Primary Pulmonary

Hypertension.
FFU
Medical faculty
Pediatric lecture
June 2020

04/12/2021 Dr Hussein 1
PATHOPHYSIOLOGY and causes.
• Primary pulmonary hypertension is characterized by pulmonary
vascular obstructive disease and right-sided heart failure.
• It occurs at any age, although in pediatric patients the diagnosis is
usually made in adolescence.
• In older patients, females outnumber males 1.7 : 1; in younger
patients, both genders are represented equally.
• Some patients have evidence of either an immunologic disorder or a
hypercoagulable state.

04/12/2021 Dr Hussein 2
Continue.
• Causes can be genetic and environmental factors.
• Pulmonary hypertension is a common complication of sickle cell
anemia and other hemolytic anemias.
• Pulmonary hypertension is associated with precapillary obstruction of
the pulmonary vascular bed as a result of hyperplasia of the muscular
and elastic tissues and a thickened intima of the small pulmonary
arteries and arterioles.
• Atherosclerotic changes may be found in the larger pulmonary
arteries as well

04/12/2021 Dr Hussein 3
Continue.
• In children, pulmonary venoocclusive disease may account for some
cases of primary pulmonary hypertension.
• Before a diagnosis of primary pulmonary hypertension can be made,
other causes of elevated pulmonary arterial pressure must be
eliminated (chronic pulmonary parenchymal disease, persistent
obstruction of the upper airway, congenital cardiac malformations,
recurrent pulmonary emboli, alveolar capillary dysplasia, liver disease,
autoimmune disease, and moyamoya disease).

04/12/2021 Dr Hussein 4
Continue.
• Pulmonary hypertension places an afterload burden on the right
ventricle, which results in right ventricular hypertrophy.
• Dilatation of the pulmonary artery is present, and pulmonary valve
insufficiency may occur.
• In the later stages of the disease, the right ventricle dilates, tricuspid
insufficiency develops, and cardiac output is decreased.
• Arrhythmias, syncope, and sudden death are common.

04/12/2021 Dr Hussein 5
CLINICAL MANIFESTATIONS.
• The predominant symptoms include exercise intolerance and
fatigability;occasionally, precordial chest pain, dizziness, syncope, or
headaches are noted.
• Peripheral cyanosis may be present, especially in patients with a
patent foramen ovale through which blood can shunt from right to
left; in the late stages of disease,patients may have cold extremities
and a gray appearance associated with low cardiac output.
• Arterial oxygen saturation is usually normal unless there is an
associated intracardiac shunt.

04/12/2021 Dr Hussein 6
Continue..
• If right-sided heart failure has supervened, jugular venous pressure is
elevated, and hepatomegaly and edema are present.
• The 1st heart sound is often followed by an ejection click emanating
from the dilated pulmonary artery.
• The 2nd heart sound is narrowly split, loud, and sometimes booming
in quality; it is frequently palpable at the upper left sternal border. A
presystolic gallop rhythm may be audible at the lower left sternal
border.

04/12/2021 Dr Hussein 7
Continue..
• The systolic murmur is soft and short and is sometimes followed by a
blowing decrescendo diastolic murmur caused by pulmonary
insufficiency.
• In later stages, a holosystolic murmur of tricuspid insufficiency is
appreciated at the lower left sternal border.

04/12/2021 Dr Hussein 8
Diagnosis.
• Chest X-ray reveal a prominent pulmonary artery and right ventricle .
• The pulmonary vascularity in the hilar areas may be prominent, in contrast
to the peripheral lung fields in which pulmonary markings are decreased.
• The ECG shows right ventricular hypertrophy, often with spiked P waves.
• Echocardiography is used to screen for any congenital cardiac
malformations.
• Doppler evaluation of the tricuspid valve, if insufficiency is present, will
allow estimation of the right ventricular (and hence pulmonary arterial)
systolic pressure.

04/12/2021 Dr Hussein 9
Continue..
• At cardiac catheterization, the presence of left-sided obstructive
lesions (pulmonary venous stenosis, mitral stenosis, restrictive
cardiomyopathy) that result in pulmonary venous hypertension can
be evaluated .
• The presence of pulmonary arterial hypertension with a normal
pulmonary capillary wedge pressure is diagnostic of primary
pulmonary hypertension.

04/12/2021 Dr Hussein 10
PROGNOSIS AND TREATMENT.
• Primary pulmonary hypertension is progressive, and no cure is
currently available.
• Some success has been reported with oral calcium channel blocking
agents such as nifedipine in children who demonstrate pulmonary
vasoreactivity when these agents are administered during
catheterization.
• Continuous intravenous infusion of the arachidonic acid metabolite,
prostacyclin provides relief as long as the infusion is continued.

04/12/2021 Dr Hussein 11
Continue..
• Despite the success of prostacyclin in reducing symptoms and
improving quality of life, it slows but does not stop the progression of
the disease.
• Continuous administration of nitric oxide via nasal cannula, nebulized
forms of prostacyclin and orally administered pulmonary vasodilators
(bosentan, an antagonist of endothelin receptors; or sildenafil, a
phosphodiesterase type 5 inhibitor) have been used with success in
adults, although clinical studies in children are of small numbers.

04/12/2021 Dr Hussein 12
Continue..
• Anticoagulation may be of value in patients with previous pulmonary
thromboemboli, and some of these patients will respond to balloon
angioplasty of narrowed pulmonary artery segments.
• Despite many advances, definitive therapy is still heart-lung or lung
transplantation .
• In patients with severe pulmonary hypertension and low cardiac
output, the terminal event is often sudden and related to a lethal
arrhythmia.
• Patients with primary pulmonary hypertension diagnosed in infancy
often have rapid progression and high mortality.
04/12/2021 Dr Hussein 13
Thanks for your attention.

04/12/2021 Dr Hussein 14