Wentworth-Douglass Hospital
(603) 742-5252
Decrease (-) Restore Default Increase (+) font size
Physicians
Ebola: what you should know
Health Library
Back to Health Library   Print This Page Print    Email to a Friend Email

Heart, section through the middle
Heart, section through the middle


Heart, front view
Heart, front view


Definition:

Pulmonary atresia is an extremely rare form of congenital heart disease in which the pulmonary valve does not form properly. The pulmonary valve is a flap-like opening on the right side of the heart that allows blood to move to the lungs.

In pulmonary atresia, a solid sheet of tissue forms where the valve opening should be. Because of this defect, blood from the right side of the heart cannot go to the lungs to pick up oxygen.



Alternative Names:

Atresia - pulmonary; PA/IVS



Causes, incidence, and risk factors:

As with most congenital heart diseases, there is no known cause of pulmonary atresia. The condition is associated with another type of congenital heart defect called a patent ductus arteriosus (PDA).

Persons with pulmonary atresia may also have a poorly developed tricuspid valve. They may also have an underdeveloped right ventricle and abnormal blood vessels feeding the heart.

Pulmonary atresia may occur with or without a ventricular septal defect (VSD) . If the person does not have a VSD, the condition is called pulmonary atresia with intact ventricular septum (PA/IVS). If the person has both problems, the condition is called pulmonary atresia with VSD. This is an extreme form of tetralogy of Fallot .



Symptoms:

Symptoms usually occur in the first few hours of life, although it may take up to a few days.

Symptoms may include:

  • Bluish colored skin (cyanosis )
  • Shortness of breath
  • Fast breathing
  • Poor eating habits (babies may get tired while nursing or sweat during feedings)
  • Fatigue


Signs and tests:

The health care provider will use a stethoscope to listen to the heart and lungs. Persons with a PDA have a characteristic heart murmur that can be heard with a stethoscope.

The following tests may be ordered:



Treatment:

A medicine called prostaglandin E1 is usually used to help the blood move (circulate) into the lungs.

Other treatments include:

  • Interventional heart catheterization to repair the problem.
  • Open heart surgery to repair or replace the valve. This surgery can also be used to place a tube between the right ventricle and the pulmonary (lung) arteries.
  • For some patients, the surgeon may reconstruct the heart as a single ventricle.
  • Heart transplant


Support Groups:



Expectations (prognosis):

Most cases can be helped with surgery. However, how well a baby does depends on the quality of the blood vessels supplying the heart, how well the heart is beating, and the amount of leakiness of the other heart valves.

Some patients who have PA/IVS where the right ventricle is extremely small usually have several surgeris to help simulate normal circulation, but the surgeries do not exactly re-create normal circulation. A subgroup of these patients also have abnormal blood supply to the heart.



Complications:
  • Delayed growth and development
  • Seizures
  • Heart attack
  • Stroke
  • Infectious endocarditis
  • Heart failure
  • Death


Calling your health care provider:

Call your health care provider if the baby has:

  • Problems breathing
  • Skin or nails that appear blue (cyanosis)


Prevention:

There is no known prevention.

All pregnant women should receive good prenatal care. Many congenital defects can be discovered on routine ultrasound examinations. If found before birth, medical specialists (such as a pediatric cardiologist, a cardiothoracic surgeon, and a neonatologist) can be present, and ready to help as necessary. Such preparation can mean the difference between life and death for some babies.



References:

Zipes DP, Libby P, Bonow RO, Braunwald E, eds. Braunwald's Heart Disease: A Textbook of Cardiovascular Medicine, 8th ed. St. Louis, Mo; WB Saunders; 2007.




Review Date: 12/10/2007
Reviewed By: David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.; and Mark A Fogel, MD, FACC, FAAP, Associate Professor of Pediatrics and Radiology, Director of Cardiac MR, The Children's Hospital of Philadelphia.

The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed medical professional should be consulted for diagnosis and treatment of any and all medical conditions. Call 911 for all medical emergencies. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. © 1997- A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.
adam.com


Find What You Need

Events
Careers
Foundation
About Us
Contact
Directions
News
Social Media Agreement
Joint Notice
Web Privacy Policy
WDH Staff Portal

Centers & Services

Cancer Center
Cardiovascular Care
Joint Replacement
Women & Children's
Physician Offices
Other Services

Conditions & Treatments

Health Library

Support Services

Support Groups
Care-Van
Dental Center
Social Work
Food & Nutrition
Integrative Wellness
Spiritual Care
Concerns & Grievances
Homecare and Hospice

For Patients

Pay Your Bill Online
Pricing Estimates
Financial Assistance
Interpreter Services
Surgery Preparation
Medical Record Request
Advance Directives
Clinical Research & Trials

For Healthcare Professionals

Work and Life
Financial Well-Being
Career and Growth

The Wentworth-Douglass Health System includes:

 

Address

Wentworth-Douglass Hospital
789 Central Avenue, Dover, NH 03820
Phone: (603) 742-5252
Toll free: 1 (877) 201-7100