Double inlet left ventricle (DILV) is a malformation of heart since birth. In this condition the two upper chambers of heart connect to the left ventricle. It is a rare disease affecting 5 out of 100000 live births. In DILV only left ventricle is functioning. The blood from both atrium flows into the left ventricle. This blood is mix blood, meaning oxygen rich and oxygen poor blood. From left ventricle this mix blood is supplied throughout the body.
This abnormality produces several symptoms which include blue discoloration of skin, breathing difficulty, poor feeding, failure to gain weight, and eventually left ventricular failure if the defect is not treated.The exact cause of double inlet left ventricle is not known, but many other cardiac malformations accompany double inlet left ventricle. Treatment consists of surgical correction of DILV.
What Is The Cause Of Double Inlet Left Ventricle?
The exact cause of double inlet left ventricle is not clear. It is a congenital defect. This means the infant is born with this type of heart defect. It is not genetically associated meaning there is no abnormality in parents gene. The malformation of heart thus occurs during the developmental stage of the fetus, usually between 2nd and 4th week of pregnancy.A child with DILV may have associated other heart malformations such as coarctation of aorta, pulmonary atresia, pulmonary stenosis, or transposition of aorta and pulmonary artery.
Normally human heart consists of 4 chambers; the top left and right atrium and bottom left and right ventricle. The oxygen poor blood enters into the right atrium through vena cava. It then flows into the right ventricle from where blood is carried to the lungs through pulmonary artery. Once the blood is oxygenated in the lungs it enters into the left atrium through pulmonary vein. From left atrium the pure oxygenated blood goes into left ventricle. The left ventricle than pumps the oxygenated blood through the great vessel called aorta throughout the body.
In infants with double inlet left ventricle, the left ventricle is normal, but the right ventricle is small and non functional. Instead of right atrium supplying blood to the right ventricle and left to left ventricle, the blood from both atrium goes into the left ventricle. This blood is mix blood; oxygen rich and oxygen poor. The mix blood flows throughout the body producing various symptoms.
Symptoms Of Double Inlet Left Ventricle
In double inlet left ventricle the oxygen rich as well as oxygen poor blood circulates in the body. This may affect oxygen saturation in the tissues giving rise to various symptoms. They include;
- Blue discoloration of skin, lips, tongue and mouth.
- Difficulty in breathing.
- Failure to thrive.
- Poor feeding.
- Increased sweating.
- Trouble in achieving normal milestone.
- Swelling in legs, face and abdomen.
- Abnormal heart sound (heart murmur).
- Left ventricular enlargement leading to heart failure.
Diagnosis And Treatment Of Double Inlet Left Ventricle
The doctor will examine the child and his heart sound with the help of stethoscope. He can hear an abnormal heart sound called murmur in a baby with double inlet left ventricle. He will recommend further tests which include echo-cardiogram, electrocardiogram, cardiac catheterization, and X-ray chest.
Treatment of double inlet left ventricle is surgical repair of the heart. The child may need many surgeries so that his heart can work properly. The surgery is generally done in first few weeks after birth. The child may need one or two or three surgeries over a period of time to reconfigure the heart.
The doctor may also prescribe the child medicines before and after surgery. These medicines are ACE inhibitors, medicine that are effective in contracting heart, anticoagulants, and diuretics. Usually with proper surgical repair the child can live into adulthood. However, he may need regular checkups. He may need to take antibiotics during dental treatment or any infection to prevent damage of the heart valve.