My 9 y/o boy was diagnosed with Idiopathic Pulmonary Arterial Hypertension (IPAH), what do you recommend?

Dear Doctors
Hello
9-year-old boy three years ago with shortness of breath at rest and when you’re talking to a doctor pulmonologist and cardiologist referral and ipah disease was diagnosed.
Sildenafild and bosentan reduced pulmonary arterial pressure to consume drugs.
Angiography and echocardiography is attached.
Please ..
Your recommendations as to best treatment(…)?
thanks

ipah-disease


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mrsshalini14docc4youDr MahaveerDr Ahmed Fawzy Recent comment authors
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Dr Ahmed Fawzy
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Dr Ahmed Fawzy

In my opinion you need to start treatment early before right ventricular hypertrophy occur
Low salt and low liquids
Early drug treatment to prevent complications
Many possible drugs can be used like nitric oxide , prostanoid(do not recommed this one) or remodulin
U should consult your doctor in which drugs will be used

Also a shunt can be done to reduce pressure (very good i would go with it if your doctor suggests it )

Good Luck

Dr Mahaveer
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Dr Mahaveer

hi
the reports are not much alarming and things appear to be under control.
give him the prescribed medicines to prevent from further complications.
thanks

shw12
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shw12

HI
REPORTS DO SUGGEST PAH. HE SHOULD TAKE THE MEDICINES REGULARLY.
A 3 MONTHLY FOLLOW UP WITH CARDIOLOGIST IS RECOMMENDED TO SEE THE PROGRESS OF THE DISEASE.

mrsshalini14
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mrsshalini14

hello there
at present the prescribed medicines shoul be taken regularly. there are chances of partial cardiac failure if PAH worsens.
Percutaneous catheterisation techniques are available and you should check it out with your cardiologist.