pressure 70–105 mmHgMean pulmonaryarterial pressure

12–16 mmHg

Pulmonary vascularresistance

50–300 dynes/min

Systemic vascularresistance

700–1400 dynes/min

Box 1Normal hemodynamic values.

suddenly, which occurs during brisk exercise or in heath, theright-to-left shunt aggravates and the patient becomes morecyanotic. The latter is particularly observed by the bluetongue. Many patients also show chest deformities related tothe heart defect. Patients may have, for example, prominentparasternal ribs because of dilated or hypertrophic right heart.

5.1.2. PalpationThe arterial pulse volume is commonly small or normal.

A right ventricular parasternal lift is present in patients withpulmonary arterial hypertension (right heart ictus): the leftventricular impulse is trivial or absent (left heart ictus). Adilated pulmonary trunk and pulmonary valve closure mightbe palpable at the upper left sternal border.

5.1.3. AuscultationThe first heart sound is normal whereas the second heart

sound is loud. In patients with ASD and VSD, the increasedflow of blood into the dilated pulmonary trunk, due to a strongleft-to-right shunt, might cause a soft systolic ejection murmur,heardmaximally at the left sternal border.When the left-to-rightshunt decreases because of flow reversal through the originaldefect, the ejection murmur will disappear. A high frequencydiastolic decrescendo murmur may be heard at the left sternalborder. This is the Graham Steel murmur of pulmonary valveregurgitation. A holosystolic murmur of tricuspid valveregurgitation may be present and increases with age.

5.2. Symptoms

Eisenmenger syndrome is associated with a myriad ofsymptoms. The most common complaint is progressiveexercise intolerance including shortness of breath withexertion, due to low systemic output [6]. Fatigue, chest painand syncope might occur, but are not specific for patients withthe Eisenmenger syndrome [5]. Palpitations are not uncom-mon and are, if pathological, most often due to atrialfibrillation or flutter [19]. Symptoms of heart failure generallydo not occur before the age of 30 years and are associated withright ventricular hypertrophy and dysfunction. The mainsymptoms of right-sided heart failure are peripheral edema andascites. In addition to the symptoms of the Eisenmengersyndrome, patients may experience symptoms associated withhyperviscosity, attributed to the associated erythrocytosis (seebelow). These symptoms are summarized in Box 2.

6. Secondary erythrocytosis

Secondary erythrocytosis is a constant feature of cyanoticcongenital heart disease and occurs as a result of an increasedproduction of erythropoietin by the kidneys. Secondaryerythrocytosis represents a compensatory or physiologicaladaptation to the chronic cyanosis and hypoxemia that isessential in maintaining adequate tissue oxygenation [20].As a result, patients with Eisenmenger syndrome presentwith elevated hemoglobin and hematocrit levels (Box 3).

Box 2Symptoms associated with hyperviscosity [22].

Reproduced from: Oechslin E. Hematological management of the cyanotic adult with congenital heartdisease. Int J Cardiol 2004; 97 (suppl. 1): 109–115 (permission provided by Elsevier).


Clinically, secondary erythrocytosis presents as compen-sated or decompensated erythrocytosis. Patients presentingwith compensated erythrocytosis have established equili-brium in an iron replete state; the hematocrits, however, canrange up to 70%; while ferritin values are in normal ranges(Box 3). In this group of patients, hyperviscosity symptomsare absent or mild. As long as hematocrits are below 70%,there is no increased risk for stroke. When, however,

hematocrits exceed 70%, red blood cells increasingly stacktogether in long chains, called ‘rouleaux formation’. In thisstate, there is an increased risk for developing a stroke [21].

By contrast, decompensated erythrocytosis comprises agroup of patients who fail to establish equilibrium with risinghematocrit levels and iron repletion state These patientsreport recurrent moderate to severe symptoms of hypervis-cosity (Box 2). Symptoms of hyperviscosity worsen if the

Hemoglobine (Hb)Males 13.5 – 18 g/dlFemales 12 – 16 g/dlHematocrit (Hct)Males 0.40 – 0.51 (40–51%)Females 0.38 – 0.44 (38–44%)Serum Ferritin 15–300 ng/mlSerum ironMales 65 – 17 µg/dlFemales 50 – 170 µg/dlMean cell volume(MCV)

80–100 fl

Box 3Normal hematological values.

