Hostname: page-component-586b7cd67f-g8jcs Total loading time: 0 Render date: 2024-11-23T12:10:04.909Z Has data issue: false hasContentIssue false

Longer-term issues for young adults with hypoplastic left heart syndrome: contraception, pregnancy, transition, transfer, counselling, and re-operation

Published online by Cambridge University Press:  13 December 2011

Sameh M. Said
Affiliation:
Department of Cardiac Surgery, Mayo Clinic, Rochester, Minnesota, Canada
Joseph A. Dearani
Affiliation:
Department of Cardiac Surgery, Mayo Clinic, Rochester, Minnesota, Canada
Candice K. Silversides
Affiliation:
Division of Cardiology, Mount Sinai and Toronto General Hospitals, Toronto, Ontario, Canada
Richard M. Martinez
Affiliation:
Division of Pediatric Cardiology, The Congenital Heart Institute of Florida (CHIF), All Children's Hospital, University of South Florida College of Medicine, Saint Petersburg, Florida, United States of America
David B. Drajpuch*
Affiliation:
Department of Cardiac Nursing, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, United States of America
*
Correspondence to: D. B. Drajpuch, MSN CRNP, Children's Hospital of Philadelphia, 12NW10, 34th Street and Civic Center Boulevard, Philadelphia, Pennsylvania 19104, United States of America. Tel: +1 215 590 2708; Fax: +1 215 590 2715; E-mail: [email protected]

Abstract

Hypoplastic left heart syndrome remained a largely untreated lesion until the 1980s. In the current era, 75–80% of patients who are managed at “centres of excellence” can be expected to survive into young adulthood after staged palliation. This improved survival has led to an emerging population of patients now entering adulthood with a new set of concerns. We discuss the realised and potential issues that will be faced by these patients, including family planning, transition, and re-operation.

