Pulmonary hypertension can be present in TOF patients due to: hypoplastic pulmonary arteries with associated high vascular resistance, excessive shunting across the surgically constructed shunts (mainly Potts or Waterston shunts) or presence of multiple pulmonary artery stenosis. https://www.textbookofcardiology.org/wiki/Grown-up_Congenital_Heart_Disease_(GUCH) 23/38 7/4/23, 12:41 AM Grown-up Congenital Heart Disease (GUCH) - Textbook of Cardiology A residual VSD shunt is present in about 20 percent of all operated TOF patients, requiring a reoperation in 5 10 percent of them. Residual shunt might be due to detachment of the patch or an additional septal defect which was not recognized during surgery. Marfan syndrome Case report Introduction Figure 19. Magnetic resonance imaging of the aorta, showing aortic root dilatation in Marfan syndrome. Marfan syndrome (MFS) is an autosomal dominant condition with a reported incidence of 1 in 3000 to 5000 individuals and is one of the most common inherited disorders of connective tissue. While most MFS patients have an affected parent, around 15 30 percent have a de novo mutation. MFS is associated with a broad range of clinical symptoms and associated disorders, ranging from classic ocular, cardiovascular, and musculoskeletal abnormalities to manifestations including involvement of the lung, skin, and central nervous system. Figure 18. Echocardiographic image of aortic root dilatation in Marfan syndrome. Progressive dilatation of the ascending aorta is one of the key features, which causes a high risk of sudden death due to aortic dissection or rupture in young Marfan patients. (Figure 18 & 19) The underlying genetic defect is localised in the fibrillin gene on chromosome 15 (FBN1) in which recently around 600 different mutations are found. However in about 10% of MFS patients there is no mutation identified in the FBN1 gene, furthermore FBN1 mutations also occur across a wide range of milder phenotypes that overlap the classic Marfan phenotype. Therefore it is not possible to diagnose MFS solely with genetic information. Criteria for diagnosis of Marfan syndrome The 2010 revised Ghent criteria puts greater weight on aortic root aneurysm/dissection and ectopia lentis as the cardinal clinical features of MFS and on testing for mutations in FBN1 and other relevant genes. https://www.textbookofcardiology.org/wiki/Grown-up_Congenital_Heart_Disease_(GUCH) 24/38 7/4/23, 12:41 AM Grown-up Congenital Heart Disease (GUCH) - Textbook of Cardiology In the absence of family history of MFS, the presence of one of any of the following criteria is diagnostic for MFS: Aortic criterion (aortic diameter Z 2 or aortic root dissection) and ectopia lentis* Aortic criterion (aortic diameter Z 2 or aortic root dissection) and a causal FBN1 mutation Aortic criterion (aortic diameter Z 2 or aortic root dissection) and a systemic score 7 Ectopia lentis and a causal FBN1 mutation as defined above that has been identified in an individual with aortic aneurysm In the presence of family history of MFS (as defined by the above criteria), the presence of one of any of the following criteria is diagnostic for MFS: Ectopia lentis Systemic score 7 points* Aortic criterion (aortic diameter Z 2 above 20 years old, Z 3 below 20 years, or aortic root dissection)* For criteria with an asterisk (*), the diagnosis of MFS can be made only in the absence of discriminating features of Shprintzen-Goldberg syndrome (SGS), Loeys-Dietz syndrome (LDS), or vascular Ehlers- Danlos syndrome (vEDS) and after TGFBR1/2, collagen biochemistry, or COL3A1 testing if indicated. The revised Ghent nosology includes the following scoring system for systemic features: Wrist AND thumb sign: 3 points (wrist OR thumb sign: 1 point) Pectus carinatum deformity: 2 (pectus excavatum or chest asymmetry: 1 point) Hindfoot deformity: 2 points (plain pes planus:1 point) Pneumothorax: 2 points Dural ectasia: 2 points Protrusio acetabuli: 2 points Reduced upper segment/lower segment ratio (US/LS) AND increased arm span/height AND no severe scoliosis: 1 point Scoliosis or thoracolumbar kyphosis: 1 point Reduced elbow extension ( 170 degrees with full extension): 1 point Facial features (at least 3 of the following 5 features: