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Hypoplastic left heart syndrome(HLHS): Causes, Types, Symptoms, Diagnosis ,Treatment , Risk factors , Complications , Prevention

 What Is Hypoplastic Left Heart Syndrome(HLHS)?

Hypoplastic left coronary heart syndrome (HLHS) is a coronary heart situation that develops earlier than birth (congenital). The left facet of a baby’s heart doesn’t shape right. This keeps blood from flowing through their coronary heart in a normal manner. Medicine can assist quickly after delivery, but infants with HLHS want a sequence of three surgeries at a very young age.

Hypoplastic left coronary heart syndrome (HLHS) is a problem that takes place while the left aspect of a toddler’s coronary heart does not shape because it ought to. It’s smaller than regular and might not pump enough blood to the frame. After the infant is born, medical doctors can deal with the problem with medicines and several surgeries. Some babies will need a heart transplant.

Hypoplastic left heart syndrome (HLHS) is a start defect that affects ordinary blood go with the flow through the coronary heart. As the baby develops in the course of being pregnant, the left side of the heart does no longer shape effectively. Hypoplastic left coronary heart syndrome is one kind of congenital heart defect. Congenital method gift at start. Because a baby with this defect needs surgical operation or different procedures quickly after beginning, HLHS is considered an important congenital heart disorder (CCHD).


What Is Hypoplastic Left Heart Syndrome(HLHS)


Explanation of medical terms and concept Hypoplastic left heart syndrome(HLHS)

Hypoplastic left coronary heart syndrome is an extraordinary heart disorder present at delivery (congenital). In this condition, the left aspect of the coronary heart is extremely underdeveloped.

If a baby is born with hypoplastic left heart syndrome, the left facet of the coronary heart can not pump blood well. Instead, the right facet of the coronary heart needs to pump blood to the lungs and to the relaxation of the body.

Treatment of hypoplastic left coronary heart syndrome calls for medicinal drugs to prevent closure of the relationship (ductus arteriosus) among the proper and left sides of the coronary heart. Plus, either surgery or a heart transplant is necessary. Advances in care have progressed the outlook for toddlers born with this circumstance.

A congenital heart defect that is the most severe form of left heart disease hypoplastic left heart syndrome occurs when the left ventricle is underdeveloped The mitral and aortic valves are also affected as well as the septum between the two upper chambers of the heart In some cases the aorta may be narrowed Because it is so severe and because of serious complications like congestive heart failure and high blood pressure more than half of babies born with this condition do not survive Those who do survive must have multiple surgeries in order to improve their quality of life Newborns born with HLHS can be treated with a combination of medications and surgery to help them develop normally.

Hypoplastic left heart syndrome affects a number of systems on the left aspect of the coronary heart that do not completely develop, for example:

  • The left ventricle is underdeveloped and too small.

  • The mitral valve is not formed or is very small.

  • The aortic valve is not formed or is very small.

  • The ascending portion of the aorta is underdeveloped or is too small.

  • Often, babies with hypoplastic left heart syndrome also have an which is a hole between the left and right upper chambers (atria) of the heart.

In a baby without a congenital heart disorder, the proper aspect of the coronary heart pumps oxygen-negative blood from the heart to the lungs. The left facet of the heart pumps oxygen-rich blood to the rest of the frame. When a child is developing in a mother’s womb all through being pregnant, there are two small openings among the left and right facets of the coronary heart: the patent ductus arteriosus and the patent foramen ovale. Normally, those openings will close a few days after birth.

In babies with hypoplastic left heart syndrome, the left facet of the coronary heart can't pump oxygen-rich blood to the body well. During the first few days of lifestyles for a toddler with hypoplastic left heart syndrome, the oxygen-rich blood bypasses the poorly functioning left aspect of the coronary heart through the patent ductus arteriosus and the patent foramen ovale. The proper facet of the coronary heart then pumps blood to each the lungs and the relaxation of the frame. However, among infants with hypoplastic left coronary heart syndrome, when those openings close, it will become difficult for oxygen-rich blood to get to the relaxation of the body.

Symptoms Hypoplastic left heart syndrome(HLHS)

The following signs of hypoplastic left heart syndrome can be given at delivery or several.Babies born with hypoplastic left coronary heart syndrome typically are critically sick quickly after start. Symptoms encompass:

  • Grayish-blue color of the lips and gums (cyanosis)

  • Rapid, difficult breathing

  • Poor feeding

  • Cold hands and feet

  • Weak pulse

  • Being unusually drowsy or inactive

If the natural connections among the coronary heart's left and proper sides (foramen ovale and ductus arteriosus) are allowed to shut within the first few days of existence in infants with hypoplastic left heart syndrome, they can pass into surprise and likely die.

