Background A circular muscle myotomy preserving the longitudinal outer esophageal muscular layer is often recommended during peroral endoscopic myotomy (POEM) for achalasia. However, because the ...longitudinal muscle fibers of the esophagus are extremely thin and fragile, and completeness of myotomy is the basis for the excellent results of conventional surgical myotomy, this modification needs to be further debated. Here, we retrospectively analyzed our prospectively maintained POEM database to compare the outcomes of endoscopic full-thickness and circular muscle myotomy. Study Design According to the myotomy depth, 103 patients with full-thickness myotomy were assigned to group A, while 131 patients with circular muscle myotomy were assigned to group B. Symptom relief, procedure-related parameters and adverse events, manometry outcomes, and reflux complications were compared between groups. Results The mean operation times were significantly shorter in group A compared with group B (p = 0.02). There was no increase in any procedure-related adverse event after full-thickness myotomy (all p < 0.05). During follow-up, treatment success (Eckardt score ≤ 3) persisted for 96.0% (95 of 99) of patients in group A and for 95.0% (115 of 121) of patients in group B (p = 0.75). There were no statistically significant differences in pre- and post-treatment D-value of symptom scores and lower esophageal sphincter pressures between groups (both p > 0.05). The overall clinical reflux complication rates were also similar (21.2% vs 16.5%, p = 0.38). Conclusions Short-term symptom relief and manometry outcomes of each method were comparable. Full-thickness myotomy significantly reduced the procedure time but did not increase the procedure-related adverse events or clinical reflux complications.
Background Peroral endoscopic myotomy (POEM) has been developed to provide a less-invasive myotomy for achalasia in adults but seldom has been used in pediatric patients. Objective To evaluate the ...feasibility, safety, and efficacy of POEM for pediatric patients with achalasia. Design Single-center, prospective study. Setting Academic medical center. Patients A total of 27 pediatric patients (mean age 13.8 years, range 6-17 years) with achalasia. Interventions POEM. Main Outcome Measurements The primary outcome was symptom relief during follow-up, defined as an Eckardt score of ≤3. Secondary outcomes were procedure-related adverse events, clinical reflux adverse events, and lower esophageal sphincter (LES) pressure on manometry before and after POEM. Results A total of 26 cases (96.3%) underwent successful POEM. A submucosal tunnelling attempt failed in 1 case because of serious inflammation and adhesion. No serious adverse events related to POEM were encountered. During a mean follow-up period of 24.6 months (range 15-38 months), treatment success was achieved in all patients (mean score before vs after treatment 8.3 vs 0.7; P < .001). Mean LES pressure also decreased from a mean of 31.6 mm Hg to 12.9 mm Hg after POEM ( P < .001). Five patients developed clinical reflux adverse events (19.2%). Limitations Single center and lack of some objective evaluations. Conclusion This relatively long-term follow-up study adds to the evidence that POEM seems to be a promising new treatment for pediatric patients with achalasia, resulting in long-term symptom relief in all cases and without serious adverse events.
Background Given the high morbidity and mortality rates for surgery and the diminishment of quality of life caused by operative resection of the gastric cardia, a minor invasive treatment without ...loss of curability is desirable for submucosal tumors (SMTs) of the esophagogastric junction (EGJ). Endoscopic submucosal dissection (ESD) has been used successfully for the removal of esophageal or gastric SMTs; however, the EGJ has been regarded as a difficult location for ESD because of its narrow lumen and sharp angle. Objective To evaluate the clinical impact of ESD for SMTs of the EGJ arising from the muscularis propria layer. Design Single-center, prospective study. Setting Academic medical center. Patients 143 patients with 143 SMTs of the EGJ originating from the muscularis propria layer. Interventions ESD. Main Outcome Measurements Complications, en bloc resection rate, local recurrence, and distant metastases. Results The average maximum diameter of the lesions was 17.6 mm (range 5 - 50 mm). The en bloc resection rate was 94.4% (135/143). All en bloc resection lesions showed both lateral and deep tumor-free margins, including 20 GI stromal tumors. Perforations occurred in 6 patients (4.2%, 6/143), and metal clips were used to occlude the defect. Four pneumoperitoneum and 2 pneumothorax caused by perforations were resolved with nonsurgical treatment. Local recurrence and distant metastasis have not occurred during a 2-year follow-up. Limitations Single-center, short follow-up. Conclusions ESD appears to be a safe, feasible, and effective procedure for providing accurate histopathologic evaluations, as well as curative treatments for SMTs of the EGJ originating from the muscularis propria layer.
