Cerebral infarction in Duchenne muscular dystrophy.

Duchenne muscular dystrophy (DMD) is an X-linked form of muscular dystrophy characterized by progressive limb-girdle distribution of muscle weakness. Morbidity related to cardiomyopathy (CMO) is common, but cerebral infarction (CI) is relatively rare in these patients. We report a case of a pontine infarct in a patient with DMD and advanced CMO, and review the published data on CMO and CI in patients with DMD.

Cardiac resynchronization therapy in patients with end-stage inotrope-dependent class IV heart failure.

Although cardiac resynchronization therapy (CRT) is beneficial in patients with drug-refractory New York Heart Association (NYHA) class III/IV heart failure (HF) and left ventricular (LV) dyssynchrony, CRT efficacy is not well established in patients with more advanced HF on inotropic support. Ten patients (age 55 +/- 13 years) with inotrope-dependent class IV HF (nonischemic [n = 6] and ischemic [n = 4]) received a CRT implantable cardioverter-defibrillator device. QRS duration was 153 +/- 25 ms (left branch bundle block [n = 7], intraventricular conduction delay [n = 2], and QRS <120 ms [n = 1]). The indication for CRT was based on either electrocardiographic criteria (n = 9) or echocardiographic evidence of LV dyssynchrony (n = 1). Intravenous inotropic therapy consisted of dobutamine (n = 6; 4.3 +/- 1.9 microg/kg/min) or milrinone (n = 4; 0.54 +/- 0.19 microg/kg/min) as inpatient (n = 3) or outpatient (n = 7) therapy for 146 +/- 258 days before CRT. One patient required ventilatory support before and during device implantation. All patients were alive at follow-up 1,088 +/- 284 days after CRT. Three patients underwent successful orthotopic cardiac transplantation after 56, 257, and 910 days of CRT. HF improved in 9 patients to NYHA classes II (n = 5) and III (n = 4). Intravenous inotropic therapy was discontinued in 9 of 10 patients after 15 +/- 14 days of CRT. LV volumes decreased (end-diastolic from 226 +/- 78 to 212 +/- 83 ml; p = 0.08; end-systolic from 174 +/- 65 to 150 +/- 78 ml; p <0.01). LV ejection fraction increased (23.5 +/- 4.3% to 32.0 +/- 9.1%; p <0.05). No implantable cardioverter-defibrillator shocks were recorded, and antitachycardia therapy for ventricular tachyarrhythmias was delivered in 1 patient. In conclusion, patients with end-stage inotrope-dependent NYHA class IV HF and LV dyssynchrony may respond favorably to CRT with long-term clinical benefit and improved LV function.

Latency during left ventricular pacing from the lateral cardiac veins: a cause of ineffectual biventricular pacing.

We report three patients with cardiomyopathy and pronounced stimulus to QRS latency during left ventricular (LV) pacing from an epicardial cardiac vein. Delayed LV activation during simultaneous biventricular pacing produced an electrocardiographic pattern dominated by right ventricular stimulation. Hemodynamic parameters improved immediately after advancing LV stimulation (in one patient) or pacing the LV only (in two patients) coupled with dramatic improvement of heart failure symptoms.

Peroxisome proliferator-activated receptor agonists inhibit inflammatory edema and hyperalgesia.

Previous studies have produced conflicting data on the contribution of the peroxisome proliferator-activated receptors (PPARs) to the inflammatory process. This study investigated the effects of several PPARalpha and PPARgamma subtype-specific agonists on the inflammation and hyperalgesia produced by intraplantar carrageenan injection in unanesthetized male Sprague-Dawley rats. Intraperitoneal administration of PPARalpha agonists reduced edema in parallel to their potencies determined in vitro. Perfluorooctanoic acid (PFOA) inhibited carrageenan-induced edema in a dose-dependent manner, and also reduced thermal hypersensitivity. Furthermore, PFOA produced much more robust effects when administered 0.5-24 hrs before carrageenan, as compared to when it was administered 1.5 hrs after carrageenan. Intraperitoneal administration of similar doses of the PPARgamma agonist rosiglitazone, but not the less potent agonist, troglitazone, reduced edema when administered before but not after carrageenan. We conclude that systemic administration of potent PPARalpha and PPARgamma agonists exert anti-hyperalgesic and/or antiinflammatory actions in vivo, possibly by interfering with the initiation of inflammation.