Grown-up Congenital Heart Disease (GUCH): Difference between revisions

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=== Case report ===  
=== Case report ===  
=== Introduction ===
=== Introduction ===
[[File:9. coarctatie.PNG|thumb|right|Figure 9. Schematic drawing of the anatomy prenatal (left) and postnatal (right) in coarctation of the aorta. In the normal situation (without coarctation) only 10 percent of the fetal cardiac output flows through the descending aorta. Therefore there are no hemodynamic consequences prenatal of coarctation of the aorta. In the postnatal situation, after closure of the ductus arteriosus, around 75% of cardiac output needs to pass the coarctation, leading to obstruction.]]
[[File:Figure 9. Schematic drawing of the anatomy prenatal and postnatal.png|thumb|right|Figure 9. Schematic drawing of the anatomy prenatal (left) and postnatal (right) in coarctation of the aorta. In the normal situation (without coarctation) only 10 percent of the fetal cardiac output flows through the descending aorta. Therefore there are no hemodynamic consequences prenatal of coarctation of the aorta. In the postnatal situation, after closure of the ductus arteriosus, around 75% of cardiac output needs to pass the coarctation, leading to obstruction.]]
Coarctation of the aorta is a narrowing of the thoracic aorta, typically located in the region of the obliterated ductus arteriosum. (Figure 9) The relation to the position of the left subclavian artery differs, in most patients the left subclavian artery is located anterior of the coarctation. Aortic coarctation is frequently associated with diffuse hypoplasia of the aortic arch and isthmus.  
Coarctation of the aorta is a narrowing of the thoracic aorta, typically located in the region of the obliterated ductus arteriosum. (Figure 9) The relation to the position of the left subclavian artery differs, in most patients the left subclavian artery is located anterior of the coarctation. Aortic coarctation is frequently associated with diffuse hypoplasia of the aortic arch and isthmus.  


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=== Treatment and outcome ===
=== Treatment and outcome ===
[[File:10._coarctatie_repair.PNG|thumb|left|Figure 10. Schematic drawing showing surgical procedures for repair of coarctation of the aorta. Left: resection with end-to-end anastomosis. Middle: dilating technique using a patch; this technique is used in coarctations involving a long segment of the aorta. Right: the subclavian flap aortoplasty, using the left subclavian artery.]]
[[File:Figure 10. Schematic drawing showing surgical procedures for repair of coarctation of the aorta.png|thumb|left|Figure 10. Schematic drawing showing surgical procedures for repair of coarctation of the aorta. Left: resection with end-to-end anastomosis. Middle: dilating technique using a patch; this technique is used in coarctations involving a long segment of the aorta. Right: the subclavian flap aortoplasty, using the left subclavian artery.]]
[[File:11. coarctatie repair2.PNG|thumb|right|Figure 11. Schematic drawing showing surgical procedures for repair of a coarctation of the aorta. Left: an interposition graft. Middle: the extended aortic arch repair. Right: the extra-anatomical bypass.]]
[[File:Figure 11. Schematic drawing showing surgical procedures for repair of a coarctation of the aorta.png|thumb|right|Figure 11. Schematic drawing showing surgical procedures for repair of a coarctation of the aorta. Left: an interposition graft. Middle: the extended aortic arch repair. Right: the extra-anatomical bypass.]]
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=== Introduction ===
=== Introduction ===
[[File:12. TGA.jpg|thumb|left|Figure 12: Schematic drawing showing transposition of the great arteries. The pulmonary artery is located above the left ventricle (LV) and the aorta is located above the right ventricle (RV).]]
[[File:12. TGA.jpg|thumb|left|Figure 12: Schematic drawing showing transposition of the great arteries. The pulmonary artery is located above the left ventricle (LV) and the aorta is located above the right ventricle (RV).]]
[[File:13. TGA.PNG|thumb|right|Figure 13. Schematic drawing of the circulation in transposition of the great arteries. Left: normal position of the great arteries with the pulmonary and systemic circulation serially connected. Right: transposition of the great arteries with a parallel circulation.]]
