A Rare Variant of Hypogenetic Lung Syndrome Mimicking Scimitar Vein.

Hypogenetic lung syndrome, also known as scimitar syndrome, is a rare and well-known congenital condition that includes hypoplastic right pulmonary artery and lung, right displacement of the heart, anomalous systemic arterial supply to the lung, and a characteristically curved anomalous right pulmonary vein draining into the inferior vena cava. In exceptional cases, the anomalous pulmonary vein may drain into left atrium. We here report a case of a 17-year-old girl with a rare variant of hypogenetic lung syndrome diagnosed by means of multimodality imaging and treated with percutaneous occlusion of the aortopulmonary collateral.

Outcomes of Surgery for Mixed Total Anomalous Pulmonary Venous Drainage in Children.

Total anomalous pulmonary venous drainage (TAPVD) is an uncommon cardiac defect in children. The mixed subset accounts for 5%-10% of the TAPVD and is variable in its anatomy. The outcomes associated with this subset of patients are rarely reported. A retrospective review of all patients with mixed TAPVD undergoing repair at a single institution (1984-2014) was conducted. A descriptive analysis was performed. Twenty-four patients underwent repair for mixed TAPVD (6 univentricular physiology, 18 biventricular physiology). The mixed TAPVD anatomy included 8 patients in group I (2 + 2 veins), 11 patients in group II (3 + 1 veins), and 5 patients in group III (atypical). Preoperative pulmonary venous obstruction occurred in 8 patients (33%). The median age at repair was 2.2 months (range 2 days to 3 years) and median weight was 4.2 kg (range 1.9 to 12.5 kg). Operative mortality was 13% (3 of 24), 33.3% (2 of 6) for patients with univentricular physiology, and 5.6% (1 of 18) for patients with biventricular physiology. There have been no operative deaths in the biventricular group since 1997 (n = 11). Survival at 30-days was 83% ± 15% (95% confidence interval: 27%-97%) and 94% ± 5% (95% confidence interval: 67%-99%) for the univentricular and biventricular groups, respectively. Reoperation for recurrent pulmonary venous obstruction was required in 2 patients (8.3%) where the sutureless technique was used. The average follow-up after surgery was 9.3 ± 6.4 years (5 months to 21 years), and all surviving patients were asymptomatic. Mixed TAPVD can be repaired with good results in children, particularly in those undergoing biventricular repair.

A rare variant of intracardiac total anomalous pulmonary venous connection.

Total anomalous pulmonary venous connection (TAPVC) with direct connection of the pulmonary veins to the morphologically right atrium is exceedingly rare other than in the setting of isomerism of the right atrial appendages. We present an interesting case of TAPVC in a patient with situs solitus that connected to the right atrium via a broad-mouthed common chamber.

A 30-year experience with mixed-type total anomalous pulmonary venous connection: a word of caution.

BACKGROUND: Patients with total anomalous pulmonary venous connection can be problematic, particularly those with mixed-type pathology. We aimed to describe a cohort of patients with mixed-type anomalous drainage, highlighting the treatment challenges, and identifying risk factors for poor outcome. METHODS: We reviewed the clinical records of patients who underwent repair for mixed-type total anomalous pulmonary venous connection between 1986 and 2015. RESULTS: A total of 19 patients were identified. The median age and weight of patients at surgery were 18 days (with a range from 1 to 185) and 3.4 kg (with a range from 1.9 to 6.5), respectively. Venous anatomy included a combination of duplicate supracardiac (four), supracardiac and cardiac (11), and supracardiac and infracardiac (four) drainage. Out of 19 patients, six (32%) died within 30 days or the initial hospital stay; two additional patients died from progressive pulmonary vein stenosis at 72 and 201 days, respectively, resulting in 42% mortality within the 1st year. Follow-up data were available for 8/11 long-term survivors. The median follow-up period was 7.3 years (with a range from 1.8 to 15.7). Only one patient underwent re-intervention for recurrent pulmonary vein stenosis. For surgical mortality, no statistically significant risk factors were identified, although the risk trended to be higher (p⩽0.1) with lower age and weight, an infracardiac component, and prolonged cardiopulmonary bypass. For 1-year mortality, the risk became significant (p⩽0.05) with a lower weight (p=0.01), an infracardiac component (p=0.03), and prolonged cardiopulmonary bypass (p=0.04). CONCLUSION: The surgical and 1-year mortality in patients with mixed-type total anomalous pulmonary venous connection is high. On the other hand, among patients who survive past the 1st year, most have good outcomes without subsequent sequelae.

Standardized likelihood ratio test for comparing several log-normal means and confidence interval for the common mean.

Standardized likelihood ratio test (SLRT) for testing the equality of means of several log-normal distributions is proposed. The properties of the SLRT and an available modified likelihood ratio test (MLRT) and a generalized variable (GV) test are evaluated by Monte Carlo simulation and compared. Evaluation studies indicate that the SLRT is accurate even for small samples, whereas the MLRT could be quite liberal for some parameter values, and the GV test is in general conservative and less powerful than the SLRT. Furthermore, a closed-form approximate confidence interval for the common mean of several log-normal distributions is developed using the method of variance estimate recovery, and compared with the generalized confidence interval with respect to coverage probabilities and precision. Simulation studies indicate that the proposed confidence interval is accurate and better than the generalized confidence interval in terms of coverage probabilities. The methods are illustrated using two examples.