AUTHOR AND EDITOR INFORMATION

Section 1 of 12  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Acknowledgments
• Multimedia
• References

INTRODUCTION

Section 2 of 12  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Acknowledgments
• Multimedia
• References

Background

In 1897, Eisenmenger reported the case of a 32-year-old man who had showed exercise intolerance, cyanosis, heart failure, and hemoptysis prior to death. Autopsy showed a large ventricular septal defect (VSD) and overriding aorta. This was the first description of a link between a large congenital cardiac shunt defect and the development of pulmonary hypertension.

Pathophysiology

Eisenmenger syndrome occurs in patients with large congenital cardiac or surgically created extracardiac left-to-right shunts. These shunts initially cause increased pulmonary blood flow. Subsequently, usually before puberty, pulmonary vascular disease causes pulmonary hypertension, ultimately resulting in reversed or bidirectional shunt flow with variable degrees of cyanosis.

For more information, see Medscape CME activity, Eisenmenger's Syndrome: Pathophysiologic Insights and Pharmacologic Treatment Rationales.

Frequency

International

• The frequency of pulmonary hypertension and the subsequent development of reversed shunting vary depending on the specific heart defect and operative interventions.
• Approximately 50% of infants with a large, nonrestrictive VSD or patent ductus arteriosus (PDA) develop pulmonary hypertension by early childhood.
• Forty percent of patients with VSD or PDA and transposition of the great arteries develop pulmonary hypertension within the first year of life.
• The natural history of a large secundum atrial septal defect (ASD) differs in that the 10% of cases that progress to pulmonary hypertension do so more slowly and usually not until after the third decade of life.
• All patients with persistent truncus arteriosus and unrestricted pulmonary blood flow, and almost all patients with common atrioventricular canal, develop severe pulmonary hypertension by the second year of life.
• The frequency of pulmonary hypertension in surgically created systemic-to-pulmonary shunts varies depending on size and anatomy.
• Ten percent of those with a Blalock-Taussig anastomosis (subclavian artery to pulmonary artery) develop pulmonary hypertension compared to 30% of those with a Waterston (ascending aorta to pulmonary artery) or a Potts (descending aorta to pulmonary artery) shunt.

Mortality/Morbidity

• Exercise tolerance is severely impaired due to an inability to increase pulmonary blood flow, thereby limiting oxygen uptake. The systemic vascular bed is prone to vasodilation and subsequent systemic arterial hypotension, which can cause syncope.
• Long-term survival depends on the age at onset of pulmonary hypertension and the coexistence of additional adverse features, such as Down syndrome. Survival predominantly depends on right ventricular function. These patients usually do not survive beyond the second or third decade. The most frequent terminal event is a combination of hypoxemia and arrhythmia in the setting of rapid increases in pulmonary vascular resistance or decreases in systemic vascular resistance (SVR).
• The complications of chronic cyanotic heart disease affect multiple organ systems, including hematologic, skeletal, renal, and neurologic, causing significant morbidity and mortality.

Sex

No overall sex predilection has been reported.

Age

Eisenmenger syndrome usually develops before puberty but may develop in adolescence and early adulthood.

CLINICAL

Section 3 of 12  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Acknowledgments
• Multimedia
• References

History

Symptoms related specifically to pulmonary hypertension result from the inability to increase pulmonary blood flow in response to physiological stress. Other symptoms are caused by the variety of multisystem complications associated with cyanotic congenital heart disease.

• Pulmonary hypertension
• • Breathlessness
  • Fatigue
  • Lethargy
  • Severely reduced exercise tolerance with a prolonged recovery phase
  • Presyncope
  • Syncope
• Heart failure
• • Exertional dyspnea
  • Orthopnea
  • Paroxysmal nocturnal dyspnea
  • Edema
  • Ascites
  • Anorexia
  • Nausea
• Erythrocytosis
• • Myalgias
  • Muscle weakness
  • Anorexia
  • Fatigue
  • Lassitude
  • Paresthesias of the digits and lips
  • Tinnitus
  • Blurred or double vision
  • Scotomata
  • Headache
  • Dizziness
  • Slowed mentation
  • Decreased alertness
  • Irritability
• Bleeding tendency
• • Mild mucocutaneous bleeding
  • Epistaxis
  • Menorrhagia
  • Pulmonary hemorrhage
• Vasodilation
• • Presyncope
  • Syncope
• Cholelithiasis
• • Right upper quadrant pain
  • Biliary colic
  • Fever
  • Pale stools
  • Jaundice
• Nephrolithiasis
• • Renal colic
  • Secondary gout
  • Joint pain and swelling
• Paradoxical embolus can cause symptoms of localized vascular insufficiency end-organ ischemia.
• Hypertrophic osteoarthropathy can cause long bone pain and tenderness.
• Retinal complications include episodes of transient visual loss and spontaneous hyphemas.

