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What are the most common heart problems in CCHS?
The most common heart problems are arrhythmias and pulmonary hypertension.
What are arrhythmias ?
Arrhythmias are irregular heartbeats. The most frequent arrhythmias in CCHS are bradycardias in which heart beats at a lower rate or even stops during few seconds. These heart abnormalities can be asymptomatic or patients can have recurrent fainting episodes named syncopes (a transient loss of consciousness and posture tone with fall down and spontaneous recovery). If these pauses are prolonged they can lead to cardiac arrest or even sudden death.

How to diagnose arrhythmias?
Abnormalities in heart rhythm can be detected by prolonged electrocardiography (ECG) monitoring lasting 24-72 hours that is also called Holter monitoring.

Can these problems be treated?
On the basis of Holter monitoring and symptoms cardiologist will decide on the need of medication or in severe cases a cardiac pacemaker. The pacemaker is a small device that is implanted under the skin. It has wires which analyses heart rhythm and if it`s below a stablish value it sends electrical signals by wires to stimulate heartbeats.

What is pulmonary hypertension / cor pulmonale?
Pulmonary hypertension is high blood pressure in the arteries, that take the blood to the lungs to be oxygenated. Chronic low blood oxygen or high carbondioxide make these vessels to constrict their walls. Therefore the pressure in these vessels raises which is called pulmonary hypertension. Due to high pressure in these vessels the heart has to work harder to make the blood circulate through the lungs. If the pressure is too high the heart can't keep up, and becomes wider. This situation is called cor pulmonale.

How do I recognize pulmonary hypertension?
Mild pulmonary hypertension can be asymptomatic or cause bluish discolouration of the lips and skin, fatigue and shortness of breath. When patients develop core pulmonale those symptoms become worse and patients can have swelling around the ankles and feet (peripheral edema), and abdominal swelling (ascites).

How to diagnose pulmonary hypertension?
Pulmonary hypertension can be diagnosed by a cardiologist using Echocardiography (ECHO or cardiac ultrasound) and ECG
Can pulmonary hypertension be prevented or treated?
Yes, it can be prevented by avoiding low blood oxygene and high carbondioxide levels. Therefore optimal ventilation settings need to be secured early on. .
Once, cor pulmonale has developed, it can be treated by different options like optimizing ventilation oxygen and different types of medication

Are all fainting episodes due to arrhythmias in CCHS?
No, these children also can have fainting episodes related to orthostatic hypotension ? also called postural hypotension. This is a consequence of low blood pressure that happens when you stand up. Postural hypotension can also make you feel dizzy or lightheaded, or causes blurred vision.


update: 07/11/2012

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