This is a cyanotic congenital heart disease. In this CHD, there is a large ventricular septal defect (VSD) along with only one large artery arising from base of the heart that gives rise to pulmonary arteries and supplies all parts of the body. There is no direct origin of pulmonary arteries from the ventricle.
Patients generally present at age of 1 and 1.5 months with sign and symptoms of heart failure like shortness of breath, frequent cough and cold etc. Initially, there may not be visible cyanosis (bluish discoloration of fingers and lips), that can be picked only by saturation monitor machine.
If patient is not getting operated early then disease may progress rapidly into inoperable phase known as Eisenmenger complex.
Echocardiography is best modality for diagnosing this form of congenital heart disease.
Treatment is only by open heart surgery with VSD surgical closure and placement of an artificial tube known as CONDUITfrom right ventricle along with attachment of pulmonary arteries to this conduit so that systemic and pulmonary circulations can be separated.
Outcome after surgeries are good and patient can live a good quality of life. Without surgery, patient can die very early.