Eisenmenger syndrome

Eisenmenger
Syndrome
Ms. Feba Geevarghese
Vice Principal & Ph.D. Scholar
College of Nursing, St. Stephen’s Hospital
Delhi

GENERAL OBJECTIVE
 At the end of the teaching, students will acquire
knowledge regarding Eisenmenger Syndrome, its
pathophysiology, clinical manifestations, medical
management and demonstrate skills in nursing
management of the patient.

SPECIFIC OBJECTIVES
At the end of the class, the students will be able to:
 define Eisenmenger Syndrome
 describe anatomy and physiology of the heart
 identify the cause of Eisenmenger Syndrome
 outline the pathophysiology of Eisenmenger Syndrome
 enumerate the clinical manifestations of Eisenmenger Syndrome
 identify the diagnostic evaluation of Eisenmenger Syndrome
 discuss the medical management of Eisenmenger Syndrome
 explain the nursing management of Eisenmenger Syndrome using
nursing process approach
 describe the points for patient education

Definition
 Eisenmenger syndrome was so named by Dr. Paul Wood after Dr. Victor
Eisenmenger, who first described the condition in 1897.
 Eisenmenger's syndrome (or ES, Eisenmenger's reaction, Eisenmenger
physiology, or tardive cyanosis) is defined as the process in which a long-
standing left-to-right cardiac shunt caused by a congenital heart
defect (typically by a ventricular septal defect, atrial septal defect, or less
commonly, patent ductus arteriosus) causes pulmonary hypertension and
eventual reversal of the shunt into a cyanotic right- to- left shunt.

Anatomy and Physiology of the Heart

Anatomy and Physiology of the Heart..

 The heart is a muscular organ which pumps blood through
the blood vessels of the circulatory system. The heart is divided
into four chambers: upper left and right atria; and lower left and
right ventricles. Commonly the right atrium and ventricle are
referred together as the right heart and their left counterparts as
the left heart. In a healthy heart blood flows one way through
the heart due to heart valves, which prevent backflow. The heart
is enclosed in a protective sac, the pericardium, which also
contains a small amount of fluid. The wall of the heart is made up
of three layers: epicardium, myocardium, and endocardium.

 The heart pumps blood with a rhythm determined by a group of pacemaking cells
in the sinoatrial node. These generate a current that causes contraction of the
heart, traveling through the atrioventricvular node and along the conduction
system of the heart. The heart receives blood low in oxygen from the systemic
circulation, which enters the right atrium from the superior and inferior
venacavae and passes to the right ventricle. From here it is pumped into the
pulmonary circulation, through the lungs where it receives oxygen and gives off
carbon dioxide. Oxygenated blood then returns to the left atrium, passes through
the left ventricle and is pumped out through the aorta to the systemic
circulation−where the oxygen is used and metabolized to carbon dioxide. The
heart beats at a resting rate close to 72 beats per minute.

 The valves between the atria and ventricles are called the atrioventricular valves.
Between the right atrium and the right ventricle is the tricuspid valve. The tricuspid
valve has three cusps, which connect to chordae tendinae and three papillary
muscles named the anterior, posterior, and septal muscles, after their relative
positions. The mitral valve lies between the left atrium and left ventricle. It is also
known as the bicuspid valve due to its having two cusps, an anterior and a posterior
cusp. These cusps are also attached via chordae tendinae to two papillary muscles
projecting from the ventricular wall.

 The papillary muscles extend from the walls of the heart to valves
by cartilaginous connections called chordae tendinae. These
muscles prevent the valves from falling too far back when they
close. During the relaxation phase of the cardiac cycle, the
papillary muscles are also relaxed and the tension on the chordae
tendineae is slight. As the heart chambers contract, so do the
papillary muscles. This creates tension on the chordae tendineae,
helping to hold the cusps of the atrioventricular valves in place
and preventing them from being blown back into the atria.

 Two additional semilunar valves sit at the exit of each of the ventricles.
The pulmonary valve is located at the base of the pulmonary artery. This has three
cusps which are not attached to any papillary muscles. When the ventricle relaxes
blood flows back into the ventricle from the artery and this flow of blood fills the
pocket-like valve, pressing against the cusps which close to seal the valve. The
semilunar aortic valve is at the base of the aorta and also is not attached to papillary
muscles. This too has three cusps which close with the pressure of the blood flowing
back from the aorta.

