Exceptional survival: double inlet left ventricle presenting with aortic dissection

Area of Science:

• Cardiology
• Congenital Heart Disease
• Adult Congenital Heart Disease

Background:

• Univentricular heart (UVH) is a rare congenital heart defect where only one ventricle adequately pumps blood to the body.
• Survival into adulthood with unoperated UVH is uncommon and typically associated with specific hemodynamic conditions, like balanced circulations.
• Balanced circulations, such as double inlet left ventricle with moderate pulmonary stenosis, offer better long-term survival prospects.

Observation:

• A case is presented of a woman who experienced an aortic dissection.
• During the diagnostic workup for aortic dissection, a previously unrecognized univentricular heart was identified.
• This presentation is unusual, as significant cardiovascular events can occur in adults with undiagnosed complex congenital heart disease.

Findings:

• The patient's univentricular heart physiology was likely a contributing factor to the aortic dissection, though the exact mechanism requires further elucidation.
• This case underscores the potential for severe cardiovascular complications in adults with undiagnosed congenital heart anomalies.
• The successful management of this complex case highlights the importance of comprehensive cardiac evaluation in patients presenting with seemingly unrelated acute cardiovascular events.

Implications:

• This case emphasizes the need for increased awareness and diagnostic vigilance for congenital heart disease in adult populations, particularly when presenting with acute cardiovascular syndromes.
• It suggests that univentricular heart physiology, even when previously unrecognized, may predispose individuals to serious vascular complications like aortic dissection.
• Further research into the long-term outcomes and specific risks associated with unoperated univentricular hearts in adults is warranted.