GROUP 2 (PULMONARY

HYPERTENSION AND PULMONARY

HEART DISEASE)
 NUR/00010/022
 NUR/03010/022
 NUR/00045/022
 NUR/03005/022
 NUR/00012/022
Pulmonary
hypertension
Pulmonary Hypertension

 Pulmonary hypertension (PH) is characterized by elevated pulmonary arterial

pressure and secondary right heart ventricular failure. It may be suspected in a
patient with dyspnea with exertion without other clinical manifestations.
 Unlike systemic blood pressure, the pulmonary pressures cannot be measured
indirectly. In the absence of these measurements, clinical recognition becomes the
only indicator of PH. However, PH is a condition that is often not clinically
evident until late in its progression.
Pathophysiology

 Conditions such as collagen vascular disease, congenital heart disease,

anorexigens (specific appetite depressants), chronic use of stimulants, portal
hypertension, and HIV infection increase the risk of PH in susceptible patients.
 Vascular injury occurs with endothelial dysfunction and vascular smooth muscle
dysfunction, which leads to disease progression (vascular smooth muscle
hypertrophy, adventitial and intimal proliferation [thickening of the wall], and
advanced vascular lesion formation).
 Normally, the pulmonary vascular bed can handle the blood volume delivered by
the right ventricle. It has a low resistance to blood flow and compensates for
increased blood volume by dilation of the vessels in the pulmonary circulation
However, if the pulmonary vascular bed is destroyed or obstructed, as in PH, the
ability to handle whatever flow or volume of blood it receives is impaired, and the
increased blood flow then increases the pulmonary artery pressure.
As the pulmonary arterial pressure increases, the pulmonary vascular resistance also
increases. Both pulmonary artery constriction (as in hypoxemia or hypercapnia) and
a reduction of the pulmonary vascular bed (which occurs with PE) result in increased
pulmonary vascular resistance and pressure.
This increased workload affects right ventricular function. The myocardium
ultimately cannot meet the increasing demands imposed on it, leading to right
ventricular hypertrophy (enlargement and dilation) and failure.
Clinical Classification of Pulmonary
Hypertension (PH)
 Group 1: Pulmonary Arterial Hypertension (PAH) Sporadic idiopathic PAH Heritable
idiopathic PAH Drug and toxin-induced PAH. PAH due to diseases such as connective
tissues disorders, HIV infection, portal hypertension, congenital heart disease
 Group 2: PH due to left heart disease Systolic dysfunction Diastolic dysfunction
Valvular heart disease
 Group 3: PH due to chronic lung diseases and/or hypoxemia Chronic obstructive
pulmonary disease Interstitial lung disease Mixed restrictive and obstructive lung
disease Sleep disordered breathing
 Group 4: Chronic thromboembolic pulmonary hypertension (CTEPH) Due to
thromboembolic occlusion of the proximal or distal pulmonary vasculature
 Group 5: PH with unclear multifactorial mechanisms Hematologic disorders Systemic
disorders (e.g., sarcoidosis) Metabolic disorder
Clinical Manifestations

 Dyspnea, the main symptom of PH, occurs at first with exertion and eventually at
rest. Substernal chest pain also is common.
 Other signs and symptoms include weakness, fatigue, syncope, occasional
hemoptysis, and signs of right-sided heart failure (peripheral edema, ascites,
distended neck veins, liver engorgement, crackles, heart murmur).
 Anorexia and abdominal pain in the right upper quadrant may also occur
Assessment and Diagnostic Findings

 Diagnostic testing is used to confirm that PH exists, determine its severity, and
identify its causes.
 Initial diagnostic evaluation includes
1. history,
2. physical examination,
3. chest x-ray,
4. pulmonary function studies,
5. electrocardiogram (ECG), and echocardiogram.
Medical Management

 The primary goal of treatment is to manage the underlying condition related to PH

if the cause is known.
 diuretics,
 oxygen,
 anticoagulation,
 digoxin,
 exercise training.
 Diuretics and oxygen should be added as needed. Appropriate oxygen therapy
reverses the vasoconstriction and reduces the PH in a relatively short time.
 Most patients with PH do not have hypoxemia at rest but require supplemental
oxygen with exercise. Anticoagulation should be considered for patients at risk for
intrapulmonary thrombosis.
 Digoxin may improve right ventricular ejection fraction in some patients and may
help to control heart rate; however, patients must be monitored closely for
potential complications
Pharmacologic Therapy

 Different classes of medications are used to treat PH; these include calcium
channel blockers, prostanoids, endothelin antagonists, and phosphodiesterase-5
inhibitors.
Surgical Management
 Lung transplantation remains an option for a select group of patients with PH
who are refractory to medical therapy. Bilateral lung or heart–lung transplantation
is the procedure of choice. Atrial septostomy may be considered for selected
patients with severe disease inhibitors.
Nursing Management

 The major nursing goal is to identify patients at high risk for PH, such as those with
COPD, PE, congenital heart disease, and mitral valve disease so that early treatment
can commence.
 The nurse must be alert for signs and symptoms, administer oxygen therapy
appropriately, and instruct the patient and family about the use of home oxygen
therapy.
 In patients treated with prostanoids (e.g., epoprostenol or treprostinil), education
about the need for central venous access (epoprostenol), subcutaneous infusion
(treprostinil), proper administration and dosing of the medication, pain at the
injection site, and potential severe side effects is extremely important.
 Emotional and psychosocial aspects of this disease must be addressed.
 Formal and informal support groups for patients and families are extremely valuable
PULMONARY
HEART DISEASE
Pulmonary heart disease (PHD)

 Also known as cor pulmonale.