 Heart tissue, like all cells in the body, needs to be supplied
with oxygen, nutrients and a way of removing metabolic wastes.
This is achieved by the coronary circulation, which
includes arteries, veins and lymphatic vessels. Blood flow through
the coronary vessels occurs in peaks and troughs relating to the
heart muscle's relaxation or contraction.

 Heart tissue receives blood from two arteries which arise just above the aortic
valve. These are the left main coronary artery and the right coronary artery.
The left main coronary artery splits shortly after leaving the aorta into two
vessels, the left anterior descending and the left circumflex artery. The left
anterior descending artery supplies heart tissue and the front, outer side, and
the septum of the left ventricle. It does this by branching into smaller arteries –
diagonal and septal branches. The left circumflex supplies the back and
underneath of the left ventricle. The right coronary artery supplies the right
atrium, right ventricle, and lower posterior sections of the left ventricle. The
right coronary artery also supplies blood to the atrioventricular node (in about
90% of people) and the sinoatrial node (in about 60% of people). The right
coronary artery runs in a groove at the back of the heart and the left anterior
descending artery runs in a groove at the front.

 The coronary sinus is a large vein that drains into the right atrium, and receives
most of the venous drainage of the heart. It receives blood from the great
cardiac vein (receiving the left atrium and both ventricles), the posterior cardiac
vein (draining the back of the left ventricle), the middle cardiac vein (draining the
bottom of the left and right ventricles), and small cardiac veins. The anterior
cardiac veins drain the front of the right ventricle and drain directly into the right
atrium.

Cause of Eisenmenger's syndrome
 A number of congenital heart defects can cause
Eisenmenger syndrome, including atrial septal
defects, ventricular septal defects, patent ductus arteriosus,
and more complex types of acyanotic heart disease.

PATHOPHYSIOLOGY OF
EISENMENGER'S SYNDROME

The larger and more muscular left side of the heart must
generate the high pressure required to supply blood to the
extensive, high-resistance systemic circulation. In contrast, the
smaller, right side of the heart must generate a much lower
pressure in order to pass blood through the low-resistance,
high compliance circulation of the lungs.
If a significant anatomic defect (i.e. a hole or
breach) exists between the two sides of the heart,
a shunt will occur, causing blood to flow down the
normal pressure gradient from the left side to the
right side.
The left-to-right shunting of blood results in abnormally high
blood flow and pressure directed to the right heart
circulation, gradually leading to maladaptive changes that
ultimately result in pulmonary hypertension.

Eventually, due to increased resistance and decreased compliance of the
pulmonary vessels, elevated pulmonary pressures cause the myocardium
of the right heart to hypertrophy(RVH)
The scar tissue also provides less flexibility and compliance than normal
lung tissue, causing further increases in pulmonary blood pressure, and
the weakened heart must pump harder to continue supplying the lungs,
leading to damage of more capillaries
Increased right-sided blood volume and pressure causes a cascade of
pathologic damage to the delicate pulmonary capillaries, causing them to
be incrementally replaced with scar tissue

The defect, now a right-to-left shunt, causes reduced oxygen saturation in
the arterial blood due to mixing of oxygenated blood returning from the
lungs with the deoxygenated blood returning from systemic circulation.
As a consequence, deoxygenated blood returning from the body bypasses
the lungs through the reversed shunt and proceeds directly to systemic
circulation, leading to cyanosis and resultant organ damage.
The onset of Eisenmenger's syndrome begins when right ventricular
hypertrophy causes right heart pressures to exceed that of the left heart,
leading to reversal of blood flow through the shunt (i.e., blood moves from
the right side of the heart to the left side)

This decreased saturation is
sensed by the kidneys,
in a compensatory increase
in erythropoietin production
and an increased production
red blood cells in an attempt
increase oxygen delivery
As the bone marrow increases
erythropoiesis, the systemic
reticulocyte count and the risk
for hyperviscosity
syndrome increases