For more information, see Medscape CME activity Uncovering Complications of Congenital Heart Disease: Eisenmenger's Syndrome and Beyond.

Physical

• Cardiovascular
• • Central cyanosis (differential cyanosis in the case of a PDA)
  • Clubbing
  • Jugular venous pulse wave may be A-wave dominant, and, in the presence of a significant tricuspid regurgitation, the V wave may be prominent; central venous pressure may be elevated.
  • Precordial palpation reveals a right ventricular heave and, frequently, a palpable S₂.
  • Loud P₂
  • High-pitched early diastolic murmur of pulmonic insufficiency
  • Right-sided fourth heart sound
  • Pulmonary ejection click
  • Single S₂ 
  • As the pulmonary vascular resistance progressively rises, the holosystolic murmur of nonrestrictive VSD shortens and softens, first becoming early systolic in timing, before disappearing entirely as the shunt is reversed.
  • The continuous murmur of a PDA disappears when Eisenmenger physiology develops; a short systolic murmur may remain audible.
• Other signs
• • Respiratory signs include cyanosis and tachypnea.
  • Hematologic signs include bruising and bleeding; funduscopic abnormalities related to erythrocytosis include engorged vessels, papilledema, microaneurysms, and blot hemorrhages.
  • Abdominal signs include jaundice, right upper quadrant tenderness, and positive Murphy sign (acute cholecystitis).
  • Vascular signs include postural hypotension and focal ischaemia (paradoxical embolus).
  • Musculoskeletal signs include clubbing, tenderness over the metacarpal or metatarsal joints (hypertrophic osteoarthropathy), and joint effusions.
  • Skin demonstrates fewer urate deposits than commonly observed in primary gout.
  • Ocular signs include conjunctival injection, rubeosis iridis, and retinal hyperviscosity changes (see Hematological signs).

Causes

• Large uncorrected cardiac shunts or palliative, surgically created systemic-to-pulmonary shunts for congenital heart disease
• Large nonrestrictive VSD
• Nonrestrictive PDA
• Atrioventricular septal defect, including large ostium primum ASD without ventricular component
• Aortopulmonary window
• Palliative, surgically created systemic-to-pulmonary anastomosis for treatment of congenital heart disease

DIFFERENTIALS

Section 4 of 12  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Acknowledgments
• Multimedia
• References

Other Problems to be Considered

Pulmonary atresia with VSD
Single ventricle with pulmonary stenosis

WORKUP

Section 5 of 12  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Acknowledgments
• Multimedia
• References

Lab Studies

• Complete blood count
• • Erythrocytosis increases hematocrit and hemoglobin concentration.
  • Phlebotomy-related iron deficiency decreases the mean corpuscular volume and mean corpuscular hemoglobin concentration.
• Red cell mass is increased with erythrocytosis.
• Bleeding time is prolonged by platelet dysfunction.
• Biochemical profile
• • Increased conjugated bilirubin
  • Increased uric acid
  • Urea and creatinine sometimes elevated
  • Erythrocytic hypoglycemia is an artifactually low blood glucose level caused by increased in vitro glycolysis in the setting of increased red cell mass.
• Iron studies
• • Reduced serum ferritin due to phlebotomy-related iron store reduction
  • Increased total iron binding capacity
• Urine biochemical analysis reveals proteinuria.
• Arterial blood gases
• • Reduced resting PaCO₂ due to resting tachypnea and reduced PaO₂ due to right-to-left shunting
  • Mixed respiratory and metabolic acidosis