dolichocephaly [reduced cephalic index or head width/length ratio], enophthalmos, downslanting palpebral fissures, malar hypoplasia, retrognathia): 1 point Skin striae: 1 point Myopia >3 diopters: 1 point Mitral valve prolapse (all types): 1 point A systemic score 7 indicates systemic involvement. Pathophysiology Aortic root disease, leading to aneurysmal dilatation, aortic regurgitation, and dissection, is the main cause of morbidity and mortality in the MFS. Dilatation of the aorta is found in approximately 50 percent of children with MFS and progresses with time. Approximately 60 to 80 percent of adult https://www.textbookofcardiology.org/wiki/Grown-up_Congenital_Heart_Disease_(GUCH) 25/38 7/4/23, 12:41 AM Grown-up Congenital Heart Disease (GUCH) - Textbook of Cardiology patients with MFS have dilatation of the aortic root (with normal range adjusted for patient body surface area and age) by echocardiography, often accompanied by aortic regurgitation. Dilatation may also involve other segments of the thoracic aorta, the abdominal aorta, the root of the pulmonary artery or even the carotid and intracranial arteries. Untreated MFS is frequently associated with aortic dissection, which begins just above the coronary ostia and extends the entire length of the aorta; it is a type I dissection in the DeBakey classification or a type A in the Dailey scheme. Approximately 10 percent of dissections begin distal to the left subclavian (type III or type B) but dissection is rarely limited to just the abdominal aorta. Many patients with MFS and aortic dissection have a family history of dissection. Mitral valve prolapse (MVP) is frequently identified in patients with MFS. However, only one point in the systemic score is assigned for MVP since it is a nonspecific feature and most patients with mitral valve prolapse do not have MFS. The frequency of MVP in MFS increases with age and is greater in women. Tricuspid valve prolapse may also occur. Treatment Beta blockers decrease myocardial contractility and may also improve the elastic properties of the aorta, particularly in patients with an aortic root diameter <40 mm thereby decreasing the risk of aortic dissection and delaying the aortic dilatation. Prophylactic treatment with beta blockers is considered the standard of care in adults with MFS. Furthermore patients with MFS are advised to avoid any contact sports, exercise at maximal capacity, and isometric activities. The exact aortic root diameter at which elective surgery should be performed is uncertain. The current guidelines recommend elective operation for patients with MFS at an external diameter of 50 mm to avoid acute dissection or rupture. Indications for repair at an external diameter less than 50 mm include rapid growth (>2 mm/y), family history of aortic dissection at a diameter less than 50 mm, desire of pregnancy or presence of progressive aortic or mitral valve regurgitation. However one must take into account that a predicted aortic root diameter varies with body size and age and may be smaller in women. Smaller patients have dissection at a smaller aortic root size and 15 percent of patients with MFS have dissection at a diameter less than 50 mm. The classic aortic root surgery is the Bentall procedure in which the ascending aorta is replaced, together with the aortic valve, by a graft with prosthetic valve. In this procedure the coronary arteries need to be reimplanted in the aortic graft. In patients with anatomically normal valves, in whom the insuf ciency is due to the dilated annulus or dissection, valve-sparing operations with root replacement by a Dacron prosthesis and with reimplantation of the coronary arteries into the prosthesis (David s procedure) or remodelling of the aortic root (Yacoub s procedure) have now become the preferred surgical procedures. Aortic regurgitation is, however, a common complication, requiring reoperation in 20% of patients after 10 years. Outcome The reported operative mortality of the Bentall procedure is 1.5% for elective and 11.7% for emergency operations. Five- and 10-year survival rates of 84 and 75%, respectively, have been reported. This relatively limited prognosis is due to late sequelae associated with MFS; 75% require