By M.elkhatib

Understand the physiology of pulmonary hypertension.

Understand the signs and symptoms of pulmonary hypertension.

Types pulmonary hypertension. Understand the treatment of a pulmonary

hypertension and a crisis.

• Pulmonary hypertension occurs when there Pulmonary hypertension occurs when there is abnormally high pressure within the blood is abnormally high pressure within the blood vessels of the lungsvessels of the lungs.

• Normally, blood that flows through these Normally, blood that flows through these vessels meets little resistance, as they vessels meets little resistance, as they naturally widen to allow blood from the naturally widen to allow blood from the heart to pass through smoothly to pick up heart to pass through smoothly to pick up oxygen and deliver it to the body.oxygen and deliver it to the body.

• In pulmonary hypertension, the blood In pulmonary hypertension, the blood vessels constrict, becoming narrow and vessels constrict, becoming narrow and thick. This means less blood is able to flow thick. This means less blood is able to flow through the blood vessels through the blood vessels

• When this happens, pressure within the When this happens, pressure within the vessels builds up and the heart muscle vessels builds up and the heart muscle must work harder to force the blood must work harder to force the blood through. through.

When the pressure becomes too high, the When the pressure becomes too high, the heart can't keep up and less blood is heart can't keep up and less blood is circulated through the lungs. This results in circulated through the lungs. This results in less oxygen to the entire body.less oxygen to the entire body.

Pulmonary Hypertension affects more women then men.

Primary pulmonary hypertensionPrimary pulmonary hypertension Also known as idiopathic pulmonary Also known as idiopathic pulmonary

hypertension has no identifiable underlying hypertension has no identifiable underlying cause. cause.

Secondary pulmonary hypertension is Secondary pulmonary hypertension is defined as elevated pulmonary pressures defined as elevated pulmonary pressures secondary to either a pulmonary or a cardiac secondary to either a pulmonary or a cardiac disorder disorder

Persistent pulmonary hypertension of the Persistent pulmonary hypertension of the newborn (PPHN)newborn (PPHN)

•Any condition that causes chronic low oxygen levels in the blood•Autoimmune diseases that damage the lungs, such as scleroderma and rheumatoid arthritis•Certain birth defects of the heart•Certain diet medications•Congestive heart failure•History of a blood clot in the lung•HIV infection•Lung or heart valve disease•Obstructive sleep apnea

TachypnoeaTachypnoea Increased WOB(work of breathing)Increased WOB(work of breathing) CyanosisCyanosis hypoxiahypoxia TachycardiaTachycardia

Shortness of breath or light-headedness during activity is often the first symptom.

Over time, symptoms occur with lighter activity or even while at rest

Ankle and leg swelling Bluish color of the lips or skin (cyanosis) Chest pain or pressure, usually in the front

of the chest Dizziness or fainting spells Fatigue Weakness

Elevated pulmonary artery pressure in Elevated pulmonary artery pressure in congenital heart disease is caused by congenital heart disease is caused by pulmonary over circulation, pulmonary pulmonary over circulation, pulmonary vasoconstriction, and pulmonary vascular vasoconstriction, and pulmonary vascular disease, either alone or in combination. disease, either alone or in combination.

Associated with 2 major categories of CHDAssociated with 2 major categories of CHD

1)Those resulting in increased pulmonary 1)Those resulting in increased pulmonary blood flow and increased pulmonary arterial blood flow and increased pulmonary arterial pressure e.g.. VSD,ASD,AVSD,PDApressure e.g.. VSD,ASD,AVSD,PDA

2)Those resulting in increased pulmonary 2)Those resulting in increased pulmonary venous pressure e.g. TAPVD, post TCPCvenous pressure e.g. TAPVD, post TCPC

In congenital heart disease with a left to right shunt, high pulmonary blood flow increases shear stress and causes obstructive pulmonary hypertension.

Existing pulmonary hypertension is exacerbated post cardiopulmonary bypass due to reperfusion injury causing further endothelial dysfunction.

Abnormal heart sounds (especially a splitting of the second heart sound)

Enlargement of the veins in the neck Feeling of a pulse over the breastbone Heart murmur Leg swelling Liver and spleen swelling Normal breathing sounds

Cardiac catheterization Chest x-ray CT scan of the chest Echocardiogram ECG Nuclear lung scan Pulmonary arteriogram Pulmonary function tests Sleep study

Right heart strain (RVH and RA enlargement)

Right axis deviation

ST depression and T wave inversion V1-V3

There is no known cure.

The goal of treatment is to control symptoms. It is important to treat medical disorders that cause pulmonary hypertension, such as obstructive sleep apnea, lung conditions, and heart valve disorders

OxygenOxygen Nitric OxideNitric Oxide Sildenafil (Viagra)Sildenafil (Viagra) ProstacyclinProstacyclin BosentanBosentan DiureticsDiuretics

1. Bag the child in 100% oxygen with high PEEP and hyperventilate them.

2. Sedate and paralyse the child immediately. Fentanyl is the drug of choice with these children.

3. Nitric Oxide at 20ppm

A pulmonary hypertensive crisis is an EMERGENCY. If it is not treated immediately, the child will DIE!!!

Avoid pregnancy Avoid strenuous activities and heavy lifting Avoid traveling to high altitudes Keep up to date with yearly flu and

pneumococcal pneumonia vaccines Stop smoking

The long-term outlook has been poor, but new therapies may produce better results. Some people with this condition may develop progressive heart failure that may lead to death

PH is elevated pulmonary pressuresPH is elevated pulmonary pressures Caused by a variety of factorsCaused by a variety of factors Very difficult to treat and not curableVery difficult to treat and not curable Some children with CHD at increased risk of Some children with CHD at increased risk of

developing PHdeveloping PH New therapies promising but still uncertainNew therapies promising but still uncertain