Severe mitral regurgitation in a young female with pansinusitis and bronchiectasis

SummaryPrimary ciliary dyskinesia (PCD) is a disease characterized by symptoms of upper and lower respiratory tract infections due to abnormal structure and function of cilia.Cardiac involvement is characterized by situs inversus (Kartagener's syndrome in PCD) and other congenital cardiovascular abnormalities. We describe a 34-year-old female with a history of recurrent sinusitis and bronchiectasis but without situs inversus or other congenital cardiac anomalies in whom an association between mitral regurgitation secondary to myxoid degeneration and primary ciliary dyskinesia was suggested

Additive Value of B-Type Natriuretic Peptide on Rest 201Tl-Dipyridamole Stress 99mTc-Sestamibi Gated Myocardial SPECT in Patients with Normal Left Ventricular Systolic Function

We evaluated whether BNP has additive value to SPECT in patients with normal left ventricular (LV) systolic function. Data from 224 consecutive patients who underwent rest 201Tl-dipyridamole stress 99mTc-sestamibi gated SPECT and coronary angiography due to chest pain were analyzed. Patients with true positive SPECT showed significant higher BNP level than those with false positive defect (38.5 (19.0–79.8) versus 19.0 (9.3–35.8), P = .01). Patients with true negative SPECT also showed significantly lower BNP level than those with false negative SPECT (39.0 (23.0–77.0) versus 22.0 (15.0–43.0), P = .002). In multivariate analyses, elevated BNP level (using a cut-off value of 23.0 pg/mL) was the strongest and independent predictor of CAD in overall patients (OR 2.75, 95% CI: 1.50–5.023, P = .001) and patients with positive SPECT (OR 3.34, 95% CI: 1.51–7.37, P = .003). The area under the receiver-operating characteristic curve for CAD in overall patients and patients with positive SPECT was 0.673 (95% CI: 0.603–0.743, P < .001) and 0.694 (95% CI: 0.602–0.786, P < .001), respectively. This study suggests that BNP level has additive diagnostic value to SPECT findings in predicting CAD in patients with normal LV systolic function

Successful Resection of a Giant Left Ventricular Pseudoaneurysm Developed Later after Mitral Valve Replacement

We present a case of successful surgical resection of a giant left ventricular (LV) pseudoaneurysm that developed 5 yr after mitral valve replacement (MVR). A 59-yr-old female was admitted with exertional chest pain radiating to left arm and back. 64-slice multidetector computed tomography (MDCT) revealed significant stenosis on the ostium of the first diagonal branch of the left anterior descending coronary artery and also a huge pseudoaneurysm compressing the right atrium and the inferior vena cava. She underwent resection of the pseudoaneurysm, and the pseudoaneurysm tunnel was repaired from the inside of LV cavity by removing the previously inserted prosthetic valve, followed by redo MVR together with coronary arterial bypass grafting (CABG) for a single-vessel disease. At the 6-month follow-up, the patient continued to do well without any complications

Acute Idiopathic Hemorrhagic Pericarditis with Cardiac Tamponade as the Initial Presentation of Acquired Immune Deficiency Syndrome

This paper presents a case of cardiac tamponade with idiopathic hemorrhagic pericarditis as the initial symptom of human immunodeficiency virus (HIV) infection. A 29-year-old male came to the emergency room with a sudden onset of dizziness. Upon arrival, he was hypotensive although not tachycardic, and his jugular venous pressure was not elevated. His chest X-rays revealed a mild cardiomegaly. Transthoracic echocardiography revealed a large amount of pericardial effusion with a diastolic collapse of the right ventricle, a dilated inferior vena cava with little change in respiration, and exaggerated respiratory variation of mitral inflow velocities, representing echocardiographic evidence of cardiac tamponade. After pericardiocentesis, his blood pressure improved to 110/70 mmHg without inotropics support. Serial 12-lead electrocardiograms during hospitalization revealed upwardly concave diffuse ST-segment elevation followed by a T-wave inversion suggestive of acute pericarditis. Pericardial fluid cytology and cultures for bacteria, mycobacteria, adenovirus, and fungus were all negative. HIV enzyme-linked immunosorbent assay (ELISA) was positive and confirmed by Western blot. The CD4 cell count was 168/mm3. Finally, the diagnosis of cardiac tamponade due to HIV-associated hemorrhagic pericarditis was made. It was concluded that HIV infection should be considered in the diagnosis of unexplained pericardial effusion or cardiac tamponade in Korea

