Lori A. Blauwet

To our knowledge, natural history has not been reported for cardiac sarcoidosis (CS) diagnosed by pathologic evaluation of the apical core at left ventricular assist device (LVAD) implantation or cardiac transplantation. We retrospectively identified 232 consecutive patients meeting CS criteria. Of these patients, 54 were diagnosed by pathologic confirmation of CS, 10 after evaluation of the apical core (LVAD implant) or explanted heart (transplant). We compared clinical characteristics at initial evaluation and outcomes for these 10 patients with those of 10 patients with known CS before LVAD implant/transplant. In the study group, five patients (50%) had confirmed extracardiac sarcoidosis before LVAD implant/transplant; five had not been diagnosed with sarcoidosis. Mean (standard deviation) left ventricular ejection fraction at initial evaluation was 23% (16%), and left ventricular end-diastolic dimension was 61 (10) mm. Four patients died during follow-up; however, no survival difference was found for the 10 patients diagnosed incidentally and the group with a previous diagnosis or institutional LVAD/transplant cohorts. Patients diagnosed with CS on pathological examination of the apical core/explanted heart may have severe dilated cardiomyopathy as the initial presentation. Outcomes for patients with CS after advanced heart failure therapies may be comparable with those of non-CS patients.

OBJECTIVE: To describe cardiac and obstetric outcomes in subsequent pregnancies of patients with peripartum cardiomyopathy and to report demographic and clinical characteristics of index pregnancies. METHODS: We conducted a retrospective cohort study of all pregnant patients with prior peripartum cardiomyopathy seen at the Mayo Clinic from January 2000 through March 2017. Maternal and neonatal outcome data of index and all subsequent pregnancies were abstracted, and all echocardiography examinations were individually reviewed. RESULTS: Twenty-five patients with prior peripartum cardiomyopathy were included; all except one had recovered left ventricular (LV) function (LV ejection fraction 50% or greater) before the subsequent pregnancy. Forty-three subsequent pregnancies were identified: six (14.0%) miscarriages, four (9.3%) terminations, and 33 (76.7%) live births. The rate of peripartum cardiomyopathy relapse was 20.9%; median LV ejection fraction nadir in patients with relapse was 43% (range 35–45%). None had LV ejection fraction decline to the level of their index pregnancy. No cardiac arrests or deaths were observed, and all patients with relapse recovered LV function. Median gestational age at delivery for all live births in subsequent pregnancies was 39.0 weeks (range 36 6/7–41 3/7 weeks). CONCLUSION: Patients with a history of peripartum cardiomyopathy who recover LV function are at risk for a transient minor decrease in LV ejection fraction during future pregnancies, but obstetric and neonatal outcomes are often favorable.

Background—Peripartum cardiomyopathy has variable disease progression and left ventricular (LV) recovery. We hypothesized that baseline right ventricular (RV) size and function are associated with LV recovery and outcome. Methods and Results—Investigations of Pregnancy-Associated Cardiomyopathy was a prospective 30-center study of 100 peripartum cardiomyopathy women with LV ejection fraction (LVEF) Conclusions—Peripartum cardiomyopathy patients had a high incidence of LV recovery, but a significant minority had persistent LV dysfunction or a major clinical event by 1 year. RV function per echocardiographic fractional area change at presentation was associated with subsequent LV recovery and clinical outcomes and thus is prognostically important.

A 63-year-old man presented with generalized fatigue, chills, malaise, dyspnea, intermittent fevers, and 50-pound weight loss of 4 months′ duration. Blood cultures were positive for pan-sensitive Streptococcus anginosus. Transesophageal echocardiography showed an 11 mm × 3 mm mobile mass attached to the mitral valve, a 16 mm × 16 mm mobile mass attached to the pulmonary valve, and a small membranous ventricular septal defect. The patient received 12 weeks of intravenous (IV) antibiotics with eventual resolution of the masses. Multi-valve endocarditis involving both the left and right chambers is rarely reported without prior history of IV drug use or infective endocarditis. Our case emphasizes the importance of careful assessment for ventricular septal defects or extra-cardiac shunts in individuals who present with simultaneous right and left-sided endocarditis.

Idiopathic giant cell myocarditis (GCM) and cardiac sarcoidosis (CS) are rare disorders that cause cardiomyopathy, often with ventricular arrhythmias or heart block. Infection, autoimmune processes, and genetics have all been implicated in the pathogenesis of these diseases, but the etiology for both diseases is likely a complex multifactorial process. Both GCM and CS are generally progressive despite treatment with standard heart failure and arrhythmia therapies. Making the diagnosis of GCM or CS on initial clinical presentation is possible in only a small percentage of patients, so myocardial tissue diagnosis is required. The use of multiple noninvasive imaging modalities may aid in diagnosis and assessment of response to treatment. Establishing the diagnosis of GCM or CS early is crucial, as tailored immunosuppressive treatment may significantly alter the clinical course of these patients. The prognosis of patients with GCM is poor, while the prognosis for patients with CS varies according to degree of left ventricular dysfunction.

Heart failure during pregnancy has been recognised since the mid 19th century, but it was not until 1971 that Demakis and colleagues proposed diagnostic criteria for PPCM.^w1 These diagnostic criteria have subsequently been revised by several groups1 2 ^w2 (table 1).

Peripartum cardiomyopathy (PPCM) is a potentially devastating disease that affects women during the last months of pregnancy or the first months after delivery. The aetiology and pathogenesis of this disease remain unclear, but oxidative stress and the generation of a cardiotoxic fragment of prolactin may play key roles. Diagnosing PPCM remains a challenge, as symptoms may mimic those women experience during normal pregnancy and the peripartum period. A high index of suspicion is thus necessary to make the diagnosis. Patients with PPCM have a varied clinical course, as some patients achieve full recovery while others progress to end-stage heart failure and even death. Standard heart failure treatment is indicated, although special provisions are necessary in pregnant and lactating women. Additional research into the pathophysiology of this disease, including possible genetic contributions, may lead to novel treatment strategies that can improve outcomes.