Risk stratification and management of women with cardiomyopathy/heart failure planning pregnancy or presenting during/after pregnancy: a position statement from the Heart Failure Association of the European Society of Cardiology Study Group on Peripartum Cardiomyopathy

Abstract

This position paper focusses on the pathophysiology, diagnosis and management of women diagnosed with a cardiomyopathy, or at risk of heart failure (HF), who are planning to conceive or present with (de novo or previously unknown) HF during or after pregnancy. This includes the heterogeneous group of heart muscle diseases such as hypertrophic, dilated, arrhythmogenic right ventricular and non‐classified cardiomyopathies, left ventricular non‐compaction, peripartum cardiomyopathy, Takotsubo syndrome, adult congenital heart disease with HF, and patients with right HF. Also, patients with a history of chemo‐/radiotherapy for cancer or haematological malignancies need specific pre‐, during and post‐pregnancy assessment and counselling. We summarize the current knowledge about pathophysiological mechanisms, including gene mutations, clinical presentation, diagnosis, and medical and device management, as well as risk stratification. Women with a known diagnosis of a cardiomyopathy will often require continuation of drug therapy, which has the potential to exert negative effects on the foetus. This position paper assists in balancing benefits and detrimental effects.

Ultrasound of Fetal Cardiac Function Changes in Pregnancy-Induced Hypertension Syndrome

“…PIH is a syndrome specific to pregnant women [ 16 , 17 ] and may trigger systemic small artery spasm in patients, leading to increased peripheral vascular resistance and consequently placental dysfunction [ 18 , 19 ]. In the case of ischemia and hypoxia, the compensatory function initiated by the fetal organism is associated with redistribution of fetal cardiac output, compensatory hypertrophy of cardiomyocytes, and impairment of cardiac diastolic and systolic function.…”

2021 ESC Guidelines for the diagnosis and treatment of acute and chronic heart failure

“…The increased demands on ventricular function due to the increased circulating volume and cardiac output of pregnancy can unmask pre‐existing, but previously undiagnosed, causes of HF such as CMPs and valve diseases 886 . Symptoms are more likely to occur in the second trimester when the demand for an increased cardiac output is the highest.…”

“…Symptoms are more likely to occur in the second trimester when the demand for an increased cardiac output is the highest. Severe emotionally stressful episodes during pregnancy and delivery can also cause Takotsubo syndrome 885 , 886 …”

Maternal Heart Failure

“…Treatment will vary depending on whether a woman is pregnant or postpartum, as once mothers have delivered there is no longer a concern about fetal stability/maturity or teratogenicity of medications and more standard HF therapies can be initiated (Figure 6 ). 67 , 68 , 73 , 74 An extensive review of the medication use in pregnancy is beyond the scope of this article but has been reviewed recently. 75 In patients with stable HF, medical treatment approach parallels that of nonpregnant patients; however, there is a need to avoid teratogenic drugs (angiotensin‐converting enzyme inhibitors, angiotensin II receptor blockers, angiotensin receptor neprilysin inhibitors, mineralocorticoid receptor antagonists, atenolol, direct factor Xa inhibitors).…”

“… 75 In patients with stable HF, medical treatment approach parallels that of nonpregnant patients; however, there is a need to avoid teratogenic drugs (angiotensin‐converting enzyme inhibitors, angiotensin II receptor blockers, angiotensin receptor neprilysin inhibitors, mineralocorticoid receptor antagonists, atenolol, direct factor Xa inhibitors). 68 , 73 , 76 Mainstays of treatment include hydralazine, nitrates, and β‐blockers. 68 Diuretics should be used in those patients with signs or symptoms of pulmonary edema; however, caution must be exercised as they have the potential to reduce placental perfusion.…”