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Treatment of congenital heart disease: risk-reducing measures in young adults

    Teun van der Bom

    Department of Cardiology, Academic Medical Center, Amsterdam, The Netherlands.

    Interuniversity Cardiology Institute of The Netherlands, Utrecht, The Netherlands

    ,
    Paul Luijendijk

    Department of Cardiology, Academic Medical Center, Amsterdam, The Netherlands.

    Interuniversity Cardiology Institute of The Netherlands, Utrecht, The Netherlands

    ,
    Berto J Bouma

    Department of Cardiology, Academic Medical Center, Amsterdam, The Netherlands.

    ,
    Dave R Koolbergen

    Department of Cardio-thoracic Surgery, Academic Medical Center, Amsterdam, The Netherlands

    ,
    Joris R de Groot

    Department of Cardiology, Academic Medical Center, Amsterdam, The Netherlands.

    &
    Barbara JM Mulder

    † Author for correspondence

    Department of Cardiology, Academic Medical Center, Amsterdam, The Netherlands.

    Interuniversity Cardiology Institute of The Netherlands, Utrecht, The Netherlands

    Published Online:https://doi.org/10.2217/fca.11.5

    Adults with congenital heart disease form a new and relatively young population, since surgical treatment of heart defects became available three to four decades ago. Owing to improved survival this population is steadily growing in number and age. Little is known regarding long-term survival; however, late complications occur frequently. During adulthood, almost half of the patients have one or more complication, such as endocarditis, stroke, systemic or pulmonary hypertension, aortic aneurysm or dissection and arrhythmias. Heart failure and sudden cardiac death are the main causes of death. Treatment of adults with congenital heart disease is aimed at the reduction of symptoms, but also at minimizing the risk and severity of late complications. In this article the most recent advances in the treatment of congenital heart disease will be discussed. The main focus of the article will be on pharmacological, interventional and surgical interventions that reduce the risk of heart failure, arrhythmias, vascular complications, pulmonary hypertension and endocarditis.

    Papers of special note have been highlighted as: ▪ of interest ▪▪ of considerable interest