[[File:Figure 13. Schematic drawing of the circulation in transposition of the great arteries.png|thumb|right|Figure 13. Schematic drawing of the circulation in transposition of the great arteries. Left: normal position of the great arteries with the pulmonary and systemic circulation serially connected. Right: transposition of the great arteries with a parallel circulation.]]
Transposition of the great arteries (TGA) accounts for 5-8% of all congenital heart defects and occurs 2-3 times more frequently in males. TGA is best defined as a normal atrioventricular connection with an abnormal ventricular–arterial connection; the morphological left atrium is connected through the left ventricle with the pulmonary artery and the morphological right atrium through the right ventricle with the aorta. (Figure 12)The aorta is often located on the right side and in front of the pulmonary artery (D-TGA). In 70 percent there is an isolated form of TGA, in 30 percent the TGA is accompanied by other heart defects, like VSD or obstruction of the left ventricle outflow tract.
Transposition of the great arteries (TGA) accounts for 5-8% of all congenital heart defects and occurs 2-3 times more frequently in males. TGA is best defined as a normal atrioventricular connection with an abnormal ventricular–arterial connection; the morphological left atrium is connected through the left ventricle with the pulmonary artery and the morphological right atrium through the right ventricle with the aorta. (Figure 12)The aorta is often located on the right side and in front of the pulmonary artery (D-TGA). In 70 percent there is an isolated form of TGA, in 30 percent the TGA is accompanied by other heart defects, like VSD or obstruction of the left ventricle outflow tract.


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=== Congenitally corrected transposition of the great arteries ===
=== Congenitally corrected transposition of the great arteries ===
=== Introduction ===
=== Introduction ===
[[File:14. ccTGA.PNG|thumb|right|Figure 14. Congenitally corrected transposition of the great arteries. RA, right atrium. LA, left atrium. RV, right ventricle. LV, left ventricle. p, pulmonary artery. ao, aorta. tric, tricuspid valve.]]
[[File:Figure 14. Congenitally corrected transposition of the great arteries.png|thumb|right|Figure 14. Congenitally corrected transposition of the great arteries. RA, right atrium. LA, left atrium. RV, right ventricle. LV, left ventricle. p, pulmonary artery. ao, aorta. tric, tricuspid valve.]]
The congenitally corrected transposition of the great arteries (ccTGA) is characterized by a normal anatomical position of both atria, with an abnormal connection between the atria and the ventricles. The right atrium is connected with the left ventricle and the left atrium is connected with the right ventricle. (Figure 14) Furthermore the aorta arises from the right ventricle and the pulmonary artery from the left ventricle. There are, in conclusion, abnormal atrioventricular connections and abnormal ventricular-arterial connections present in ccTGA.  
The congenitally corrected transposition of the great arteries (ccTGA) is characterized by a normal anatomical position of both atria, with an abnormal connection between the atria and the ventricles. The right atrium is connected with the left ventricle and the left atrium is connected with the right ventricle. (Figure 14) Furthermore the aorta arises from the right ventricle and the pulmonary artery from the left ventricle. There are, in conclusion, abnormal atrioventricular connections and abnormal ventricular-arterial connections present in ccTGA.  
CcTGA is a very rare defect, accounting for about 1% of all congenital heart disease.
CcTGA is a very rare defect, accounting for about 1% of all congenital heart disease.
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=== Case report ===
=== Case report ===
=== Introduction ===
=== Introduction ===
[[File:20. Wilhelm Ebstein.jpg|thumb|right|Figure 20. Wilhelm Ebstein (1836 – 1912).]]
[[File:20. Wilhelm Ebstein.jpg|thumb|left|Figure 20. Wilhelm Ebstein (1836 – 1912).]]
[[File:Figure 21. Schematic drawing showing Ebstein’s anomaly of the tricuspid valve.png|thumb|right|Figure 21. Schematic drawing showing Ebstein’s anomaly of the tricuspid valve. Left: normal heart with openend right ventricle. Right: Ebstein’s anomaly with displacement of the septal and posterior tricuspid leaflet, leading to atrialisation of a significant part of the right ventricle.]]
Ebsteins anomaly, named after Wilhelm Ebstein (1836 – 1912) (Figure 20) is a congenital heart defect of the morphological tricuspid valve. The prevalence of Ebstein's anomaly is about 1 in 50.000 – 200.000 with a similar incidence in both males and females.
