Partial anomalous pulmonary venous return (PAPVR) is a rare congenital cardiac anomaly that can be difficult to detect and often remains undiagnosed. PAPVR is diagnosed using non-invasive imaging techniques such as echocardiography, CT, and MRI. Image data are reviewed on a 2-dimensional (D) monitor, which may not facilitate a good understanding of the complex 3D heart structure. In recent years, 3D printing technology, which allows the creation of physical cardiac models using source image datasets obtained from cardiac CT or MRI, has been increasingly used in the medical field. We report a case involving a 3D-printed model of PAPVR with a biatrial connection. This model demonstrated separate drainages of the right upper and middle pulmonary veins into the lower superior vena cava (SVC) and the junction between the SVC and the right atrium, respectively, with biatrial communication through the right middle pulmonary vein.

Partial anomalous pulmonary venous return (PAPVR) is a rare congenital cardiac anomaly that can be difficult to detect and often remains undiagnosed. PAPVR is diagnosed using non-invasive imaging techniques such as echocardiography, CT, and MRI. Image data are reviewed on a 2-dimensional (D) monitor, which may not facilitate a good understanding of the complex 3D heart structure. In recent years, 3D printing technology, which allows the creation of physical cardiac models using source image datasets obtained from cardiac CT or MRI, has been increasingly used in the medical field. We report a case involving a 3D-printed model of PAPVR with a biatrial connection. This model demonstrated separate drainages of the right upper and middle pulmonary veins into the lower superior vena cava (SVC) and the junction between the SVC and the right atrium, respectively, with biatrial communication through the right middle pulmonary vein.

BACKGROUND AND OBJECTIVES: The early assessment of treatment is not done for benign paroxysmal positional vertigo (BPPV) since the well-known phenomenon of fatigability after a repeated positional test can mimic successful treatment. The aim of this study is to evaluate the clinical implication of ‘fatigability’ after Epley maneuver and to identify the therapeutic efficacy of Epley maneuver in posterior canal BPPV (PC-BPPV). SUBJECTS AND METHOD: This study was prospectively conducted by two dizziness clinics on 51 consecutive patients diagnosed with PC-BPPV. All patients included in the study received Epley maneuver treatment. The therapeutic results were reassessed immediately after a single trial of Epley maneuver. After 30 minutes, results were reassessed repeatedly to confirm the fatigability of diagnostic procedure immediately after treatment. If the treatment was not successful after 30 minutes, Epley maneuver was repeatedly performed until complete resolution. RESULTS: Immediately after the first maneuver, 45 of 51 (88.2%) patients had neither vertigo nor nystagmus during the positional test. All patients demonstrated complete resolution after receiving one to three Epley maneuvers on the day of diagnosis. ‘Fatigability (false negative result)’ was confirmed for only one case (1 of 6 patients, 16.7%), in which nystagmus was observed after 30 minutes but not identified immediately after the first Epley maneuver. CONCLUSION: The therapeutic efficacy of Epley maneuver is very high in PC-BPPV. Considering the possibility of fatigability when reassessment is performed immediately after therapeutic maneuver, clinicians should avoid assessing the outcome immediately after treatment in patients with PC-BPPV.
Benign Paroxysmal Positional Vertigo* ; Diagnosis ; Dizziness ; Fatigue ; Humans ; Methods ; Prospective Studies ; Vertigo

The objectives of this study were to assess the clinical outcomes of adults with Ebstein Anomaly (EA) according to their treatment modalities. All adult EA patients diagnosed between October 1994 and October 2014 were retrospectively evaluated by medical record review. Total 60 patients were categorized into 3 groups according to their treatment strategy, i.e. non-operative treatment (Group I, n = 23), immediate operative treatment (Group II, n = 27), and delayed operative treatment (Group III, n = 10). A composite of major adverse cardiac and cerebrovascular events (MACCE) and factors associated with MACCE were assessed in each treatment group. MACCE occurred in 13.0% patients in Group I, 55.6% patients in Group II and 50% in Group III (P = 0.006). Event free survivals at 5 years were 90% in Group I, 52.7% in Group II, 50.0% in Group III (P = 0.036). Post-operatively, most patients showed improvement on clinical symptoms. However, event free survival rate was lower in patients with operation compared to those with non-operative treatment (58.7% vs. 90.9%; P = 0.007). Major arrhythmic event occurred more frequently even after surgical ablation (50.0% vs. 20.0%; P = 0.034). Re-operation was more frequent in patients underwent delayed surgery compared to those with immediate surgery (50.0% vs. 18.5%; P = 0.001). Current guideline to decide patient's treatment strategy appeared to be appropriate in adult patients with EA. However, surgical ablation for arrhythmia was not enough so that concomitant medical treatment should be considered. Therefore, attentive risk stratification and cautious decision of treatment strategy by experienced cardiac surgeon are believed to improve clinical outcome.
Adult* ; Arrhythmias, Cardiac ; Disease-Free Survival ; Ebstein Anomaly* ; Humans ; Medical Records ; Retrospective Studies

p21-activated kinases (PAKs) are key regulators of actin dynamics, cell proliferation and cell survival. Deregulation of PAK activity contributes to the pathogenesis of various human diseases, including cancer and neurological disorders. Using an ELISA-based screening protocol, we identified naphtho(hydro)quinone-based small molecules that allosterically inhibit PAK activity. These molecules interfere with the interactions between the p21-binding domain (PBD) of PAK1 and Rho GTPases by binding to the PBD. Importantly, they inhibit the activity of full-length PAKs and are selective for PAK1 and PAK3 in vitro and in living cells. These compounds may potentially be useful for determining the details of the PAK signaling pathway and may also be used as lead molecules in the development of more selective and potent PAK inhibitors.
Actins ; Cell Proliferation ; Cell Survival ; Humans ; In Vitro Techniques ; Mass Screening ; Nervous System Diseases ; p21-Activated Kinases ; Phosphotransferases* ; rho GTP-Binding Proteins

