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Pulmonary Atresia or Right Ventricular Outflow Tract Obstruction

This is a congenital (present at birth) heart defect.

What it is

Right ventricular outflow tract obstruction (RVOTO) refers to a group of malformations that includes several defects, including pulmonary atresia and pulmonary valve stenosis. All RVOTO defects are evidenced by some kind of blockage or obstruction in the right ventricular outflow tract, which is the path through which blood flows from the right ventricle to the lungs. RVOTO usually appears as a part of other defects including tetralogy of Fallot, Williams syndrome, Noonan syndrome or in conjunction with an atrial septal defect.

Pulmonary atresia refers to the blockage (by a sheet of tissue) of the opening to the right side of the heart. Normally blood flows through this opening from the heart to the lungs to be reoxygenated. In babies with pulmonary atresia, blood cannot reach the lungs except via a blood vessel called the ductus arteriosus, which normally closes shortly after birth. Pulmonary valve stenosis is a similar condition, in which the opening is not blocked but is narrowed so that blood flow is limited.

Symptoms

Pulmonary Valve Stenosis:

  • Abdominal bloating
  • Cyanosis (bluish skin color)
  • Chest pain
  • Fainting
  • Fatigue
  • Poor weight gain
  • Shortness of breath
  • Sudden death

Pulmonary Atresia:

  • Cyanosis (bluish skin color)
  • Fast breathing/ shortness of breath
  • Fatigue
  • Poor eating habits

Causes

Anti-depressants: Maternal use of anti-depressants during pregnancy can double the risk of a baby to developing RVOTO, according to research published in the New England Journal of Medicine. The anti-depressants linked to this and other birth defects may include:

Painkillers: Exposure to opioid painkillers before birth can increase the likelihood of a baby developing RVOTO by 60%and pulmonary valve stenosis by 70%, according to research published in the American Journal of Obstetrics & Gynecology. Opioid painkillers include:

Clomid: Research conducted by the Centers for Disease Control and Prevention (CDC) found that babies exposed to the fertility drug Clomid face a 30% increased risk of being born with pulmonary valve stenosis.

Treatment

Treatment can vary based on whether RVOTO is part of another condition. Babies with pulmonary atresia or pulmonary valve stenosis will be administered medication that will keep the ductus arteriosus open so that blood can flow to the lungs. Other procedures will be necessary to provide a long-term solution, ranging from minor heart catheterization to open heart surgery to repair or replace the valve. In the most serious cases, the baby’s heart may need to be reconstructed or replaced in a heart transplantation procedure.

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