Imaging Studies

• Chest radiograph
• • Right ventricular and right atrial enlargement
  • Features of pulmonary hypertension, including dilated main pulmonary artery, increased hilar vascular markings, and pruned peripheral vessels
• Transthoracic echocardiogram
• • The structural cardiac defect responsible for the shunt can be defined by the 2-dimensional imaging.
  • The location of cardiac shunt can usually be demonstrated by color Doppler.
  • The pressure gradient across the defect can be estimated.
  • Estimated pulmonary artery systolic and diastolic pressures
  • Identification of coexistent structural abnormalities
  • Left and right ventricular size and function: A study by Salehian et al reported that left ventricular dysfunction (defined as LVEF <50%), right ventricular hypertrophy, and signs and symptoms of heart failure predict mortality in patients with Eisenmenger syndrome.¹
  • Identification of surgical systemic-to-pulmonary shunts
  • The addition of supine bicycle ergometry can demonstrate increased right-to-left shunting with exercise.
• Transesophageal echocardiogram is useful for imaging posterior structures, including the atria and pulmonary veins.
• Magnetic resonance imaging
• • Can estimate magnitude of the right-to-left shunt
  • Useful for anatomical definition in some cases

Other Tests

• Electrocardiogram
• • Almost always abnormal results and includes signs of right heart hypertrophy in addition to abnormalities associated with the underlying defect
  • Frontal plane QRS right axis deviation
  • Tall monophasic R wave in V₁, deep S wave in V₆, ± ST and T wave abnormalities
  • P pulmonale

Procedures

• Cardiac catheterization can be of value, after collecting clinical and noninvasive data, to confirm and/or demonstrate the following:
• • Severity of pulmonary arterial hypertension
  • Conduit patency and pressure gradient
  • Coexisting coronary artery anomalies (rare)
  • Degree of shunting

Histologic Findings

In severe pulmonary vascular disease, histological analysis reveals abnormal extension of muscle into small peripheral arteries, severe medial smooth muscle hypertrophy of existing muscular arteries, plexiform lesions and increased intercellular material, and a reduction in the overall concentration and size of arteries.

TREATMENT

Section 6 of 12  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Acknowledgments
• Multimedia
• References

Medical Care

• Fluid balance and climate control
  • Avoid sudden fluid shifts or dehydration, which may increase right-to-left shunting.
  • Avoid very hot or humid conditions, which may exacerbate vasodilation, causing syncope and increased right-to-left shunting.
• Oxygen therapy
  • The use of oxygen supplementation is controversial.
  • Oxygen therapy has been shown to have no impact on exercise capacity and survival in adult patients with Eisenmenger syndrome.^{2, 3}
  • Some patients might benefit from nocturnal supplementation, although it is most useful as a bridge to heart-lung transplant.
  • Air travel appears to be safe as long as the airplanes are adequately pressurized. Supplemental oxygen during commercial air travel is often recommended, but limited data exist regarding this issue.⁴
• Pulmonary vasodilator therapy
• • The pathophysiology of pulmonary hypertension in patients with Eisenmenger syndrome is somewhat similar to that of idiopathic pulmonary arterial hypertension (IPAH); both are associated with a neurohormonal imbalance of endogenous pulmonary vasodilators and vasoconstrictors. This imbalance leads to vascular remodeling, intimal fibrosis, and increased pulmonary vascular resistance (PVR). Therefore, in the management of patients with Eisenmenger syndrome, use of pulmonary vasodilating agents that have been shown to be useful in the management of patients with IPAH is conceptually appealing. Recent data support this use.
  • Long-term prostacyclin therapy was shown to improve hemodynamics (decrease in mean PA pressure, improvement in cardiac index, and decrease in PVR) and quality of life in patients with congenital heart disease and PAH.⁵ Another study evaluated epoprostenol infusion in adolescents with congenital heart disease and Eisenmenger physiology showing improved oxygenation (from 69% to 85%) and improvement in a 6-minute walk test distance (from 48 yd to 375 yd).⁶
  • Bosentan, an endothelin receptor antagonist, was the second vasodilator to be evaluated in patients with Eisenmenger syndrome. In a small retrospective study of 9 patients with congenital heart disease and Eisenmenger syndrome, bosentan therapy resulted in improved oxygen saturation (from 79% to 88%) and improved New York Heart Association (NYHA) class.⁷ A larger, multicenter, prospective study by Schulze-Neick et al tested the same hypothesis. In this study, bosentan titrated to 125 mg orally bid was associated with improvement in 6-minute walk test distance (362 m to 434 m), improvement in NYHA class (3.1 to 2.4), and a decrease in systolic PA pressure (111 mm Hg to 106 mm Hg).⁸
    
