Diagnosis
There are various ways of diagnosing this congenital heart defect both prenatally and postnatally, these methods include:
Ultrasound
Pulse oximetry
Chest X-ray
Echocardiogram
Electrocardiogram
Cardiac catheterization
Cardiac CT scan
Genetic testing (particularly if other systemic birth anomalies are seen alongside the pulmonary atresia and ventricular septal defect). Management
When the disorder is detected (usually before or soon after birth), prostaglandin will be temporarily used as soon as possible in order to keep the ductus arteriosus open for as long as possible until surgery can be done, this is done so that blood can keep flowing to the lungs, since the bodies of babies with pulmonary atresia usually use the ductus arteriosus for lung blood flow pre-natally until birth, after which it closes.Afterwards, this anomaly is usually managed with surgeries for improvement of blood flow and function of the heart, although what kind of treatment one gets depends on the structure of the cardiorespiratory system.The surgical methods that can be used to treat (for the long-term) this condition include:
Catheter procedure for pulmonary artery branches
Systemic-to-pulmonary artery shunt
Neonatal complete repair
One-stage complete repair
Balloon septostomy

Staged unifocalization

Frequency
Frequency estimates vary between populations, estimates range from 0.01% to 0.2% of live births with PAVSD. It is believed to make up for 1-2% of cases of congenital heart defects worldwide.Of all patients with PAVSD, around 25-32% of them have a microdeletion of the 22q11.2 chromosome. Prognosis
Without treatment, it is a highly life-threatening condition, so prognosis is poor.
Radiological studies showed a ventricular septal defect alongside cardiac and arterial anomalies (heart silhouette enlargement, elevation of the cardiac apex, presence of a right aortic arch, enlargement affecting the main pulmonary arteries and their major branches, high pulmonary artery vascularity, and ventricular septal defect). See also
Tricuspid atresia
Mitral regurgitation


== References ==