cctga patients need lifelong follow-up in an age-appropriate facility with expertise in

Transcription

1 ONLINE SUPPLEMENT ONLY: ISSUES IN THE ADULT WITH CCTGA General cctga patients need lifelong follow-up in an age-appropriate facility with expertise in congenital heart disease care at annual intervals. 6,s1 In selected cases shorter intervals may be required. Due to the abnormality of the conduction system an increasing incidence of AV block is seen with increasing age; the incidence of complete heart block being 2% per year. s1 Therefore, an electrocardiogram should be performed regularly. For some patients Holter monitoring or electrocardiographic event recording may be helpful, especially if symptoms are intermittent. Considering that many patients with cctga may require a pacemaker, the use of MRI-compatible devices is recommended. RV as systemic ventricle Late onset congestive heart failure is common in cctga. 6 In a study of adults with complex CHD and a systemic RV, the clinical syndrome of heart failure occurred in 32% of patients with cctga. s2 Heart failure is more common in those with associated cardiac lesions (VSD or PS). By the age of 45 years, 67% of patients with associated lesions (and 25% of those without) had congestive heart failure. 19 Clinical heart failure is also associated with arrhythmia, pacemaker implantation, prior surgery of any type, and tricuspid valvuloplasty or replacement. 19 Systemic ventricular dysfunction was found in 70% of cctga with associated lesions and in 55% of cctga patients without other lesions, so regular surveillance of ventricular function is of critical importance. 19 CMR imaging and exercise testing are important in the risk assessment. Patients with a CMR derived RV end-diastolic volume index >150 ml/m2 and peak exercise systolic

2 blood pressure below 180 mm Hg had a higher annual event rate than patients without these risk factors. s3 In patients with a systemic RV, regular physical activity is positively associated with exercise capacity and quality of life, irrespective of cardiac performance. s4 Tricuspid regurgitation is encountered in up to 80% of patients 19 and may necessitate tricuspid valve replacement. The importance of assessing tricuspid valve function has been emphasized, 6 however the timing of tricuspid valve intervention is a topic of ongoing discussion. A recent study showed that the best predictor for post-operative systemic RV ejection fraction was the preoperative ejection fraction with a threshold of 40%. s5 Atrial fibrillation and NYHA class III- IV were additional pre-operative variables associated with late mortality. s5 An earlier study found substantial mortality below a threshold RV ejection fraction of 44%. s6 Taken together, these results suggest that an earlier intervention is indicated in patients with significant tricuspid regurgitation before RV function deteriorates. Percutaneous pulmonary valve replacement is feasible in adult CCTGA patients with dysfunctional left ventricular outflow tract conduits, but associated with a 15% rate of valve nonimplantation and serious adverse events. s7 LV as systemic ventricle Among the late complications of anatomic repair are systemic LV dysfunction and atrial baffle stenosis or leaks, so regular echocardiographic surveillance is necessary. CMR is superior for assessing the baffles. Late LV dysfunction or angina pectoris after arterial switch are indications for an assessment of the coronary arteries. Depending on local availability, stress perfusion echocardiography, s8 CT, myocardial SPECT imaging, or stress CMR are valid options. Systemic LV dysfunction should be treated according to current guidelines. s9 Cardiac resynchronization

3 therapy (CRT) is a valid option, but data is sparse. s10 Also of concern is the development of neoaortic valve regurgitation. Previous pulmonary artery banding has been associated with the need for aortic valve surgery and significant neoartic regurgitation. s11 Regular follow up assessment is therefore mandatory. The Rastelli repair requires an RV to pulmonary artery conduit, so conduit stenosis is common late complication with anticipated need for reoperation or reintervention in the catheterization laboratory. Echocardiography is well suited for follow up assessment in most patients, with CMR delivering important additional information in adult patients, especially when considering a percutaneous intervention. Electrophysiology and Exercise testing Tachyarrhythmias can complicate cctga. In a retrospective review of adult cctga with 544 patient years of follow up, there were 5 five cases of sudden dealth, 2 in those who had previously documented supraventricular or non-sustained ventricular tachycardia. All who died were classified as New York Heart Association class I or II, suggesting the need for improved risk stratification. s12 Feasibility of successful radiofrequency catheter ablation of atrioventricular node reentrant tachycardia has been shown, with the slow pathway input region mainly found in the posterior midseptum. s13 Cardiopulmonary exercise testing should be performed on a regular basis, because many patients may not report symptoms despite an objective reduced exercise capacity. In adult cctga patients studied with cardiopulmonary exercise testing, aerobic capacity was severely diminished, varying from 30-50% of the results achieved by healthy subjects. s14 Patients with a systemic RV also have an abnormal response during stress CMR, manifesting as an inability to

