All You Need to Know About Single Ventricle Heart Defects

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“Single ventricle heart defects”

You all know that heart has four chambers. The first two upper chambers of heart are collectively called as atria, and atria collect the blood flowing into the heart. The last two chambers of heart called as ventricle helps to pump out the blood from heart. Single ventricle defect is called for a child born with heart that has problems for one lower chamber of heart. This lower chamber, ventricle may be small, underdeveloped or missing a valve.

Signs and symptoms for single ventricle heart defects

  1. Difficulty in breathing and feeding
  2. Skin, lips and nails will have blue or purple tint.
  3. Lethargy

Treatments for Single ventricle defects

Single ventricle defects need a series of open heart procedures called staged reconstruction. In some cases cardiac catheterization is also performed.

Single ventricle heart defects include:-----

  • Hypoplastic left heart syndrome (HLHS)

This is a severe heart defect (congenital heart defect) in which left side of heart is not fully developed. Child with hypoplastic left heart syndrome will have a small left ventricle and also have a closed or too small mitral and aortic valve. 

Signs and symptoms for hypoplastic left heart syndrome

  1. Difficulty in breathing and feeding
  2. Skin, lips and nails will have blue or purple tint.
  3. Lethargy

Diagnosis for hypoplastic left heart syndrome

  1. Cardiac MRI
  2. Cardiac catheterization
  3. Chest X-ray
  4. Electrocardiogram
  5. Pulse oximetry (to detect the content of oxygen in blood)
  6. Echocardiogram

Treatment for hypoplastic left heart syndrome

Open heart surgery is done to correct hypoplastic left heart syndrome. In some cases additionally catheter therapies are done. And in rare cases heart transplantation is needed for hypoplastic left heart syndrome.

  • Tricuspid atresia

Tricuspid valve opens to right atrium and right ventricle. A child with tricuspid atresia will have only one ventricle (left ventricle) functioning and have poorly developed right heart structures. Tricuspid atresia have the following characteristic

  1. No tricuspid valve  
  2. Have smaller right ventricle
  3. ASD (atrial septal defect)
  4. VSD (ventricular septal defect)
  5. Tricuspid atresia is often associated with “pulmonary stenosis” and “transposition of great arteries”

Signs and symptoms for tricuspid atresia

  1. Fatigue
  2. Poor weight gain
  3. Shortness of breath
  4. Heart murmur
  5. Difficulty feeding
  6. Cyanosis (Skin, lips and nails will have blue or purple tint)
  7. In older children, there will be abnormal shape for finger tips.

Diagnosis for tricuspid atresia

  1. Cardiac catheterization
  2. Electrocardiogram
  3. Blood test
  4. Echocardiogram
  5. Chest X-ray

Treatment for tricuspid atresia

Baby with tricuspid atresia require prostaglandin medication and three surgeries namely “Blalock-Taussig shunt”, “Hemi-Fontan/Glenn”,and “Fontan”

  • Double outlet left ventricle (DOLV)

Double outlet left ventricle is a rare birth defect (congenital heart defect) appears in children and it occurs when the aorta and pulmonary artery both exit from the left ventricle. The only pathway for blood to flow into the lungs is a hole that connects the two ventricles (ventricular septal defect). 

Symptoms for Double outlet left ventricle

  1. Skin, lips and nails will have blue or purple tint
  2. Poor weight gain
  3. Shortness of breath
  4. Unresponsiveness
  5. Sweating during feeding
  6. Irregular fatigue
  7.  In some cases your child will have pulmonary outflow tract obstruction, aortic outflow tract obstruction, and other cardiac abnormalities

Diagnosis for Double outlet left ventricle

  1. Magnetic resonance imaging (MRI)
  2. Cardiac catheterization
  3. Chest X-ray
  4. Electrocardiogram
  5. Echocardiography

Treatment for Double outlet left ventricle

Surgery is performed for Double outlet left ventricle, and during this procedure VSD is corrected. In some cases cardiac surgeons will perform a bidirectional Glenn procedure followed by a Fontan operation

  • Heterotaxy syndrome

It is a rare birth defect that involves heart and other organs.  There are two types of heterotaxy syndrome and they are

Right atrial isomerism

Children with right atrial isomerism have multiple heart defects including

  1. Septal defects
  2. Problems with heart valves (particularly pulmonary heart valve)
  3. Anomalous pulmonary venous connection (abnormalities of blood coming back from lungs to heart)
  4. Absence of spleen
  5. Liver and other organs on wrong side of the body

Left atrial isomerism

Children with left atrial isomerism have

  1. Septal defects
  2. Problems with heart valves
  3. Problems with heart’s electrical system
  4. Complete heart block
  5. There will be no spleen or may have polysplenia (several small spleens)

Signs and symptoms for heterotaxy syndrome

  1. Difficulty in breathing and feeding
  2. Skin, lips and nails will have blue or purple tint.
  3. Lethargy

Diagnosis for heterotaxy syndrome

  1. Cardiac MRI
  2. Cardiac catheterization (some rare cases)
  3. Chest X-ray
  4. Electrocardiogram
  5. Echocardiogram

Treatment for heterotaxy syndrome

  1. Cardiac catheterization and surgical procedures are done for “left atrial isomerism”
  2. For abnormal heart rhythm implantable pacemakers (defibrillators) are required.
  3. For right atrial isomerism, no treatments are required, but patients with left atrial isomerism need to visit pediatric cardiologist regularly for medical checkups.

Most of the patients with Left atrial isomerism and right atrial isomerism need lot of heart surgeries

  • Congenital heart disease

These are inborn birth defect affecting different conditions of heart. Different types of congenital defects are PDA, ASD, VSD, AVC, TGA, TOF, DORV, truncus arteriosus, tricuspid atresia, pulmonary atresia, CoA, AS, HLHS

Diagnosis for congenital heart disease

  1. Fetal echocardiogram
  2. Electrocardiogram
  3. Cardiac magnetic resonance imaging (MRI)
  4. Cardiac catheterization

Treatment for congenital heart disease

Treatment for congenital heart disease are

  1. Medications
  2. Heart surgery
  3. Cardiac catheterization

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