    Bibliography

    • Khairy P, Ionescu-Ittu R, Mackie AS et al.: Changing mortality in congenital heart disease. J. Am. Coll. Cardiol.56(14),1149–1157 (2010).
    • Marelli AJ, Mackie AS, Ionescu-Ittu R, Rahme E, Pilote L: Congenital heart disease in the general population: changing prevalence and age distribution. Circulation115(2),163–172 (2007).
    • Engelfriet P, Boersma E, Oechslin E et al.: The spectrum of adult congenital heart disease in Europe: morbidity and mortality in a 5 year follow-up period. The Euro Heart Survey on adult congenital heart disease. Eur. Heart J.26(21),2325–2333 (2005).
    • Warnes CA: The adult with congenital heart disease: born to be bad? J. Am. Coll. Cardiol.46(1),1–8 (2005).
    • Oechslin EN, Harrison DA, Connelly MS, Webb GD, Siu SC: Mode of death in adults with congenital heart disease. Am. J. Cardiol.86(10),1111–1116 (2000).
    • Pillutla P, Shetty KD, Foster E: Mortality associated with adult congenital heart disease: trends in the US population from 1979 to 2005. Am. Heart J.158(5),874–879 (2009).
    • Zomer AC, Uiterwaal CSPM, der Velde ETV et al.: Mortality in adult congenital heart disease: are national registries reliable for cause of death? Int. J. Cardiol. (2010) (Epub ahead of print).
    • Baumgartner H, Bonhoeffer P, De Groot NMS et al.: ESC Guidelines for the management of grown-up congenital heart disease (new version 2010): the Task Force on the Management of Grown-up Congenital Heart Disease of the European Society of Cardiology (ESC). Eur. Heart J.31(23),2915–2957 (2010).
    • Warnes CA, Williams RG, Bashore TM et al.: ACC/AHA 2008 guidelines for the management of adults with congenital heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Develop Guidelines on the Management of Adults With Congenital Heart Disease). Developed in Collaboration With the American Society of Echocardiography, Heart Rhythm Society, International Society for Adult Congenital Heart Disease, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic Surgeons. J. Am. Coll. Cardiol.52(23),e1–e121 (2008).
    • 10  Piran S, Veldtman G, Siu S, Webb GD, Liu PP: Heart failure and ventricular dysfunction in patients with single or systemic right ventricles. Circulation105(10),1189–1194 (2002).
    • 11  McKee PA, Castelli WP, McNamara PM, Kannel WB: The natural history of congestive heart failure: the Framingham study. N. Engl. J. Med.285(26),1441–1446 (1971).
    • 12  Verheugt CL, Uiterwaal CSPM, van der Velde ET et al.: Mortality in adult congenital heart disease. Eur. Heart J.31(10),1220–1229 (2010).
    • 13  Warnes CA: Adult congenital heart disease importance of the right ventricle. J. Am. Coll. Cardiol.54(21),1903–1910 (2009).
    • 14  Ikeda U, Furuse M, Suzuki O et al.: Long-term survival in aged patients with corrected transposition of the great arteries. Chest101(5),1382–1385 (1992).
    • 15  Pézard P, Banus Y, Laporte J et al.: Corrected transposition of the great vessels in aged adults. Apropos of 2 patients aged 72 and 80. Arch. Mal Coeur Vaiss.79(11),1637–1642 (1986).
    • 16  Book W: Heart failure in the adult patient with congenital heart disease. J. Card. Fail.11(4),306–312 (2005).
    • 17  Bolger AP, Sharma R, Li W et al.: Neurohormonal activation and the chronic heart failure syndrome in adults with congenital heart disease. Circulation106(1),92–99 (2002).
    • 18  Effect of enalapril on survival in patients with reduced left ventricular ejection fractions and congestive heart failure. The SOLVD Investigators. N. Engl. J. Med.325(5),293–302 (1991).
    • 19  Pfeffer MA, Swedberg K, Granger CB et al.: Effects of candesartan on mortality and morbidity in patients with chronic heart failure: the CHARM-Overall programme. Lancet362(9386),759–766 (2003).
