Pulmonary hypertension

By Asmaa Othman
Lecturer int. Medicine
 The definition of pulmonary hypertension (PH) is based upon right
heart catheterization measurements. PH is defined as a mean
pulmonary artery pressure greater than 25 mmHg at rest.
 CLASSIFICATION
1 Pulmonary arterial hypertension (PAH)
 1.1. Idiopathic PAH
 1.2. Heritable
 1.3. Drug- and toxin-induced
(aminorex, fenfluramine, SSRI?)
 1.4. Associated with
1.4.1. Connective tissue diseases
1.4.2. HIV infection
1.4.3. Portal hypertension
1.4.4. Congenital heart diseases
1.4.5. Schistosomiasis
1.4.6. Chronic hemolytic anemia
 1.5 Persistent pulmonary hypertension of the newborn
2.Pulmonary hypertension owing to left heart disease
3.Pulmonary hypertension owing to lung diseases and/or hypoxia
4.Chronic thromboembolic pulmonary hypertension (CTEPH)
5. Pulmonary hypertension with unclear multifactorial
mechanisms
CLINICAL OVERVIEW
( Low COP, Rt HF, Cause)

 Findings may be difficult to elucidate in patients with PH that is caused by an underlying

condition, because manifestations of the underlying disease frequently obscure those of
the PH.
 Most patients with PH initially experience exertional dyspnea, lethargy, and fatigue
(inability to increase cardiac output )
 As the PH progresses and right ventricular failure develops, exertional chest pain (ie,
angina), exertional syncope, and peripheral edema may develop
 In most circumstances, angina is due to subendocardial hypoperfusion caused by
increased myocardial oxygen demand.
 However, angina is occasionally caused by dynamic compression of the left main
coronary artery by an enlarged pulmonary artery; this risk is greatest for patients with a
pulmonary artery trunk at least 40 mm in diameter .
 Passive hepatic congestion may cause anorexia and abdominal pain in the right upper
quadrant.
 Less common symptoms of PH include cough, hemoptysis, and hoarseness (ie, Ortner's
syndrome) due to compression of the left recurrent laryngeal nerve by a dilated main
pulmonary artery
World Health Organization (WHO) functional
classification for pulmonary hypertension

7
PATHOGENESIS
 The primary cause of significant PH is almost always increased pulmonary vascular
resistance.
 Increased flow and increased pulmonary venous pressure alone does not usually cause
significant pulmonary hypertension because the pulmonary vascular bed vasodilates and
recruits vessels in response to increased flow, so that little, increased pressure results.
 Increased pulmonary blood flow through the pulmonary vasculature may be due to
congenital heart defects with left-to-right shunt (eg, atrial septal defects, ventricular septal
defects, patent ductus arteriosus) or liver cirrhosis.

 Increased pulmonary venous pressure may be due to mitral valve disease, left ventricular
systolic or diastolic dysfunction, constrictive pericarditis, or pulmonary venous obstruction
(eg, veno-occlusive disease).
 Increased pulmonary vascular resistance may be due to conditions associated with
occlusive vasculopathy of the small pulmonary arteries and arterioles (eg, idiopathic
PAH, collagen vascular disease, HIV infection), conditions that decrease the area of the
pulmonary vascular bed (eg, pulmonary emboli, interstitial lung disease), or conditions
that induce hypoxic vasoconstriction (eg, chronic obstructive pulmonary disease).
 On pathology, patients with PAH are found to have hyperplasia and hypertrophy of the
intima, media, and adventitia of the pulmonary arterial vasculature. On the molecular
level, this is related to endothelial dysfunction, which leads to disorganized endothelial
cell proliferation, decreased production of vasodilators such as prostacyclin and nitric
oxide, and overexpression of vasoconstrictors like endothelin. These pathophysiologic
mechanisms are particularly important as they guide the therapeutic targets of
pharmacotherapies for advanced PAH disease.
investigation

