Eisenmenger Syndrome

Madison Bohon
DMS 497 Clinical Practicum IV Fall 2015
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Abstract
The purpose of this research is to educate the reader on a particular patients case study and the
pathology associated with this patient. The pathology mentioned in this paper is Eisenmenger
Syndrome. The pathology is defined, and the clinical and sonographic findings of the pathology
are described. The literature descriptions of Eisenmenger Syndrome are compared to the
characteristics of this specific case. Appropriate treatment and follow up is discussed, along with
how sonographers can learn from this disease process.
Key Words
Cyanosis
Shunting
Congenital Heart Disease
Ventricular Septal Defect
Atrial Septal Defect
Right-to-left Shunting
Left-to-right Shunting
Pulmonary Hypertension
Eisenmenger Syndrome is a severe effect of pulmonary arterial hypertention due to congenital
anomalies of the heart. It is a right-to-left shunting that causes reversal of flow and cyanosis.1
This pathology is fatal, however the person may live into their sixth decade of life.2
Case History
Patient had a known history of congenital Eisenmenger Syndrome. Patient was a thirty two year
old African American who presented with hypoxemia. The patient had complex congenital heart
disease (CHD). Patient was 53 and weighed 98 pounds with a body mass index of 16.82

kg/m^2. Patients heart rate was 113 beats per minute. The patients labs indicated low levels of
BUN and BUN/creatinine ratio. The echocardiogram was ordered because the patient
experienced a new ischemic cerebrovascular incident, and the ordering physician was concerned
about a cardioembolic source. Fourteen days after the echocargiogram, the patient had a
magnetic resonance imaging (MRI) test done without contrast that concluded there were no new
areas of infarction, there were evolving bilateral subdural hematomas which were stable in size,
and there was a stable subacute left parietal infarct. The patient also had a later bilateral upper
extremity venous sonogram due to upper extremity swelling that found an absence of deep vein
thrombosis in both the right and left upper extremity.
Sonographic Findings
There was a large single appearing ventricle with global hypokinesis (Figure 1). The ejection
fraction of this patient was 20%. A large anterior aortic systemic vessel was seen (Figure2). The
aortic valve was moderately thickened and echogenic but still functional. There appeared to be a
large anechoic space between the two atrium (Figure 3), which would indicate a secundum atrial
septal defect (ASD). The posterior pulmonic artery was atretic, and the valve was calcified with
diminished systolic motion. There was also a small amount of pericardial effusion.
Diagnosis
The reading physician confirmed the previous diagnosis of Eisenmenger Syndrome. A diagnosis
of complex CHD, probable secundum ASD, probable transposition of the great vessels (TGV),
mild regurgitation of the aortic valve, and pericardial effusion was made for this patient.
Eisenmenger syndrome is any congenital anomaly that includes ventricular septal defect (VSD),
ASD, or patent ductus arteriosus that is not treated and results in pulmonary hypertension,
reversal of flow, and cyanosis.1, 3 Eisenmenger Syndrome represents the most advanced form of

pulmonary arterial hypertension (PAH).1 Approximately 50% of all patients with large
unrepaired VSDs, approximately 10% of patients with large unrepaired ASDs, and almost all
patients with unrepaired truncus arteriosus are at risk of developing Eisenmenger Syndrome.1
This syndrome usually develops before puberty and is more prevalent in patients located in
underdeveloped countries.1 Clinical findings of this pathology include cyanosis, swollen finger
tips, syncope, arrhythmia, kidney problems, and stroke.4 Sonographic findings include a VSD, an
overriding aorta, patent ductus arteriosus, or an ASD.3 These left-to-right shunts cause elevated
pulmonary pressures. This pulmonary hypertension leads to a change to right-to-left shunting.
Reversal of flow occurs because of pulmonary hypertension. The lack of oxygen being
distributed to the rest of body causes cyanosis.
Conclusion
The patient had similar characteristics compared to the ones listed in literature under
Eisenmenger Syndrome. This patient had low BUN and BUN/Creatinine ratios, which indicate
kidney problems. Kidney issues are associated with Eisenmenger Syndrome.4 This patient was
also experiencing brain infarcts, which would be cause by the lack of oxygen getting to his brain
because of the right-to-left shunting and reversal of flow.1,4 A day after this patient has the
echocardiogram done, an upper extremity venous was ordered because of swelling. Swelling of
the hands is a symptom of Eisenmenger Syndrome.4 Literature lists causes of Eisenmenger as
VSD, ASD, and patent ductus arteriosus.3 This patient did present with VSD and ASD, but not a
patent ductus arteriosus. The BMI of this patient was low, which could be a cause of the lack of
physical activity the patient was able to perform due to limited oxygen uptake resulting from an
inability to increase pulmonary blood flow.3 Even though this patient has survived into his third
decade of life, his prognosis seems to be getting worse. The patent was presenting with

hypoxemia, which is one of the three predictions for a decreasing prognosis. The other two are
syncope and increased right sided pressures.3 Sonographers should be aware that Eisenmenger
Syndrome progresses from certain defects like the VSD, ASD, and patent ductus arteriosus.
Proper diagnosis may assist in any therapy or surgeries that may be available to the patient.
Advanced therapy for patients with PAH is associated with lower risk of death, and selected
patients with Eisenmenger Syndrome are undergoing palliative measures or lung/heart-lung
transplant.5 If patient history is caught, recorded, diagnosed, and watched some patients may live
a longer more active life. Even though Eisenmenger Syndrome is fatal, some people may live
into their sixth decade.2

References
1. Beghetti M, Gali N. Eisenmenger Syndrome: A Clinical Perspective in a New
Therapeutic Era of

Pulmonary Arterial Hypertension. J Am Coll

Cardiol. 2009;53(9):733-740. doi:10.1016/j.jacc.2008.11.025

2. Diller G, Dimopoulos K, Broberg C, Kaya M, Naghotra U, Uebing A, Harries C,
Goktekin O, Gibbs S, Gatzoulis M. Presentation, Survival Prospects, and Predictors of
Death in Eisenmenger Syndrome: a Combined Retrospective and Case-Control Study.
European Heart Journal, July 2006, 27 (14) 1737-1742; DOI: 10.1093/eurheartj/ehl116
3. El-Chami M. Eisenmenger Syndrome. Medscape.
http://emedicine.medscape.com/article/154555-overview. Accessed November 27, 2015.
4. UCSF Medical Center. Eisenmengers Syndrome. UCSF Health.
http://www.ucsfhealth.org/conditions/eisenmengers_syndrome/. Accessed November 27,
2015.
5. Dimopoulos K, Inuzuka R, Goletto S, Giannakoulas G, Swan L, Wort S, Gatzoulis M.
Congenital Heart Disease: Improved Survival Among Patients with Eisenmenger
Syndrome Receiving Advanged Therapy for Pulmonary Arterial Hypertension.
Circulation. 2010;121:20-25.

Figure 1:

This figure is showing the single appearing ventricle with a VSD in the apical four chamber
view.
Figure 2.

Figure two is taken from the parasternal long axis view. It shows the left atrium, left ventricle,
and the enlarged aortic systemic vessel.

Figure 3.

Figure three is also from the apical four chamber view, and it is showing an absence of a
interatrial septum, suggesting an ASD.