Action Additional informationStep 1 Check blood

pressure andheart rate.

If systolic bloodpressure is lower than100 mmHg, requestmedical supervision

Step 2 Access thebrachial vein.

Use preferentially anintravenous catheterof 18 Gauge×1.16inch (1.3×30 mm)

Step 3 Evacuate 250ml of bloodover 30 min

Free flow to collectorbag

Step 4 Administer 250ml saline (NaCl0.9%) over30 min

Use a 3-way stopcock(Caveat: Avoidparadoxal airembolism)

Step 5 Evacuate 250ml of bloodover 30 min

Free flow to collectorbag

Step 6 Administer 250ml saline(NaCl 0.9%)over 30 min

Use a 3-way stopcock(Caveat: Avoidparadoxal airembolism)

Step 7 Remove theintravenouscatheter

Be aware about apotential bleedingdiathesis

Step 8 Check bloodpressure andheart rate again

If systolic bloodpressure is lower than100 mmHg, requestmedical supervision

Box 4Procedure for isovolumic phlebotomy at the Univer-sity Hospital Leuven, Belgium.


red cell volume, expressed as mean cell volume (MCV),decreases or if the patient becomes iron deficient, which canbe evaluated by ferritin levels (box 3).

Hyperviscosity is, paradoxically enough, associated withincreased bleeding diatheses, which is attributed to platelet(low number and dysfunctional) and coagulation-factorabnormalities [22]. Bleeding tendencies and hemostaticproblems are characterized by a number of symptoms,such as hemoptysis, epistaxis, and in some female patientsthe occurrence of heavy menses. These problems, may leadto anemia and subsequent to iron deficiency. Metaboliccomplications associated with cyanotic congenital heartdisease include impaired renal function, diminished glomer-ular filtration rate, proteinuria and hyperuricemia. Thechanges in urate metabolism can result in arthralgias andacute gouty arthritis [23].

7. Medical management

Patients with Eisenmenger syndrome have uniquelycomplex needs and it is essential that their care be followedclosely by adult congenital heart specialists trained in thetreatment of this physiology [6]. Medical therapy is directedby the clinical presentation of the patient. Current bestpractice consist successively of (i) conservative treatment;(ii) the use of selective pulmonary vasodilating agents; and(iii) transplantation [3].

7.1. Conservative treatment

The conservative treatment comprise: preservation offluid balance and avoidance of dehydration and large fluidshifts; treatment of iron-deficiency anemia; relief ofhyperviscosity syndrome; contraception and avoidance ofpregnancy; antiarrhythmic therapy; anticoagulation in spe-cific circumstances; and sometimes oxygen therapy [5].

7.1.1. PhlebotomyErythrocytosis in cyanotic patients tends to remain stable,

therefore phlebotomy is only recommended in patients whoare markedly symptomatic with hematocrits N65%. Prophy-lactic phlebotomy should not be performed in patients who

are asymptomatic or mildly symptomatic, regardless of thehematocrit [19]. The reporting of hyperviscosity-likesymptoms in the setting of a hematocrit b65% is morefrequently due to iron deficiency and is not an indication forphlebotomy. Fluctuations in the hematocrit and symptoms ofhyperviscosity may also be caused by dehydration caused byheat, fever, vomiting, nutritional changes or infection. Whenphlebotomy is required, it should be done concomitant withisovolumic fluid replacement. Phlebotomy without adequatereplacement is potentially hazardous as it may further reducesystemic blood flow, oxygen delivery, cerebral perfusion andcardiac output. Repeated phlebotomy should be avoided asthis can lead to rebound response from the bone marrow andiron deficiency anemia, which may worsen symptoms ofhyperviscosity, since iron-deficient erythrocytes (microcyticanemia) are less deformable than iron-replete erythrocytes[19]. Close monitoring of the heart rate and blood pressureduring phlebotomy is of critical importance. A suggestedprotocol for phlebotomy is described in box 4.

Patients with Eisenmenger syndrome who undergononcardiac surgery are prone to develop hemorrhagic andthrombotic complications. In preparation for noncardiacsurgery, therefore, prophylactic phlebotomy (usually of 1 to2 units of blood, with isovolumic replacement) is recom-mended for patients with a hematocrit above 65% in order to


reduce the likelihood of perioperative hemorrhagic andthrombotic complications [19]. A suggested algorithm toassess the need for management of patients with erythrocy-tosis is described in Fig. 1.