    • 20  Effect of metoprolol CR/XL in chronic heart failure: Metoprolol CR/XL Randomised Intervention Trial in Congestive Heart Failure (MERIT-HF). Lancet353(9169),2001–2007 (1999).
    • 21  Brophy JM, Joseph L, Rouleau JL: β-blockers in congestive heart failure. A Bayesian meta-analysis. Ann. Intern. Med.134(7),550–560 (2001).
    • 22  Winter MM, van der Plas MN, Bouma BJ et al.: Mechanisms for cardiac output augmentation in patients with a systemic right ventricle. Int. J. Cardiol.143(2),141–146 (2009).
    • 23  Uebing A, Diller G, Li W et al.: Optimized rate-responsive pacing does not improve either right ventricular hemodynamics or exercise capacity in adults with a systemic right ventricle. Cardiol. Young20(5),485–494 (2010).
    • 24  Haddad F, Doyle R, Murphy DJ, Hunt SA: Right ventricular function in cardiovascular disease, part II: pathophysiology, clinical importance, and management of right ventricular failure. Circulation117(13),1717–1731 (2008).
    • 25  Winter MM, Bouma BJ, Groenink M et al.: Latest insights in therapeutic options for systemic right ventricular failure: a comparison with left ventricular failure. Heart95(12),960–963 (2009).
    • 26  Dore A, Houde C, Chan K et al.: Angiotensin receptor blockade and exercise capacity in adults with systemic right ventricles: a multicenter, randomized, placebo-controlled clinical trial. Circulation112(16),2411–2416 (2005).
    • 27  van der Bom T, Winter MM, Bouma BJ et al.: Rationale and design of a trial on the effect of angiotensin II receptor blockers on the function of the systemic right ventricle. Am. Heart J.160(5),812–818 (2010).
    • 28  Schuuring MJ, Vis JC, Duffels MG, Bouma BJ, Mulder BJ: Adult patients with pulmonary arterial hypertension due to congenital heart disease: a review on advanced medical treatment with bosentan. Ther. Clin. Risk Manag.6,359–366 (2010).
    • 29  Beghetti M: Fontan and the pulmonary circulation: a potential role for new pulmonary hypertension therapies. Heart96(12),911–916 (2010).
    • 30  Ishibashi N, Park I, Takahashi Y et al.: Effectiveness of carvedilol for congestive heart failure that developed long after modified Fontan operation. Pediatr. Cardiol.27(4),473–475 (2006).
    • 31  Kouatli AA, Garcia JA, Zellers TM, Weinstein EM, Mahony L: Enalapril does not enhance exercise capacity in patients after Fontan procedure. Circulation96(5),1507–1512 (1997).
    • 32  Hsu DT, Zak V, Mahony L et al.: Enalapril in infants with single ventricle: results of a multicenter randomized trial. Circulation122(4),333–340 (2010).
    • 33  Haddad F, Ashley E, Michelakis ED: New insights for the diagnosis and management of right ventricular failure, from molecular imaging to targeted right ventricular therapy. Curr. Opin. Cardiol.25(2),131–140 (2010).
    • 34  Nagendran J, Archer SL, Soliman D et al.: Phosphodiesterase type 5 is highly expressed in the hypertrophied human right ventricle, and acute inhibition of phosphodiesterase type 5 improves contractility. Circulation116(3),238–248 (2007).
    • 35  Michelakis E, Tymchak W, Lien D et al.: Oral sildenafil is an effective and specific pulmonary vasodilator in patients with pulmonary arterial hypertension: comparison with inhaled nitric oxide. Circulation105(20),2398–2403 (2002).
    • 36  Graham EM, Bandisode VM, Bradley SM et al.: Effect of preoperative use of propranolol on postoperative outcome in patients with tetralogy of Fallot. Am. J. Cardiol.101(5),693–695 (2008).
    • 37  Norozi K, Bahlmann J, Raab B et al.: A prospective, randomized, double-blind, placebo controlled trial of β-blockade in patients who have undergone surgical correction of tetralogy of Fallot. Cardiol. Young17(4),372–379 (2007).
