Introduction/Background

Peripartum Cardiomyopathy (PPCM) is a rare but serious disease, characterized by left ventricular systolic dysfunction with left ventricular ejection fraction (LVEF) <45% within the last month of pregnancy till 6 months after birth. In some cases, a complete recovery of LVEF can be seen. However, deterioration of LVEF and a significant mortality despite conventional therapy can be seen in other cases. As one of the possible factors, cardiac antibodies have been described in the development of PPCM. Elimination of these antibodies by immunoadsorption (IA) has shown promising results, with significant improvement in cardiac performance and clinical symptoms in heart failure patients.

Case description

We report the case of a 26-year-old woman, which presented with a history of increasing dyspnea at rest within 6 weeks after delivery. The pregnancy (II. gravida, II. para) itself was without complications. Acute heart failure with severe left ventricular dysfunction (EF 15%) and 4 ventricular thrombi was diagnosed. Coronary artery disease and spontaneous coronary artery dissection was ruled out by coronary angiography. Cardiac MRI showed biventricular dilatation with severe impairment of LVEF without signs of acute myocarditis. Despite the introduction of heart failure therapy (dapagliflozin, spironolactone, ivabradine, sacubitril/valsartan, torasemide, metoprolol) and bromocriptine severe impairment of LVEF (~23%) persisted after 4 weeks of treatment. Analysis of cardiac antibodies showed a positive result for M2-, alpha1- and endothelin-autoantibodies, hence we started a cycle of five immunoadsorption treatments using ADAsorb with GLOBAFFIN-adsorber on five consecutive days, treating 2.5 times the plasma volume per session. IA was well tolerated  and a good reduction of Immunoglobulin G level could be achieved. The last immunoadsorption session was followed by substitution of human polyclonal IgG (0.5 mg/kg body weight, absolute: 500 mg). Cardiac MRI follow-up 2 months after IA showed a significant improvement in left and right ventricular function (LVEF 34 vs. 16%, RVEF 42 vs. 18%). Moreover, 6 months after IA cardiac MRI revealed further improvement of LVEF (45%) and the auto-antibodies were not detectable anymore.

Conclusion

Although a highly reduced left ventricular ejection fraction and a greater degree of left ventricular dilatation at presentation can predict a poor recovery of the left ventricular ejection fraction under conventional therapy in PPCM patients, our patient (LVEF 16%, LVEDD 7.0 cm) showed an improvement of LVEF 6 months after induction of IA (LVEF 45%). While the definite cause of PPCM remains unclear, the analysis of cardiac antibodies indicates an, at least partial, immunological pathogenesis in the worsening of LVEF. Several studies revealed that removing these antibodies resulted in a recovery of the left ventricular function and a better clinical outcome. Therefore, even if signs of myocarditis in cMRI are absent, the analysis of cardiac antibodies in patients with severe PPCM  could help to identify those patients in whom IA might be a promising option.