7.1.2. Iron deficiencyWhile inappropriate phlebotomy is the primary cause of

microcytic hypochromic anemia, other causes includerecurrent hemoptyses or epistaxes and menorrhagia infemales. A good nutritional status with sufficient iron intakeis also important to avoid iron deficiency. Iron replacementin patients with microcytic iron deficiency (low Hb, ferritinand MCV) must be carefully administered beginning with asmall daily dose of ferrous sulfate (325 mg which is 65 mg ofelemental iron) given orally. Iron therapy should bediscontinued once indices respond or at the first discerniblerise in hematocrit. Patients should also be cautioned to avoidover-the-counter vitamins or dietary supplemental thatcontain iron. Regular checks of hemoglobin, hematocrit,MCV, ferritin and serum iron will help determine if recurringsymptoms of hyperviscosity are related to increased red cellmass or to iron deficiency.

7.1.3. Drug treatmentThe prophylactic administration of anticoagulants or

aspirin remains controversial. Although there is no empiricaldata to support its use, in practice, anticoagulation therapy isprescribed in patients with recurrent thromboembolic eventsand atrial fibrillation [5]. It is, however, generally held thatthe use of anticoagulants or aspirin should be avoided,because of the underlying hemostatic abnormalities andbleeding tendencies associated with Eisenmenger physiol-

Fig. 1. Management of the patient with the Eisenmenger syndrome anderythrocytosis. Reproduced from: Vongpatanasin W, Brickner ME, HillisLD, Lange RA. The Eisenmenger syndrome in adults. Ann InternMed 1998;128: 745–755 (with kind permission of the American College ofPhysicians).

ogy [6,19]. Individualized recommendations outlining risksand benefits should be further defined.

Studies in patients with primary pulmonary hypertensionsuggested beneficial effects of calcium channel blockers indecreasing the pulmonary vascular resistance [24]. Hence,the use of calcium channel blockage has been advocatedbecause of its vasodilating characteristics. However, atten-tion should be paid to the use of vasodilator therapy. Theseagents can cause a sudden decrease in systemic vascularresistance, without proportionally decreasing the pulmonaryvascular resistance [3]. Reduced stroke volume increase theright-to-left shunt which can lead to increased cyanosis andtissue hypoxia. Calcium channel blockers, as well as otherafterload reducing drugs, should be avoided in patients withEisenmenger syndrome [3].

7.1.4. OxygenTo date, the use of oxygen therapy for adults with

Eisenmenger syndrome has not been sufficiently examinedso as to fully assess the risks and benefits. Initially, it wasthought that oxygen therapy would improve arterial oxygensaturation [25], however patients with right -to-left shunts,remain hypoxemic despite the use of inhaled high-flowoxygen [26]. Routine use of supplemental oxygen should bedeferred because it predisposes patients to epistaxis becauseof its drying effect of nasal oxygen on fragile mucousmembranes [5]. When required, use of humified air isrecommended [27].

7.1.5. VaccinationPatients with Eisenmenger syndrome are susceptible to

pneumonia, which may result in deterioration and death [28].Yearly vaccination for the prevention of influenza andpneumococcal pneumonia is advocated [28].

7.1.6. EndocarditisCongenital heart defects underlying to the Eisenmenger

syndrome, are associated with an ongoing risk for endo-carditis. If endocarditis occurs in these patients, it isassociated with a high morbidity and mortality [11,16],because the likelihood to develop cerebral abscesses is high[5]. Therefore, patients should be instructed about preventivemeasures and antibiotic prophylaxis, according to the newguidelines for the prevention of infective endocarditis [29].

7.2. Pulmonary vasodilating agents

The role of pulmonary vasodilator therapy has becomeincreasingly important in the treatment of pulmonary arterialhypertension. While initially described for the treatment ofprimary pulmonary arterial hypertension, there is nowgrowing evidence that patients with Eisenmenger syndromemay also have improved hemodynamics and exercisecapacity [30–35]. Pulmonary vasodilating therapy consistsof either prostacyclins, endothelin receptor antagonists, orphosphodiesterase inhibitors (PDE-inhibitor). Prostacyclin


can be administered intravenously (epoprostenol), subcuta-neously (treprostinil), orally (Beraprost sodium), or viainhalation (iloprost); endothelin antagonists (bosentan,sitaxsentan) are administered orally; and PDE-inhibitors(sildenafil) can be administered both orally as intravenously[3]. More studies are ongoing to determine the efficacy ofnewer molecules, and the timing when to start with thesespecific agents.