Ebsteins anomaly, named after Wilhelm Ebstein (1836 – 1912) (Figure 20) is a congenital heart defect of the morphological tricuspid valve. The prevalence of Ebstein's anomaly is about 1 in 50.000 – 200.000 with a similar incidence in both males and females.


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=== Pathophysiology ===
=== Pathophysiology ===
 
[[File:22. Eisenmenger.jpg|thumb|right|Figure 22. Photo showing typical features of chronic hypoxemia in Eisenmenger syndrome, with typical digital clubbing with cyanotic nail beds.]]


The pathogenesis of PH is complex and just beginning to be elucidated. In patients with congenital heart disease, left-to-right intracardiac shunting increases flow through the pulmonary vasculature, this causes shear forces that disrupt the vascular endothelium and activate cellular mechanisms critical to the pathogenesis and progression of PAH.
The pathogenesis of PH is complex and just beginning to be elucidated. In patients with congenital heart disease, left-to-right intracardiac shunting increases flow through the pulmonary vasculature, this causes shear forces that disrupt the vascular endothelium and activate cellular mechanisms critical to the pathogenesis and progression of PAH.
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Therapy improves exercise capacity and functional class, however the impact on mortality has been less well established.
Therapy improves exercise capacity and functional class, however the impact on mortality has been less well established.
== References ==
<biblio>
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#Basow2012c Basow, D. S. (2012c). Management and outcome of isolated atrial septal defects in children. UpToDate. Waltham, MA.: UpToDate.
#Basow2012d Basow, D. S. (2012d). Pathophysiology and clinical features of atrial septal defects in adults. UpToDate. Waltham, MA.: UpToDate.
#Basow2012e Basow, D. S. (2012e). Identification and assessment of atrial septal defects in adults. UpToDate. Waltham, MA.: UpToDate.
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#Roos-Hesselink Roos-Hesselink, J. W., Meijboom, F. J., Spitaels, S. E. C., van Domburg, R., van Rijen, E. H. M., Utens, E. M. W. J., Bogers, A. J. J. C., e.a. (2003). Excellent survival and low incidence of arrhythmias, stroke and heart failure long-term after surgical ASD closure at young age. A prospective follow-up study of 21-33 years. European Heart Journal, 24(2), 190-197.
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#Luijendijk2 Luijendijk, Paul, Bouma, B. J., Vriend, J. W. J., Vliegen, H. W., Groenink, M., & Mulder, B. J. M. (2011). Usefulness of exercise-induced hypertension as predictor of chronic hypertension in adults after operative therapy for aortic isthmic coarctation in childhood. The American Journal of Cardiology, 108(3), 435-439.
#Mulder4 Mulder, B.J.M., Pieper, P. G., Meijboom, F. J., & Hamer, J. P. M. (2006). Coarctation of the aorta. Adult Congenital Heart Disease (Aangeboren hartafwijkingen bij volwassenen) (Second edition.). Houten: Bohn Stafleu van Loghum.
#Vriend1 Vriend, J. W. J., & Mulder, B. J. M. (2005). Late complications in patients after repair of aortic coarctation: implications for management. International Journal of Cardiology, 101(3), 399-406.
#Vriend2 Vriend, J. W. J., Oosterhof, T., & Mulder, B. (2005). Noninvasive imaging for the postoperative assessment of aortic coarctation patients. Chest, 127(6), 2295.
#Drenthen Drenthen, W., Pieper, P. G., Ploeg, M., Voors, A. A., Roos-Hesselink, J. W., Mulder, B. J. M., Vliegen, H. W., e.a. (2005). Risk of complications during pregnancy after Senning or Mustard (atrial) repair of complete transposition of the great arteries. European Heart Journal, 26(23), 2588-2595. doi:10.1093/eurheartj/ehi472
#Mulder5 Mulder, B.J.M., Pieper, P. G., Meijboom, F. J., & Hamer, J. P. M. (2006). Transposition of the great arteries. Adult Congenital Heart Disease (Aangeboren hartafwijkingen bij volwassenen) (Second edition.). Houten: Bohn Stafleu van Loghum.
