Heterotaxy syndrome is a complex syndrome that occurs when the axes of the body fail to rotate correctly when developing in the womb. This can result in many different organ systems being affected, and also means that each individual with heterotaxy is unique.
The ductus arteriosus is small blood vessel connecting the pulmonary artery to the aorta. In utero, the ductus arteriosus allows for blood to bypass the lungs and flow directly out to the body. Normally this structure closes within the first 72 hours of life. When it fails to properly close, it is termed a patent ductus arteriosus (PDA). A PDA is considered a congenital heart defect, in other words, a birth defect of the heart. Congenital heart defects are the most common form of birth defects, occurring in approximately 1 in 150 individuals. A PDA is one of the more common congenital heart defects; the overall incidence is 2-3 per 1000. PDA’s are much more commonly found in premature infants as opposed to full term infants.
A patent ductus arteriosus (PDA) is an abnormal communication between the aorta and pulmonary artery that allows for oxygenated (red) blood to pass into the lungs. If the amount of blood flowing through the PDA is enough to cause problems, then intervention may be indicated. This most commonly occurs in the setting of a large or moderate size PDA. Indications for intervention in infancy include symptoms unresponsive to medication, elevated blood pressure in the lungs, and significant dilation of the heart due to excess blood flow. Usually the need for intervention in infancy becomes clear by 6-12 months of age, and often much earlier.
The pulmonary valve connects the right ventricle and the pulmonary artery. When the heart squeezes, the valve opens, allowing blood to pass from the right ventricle to the pulmonary artery. When the heart relaxes, the valve closes, preventing backflow of blood into the heart. Pulmonary valve stenosis refers to a condition in which the valve is abnormally narrowed. Pulmonary valve stenosis is a congenital heart defect, in other words, a birth defect of the heart. Congenital heart defects are the most common form of birth defects, occurring in approximately 1 in 150 individuals. Pulmonary valve stenosis is one of the more common congenital heart defects; the overall incidence is 0.5-1 per 1000. The cause of pulmonary valve stenosis is not entirely clear; rare cases can be associated with genetic defects or inherited syndromes.