    • 38  Cleland JGF, Daubert J, Erdmann E et al.: The effect of cardiac resynchronization on morbidity and mortality in heart failure. N. Engl. J. Med.352(15),1539–1549 (2005).
    • 39  Janousek J: Cardiac resynchronization in congenital heart disease. Heart95(11),940–947 (2009).▪ Important study on cardiac resynchronization therapy in congenital heart disease.
    • 40  Cecchin F, Frangini PA, Brown DW et al.: Cardiac resynchronization therapy (and multisite pacing) in pediatrics and congenital heart disease: five years experience in a single institution. J. Cardiovasc. Electrophysiol.20(1),58–65 (2009).
    • 41  Dubin AM, Janousek J, Rhee E et al.: Resynchronization therapy in pediatric and congenital heart disease patients: an international multicenter study. J. Am. Coll. Cardiol.46(12),2277–2283 (2005).
    • 42  Khairy P: EP challenges in adult congenital heart disease. Heart Rhythm.5(10),1464–1472 (2008).
    • 43  Moss AJ, Hall WJ, Cannom DS et al.: Cardiac-resynchronization therapy for the prevention of heart-failure events. N. Engl. J. Med.361(14),1329–1338 (2009).
    • 44  Oosterhof T, van Straten A, Vliegen HW et al.: Preoperative thresholds for pulmonary valve replacement in patients with corrected tetralogy of Fallot using cardiovascular magnetic resonance. Circulation116(5),545–551 (2007).
    • 45  Therrien J, Siu SC, McLaughlin PR et al.: Pulmonary valve replacement in adults late after repair of tetralogy of fallot: are we operating too late? J. Am. Coll. Cardiol.36(5),1670–1675 (2000).
    • 46  Oosterhof T, Meijboom FJ, Vliegen HW et al.: Long-term follow-up of homograft function after pulmonary valve replacement in patients with tetralogy of Fallot. Eur. Heart J.27(12),1478–1484 (2006).
    • 47  Bouzas B, Kilner PJ, Gatzoulis MA: Pulmonary regurgitation: not a benign lesion. Eur. Heart J.26(5),433–439 (2005).
    • 48  Vliegen HW, van Straten A, de Roos A et al.: Magnetic resonance imaging to assess the hemodynamic effects of pulmonary valve replacement in adults late after repair of tetralogy of Fallot. Circulation106(13),1703–1707 (2002).
    • 49  Mulder BJM, de Winter RJ, Wilde AAM: Percutaneous pulmonary valve replacement: a new development in the lifetime strategy for patients with congenital heart disease. Neth. Heart J.15(1),3–4 (2007).
    • 50  Oosterhof T, Hazekamp MG, Mulder BJM: Opportunities in pulmonary valve replacement. Expert Rev. Cardiovasc. Ther.7(9),1117–1122 (2009).
    • 51  Bonhoeffer P, Boudjemline Y, Saliba Z et al.: Percutaneous replacement of pulmonary valve in a right-ventricle to pulmonary-artery prosthetic conduit with valve dysfunction. Lancet356(9239),1403–1405 (2000).
    • 52  Lurz P, Gaudin R, Taylor AM, Bonhoeffer P: Percutaneous pulmonary valve implantation. Semin. Thorac. Cardiovasc. Surg. Pediatr. Card. Surg. Annu.12,112–117 (2009).▪▪ Study describing the potential clinical importance of percutaneuous pulmonary valve implantation.
    • 53  Lurz P, Coats L, Khambadkone S et al.: Percutaneous pulmonary valve implantation: impact of evolving technology and learning curve on clinical outcome. Circulation117(15),1964–1972 (2008).
    • 54  Romeih S, Kroft LJ, Bokenkamp R et al.: Delayed improvement of right ventricular diastolic function and regression of right ventricular mass after percutaneous pulmonary valve implantation in patients with congenital heart disease. Am. Heart J.158(1),40–46 (2009).
    • 55  Koyak Z, de Groot JR, Mulder BJ: Interventional and surgical treatment of cardiac arrhythmias in adults with congenital heart disease. Expert Rev. Cardiovasc. Ther.8(12),1753–1766 (2010).