 This is the altered structure or function of the right ventricle occurring in
association with abnormal respiratory function.
 Normally, the Right ventricle (RV) is thin walled, it pumps blood a short
distance to a low resistance system ;lungs
 In PHD the right ventricle enlarges and ultimately fails as a response to increased
vascular resistance thus high blood pressure in the lungs. This is referred to as
pulmonary hypertension.
 Abnormal gas exchange is a fundamental underpinning of PHD
Causes of PHD.
1. Autoimmune diseases that damage the lungs such as scleroderma
2. Chronic obstructive pulmonary disease
3. Severe bronchiectasis- damaged large airways in the lungs being
permanently wide.
4. Damage to lung tissue in conditions such as in severe scarring by interstitial
lung disease.
5. Damage to pulmonary vessels e.g. in chronic thromboembolisms or
recurrent blood clots
6. Anything affecting the spine or ribcage e.g. kyphoscoliosis where the spine
is curved and the lungs cannot fully expand.
7. Cystic fibrosis- thick sticky mucus builds up in the lungs
8. Obstructive sleep apnea that causes a drop in oxygen levels.
Pathophysiology.

 Pulmonary circulation a low pressure system with pressures ranging from

10mmHg – 14mmHg.
 Lung disorders make it harder to oxygenate blood which leads to hypoxia or low
oxygen levels.
 These low oxygen levels lead to hypoxic pulmonary vasoconstriction.
 In turn leading to an increased resistance in pulmonary vessels and thus
pulmonary hypertension with pressure rising above 25mmHg.
 The high pulmonary pressure makes it hard for the right ventricle to pump blood
into the pulmonary circulation
Pathophysiology cont.

 Acute lung disorders such a pulmonary embolisms cause rapid rise in pressure
leading to the right ventricle dilating.
 Chronic lung disorders such as chronic obstructive pulmonary disease cause
prolonged high pressure on the right ventricle leading to hypertrophy of muscles
of the right ventricle so it can contract with more force.
 This enlargement of the muscle wall results in reduced ventricular volume less
blood occupies this space leading to diastolic heart failure.
Pathophysiology cont.

 An increase in RV muscle mass would mean an increased demand for oxygen.

Coronaries get squeezed down by the extra muscle so that even less blood is
supplied to the RV.
 More demand and reduced supply leads to right ventricular ischemia and weaker
contractions hence systolic heart failure.
NOTE Pulmonary hypertension is different from primary pulmonary
hypertension. Primary HTN is an vasculopathy exclusively affecting
pulmonary circulation whereas pulmonary HTN previously known as secondary
HTN ,is associated with causal underlying disease process.
Clinical manifestations.
 With the distressed lungs shortness of breath , chronic cough, chest pain,
fatigue and fainting will be experienced.
 Hemoptysis –spitting of blood that originated from lungs or bronchial tubes
 Physical findings may reflect the underlying lung disease
 RV dilation in acute PHD and RV hypertrophy in chronic PHD
 Increased pulmonary pressure
 PHD causes back up of blood in the venous system. This fluid congestion
presents as :
 Jugular venous distention
 Hepatomegaly
 Pitting edema in lower extremities
Complications

 Fainting
 Hypoxia
 Pedal edema
 Passive hepatic congestion
 Death
Differential diagnosis
 Consider :
 Right sided heart failure due to congestive heart diseases.
 Biventricular heart failure
 Primary pulmonary hypertension
 Blood disorders that increase blood viscosity
 Thromboembolic disease
 Atrial myxoma-noncancerous tumor in the upper left or right side of the heart
often on atrial septum.
Diagnosis of PHD
 Diagnosis is made with an echocardiogram that shows evidence of increased
pressure in the pulmonary arteries and right ventricle.
 Right heart catheterization directly measures and assesses for response to
vasodilating medication. This is the most accurate but also most invasive.
 Follow up tests are done to identify the underlying cause e.g. spirometry for
chronic lung diseases.
Medical management

 Treatment targets the underlying lung condition.

 Supplemental oxygen given for hypoxia induced vasoconstriction
 Diuretics
 Vasodilators for chronic PHD
 Vasoconstrictors for acute PHD
 Physical exercise
 Anticoagulation therapy
surgical management

 Single – lung, double - lung and heart transplantation.

 Uvulopalatopharangoplastasty ; opening upper airway by taking out extra tissue in
the throat to treat mild obstructive sleep apnea.
 Surgical embolectomy (open heart surgery) for massive pulmonary embolism
 Phlebotomy for chronic cor pulmonale and chronic hypoxia- reveals a hematocrit
of 65% or more this is known as polycythaemia.
 Pulmonary embolectomy
Nursing intervention
 Patient education on importance of drug adherence to improve morbidity and
mortality.
 Promoting activity tolerance – 30 minutes of physical activity every day
should be encouraged.
 Managing fluid volume- fluid status be monitored closely as well as adhering
to a low sodium diet.
 Controlling anxiety- breathing difficulties tend to cause anxiety. Help client
manage anxiety, ensure physical comfort, breathing exercises and provide
psychological support
 Provide information on disease therapy needs and prevention of recurrences.
Prevention of PHD
 Controlling certain medical conditions such as blood clotting disorders.
 Regular exercise
 Eating well balanced diet to avoid hypertension and heart disease
 Treating sleep apnea
 Avoiding lung irritants where possible such as chemical fumes and dust.
 Maintaining a healthy weight.
 Stop smoking.