Epidemiology
HLHS occurs in an estimated 1 out of 3,841 live births in the United States, or an estimated total of 1,025 live births per year in the US. Overall, it is estimated to make up 2-3% of all cases of congenital heart disease, and is the most common single-ventricle defect. It is thought to be more common in male infants, 1.5 times as common as in female infants. A recent systematic review found a slight decrease in prevalence of HLHS from 0.689/1000 in 1995 -1999, to 0.475/1000 in 2010 - 2017. This is thought to be due to improvements in prenatal detection and prenatal counseling and subsequent pregnancy. Signs and symptoms
Closing of the ductus arteriosus in a heart that is severely underdeveloped on the left results in cyanosis and respiratory distress which can progress to cardiogenic shock and death. Early symptoms might include poor feeding or cyanosis that does not respond to oxygen administration. Peripheral pulses may be weak and extremities cool to the touch.HLHS often co-occurs with low birth weight and premature birth.In neonates with a small atrial septal defect, termed "restrictive", there is inadequate mixing of oxygenated and deoxygenated blood. These neonates quickly decompensate and develop acidosis and cyanosis.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.
Haber syndrome is a cutaneous disorder of hyperpigmentation characterized by reticulated pigmentation of the persons skin. A rare genodermatosis, its key features include "rosacea-like facial eruption[,] reticulated hyperpigmentation of major flexures, comedones on the back and neck, and pitted facial scars." See also
List of cutaneous conditions

References


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