    A multicenter, prospective, double-blind, placebo-controlled study (the BREATH-5 study) tested the effect of bosenten titrated to 125 mg bid in 54 patients with Eisenmenger syndrome. Bosentan reduced the mean pulmonary arterial pressure and improved exercise capacity and World Health Organization (WHO) class.⁹ A longer follow-up of the BREATH-5 population (up to 40 wk) showed that bosentan remained safe and had a positive impact on patients with Eisenmenger syndrome.¹⁰  
  • A retrospective study that examined the outcomes of patients with Eisenmenger syndrome who were treated with pulmonary vasodilators versus those who were not showed that treatment with prostacyclin analogues and/or endothelin receptor antagonists delayed the need for transplantation.¹¹
  • Sildenafil is another vasodilatory agent originally used for erectile dysfunction but recently approved for IPAH. Studies suggest that sildenafil is safe and effective in patients with Eisenmenger syndrome. In a randomized, placebo-controlled study of 20 patients with PAH (10 patients with Eisenmenger syndrome and 10 with IPAH), sildenafil improved NYHA class, 6-minute walk test distance, and exercise duration. Furthermore, sildenafil therapy resulted in a decrease in systolic PA pressure from 98 mm Hg to 78 mm Hg. The effects of the drug were similar for the 2 patient populations in this study.¹²
    
    In a study by Chau et al, sildenafil improved hemodynamics and symptoms in patients with pulmonary hypertension secondary to Eisenmenger syndrome and a similar group of patients with idiopathic arterial hypertension.¹³
    
  • In a study from India, another phosphodiesterase inhibitor (tadalafil) was shown to be safe and effective in 16 symptomatic patients with Eisenmenger syndrome. Tadalafil improved oxygen saturation (84% to 89% at 12 wk), and the mean WHO class (2.31 to 1.25).¹⁴
  • In summary, vasodilator therapy improves symptoms in patients with Eisenmenger syndrome and should be used routinely in the management of this patient population. However, a single medical therapy has not been consistently shown to reduce mortality in patients with Eisenmenger syndrome.
• Anticoagulation: Silversides et al reported that the incidence of proximal pulmonary artery thrombus in this patient population was 21%.¹⁵ This study raised the issue of anticoagulation in Eisenmenger patients. These patients are at increased risk of bleeding; hence, the use of anticoagulation is still not routinely recommended.
• Contraception, pregnancy, and genetic counseling
• • Avoid pregnancy. Tubal ligation is strongly recommended in women with Eisenmenger syndrome.
  • If patients refuse tubal ligation, hormone therapy (controlled release levonorgestrel or norethindrone and ethinyl estradiol preparations) is preferred over intrauterine devices, which can cause significant menorrhagia and potentially increase the risk of endocarditis.
  • The fetal mortality rate is approximately 25%, and the maternal mortality rate exceeds 50%.
  • Therapeutic abortion is recommended for women in the early stages of pregnancy.
  • The risk of congenital heart defects in offspring is approximately 10% and sometimes higher, depending on the primary natural cardiac defect.
  • Fetal echocardiography is recommended for pregnant patients or siblings.
• For resuscitation in the event of massive acute bleeding, replace losses with fresh frozen plasma, cryoprecipitate, and platelets.
• Endocarditis
• • Patients with Eisenmenger syndrome are at very high risk for endocarditis. Endocarditis prophylaxis should be emphasized and patients should be given repeat instructions about this issue. For standard general prophylaxis for dental, oral, respiratory tract, esophageal, genitourinary, and other gastrointestinal procedures, refer to the American Heart Association recommendations for the prevention of bacterial endocarditis.¹⁶
  • Infective endocarditis prophylaxis (nonchemotherapeutic)
  • • Encourage good oral hygiene (soft-bristle toothbrushing twice a day, mouthwash or hydrogen peroxide rinses, soft conical rubber gum stimulator, semiannual dental visits).
    • Skin care advice should include using a nonabrasive cleanser and avoidance of squeezing skin.
    • Nail biting should be avoided.
• Right heart failure is often present in patients with Eisenmenger syndrome, but limited treatment options are available for this complication. Typically, digoxin and diuretics have been used. Diuretics, specifically loop diuretics, are used for symptomatic relief of congestion. However, diuretics should be used cautiously in these patients given their preload-dependent state.
• Erythrocytosis
• • Erythrocytosis is almost always present in patients with Eisenmenger syndrome. This could result in symptoms of hyperviscosity that include visual disturbances, fatigue, headache dizziness, and paresthesias. Routine phlebotomy is not usually recommended for this condition, except in the presence of hyperviscosity symptoms. Before initiating phlebotomy, dehydration must be ruled out since it can falsely increase the hematocrit level. Furthermore, phlebotomy should always be performed with concomitant fluid replacement.
  • Repeated phlebotomy can result in iron deficiency anemia. Patients with an iron deficiency have apparent normal hematocrit level and low mean corpuscular volume (MCV). The iron deficient erythrocytes are less deformable than normal erythrocytes, and this lack of deformability can worsen hyperviscosity.¹⁷
  • To manage erythrocytosis, first rule out dehydration. Then, if the patient has symptoms of hyperviscosity and the hematocrit level is greater than 65%, venesect 250-500 mL of blood and replace with an equivalent volume of isotonic sodium chloride (or 5% dextrose if in heart failure).
• Thrombotic and bleeding complications
• • Eisenmenger syndrome patients are prone to thrombotic events as part of their hyperviscosity. At the same time, they are susceptible to bleeding because their platelets are dysfunctional. Therefore, patients who have a hematocrit level greater than 65% and are undergoing noncardiac surgery should receive phlebotomy and concomitant fluid replacement in order to decrease the risk of thrombotic and bleeding events.¹⁸
• Central nervous system (CNS) events can occur secondary to paradoxical embolus, CNS venous thrombosis, intracranial hemorrhage, or brain abscesses in the setting of endocarditis.¹⁸ Endocarditis prophylaxis, the use of air filters with all intravenous catheters during hospital admissions, and adequate management of hyperviscosity can help decrease these potentially fatal complications.