Signs of shock include:

  • Cool, clammy skin that can be pale or lips that can be bluish-gray

  • A weak and rapid pulse

  • Breathing that may be slow and shallow or very rapid

  • Dull eyes that seem to stare

A toddler in shock is probably aware or subconscious.

When to see a doctor

Most toddlers with hypoplastic left heart syndrome are diagnosed both before birth or quickly after. However, are seeking medical help in case you word that your toddler has any of the symptoms of the condition.

If you believe you studied that your infant is in surprise, straight away call 911 or your local emergency

Causes Hypoplastic left heart syndrome(HLHS)

Hypoplastic left coronary heart syndrome happens within the womb while a child's coronary heart is growing. The cause is unknown. However, having one toddler with hypoplastic left coronary heart syndrome, will increase the chance of getting any other with a similar situation.

The heart has 4 chambers, two on the right and  at the left. In doing its fundamental activity — pumping blood through the frame — the coronary heart makes use of its left and proper sides for one of a kind duties.

The proper facet moves blood to the lungs. In the lungs, oxygen enriches the blood, which then moves to the coronary heart's left facet. The left side of the heart pumps blood right into a large vessel referred to as the aorta, which sends the oxygen-wealthy blood to the relaxation of the frame.

Hypoplastic left coronary heart is a rare form of congenital coronary heart disease. It is more not unusual in men than in women.

As with maximum congenital heart defects, there may be no acknowledged reason. About 10% of infants with hypoplastic left heart syndrome also produce other delivery defects. It is likewise related to some genetic illnesses inclusive of Turner syndrome, Jacobsen syndrome, trisomy 13 and 18.

The trouble develops earlier than beginning when the left ventricle and other structures do not develop properly, which include the:

  • Aorta (the blood vessel that carries oxygen-rich blood from the left ventricle to the entire body)

  • Entrance and exit of the ventricle

  • Mitral and aortic valves

What happens in hypoplastic left heart syndrome

In hypoplastic left coronary heart syndrome, the left side of the coronary heart cannot properly supply blood to the frame. That's because the decreased left chamber (left ventricle) is simply too small or is not there. Also, the valves on the left facet of the heart (aortic valve and mitral valve) do not paint well, and the main artery leaving the heart (aorta) is smaller than usual.

After beginning, the proper aspect of a baby's coronary heart can pump blood both to the lungs and to the rest of the frame thru a blood vessel that connects the pulmonary artery at once to the aorta (ductus arteriosus). The oxygen-rich blood returns to the right side of the heart through a herbal establishment (foramen ovale) between the right chambers of the coronary heart.

When the ductus arteriosus and the foramen ovale close — which they commonly do after the first day or  of lifestyles — the right side of the heart has no way to pump blood to the frame. Babies with hypoplastic left heart syndrome want medicinal drugs to maintain these connections open and hold blood flowing to the frame until they have coronary heart surgery.

Risk factors Hypoplastic left heart syndrome(HLHS)

The causes of heart defects which include hypoplastic left heart syndrome amongst maximum toddlers are unknown. Some babies have heart defects because of changes in their genes or chromosomes. These varieties of coronary heart defects also are thought to be as a result of an aggregate of genes and other chance factors, such as matters the mother comes in touch with inside the environment or what the mom eats or drinks or the drugs the mom makes use of.

People who've a baby with hypoplastic left heart syndrome have a higher risk of having every other toddler with this or a comparable circumstance.

There are no other clean threat factors for hypoplastic left heart syndrome.

Complications

With proper remedy, many babies with hypoplastic left coronary heart syndrome live on. But maximum have complications later, which may additionally include:

  • Tiring easily during sports or other exercise

  • Heart rhythm problems (arrhythmias)

  • Fluid buildup in the lungs, abdomen, legs and feet (edema)

  • Not growing well

  • Developing blood clots that may lead to a pulmonary embolism or stroke

  • Developmental problems related to the brain and nervous system

  • Need for additional heart surgery or a heart transplant

Prevention

There's no way to prevent hypoplastic left heart syndrome. Before getting pregnant, human beings with a family history of heart defects or who've a toddler with a congenital heart illness would possibly need to meet with a genetic counselor and a heart specialist who treats congenital heart defects.

Diagnosis Hypoplastic left heart syndrome(HLHS)

This heart defect is identified on fetal fetal echocardiograms. It is one of the most commonplace cardiac defects discovered on screening ultrasounds. Early diagnosis of the defect lets in for set off intervention on the time of delivery.


Planning to supply an infant at a health facility able to aggressive newborn resuscitation is crucial in improving the probabilities for an amazing outcome.


Echocardiograms can give designated statistics of the anatomy of the diverse cardiac systems affected in hypoplastic left heart syndrome. They additionally give vital facts approximately the features of the proper ventricle, the heart valves, the size of the atrial septal defect and the size of the patent ductus arteriosus.