reoperations, 10% mitral valve surgery, 1% develop endocarditis and 1% a CVA. Marfan syndrome has also been associated https://www.textbookofcardiology.org/wiki/Grown-up_Congenital_Heart_Disease_(GUCH) 26/38 7/4/23, 12:41 AM Grown-up Congenital Heart Disease (GUCH) - Textbook of Cardiology with a considerably higher risk of re-dissection and recurrent aneurysm than other aetiologies of aortic disease. Long-term results of valvesparing aortic root replacement in Marfan syndrome are still unknown. Ebsteins anomaly Case report Introduction 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 tricuspid valve. The prevalence of morphological Figure 20. Wilhelm Ebstein (1836 1912). Ebstein's anomaly is about 1 in 50.000 200.000 with a similar incidence in both males and females. As its name clearly indicates, the tricuspid valve consists of three leaflets; anterosuperior, septal and inferior. The Ebstein s anomaly consists of a variety of anatomical and functional abnormalities of the tricuspid valve. Typical features are: displacement of the septal and inferior leaflet downwards from the atrioventricular junction and toward the body of the right ventricle or the apex the anterior leaflet is not displaced, enlarged (sail-like) and can show fenestrations the tricuspid valve inlet is displaced towards the right ventricular outflow tract and often stenotic atrialisation of the right ventricle because of a downward extension of the tricuspid valve, leaving a small right ventricle or only a right ventricular outflow tract Pathophysiology https://www.textbookofcardiology.org/wiki/Grown-up_Congenital_Heart_Disease_(GUCH) 27/38 7/4/23, 12:41 AM Grown-up Congenital Heart Disease (GUCH) - Textbook of Cardiology The Ebstein s anomaly can be isolated or associated with other cardiac defects like ASD or patent foramen ovale, pulmonary outflow tract obstruction, VSD, coarctation of the aorta, ccTGA, one or more accessory conduction pathways or patent ductus arteriosus. The primary hemodynamic consequence of Ebstein s anomaly is tricuspid regurgitation (TR), which varies in severity. Severe TR causes a volume overload and right-sided cardiac chamber dilation and dysfunction, leading to a decrease in cardiac output. In some cases even hepatic congestion and failure can arise. Ebstein's anomaly can be classified as mild, moderate, or severe based upon the extent of apical displacement of the valve leaflets with resultant tricuspid regurgitation, and the degree of right ventricular dysfunction. Symptoms such as cyanosis and heart failure from severe TR may appear soon after birth, because of high pulmonary vascular resistance. However, symptoms often improve as pulmonary vascular resistance decreases. At a later age, symptoms such as exertional dyspnea, fatigue, cyanosis, and palpitations may recur; these symptoms may be insidious in onset. Palpitations due to atrial tachyarrhythmia are present in 20 to 30 percent of cases. Some of these arrhythmias may be due to Wolff-Parkinson-White syndrome, since up to 20 percent of patients have one or more accessory pathways; the majority of these pathways are located around the orifice of the malformed tricuspid valve. Patients with Ebstein's anomaly who have an interatrial communication are at risk for paradoxical embolization, brain abscesses, and sudden death. Treatment Indications for surgical tricuspid valve repair or replacement are: decrease in exercise capacity progressive cyanosis progressive right ventricular dilatation or dysfunction occurrence of paradoxal embolism supraventricular arrhythmias despite pharmacological or ablation therapy progressive cardiomegaly on chest radiograph There are two main surgical techniques used in tricuspid valve repair. The Danielson repair consists of horizontally plicating the atrialized portion of the RV while multiple commissuroplasty stitches are placed. The Carpentier technique consists of detaching the large sail-like anterior leaflet from the valve annulus and translocating it in a clockwise division in order to create what essentially becomes a single leaflet valve. The atrialized portion of the RV is plicated but in a vertical not a horizontal fashion. If repair is not possible and the patient has reached adult size, tricuspid