[Primary angioplasty in a patient with the May-Hegglin anomaly, a rare heredity thrombocytopenia. A case report and review of the literature]. / Infarto miocardico acuto trattato con angioplastica primaria in una rara forma di piastrinopenia ereditaria: anomalia di May-Hegglin. Caso clinico e revisione della letteratura.

The May-Hegglin anomaly (MHA) is a rare autosomal dominant platelet disorder characterized by thrombocytopenia, giant platelets and leukocyte inclusion bodies. Many patients affected by the MHA have a marked hemorrhagic tendency, a well known contraindication to thrombolytic and anticoagulant therapies. We report a case of a 56-year-old woman with the MHA, referred to our department with an evolving acute ST-elevation myocardial infarction. The patient underwent urgent coronary angiography revealing the acute occlusion of the distal left anterior descending coronary artery, treated with a thrombus aspiration system. In view of the absence of residual stenosis, no balloon dilation and stent deployment were performed. No antiaggregant and anticoagulant therapy was administered. The procedure has been successful, the hospital course was uneventful and the patient was discharged 5 days later. At a 30-day follow-up the patient was asymptomatic and in a good hemodynamic state. To the best of our knowledge, this report is the first description of managing a myocardial infarction in a patient affected by the MHA in the reperfusion era.

[Initial experience in the management of myocardial infarction with primary angioplasty: results of the activity in two hospitals of the Turin area without on-site cardiac surgery]. / Iniziale esperienza organizzativa nel trattamento con angioplastica primaria dell'infarto miocardico acuto in due ospedali torinesi territoriali non dotati di cardiochirurgia: primi tre anni di attività.

BACKGROUND: Reperfusion therapy of ST-elevation myocardial infarction (STEMI) with primary coronary angioplasty (PTCA) is becoming an accepted therapeutical strategy because of a lower incidence of reinfarction, of hemorrhagic stroke and for a greater reduction of the infarct size in comparison to thrombolytic therapy. In this study we evaluated the feasibility and the effectiveness of such a strategy in two hospitals without on-site heart surgery but with a high volume of admission for acute coronary syndrome and a high caseload of elective interventional procedures. METHODS: Since January 2001 we started a program of primary PTCA for all STEMI patients presenting within 12 hours of symptom onset. An interventional team (physician, nurse and technician) were on call in a 24/7/365 fashion. Aspirin, heparin and abciximab were administered in the emergency room to all patients. Immediately after the procedure patients were given clopidogrel. RESULTS: Up to December 2003, 464 patients (mean age 63 +/- 12 years, 19.8% female) underwent primary PTCA. The symptom-emergency room interval was 3 +/- 3.9 hours, while the door-to-balloon time was 52.5 +/- 39.4 min. A TIMI 0-1 flow in the infarct-related artery was present in 55.8% of patients. Seventy patients (15.1%) presented with shock. In 430 patients (92.7%) a TIMI 3 flow was restored followed by a reduction in ST-segment elevation > 50% in 356 patients (76.7%). Total in-hospital mortality was 4.9% (23 out of 464 patients). The mortality of patients with shock was 31.4% (22 out of 70 patients). Two patients (0.4%) underwent emergency bypass. Four patients (0.8%) were electively referred to surgery prior to discharge in order to complete revascularization, which could not be obtained with further PTCA. The rate of major hemorrhagic complications was 0.8%. CONCLUSIONS: Primary PTCA for STEMI is a reperfusion strategy feasible and effective even in hospitals without on-site heart surgery, provided that a high volume of routine and emergency interventional procedures is maintained and when such a strategy is timely performed according to international guidelines.