Cardiac catheterization is not often used as part of the preliminary assessment. This is due to the high dangers in an unstable new child. Catheterization does assist in comparing cardiopulmonary features and anatomy in older youngsters with hypoplastic left coronary heart syndrome. It can assist with making plans for later tiers inside the remedy.

Often, hypoplastic left coronary heart syndrome is recognized before birth, with fetal echocardiogram (ultrasound). Our Fetal Heart Program allows you to prepare a plan for shipping and care immediately after beginning.


Sometimes HLHS is identified hours or days after delivery and the toddler will need instantaneous remedy. Diagnosis of HLHS may additionally require some or all of those checks:

 tests:

  • Echocardiogram (also called "echo" or ultrasound): sound waves create an image of the heart

  • Electrocardiogram (ECG): a record of the electrical activity of the heart

  • Chest X-ray

  • Pulse oximetry: a noninvasive way to monitor the oxygen content of the blood

  • Cardiac catheterization: a thin tube is inserted into the heart through a vein and/or artery in either the leg or through the umbilicus ("belly button")

  • Cardiac MRI: a three-dimensional image shows the heart's abnormalities

Before birth

It's possible for a child to be identified with hypoplastic left coronary heart syndrome inside the womb. A routine ultrasound examination at some stage in the second trimester of being pregnant might show the condition.

After birth

A toddler with grayish-blue lips or trouble respiration at delivery may have a coronary heart illness, including hypoplastic left coronary heart syndrome. Other possible symptoms of a coronary heart problem are a coronary heart murmur, that's a sound because of speeding blood glide that can be heard through a stethoscope.

An echocardiogram can diagnose hypoplastic left coronary heart syndrome. This test uses sound waves that bounce off the infant's coronary heart to provide shifting pictures that show on a video display screen.

For toddlers with hypoplastic left coronary heart syndrome, the echocardiogram shows a small left ventricle and aorta. The echocardiogram may additionally display problems with heart valves.

Because this test can sing blood flow, it suggests blood shifting from the right ventricle into the aorta. And an echocardiogram can pick out other coronary heart situations, inclusive of an atrial septal defect.

Treatment Hypoplastic left heart syndrome(HLHS)

Hypoplastic left heart syndrome is most customarily fatal without early intervention. Compared to 25 years in the past, there are many distinctive options for treatment of this complex heart situation; an individualized approach is taken for each and every child. Your physician will explain every character option, and why one precise method might be endorsed for your toddler.

Hypoplastic left coronary heart syndrome is dealt with through several surgical processes or a heart transplant. Your child's health care issuer will talk about remedy options with you.

If the diagnosis is made before birth, care vendors usually propose delivery at a health facility with a cardiac surgery center.

Options to assist control a toddler's condition earlier than surgical operation or transplant encompass:

  • Medication. The medication alprostadil (Prostin VR Pediatric) enables widen the blood vessels and continues the ductus arteriosus open.

  • Breathing assistance. Babies who've respiratory trouble would possibly need help from a respiratory machine (ventilator).

  • Intravenous fluids. A baby might receive fluids through a tube inserted into a vein.

  • Feeding tube. Babies who have hassle feeding or who tire whilst feeding may be fed through a feeding tube.

  • Atrial septostomy. This system creates or enlarges the outlet between the heart's top chambers to allow extra blood to float from the proper atrium to the left atrium. This is achieved if the foramen ovale closes or is just too small. Babies who have already got a gap (atrial septal illness) may not want this technique.

Surgeries and other procedures

Children with hypoplastic left coronary heart syndrome will possibly need several surgical procedures. Surgeons carry out these methods to create separate pathways to get oxygen-rich blood to the frame and oxygen-terrible blood to the lungs. The procedures are accomplished in 3 degrees.

  • Norwood procedure. This surgery is commonly executed within the first two weeks of existence. There are several ways to do this process.
    Surgeons reconstruct the aorta and join it to the coronary heart's decreased right chamber. Surgeons insert a tube (shunt) that connects the aorta to the arteries leading to the lungs (pulmonary arteries), or they place a shunt that connects the proper ventricle to the pulmonary arteries. This allows the right ventricle to pump blood to each the lungs and the body.
    In a few cases, a mixed method is done. Surgeons implant a stent in the ductus arteriosus to preserve the hole among the pulmonary artery and the aorta. Bands located around the pulmonary arteries lessen blood waft to the lungs and create an opening between the atria of the coronary heart.
    After the Norwood procedure, a toddler's pores and skin will nevertheless be discolored due to the fact oxygen-rich and oxygen-poor blood preserve to combine in the heart. Once a toddler successfully passes via this stage of treatment, the odds of survival can boom.

