In contrast to the normal anatomical origin of the aorta and pulmonary artery from the left and right ventricle, respectively, infants with double outlet right ventricle (DORV) have both great vessels predominantly originating from the right ventricle. While the specific cause of DORV is yet to be identified, this pathological condition is usually associated with a ventricular septal defect (VSD), which determines the type of DORV.
Based on the location of the VSD, a DORV may be subaortic, subpulmonary, doubly committed, or non-committed. In addition to DORVs being typically accompanied by VSDs, other cardiac anomalies such as pulmonary artery stenosis and transposition of the great vessels may also be seen. Diagnosis is made on the grounds of imaging techniques like echocardiograms, which uses sound waves to illustrate the great vessels and their spatial relationship to each other, the VSD, and any other cardiac anomaly.
The majority of infants affected tend to present within their first few weeks of life with cyanosis, which is a bluish discoloration of the skin due to poor oxygen saturation. These infants may also have difficulties breathing as well as poor feeding and a general failure to thrive. In severe cases, infants may have congestive heart failure, which requires immediate and aggressive therapy. Surgery is generally the treatment of choice, but there are several complications that may be associated with DORV.
Thanks to improved contemporary treatment techniques and thorough follow-up care guidelines, complications arising as a result of DORV are becoming increasingly uncommon. Nonetheless, complications include pulmonary hypertension and irreversible pulmonary damage due to the uncontrolled high pulmonary blood pressure. Complications may arise due to the surgical treatment of DORV as well. These depend on the type of surgical operation that was performed and the anatomy of the lesion.
Patients who have subpulmonary and subaortic DORVs may experience a decrease in the diameter of the VSD immediately following surgery. These patients may as a result develop subaortic obstruction. Other structural and functional abnormalities that may arise from complex repairs include ventricular dysfunction, aortic insufficiency, and insufficiency of the atrioventricular valve. Postoperative arrhythmias and even sudden death may also occur in some patients who are at risk.
Prognosis and follow-up
Not withstanding the potential complications that may arise, enormous strides in our understanding, diagnosis, and treatment of the condition make it possible for most people born with DORV to lead relatively normal and productive lives. In fact, some patients will not experience any further cardiac problems, postoperatively. On the flip side, however, there are some instances where problems with the cardiac valves or rhythm may occur. New problems may require catheterization or additional surgeries.
With the advancement in surgical techniques the odds are great that most infants will undergo successful DORV surgical repairs. Children are carefully followed after their surgeries and they may need to continue seeing a cardiologist well into their teenage years as well as into adulthood for optimal results and a great long-term outlook. Cardiologists assist with the crafted care program and give guidelines with regards to nutrition, pregnancy,exercise and activity levels.
Reviewed by Susha Cheriyedath, MSc