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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
Difficulty in breathing and feeding
Skin, lips and nails will have blue or purple tint.
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
Difficulty in breathing and feeding
Skin, lips and nails will have blue or purple tint.
Lethargy
Diagnosis for hypoplastic left heart syndrome
Cardiac MRI
Cardiac catheterization
Chest X-ray
Electrocardiogram
Pulse oximetry (to detect the content of oxygen in blood)
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
No tricuspid valve
Have smaller right ventricle
ASD (atrial septal defect)
VSD (ventricular septal defect)
Tricuspid atresia is often associated with pulmonary stenosis and transposition of great arteries
Signs and symptoms for tricuspid atresia
Fatigue
Poor weight gain
Shortness of breath
Heart murmur
Difficulty feeding
Cyanosis (Skin, lips and nails will have blue or purple tint)
In older children, there will be abnormal shape for finger tips.
Diagnosis for tricuspid atresia
Cardiac catheterization
Electrocardiogram
Blood test
Echocardiogram
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
Skin, lips and nails will have blue or purple tint
Poor weight gain
Shortness of breath
Unresponsiveness
Sweating during feeding
Irregular fatigue
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
Magnetic resonance imaging (MRI)
Cardiac catheterization
Chest X-ray
Electrocardiogram
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
Septal defects
Problems with heart valves (particularly pulmonary heart valve)
Anomalous pulmonary venous connection (abnormalities of blood coming back from lungs to heart)
Absence of spleen
Liver and other organs on wrong side of the body
Left atrial isomerism
Children with left atrial isomerism have
Septal defects
Problems with heart valves
Problems with hearts electrical system
Complete heart block
There will be no spleen or may have polysplenia (several small spleens)
Signs and symptoms for heterotaxy syndrome
Difficulty in breathing and feeding
Skin, lips and nails will have blue or purple tint.
Lethargy
Diagnosis for heterotaxy syndrome
Cardiac MRI
Cardiac catheterization (some rare cases)
Chest X-ray
Electrocardiogram
Echocardiogram
Treatment for heterotaxy syndrome
Cardiac catheterization and surgical procedures are done for left atrial isomerism
For abnormal heart rhythm implantable pacemakers (defibrillators) are required.
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
Fetal echocardiogram
Electrocardiogram
Cardiac magnetic resonance imaging (MRI)
Cardiac catheterization
Treatment for congenital heart disease
Treatment for congenital heart disease are
Medications
Heart surgery
Cardiac catheterization
