Cardiomyopathies and Congenital Heart Disease in Pregnancy

Abstract

Pregnancy-associated diseases of the cardiovascular system occur in up to 10% of all pregnancies and the incidence is increasing. Besides congenital heart disease or pre-existing cardiomyopathy in the mother, the clinical focus has moved especially to peripartum cardiomyopathy (PPCM) because of the conditionʼs dramatic clinical course and the identification of the underlying mechanisms. This review article concentrates therefore on PPCM, which occurs either in the last month of pregnancy or in the first 6 months following delivery in women with previously healthy hearts. The global incidence is estimated today at roughly 1 : 1000 pregnancies. The condition is heterogeneous, ranging from mild disease to severe acute heart failure with cardiogenic shock and sudden cardiac death of the mother. Important risk factors are pregnancy-associated hypertensive complications, multiple pregnancy and greater maternal age. The pathogenesis comprises cleavage, induced by increased oxidative stress, of the lactation hormone prolactin into a toxic hormone fragment that damages blood vessels, known as the 16-kDalton protein fragment. The lactation-blocking drug bromocriptine prevents prolactin release and promotes healing of PPCM in combination with pharmacological heart failure therapy; it appears to prevent recurrence in subsequent pregnancies. Uncomplicated pregnancy is possible in most patients with congenital heart disease. The foetal complications include an increased abortion rate, prematurity and smallness for gestational age, as well as an increased risk of cardiac malformations. The maternal risk comprises mainly arrhythmias, progressive heart failure and thrombembolic complications, with the risk of vessel dissection with a low mortality risk of < 1% in the case of aortopathies. Individual risk assessment and corresponding close monitoring of the pregnancy are required.