Clinical Manifestations of Eisenmenger
syndrome
 Cyanosis (a blue tinge to the skin
resulting from lack of oxygen)
 High red blood cell count
 Swollen or clubbed finger tips
(clubbing)
 Fainting (also known as syncope)
 Heart failure
 Abnormal heart rhythms
 Bleeding disorders
 Coughing up blood
 Iron deficiency
 Infections (endocarditis and
pneumonia)
 Kidney problems
 Stroke
 Gout (rarely) due to increased uric
acid resorption and production
with impaired excretion
 Gallstones

Diagnostic Evaluation
 Imaging tests like an X-rays, CT scan, or an echocardiogram
 An electrocardiogram (EKG or ECG) or a walking test (to measure your heart rate and
blood flow)
 Blood tests (to look for abnormally high or abnormally low red blood cell counts)
 Cardiac catheterization (for detailed information about the heart)
 Lung function tests (to measure how much oxygen is getting to the bloodstream)

Medical Management
Much of the therapy being used for Eisenmenger syndrome has been studied in the
treatment of Idiopathic Pulmonary Arterial Hypertension (IPAH)
 Calcium channel blockers- These medicines lower blood pressure in the lungs and
the rest of the body.
 Anti- coagulants- Survival is increased when patients are treated with anticoagulant
therapy. Warfarin should be used, provided the patient has no contraindications to
anticoagulation. Maintain an international normalized ratio (INR) of 1.5 to 2.
 Nitric Oxide (NO)- Nitric oxide (NO) is a potent and selective pulmonary arteriolar
vasodilator produced in endothelial cells. Studies have shown inhaled NO to reduce
pulmonary vascular resistance with minimal systemic effects in patients with ES and
acute pulmonary hypertensive states of other etiologies.

Medical Management…
 Endothelin-1 (ET-1) Receptor Antagonists- ET-1 is a powerful vasoconstrictor with
elevated concentrations in the plasma and lung tissue of patients with PAH. It plays a
key role in the pathogenesis of PAH including in vitro effects on proliferation, fibrosis
and inflammation.
 Bosentan- Bosentan is a non-selective endothelin receptor antagonist
 Sildenafil(Viagra) and tadalafil - have shown beneficial effects on pulmonary
selectivity and arterial oxygenation. demonstrating an improvement in exercise
capacity, as assessed according to the six-minute walking test, functional class and
hemodynamics in PAH patients.

 Prostacyclin and Prostacyclin Analogs- Prostanoids can be
administered by continuously intravenous or subcutaneous infusion,
by inhalation and orally. Due to their active profile with vasodilatory,
antiproliferative, anti-inflammatory and anticoagulant effects, they are
suitable drugs for the treatment of PAH.
 Epoprostenol- Prostacyclin is a potent endogenous vasodilator
produced in the vascular endothelium. Epoprostenol was the first
synthetic prostacyclin analog, which became standard therapy of
severe PAH in many countries.
 Digoxin- Digoxin therapy can be used to improve right ventricular
function in patients with right ventricular failure

 Diuretics- Use diuretics to manage peripheral edema. The use of loop
diuretics (eg, furosemide, bumetanide) requires potassium
supplementation and close monitoring of serum potassium.
Potassium-sparing diuretics may have a role in ameliorating the
sometimes-intractable hypokalemia observed with daily diuretic use.
 Oxygen therapy- Give supplemental oxygen in patients with resting
or exercise-induced hypoxemia.
 Therapeutic erythropheresis
 Prevent infections (antibipotics)

Surgical Management
 Surgical palliation or repair should be performed early in patients
with congenital heart disease to prevent progression to
Eisenmenger syndrome. No surgical care is available to correct the
congenital cardiac defect that caused the pathologic pulmonary
vascular changes once Eisenmenger syndrome has developed to
the irreversible stage.
 Heart-lung transplantation and single or bilateral, sequential lung
transplantation
 Atrial septostomy- A surgeon creates an opening between the
right and left sides of the heart.

NURSING MANAGEMENT USING NURSING
PROCESS APPROACH
Assessment
 Vital signs
 Current weight
 Cardiac auscultation for a systolic murmur
 Pulmonary auscultation for crackles
 Measurement of jugular vein distention.

