Absent Pulmonary Valve

September 16, 2022
Janelle Thomas MSN, RN
feature image

Finding out your child is dealing with a congenital heart defect can mean an immediate world of challenges, questions, and uncertainty. While this is a serious condition that requires immediate and extensive medical care, the bright spot is that timely surgical intervention leads to a positive outcome in many cases.

A clear picture of this condition, the treatment options, and how to care for a child who receives this diagnosis can help your family along the care journey. We’re happy to share this helpful overview so you can be as engaged and proactive as possible with your little one’s health.

What is absent pulmonary valve?

An absent pulmonary valve is caused by a congenital heart defect where the pulmonary valve in the heart does not develop, or does not develop fully. This valve flows from the heart to the lungs, delivering essential oxygen to the blood supply. The resulting lack of oxygen in the blood can cause a number of serious health problems and complications.

Absent pulmonary valve is often closely related to a condition called tetralogy of Fallot, which is a collection of four congenital heart defects. In addition to an absent or restricted pulmonary valve, these defects include a hole in the right and left ventricle wall, thickened right ventricle wall, and shifting of the aorta. A completely absent pulmonary valve is found in about 3% to 6% of cases of tetralogy of Fallot.

Absent Pulmonary Valve Causes

As a congenital heart defect, an absent pulmonary valve develops while the baby is still in the mother’s womb. There can be different underlying causes for heart defects of this kind, but it is often related to a genetic problem that can be hereditary or the result of spontaneous mutation. In many situations, diagnosing physicians are not able to fully identify the exact cause.

Absent Pulmonary Valve Risk Factors

Risk factors for an absent pulmonary valve are similar to tetralogy of Fallot and other congenital heart defects, and can include:

  • Family history of heart defects
  • Maternal age over 40
  • Nutritional issues before or during pregnancy
  • Consuming alcohol or drugs during pregnancy
  • Serious health condition or disease during pregnancy

Absent Pulmonary Valve Symptoms

Symptoms will often depend on the presence of other heart defects and problems, but can consist of:

  • Blue tint to the skin, particularly in the lips and extremities
  • Coughing, wheezing, and breathing difficulties
  • Diminished appetite and delayed growth
  • Overall failure to thrive
  • Respiratory complications and failure if overgrown blood vessels put pressure on the lungs

Absent Pulmonary Valve Complications

Serious complications can include collapsed lungs, stroke, brain infection, pneumonia, and heart failure, particularly on the right side. When recognized, and absent pulmonary valve will require immediate medical care to avoid long-term complications up to and including death.

Diagnosing Absent Pulmonary Valve

Absent pulmonary valve is usually diagnosed immediately at birth, or even before with the help of an echocardiogram. To confirm the diagnosis, specific tests can include an electrocardiogram, chest X-ray, MRI, and heart CT scan.

Absent Pulmonary Valve Treatment

If severe respiratory issues develop, life-saving heart surgery will be needed right away. Less severe cases will still need a procedure within the first three to six months of life.

Surgical goals for absent pulmonary valve include:

  • Replacement of the absent or defective pulmonary valve with donor tissue
  • Moving the pulmonary artery and rebuilding the artery wall to reduce pressure on the lungs and airways
  • Possible rebuilding of the windpipe and other airways

If other heart defects are present, additional surgery may be required. Treatment may include being placed on a ventilator before the procedure and during surgical recovery.

Caring for a Child with Absent Pulmonary Valve

After surgery to address an absent pulmonary valve, it is normal for children to have extensive care and monitoring needs. Families can expect regular appointments and imaging tests for heart function, activity changes, medications, and the use of supporting equipment such as a pacemaker.

There are many steps you can take to improve wellness and achieve a healthy and happy childhood for children with congenital heart defects such as absent pulmonary valve syndrome. Counseling and support groups can be especially helpful for managing emotional and behavioral health. A large number of families also turn to pediatric home health services to meet a wide range of care and support needs.

Contact Care Options for Kids For Pediatric Home Health Care

It can be hard to balance your time between work, home, and caring for a child. That’s why our team of skilled professionals at Care Options for Kids is here to help.

Our home health care services offer support in the comfort of your home. We refer loving and competent nurses to provide customized care for families — from a few hours a day to around-the-clock supervision. Contact us directly to speak with a home health care professional or request a free in-home assessment. Together we can determine the best plan of action to keep your loved ones happy and healthy.

If you are considering pediatric home health care services, contact the caring staff at Care Options for Kids. Call today at (888) 592-5855.