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Background: Duchenne's muscular dystrophy (DMD) is an X-linked muscular disease which is caused by the absence of dystrophin. This results in the death of muscle cells and cardiomyocytes and consequent substitution by fat and fibrous tissue. The clinical translation of this is muscle weakness and cardiomyopathy. We report on the case of a young patient with dilated cardiomyopathy on a background of DMD who developed ST-elevation myocardial infarction (STEMI). Case summary: A 19-year-old male patient with DMD, known dilated cardiomyopathy, and no risk factors for ischaemic heart disease presented with central crushing chest pain. His electrocardiogram revealed anterior ST elevation. His angiogram revealed distally occluded left anterior descending and second diagonal branch with no evidence of underlying coronary artery disease. He was treated with balloon angioplasty. An echocardiogram raised the suspicion of a left ventricular thrombus, and the mechanism of STEMI was felt to be embolism from the left ventricular thrombus on a background of dilated cardiomyopathy in the context of DMD. The patient was treated with anticoagulants (warfarin). On a repeat echocardiogram a few months later, the thrombus had resolved. At 3 years of follow-up, the patient did not present any more embolic events. Discussion: To our knowledge, this is the first case of STEMI secondary to thrombotic coronary occlusion that has been described in a patient with DMD. This case highlights an unusual complication of DMD. Based on this case, we discuss the dilemmas in the management and follow-up of this complex patient population.
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OBJECTIVES: To evaluate the outcome of unprotected left main stem (LMS) percutaneous coronary intervention (PCI) in a large UK nonsurgical center. BACKGROUND: PCI on unprotected LMS is increasingly regarded as a viable alternative to coronary artery bypass grafting (CABG) with comparable outcome and safety profile in select groups. The safety and efficacy of unprotected LMS PCI without on-site surgical back up has not been reported. METHODS: Data on all unprotected LMS PCI performed between January 2011 and December 2015, was collected from the local PCI database and electronic patient records. In hospital and 1-year major adverse cardiovascular events (MACE) (all-cause mortality, myocardial infarction [MI], stroke, and target vessel revascularization [TVR]) was recorded. RESULTS: 249 patients had unprotected LMS intervention during the study period. 77% of patients (n = 192) were male and mean age was 70 ± 12 years. 31% (n = 78) of cases were elective, 44% (n = 109) NSTEMI, and 25% (n = 62) STEMI. Anatomical distribution: 19% (n = 47) ostial left main, 31% (n = 77) shaft, and 50% (n = 125) bifurcation. The mean SYNTAX score was 24.4 ± 10.6. 22% (n = 55) of patients had severe LV impairment preprocedure and 13% (n = 33) were in cardiogenic shock at presentation. 35% (14%) required IABP support. The vast majority (98.4%) of procedures were successful. No patients required emergency transfer for CABG surgery. There were 25 (10%) in-hospital deaths. 68% of in-hospital deaths occurred in patients undergoing primary PCI for STEMI. 72% of patients who died were in cardiogenic shock at presentation. The 12-month MACE rate was 17.2%. Death occurred in 11.6%, MI in 2.4%, TVR in 2.4%, and stroke in 0.8% of patients. CONCLUSION: These results highlight the safety and efficacy of unprotected LMS PCI in a high volume non-surgical center.