Type
Original Article
Copyright
Copyright © Cambridge University Press 2011

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

1.Sable, C, Foster, E, Uzark, K, et al. Best practices in managing transition to adulthood for adolescents with congenital heart disease: the transition process and medical and psychosocial issues: a scientific statement from the American Heart Association. Circulation. 2011; 123: 14541485.CrossRefGoogle ScholarPubMed
2.Dearani, JA, Connolly, HM, Martinez, R, Fontanet, H, Webb, GD. Caring for adults with congenital cardiac disease: successes and challenges for 2007 and beyond. Cardiol Young 2007; 17 Suppl 2: 8796.CrossRefGoogle Scholar
3.Kovacs, AH, Harrison, JL, Colman, JM, Sermer, M, Siu, SC, Silversides, CK. Pregnancy and contraception in congenital heart disease: what women are not told. J Am Coll Cardiol 2008; 52: 577578.CrossRefGoogle Scholar
4.Opotowsky, AR, Shellenberger, D, Dharan, V, et al. Successful pregnancies in two women with hypoplastic left heart syndrome. Congenit Heart Dis 2010; 5: 476481.CrossRefGoogle ScholarPubMed
5.Canobbio, MM, Mair, DD, van der Velde, M, Koos, BJ. Pregnancy outcomes after the Fontan repair. J Am Coll Cardiol 1996; 28: 763767.CrossRefGoogle ScholarPubMed
6.Hoare, JV, Radford, D. Pregnancy after Fontan repair of complex congenital heart disease. Aust N Z J Obstet Gynaecol 2001; 41: 464468.CrossRefGoogle ScholarPubMed
7.Drenthen, W, Pieper, PG, Roos-Hesselink, JW, et al. Pregnancy and delivery in women after Fontan palliation. Heart 2006; 92: 12901294.CrossRefGoogle ScholarPubMed
8.Drenthen, W, Pieper, PG, Roos-Hesselink, JW, et al. Outcome of pregnancy in women with congenital heart disease: a literature review. J Am Coll Cardiol 2007; 49: 23032311.CrossRefGoogle ScholarPubMed
9.Silversides, CK, Sermer, M, Siu, SC. Choosing the best contraceptive method for the adult with congenital heart disease. Curr Cardiol Rep 2009; 11: 298305.CrossRefGoogle ScholarPubMed
10.Thorne, S, MacGregor, A, Nelson-Piercy, C. Risks of contraception and pregnancy in heart disease. Heart 2006; 92: 15201525.CrossRefGoogle ScholarPubMed
11.Thorne, S, Nelson-Piercy, C, MacGregor, A, et al. Pregnancy and contraception in heart disease and pulmonary arterial hypertension. J Fam Plann Reprod Health Care 2006; 32: 7581.CrossRefGoogle ScholarPubMed
12.Ghai, A, Harris, L, Harrison, DA, Webb, GD, Siu, SC. Outcomes of late atrial tachyarrhythmias in adults after the Fontan operation. J Am Coll Cardiol 2001; 37: 585592.CrossRefGoogle ScholarPubMed
13.Siu, SC, Sermer, M, Harrison, DA, et al. Risk and predictors for pregnancy-related complications in women with heart disease. Circulation 1997; 96: 27892794.Google ScholarPubMed
14.Siu, SC, Sermer, M, Colman, JM, et al. Prospective multicenter study of pregnancy outcomes in women with heart disease. Circulation 2001; 104: 515521.CrossRefGoogle ScholarPubMed
15.Silversides, CK, Harris, L, Haberer, K, Sermer, M, Colman, JM, Siu, SC. Recurrence rates of arrhythmias during pregnancy in women with previous tachyarrhythmia and impact on fetal and neonatal outcomes. Am J Cardiol 2006; 97: 12061212.CrossRefGoogle ScholarPubMed
16.Varma, C, Warr, MR, Hendler, AL, Paul, NS, Webb, GD, Therrien, J. Prevalence of “silent” pulmonary emboli in adults after the Fontan operation. J Am Coll Cardiol 2003; 41: 22522258.CrossRefGoogle ScholarPubMed
17.Presbitero, P, Somerville, J, Stone, S, Aruta, E, Spiegelhalter, D, Rabajoli, F. Pregnancy in cyanotic congenital heart disease. Outcome of mother and fetus. Circulation 1994; 89: 26732676.CrossRefGoogle ScholarPubMed
18.Rossiter, JP, Repke, JT, Morales, AJ, Murphy, EA, Pyeritz, RE. A prospective longitudinal evaluation of pregnancy in the Marfan syndrome. Am J Obstet Gynecol 1995; 173: 15991606.CrossRefGoogle ScholarPubMed
19.Meijboom, LJ, Vos, FE, Timmermans, J, Boers, GH, Zwinderman, AH, Mulder, BJ. Pregnancy and aortic root growth in the Marfan syndrome: a prospective study. Eur Heart J 2005; 26: 914920.CrossRefGoogle ScholarPubMed
20.Cripe, L, Andelfinger, G, Martin, LJ, Shooner, K, Benson, DW. Bicuspid aortic valve is heritable. J Am Coll Cardiol 2004; 44: 138143.CrossRefGoogle ScholarPubMed
21.McBride, KL, Pignatelli, R, Lewin, M, et al. Inheritance analysis of congenital left ventricular outflow tract obstruction malformations: segregation, multiplex relative risk, and heritability. Am J Med Genet A 2005; 134A: 180186.CrossRefGoogle ScholarPubMed
22.Warnes, CA, Williams, RG, Bashore, TM, et al. 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). Circulation 2008; 118: e714e833.Google Scholar
23.Van Deyk, K, Moons, P, Gewillig, M, Budts, W. Educational and behavioral issues in transitioning from pediatric cardiology to adult-centered health care. Nurs Clin North Am 2004; 39: 755768.CrossRefGoogle ScholarPubMed
24.Drenthen, W, Pieper, PG, Roos-Hesselink, JW. Pregnancy and delivery in women after Fontan palliation. Heart 2006; 92: 12901294.CrossRefGoogle ScholarPubMed
25.Benson, CB, Bluth, EI. Family history of congenital heart disease. In: Benson CB, Arger PH, Bluth EI (eds.). Ultrasound in Obstetrics and Gynecology: A Practical Approach. Thieme Medical Publishers, New York, NY, 2000: 155156.Google Scholar