Activated forkhead transcription factor inhibits neointimal hyperplasia after angioplasty through induction of p27

OBJECTIVE: We examined the effects of FKHRL1 (forkhead transcription factor in rhabdomyosarcoma like-1) overexpression on vascular smooth muscle cell (VSMC) proliferation, apoptosis, and cell cycle, in vitro, and the role of FKHRL1 and p27 in the pathophysiology of neointimal growth after balloon angioplasty, in vivo. Furthermore, we tested whether FKHRL1 overexpression can inhibit neointimal hyperplasia in a rat carotid artery model. METHODS AND RESULTS: Adenovirus expressing the constitutively active FKHRL1 (FKHRL1-TM; triple mutant) with 3 Akt phosphorylation sites mutated was transfected to subconfluent VSMCs. FKHRL1 overexpression in cultured VSMCs increased p27 expression, leading to G1 phase cell-cycle arrest and increased apoptosis. In vivo, the phosphorylation of FKHRL1 increased significantly 3 hours after balloon injury and decreased thereafter, with the subsequent downregulation of p27. Although the phosphorylation of FKHRL1 was greatest at 3 hours, the downregulation of p27 showed a temporal delay, only slightly starting to decrease after 3 hours and reaching a nadir at 72 hours after balloon injury. Gene transfer of FKHRL1-TM increased p27, decreased proliferation, and increased apoptosis of VSMCs, which resulted in a marked reduction in neointima formation (intima-to-media ratio: 0.31+/-0.13 versus 1.17+/-0.28, for FKHRL1-TM versus Adv-GFP; P<0.001). CONCLUSIONS: Balloon angioplasty leads to the phosphorylation of FKHRL1 and decreased expression of p27, thereby promoting a proliferative phenotype in VSMCs in vitro and in vivo. This study reveals the importance of FKHRL1 in proliferation and viability of VSMCs and suggests that it may serve as a molecular target for interventions to reduce neointima formation after angioplasty

Hypereosinophilia Presenting as Eosinophilic Vasculitis and Multiple Peripheral Artery Occlusions without Organ Involvement

We report here a case with hypereosinophilia and peripheral artery occlusion. A 32-yr-old Korean woman presented to us with lower extremity swelling and pain. Angiography revealed that multiple lower extremity arteries were occlusive. The biopsy specimen showed perivascular and periadnexal dense eosinophilic infiltration in dermis and subcutaneous adipose tissue. Laboratory investigations revealed a persistent hypereosinophilia. She was prescribed prednisolone 60 mg daily. Her skin lesion and pain were improved and the eosinophil count was dramatically decreased. After discharge, eosinophil count gradually increased again. Cyanosis and pain of her fingers recurred. She had been treated with cyclophosphamide pulse therapy. Her eosinophilia was decreased, but the cyanosis and tingling sense were progressive. The extremity arterial stenoses were slightly progressed. Skin biopsy showed perivascular eosinophilic infiltration in the dermis and CD40 ligand (CD40L) positive eosinophilic infiltration. The serum TNF-α was markedly increased. These results suggest that CD40L (a member of TNF-α superfamily) could play a role in the inflammatory processes when eosinophil infiltration and activation are observed. We prescribed prednisolone, cyclophosphamide, clopidogrel, cilostazol, beraprost and nifedipine, and she was discharged