Alveolar Capillary Dysplasia with Misalignment of Pulmonary Veins (ACD/MPV)

What is Alveolar Capillary Dysplasia / Misalignment of Pulmonary Veins (ACD/MPV)?

Lungs start to develop early during pregnancy. They continue to grow during childhood and don’t stop until adulthood. It is important that the airways, air sacs, and blood vessels are lined up in the right way.

ACD/MPV happens when the blood vessels of the lungs do not develop normally. The small blood vessels (capillaries) around the air sacs may be abnormal. This makes it hard for oxygen to move from the lungs to the blood. The small vessels that carry blood out of the lung (veins) may be abnormal or in the wrong place. This makes it hard for blood to leave the lungs with oxygen.

Babies with ACD/MPV have a lot of trouble breathing. They have low oxygen levels and high pressures in their heart. Babies can have trouble very quickly after birth and usually need help breathing to stay alive.

Diagnosis

ACD/MPV is possible when babies have trouble breathing soon after birth. Several tests may need to be done. These include:

• A chest x-ray could look normal. It might show areas of haziness in the lungs.
• A lung scan (chest CT) may show signs of abnormal lung development.
• A heart ultrasound (ECHO) can show high pressures in the heart.
• A lung biopsy is usually needed for the right diagnosis. This will show abnormal lung structure. The air sacs may look abnormal. The blood vessels and airways are not in the right places.
• Genetic testing may show mutations in genes that may cause abnormal lungs. Problems with the FOXF1 gene can cause this.

Infants with ACD/MPV may also have problems in other parts of their body.

Treatment

Treatment of ACD/MPV is to support breathing. Babies usually need help with oxygen and positive pressure. They may need a breathing tube and a ventilator. Medicines are used to help with breathing and with high pressures in the heart.

Babies who are very sick may need a treatment called ECMO (extracorporeal membrane oxygenation). They may need lung transplant to survive.

What Does ACD/MPV Mean for My Child?

Babies with ACD/MPV can be very sick and may die without lung transplant. There are some mild types of ACD/MPV that may be less serious. Your medical team will help you learn what your baby needs.

Support Group: ACD Association

What to Watch Out For

• If your child is working hard to breathe, talk to a health care provider. If possible, ask to see a lung specialist who is experienced with these conditions.
• Try to prevent infections from common childhood illnesses. Wash your hands often and ask your friends and family to let you know if they are sick before they are around your child.
• Get your child’s vaccines and yearly flu shot.

---

This information is for educational purposes only. It should not be used as a substitute for the medical advice of one’s healthcare provider.