7.3. Transplantation

Once the Eisenmenger syndrome has developed, closureof the systemic-to-pulmonary connection is no longerpossible. The only definitive treatment is lung transplanta-tion and repair of the congenital heart defect(s) or heart-lungtransplantation. Lung transplantation or heart-lung trans-plantation will only be considered in cases with irreversiblepulmonary hypertension; if the pulmonary vascular resis-tance is more than 5 Wood units or when the transpulmonarygradient is 12 mmHg or more [36]. In other cases, cardiacrepair or isolated heart transplantation can be considered.Hence, lung transplantation is only an option for patientswho have markers of a poor prognosis (syncope, refractoryright-sided heart failure, NYHA functional class IV, orsevere hypoxemia) [19]. Because of the somewhat limitedsuccess of transplantation [3] and the reasonably goodsurvival among patients treated medically, careful selectionof patients for transplantation is imperative [19].

8. Counseling and Education

8.1. Quality of life and perceived health status

Studies in which quality of life and perceived health statusin patients with Eisenmenger syndrome are investigated arescarce. These studies found that patients with Eisenmengerand persistent cyanosis have a lower perceived health status[37,38], but that the quality of life is only marginallydifferent from that of patients with non-cyanotic heartdefects [37]. Moreover, it is argued that many patients canhave a reasonably active and productive live [16].

However, uncertainty always looms close to thesepatients and their families. Individual responses to thediagnosis also may vary, thus nurses, along with other healthprofessionals, must be prepared to guide these patients inorder to help them achieve an optimal level of life. Any whilethe diagnosis Eisenmenger Syndrome renders them inoper-able, it is important to explain that with proper management,they may have a stable and productive life. As with anychronic disease, a sense of hopelessness may causeemotional immobility, but as nurses, the role is to restorehope and to assist patients and their families to gain controlof their life. This often begins by informing the patient aboutthe disease, its symptoms and causes. Next, utilizing theconcept of self-care and health maintenance, the patients

must be given shared responsibility for their physical well-being.

8.2. Gynecologic issues, Contraception and PregnancyManagement

8.2.1. Gynecologic IssuesWomen with cyanotic heart defects may present with

dysfunctional uterine bleeding ranging from amenorrhea toexcessive menstrual bleeding [39]. For adolescents,menarche may be delayed [40]. Dysfunctional menstrualbleeding may reflect anovulation, which, if uncorrected,increases the risk of endometrial hyperplasia and adenocar-cinoma [41]. Females reporting menorrhagia are at risk foriron deficiency and relative anemia. Menstrual histories,therefore, must be included in routine evaluation withgynecologic referral as indicated.

8.2.2. ContraceptionThe use of combined oral contraceptives is absolutely

contra-indicated [42,43]. The relationship between oralcontraceptives, particularly the estrogenic type, and venousthromboembolism makes this form of contraception poten-tially hazardous. While sterilization is often recommended,some patients may resist because of religious or emotionalreasons. In addition, there is a significant surgical andanesthetic risk with the surgical approach; and the abdominalCO2 inflation for the laparoscopic approach substantiallyworsens the hemodynamic equilibrium. Newer approaches,such as coil embolization of fallopian tubes may carry lessrisk, but this technique has not been clearly defined inpatients with Eisenmenger syndrome. Therefore, the levo-norgestrel-releasing intra-uterine device (Mirena®) can berecommended. It is highly effective and carries low risk ofinfection. Caution should be given at time of insertionbecause a vagal reaction—which occurs in 5% of women—could prove fatal in patients with Eisenmenger physiology[42]. Progestogen-only pill (mini-pill), subdermal implants,Depo-Provera®, barrier method, and emergency hormonalcontraception are alternatives and maybe used [42,43].Because of the absolute contra-indication of pregnancy andthe potential risks of permanent sterilization, vasectomycould be an option. Many couples consider this optionbecause there is no risk to the patient. However, this requiresthe clinician to be honest with the couple about lifeexpectancy; and for the couple to consider future relation-ships and family plans for the male partner after the patienthas died. This is a difficult topic, but an important discussionto have with patients and their partners.

