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Pregnancy in cardiomyopathies and congenital heart disease

Session presentations
Pregnancy and Heart Disease

Dr. F. Mouquet  gave an excellent overview of the diagnosis, management and prognosis of peripartum cardiomyopathy (PPCM). PPCM is an idiopathic cardiomyopathy presenting with heart failure secondary to left ventricular systolic dysfunction towards the end of pregnancy (5-10% of patients) or in the months following delivery (90-95% of patients), where no other cause of heart failure is found. The LV may not be dilated but the ejection fraction is nearly always reduced below 45% . Echocardiography is mandatory for the diagnosis. Patients at high risk include women with LV ejection fraction < 30% or  biventricular dysfunction, the need for inotropic agents and intolerance to low-dose beta-blockers or ACE-inhibitors. These patients should be managed in intensive care units. Women with PPCM have a risk of approximately 60% of persistent LV dysfunction or death. Preliminary data about the use of bromocryptin to improve prognosis are promising, but there is yet no formal recommendation for its use. A subset of patients may have previously undiagnosed dilated cardiomyopathy (DCM) rather than PPCM, therefore family history for DCM and screening of first degree relatives is important. The risk of recurrence is dependant on persistence of LV dysfunction. Subsequent pregnancies are always high risk and should be managed by a multidisciplinary team in a specialist centre.

Dr. C. Autore gave a well-structured, very informative presentation concerning inherited cardiomyopathies during pregnancy. Data in the literature are scarce. Women with dilated cardiomyopathy have a risk of around 30% of heart failure during pregnancy, while death rate is up to 10%. The risk depends on functional class, LV function and previous cardiac events. When LV dysfunction is severe, pregnancy should be discouraged because of the high mortality risk. The risk of pregnancy for women with hypertrophic cardiomyopathy depends on severity of left ventricular outflow tract obstruction and on functional class. Mortality is low, and pregnancy induces no increase in malignant arrhythmias or progression of the disease. Women with arrhythmogenic right ventricular cardiomyopathy should have careful follow-up in the last trimester and puerperium since arrhythmic events tend to occur around that time. Betablockers should be continued.  The presence of an ICD is no reason to discourage pregnancy. Pregnant women with cardiomyopathies should be managed by multidisciplinary teams.

Dr. A. Pijuan Domenech discussed pregnancy and cyanotic congenital heart diseases. Women with uncorrected cyanotic heart disease have a high pregnancy risk due to increase of right-to left shunting. Women with Eisenmenger syndrome have the highest risk because of additional inability to increase cardiac output. In both groups, heart failure occurs in 20-25%. Maternal mortality in Eisenmenger syndrome is still around 30% and pregnancy is not advised. Sildenafil and prostcyclin may improve outcome. The offspring is at risk of recurrence of congenital heart disease, growth retardation, preterm birth and mortality.  Women with cyanotic heart disease and/or pulmonary hypertension should be cared for by a multidisciplinary team in a tertiairy centre. Preconceptional counselling is recommended in all women.

The last, outstanding,  presentation by dr. F. Walker concentrated on mechanical valve prostheses. Pregnancies in these women are difficult to manage because pregnancy is a pro-thrombotic state that starts at the first day of pregnancy, while there is no ideal anticoagulant. Warfarin is associated with embryopathy in the first trimester, while some authors reported a low live birth rate with warfarin use later in pregnancy. However, low molecular weight heparin (LMWH) has a higher risk of  valve thrombosis which is a life-threatening complication. This risk is lower with dose-adjustment according to anti-Xa monitoring. There is still disagreement between experts about optimal anti-Xa levels the necessity of trough level monitoring, which is advised by recent ACC/AHA recommendations but not by the ESC guidelines. Dr Walker emphasized that pregnant women with a mechanical prosthesis require multidisciplinary care in expert centres. LMWH should only be used in when anti-Xa levels can monitored. LMWH throughout pregnancy should be reserved for ‘the perfect patient’ who has an obsessive treatment compliance.

This well-attended session was of high quality due to the excellent presentations with a content relevant to the subjects discussed, which induced lively interaction with the audience.




Pregnancy in cardiomyopathies and congenital heart disease

The content of this article reflects the personal opinion of the author/s and is not necessarily the official position of the European Society of Cardiology.