p21-activated kinases (PAKs) are key regulators of actin dynamics, cell proliferation and cell survival. Deregulation of PAK activity contributes to the pathogenesis of various human diseases, including cancer and neurological disorders. Using an ELISA-based screening protocol, we identified naphtho(hydro)quinone-based small molecules that allosterically inhibit PAK activity. These molecules interfere with the interactions between the p21-binding domain (PBD) of PAK1 and Rho GTPases by binding to the PBD. Importantly, they inhibit the activity of full-length PAKs and are selective for PAK1 and PAK3 in vitro and in living cells. These compounds may potentially be useful for determining the details of the PAK signaling pathway and may also be used as lead molecules in the development of more selective and potent PAK inhibitors.
Actins ; Cell Proliferation ; Cell Survival ; Humans ; In Vitro Techniques ; Mass Screening ; Nervous System Diseases ; p21-Activated Kinases ; Phosphotransferases* ; rho GTP-Binding Proteins

We report a case of the isolation of the Aspergillus versicolor complex, initially misidentified by morphological characteristics as the Scopulariopsis species, from a homograft with a bicuspidalized pulmonary valve. An eighteen-month-old female, who had critical pulmonary stenosis, underwent pulmonary valve replacement. On postoperative day 8, she developed a fever, which did not respond to empiric broad-spectrum antibiotics. While no definitive source was identified, a filamentous fungus was isolated from the thawed homograft tissue culture prior to implantation on the operation day. The colonies were powdery green with white edges on Sabouraud dextrose agar. Microscopic examination showed septate hyphae with branched conidiophores and chains of spiny conidia, which suggested Scopulariopsis species. After direct sequencing of the internal transcribed spacer (ITS) regions, the fungus was identified as the A. versicolor complex. To our knowledge, the isolation of the A. versicolor complex from a homograft valve has not been previously described. This case shows that laboratory staff should be aware that microscopic morphology of the A. versicolor complex can resemble that of a number of other genera, including Scopulariopsis species.
Agar ; Anti-Bacterial Agents ; Aspergillus ; Bicuspid ; European Continental Ancestry Group ; Female ; Fever ; Fungi ; Glucose ; Humans ; Hyphae ; Pulmonary Valve ; Pulmonary Valve Stenosis ; Scopulariopsis ; Spores, Fungal ; Transplantation, Homologous

The congenital complete atrioventricular block(CCAVB) is a rare disease, which is the most serious complication of neonatal lupus erythematosus. Newborn with CCAVB are at risk of diminished cardiac output and the subsequent development of congestive heart failure. Transplacental steroid and beta-adrenergic agonist therapies are useful for the first and second degree atrioventricular block. But those therapies are usually not effective for complete atrioventricular block. If the fetus has a complete atrioventricular block, delivery should be considered unless other delivery contraindications. In this situation, early pacemaker implantation surgery can improve the survival rate of patients. We report one case of premature newborn with congenital complete atrioventricular block who is successfully recovered by pacemaker implantation.
Adrenergic beta-Agonists ; Atrioventricular Block ; Cardiac Output ; Fetus ; Heart Failure ; Humans ; Infant, Newborn ; Lupus Erythematosus, Systemic ; Rare Diseases ; Survival Rate

PURPOSE: This study analyzed the type of acute urinary retention (AUR) and evaluated the treatments used, including trial without catheter (TWOC). MATERIALS AND METHODS: This study was based on 299 patients who were treated for AUR from January 2007 to August 2009. The patients were classified into the spontaneous AUR group (group S) and the precipitated AUR group (group P), in which AUR was consecutive to triggering events. The treatment modalities including TWOC, the success rate of TWOC, age, prostate-specific antigen (PSA) levels, the volume of the prostate, and the drained volume at catheterization were analyzed in each group. RESULTS: Of 299 men with AUR, 160 (54%) had spontaneous AUR and 139 (46%) had precipitated AUR. Compared with group P, patients in group S were more likely to be treated by surgery, either immediately (16.9% vs. 3.6%, p<0.05) or after prolonged catheterization (42.2% vs. 29.1%, p<0.05). The success rate of TWOC was lower in men of older ages (> or =70 years) and in those with enlarged prostates (> or =50 ml), higher PSA levels (> or =3 ng/ml), and a large drained volume at catheterization (> or =1,000 ml). CONCLUSIONS: In this group of AUR patients, there were slightly more patients with spontaneous AUR (54%) than with precipitated AUR (46%). The success rate of TWOC was more than 70% regardless of the type of AUR. Although TWOC is recommended primarily in the treatment of AUR, early surgical intervention should be considered if the patient has an enlarged prostate (> or =50 ml) or a large drained volume at catheterization (> or =1,000 ml).
Catheterization ; Catheters ; Humans ; Male ; Prostate ; Prostate-Specific Antigen ; Prostatic Hyperplasia ; Urinary Catheterization ; Urinary Retention

Early spontaneous recanalization of the middle cerebral artery in acute ischemic phase artery is not uncommon, whereas the late spontaneous recanalization of chronic occluded artery is a very rare phenomenon and exact incidence and the timing of this event have not been quantified. We present a case in which late spontaneous recanalization of long-lasting middle cerebral artery occlusion occurred in the absence of surgical, endovascular and thrombolytic treatments.
Angiography ; Arteries ; Incidence ; Infarction, Middle Cerebral Artery ; Middle Cerebral Artery