HLHS
Hypoplastic Left Heart Syndrome (HLHS) is a catastrophic birth-defect of the heart. It means the entire left-side of the heart was under developed while still in the womb. Because of the specific characteristics of the prenatal heart, babies with HLHS do not experience any problems while still in the womb (typically). When a baby is born, the heart actually changes within the first few days and weeks after birth. When these change occurs, babies with HLHS experience significant problems. If not treated, HLHS babies will die within a few weeks (100% mortality at 6 weeks). The only course of action before about 25 years ago was to make the baby comfortable until their eventual death, thankfully times have changed.
Normally, diagnosis of any cardiac defect is done prenatally somewhere starting in the 4-5 month period (second trimester). Although, not all medical staff will catch this defect during normal visits for pregnancy checkups. We strongly recommend keeping on schedule with office visits and insisting on sonogram verified good 4-chamber heart of the baby. Before high resolution sonogram technology (or if you doctor misses this defect), parents would often be able to take their baby home only to discover there is something wrong within the first few days at home. Parents are often in shock when they find out their perfect bundle of joy has a serious heart condition that requires significant intervention.
The primary plan of action is a series of major open-heart and around the heart surgeries that will eventually reroute circulation so only the right side of the heart does all the work that is normally done by both sides. The first surgery is called the Norwood, the second one is the Glen, and the last one is the Fontan. The only other possible option is a heart transplant — which may still be evenaully needed, depending on the specific case of the child.
One of the most common questions regarding HLHS and the three-stange repair is about long-term prognosis. First of all, this area of medicine is advancing rapidly, so today’s estimations may be way off tomorrow. Likewise, the estimates from just a few years ago is vastly different than what might be expected today. Because of the shortage of stable data, there is no clear prognosis. We like to take things day by day and appreciate the precious gift of life while we have it.
Of course, HLHS is a very serious diagnosis but is not the end of the world. In most cases, is no longer the death sentence it once was. Even better, these days, HLHS patients often can live almost normal childhoods due to the great advances in medical science and great treatment options. Many of the first HLHS patients are starting to enter adulthood (early 20s). Some of the earliest patients have had heart transplants eventually at various ages, but we are hopeful today’s patients can make it into their early adulthood and beyond before this becomes a consideration.
We are excited about the wonderful advancements in the treatment and management of HLHS.