Discontinuation of Cardiac Resynchronization Therapy for Heart Failure Due to Dilated Cardiomyopathy in a 61-Year-Old Female "-Super-Responder" with Return of a Reduced Left Ventricular Ejection Fraction to Normal.

BACKGROUND Although cardiac resynchronization therapy (CRT) is widely used, it has been validated only during active pacing. "Super-responders" are patients with normalized or markedly improved left ventricular (LV) systolic function with CRT who may experience a decline in cardiac function with CRT discontinuation. CASE REPORT A 61-year-old woman with a nonischemic cardiomyopathy was admitted to our hospital in September 2008 for the treatment of heart failure (HF). Cardiac assessment revealed impaired LV function with an ejection fraction of 18%, LV dilatation, and left bundle branch block (LBBB). Despite optimized medical treatment, her HF progressed, with a rapid increase in LV chamber size, mitral regurgitation, and widening of the QRS complex. In July 2011, the patient initially refused CRT, but later consented to the procedure; CRT pacemaker implantation was subsequently performed. Thereafter, the LVEF improved from 27% to 46%, LV diastolic dimension decreased rapidly from 79 mm to 56 mm, and LVEF (65%) and LV size (47 mm) normalized within 1 year later. As of August 2012, battery exchange was needed within 1 year because of high LV pacing thresholds. In October 2012, although CRT discontinuation was not recommended, we discontinued CRT to conserve battery life with the patient's consent, hoping to maintain her condition with pharmaceutical treatment. She remained stable through January 2020, with no indication of re-exacerbation. CONCLUSIONS We describe a female patient with a nonischemic cardiomyopathy and LBBB who demonstrated a super-response to CRT and maintained improvement in LV function and functional status for 8 years after discontinuing CRT.

A familial case of multiple recurrent cardiac myxomas.

The familial variant of cardiac myxomas is an autosomally dominant disease. Here, we report a case of recurrent multiple cardiac myxomas wherein the patient, a 36-year-old woman who was referred for palpitations and nocturnal dyspnea, had a relevant familial history. Her mother had undergone extirpation of cardiac myxoma when she was about 50 years old. Echocardiography showed the presence of multiple cardiac myxomas. One of the cardiac myxomas nearly obstructed the left ventricular inflow; therefore, we extirpated the myxomas under cardiopulmonary bypass and cardioplegic arrest. The operation was uneventful and the postoperative clinical course was good. Pathological examination confirmed that the extirpated mass was a myxoma. Approximately 3 years after the first cardiac operation, we found a recurrent cardiac myxoma in the same patient. She then underwent a second operation and was discharged without any complications. Pathological examination also confirmed that the cardiac mass was a myxoma and that there was no malignancy. .

A case of takotsubo cardiomyopathy leading to the diagnosis of myasthenia gravis.

A 52-year-old woman presenting with shortness of breath and having no related past medical history was diagnosed with takotsubo cardiomyopathy. However, she revealed respiratory failure atypical with takotsubo cardiomyopathy. We diagnosed myasthenia gravis with myasthenic crisis by acetylcholine receptor-binding antibody titer with mediastinal tumor. Physical or emotional stress is well known to trigger the onset of takotsubo cardiomyopathy. Similarly, myasthenia crisis is also triggered by stress. Here, we report a case of simultaneous occurrence of takotsubo cardiomyopathy and myasthenia crisis.

Detection of von Willebrand factor and tissue factor in platelets-fibrin rich coronary thrombi in acute myocardial infarction.

The rapid closure of coronary arteries due to occlusive thrombi is the major cause of acute myocardial infarction. However, the mechanisms of coronary thrombus formation have not been elucidated. We immunohistochemically assessed the localizations and their changes over time of glycoprotein IIb/IIIa, fibrin, von Willebrand factor (vWF), and tissue factor (TF), after the onset of chest pain (<4, 4 to 6, or 6 to 12 hours), in fresh coronary thrombi causing acute myocardial infarction. The occlusive thrombi were consistently composed of platelets, fibrin, vWF, and TF from the early phase of onset, and glycoprotein IIb/IIIa and fibrin were closely associated with vWF and TF, respectively. vWF and/or TF may contribute to occlusive thrombus formation and be novel therapeutic candidates for treating patients with coronary thrombosis.

Detection of right to left shunting through a patent foramen ovale in Japanese patients with ischemic stroke by transesophageal echocardiography using a standardized Valsalva maneuver.

We attempted to clarify the usefulness of transesophageal echocardiography performed using a standardized Valsalva maneuver to detect the presence of a patent foramen ovale in Japanese patients with ischemic stroke. Four hundred ninety six patients with ischemic stroke who were admitted to the Yokohama City Brain and Stroke Center between September 1999 and February 2002 were enrolled for the study. All the enrolled patients underwent transesophageal echocardiography with contrast injection and color Doppler imaging. During the procedure, a standardized Valsalva maneuver was performed to induce right to left shunting through a patent foramen ovale. Other related structural abnormalities, such as atrial septal aneurysm and the Chiari network anomaly, were also detected by the test. Transesophageal echocardiography without the Valsalva maneuver revealed a functional right to left communication in only 8.2% of the ischemic stroke patients, whereas the procedure conducted using a standardized Valsalva maneuver to provoke shunting revealed a patent foramen ovale in 15.3% of the patients. The presence of an atrial septal aneurysm or the Chiari network anomaly was not sensitive or specific enough to predict the presence of a patent foramen ovale as diagnosed by transesophageal echocardiography using the standardized Valsalva maneuver. Our results suggest that the standardized Valsalva maneuver is a safe and useful technique to detect the presence of a patent foramen ovale, which is potentially known to be associated with paradoxical embolism.

Possible contribution of acetylamrinone and its enhancing effects on platelet aggregation under shear stress conditions in the onset of thrombocytopenia in patients treated with amrinone.

BACKGROUND: Thrombocytopenia is recognized as one of the most common complications when the patients with severe heart failure are treated with cardiotropic phosphodiesterase (PDE)-3 inhibitors. To understand the mechanism of the onset of this complication, we focused on the effects of various PDE-3 inhibitors and its stable metabolite of acetylamrinone on platelet aggregation occurring under physiological shear stress conditions. METHOD: Blood specimens were obtained from eight apparently healthy adult donors. Platelet-rich plasma was separated after anticoagulation by citrate. The effects of PDE-3 inhibitors of amrinone and olprinone, as well as the stable metabolite of the former of acetylamrinone, on platelet aggregation induced by its exposure to a shear rate of 1200 and 10,800 s(-1) were determined by optically modified cone-plate viscometer. RESULTS: Both olprinone and amrinone inhibited platelet aggregation at 10,800 s(-1) in a dose-dependent manner with the IC(50) value of 14 +/- 1 and 61 +/- 8 microM (mean +/- S.D.), respectively, while amrinone significantly inhibited platelet aggregation at 1200 s(-1) only at highest concentration tested (100 microM). Contrary to the effects shown with PDE-3 inhibitors, acetylamrinone did not inhibit platelet aggregation at all. Moreover, it even enhanced the aggregation at 1200 s(-1) when used with 5 microM. CONCLUSIONS: Our results demonstrate possible contribution of the enhancing effects of acetylamrinone on platelet aggregation occurring under blood flow conditions, which reduced the platelet count when occurring in real circulation, to the higher incidence of thrombocytopenia in patients treated with amrinone.