Supraventricular arrhythmias (SVA) may be risk factors of prognosis in patients with pulmonary arterial hypertension. SVA are increasingly reported in patients with pulmonary hypertension, but little ...is known about their incidence and outcomes for patients with idiopathic pulmonary arterial hypertension (IPAH) in a large cohort. In this 6-year prospective multicenter study, 280 patients with IPAH were enrolled to investigate the incidence of SVA and assess risk factors, clinical manifestation, management, and impact on survival. The cumulative 6-year incidence of SVA was 15.8%. The most common types of SVA were atrial fibrillation (n = 16) and atrial flutter (n = 13), followed by atrial tachycardia (n = 11). Most episodes of SVA were associated with significant clinical deterioration and right-sided cardiac failure. Sinus rhythm was successfully restored in most patients, resulting in clinical recovery. Increased right ventricular diameter, left atrial area, and higher right atrial pressure and pulmonary vascular resistance were associated with increased risk for experiencing SVA. SVA predicted a greater risk for mortality in a stepwise forward Cox analysis (hazard ratio 4.757, 95% confidence interval 2.695 to 8.397, p <0.001). Kaplan-Meier survival curves showed that patients with SVA, mainly permanent SVA, had a lower survival rate than those who did not develop SVA (p = 0.008). In conclusion, SVA often lead to clinical deterioration and may be associated with an increased risk for death in a large cohort of patients with IPAH. Restoration and maintenance of sinus rhythm are important treatment goals in patients with IPAH.
Abstract Background Both low tube voltage and sinogram-affirmed iterative reconstruction (IR) techniques hold promise to decrease radiation dose at coronary CT angiography (CCTA). The increased ...iodine contrast at low tube voltage allows for minimizing iodine load. Objective To assess the effect of reduced x-ray tube voltage, low iodine concentration contrast medium and IR on image quality and radiation dose at CCTA. Methods Two hundred thirty-one consecutive patients with suspected coronary artery disease were enrolled in this prospective, multicenter trial and randomized to 1 of 2 dual-source CCTA protocols: 120-kVp with 370 mgI/mL iopromide or iopamidol (n = 116; 44 women; 55.3 ± 9.8 years) or 100 kVp with 270 mgI/mL iodixanol (n = 115; 48 women; 54.2 ± 10.4 years). Reconstruction was performed with filtered back projection and IR. Attenuation, image noise, signal-to-noise ratio, and contrast-to-noise ratio were measured and image quality scored. Size-specific dose estimates and effective doses were calculated. Results There were no significant differences in mean arterial attenuation (406.6 ± 76.7 vs 409.7 ± 65.2 Hounsfield units; P = .739), image noise (18.7 ± 3.8 vs 17.9 ± 3.4 Hounsfield units; P = .138), signal-to-noise ratio (22.5 ± 5.4 vs 23.7 ± 6.1; P = .126), contrast-to-noise ratio (17.5 ± 5.5 vs 18.3 ± 6.1; P = .286), or image quality scores (4.1 ± 0.9 vs 4.0 ± 0.9; P > .05) between 120-kVp filtered back projection–reconstructed and 100-kVp IR-reconstructed series. Mean iodine dose was 26.5% lower (18.3 ± 0.5 vs 24.9 ± 0.9 g; P < .0001), mean size-specific dose estimate was 35.1% lower (17.9 ± 6.6 vs 27.5 ± 8.2 mGy; P < .0001), and effective dose was 34.9% lower (2.3 ± 1.0 vs 3.5 ± 1.1 mSv; P < .0001) with the 100 kVp compared with the 120-kVp protocol, respectively. Conclusion Using low x-ray tube voltage and IR allows for decreasing the iodine load and effective radiation dose at CCTA while maintaining image quality.