For more information, see Medscape CME activity, Eisenmenger's Syndrome: Pathophysiologic Insights and Pharmacologic Treatment Rationales.

Surgical Care

• Heart-lung transplantation
• • Heart-lung transplantation is the procedure of choice if repair of the underlying cardiac defect is not possible.
  • It was performed successfully for the first time in 1981. Since then, the outcome has improved due to better immunosuppressive therapy, new antiviral agents, and improved patient selection.
  • Reported actuarial survival rates are 68% at 1 year, 43% at 5 years, and 23% at 10 years.
  • The main complications are infection, rejection, and obliterative bronchiolitis.
• Bilateral lung transplantation
• • Repair of the underlying cardiac defect is required, but it is considered the preferable procedure if the cardiac defect is simple.
  • Bilateral lung transplantation is better than single-lung transplantation in terms of mortality, New York Heart Association functional class, cardiac output, and postoperative pulmonary edema.
• Advantages over heart-lung grafting include no transplant coronary artery disease or cardiac rejection, and this approach may be considered an option in current times of donor organ shortage, although exact indications have yet to be defined.
• Corrective surgery
• • Repair of the primary defect is contraindicated in the context of established severe pulmonary arterial hypertension. However, corrective surgery may be possible in certain cases if a significant degree of left-to-right shunting remains and if responsiveness of the pulmonary circulation to vasodilator therapy can be demonstrated.
  • Limitations include a transient dynamic right ventricular outflow tract obstruction, and exact indications for this approach have not yet been defined.

Consultations

• Pediatric cardiologist (pediatric patients)
• Adult cardiologist who specializes in adult congenital heart disease (adult patients)
• Early input from heart-lung transplant team

Diet

• Patients with right-sided congestive heart failure should avoid excessive sodium intake.
• Sodium restriction must be balanced with the need to maintain intravascular volume.

Activity

• Intense athletic activities carry the risk of sudden death.
• Patients should not participate in competitive sports.
• In certain patients, an exercise prescription can be individualized based on exercise testing that documents a level of activity that meets the following 3 criteria:
  1. Oxygen saturations remain greater than 80%.
  2. No symptomatic arrhythmias occur.
  3. No evidence of symptomatic ventricular dysfunction is present.

MEDICATION

Section 7 of 12  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Acknowledgments
• Multimedia
• References

The medical treatment of Eisenmenger syndrome is directed toward improvement of symptoms related to heart failure and pulmonary hypertension and preventing and managing complications related to cyanotic congenital heart disease.

