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Absent pulmonary valve syndrome (APVS) is a congenital heart defect that occurs when the flaps of the pulmonary valve do not develop or are severely underdeveloped resulting in aneurysms (dilation) of the pulmonary arteries and softening of the trachea and bronchi (tracheobronchomalacia).
When the right side of the heart is more underdeveloped than the left side, this is known as hypoplastic right heart syndrome. HRHS is known for the pulmonary valve, the tricuspid valve, right ventricle, and the pulmonary artery all failing to form properly. HRHS also causes the right ventricle to be a fair amount smaller than the left side.
On EKG, right axis deviation and right ventricular hypertrophy are common, but not indicative of HLHS. Chest x-ray may show a large heart (cardiomegaly) or increased pulmonary vasculature. Neonates with HLHS do not typically have a heart murmur, but in some cases, a pulmonary flow murmur or tricuspid regurgitation murmur may be audible. [5]
Without treatment, it is a highly life-threatening condition, so prognosis is poor. [35] [34] If surgery isn't performed in severe cases, the child can (and will) die, since the phenotype of pulmonary atresia is not compatible with life due to the pulmonary valve atresia resulting in reduced blood oxygenation. [9] [62] [63]
The Melody Transcatheter Pulmonary Valve (TPV), approved in Europe in 2006 and in the U.S. in 2010 under a Humanitarian Device Exemption (HDE), is designed to treat congenital heart disease patients with a dysfunctional conduit in their right ventricular outflow tract (RVOT).
The Fontan Kreutzer procedure is used in pediatric patients who possess only a single functional ventricle, either due to lack of a heart valve (e.g. tricuspid or mitral atresia), an abnormality of the pumping ability of the heart (e.g. hypoplastic left heart syndrome or hypoplastic right heart syndrome), or a complex congenital heart disease where a bi-ventricular repair is impossible or ...
The filmmaker, who was born without a womb, has now created a short film to raise awareness of the rare condition. "To be told I haven't got a womb and not be able to carry my own child, was ...
The most common cause of reoperation is a leaky pulmonary valve (pulmonary valve insufficiency). [61] This is usually corrected with a procedure called pulmonary valve replacement. [24]: 136 One common prognostic factor with TOF is the development of ischemia reperfusion injury. Insufficient myocardial protection is considered one of the main ...