Transcatheter closure of ventricular septal defects (VSDs) is now offered as primary therapy at many institutions. We sought to evaluate the clinical feasibility and safety of device closure of ...intracristal VSDs using perimembranous occluders. A total of 49 patients were diagnosed with intracristal VSDs and assigned to the intracristal VSD group, and another 49 patients with the same size perimembranous VSDs were selected and assigned to the perimembranous VSD group. Two types of perimembranous occluders, symmetric and asymmetric, were used, and no difference was found between the groups with respect to successful closure. The diameter of the intracristal VSD was 3 to 10 mm (mean 5.8 ± 1.4) on the transthoracic echocardiogram. The procedure time and fluoroscope time in the intracristal VSD group were significantly greater than those in the perimembranous VSD group. More defects with a subaortic rim ≤2 mm on the transthoracic echocardiogram were present in the intracristal VSD group than in the perimembranous VSD group; thus, more asymmetric occluders were used in the intracristal VSD group. All devices remained in a stable position and in an optimal shape during follow-up. In conclusion, transcatheter closure of intracristal VSDs with the perimembranous occluder is feasible, safe, and effective.
Abstract Background PRKAG2 gene encodes the γ2 regulatory subunit of AMP-activated protein kinase (AMPK) that acts as a sensor of cellular energy status, and its germline mutations are responsible ...for PRKAG2 cardiac syndrome (PCS). The majority of missense mutations of cystathionine beta-synthase (CBS) domains found in PCS impair the binding activity of PRKAG2 to adenosine derivatives, and therefore lead to PRKAG2 function impairment and AMPK activity alteration, resulting in a familial syndrome of ventricular preexcitation, conduction defects, and cardiac hypertrophy. However, it is unclear about the PRKAG2 mutation in the non-CBS domain. Here, a Chinese family exhibiting the cardiac syndrome associated with a novel heterozygous PRKAG2 mutation (Gly100Ser) mapped to exon 3 encoding a non-CBS domain is described and the function of this novel mutation was investigated in vitro. Methods The PRKAG2 G100S and R302Q mutations were constructed by a two-step polymerase chain reaction and then transfected into CCL13 cells by lentivirus vectors. Wild-type PRKAG2 gene transfection was used as a negative control. PRKAG2 expression was determined by Western blot. Immunofluorescence was used to localize the intracellular PRKAG2 proteins. MTT assay was performed to explore the effect of mutations on cell proliferation. Periodic acid-Schiff staining was used for detecting glycogen accumulation. AMPK concentration was measured with enzyme-linked immunosorbent assay. Results Our results showed neither intracellular localization of PRKAG2 nor cell growth was altered. In contrast, PRKAG2 protein expression levels were significantly reduced by this mutation. Furthermore, PRKAG2-mediated activity of AMPK was attenuated, resulting in glycogen metabolism dysregulation. These findings revealed that non-CBS domains of PRKAG2 were essential to the regulation of AMPK activity, similar to CBS. Conclusions Our study ascribes a crucial regulatory role to the novel PRKAG2 G100S mutation, and reiterates that PCS occurs as a consequence of AMPK signaling abnormality caused by PRKAG2 gene mutations.
Summary Port wine stain (PWS) birthmarks are congenital vascular malformations characterized by ectatic capillaries in the papillary layer of the dermis. They usually appear at birth and tend to ...become darker and thicker with age. Cosmetic and pathological changes can cause great depression in all ages. Laser-mediated selective photothermolysis is the treatment of choice but vascular-targeted photodynamic therapy (PDT) might be an alternative approach in the treatment of PWS. PDT has shown therapeutic benefit in pink and resistant lesions. In theory, vascular-targeted PDT may be more efficient in terms of better lightening and less treatment sessions than pulsed dye laser (PDL) irradiation. Chinese clinicians have gained profound knowledge and accumulated a large body of clinical experience in PWS PDT in the past years. The pursuing of regulatory approval on PWS PDT is currently underway. In this article we will report our experience in PWS PDT, discuss current PDT protocols and introduce the novel combination therapy, such as PDT + PDL and PDT + topical angiogenic inhibitor.
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