eisenmenger syndrome (pulmonary arterial hypertension)

eisenmenger syndrome due to sinus venousus ASD with bidirectional shunt. kindly advice need of other drugs eg. hydroxyurea if needed

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It is a condition associated with VENTRICULAR SEPTAL DEFECT and other congenital heart defects that allow the mixing of pulmonary and systemic circulation, increase blood flow into the lung, and subsequent responses to low oxygen in blood. The left-to-right shunting of blood results in abnormally high blood flow and pressure directed to the right heart circulation, gradually leading to maladaptive changes that ultimately result in pulmonary hypertension. Increased right-sided blood volume and pressure causes a cascade of pathologic damage to the delicate pulmonary capillaries, causing them to be incrementally replaced with scar tissue. Although there's no cure, medications may help you feel better, improve your quality of life and prevent serious complications. Doctors don't generally recommend surgery to repair the hole in your heart once Eisenmenger syndrome has developed, and any surgery may be life-threatening. Sidanefil group of medicine is helpful to some extent in PAHT.

Do cath study and oximetry for reversibilty. Doesnot seems to me inoperable. He may undergo surgery and closure of ASD with leaving residual PFO. You may use Sildenafil 20 mg TDS or Boseantan 62.5 BD or Ambrisantan etc

Tab. Bosentan 62.5 mg. Cynosis and clubbing present. Heart lung transplant is only treatment for complete cure.

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