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Prof. Johann Bauersachs,
Denise Hilfiker Kleiner reviewed the definition of peripartum cardiomyopathy (PPCM) according to the ESC, i.e. systolic heart failure (ejection fraction <45%) one month before or within 5 months after delivery in the absence of pre-existing heart disease. The incidence is around 1:2000 pregnancies in Caucasians, but markedly higher in Africans. We need to increase the awareness of this disease, as too often it takes weeks to months until PPCM is diagnosed in these young females.
According to a large German registry, risk factors are twin pregnancies, Caesarean section, pregnancy -associated hypertensive disorders, and smoking. Only 50 % of the patients have full recovery of left ventricular function within 6 months after diagnosis. NT-proBNP appears to be a reasonable screening marker, however, it lacks specificity. microRNA-146 was identified as a specific marker, and may also be a future target for therapy.
Karen Sliwa discussed the pharmacological management of PPCM, which consists mainly of standard heart failure drugs such as betablockers and ACE inhibitors; and diuretics as needed. It is important to note that ACE inhibitors and ARBs are contraindicated during pregnancy, as they harm the foetus. Based on pre-clinical work from D. Hilfiker-Kleiner, Bromocriptine has emerged as a potential useful treatment for patients with PPCM, and both a small pilot study, as well as registry data, support its use.However, more definitive proof of its value will derive from the ongoing randomized multicenter study in Germany. K. Sliwa also presented data on the use of Bromocriptine in patients with a second pregnancy after PPCM who benefitted from Bromocriptine given 4 hours after the second delivery.
Given the reported procoagulant effects of bromocriptine, it may be wise to give additional LMWH. As patients with PPCM often display left ventricular thrombus or have concurrent pulmonary embolism, it is necessary to search for these potential problems and treat with anticoagulation.
Frederic Mouquet reported that it is hard to predict whether LV function in PPCM patients will recover; therefore it is wise to postpone cardiac resynchronisation therapy and/or ICD implantation up to one year. As patients with markedly reduced LV function are at elevated risk for early ventricular fibrillation, the use of a life vest is advocated. In patients with cardiogenic shock not responding to therapy, left ventricular support by intra-aortic balloon pump, Impella device, extracorporeal membrane oxygenation or left-ventricular assist device implantation may be necessary in selected cases.
Heart transplantation is also an option in patients who do not recover; however, results in PPCM patients may be less favourable than in patients with dilated cardiomyopathy, with more graft rejections and lower survival in PPCM patients.
The important question of how to counsel patients who want to become pregnant again was addressed by Mark Petrie.
Although prospective data is scarce, patients who did not have full recovery of LV function have a higher risk of death and worsening of heart failure. Patients with full recovery have better outcome with only a low risk of death, but may nevertheless suffer from recurrence of heart failure. Therefore, in all patients with PPCM, a second pregnancy requires intense care by an experienced interdisciplinary team. As even after an uneventful second pregnancy, LV function can deteriorate at a later stage, long-term follow-up is advocated.
Given the high risk of worsening heart failure and foetal damage by ACE inhibitors/aldosterone antagonists, all patients with PPCM need to be counselled on contraception, which may be achieved by intrauterine devices, which appear to be safe and effective in these patients.
Session Title: Controversies in peripartum cardiomyopathy
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