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Pulmonary atresia is a form of congenital Heart disease in which the pulmonary valve does not form properly. The pulmonary valve is an opening on the right side of the heart that regulates blood flow from the right ventricle (right side pumping chamber) to the lungs.
In pulmonary atresia, a solid sheet of tissue forms where the valve opening should be, and the valve remains closed. Because of this defect, blood from the right side of the heart cannot go to the lungs to pick up oxygen.
Alternative NamesPulmonary atresia - intact ventricular septum; PA/IVS
Causes, incidence, and risk factorsAs 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. Although both conditions are called pulmonary atresia, they are actually different defects.
SymptomsSymptoms 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)
- Fast breathing
- Fatigue
- Poor eating habits (babies may get tired while nursing or sweat during feedings)
- Shortness of breath
The health care provider will use a stethoscope to listen to the heart and lungs. Persons with a PDA have a heart murmur that can be heard with a stethoscope.
The following tests may be ordered:
- Chest x-ray
- Echocardiogram
- Electrocardiogram (ECG)
- Heart catheterization
- Pulse oximetry - shows the amount of oxygen in the blood
A medicine called prostaglandin E1 is usually used to help the blood move (circulate) into the lungs. This medicine keeps a blood vessel open between the pulmonary artery and aorta. The vessel is called a patent ductus arteriosus (PDA).
Other treatments include:
- Heart catheterization to repair the problem
- Open heart surgery to repair or replace the valve, or to place a tube between the right ventricle and the pulmonary (lung) arteries
- Reconstructing the heart as a single ventricle (for some patients)
- Heart transplant
Most cases can be helped with surgery. However, how well a baby does depends on:
- Quality of the blood vessels supplying the heart
- How well the heart is beating
- Amount of leakiness of the other heart valves
Outcomes can vary because of the different forms of this defect. The baby could have only a single catheter-based procedure, or could need three or more surgeries and have only a single working ventricle. The outcome is difficult to predict without knowing all details about the patient's condition.
Complications- Delayed growth and development
- Seizures
- Stroke
- Infectious endocarditis
- Heart failure
- Death
Call your health care provider if the baby has:
- Problems breathing
- Skin or nails that appear blue (cyanosis)
There is no known prevention.
All pregnant women should receive routine prenatal care. Many congenital defects can be discovered on routine ultrasound examinations. If the defect is found before birth, medical specialists (such as a pediatric cardiologist, cardiothoracic surgeon, and neonatologist) can be present at the birth, and ready to help as needed. This preparation can mean the difference between life and death for some babies.
ReferencesZipes 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.
What else can I help you with?
What are the release dates for Birth Day - 2000 Pulmonary Atresia?
Birth Day - 2000 Pulmonary Atresia was released on: USA: 2 January 2004
What is pulmonary atresia?
Pulmonary atresia is a congenital heart defect where the pulmonary valve does not form properly, leading to a blockage of blood flow from the heart to the lungs. This condition can result in low oxygen levels in the blood and may require surgical intervention to improve blood flow to the lungs.
What are the four defects in tetralogy of fallot?
Pulmonary Atresia (or Pulmonary Stenosis) Right Ventricular Hypertrophy Ventricular Septal Defect Overriding Aorta
What is atresia?
* Anal atresia -- congenital absence of a hole at the bottom end of the intestine. Also called imperforate anus. * Aortic atresia -- congenital absence of the normal valvular opening into the aorta. * Biliary atresia -- absence of the major bile ducts. * Choanal atresia -- congenital failure of one or both nasal passages to open. * Esophageal atresia -- a birth defect in which part of esophagus is not hollow. * Intestinal atresia -- obliteration of the hollow of the small intestine, involving the ileum (50% of cases) or the jejunum or duodenum. * Laryngeal atresia -- congenital failure of the laryngeal opening to develop, resulting in partial or total obstruction at or just above or below the glottis. * Pulmonary atresia -- congenital absence of the pulmonary valve opening in the heart. * Tricuspid atresia -- congenital lack of the tricuspid valve opening. * Vaginal atresia -- congenital occlusion of the vagina or subsequence adhesion (sticking together) of the walls of the vagina occluding it.
Is atresia a cavity?
Atresia is a term related to cavities. Atresia is the condition in which a tube is blocked, or absent.
Does duodenal atresia always mean Down syndrome?
No, there is a 30-40% chance of Down Syndrome when duodenal atresia is diagnosed.
What are the effects of Lexapro on a fetus?
There are a long list of Zoloft side effects that have been discovered over the past few years. Most common are the one's associated with birth defects. These are cleft lip, pulmonary lung, neural-tube defects, club foot, ventricular septal defect, anal atresia, persistent pulmonary hypertension and infant omphalocele. http://www.zoloftsideeffectsandbirthdefects.info
What are sign of Ovarian follicle atresia?
An ovarian follicle atresia is a process in which immature ovarian follicles physically decline. Roughly 20 ovarian follicles mature every month but only one of them is ovulated and the rest go through the process of atresia. As such, there is no specific sign of ovarian follicle atresia as it is a natural process in the female reproductive cycle.
Congenital lack of continuity of the esophagus?
atresia
What is lack of a normal body opening?
Atresia
What are the different nursing intervention of choanal atresia?
there's nothing to do about it
The congenital absence of a normal body opening is called?
atresia