26.Hinton, RB Jr, Martin, LJ, Tabangin, ME, Mazwi, ML, Cripe, LH, Benson, DW. Hypoplastic left heart syndrome is heritable. J Am Coll Cardiol 2007; 50: 15901595.CrossRefGoogle ScholarPubMed
27.Singh, TP, Curran, TJ, Rhodes, J. Cardiac rehabilitation improves heart rate recovery following peak exercise in children with repaired congenital heart disease. Pediatr Cardiol 2007; 28: 276279.CrossRefGoogle ScholarPubMed
28.Uzark, K, Jones, K, Slusher, J, Limbers, CA, Burwinkle, TM, Varni, JW. Quality of life in children with heart disease as perceived by children and parents. Pediatrics 2008; 121: e1060e1067.CrossRefGoogle ScholarPubMed
29.Gutgesell, HP, Gessner, IH, Vetter, VL, Yabek, SM, Norton, JB Jr. Recreational and occupational recommendation for young patients with heart disease: a statement for physicians by the committee on Congenital Cardiac Defects of the Council on Cardiovascular Disease in the Young, American Heart Association. Circulation 1986; 74: 1195A1198A.Google Scholar
30.Callahan, ST, Cooper, WO. Uninsurance and health care access among young adults in the United States. Pediatrics 2005; 116: 8895.CrossRefGoogle ScholarPubMed
31.Collins, SR, Schoen, C, Kriss, JL, Doty, MM, Mahato, B. Rite of passage? Why young adults become uninsured and how new policies can help. The Commonwealth Fund (USA) 2006; 20: 114.Google ScholarPubMed
32.Ishino, K, Stumper, O, De Giovanni, JJ, et al. The modified Norwood procedure for hypoplastic left heart syndrome: early to intermediate results of 120 patients with particular reference to aortic arch repair. J Thorac Cardiovasc Surg 1999; 117: 920930.CrossRefGoogle ScholarPubMed
33.Ballweg, JA, Dominguez, TE, Tabbutt, S, et al. Reintervention for arch obstruction after stage 1 reconstruction does not adversely affect survival or outcome at Fontan completion. J Thorac Cardiovasc Surg 2010; 140: 545549.CrossRefGoogle ScholarPubMed
34.Cua, CL, Sanghavi, D, Voss, S, et al. Right ventricular pseudoaneurysm after modified Norwood procedure. Ann Thorac Surg 2004; 78: e72e73.CrossRefGoogle ScholarPubMed
35.Karimi, M, Farouk, A, Stork, J, Hennein, HA. Right ventricular aneurysm following modified Norwood-Sano operation for hypoplastic left heart syndrome. Interact CardioVasc Thorac Surg 2008; 7: 664666.CrossRefGoogle ScholarPubMed
36.McGuirk, SP, Griselli, M, Stumper, OF, et al. Staged surgical management of hypoplastic left heart syndrome: a single institution 12 year experience. Heart 2006; 92: 364370.CrossRefGoogle Scholar
37.Januszewska, K, Kozlik-Feldmann, R, Dalla-Pozza, R, et al. Right ventricle-to-pulmonary artery shunt related complications after Norwood procedure. Eur J Cardiothorac Surg 2011; 40: 508513.Google ScholarPubMed
38.Bove, EL, Ohye, RG, Devaney, EJ, Hirsch, J. Tricuspid valve repair for hypoplastic left heart syndrome and the failing right ventricle. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2007; 10: 101104.CrossRefGoogle Scholar
39.Sreeram, N, Emmel, M, Trieschmann, U, et al. Reopening acutely occluded cavopulmonary connections in infants and children. Interact Cardiovasc Thorac Surg 2010; 10: 383388.CrossRefGoogle ScholarPubMed
40.Iyengar, AJ, Brizard, CP, Konstantinov, IE, d'Udekem, Y. The option of taking down the Fontan circulation: the Melbourne experience. J Thorac Cardiovasc Surg 2010; 139: 13461348.CrossRefGoogle ScholarPubMed
41.Kadoba, K, Armiger, LC, Sawatari, K, Jonas, RA. Mechanical durability of pulmonary allograft conduits at systemic pressure. Angiographic and histologic study in lambs. J Thorac Cardiovasc Surg 1993; 105: 132141.CrossRefGoogle ScholarPubMed
42.Ehsan, A, Singh, H, Vargas, SO, et al. Neoaortic aneurysm after stage I Norwood reconstruction. Ann Thorac Surg 2005; 79: e23e25.CrossRefGoogle Scholar
43.Laudito, A, Brook, MM, Suleman, S, et al. The Ross procedure in children and young adults: a word of caution. J Thorac Cardiovasc Surg 2001; 122: 147153.CrossRefGoogle Scholar
44.Pizarro, C, Baffa, JM, Derby, CD, et al. Valve-sparing neo-aortic root replacement after Fontan completion for hypoplastic left heart syndrome. J Thorac Cardiovasc Surg 2011; 141: 10831084.CrossRefGoogle ScholarPubMed
45.Knosalla, C, Weng, Y, Warnecke, H, et al. Mycotic aortic aneurysms after orthotopic heart transplantation: a three-case report and review of the literature. J Heart Lung Transplant 1996; 15: 827839.Google ScholarPubMed
46.Vigano, M, Rinaldi, M, D'Armini, AM, et al. The spectrum of aortic complications after heart transplantation. Ann Thorac Surg 1999; 68: 105111.CrossRefGoogle ScholarPubMed
47.Kanter, KR, Mahle, WT, Vincent, RN, Berg, AM. Aortic complications after pediatric cardiac transplantation in patients with a previous Norwood reconstruction. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2011; 14: 2428.CrossRefGoogle ScholarPubMed
48.Jacobs, JP, Iyer, RS, Weston, JS, et al. Expanded PTFE membrane to prevent cardiac injury during resternotomy for congenital heart disease. Ann Thorac Surg 1996; 62: 17781782.CrossRefGoogle ScholarPubMed
49.Salminen, JT, Mattila, IP, Puntila, JT, Sairanen, HI. Prevention of postoperative pericardial adhesions in children with hypoplastic left heart syndrome. Interact Cardiovasc Thorac Surg 2011; 12: 270272.CrossRefGoogle ScholarPubMed