PROCESS APPROACH…
Nursing Diagnoses
 Decreased cardiac output related to the disease process and
medical treatments
 Ineffective cardiopulmonary, cerebral, peripheral, and renal
tissue perfusion related to decreased cardiac output
 Impaired gas exchange related to pulmonary congestion caused
by cardiac failure
 Activity intolerance related to failing heart function
 Powerlessness related to disease prognosis

PROCESS APPROACH…
Planning and Goals
The major goals for the patient include:
 improved or maintained cardiac output
 increased activity tolerance
 reduction of anxiety
 absence of complications

PROCESS APPROACH…
Nursing Interventions
 Improving Cardiac Output
 During a symptomatic episode, rest is indicated
 Assessing the patient’s oxygen saturation at rest and during activity
 Provide supplemental oxygen
 Ensuring that medications are taken as prescribed to preserve adequate cardiac
output
 Determining the patient’s weight every day
 Keeping patient warm and frequently changing position to stimulate circulation and
reduce the possibility of skin breakdown

PROCESS APPROACH…
 Increasing Activity Tolerance
 Plan the patient’s activities so that they occur in cycles, alternating rest with activity
periods
 Avoid strenuous activity and sports
 Provide oxygen supplementation and medications
 Reducing Anxiety
 Spiritual, psychological, and emotional support may be indicated for the patient,
family, and signiﬁcant others
 If the patient is facing death or awaiting transplantation, time must be provided to
discuss these issues
 Nurses help the patient, family, and signiﬁcant others with anticipatory grieving

PROCESS APPROACH…
 Promoting Home and Community-Based Care
(a). Teaching Patients Self-Care-
 Teaching patients about the medication regimen, symptom
monitoring
 Helping patients cope with their disease status assists them
in adjusting their lifestyles

PROCESS APPROACH…
(b). Continuing Care-
 The nurse reinforces previous teaching and performs ongoing assessment of the
patient’s symptoms and progress
 Teaching patients diet and ﬂuid restrictions
 Maintain a record of daily weights and symptoms
 Organize daily activities to increase activity tolerance can be helpful
 Patient’s responses to and to the medication regimen are assessed
 Explanations about symptoms that should be reported to the physician are emphasized
 Because of the risk of dysrhythmia, the patient’s family may be taught cardiopulmonary
resuscitation. Women are often advised to avoid pregnancy
 The nurse assesses the psychosocial needs of the patient and family on an ongoing
basis

PROCESS APPROACH…
 Evaluation
Expected Patient Outcomes
Maintains or improves cardiac function
a. Exhibits heart and respiratory rates within normal limits
b. Reports decreased dyspnea and increased comfort
c. Reports no weight gain
d. Maintains or increases activity tolerance
e. Carries out activities of daily living
f. Reports increased tolerance to activity

PROCESS APPROACH…
2. Is less anxious
a. Discusses prognosis freely
b. Verbalizes fears and concerns
3. Adheres to the self-care program
a. Takes medications according to prescribed schedule
b. Modifies diet to accommodate sodium and fluid restrictions
c. Modifies lifestyle to accommodate recommended activity and rest behaviors

Living with Eisenmenger Syndrome
 Pregnancy is not recommended for women with Eisenmenger’s syndrome.
Pregnancy may be dangerous for the mother and may cause complications for
the fetus due to effects of low oxygen levels in the blood.
 Any anesthesia and surgery is considered high risk and should be carefully
planned. Collaboration with a cardiac specialist is recommended.
 Air travel and high altitude exposure requires adequate hydration (fluid intake) and
supplemental oxygen to prevent complications.
 Severe headache, dizziness, syncope (fainting), or changes in level of
consciousness should be taken seriously and evaluated emergently.

Living with Eisenmenger Syndrome…
 Smoking and alcohol intake are not recommended.
 Coughing should be controlled or prevented with a strong
suppressant medication to prevent risk of pulmonary hemorrhage
(bleeding from the lungs).
 Flu shots are recommended annually, and pneumococcal vaccine
should be received according to the doctor’s recommendation.
 Avoid hemodynamic shifts such as dehydration

Eisenmenger syndrome

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