Ji Hyun Lim

Rapid progression from hypertrophic cardiomyopathy to heart failure in a patient with Becker's muscular dystrophy.

We describe the case of a 17‐year‐old boy with Becker*s muscular dystrophy (BMD) presenting with rapid progression from hypertrophic cardiomyopathy to heart failure within 2 years. Initial echocardiogram showed severe hypertrophy of left ventricle (LV) and right ventricle (RV) with normal chamber size, and preserved LV systolic function. Microscopic study of cardiac muscle obtained by endomyocardial biopsy of the interventricular septum showed severe hypertrophy of the muscle fibers and interstitial fibrosis. Follow‐up echocardiogram 2 years after the first examination exhibited marked dilated LV and RV with severe LV global hypokinesia. Follow‐up endomyocardial biopsy demonstrated increased interstitial cellular matrix. Immunohistochemical staining for dystrophin revealed significant loss of dystrophin along the sarcoplasmic membrane of the right biceps brachii muscle, compatible with BMD.

Long-term Predictive Factors of Major Adverse Cardiac Events in Patients with Acute Myocardial Infarction Complicated by Cardiogenic Shock

Background Cardiogenic shock (CS) after acute myocardial infarction (AMI) develops in 5~10% of patients and it is associated with high mortality. The aim of this study is to assess the predictive factors of mortality for patients with AMI and CS. Methods Two hundred fifty five AMI patients with CS (the mean age was 66.0±11.0 years, M:F=156:99) out of 1,268 AMI patients who admitted at Chonnam National University Hospital between July 2000 and June 2002 were analyzed according to the clinical characteristics, coronary angiographic findings and MACE during admission and for the 1-year clinical follow-up. Results Among the enrolled patients, 129 patients survived without MACE (Group I, mean age 64.2±10.6 years, M:F=76:53), and 126 patients had MACE (Group II, mean age 68.1±10.0 years, M:F=80:46) during admission or during the 1-year follow-up period. There were significant differences in age between the Groups I and II (64.2±10.6 vs. 68.1±11.0 years, respectively, p=0.004) and the previous MI history (0 vs. 17.4%, respectively, p<0.001). The left ventricular ejection fraction (EF) was lower in Group II (Group I vs. II: 49.1±13.0 vs. 39.1±12.9%, p<0.001). The levels of troponin (Tn) I and C-reactive protein (CRP) were higher in Group II (Group I vs. II: 29.2±7.72 vs. 50.8±5.17 ng/dL, p=0.017, 3.8±0.48 vs. 9.9±1.21 mg/dL, p<0.001 respectively). Left main stem lesion (LMSL) was more common in Group II than in Group I (0.7% vs. 22.0%, respectively, p=0.004). In-hospital death was associated with low Thrombolysis In Myocardial Infarction (TIMI) flow after coronary revascularization. Conclusion Old age, a previous MI history, high Tn and CRP, low EF and LMSL are associated with higher MACE for patients with AMI and CS. Coronary revascularization with TIMI 3 flow lowers the in-hospital mortality.

The Effects of QRS Duration and Pacing Sites on the Acute Hemodynamic Changes during Right Ventricular Pacing

Background Has been reported that patients exhibiting prolonged paced QRS duration tend to have more serious heart disease, and the paced QRS duration can be an effective indicator of impaired left ventricular function. However, the acute and chronic hemodynamic effects of paced QRS duration and pacing sites during right ventricular (RV) pacing remain unknown. Methods A total of 14 patients who underwent electrophysiologic study for paroxysmal supraventricular tachycardia were examined. RV pacing was performed at 10 different sites with cycle lengths of 600 ms and 500 ms utilizing a 6-7F deflectable quadripolar electrode catheter. Systolic, diastolic, and mean blood pressures during pacing were measured once the blood pressure was stabilized. Results During RV pacing, blood pressures (systolic/diastolic/mean) decreased. The change of post-pacing QRS duration and pre-pacing the systolic blood pressure (SBP) were greater in the group with paced QRS duration. The differences overall were greater than 140 ms. The SBP decrease during pacing was larger in the group exhibiting paced QRS duration of greater than 140 ms. The SBP decrease during pacing showed relation to QRS duration during pacing (r=0.500, p=0.001), the change of QRS duration post-pacing (r=0.426, p=0.001), and SBP during sinus rhythm (r=0.342, p=0.001) on linear correlation analysis. The pacing site, on the other hand, did not affect acute hemodynamic changes during pacing. Conclusion Ventricular pacing of less than 40 ms at the area of paced QRS duration is recommended.

Successful stenting for congenital coarctation of the distal thoracic aorta (middle aortic syndrome) in an adult.

Abstract  We report a successful stent implantation for a coarctation lesion of the lower thoracic aorta in a 44‐year‐old male. This patient had suffered from claudication of both legs for 30 years. An aortogram revealed a coarctation of the distal thoracic aorta at T11 level with 60 mmHg peak systolic pressure gradient of across the lesion. A balloon angioplasty followed by an 18 mm × 40 mm sized stent implantation was performed successfully. The peak systolic gradient across the coarctation decreased from 60 to 15 mmHg. The patients symptom was relieved immediately after stenting. No significant or adverse events were observed during 7 months clinical follow‐up. Follow‐up aortogram after 7 months revealed no restenosis with an improved pressure gradient. Adults with congenital coarctation of the descending thoracic aorta can be successfully treated by stent implantation.

Congenital anomaly of the true double-lumen right coronary artery: An extremely rare case

Double right coronary artery is a rare anomaly which is mostly identified by two separate pathways with a common ostium (one-two way). We report herein an extremely rare case of congenital true double-lumen right coronary artery, an anomaly, where a common pathway from the ostium to the proximal segment diverged into two separate pathways from the proximal to the mid-segment and then converged into one pathway (one-two-one way).