#vanderZedde van der Zedde, J., Oosterhof, T., Tulevski, I. I., Vliegen, H. W., & Mulder, B. J. M. (2005). Comparison of segmental and global systemic ventricular function at rest and during dobutamine stress between patients with transposition and congenitally corrected transposition. Cardiology in the Young, 15(2), 148-153. doi:10.1017/S1047951105000326
#Mulder6 Mulder, B.J.M., Pieper, P. G., Meijboom, F. J., & Hamer, J. P. M. (2006). Congenitally corrected transposition of the great arteries. Adult Congenital Heart Disease (Aangeboren hartafwijkingen bij volwassenen) (Second edition.). Houten: Bohn Stafleu van Loghum.
#Winlaw Winlaw, D. S., McGuirk, S. P., Balmer, C., Langley, S. M., Griselli, M., Stümper, O., De Giovanni, J. V., e.a. (2005). Intention-to-treat analysis of pulmonary artery banding in conditions with a morphological right ventricle in the systemic circulation with a view to anatomic biventricular repair. Circulation, 111(4), 405-411.
#Winter1 Winter, M. M., Bouma, B. J., van Dijk, A. P. J., Groenink, M., Nieuwkerk, P. T., van der Plas, M. N., Sieswerda, G. T., e.a. (2008). Relation of physical activity, cardiac function, exercise capacity, and quality of life in patients with a systemic right ventricle. The American Journal of Cardiology, 102(9), 1258-1262.
#Winter2 Winter, M. M., van der Bom, T., de Vries, L. C. S., Balducci, A., Bouma, B. J., Pieper, P. G., van Dijk, A. P. J., e.a. (2011). Exercise training improves exercise capacity in adult patients with a systemic right ventricle: a randomized clinical trial. European Heart Journal.
#Winter3 Winter, M. M., van der Plas, M. N., Bouma, B. J., Groenink, M., Bresser, P., & Mulder, B. J. M. (2010). Mechanisms for cardiac output augmentation in patients with a systemic right ventricle. International Journal of Cardiology, 143(2), 141-146.
#Basow2012 Basow, D. S. (2012). Hypoplastic left heart syndrome. UpToDate. Waltham, MA.: UpToDate.
#Fontan Basow, D. S. (2012). Hypoplastic left heart syndrome. UpToDate. Waltham, MA.: UpToDate.
#Mulder7 Mulder, B.J.M., Pieper, P. G., Meijboom, F. J., & Hamer, J. P. M. (2006). Univentricular heart and the Fontan circulation. Adult Congenital Heart Disease (Aangeboren hartafwijkingen bij volwassenen) (Second edition.). Houten: Bohn Stafleu van Loghum.
#Schuuring Schuuring, M. J., Vis, J. C., Bouma, B. J., van Dijk, A. P. J., van Melle, J. P., Pieper, P. G., Vliegen, H. W., e.a. (2011). Rationale and design of a trial on the role of bosentan in Fontan patients: Improvement of exercise capacity? Contemporary Clinical Trials, 32(4), 586-591.
#vandenBosch van den Bosch, A. E., Roos-Hesselink, J. W., Van Domburg, R., Bogers, A. J. J. C., Simoons, M. L., & Meijboom, F. J. (2004). Long-term outcome and quality of life in adult patients after the Fontan operation. The American Journal of Cardiology, 93(9), 1141-1145.
#Balci Balci, A., Drenthen, W., Mulder, B. J. M., Roos-Hesselink, J. W., Voors, A. A., Vliegen, H. W., Moons, P., e.a. (2011). Pregnancy in women with corrected tetralogy of Fallot: occurrence and predictors of adverse events. American Heart Journal, 161(2), 307-313.
#Lillehei Lillehei, C. W., Cohen, M., Warden, H. E., Read, R. C., Aust, J. B., Dewall, R. A., & Varco, R. L. (1955). Direct vision intracardiac surgical correction of the tetralogy of Fallot, pentalogy of Fallot, and pulmonary atresia defects; report of first ten cases. Annals of Surgery, 142(3), 418-442.