    • 56  de Groot NMS, Atary JZ, Blom NA, Schalij MJ: Long-term outcome after ablative therapy of postoperative atrial tachyarrhythmia in patients with congenital heart disease and characteristics of atrial tachyarrhythmia recurrences. Circ. Arrhythm Electrophysiol.3(2),148–154 (2010).
    • 57  Thorne SA, Barnes I, Cullinan P, Somerville J: Amiodarone-associated thyroid dysfunction: risk factors in adults with congenital heart disease. Circulation100(2),149–154 (1999).
    • 58  Dobrev D, Nattel S: New antiarrhythmic drugs for treatment of atrial fibrillation. Lancet375(9721),1212–1223 (2010).
    • 59  Khairy P, Aboulhosn J, Gurvitz MZ et al.: Arrhythmia burden in adults with surgically repaired tetralogy of Fallot: a multi-institutional study. Circulation122(9),868–875 (2010).▪▪ Important study on risk stratification for arrhythmia in patients with surgically repaired tetralogy of Fallot.
    • 60  Kiesewetter C, Michael K, Morgan J, Veldtman GR: Left ventricular dysfunction after cardiac resynchronization therapy in congenital heart disease patients with a failing systemic right ventricle. Pacing Clin. Electrophysiol.31(2),159–162 (2008).
    • 61  Bardy GH, Smith WM, Hood MA et al.: An entirely subcutaneous implantable cardioverter–defibrillator. N. Engl. J. Med.363(1),36–44 (2010).
    • 62  Dave AS, Aboulhosn J, Child JS, Shivkumar K: Transconduit puncture for catheter ablation of atrial tachycardia in a patient with extracardiac Fontan palliation. Heart Rhythm.7(3),413–416 (2010).
    • 63  Khairy P, Fournier A, Ruest P, Vobecky SJ: Transcatheter ablation via a sternotomy approach as a hybrid procedure in a univentricular heart. Pacing Clin. Electrophysiol.31(5),639–640 (2008).
    • 64  Biviano A, Garan H, Hickey K et al.: Atrial flutter catheter ablation in adult patients with repaired tetralogy of Fallot: mechanisms and outcomes of percutaneous catheter ablation in a consecutive series. J. Interv. Card. Electrophysiol.28(2),125–135 (2010).
    • 65  Cappato R, Schlüter M, Weiss C et al.: Radiofrequency current catheter ablation of accessory atrioventricular pathways in Ebstein’s anomaly. Circulation94(3),376–383 (1996).
    • 66  Hoffmann A, Chockalingam P, Balint OH et al.: Cerebrovascular accidents in adult patients with congenital heart disease. Heart96(15),1223–1226 (2010).
    • 67  Perloff JK, Rosove MH, Child JS, Wright GB: Adults with cyanotic congenital heart disease: hematologic management. Ann. Intern. Med.109(5),406–413 (1988).
    • 68  Thanassoulis G, Yip JWL, Filion K et al.: Retrospective study to identify predictors of the presence and rapid progression of aortic dilatation in patients with bicuspid aortic valves. Nat. Clin. Pract. Cardiovasc. Med.5(12),821–828 (2008).
    • 69  Vriend JWJ, Mulder BJM: Late complications in patients after repair of aortic coarctation: implications for management. Int. J. Cardiol.101(3),399–406 (2005).
    • 70  Vriend JWJ, van Montfrans GA, Romkes HH et al.: Relation between exercise-induced hypertension and sustained hypertension in adult patients after successful repair of aortic coarctation. J. Hypertens.22(3),501–509 (2004).
    • 71  Clarkson PM, Nicholson MR, Barratt-Boyes BG, Neutze JM, Whitlock RM: Results after repair of coarctation of the aorta beyond infancy: a 10 to 28 year follow-up with particular reference to late systemic hypertension. Am. J. Cardiol.51(9),1481–1488 (1983).
    • 72  O’Sullivan JJ, Derrick G, Darnell R: Prevalence of hypertension in children after early repair of coarctation of the aorta: a cohort study using casual and 24 hour blood pressure measurement. Heart88(2),163–166 (2002).
    • 73  Beekman RH, Katz BP, Moorehead-Steffens C, Rocchini AP: Altered baroreceptor function in children with systolic hypertension after coarctation repair. Am. J. Cardiol.52(1),112–117 (1983).