4 increase RV ejection fraction during pharmacological or exercise provocation, which, in a single center series was shown to predict hospitalization and cardiac death. 6,s15 Pregnancy Successful pregnancy can be achieved in most women with cctga, and patients must be under the direct management of an experienced multidisciplinary team. s16 In a series of 19 patients who had 45 pregnancies, 5 developed cardiovascular complications during pregnancy. Twenty-seven pregnancies resulted in live births, while 12 miscarriages, and 6 elective terminations of pregnancy were observed. s17 Of 60 pregnancies in 22 patients reported by Connolly et al., 49 resulted in live births, while 11 were unsuccessful. s18 In a more contemporary series of 20 pregnancies in 13 women, 19 were successful, and maternal complications (supraventricular arrhythmia and deterioration of RV function) occurred in 3 patients. s19 During a 19-year median follow-up after pregnancy, rate of hospitalization for heart failure was similar in women who had prior pregnancy and controls. s19 It is reassuring that no maternal death occurred in any of these studies. The American Heart Association has recently published guidelines for the management of pregnant women with complex congenital heart disease including cctga. s16 The reported recurrence risk of CHD in live births for mothers with cctga is between 0-5%, and this may guide patient education and counseling. s17-s19

5 References ONLINE SUPPLEMENT REFERENCES ONLY s1. Baumgartner H, Bonhoeffer P, De Groot NM, et al. ESC Guidelines for the management of grown-up congenital heart disease (new version 2010). Eur Heart J. 2010;31(23): s2. Piran S, Veldtman G, Siu S, Webb GD, Liu PP. Heart failure and ventricular dysfunction in patients with single or systemic right ventricles. Circulation. 2002;105(10): s3. van der Bom T, Winter MM, Groenink M, et al. Right ventricular end-diastolic volume combined with peak systolic blood pressure during exercise identifies patients at risk for complications in adults with a systemic right ventricle. J Am Coll Cardiol. 2013;62(10): s4. Winter MM, Bouma BJ, van Dijk AP, et al. Relation of physical activity, cardiac function, exercise capacity, and quality of life in patients with a systemic right ventricle. Am J Cardiol. 2008;102(9): s5. Mongeon FP, Connolly HM, Dearani JA, Li Z, Warnes CA. Congenitally corrected transposition of the great arteries ventricular function at the time of systemic atrioventricular valve replacement predicts long-term ventricular function. J Am Coll Cardiol. 2011;57(20): s6. van Son JA, Danielson GK, Huhta JC, et al. Late results of systemic atrioventricular valve replacement in corrected transposition. J Thorac Cardiovasc Surg. 1995;109(4): ; discussion s7. Whiteside W, Tretter JT, Aboulhosn J, et al. Acute and Midterm Outcomes of Transcatheter Pulmonary Valve Replacement for Treatment of Dysfunctional Left Ventricular Outflow Tract Conduits in Patients With Aortopulmonary Transposition and a Systemic Right Ventricle. Circ Cardiovasc Interv. 2017;10(9). s8. Kutty S, Xiao Y, Olson J, et al. Safety and Efficacy of Cardiac Ultrasound Contrast in Children and Adolescents for Resting and Stress Echocardiography. J Am Soc Echocardiogr. 2016;29(7): s9. Budts W, Roos-Hesselink J, Radle-Hurst T, et al. Treatment of heart failure in adult congenital heart disease: a position paper of the Working Group of Grown-Up Congenital Heart Disease and the Heart Failure Association of the European Society of Cardiology. Eur Heart J. 2016;37(18): s10. Sachdeva S, Jacobsen RM, Woods RK, et al. Anatomic Repair of Congenitally Corrected Transposition of the Great Arteries: Single-Center Intermediate-Term Experience. Pediatr Cardiol s11. Brizard CP, Lee A, Zannino D, et al. Long-term results of anatomic correction for congenitally corrected transposition of the great arteries: A 19-year experience. J Thorac Cardiovasc Surg. 2017;154(1): e254. s12. McCombe A, Touma F, Jackson D, et al. Sudden cardiac death in adults with congenitally corrected transposition of the great arteries. Open Heart. 2016;3(2):e s13. Liao Z, Chang Y, Ma J, et al. Atrioventricular node reentrant tachycardia in patients with congenitally corrected transposition of the great arteries and results of radiofrequency catheter ablation. Circ Arrhythm Electrophysiol. 2012;5(6): s14. Fredriksen PM, Chen A, Veldtman G, Hechter S, Therrien J, Webb G. Exercise capacity in adult patients with congenitally corrected transposition of the great arteries. Heart. 2001;85(2): s15. Winter MM, Scherptong RW, Kumar S, et al. Ventricular response to stress predicts outcome in adult patients with a systemic right ventricle. Am Heart J. 2010;160(5):

6 s16. Canobbio MM, Warnes CA, Aboulhosn J, et al. Management of Pregnancy in Patients With Complex Congenital Heart Disease: A Scientific Statement for Healthcare Professionals From the American Heart Association. Circulation. 2017;135(8):e50-e87. s17. Therrien J, Barnes I, Somerville J. Outcome of pregnancy in patients with congenitally corrected transposition of the great arteries. Am J Cardiol. 1999;84(7): s18. Connolly HM, Grogan M, Warnes CA. Pregnancy among women with congenitally corrected transposition of great arteries. J Am Coll Cardiol. 1999;33(6): s19. Kowalik E, Klisiewicz A, Biernacka EK, Hoffman P. Pregnancy and long-term cardiovascular outcomes in women with congenitally corrected transposition of the great arteries. Int J Gynaecol Obstet. 2014;125(2):