Key words

peripartum cardiomyopathy - PPCM - pregnancy - congenital heart defects

• References/Literatur

• 1 Elliott P, Andersson B, Arbustini E. et al. Classification of the cardiomyopathies: a position statement from the European Society of Cardiology Working Group on Myocardial and Pericardial Diseases. Eur Heart J 2008; 29: 270-276
• 2 Sliwa K, Hilfiker-Kleiner D, Petrie MC. et al. Heart Failure Association of the European Society of Cardiology Working Group on Peripartum Cardiomyopathy. Current state of knowledge on aetiology, diagnosis, management, and therapy of peripartum cardiomyopathy: a position statement from the Heart Failure Association of the European Society of Cardiology Working Group on peripartum cardiomyopathy. Eur J Heart Fail 2010; 12: 767-778
• 3 Sliwa K, Petrie MC, Hilfiker-Kleiner D. et al. Long-term prognosis, subsequent pregnancy, contraception and overall management of peripartum cardiomyopathy: practical guidance paper from the Heart Failure Association of the European Society of Cardiology Study Group on Peripartum Cardiomyopathy. Eur J Heart Fail 2018; 20: 951-962 doi:10.1002/ejhf.1178
• 4 Hilfiker-Kleiner D, Bauersachs J. [Cardiovascular disease in pregnancy]. Dtsch Med Wochenschr 2016; 141: 110-114
• 5 Silversides CK, Grewal J, Mason J. et al. Pregnancy Outcomes in Women With Heart Disease: The CARPREG II Study. J Am Coll Cardiol 2018; 71: 2419-2430 doi:10.1016/j.jacc.2018.02.076
• 6 Sliwa K, van Hagen IM, Budts W. et al. ROPAC investigators. Pulmonary hypertension and pregnancy outcomes: data from the Registry Of Pregnancy and Cardiac Disease (ROPAC) of the European Society of Cardiology. Eur J Heart Fail 2016; 18: 1119-1128 doi:10.1002/ejhf.594
• 7 van Hagen IM, Roos-Hesselink JW, Ruys TP. et al. ROPAC Investigators; EURObservational Research Programme (EORP) Team. Pregnancy in Women With a Mechanical Heart Valve: Data of the European Society of Cardiology Registry of Pregnancy and Cardiac Disease (ROPAC). Circulation 2015; 132: 132-142 doi:10.1161/CIRCULATIONAHA.115.015242
• 8 Gouton M, Nizard J, Patel M. et al. Maternal and fetal outcomes of pregnancy with Fontan circulation: A multicentric observational study. Int J Cardiol 2015; 187: 84-89 doi:10.1016/j.ijcard.2015.03.344
• 9 Elkayam U, Goland S, Pieper PG. et al. High-Risk Cardiac Disease in Pregnancy: Part I. J Am Coll Cardiol 2016; 68: 396-410 doi:10.1016/j.jacc.2016.05.048
• 10 Elkayam U, Goland S, Pieper PG. et al. High-Risk Cardiac Disease in Pregnancy: Part II. J Am Coll Cardiol 2016; 68: 502-516 doi:10.1016/j.jacc.2016.05.050
• 11 Hassan N, Patenaude V, Oddy L. et al. Pregnancy outcomes in Marfan syndrome: a retrospective cohort study. Am J Perinatol 2015; 30: 123-130 doi:10.1055/s-0034-1376179
• 12 Hilfiker-Kleiner D, Haghikia A, Nonhoff J. et al. Peripartum cardiomyopathy: current management and future perspectives. Eur Heart J 2015; 36: 1090-1097
• 13 Hilfiker-Kleiner D, Sliwa K. Pathophysiology and epidemiology of peripartum cardiomyopathy. Nat Rev Cardiol 2014; 11: 364-370
• 14 Selle T, Renger I, Labidi S. et al. Reviewing peripartum cardiomyopathy: current state of knowledge. Future Cardiol 2009; 5: 175-189
• 15 Bauersachs J, Arrigo M, Hilfiker-Kleiner D. et al. Current management of patients with severe acute peripartum cardiomyopathy: practical guidance from the Heart Failure Association of the European Society of Cardiology Study Group on peripartum cardiomyopathy. Eur J Heart Fail 2016; 18: 1096-1105
• 16 Elkayam U. Risk of subsequent pregnancy in women with a history of peripartum cardiomyopathy. J Am Coll Cardiol 2014; 64: 1629-1636
• 17 Hilfiker-Kleiner D, Haghikia A, Masuko D. et al. Outcome of subsequent pregnancies in patients with a history of peripartum cardiomyopathy. Eur J Heart Fail 2017; 19: 1723-1728
• 18 Haghikia A, Podewski E, Libhaber E. et al. Phenotyping and outcome on contemporary management in a German cohort of patients with peripartum cardiomyopathy. Basic Res Cardiol 2013; 108: 366
• 19 Hilfiker-Kleiner D, Kaminski K, Podewski E. et al. A cathepsin D-cleaved 16 kDa form of prolactin mediates postpartum cardiomyopathy. Cell 2007; 128: 589-600
• 20 Hilfiker-Kleiner D, Haghikia A, Berliner D. et al. Bromocriptine for the treatment of peripartum cardiomyopathy: a multicentre randomized study. Eur Heart J 2017; 38: 2671-2679
• 21 Arrigo M, Blet A, Mebazaa A. Bromocriptine for the treatment of peripartum cardiomyopathy: welcome on BOARD. Eur Heart J 2017; 38: 2680-2682
• 22 Sliwa K, Forster O, Tibazarwa K. et al. Long-term outcome of peripartum cardiomyopathy in a population with high seropositivity for human immunodeficiency virus. Int J Cardiol 2011; 147: 202-208
• 23 Stapel B, Kohlhaas M, Ricke-Hoch M. et al. Low STAT3 expression sensitizes to toxic effects of beta-adrenergic receptor stimulation in peripartum cardiomyopathy. Eur Heart J 2017; 38: 349-361
• 24 Regitz-Zagrosek V, Roos-Hesselink J, Bauersachs J. et al. 2018 ESC Guidelines on the management of cardiovascular diseases during pregnancy: the Task Force on the Management of Cardiovascular Diseases during Pregnancy of the European Society of Cardiology (ESC). Eur Heart J 2018; 39: 3165-3241 doi:10.1093/eurheartj/ehy340
• 25 Canobbio MM, Warnes CA, Aboulhosn J. et al. American Heart Association Council on Cardiovascular and Stroke Nursing; Council on Clinical Cardiology; Council on Cardiovascular Disease in the Young; Council on Functional Genomics and Translational Biology; Council on Quality of Care and Outcomes Research. Management of Pregnancy in Patients With Complex Congenital Heart Disease: A Scientific Statement for Healthcare Professionals From the American Heart Association. Circulation 2017; 135: e50-e87 doi:10.1161/CIR.0000000000000458
• 26 Kampman MA, Balci A, van Veldhuisen DJ. et al. ZAHARA II investigators. N-terminal pro-B-type natriuretic peptide predicts cardiovascular complications in pregnant women with congenital heart disease. Eur Heart J 2014; 35: 708-715 doi:10.1093/eurheartj/eht526
• 27 Krexi D, Sheppard MN. Pulmonary hypertensive vascular changes in lungs of patients with sudden unexpected death. Emphasis on congenital heart disease, Eisenmenger syndrome, postoperative deaths and death during pregnancy and postpartum. J Clin Pathol 2015; 68: 18-21 doi:10.1136/jclinpath-2014-202613
• 28 Van Ommen CH, Ottenkamp J, Lam J. et al. The risk of postthrombotic syndrome in children with congenital heart disease. J Pediatr 2002; 141: 582-586