    • 74  Polson JW, McCallion N, Waki H et al.: Evidence for cardiovascular autonomic dysfunction in neonates with coarctation of the aorta. Circulation113(24),2844–2850 (2006).
    • 75  Gibbons GH, Dzau VJ: The emerging concept of vascular remodeling. N. Engl. J. Med.330(20),1431–1438 (1994).
    • 76  Therrien J, Webb G: Clinical update on adults with congenital heart disease. Lancet362(9392),1305–1313 (2003).
    • 77  Niwa K, Siu SC, Webb GD, Gatzoulis MA: Progressive aortic root dilatation in adults late after repair of tetralogy of Fallot. Circulation106(11),1374–1378 (2002).
    • 78  Dodds GA, Warnes CA, Danielson GK: Aortic valve replacement after repair of pulmonary atresia and ventricular septal defect or tetralogy of Fallot. J. Thorac. Cardiovasc. Surg.113(4),736–741 (1997).
    • 79  Niwa K, Perloff JK, Bhuta SM et al.: Structural abnormalities of great arterial walls in congenital heart disease: light and electron microscopic analyses. Circulation103(3),393–400 (2001).
    • 80  Tan JL, Davlouros PA, McCarthy KP, Gatzoulis MA, Ho SY: Intrinsic histological abnormalities of aortic root and ascending aorta in tetralogy of Fallot: evidence of causative mechanism for aortic dilatation and aortopathy. Circulation112(7),961–968 (2005).
    • 81  Cañadas V, Vilacosta I, Bruna I, Fuster V: Marfan syndrome. Part 1: pathophysiology and diagnosis. Nat. Rev. Cardiol.7(5),256–265 (2010).
    • 82  Engelfriet P, Mulder B: Is there benefit of β-blocking agents in the treatment of patients with the Marfan syndrome? Int. J. Cardiol.114(3),300–302 (2007).
    • 83  Gersony DR, McClaughlin MA, Jin Z, Gersony WM: The effect of β-blocker therapy on clinical outcome in patients with Marfan’s syndrome: a meta-analysis. Int. J. Cardiol.114(3),303–308 (2007).
    • 84  Williams A, Davies S, Stuart AG, Wilson DG, Fraser AG: Medical treatment of Marfan syndrome: a time for change. Heart94(4),414–421 (2008).
    • 85  Habashi JP, Judge DP, Holm TM et al.: Losartan, an AT1 antagonist, prevents aortic aneurysm in a mouse model of Marfan syndrome. Science312(5770),117–121 (2006).▪▪ Demonstration of the efficacy of losartan in preventing aortic aneurysm formation in a mouse model of Marfan syndrome.
    • 86  Jiménez SA, Rosenbloom J: Angiotensin II blockade in Marfan’s syndrome. N. Engl. J. Med.359(16),1732–1733; author reply 1733–1734 (2008).
    • 87  Xiong W, Knispel RA, Dietz HC, Ramirez F, Baxter BT: Doxycycline delays aneurysm rupture in a mouse model of Marfan syndrome. J. Vasc. Surg.47(1),166–172; discussion 172 (2008).
    • 88  Lindeman JHN, Ashcroft BA, Beenakker JM et al.: Distinct defects in collagen microarchitecture underlie vessel-wall failure in advanced abdominal aneurysms and aneurysms in Marfan syndrome. Proc. Natl Acad. Sci. USA107(2),862–865 (2010).
    • 89  Vriend JWJ, de Groot E, de Waal TT et al.: Increased carotid and femoral intima-media thickness in patients after repair of aortic coarctation: influence of early repair. Am. Heart J.151(1),242–247 (2006).
    • 90  Tanous D, Benson LN, Horlick EM: Coarctation of the aorta: evaluation and management. Curr. Opin. Cardiol.24(6),509–515 (2009).
    • 91  Pedra CAC, Fontes VF, Esteves CA et al.: Stenting vs. balloon angioplasty for discrete unoperated coarctation of the aorta in adolescents and adults. Catheter Cardiovasc. Interv.64(4),495–506 (2005).
    • 92  Kenny D, Margey R, Turner MS et al.: Self-expanding and balloon expandable covered stents in the treatment of aortic coarctation with or without aneurysm formation. Catheter Cardiovasc. Interv.72(1),65–71 (2008).
    • 93  Tzifa A, Ewert P, Brzezinska-Rajszys G et al.: Covered Cheatham-platinum stents for aortic coarctation: early and intermediate-term results. J. Am. Coll. Cardiol.47(7),1457–1463 (2006).
