Pulmonary atresia
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| Pulmonary atresia | |
|---|---|
| Synonyms | |
| Pronounce | N/A |
| Specialty | N/A |
| Symptoms | Cyanosis, shortness of breath, fatigue, poor feeding |
| Complications | Heart failure, arrhythmia, growth retardation |
| Onset | Neonatal |
| Duration | Lifelong |
| Types | Pulmonary atresia with intact ventricular septum, Pulmonary atresia with ventricular septal defect |
| Causes | Congenital heart defect |
| Risks | Family history, genetic disorders |
| Diagnosis | Echocardiogram, cardiac catheterization, chest X-ray |
| Differential diagnosis | Tetralogy of Fallot, tricuspid atresia, transposition of the great arteries |
| Prevention | None |
| Treatment | Surgery, medication, cardiac catheterization |
| Medication | Prostaglandin E1 |
| Prognosis | Variable, depends on severity and treatment |
| Frequency | Rare |
| Deaths | |
Pulmonary atresia is a form of heart disease that occurs from birth (congenital heart disease). It is characterized by the absence or underdevelopment of the pulmonary valve, which regulates blood flow from the right ventricle (lower chamber) of the heart to the pulmonary artery. This condition can lead to a lack of blood flow to the lungs, preventing the normal oxygenation of blood.
Symptoms
The symptoms of pulmonary atresia may vary depending on the specific type of the condition. Common symptoms include cyanosis (a bluish coloration of the skin due to lack of oxygen), shortness of breath, fatigue, and rapid breathing. In severe cases, the condition can lead to heart failure.
Causes
The exact cause of pulmonary atresia is unknown. However, it is believed to occur during the first 8 weeks of fetal development. Some factors that may increase the risk of developing this condition include maternal use of certain medications during pregnancy, maternal alcohol use, and a family history of congenital heart disease.
Diagnosis
Pulmonary atresia is usually diagnosed shortly after birth through a physical examination and the use of imaging tests such as an echocardiogram, cardiac catheterization, and magnetic resonance imaging (MRI).
Treatment
The treatment for pulmonary atresia depends on the severity of the condition and the presence of other heart defects. Treatment options may include medications, catheter procedures, and surgery. In some cases, a heart transplant may be necessary.
Prognosis
The prognosis for individuals with pulmonary atresia varies. With early diagnosis and treatment, many individuals can lead a normal life. However, ongoing medical care is often necessary.
See also
References
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Contributors: Prab R. Tumpati, MD