valve replacement becomes necessary. A biologic prosthesis, such as a porcine valve, is usually chosen because of the high incidence of thromboembolism with a mechanical prosthesis placed in the right heart. Outcome The prognosis varies with the severity of the disease. The 1 and 10 year survival rates for all liveborn patients have been estimated 67 percent and 59 percent respectively. The major causes of death are heart failure, perioperative, and sudden death. https://www.textbookofcardiology.org/wiki/Grown-up_Congenital_Heart_Disease_(GUCH) 28/38 7/4/23, 12:41 AM Grown-up Congenital Heart Disease (GUCH) - Textbook of Cardiology However, survival is probably increasing as advances in diagnostic and surgical techniques and postoperative care have led to improvements in surgical outcome. Pulmonary hypertension Case report Introduction Pulmonary hypertension (PH) is a progressive life-threatening condition and associated with a high rate of mortality, despite medical intervention. PH is characterized by elevated pulmonary arterial pressure and secondary right ventricular failure. The definition of pulmonary hypertension (PH) is based upon right heart catheterization where PH is defined as a mean pulmonary artery pressure greater than 25 mmHg at rest. Classification PH is classified into five groups: 1. Pulmonary arterial hypertension (PAH). This group consists of idiopathic PAH and PAH due to connective tissue diseases, HIV infection, portal hypertension, congenital heart disease, schistosomiasis, chronic hemolytic anemia, persistent pulmonary hypertension of the newborn, pulmonary veno-occlusive disease, drug- and toxin-induced PH and pulmonary capillary hemangiomatosis. 2. Pulmonary hypertension owing to left heart disease. PH due to systolic dysfunction, diastolic dysfunction, or valvular heart disease is included in this group. 3. Pulmonary hypertension owing to lung diseases or hypoxemia. This group includes PH due to chronic obstructive pulmonary disease, interstitial lung disease, other pulmonary diseases with a mixed restrictive and obstructive pattern, sleep-disordered breathing, alveolar hypoventilation disorders, and other causes of hypoxemia [4]. 4. Chronic thromboembolic pulmonary hypertension. This group includes patients with PH due to thromboembolic occlusion of the proximal or distal pulmonary vasculature. 5. Pulmonary hypertension with unclear multifactorial mechanisms. These patients have PH caused by hematologic disorders (eg, myeloproliferative disorders), systemic disorders (eg, sarcoidosis), metabolic disorders (eg, glycogen storage disease), or miscellaneous causes Pulmonary arterial hypertension in congenital heart disease In approximately 6% of adults with congenital heart disease PAH develops. In fact congenital heart disease has emerged to be one of the commonest associated causes of PAH. https://www.textbookofcardiology.org/wiki/Grown-up_Congenital_Heart_Disease_(GUCH) 29/38 7/4/23, 12:41 AM Grown-up Congenital Heart Disease (GUCH) - Textbook of Cardiology The occurrence of PAH in congenital heart disease is usually the result of systemic-to-pulmonary shunting, leading to a blood volume overload of the pulmonary vasculature. Left to right shunts are most common; VSD, ASD, patent ductus arteriosus or AVSD. Pathophysiology 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 mechanism of damage to the pulmonary vasculature differs in patients with ASD compared to VSD. Vascular injury is related to the degree and duration of volume overload alone with an ASD, whereas high pressure shear forces also contribute with a VSD. Figure 22. Photo showing typical features of chronic hypoxemia in Eisenmenger syndrome, with typical digital clubbing with cyanotic nail beds. In patients with an ASD, shunting is delayed until maturation of the pulmonary vasculature occurs. The normal pulmonary vasculature is able to accommodate the increased volume of flow by vasodilating and recruiting previously unperfused vessels; thus, pulmonary artery pressures do not rise significantly in most patients with an ASD until adult life. In contrast to patients with an ASD, clinical sequelae always develop in patients with a large (nonrestrictive) VSD. Severe PAH is present from birth