Pulmonary atresia
Pulmonary atresia | |
---|---|
Other names | Pulmonary atresia - intact ventricular septum[1] |
Diagram of pulmonary atresia | |
Specialty | Cardiology |
Symptoms | Cyanosis[1] |
Causes | No known cause for pulmonary atresia[1] |
Diagnostic method | Chest x-ray, Echocardiogram[1] |
Treatment | Prostaglandin E1, Heart catheterization[1] |
Pulmonary atresia is a
In
Signs and symptoms
The symptoms/signs of pulmonary atresia that will occur in babies are consistent with
In the case of pulmonary atresia with ventricular septal defect, decreased pulmonary blood flow may cause associated defects such as:[5]
Cause
In terms of the cause of pulmonary atresia, there is uncertainty as to what instigates this congenital heart defect.[2] Potential risk factors that can cause this congenital heart defect are those the pregnant mother may come in contact with, such as:[2][6]
Diagnosis
In regards to the diagnosis of pulmonary atresia the body requires oxygenated blood for survival. pulmonary atresia is not threatening to a developing fetus however, because the mother's placenta provides the needed oxygen since the baby's lungs are not yet functional. Once the baby is born its lungs must now provide the oxygen needed for survival, but with pulmonary atresia there is no opening on the pulmonary valve for blood to get to the lungs and become oxygenated. Due to this, the newborn baby is blue in color and pulmonary atresia can usually be diagnosed within hours or minutes after birth.[medical citation needed]
The diagnosis of pulmonary atresia can be done via the following exams/methods: an
2 in the body.[1]
Classification
There are two types of pulmonary atresia.
- Pulmonary atresia with intact ventricular septum (PA-IVS) is a rare congenital malformation. PA-IVS involves complete blockage of the pulmonary valve located on the right side of the heart. This blockage prevents the flow of blood to the lungs. Because of this lack of blood flowing through the right side of the heart, the structures on that side, such as the pulmonary valve and the tricuspid valve, are abnormally small.[2][7]
Treatment
The treatment of pulmonary atresia consists of: an IV medication called
The type of surgery recommended depends on the size of the
Prognosis
The prognosis for pulmonary atresia varies for every child, if the condition is left uncorrected it may be fatal, but the prognosis has greatly improved over the years for those with pulmonary atresia. Some factors that affect how well the child does include how well the heart is beating, and the condition of the blood vessels that supply the heart. Most cases of pulmonary atresia can be helped with surgery, if the patient's
See also
- Heart valves
- Percutaneous pulmonary valve implantation
References
- ^ a b c d e f g h MedlinePlus Encyclopedia: Pulmonary atresia
- ^ a b c d e "About Pulmonary Atresia". Congenital Heart Defects (CHDs). Center for Disease Control.
- ^ Pulmonic Valve Anatomy: Overview, Pathophysiologic Variants at eMedicine
- ^ "Congenital Heart Defects". Health Topics. MedlinePlus.
- ^ a b c Pulmonary Atresia With Ventricular Septal Defect: Background, Epidemiology, Etiology at eMedicine
- ^ "Research | Congenital Heart Defects | NCBDDD | CDC". www.cdc.gov. Retrieved 24 June 2016.
- ^ Pulmonary Atresia With Intact Ventricular Septum: Background, Pathophysiology, Etiology at eMedicine
- ^ PMID 21234191.
- ^ "Alprostadil (PGE1) for Maintaining Ductal Patency".
- PMID 21415190.
- ISBN 978-0-19-982948-4.
- PMID 21536996.
- ISBN 978-1-4511-4780-3.
Further reading
- Daubeney, Piers E. F; Delany, David J; Anderson, Robert H; Sandor, George G. S; Slavik, Zdenek; Keeton, Barry R; Webber, Steven A (2002-05-15). "Pulmonary atresia with intact ventricular septum: Range of morphology in a population-based study". Journal of the American College of Cardiology. 39 (10): 1670–9. PMID 12020496.
- Tzifa, Aphrodite; Barker, Claire; Tibby, Shane M; Simpson, John M (2007-05-01). "Prenatal diagnosis of pulmonary atresia: impact on clinical presentation and early outcome". Archives of Disease in Childhood: Fetal and Neonatal Edition. 92 (3): F199–F203. PMID 16840499.
- Awori, Mark Nelson; Mehta, Nikita P.; Mitema, Frederick O.; Kebba, Naomi (18 May 2017). "Optimal -Score Use in Surgical Decision-Making in Pulmonary Atresia With Intact Ventricular Septum". World Journal for Pediatric and Congenital Heart Surgery. 8 (3): 385–8. S2CID 26067553.