    • 94  Svensson LG, Kouchoukos NT, Miller DC et al.: Expert consensus document on the treatment of descending thoracic aortic disease using endovascular stent-grafts. Ann. Thorac. Surg.85(Suppl. 1),S1–S41 (2008).
    • 95  Botta L, Russo V, La Palombara C et al.: Stent graft repair of descending aortic dissection in patients with Marfan syndrome: an effective alternative to open reoperation? J. Thorac. Cardiovasc. Surg.138(5),1108–1114 (2009).
    • 96  Nordon IM, Hinchliffe RJ, Holt PJ et al.: Endovascular management of chronic aortic dissection in patients with Marfan syndrome. J. Vasc. Surg.50(5),987–991 (2009).
    • 97  David TE, Armstrong S, Maganti M, Colman J, Bradley TJ: Long-term results of aortic valve-sparing operations in patients with Marfan syndrome. J. Thorac. Cardiovasc. Surg.138(4),859–864; discussion 863–864 (2009).
    • 98  Emery RW, Erickson CA, Arom KV et al.: Replacement of the aortic valve in patients under 50 years of age: long-term follow-up of the St. Jude Medical prosthesis. Ann. Thorac. Surg.75(6),1815–1819 (2003).
    • 99  Patel ND, Weiss ES, Alejo DE et al.: Aortic root operations for Marfan syndrome: a comparison of the Bentall and valve-sparing procedures. Ann. Thorac. Surg.85(6),2003–2010; discussion 2010–2011 (2008).
    • 100  David TE, Feindel CM, Webb GD et al.: Aortic valve preservation in patients with aortic root aneurysm: results of the reimplantation technique. Ann. Thorac. Surg.83(2),S732–735; discussion S785–S790 (2007).
    • 101  Cameron DE, Alejo DE, Patel ND et al.: Aortic root replacement in 372 Marfan patients: evolution of operative repair over 30 years. Ann. Thorac. Surg.87(5),1344–1349; discussion 1349–1350 (2009).
    • 102  de Kerchove L, Boodhwani M, Etienne P et al.: Preservation of the pulmonary autograft after failure of the Ross procedure. Eur. J. Cardiothorac. Surg.38(3),326–332 (2010).
    • 103  Badiu CC, Eichinger W, Bleiziffer S et al.: Should root replacement with aortic valve-sparing be offered to patients with bicuspid valves or severe aortic regurgitation? Eur. J. Cardiothorac. Surg.38(5),515–522 (2010).
    • 104  Luciani GB, Viscardi F, Pilati M et al.: The Ross-Yacoub procedure for aneurysmal autograft roots: a strategy to preserve autologous pulmonary valves. J. Thorac. Cardiovasc. Surg.139(3),536–542 (2010).
    • 105  Fogelman R, Nykanen D, Smallhorn JF et al.: Endovascular stents in the pulmonary circulation clinical impact on management and medium-term follow-up. Circulation92(4),881–885 (1995).
    • 106  Bergersen L, Lock JE: What is the current option of first choice for treatment of pulmonary arterial stenosis? Cardiol. Young16(4),329–338 (2006).
    • 107  van Gameren M, Witsenburg M, Takkenberg JJM et al.: Early complications of stenting in patients with congenital heart disease: a multicentre study. Eur. Heart J.27(22),2709–2715 (2006).
    • 108  Bökenkamp R, Blom NA, De Wolf D et al.: Intraoperative stenting of pulmonary arteries. Eur. J. Cardiothorac. Surg.27(4),544–547 (2005).
    • 109  Menon SC, Cetta F, Dearani JA et al.: Hybrid intraoperative pulmonary artery stent placement for congenital heart disease. Am. J. Cardiol.102(12),1737–1741 (2008).▪ Promising risk-reducing results of the hybrid procedure in patients with congenital heart disease.
    • 110  Beghetti M, Galie N: Eisenmenger syndrome a clinical perspective in a new therapeutic era of pulmonary arterial hypertension. J. Am. Coll. Cardiol.53(9),733–740 (2009).
    • 111  Galie N, Torbicki A, Barst R et al.: Guidelines on diagnosis and treatment of pulmonary arterial hypertension. The Task Force on Diagnosis and Treatment of Pulmonary Arterial Hypertension of the European Society of Cardiology. Eur. Heart J.25(24),2243–2278 (2004).