#Mulder8 Mulder, B.J.M., Pieper, P. G., Meijboom, F. J., & Hamer, J. P. M. (2006). Tetralogy of Fallot. Adult Congenital Heart Disease (Aangeboren hartafwijkingen bij volwassenen) (Second edition.). Houten: Bohn Stafleu van Loghum.
#Mulder9 Mulder, Barbara J M, & van der Wall, E. E. (2009). Tetralogy of Fallot: in good shape? The International Journal of Cardiovascular Imaging, 25(3), 271-275.
#Oosterhof Oosterhof, T., Mulder, B. J. M., Vliegen, H. W., & de Roos, A. (2006). Cardiovascular magnetic resonance in the follow-up of patients with corrected tetralogy of Fallot: a review. American Heart Journal, 151(2), 265-272.
#Vliegen Vliegen, H. W., van Straten, A., de Roos, A., Roest, A. A. W., Schoof, P. H., Zwinderman, A. H., Ottenkamp, J., e.a. (2002). Magnetic resonance imaging to assess the hemodynamic effects of pulmonary valve replacement in adults late after repair of tetralogy of fallot. Circulation, 106(13), 1703-1707.
#Windhausen Windhausen, F., Boekholdt, S. M., Bouma, B. J., Groenink, M., Backx, A. P. C. M., de Winter, R. J., Mulder, B. J. M., e.a. (2011). Per-operative stent placement in the right pulmonary artery; a hybrid technique for the management of pulmonary artery branch stenosis at the time of pulmonary valve replacement in adult Fallot patients. Netherlands Heart Journal: Monthly Journal of the Netherlands Society of Cardiology and the Netherlands Heart Foundation, 19(10), 432-435.
#deWitte de Witte, Piet, Aalberts, J. J. J., Radonic, T., Timmermans, J., Scholte, A. J., Zwinderman, A. H., Mulder, B. J. M., e.a. (2011). Intrinsic biventricular dysfunction in Marfan syndrome. Heart (British Cardiac Society), 97(24), 2063-2068.
#Engelfriet2 Engelfriet, P., & Mulder, B. (2007). Is there benefit of beta-blocking agents in the treatment of patients with the Marfan syndrome? International Journal of Cardiology, 114(3), 300-302.
#Mulder9 Mulder, B.J.M., Pieper, P. G., Meijboom, F. J., & Hamer, J. P. M. (2006). Marfan’s syndrome. Adult Congenital Heart Disease (Aangeboren hartafwijkingen bij volwassenen) (Second edition.). Houten: Bohn Stafleu van Loghum.
#Radonic Radonic, T, de Witte, P., Groenink, M., de Bruin-Bon, R., Timmermans, J., Scholte, A., van den Berg, M., e.a. (2011). Critical appraisal of the revised Ghent criteria for diagnosis of Marfan syndrome. Clinical Genetics.
#Basow20122 Basow, D. S. (2012). Ebstein’s anomaly of the tricuspid valve. UpToDate. Waltham, MA.: UpToDate.
#Carpentier Carpentier, A., Chauvaud, S., Macé, L., Relland, J., Mihaileanu, S., Marino, J. P., Abry, B., e.a. (1988). A new reconstructive operation for Ebstein’s anomaly of the tricuspid valve. The Journal of Thoracic and Cardiovascular Surgery, 96(1), 92-101.
#Celermajer Celermajer, D. S., Bull, C., Till, J. A., Cullen, S., Vassillikos, V. P., Sullivan, I. D., Allan, L., e.a. (1994). Ebstein’s anomaly: presentation and outcome from fetus to adult. Journal of the American College of Cardiology, 23(1), 170-176.
#Ebstein Ebstein W. (1866) Ueber einen sehr seltenen Fall von Insufficienz der Valvula tricuspidalis, bedingt durch eine angeborene hochgradige Missbildung. Arch Anat physiol;33:238.
#Mulder10 Mulder, B. J. M. (2002). Ebstein’s anomaly in adults. The International Journal of Cardiovascular Imaging, 18(6), 439-441.
#Mulder11 Mulder, B. J. M., Pieper, P. G., Meijboom, F. J., & Hamer, J. P. M. (2006). Ebstein anomaly of the tricuspid valve. Adult Congenital Heart Disease (Aangeboren hartafwijkingen bij volwassenen) (Second edition.). Houten: Bohn Stafleu van Loghum.