For more information, see Medscape CME activity, Eisenmenger's Syndrome: Pathophysiologic Insights and Pharmacologic Treatment Rationales.

Drug Category: Diuretics

These agents are useful to remove fluid and reduce preload and afterload in the treatment of heart failure.

Drug Category: Cardiac glycosides

Positive inotropic and negative chronotropic effects are useful in the setting of left or right heart failure.

Drug Category: Prostaglandins

These drugs can be effective in reversing reactive pulmonary vasoconstriction and can, therefore, lower pulmonary vascular resistance, decrease afterload, reduce the right ventricle, and reduce right-to-left shunting. In some patients, chronic prostacyclin analogue therapy (epoprostenol) can be of benefit, particularly as a bridge to heart-lung transplantation.

Drug Category: Vitamins

Recurrent phlebotomy for erythrocytosis can lead to microcytic anemia. Iron stores should be replaced if deficiency is symptomatic and hematocrit is <65%.

Drug Category: Anti-inflammatory medications

Indicated for symptomatic secondary gout.

Drug Category: Endothelin antagonists

These agents competitively bind to endothelin-1 (ET-1) receptors EtA and EtB in endothelium and vascular smooth muscle, inhibiting vessel constriction and elevation of blood pressure.

Drug Category: Phosphodiesterase (type 5) enzyme inhibitors

The antiproliferative effects of the phosphodiesterase type 5 pathway (PDE5), which regulates cyclic guanosine monophosphate hydrolysis, may be significant in the chronic treatment of pulmonary hypertension with PDE5 inhibitors such as sildenafil.

FOLLOW-UP

Section 8 of 12  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Acknowledgments
• Multimedia
• References

Further Inpatient Care

• Transfer patient to a specialized cardiology unit.
• Monitor patient in an intensive care unit.

Further Outpatient Care

• Patient should follow up at a specialized cardiology clinic within 2 weeks of discharge, and every 3 months when stable.

Deterrence/Prevention

• Educate patients regarding infective endocarditis risk reduction.
• Avoid situations that exacerbate vasodilation.
• Avoid abrupt, strenuous, or isometric exercise.
• Restrict activities that may result in further oxygen desaturation, symptomatic arrhythmias, or right ventricular dysfunction.
• Stop steady-state exercise at onset of symptoms.

Complications

• Hematological complications include hyperviscosity syndromes related to secondary erythrocytosis and bleeding diatheses.
• Nervous system complications include brain abscess, transient cerebral ischaemia, thrombotic stroke, and intracerebral hemorrhage.
• Hyperbilirubinemia increases the risk of gallstones.
• Hyperuricemia can cause nephrolithiasis and secondary gout.
• Hypertrophic osteoarthropathy causes bone pain and tenderness.
• Reports document transient visual loss related to peripheral retinal microvascular abnormalities.

Prognosis

• Survival is limited by complications and deteriorating ventricular function.
• Sudden death always is a threat.

MISCELLANEOUS

Section 9 of 12  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Acknowledgments
• Multimedia
• References

Medical/Legal Pitfalls

• Avoid significant reductions in SVR.
• • Lowering systemic arterial blood pressure can cause syncope.
  • A reduced SVR increases right-to-left shunting and worsens systemic arterial oxygen saturation.
• Remember to administer antibiotic prophylaxis for infective endocarditis according to the recommendations of the American Heart Association.
• Advise unequivocally against pregnancy; provide adequate contraceptive advice and genetic counseling.
• • Pregnancy carries a maternal mortality rate of approximately 50%.
  • Intrauterine devices are not recommended due to the risk of infection and bleeding.
  • Congenital cardiac abnormalities occur in approximately 10% of offspring.

