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Hypoplastic left heart syndrome (HLHS) is a rare congenital heart defect in which the left side of the heart is severely underdeveloped and incapable of supporting the systemic circulation. [2] It is estimated to account for 2-3% of all congenital heart disease. [ 3 ]
If a baby is born with cyanotic heart disease, the diagnosis is usually made shortly after birth due to the blue colour of their skin (called cyanosis). [44] If a baby is born with a septal defect or an obstruction defect, often their symptoms are only noticeable after several months, or sometimes even after many years. [44]
Without surgical repair, infants born with a single ventricle cardiac defect face almost certain mortality in the first year of life. [8] [9] [10] In these conditions, the most urgent problem is that the heart is unable to pump blood to the systemic circulation (i.e. to the body). The goal of these three surgeries is to ultimately connect the ...
The timing of surgery depends on the baby's symptoms and size. [8] The procedure involves increasing the size of the pulmonary valve and pulmonary arteries and repairing the ventricular septal defect. [8] In babies who are too small, a temporary surgery may be done with plans for a second surgery when the baby is bigger. [8]
Out of all the babies born with congenital heart defects, about 25 % have cyanosis as a result. Tetralogy of Fallot is the most common cyanotic cardiac heart defect. [37] Methemoglobinemia is considered to be rare, with acquired methemoglobinemia encountered more than the congenital form. [38]
The most typical form of APVS is a tetralogy of Fallot variant, [4] however, case studies have linked APVS to several different congenital cardiac syndromes, such as agenesis of ductus arteriosus, [5] persistent ductus arteriosus, [6] atrioventricular septal defect, [7] pulmonary branching abnormalities, [8] [9] transposition of the great arteries, [10] and type B interrupted aortic arch. [11]
An infant will begin to show signs of congestive heart failure, which can include rapid breathing, feeding problems, slow weight gain, low energy, and cold, clammy sweating. [4] Symptoms often appear between 1-2 months of age but can occur earlier in some newborns.
The embryonic heart rate (EHR) then accelerates linearly for the first month of beating, peaking at 165-185 BPM during the early 7th week, (early 9th week after the LMP). This acceleration is approximately 3.3 BPM per day, or about 10 BPM every three days, an increase of 100 BPM in the first month.