8.2.3. PregnancyPregnancy in women with Eisenmenger syndrome is

associated with significant morbidity and mortality for bothmother and fetus. Maternal mortality rates are reported to bebetween 30% to 50% [44], The decrease in systemic vascularresistance, normally associated with pregnancy, will increase


the degree of right-to-left shunt in patients with Eisenmengersyndrome, resulting in worsening of maternal cyanosis [45].Complications mainly occur at term and during the firstpostpartum week [45], and are attributed to thromboembo-lism, bleeding during delivery, syncope and symptomaticdeterioration post-delivery [46].

The increased maternal cyanosis is also associated withadverse effect on fetal outcome. Indeed, spontaneous abortionis common, intrauterine growth restriction is seen in 30% ofpregnancies, and preterm labor is frequent [45]. Fetal lossranges up to 47% [47]. In some series, only one third of allpregnancies in women with Eisenmenger's syndrome weredischarged with both mother and infant alive [47].

Despite the warnings against pregnancy, women withEisenmenger physiology will present pregnant and forpersonal reasons will choose to proceed with the pregnancyregardless of risk. For these women, a well coordinatedmultidisciplinary plan of care is essential to ensure the healthof the mother and her child [6]. The recent attention given tothe use of selective pulmonary vasodilators in improvinghemodynamics and exercise capacity in patients withpulmonary hypertension offer the potential for improvedsurvival for these high risk patients. Additionally continuousadministration of oxygen or inhaled nitrous oxide isrecommended because of their beneficial effect on reducingpulmonary resistance and shunt flow and increasingoxygenation [48,49].

8.3. Physical Activity

Patient should be cautioned against sudden strenuousexercise but should be encouraged to seek activities andoccupations that are within their physical tolerance and toavoid exercising during the hottest part of day. Patientsshould be advised to drink large quantities of fluids,particularly during hot summer months, in order to avoiddehydration and hemoconcentration. In general, a fluidintake of 1.5 liters is recommended, except if patientsdemonstrate signs and symptoms of heart failure.

8.4. Travel

Most patients with Eisenmenger syndrome do well at anatmospheric pressure of 760 mmHg (sea level) in which theatmospheric PO2 is 159 mm Hg [50]. Hypoxia mayaggravate vasoconstriction in these patients and hypobarichypoxia starts at altitudes between 1500 and 2000 meter(5000–6500 feet) above sea level [28]. At higher levels, thecyanosis and breathlessness may become more pronounced.Therefore, these patients should be cautious when travelingto high altitude levels.

While air travel is not contraindicated, each patient shouldcheck with their health care provider particularly for long-distance air travel. Most commercial airlines cabins arepressurized between 1800–2500 meter (6000–8000 ft) whenflying at 10,000 meter (30,000 ft) [28]. In healthy people this

causes a marked decrease in the arterial PO2 but only a mildreduction of the arterial oxygen saturation. In patients withcyanotic congenital heart disease, the commonly feared decreaseof SaO2 during air travel does not seem to occur [51] Similarresults have been reported for patients with Eisenmengerphysiology [52]. Patients should, nevertheless, be advised toavoid dehydration by drinking plenty of non-alcoholic fluidsand for long aircraft flights avoid prolonged periods of inactivityby getting up and walking at regular intervals [52].

8.5. Smoking

Obviously, patients with Eisenmenger syndrome shouldbe advised against both active and passive smoking or use ofany tobacco products, as tobacco has been shown to causeelevated levels of carboxyhemoglobin resulting in signifi-cant hypoxemia and increase in erythrocytosis [53].

9. Conclusion

Eisenmenger syndrome is a medical condition, character-ized by elevated pulmonary vascular resistance and right-to-left shunting of blood through a systemic-to-pulmonarycirculation connection. The management of patients withEisenmenger syndrome is complex and should be taken on byhealth care professionals who are specialized in the treatmentof this physiology. However, cardiovascular nurses should beknowledgeable about the pathophysiology, medical manage-ment and counseling issues of this condition. They need toknow that normal reference values are not applicable forpatients with Eisenmenger syndrome and that specificprecautions have to be taken, in case they need to care for apatient with Eisenmenger syndrome in their clinical practice.

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