Pathophysiology of Congenital Heart Disease in the Adult

摘要

HomeCirculationVol. 117, No. 8Pathophysiology of Congenital Heart Disease in the Adult Free AccessReview ArticlePDF/EPUBAboutView PDFView EPUBSections ToolsAdd to favoritesDownload citationsTrack citationsPermissions ShareShare onFacebookTwitterLinked InMendeleyReddit Jump toFree AccessReview ArticlePDF/EPUBPathophysiology of Congenital Heart Disease in the AdultPart I: Shunt Lesions Robert J. Sommer, Ziyad M. Hijazi and John F. RhodesJr Robert J. SommerRobert J. Sommer From the Center for Interventional Vascular Therapy, Cardiovascular Research Foundation, Columbia University Medical Center, New York, NY (R.J.S.); Department of Pediatrics and Medicine, Rush Center for Congenital and Structural Heart Disease, Rush University Medical Center, Chicago, Ill (Z.M.H.); and Department of Pediatrics, Division of Pediatric Cardiology, Duke University Medical Center, Durham, NC (J.F.R.). , Ziyad M. HijaziZiyad M. Hijazi From the Center for Interventional Vascular Therapy, Cardiovascular Research Foundation, Columbia University Medical Center, New York, NY (R.J.S.); Department of Pediatrics and Medicine, Rush Center for Congenital and Structural Heart Disease, Rush University Medical Center, Chicago, Ill (Z.M.H.); and Department of Pediatrics, Division of Pediatric Cardiology, Duke University Medical Center, Durham, NC (J.F.R.). and John F. RhodesJrJohn F. RhodesJr From the Center for Interventional Vascular Therapy, Cardiovascular Research Foundation, Columbia University Medical Center, New York, NY (R.J.S.); Department of Pediatrics and Medicine, Rush Center for Congenital and Structural Heart Disease, Rush University Medical Center, Chicago, Ill (Z.M.H.); and Department of Pediatrics, Division of Pediatric Cardiology, Duke University Medical Center, Durham, NC (J.F.R.). Originally published26 Feb 2008https://doi.org/10.1161/CIRCULATIONAHA.107.714402Circulation. 2008;117:1090–1099Congenital heart disease is common, occurring in ≈8 of 1000 live births.1 With the successes in cardiothoracic surgery over the past 3 decades and the ongoing improvements in the diagnostic, interventional, and critical care skills of pediatric cardiologists, ≈90% of children born with heart defects now survive to adulthood.2 In addition, using improved noninvasive techniques, adult cardiologists are increasingly identifying adults with septal defects that were undiagnosed in childhood. The adult congenital heart disease patients carry a spectrum of disease, from small septal defects and minor valvar obstructions to complex single-ventricle lesions that have been palliated with staged surgical repairs. It is estimated that >1 million adults in the United States now have congenital heart disease, outnumbering their pediatric counterparts for the first time.3,4While the adult cardiology community struggles with a population that once was the exclusive domain of pediatricians, governmental agencies, national physician associations, and cardiology advisory boards are trying to define the scope of this national healthcare issue and to figure out how to train current and future generations of doctors.5 This specialized cardiac care will require the diagnosis of adult congenital heart disease in patients presenting de novo with new or chronic symptoms, the long-term maintenance of those previously diagnosed, and the ability to recognize when primary or additional interventions are required. As these patients increasingly present to cardiologists' offices for care, healthcare professionals will need to develop a better level of comfort with adult congenital heart disease.This 3-part series focuses on the pathophysiology of congenital heart lesions, which are seen commonly in adult patients. In this first portion, simple shunt lesions are reviewed. For each, the natural history and common clinical presentations resulting from the shunt are discussed. A discussion of therapeutic options and the literature supporting these options is beyond the scope of this series. Pati