Pulmonary valve lets the blood flow in pulmonary artery between the right ventricle and lungs for oxygenation. Blockage of blood flow between the right ventricle and pulmonary artery due to narrowing or absence of pulmonary valve causes pulmonary atresia (AHA, 2009). The flow of oxygen-rich blood (red in color) into the lung is obstructed and low-oxygen blood (blue-in-color) circulates in the body which causes blue appearance of the child (AHA, 2009; BHF, 2010).
Wide range of the levels of defect can be found. Rodriguez-Cruz (2013) suggested considering the pulmonary atresia with ventricular septal defect as most severe. According to Rodriguez-Cruz (2013), the prevalence of pulmonary atresia is higher in males and children of patients with the defect. Ultrasound scan of the heart or echo cardiogram, chest radiography and MRI are diagnostic tools for pulmonary atresia (BHF, 2010).
Treatment of pulmonary atresia involves maintaining the normal blood flow and correcting the septal defects. Palliative treatment or the improvement of the heart condition is one of the treatment methods of pulmonary atresia but complete repair may also be possible with several operations (CHFED, n.d.; Royal Children’s Hospital, 2012). Percutaneous perforation of pulmonary valve and dilation of the valve is carried out in palliative surgery (Rodriguez-Cruz, 2013).
Valvotomy, Shunt operation, heart catheterization and heart-lung transplantation are treatment options for pulmonary atresia (Rodriguez-Cruz, 2013). According to BHF (2010), valvotomy or the process of opening the blocked valve can be catheter valvotomy or surgical valvotomy. Shunt or tube is the synthetic material placed between the pulmonary artery and the aorta (Cave Point Foundation, 2011).
American Heart Association (AHA) (2009) Pulmonary Atresia/ Intact Ventricular Septum. [Online] http://www.heart.org/idc/ [Accessed on 5 June 2013].
British Heart Foundation (BHF) (2006) Understanding Your Child’s Heart Pulmonary Atresia with Intact Ventricular Septum. [Online] Available at http://www.bhf.org.uk [Accessed on 6 June].
Cave Point Foundation (2011) Congenital Heart Disease. [Online] Available at http://www.pted.org/ [Accessed on 17 June 2013].
Children’s Heart Federation (CHFED, n.d.) Pulmonary Atresia with Intact Ventricular Septum. [Online] Available at http://www.youngatheart.org.uk/ [Accessed on 6 June 2013].
Rodriguez-Cruz, E. (2013) Pulmonary Atresia with Ventricular Septal Defect Treatment and Management. [Online] Available at http://emedicine.medscape.com/%5BAccessed on 17 June 2013].
Royal Children’s Hospital Melbourne (2012) Pulmonary Atresia with Intact Ventricular Septum. [Online] Available at http://www.rch.org.au/ [Accessed on 6 June 2013].