HYPOPLASTIC LEFT HEART SYNDROME

Hypoplastic left heart syndrome is a serious and uncommon congenital heart condition. This illness causes the left side of the heart to grow abnormally. The left portion of your baby’s heart can’t adequately pump blood throughout the body if they are born with hypoplastic left heart syndrome. The heart’s right side is in charge of producing blood to the lungs and throughout the body.

Hypoplastic left heart syndrome requires medication to prevent the closure of the link between the left and right sides, accompanied by a heart transplant or surgery. The outlook for newborns born with this syndrome has improved thanks to advances in care.

SYMPTOMS

Hypoplastic left heart syndrome causes babies to become very unwell soon after birth. Some of the signs are as follows:

  • Poor feeding
  • Difficult or fast breathing
  • Cyanosis
  • Weak pulse
  • Cold feet and hands
  • Being inactive or drowsy

An infant with hypoplastic left heart syndrome can go into stress and die if the natural interconnections between the right and left sides of the heart are forced to collapse in the first few days after birth.

Symptoms of shock include:

  • Rapid, weak pulse
  • Clammy, cool skin
  • Lackluster eyes
  • Unusual breathing

A newborn in shock may or may not be cognizant. If you think your kid is in shock, dial 911 immediately.

DIAGNOSIS

A physical examination and a family history may be used to identify hemorrhagic telangiectasia, hereditary (HHT). Some symptoms, however, may not be visible in children or young adults. Your doctor may also recommend that you have HHT genetic testing, which can verify a probable diagnosis.

  • Magnetic resonance imaging (MRI)
  • Ultrasound imaging
  • CT Scan
  • Bubble study

TREATMENT

If you or your child has hereditary hemorrhagic telangiectasia (HHT), seek therapy in a medical center that has managed the condition before. However, finding a specialist can be challenging due to the disorder’s rarity.

Hormone-related medicines may be one of the treatment procedures for HHT. These medications slow the breakdown of clots and drugs that stop blood vessels from growing. 

Among the other procedures are:

  • Surgical removal. Surgical removal of AVMs in the lungs, brain, or liver may be the best option for some people. The AVM’s location, especially in the brain, can enhance surgical risks.
  • Embolization. A long, narrow tube is threaded into your blood vessels to the problematic area, where a metal coil is inserted to prevent blood from entering the AVM, which diminishes and cures over time.
  • Liver transplant. A liver transplant is an unusual treatment option for AVMs in the liver.
  • Stereotactic radiotherapy. AVMs in the brain are treated with this treatment. To kill the AVM, it uses radiation beams from various directions that converge at the AVM.

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