#Beghetti1 Beghetti, Maurice, & Tissot, C. (2009). Pulmonary arterial hypertension in congenital heart diseases. Seminars in Respiratory and Critical Care Medicine, 30(4), 421-428.
#Beghetti2 Beghetti, Maurice, & Tissot, C. (2010). Pulmonary hypertension in congenital shunts. Revista Española De Cardiología, 63(10), 1179-1193.
#Diller Duffels, M G J, Engelfriet, P. M., Berger, R. M. F., van Loon, R. L. E., Hoendermis, E., Vriend, J. W. J., van der Velde, E. T., e.a. (2007). Pulmonary arterial hypertension in congenital heart disease: an epidemiologic perspective from a Dutch registry. International Journal of Cardiology, 120(2), 198-204.
#Engelfriet3 Engelfriet, Peter M, Duffels, M. G. J., Möller, T., Boersma, E., Tijssen, J. G. P., Thaulow, E., Gatzoulis, M. A., e.a. (2007). Pulmonary arterial hypertension in adults born with a heart septal defect: the Euro Heart Survey on adult congenital heart disease. Heart (British Cardiac Society), 93(6), 682-687.
#Galie Galie, N., Hoeper, M. M., Humbert, M., Torbicki, A., Vachiery, J.-L., Barbera, J. A., Beghetti, M., e.a. (2009). Guidelines for the diagnosis and treatment of pulmonary hypertension: The Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS), endorsed by the International Society of Heart and Lung Transplantation (ISHLT). European Heart Journal, 30, 2493-2537.
#Gatzoulis Gatzoulis, M A, Alonso-Gonzalez, R., & Beghetti, M. (2009). Pulmonary arterial hypertension in paediatric and adult patients with congenital heart disease. European Respiratory Review: An Official Journal of the European Respiratory Society, 18(113), 154-161.
#Lau Lau, E. M. T., Manes, A., Celermajer, D. S., & Galiè, N. (2011). Early detection of pulmonary vascular disease in pulmonary arterial hypertension: time to move forward. European Heart Journal, 32(20), 2489-2498.
#Mulder12 Mulder, B J M. (2010). Changing demographics of pulmonary arterial hypertension in congenital heart disease. European Respiratory Review: An Official Journal of the European Respiratory Society, 19(118), 308-313. doi:10.1183/09059180.00007910
#Mulder13 Mulder, B.J.M., Pieper, P. G., Meijboom, F. J., & Hamer, J. P. M. (2006). Eisenmenger syndrome. Adult Congenital Heart Disease (Aangeboren hartafwijkingen bij volwassenen) (Second edition.). Houten: Bohn Stafleu van Loghum.
#Schuuring2 Schuuring, M J, van Riel, A. C. M. J., Bouma, B. J., & Mulder, B. J. M. (2011). Recent progress in treatment of pulmonary arterial hypertension due to congenital heart disease. Netherlands Heart Journal: Monthly Journal of the Netherlands Society of Cardiology and the Netherlands Heart Foundation, 19(12), 495-497.
#Schuuring Schuuring, Mark J, Vis, J. C., Duffels, M. G., Bouma, B. J., & Mulder, B. J. (2010). Adult patients with pulmonary arterial hypertension due to congenital heart disease: a review on advanced medical treatment with bosentan. Therapeutics and Clinical Risk Management, 6, 359-366.
#Simonneau Simonneau, G., Robbins, I. M., Beghetti, M., Channick, R. N., Delcroix, M., Denton, C. P., Elliott, C. G., e.a. (2009). Updated clinical classification of pulmonary hypertension. Journal of the American College of Cardiology, 54(1 Suppl), S43-54.
#Vis Vis, J. C., Duffels, M. G., Mulder, P., de Bruin-Bon, R. H. A. C. M., Bouma, B. J., Berger, R. M. F., Hoendermis, E. S., e.a. (2011). Prolonged beneficial effect of bosentan treatment and 4-year survival rates in adult patients with pulmonary arterial hypertension associated with congenital heart disease. International Journal of Cardiology.
</biblio>
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