  • Bidirectional Glenn procedure. This process is usually the second one. It's executed whilst a baby is between 3 and six months of age. It entails putting off the primary shunt and connecting one of the massive veins that returns blood to the coronary heart (the superior vena cava) to the pulmonary artery. If surgeons formerly performed a hybrid process, they may observe extra steps at some stage in this manner.
    This technique reduces the work of the right ventricle by permitting it to pump blood especially to the aorta. It additionally lets in most of the oxygen-bad blood returning from the body to drift directly into the lungs without a pump.
    After this process, all the blood coming back from the higher frame is sent to the lungs, so blood with more oxygen is pumped to the aorta to supply organs and tissues in the course of the body.

  • Fontan procedure. This surgery is generally carried out whilst an infant is between 18 months and 4 years of age. The general practitioner creates a path for the oxygen-bad blood in one of the blood vessels that returns blood to the coronary heart (the inferior vena cava) to flow without delay into the pulmonary arteries. The pulmonary arteries then send the blood into the lungs.
    The Fontan technique allows the rest of the oxygen-negative blood returning from the body to drift to the lungs. After this procedure, there's little mixing of oxygen-wealthy and oxygen-terrible blood within the coronary heart. So the skin will no longer look discolored.

  • Heart transplant. Another surgical option is a heart transplant. However, the wide variety of hearts for transplant is restrained, so this feature isn't always used as often. Children who have heart transplants want medicines during life to prevent rejection of the donor coronary heart.

Follow-up care

After surgery or a transplant, a baby desires lifelong care with a cardiologist trained in congenital heart illnesses. Some medicinal drugs are probably wished for coronary heart characteristics. Various complications can arise over time and may require similar remedy or other medications.

The child's cardiologist will determine whether the child wishes to take preventive antibiotics earlier than sure dental or different approaches to prevent infections. Some children may need to limit physical interest.

Follow-up care for adults

Adult care requires a heart specialist skilled in congenital coronary heart disorder in adults. Recent advances in surgical care have allowed children with hypoplastic left coronary heart syndrome to grow into adulthood. So it is not but clear what demanding situations an adult with the situation may face. Adults want normal, lifelong follow-up care to watch for modifications inside the circumstance.

People thinking about being pregnant must discuss pregnancy risks and delivery manipulation options with their health care carriers. Having this situation will increase the threat of cardiovascular troubles in the course of pregnancy, the chance of miscarriage and the hazard of having a infant with congenital coronary heart disease.

Coping and support

It can be tough to stay with hypoplastic left heart syndrome or to care for a child with the situation. Here are some strategies that would help:

  • Seek support. Ask family participants and pals for assistance. Caregivers want breaks. Talk along with your baby's cardiologist about aid businesses and other types of assistance.
    If you're a teen or an adult with hypoplastic left heart syndrome, ask your health care issuer if there are help companies for adults with congenital coronary heart situations. It can be beneficial to speak to other people who share your challenges.

  • Keep health records. Write down your or your infant's diagnosis, medicines, surgery and different tactics and the dates they were performed. Include the name and get in touch with a variety of the heart specialist, emergency touch numbers for fitness care companies and hospitals, and other essential facts. Include a copy of the file from the medical professional inside the data.
    This information will help you hold track of the care received. It might be beneficial for care providers who don't know the complex health history. This statistics is likewise useful as your infant actions from pediatric care to grownup cardiology care.

  • Talk about your concerns. Talk with your child's heart specialist about which activities are great for your toddler. If a few are off-limits, inspire your infant in different pastimes in preference to those that specialize in what can't be completed.
    If different issues about your child's fitness concern you, speak them together with your toddler's cardiologist too. If you are a grownup with hypoplastic left coronary heart syndrome, ask your care provider about activities you may do. Talk about your issues.

General summary

Can you live a normal life with HLHS?

Although heart defects are often detected in the womb or shortly after birth, HLHS can be a shock to parents. But with the help of a pediatric cardiologist and a multidisciplinary team at an experienced center many will do well after surgery.

Can HLHS be cured?

Yes HLHS can be treated In fact the survival rate for babies with HLHS has increased from about 50 percent in the 1990s to more than 90 percent today Children who have had open heart surgery are monitored daily and have regular follow-up care which may include frequent visits to a pediatric cardiologist for the first year or two.

Can a baby survive with only 3 heart chambers?

It is possible for a baby to survive with only three heart chambers The condition known as hypoplastic left heart syndrome (HLHS) is the most common congenital heart defect occurring in about one out of every 5,000 births In this condition the left side of the heart is severely underdeveloped and unable to pump blood to the body Without surgery to correct the problem babies born with HLHS usually do not live past their first birthday.

Is hypoplastic left heart genetic?

No Hypoplastic left heart syndrome is not a genetic disorder. It is associated with other congenital heart defects so this condition can be inherited from parents through faulty genes.

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Hypoplastic left heart syndrome(HLHS): Causes, Types, Symptoms, Diagnosis ,Treatment , Risk factors  , Complications , Prevention

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