Hypoplastic Left Heart Syndrome Complications

Hypoplastic left heart syndrome (HLHS) is a rare and potentially life threatening congenital heart defect (present at birth). Hypoplastic left heart syndrome (HLHS) happens when the left side of a baby’s heart is severely underdeveloped.

Upon birth, the ductus arteriosis (hole between left and right sides of the heart) lets blood continue to flow properly. Because of that hole, hypoplastic left heart syndrome (HLHS) is often not diagnosed immediately upon birth. However, the ductus arteriosis typically closes within the first few hours or days of life. Once the hole closes in infants with hypoplastic left heart syndrome (HLHS), the effects on the infant occur rapidly and shock may ensue.

Without surgery, hypoplastic left heart syndrome is typically fatal, usually within the first week or two of the infant’s life.

With treatment, many infants survive, however most with complications later in life. Some of the possible hypoplastic left heart syndrome (HLHS) complications may include:

• Tiring easily – many children who have hypoplastic left heart syndrome (HLHS) and have had repair surgeries may have difficulty with strenuous activity later in life. This may limit their participation in sports or other strenuous outdoor activities. Some patients with hypoplastic left heart syndrome (HLHS) may still be able to participate, although under close supervision.
• Heart arrhythmias – A heart arrhythmia may occur in a hypoplastic left heart syndrome (HLHS) patient when the heart’s natural pacemaker (the sinus node) develops an abnormal rate. Some arrhythmias are not dangerous, but others may be life-threatening. Some symptoms of a heart arrhythmia in a hypoplastic left heart syndrome (HLHS) patient may include: fatigue, palpitations, dizziness, or fainting.
• Edema – Edema is a swelling caused by fluid buildup trapped in the body’s tissues. Edema in hypoplastic left heart syndrome (HLHS) patients is typically noticed in the hands, feet, arms, legs, and ankles. Typical symptoms of edema include: stretched or shiny skin, swelling of the tissue under the skin, skin that retains a dimple after pressure for several seconds, and increased stomach size.
• Blood clots – Blood clots may appear under a myriad of different circumstances. Typically the clotting of blood near an injury is useful to stop the outflow of blood, but hypoplastic left heart syndrome (HLHS) may cause the body to form blood clots in critical locations that may lead to a pulmonary embolism or stroke.
• Developmental problems – many of the developmental problems caused by hypoplastic left heart syndrome (HLHS) are a result of lethargy or poor feeding during infancy. Infants with hypoplastic left heart syndrome (HLHS) typically have difficulty feeding which causes them to receive inadequate nutrition, and possibly leading to developmental complications later in life.
• Need for additional heart surgery or transplantation – most procedures done initially to treat patients with hypoplastic left heart syndrome (HLHS) are temporary in nature. They are meant to keep the infant alive and stable, but may require further surgery later in life. The Fontan procedure, for example gives the heart a 15-30 year life expectancy. Once the heart begins to deteriorate, the hypoplastic left heart syndrome (HLHS) patient will likely need to be placed on a heart transplant list.

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