In the past 2 decades, it has become evident that individuals born with congenital heart disease (CHD) are at risk of developing life-long neurological deficits. Multifactorial risk factors contributing to neurodevelopmental abnormalities associated with CHD have been identified; however, the underlying causes remain largely unknown, and efforts to address this issue have only recently begun. There has been a dramatic shift in focus from newly acquired brain injuries associated with corrective and palliative heart surgery to antenatal and preoperative factors governing altered brain maturation in CHD. In this review, we describe key time windows of development during which the immature brain is vulnerable to injury. Special emphasis is placed on the dynamic nature of cellular events and how CHD may adversely impact the cellular units and networks necessary for proper cognitive and motor function. In addition, we describe current gaps in knowledge and offer perspectives about what can be done to improve our understanding of neurological deficits in CHD. Ultimately, a multidisciplinary approach will be essential to prevent or improve adverse neurodevelopmental outcomes in individuals surviving CHD.

Congenital heart disease depletes SVZ neural stem/progenitor cell pools critical for normal cortical growth.

Truncus arteriosus (common arterial trunk) is an uncommon but complex congenital heart anomaly. Until the early 1970s, typically, patients died between the age of a few weeks to six months. Congestive heart failure owing to large pulmonary blood flow and truncal valve regurgitation was the major cause of death until innovative surgical techniques were discovered. In 1963, Herbert Sloan at the University of Michigan completed the first repair using a nonvalved conduit with long-term survival (not reported until 1974). At the Mayo Clinic, Rastelli and McGoon studied and completed the first repair with a valved homograft in 1967. In 1976, Ebert used the 12-mm Hancock valved conduit in infants under six months of age (University of California, San Francisco). In Boston (mid-1980s), Jonas and Castañeda used aortic homografts, which greatly reduced bleeding as a postoperative complication. In the early 1990s, Bove (University of Michigan) reported outstanding results with an approach based on primary repair within the first few days of life for patients with truncus arteriosus. Improved prognosis for patients with truncus arteriosus resulted from these corrective operations by analyzing the natural history of this condition while applying innovative ideas, improved technology, and perioperative care.

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