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heart disease J Am Coll Cardiol 2002;39:1890–1900.) FIG 13.4 Association of the reported prevalence of ventricular septal defects (VSD) to total (left), total exclusive of ventricular septal defects (middle), and cyanotic cardiac disease (right), as assessed from reported studies (From Hoffman JI, Kaplan S The incidence of congenital heart disease J Am Coll Cardiol 2002;39:1890–1900.) Table 13.9 Mean and Median Prevalence of Specific Congenital Cardiac Lesions for Each One Million Live Births From Multiple Reports, the New England Regional Infant Cardiac Program, and the BaltimoreWashington Infant Study Lesion Ventricular septal defect Patent arterial duct Atrial septal defect Atrioventricular septal defect Pulmonary stenosis Aortic stenosis Coarctation of the aorta Tetralogy of Fallot Transposition Hypoplastic right heart Tricuspid atresia Ebstein malformation Pulmonary atresia with intact ventricular septum Hypoplastic left heart Common arterial trunk Double-outlet right ventricle Double-inlet ventricle Number of Studies 43 40 43 40 39 37 39 41 41 32 11 11 36 30 16 23 3570 799 941 348 729 401 409 421 315 222 79 114 132 Lower Quartile 1757 324 372 242 355 161 289 291 231 105 24 38 76 266 107 157 106 154 61 82 54 Mean 2829 567 564 340 532 256 356 356 303 160 92 40 83 Upper Quartile 4482 782 1059 396 836 388 492 577 388 224 118 161 147 NERICP 1975–77 345 135 65 110 73 41 165 196 218 – 56 12 69 BWIS 1981–89 1557 115 375 360 436 141 224 328 229 81 35 47 81 226 94 127 85 279 136 245 136 163 30 32 54 184 56 95 54 Median Totally anomalous pulmonary venous connection All cyanotic All congenital cardiac diseasea Bifoliate aortic valve 25 94 60 37 43 10 1391 1078 9596 6020 13,556 5336 91 120 58 66 1270 7669 9244 1533 10,567 13,817 888 2,033 – – – 93 aExcluding bifoliate nonstenotic aortic valves, isolated partially anomalous pulmonary venous connection, and silent arterial ducts BWIS, Baltimore Washington Infant Study; NERICP, New England Regional Infant Cardiac Program Modified from Hoffman JI, Kaplan S The incidence of congenital heart disease J Am Coll Cardiol 2002;39:1890–1900; Report of the New England Regional Infant Cardiac Program Pediatrics 1980;65:375–461; and Ferencz C, Rubin JD, Loffredo CA, Magee, CA Epidemiology of Congenital Heart Disease: The Baltimore-Washington Infant Study: 1981–1989 Vol 4 Mount Kisco, NY: Futura; 1993 Another recent systematic review included 114 reports, comprising a total study population of 24,091,867 live births A total of 164,396 cases of congenital heart disease were identified The reported prevalence increased substantially over time, from 0.6 per 1000 live births in the 1930s to 9.1 per 1000 live births after 1995 Interestingly, over the past 15 years, stabilization occurred Significant geographic differences were found Asia reported the highest prevalence, with 9.3 per 1000 live births, with relatively more pulmonary outflow obstructions and fewer left ventricular outflow tract obstructions The prevalence in Europe was significantly higher than in North America (8.2 per 1000 live births vs 6.9 per 1000 live births) There was a lower prevalence in low-income countries Access to health care is still limited in many parts of the world, as are diagnostic facilities, probably accounting for differences in reported birth prevalence between high- and low-income countries.43 Adults With Congenitally Malformed Hearts It is predicted that 83% of patients born with congenitally malformed hearts will now survive to adulthood Projections suggest that this will result in the need for follow-up for more than 200 extra cases per 100,000 live births each year.44,45 In a representative population in 2000, there were equal numbers of children and adults alive with severe congenital cardiac disease.44 The Congenital Cardiac Centre for Adults in Toronto, Canada, reported a significant and steady fall in the mean age of their population over the period from 1987 to 1997, reflective of the number of young adults referred to their center who had undergone previous repair as opposed to palliation.46 Estimates indicate that, of the patients, 25% would have complex lesions, more than half would have significant lesions, and less than 20% would have minor lesions.47 Morbidity is still relatively high in this population, especially for those with complex defects Overall Kaplan-Meier estimates of mortality after 5 years for patients with a congenital cardiac defect after the age of 17 have been reported at 3.0% to as high as 12.6% for patients with cyanotic defects, and 8.2% for patients with the Fontan circulation.48 Another study used the Canadian prevalence data to statistically model the prevalence of congenitally malformed hearts in the United States after adjusting for race and ethnicity The study estimated that in year 2010, there were 2.4 million people in the United States with congenital heart disease; of them, 1.4 million are adults and 300,000 had severe disease.49 Therefore the overall point prevalence of congenital cardiac disease has and will continue to increase, with the spectrum shifting toward adults with repaired or palliated lesions This will have an important impact on increasing the need for specialized care, with services needing to expand to meet this growing need With the increase in prevalence, tertiary care centers with adult congenital cardiology clinics have seen increases of up to threefold in workload between 1992 and 1997.46 Annual rates of hospitalization for patients with all other congenital cardiac defects was also not inconsequential.50 Expenditures have been reported to be higher for adults with congenitally malformed hearts than for the general population.51 With the greater potential need for reoperation and care during follow-up, an economic and logistical burden can be expected in the ... J Am Coll Cardiol 2002;39:1890–1900; Report of the New England Regional Infant Cardiac Program Pediatrics 1980;65:375–461; and Ferencz C, Rubin JD, Loffredo CA, Magee, CA Epidemiology of Congenital Heart Disease: The Baltimore-Washington Infant Study: 1981–1989... services needing to expand to meet this growing need With the increase in prevalence, tertiary care centers with adult congenital cardiology clinics have seen increases of up to threefold in workload between 1992 and 1997.46 Annual rates of hospitalization for patients with all other