SURVIVAL PATTERNS

Unoperated Patients

The term natural history is a misnomer, because mortality and morbidity for patients who have not undergone cardiac surgery have been materially influenced by advances in medical management. Accordingly, “natural history” should be replaced by “unoperated survival.”

Unoperated patients include those with malformations that do not require surgery, malformations that are amenable to operation in adulthood, and malformations that are inoperable except for heart, lung, or heart-lung transplantation. Management of unoperated adults must take into account not only the congenital disorder per se but also the medical disorders that are inherent components of certain types of congenital heart disease, as well as acquired disorders of the heart and circulation that coexist with and modify the physiological expressions of the congenital malformation. This section deals chiefly with common or uncommon defects in which unoperated adult survival is expected and with certain defects that are common and therefore familiar but for which adult survival is exceptional.

Common Defects in Which Unoperated Adult Survival is Expected

Common Defects in Which Unoperated Adult Survival is Expected

BICUSPID AORTIC VALVE

BICUSPID AORTIC VALVE (see also [For More Information] , and Fig. 3–67 Fig. 3–67 , and Fig. 29–33 Fig. 29–33 ). This malformation is the most frequent congenital anomaly to which that structure is subject and is one of the most common gross morphological congenital anomalies of the heart or great arteries.13 Bicuspid aortic valves that are functionally normal at birth can remain so throughout a normal life span. Progressive stenosis results from fibrocalcific thickening, a substrate that accounts for about one-half of surgical cases of isolated calcific aortic valve stenosis in adults (Fig. 30–1 Fig. 30–1 ).14 Conversely, a functionally normal bicuspid aortic valve may develop progressive incompetence and is an important cause of anatomically isolated aortic regurgitation in adults.5 A bicuspid aortic valve may be modified, sometimes suddenly and appreciably, by infective endocarditis, to which the malformation is highly susceptible.15 An inherent relationship exists between a congenital bicuspid aortic valve and an abnormality of the aortic root that takes the form of cystic medial necrosis related to the bicuspid valve, whether functionally normal, stenotic, or incompetent.5,16,16a This aortic root disease can express itself in older adults as an aneurysm with aortic regurgitation or can announce itself dramatically in younger adults as aortic dissection (Fig. 30–2 Fig. 30–2 ).

Date: 2014-12-21; view: 1006