Special Concerns

• Maternal considerations with pregnancy
• • Despite the fact that more women with congenital heart disease than ever before are reaching reproductive age, maternal mortality rates in patients with congenital heart disease have not improved in the last 50 years and pregnancy is absolutely contraindicated in the Eisenmenger syndrome.
  • Although the maternal mortality rate is reported to range from 23-52% in different series, most experienced physicians estimate that the mortality rate is in excess of 50%.
  • The most critical time is postpartum, and the majority of deaths occur in the first week.
  • Factors that increase the risk of a peripartum death include congestive heart failure, sudden increases in pulmonary vascular resistance or decreases in SVR, bleeding/anemia, hematocrit greater than 60%, oxygen saturation less than 80%, and syncope.
  • Excessive straining should be avoided during the second stage of labor. Therefore, assisted delivery usually is recommended.
  • Caesarian delivery carries a higher mortality rate and should be reserved for obstetric indications, such as cephalopelvic disproportion.
  • The use of anticoagulants is controversial. The rationale for anticoagulation is that the risk of clotting during pregnancy is increased when associated with preexisting cyanosis. However, reports indicate that anticoagulation has contributed to mortality in several patients.
  • If anticoagulants are used, a suggested protocol is heparin until 12 hours predelivery, then warfarin from 48 hours postdelivery to the end of the puerperium. At the minimum, ensure proper leg care, use of elastic bandages, sufficient hydration, and early mobilization to prevent deep venous thrombosis.
• Fetal consideration with pregnancy
• • The main risks to the fetus include arterial oxygen desaturation, hypoxemia, and polycythemia
  • The fetal mortality rate ranges from 7.8-28.0%.
  • Only 15% of babies are born at term.
• Flying on commercial airplanes
• • Flying in patients with Eisenmenger syndrome carries the following risk:
  • • Deep venous thrombosis, especially since this group of patients is predisposed to thrombotic events
    • Compromised oxygen delivery at high altitude
  • A study comparing air travel history over the past decade in 53 patients with Eisenmenger syndrome 48 patients with acyanotic found no major adverse events in either group. One patient in the Eisenmenger group had a probable transient ischemic attack (TIA) and one required supplemental oxygen after exposure to ambient cigarette smoke in flight.¹⁹
  • This study revealed that patients with Eisenmenger syndrome fly frequently and safely.
  • Nevertheless, it is recommended that patients avoid dehydration and inactivity during travel.

ACKNOWLEDGMENTS

Section 10 of 12  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Acknowledgments
• Multimedia
• References

The authors and editors of eMedicine gratefully acknowledge the contributions of previous authors Lisa A Hourigan, MBBS and Elyse Foster, MD to the development and writing of this article.

MULTIMEDIA

Section 11 of 12  

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• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Acknowledgments
• Multimedia
• References

Media file 1:  Apical 4-chamber transthoracic view demonstrating an ostium primum atrial septal defect (ASD) with enlarged right-side chambers. RA = right atrium, RV = right ventricle, LA = left atrium, LV = left ventricle.
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Media type:  Photo

Media file 2:  This radiograph reveals an enlarged right heart and pulmonary artery dilatation in a 24-year-old woman with an unrestricted patent ductus arteriosus (PDA) and Eisenmenger syndrome.
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Media type:  X-RAY

Media file 3:  This CT chest scan shows a large, unrestricted patent ductus arteriosus (PDA) in a 24-year-old woman with Eisenmenger syndrome. Asc Ao = ascending aorta.
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Media type:  CT

Media file 4:  This transthoracic apical 4-chamber segment shows color Doppler flow across the interatrial septum at the site of a large ostium primum atrial septal defect (ASD). RA = right atrium, LA = left atrium.
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Media type:  Photo

Media file 5:  This transesophageal image is from the mid esophagus of a patient with Eisenmenger syndrome secondary to an unrestricted patent ductus arteriosus (PDA). It shows a severely dilated pulmonary artery. Pulm a = pulmonary artery, Asc Ao = ascending aorta.
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Media type:  Photo

Media file 6:  This is a color Doppler interrogation of the tricuspid valve in a case of Eisenmenger syndrome. It demonstrates an elevated estimated right ventricular systolic pressure of 106 mm Hg + right atrial pressure, reflecting pulmonary hypertension. TR = tricuspid regurgitation.
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Media type:  Photo

Media file 7:  This is the transthoracic Doppler examination of the pulmonic valve in a 24-year-old woman with Eisenmenger syndrome secondary to an uncorrected ostium primum atrial septal defect (ASD). This reveals an elevated estimated pulmonary artery diastolic pressure of 51 mm Hg + right atrial pressure. PR = pulmonic regurgitation.
	View Full Size Image
Media type:  Photo

REFERENCES

Section 12 of 12

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Acknowledgments
• Multimedia
• References

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