    • 112  Galiè N, Rubin L, Hoeper M et al.: Treatment of patients with mildly symptomatic pulmonary arterial hypertension with bosentan (EARLY study): a double-blind, randomised controlled trial. Lancet371(9630),2093–2100 (2008).
    • 113  Vongpatanasin W, Brickner ME, Hillis LD, Lange RA: The Eisenmenger syndrome in adults. Ann. Intern. Med.128(9),745–755 (1998).
    • 114  Duffels MGJ, Vis JC, van Loon RLE et al.: Effect of bosentan on exercise capacity and quality of life in adults with pulmonary arterial hypertension associated with congenital heart disease with and without Down’s syndrome. Am. J. Cardiol.103(9),1309–1315 (2009).
    • 115  Galie N, Beghetti M, Gatzoulis M et al.: Bosentan therapy in patients with Eisenmenger syndrome: a multicenter, double-blind, randomized, placebo-controlled study. Circulation114(1),48–54 (2006).
    • 116  Reinhardt Z, Uzun O, Bhole V et al.: Sildenafil in the management of the failing Fontan Circulation. Cardiol. Young20(5),522–525 (2010).
    • 117  van Loon RLE, Hoendermis ES, Duffels MGJ et al.: Long-term effect of bosentan in adults versus children with pulmonary arterial hypertension associated with systemic-to-pulmonary shunt: does the beneficial effect persist? Am. Heart J.154(4),776–782 (2007).
    • 118  Tay ELW, Papaphylactou M, Diller GP et al.: Quality of life and functional capacity can be improved in patients with Eisenmenger syndrome with oral sildenafil therapy. Int. J. Cardiol. (2010) (Epub ahead of print).
    • 119  Predescu D, Chaturvedi RR, Friedberg MK et al.: Complete heart block associated with device closure of perimembranous ventricular septal defects. J. Thorac. Cardiovasc. Surg.136(5),1223–1228 (2008).
    • 120  Morris C, Reller M, Menashe V: Thirty-year incidence of infective endocarditis after surgery for congenital heart defect. JAMA279(8),599–603 (1998).
    • 121  Gersony W, Hayes C, Driscoll D et al.: Bacterial endocarditis in patients with aortic stenosis, pulmonary stenosis, or ventricular septal defect. Circulation87(Suppl. 2),I121–I126 (1993).
    • 122  Roberts GJ: Dentists are innocent! ‘Everyday’ bacteremia is the real culprit: a review and assessment of the evidence that dental surgical procedures are a principal cause of bacterial endocarditis in children. Pediatr. Cardiol.20(5),317–325 (1999).
    • 123  Li W, Somerville J: Infective endocarditis in the grown-up congenital heart (GUCH) population. Eur. Heart J.19(1),166–173 (1998).
    • 124  Di Fillipo FS, Delahaye F, Semiond B et al.: Current patterns of infective endocarditis in congenital heart disease. Heart92(10),1490–1495 (2006).
    • 125  Habib G, Hoen B, Tornos P et al.: Guidelines on the prevention, diagnosis, and treatment of infective endocarditis (new version 2009): the task force on the prevention, diagnosis, and treatment of infective endocarditis of the European Society of Cardiology (ESC). Eur. Heart J.30(19),2369–2413 (2009).
    • 126  van der Velde ET, Vander VET, Vriend JWJ et al.: CONCOR, an initiative towards a national registry and DNA-bank of patients with congenital heart disease in the Netherlands: rationale, design, and first results. Eur. J. Epidemiol.20(6),549–557 (2005).▪▪ Demonstrates the importance of registries of patients with congenital heart disease to expand knowledge and treatment options.
    • 127  Joziasse IC, van de Smagt JJ, Smith K et al.: Genes in congenital heart disease: atrioventricular valve formation. Basic Res. Cardiol.103(3),216–227 (2008).
    • 128  Smith KA, Joziasse IC, Chocron S et al.: Dominant-negative ALK2 allele associates with congenital heart defects. Circulation119(24),3062–3069 (2009).