because of the unique hemodynamics. The combined effect of volume overload and shear forces elevates the pulmonary vascular resistance, which becomes fixed during childhood. As a result, shunt reversal (right-to-left flow) is common, with hypoxemia resulting. This is often referred to as the Eisenmenger syndrome. Eisenmenger syndrome forms a small percentage (1%) of CHD patients and is defined as CHD with an initial large systemic-to-pulmonary shunt that induces progressive pulmonary vascular disease and PAH, with resultant reversal of the shunt and central cyanosis. Eisenmenger syndrome represents the most advanced form of PAH associated with CHD. An important subgroup in this Eisenmenger population is patients with Down syndrome. Classification Pulmonary arterial hypertension in congenital heart disease can be divided into four quite distinct phenotypes, which may be feasible to use in clinical practice. A. Eisenmenger syndrome. This includes all systemic-to-pulmonary shunts resulting from large defects and leading to a severe increase in pulmonary vascular resistance (PVR) and a reversed (pulmonary-to- systemic) or bidirectional shunt; cyanosis, erythrocytosis, and multiple organ involvement are present. B. PAH associated with systemic-to-pulmonary shunts. This includes moderate to large defects; PVR is mildly to moderately increased, systemic-to-pulmonary shunt is still prevalent, and no cyanosis is present at rest. https://www.textbookofcardiology.org/wiki/Grown-up_Congenital_Heart_Disease_(GUCH) 30/38 7/4/23, 12:41 AM Grown-up Congenital Heart Disease (GUCH) - Textbook of Cardiology C. PAH associated with small defects. This includes small defects (usually VSD<1 cm and ASD <2 cm of effective diameter assessed by echocardiography). D. PAH after corrective cardiac surgery. In this group of patients the congenital heart disease has been corrected, but PAH is still present immediately after surgery or recurs several months or years after surgery in the absence of significant postoperative residual lesions. Treatment Early treatment of PH is generally suggested because advanced disease may be less responsive to therapy. PAH is characterized by symptoms of dyspnea, fatigue, chest pain, and syncope. Primary therapeutic strategy should be considered in all patients with PAH and consists of diuretic, oxygen, anticoagulant, and digoxin therapy, as well as exercise and lifestyle advices. Injured endothelial cells release factors that are known to contribute to PAH. Inhibition of these factors forms the basis of some of the advanced therapies for PAH. Patients with CHD-PAH who progress to NYHA functional class II, III, or IV, qualify for advanced therapy. This is irrespective of any primary therapy given. Advanced therapy is directed at the PAH itself, rather than the underlying cause of the PAH. For PAH treatments with advanced therapy three main pathways have been detected: prostacyclin, nitric oxide and endothelin-1.This resulted in therapies with prostanoids such as epoprostenol, phosphodiesterase-5 inhibitors such as sildenafil and endothelin-1 receptor antagonists such as bosentan. Outcome The prognosis is generally poor but varies according to the severity of the underlying cause, the functional abnormalities, and the hemodynamic abnormalities. Factors that may indicate a poor prognosis include age at presentation greater than 45 years, NYHA functional class III or IV, failure to improve to a lower NYHA functional class during treatment, pericardial effusion, large right atrial size, elevated right atrial pressure, septal shift during diastole, decreased pulmonary arterial capacitance (ie, the stroke volume divided by the pulmonary arterial pulse pressure) and increased N-terminal brain natriuretic peptide level. Therapy improves exercise capacity and functional class, however the impact on mortality has been less well established. References 1. Basow, D. S. (2012a). Classification and clinical features of isolated atrial septal defects in children. UpToDate. Waltham, MA.: UpToDate. 2. Basow, D. S. (2012b). Management of atrial septal defects in adults. UpToDate. Waltham, MA.: UpToDate. https://www.textbookofcardiology.org/wiki/Grown-up_Congenital_Heart_Disease_(GUCH) 31/38 7/4/23, 12:41 AM Grown-up Congenital Heart Disease (GUCH) - Textbook of Cardiology 3. Basow, D. S. (2012c). Management and outcome of isolated atrial septal defects in children. UpToDate. Waltham, MA.: UpToDate. 