 Routine blood test

 CXR: changes plus cause
 ECG: rt ventricle hypertrophy ( tall R in V1and deep S in V6) and rt atrial enlargement (P
pulmonale)
 ECHO:
 Rt heart catheterization: to confirm PAP, determine pul wedge pressure, calculate COP,
vasodilator challenge(inhaled nitric oxide, IV adenosine or epoprostenol)
DIAGNOSTIC APPROACH
 TREATMENT
 Physical activity
 Pregnancy
 Vaccination
 Anesthesia
 Oral anticoagulant
 Diuretic
 Ca channel blockers
 Prostanoids: prostacyclin is a potant VD and inhibit plt aggregation
 Endothelin receptor antagonists (bosentan)
 Phosphodiestrase inhibitors (sildenafil)
TRANSPLANTATION
Transplantation has been performed in
patients with idiopathic pulmonary arterial Guidelines for when to refer a patient for
transplant evaluation are as follows :
hypertension (IPAH) and is considered by
some to be the final effective treatment for
• World Health Organization (WHO)
.selected patients with IPAH functional class III or IV
Bilateral lung or heart-lung transplantation is
the procedure of choice • Mean right atrial pressure >10 mmHg

• Mean pulmonary arterial pressure >50

mmHg

• Cardiac index <2.5 L/min per m2

• Failure to improve functionally despite

medical therapy

• Rapidly progressive disease

 Pulmonary embolism
 Pulmonary embolism (PE) is when a blood clot (thrombus) becomes lodged in an artery in
the lung and blocks blood flow to the lung. Pulmonary embolism usually arises from a
thrombus that originates in the deep venous system of the lower extremities; however, it
rarely also originates in the pelvic, renal, upper extremity veins, or the right heart chambers
(see the image below). After traveling to the lung, large thrombi can lodge at the bifurcation
of the main pulmonary artery or the lobar branches and cause hemodynamic compromise.

 Air, amniotic fluid amniotic fluid and fat emboli fat emboli are rarer causes
Etiology of Pulmonary Embolism

Three primary influences predispose a patient to blood clot formation; these form the so-called
Virchow triad:

 Endothelial injury
Trauma or inflammation

 Stasis or turbulence of blood flow

Venous stasis leads to accumulation of platelets and thrombin in veins. Increased viscosity
may occur due to polycythemia and dehydration, immobility, raised venous pressure in cardiac
failure, or compression of a vein by a tumor.

 Blood hypercoagulability
Sources of the embolus

 Most common is DVT (unilateral limb swelling and tenderness)

 Thrombus at Rt side of the HT; (Infective endocarditis, VSD, MI )
 Thrombus at Lt side of the HT: ( paradoxical shunt)
Epidemiology
 Pulmonary embolism is present in 60-80% of patients with DVT,
 even though more than half these patients are asymptomatic.
 Pulmonary embolism is the third most common cause of death in hospitalized patients,
with at least 650,000 cases occurring annually.
 Autopsy studies have shown that approximately 60% of patients who have died in the
hospital had pulmonary embolism, with the diagnosis having been missed in up to 70% of
the cases.
 Prospective studies have demonstrated DVT in 10-13% of all medical patients placed on
bed rest for 1 week, 29-33% of all patients in medical intensive care units, 20-26% of
patients with pulmonary diseases who are given bed rest for 3 or more days, 27-33% of
patients admitted to a critical care unit after a myocardial infarction, and 48% of patients
who are asymptomatic after a coronary artery bypass graft.
Risk factors for DVT
 Lower limb or pelvic injury or surgery
 Congestive heart failure
 Prolonged immobility
 Pregnancy
 Postpartum
 Oral contraceptive pills
 Malignancy
 Obesity
 Advanced age
 Coagulation problems
Clinical presentation :
vary according to size, site, no and lung condition
 Minute: may not cause infarction (double blood supply), asymptomatic if recurrent
complicate with PH and corpulmonale
 Medium sized (less than 65%): pul. Infarction (Chest pain, dyspnea, hemoptysis,…)
 Big size (65%-85%): Large infarction area, release of mediators (fever),RBCs hemolysis
(jaundice), acute PH and cor pulmonal (cyanosis, low COP, shock)
 Massive Pulmonary Embolism (more than 85%): It is a catastrophic entity which often
results in acute right ventricular failure and death, Frequently undiscovered until autopsy.
investigation

 CXR; may be normal, not diagnostic nor specific, wedge shaped opacity with central apex.
 ABG: hypoxia, hypocapnia,
 ECG: Rt v strain, Rt A enlarge, Rt axis deviation, S1Q3 T3 pattern, may be normal
 V/Q
 Angio
 Fibrin Split Products/D-dimer
 CT angiography
 Inv for cause: Doppler, ECHO
S1 Q3 T3 Pattern
Rt. Ventricular Strain
Chest radiograph showing pulmonary
infarct in right lower lobe
27

Pulmonary Angiogram
Treatment

 Respiratory support
 Hemodynamic Support
 Anticoagulation