Treatment of hypoplastic left heart syndrome: a systematic review and meta-analysis of randomised controlled trials.

BACKGROUND: This meta-analysis aimed to consolidate existing data from randomised controlled trials on hypoplastic left heart syndrome. METHODS: Hypoplastic left heart syndrome specific randomised controlled trials published between January 2005 and September 2021 in MEDLINE, EMBASE, and Cochrane databases were included. Regardless of clinical outcomes, we included all randomised controlled trials about hypoplastic left heart syndrome and categorised them according to their results. Two reviewers independently assessed for eligibility, relevance, and data extraction. The primary outcome was mortality after Norwood surgery. Study quality and heterogeneity were assessed. A random-effects model was used for analysis. RESULTS: Of the 33 included randomised controlled trials, 21 compared right ventricle-to-pulmonary artery shunt and modified Blalock-Taussig-Thomas shunt during the Norwood procedure, and 12 regarded medication, surgical strategy, cardiopulmonary bypass tactics, and ICU management. Survival rates up to 1 year were superior in the right ventricle-to-pulmonary artery shunt group; this difference began to disappear at 3 years and remained unchanged until 6 years. The right ventricle-to-pulmonary artery shunt group had a significantly higher reintervention rate from the interstage to the 6-year follow-up period. Right ventricular function was better in the modified Blalock-Taussig-Thomas shunt group 1-3 years after the Norwood procedure, but its superiority diminished in the 6-year follow-up. Randomised controlled trials regarding medical treatment, surgical strategy during cardiopulmonary bypass, and ICU management yielded insignificant results. CONCLUSIONS: Although right ventricle-to-pulmonary artery shunt appeared to be superior in the early period, the two shunts applied during the Norwood procedure demonstrated comparable long-term prognosis despite high reintervention rates in right ventricle-to-pulmonary artery shunt due to pulmonary artery stenosis. For medical/perioperative management of hypoplastic left heart syndrome, further randomised controlled trials are needed to deliver specific evidence-based recommendations.

Uterine cervical carcinoma: a comparison of two- and three-dimensional T2-weighted turbo spin-echo MR imaging at 3.0 T for image quality and local-regional staging.

OBJECTIVE: To compare three-dimensional (3D) T2-weighted turbo spin-echo (TSE) with multiplanar two-dimensional (2D) T2-weighted TSE for the evaluation of invasive cervical carcinoma. METHODS: Seventy-five patients with cervical carcinoma underwent MRI of the pelvis at 3.0 T, using both 5-mm-thick multiplanar 2D (total acquisition time = 12 min 25 s) and 1-mm-thick coronal 3D T2-weighted TSE sequences (7 min 20 s). Quantitative analysis of signal-to-noise ratio (SNR) and qualitative analysis of image quality were performed. Local-regional staging was performed in 45 patients who underwent radical hysterectomy. RESULTS: The estimated SNR of cervical carcinoma and the relative tumour contrast were significantly higher on 3D imaging (P < 0.0001). Tumour conspicuity was better with the 3D sequence, but the sharpness of tumour margin was better with the 2D sequence. No significant difference in overall image quality was noted between the two sequences (P = 0.38). There were no significant differences in terms of the diagnostic accuracy, sensitivity, and specificity of parametrial invasion, vaginal invasion, and lymph node metastases. CONCLUSION: Multiplanar reconstruction 3D T2-weighted imaging is largely equivalent to 2D T2-weighted imaging for overall image quality and staging accuracy of cervical carcinoma with a shorter MR data acquisition, but has limitations with regard to the sharpness of the tumour margin.

Application of different prognostic scoring systems and comparison of the FAB and WHO classifications in Korean patients with myelodysplastic syndrome.

We retrospectively studied 227 patients with MDS (1) to identify the prognostic factors of survival and acute leukemia evolution in Korean patients with MDS, (2) to apply different prognostic scoring systems to the same group of patients, and (3) to compare the FAB with the WHO classification. Six scoring systems were applied to the patients, and the FAB and WHO classifications were compared. The patients' median age was 57 years. The median survival time was 21 months, and age, dysgranulopoiesis and the IPSS cytogenetic groups were independent prognostic factors for survival. Acute leukemia occurred in 34 patients, and the cumulative incidence was 27.1% at 3 years. Marrow blast percentage was the only independent prognostic factor for acute leukemia evolution. Most scoring systems successfully discriminated risk groups for survival and acute leukemia evolution, but patient distribution into risk groups varied according to the scoring systems. Refractory cytopenia with multilineage dysplasia and RAEB II seemed to have different prognoses from RA or RARS and RAEB I, respectively. In summary, our MDS patients had different disease natures from those of Western countries regarding clinical features, prognostic factors and cytogenetic profiles. Although the WHO classification seems to improve the FAB classification, further studies are warranted to validate the utility of the WHO classification before it is accepted for routine clinical use. Our study has the limitations of retrospective analysis, and our results should be verified in future prospective studies.

Spontaneous remission of aplastic anemia: a retrospective analysis.

BACKGROUND AND OBJECTIVES: Although acquired aplastic anemia is usually a chronic disorder, a small proportion of patients with aplastic anemia was reported to recover spontaneously without specific therapy such as allogeneic bone marrow transplantation or immunosuppressive treatment. We attempted to determine the rate of spontaneous remission in the patients with AA. DESIGN AND METHODS: We reviewed the medical records of adult patients (n=136) who were diagnosed as aplastic anemia at the Asan Medical Center from 1990 to 2000. The hematologic criteria of spontaneous remission were defined according to the proposed remission criteria of the SAA Working Party of the EBMT. RESULTS: Eighteen (13%) of 136 patients satisfied the criteria for spontaneous remission at median 14 days (range, 4-332) from the diagnosis of aplastic anemia. In fifteen (83%) of 18 patients, spontaneous remission occurred within 50 days. Spontaneous remission was complete in fourteen patients (78%). Only two patients relapsed at 208 and 1,857 days after spontaneous remission, respectively. Multivariate logistic regression analysis showed that presence of infection at diagnosis (p = 0.017), drug as an etiology of aplastic anemia (p = 0.028), and serum albumin level less than 3.4 g/dL (p = 0.005) were independent predictive factors for spontaneous remission. INTERPRETATION AND CONCLUSIONS: Our study confirmed that a minority of patients with AA recovered spontaneously. Spontaneous remission was rapid and complete in most patients, and relapses were rarely observed. In most cases with spontaneous remission, aplastic anemia might be associated with external factors such as drug or infection.

Blunt traumatic rupture of the diaphragm: sonographic diagnosis.

Ultrasonographic features of seven patients with diaphragmatic rupture due to blunt trauma were analyzed. The ruptures occurred at the left hemidiaphragm in four patients and at the right in three. Direct ultrasonographic findings were as follows: disrupted diaphragm in four patients; nonvisualized diaphragm in three patients; floating diaphragm in two patients; and herniation of the liver or bowel loops through the diaphragmatic defect in three patients. Indirect sonographic findings included pleural effusion or subphrenic fluid collection in five patients and splenic laceration in one. Although the number of patients was limited, ultrasonography was very useful for the diagnosis of diaphragmatic rupture.