4. Basow, D. S. (2012d). Pathophysiology and clinical features of atrial septal defects in adults. UpToDate. Waltham, MA.: UpToDate. 5. Basow, D. S. (2012e). Identification and assessment of atrial septal defects in adults. UpToDate. Waltham, MA.: UpToDate. 6. Basow, D. S. (2012f). Devices for percutaneous closure of a secundum atrial septal defect. UpToDate. Waltham, MA.: UpToDate. 7. Berger, F., Vogel, M., Alexi-Meskishvili, V., & Lange, P. E. (1999). Comparison of results and complications of surgical and Amplatzer device closure of atrial septal defects. The Journal of Thoracic and Cardiovascular Surgery, 118(4), 674-678; discussion 678-680 8. Engelfriet, P., Boersma, E., Oechslin, E., Tijssen, J., Gatzoulis, M. A., Thil n, U., Kaemmerer, H., e.a. (2005). 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. European Heart Journal, 26(21), 2325-2333. doi:10.1093/eurheartj/ehi396 9. Mulder, B.J.M., Pieper, P. G., Meijboom, F. J., & Hamer, J. P. M. (2006a). Atrial Septal Defect. Adult Congenital Heart Disease (Aangeboren hartafwijkingen bij volwassenen) (Second edition.). Houten: Bohn Stafleu van Loghum. 10. 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. 11. Basow, D. S. (2012h). Pathophysiology and clinical features of isolated ventricular septal defects in infants and children. UpToDate. Waltham, MA.: UpToDate. 12. Basow, D. S. (2012i). Management of isolated ventricular septal defects in infants and children. UpToDate. Waltham, MA.: UpToDate. 13. Baumgartner, H., Bonhoeffer, P., De Groot, N. M. S., de Haan, F., Deanfield, J. E., Galie, N., Gatzoulis, M. A., e.a. (2010). ESC Guidelines for the management of grown-up congenital heart disease (new version 2010). European Heart Journal, 31(23), 2915-2957. https://www.textbookofcardiology.org/wiki/Grown-up_Congenital_Heart_Disease_(GUCH) 32/38 7/4/23, 12:41 AM Grown-up Congenital Heart Disease (GUCH) - Textbook of Cardiology 14. Mulder, B.J.M., Pieper, P. G., Meijboom, F. J., & Hamer, J. P. M. (2006b). Ventricular Septal Defect. Adult Congenital Heart Disease (Aangeboren hartafwijkingen bij volwassenen) (Second edition.). Houten: Bohn Stafleu van Loghum. 15. Verheugt, C. L., Uiterwaal, C. S. P. M., Grobbee, D. 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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. 21. 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. 22. 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. 23. Vriend, J. W. J., Oosterhof, T., & Mulder, B. (2005). Noninvasive imaging for the postoperative assessment of aortic coarctation patients. Chest, 127(6), 2295. 24. 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 https://www.textbookofcardiology.org/wiki/Grown-up_Congenital_Heart_Disease_(GUCH) 33/38 7/4/23, 12:41 AM Grown-up Congenital Heart Disease (GUCH) - Textbook of Cardiology 25. 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. 26. 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 27. 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. 28. Winlaw, D. S., McGuirk, S. P., Balmer, C., Langley, S. M., Griselli, M., St mper, 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. 29. 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. 30. 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. 31. 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. 32. Basow, D. S. (2012). Hypoplastic left heart syndrome. UpToDate. Waltham, MA.: UpToDate. 33. Basow, D. S. (2012). Hypoplastic left heart syndrome. UpToDate. Waltham, MA.: UpToDate. 34. 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. 35. 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. https://www.textbookofcardiology.org/wiki/Grown-up_Congenital_Heart_Disease_(GUCH) 34/38 7/4/23, 12:41 AM Grown-up Congenital Heart Disease (GUCH) - Textbook of Cardiology 35. 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. 36. 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. 37. Balci, A., Drenthen, W., Mulder, B. J. M., Roos-Hesselink, J. W., Voors, A. A., Vliegen, H. 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