New insights learned from the pulmonary to systemic blood flow ratio to predict the outcome in patients with hypoplastic left heart syndrome in the pre-Glenn stage: a single-center study

BackgroundTo the best of our knowledge, no study has been made until now to determine whether the ratio between pulmonary and systemic blood flow (Qp/Qs) in the pre-stage II (PS2) or pre-Glenn stage can predict the outcome in patients with hypoplastic left heart syndrome (HLHS) who underwent Norwood (NW) palliation.Patients and methodsFrom January 2016 to August 2022, 80 cardiac catheterizations in 69 patients with HLHS in NW palliation stage with modified Blalock–Taussig shunt (MBTS) were retrospectively recruited. The Qp/Qs was measured under stable conditions using the Fick formula. None of the patients were intubated. Patients were divided into two groups: Group 1 included patients who underwent planned cardiac catheterization (n = 56), and Group 2 had unplanned examination (n = 13), in which the indication for cardiac catheterization was desaturation in 11 patients and pulmonary over-circulation in two. The composite primary outcome was defined as accomplishing the planned operations (Glenn and Fontan) with freedom from death and reoperation, referring to palliative therapy or heart transplantation. The secondary outcome was freedom from transcatheter intervention in MBTS or pulmonary arteries.ResultsThe median follow-up was 48 months (range 6–72 months). The median value of Qp/Qs in Group 1 was 1.75 (range 1.5–2.2). In Group 2, the 11 patients with desaturation, the median value of Qp/Qs was 1.25 (range 0.9–1.45). The two patients with suspected pulmonary overcalculation showed Qp/Qs of 2.3 and 2.5, respectively; a reduction of the shunt size was required. Approximately 96.4% of patients in Group 1 achieved the primary outcome compared with only 30.7% in Group 2. The need for reintervention was 1.8% in Group 1 compared with 61.3% in Group 2. There is a significant relationship between Qp/Qs and the impaired outcome (death, palliative therapy, or heart transplantation) with a p-value of 0.001, a relative risk factor of 3.1, and a 95% confidence interval of 1.4–7.1. No significant relationship between the Qp/Qs and the size of MBTS (p-value of 0.073) was noted.ConclusionThe Qp/Qs in PS2 can predict outcomes in patients with HLHS in Norwood stage with MBTS. The Qp/Qs between 1.5 and 2.2 with a median of 1.75 seems to be optimal in the patients in PS2. Qp/Qs of <1.5 is associated with pulmonary stenosis, shunt stenosis, and pulmonary hypertension

All-oral low-dose chemotherapy TEPIP is effective and well-tolerated in patients with peripheral T-cell lymphoma

Purpose: Peripheral T-cell lymphoma (PTCL) is a rare and heterogenous hematologic malignancy with poor prognosis especially in elderly and frail patients who are not eligible for intensive treatment. The resulting palliative setting necessitates tolerable but effective schedules for outpatient treatment. TEPIP is a locally developed, all-oral low-dose regimen comprising trofosfamide, etoposide, procarbazine, idarubicin, and prednisolone. Methods: In this observational retrospective, single-center study, the safety and efficacy of TEPIP was evaluated in 12 patients (pts.) with PTCL treated at the University Medical Center Regensburg between 2010 and 2022. The endpoints were overall response rate (ORR) and overall survival (OS), and adverse events were individually reported according to the Common Terminology Criteria for Adverse Events (CTCAE) criteria. Results: The enrolled cohort was characterized by advanced age (median 70 years), extensive disease (100% Ann Arbor ≥stage 3), and poor prognosis (75% high/high-intermediate international prognostic index). The most common subtype was angioimmunoblastic T-cell lymphoma (8/12), and 11/12 patients had relapsed or refractory disease at TEPIP onset with a median of 1.5 prior treatment regimens. After a median of 2.5 TEPIP cycles (total of 83 cycles), the ORR was 42% (complete remission 25%), and the OS reached a median of 185 days. Any grade of adverse event (AE) occurred in 8/12 patients, with four patients showing AE ≥CTCAE grade 3 (33%), and the AEs were mainly non-hematological. Conclusion: TEPIP demonstrated competitive efficacy with a tolerable safety profile in a highly palliative cohort of patients with difficult-to-treat PTCL. The all-oral application, which makes outpatient treatment possible, is particularly noteworthy

Congenital and childhood atrioventricular blocks: pathophysiology and contemporary management

Atrioventricular block is classified as congeni- tal if diagnosed in utero, at birth, or within the first month of life. The pathophysiological process is believed to be due to immune-mediated injury of the conduction system, which occurs as a result of transplacental pas- sage of maternal anti-SSA/Ro-SSB/La antibodies. Childhood atrioventricular block is therefore diagnosed between the first month and the 18th year of life. Genetic variants in multiple genes have been described to date in the pathogenesis of inherited progressive car- diac conduction disorders. Indications and techniques of cardiac pacing have also evolved to allow safe perma- nent cardiac pacing in almost all patients, including those with structural heart abnormalities

Influence of band width on the scattered ion yield spectra of a He + Ion by resonant or quasi-resonant charge exchange neutralization

The influence of the band structure, especially the bandwidth, on the scattered ion yield spectra of a He+ ion by the resonant or quasi-resonant neutralization was theoretically examined using quantum rate equations. When calculating the scattered ion yield spectra of He+ to simulate the experimental data, we observed that the band structure, especially the bandwidth, had a strong influence on the spectra at relatively low incident He+ ion energies of less than several hundred eV. Through many simulations, it was determined that theoretical calculations that include bandwidth calculation can simulate or reproduce the experimentally observed spectra of He+-In, He+-Ga, and He+-Sn systems. In contrast, simulations not including bandwidth simulation could neither reproduce nor account for such spectra. Furthermore, the calculated ion survival probability (ISP) at low incident ion energies tended to decrease with increasing bandwidth. This decrease in ISP probably corresponds to the relatively small scattered ion yield usually observed at low incident ion energies. Theoretically, such a decrease indicates that a He+ ion with a low incident energy can be easily neutralized on the surface when the bandwidth is large

Echocardiogram-Guided Stenting of a Critical Aortic Coarctation in an Extremely Low Weight Preterm Infant

We report a case of critical aortic coarctation in an extremely low birth weight preterm infant weighing 600 g that was successfully treated with interventional stent implantation. The intervention was guided by echocardiography without using contrast agent due to associated renal failure. (Level of Difficulty: Intermediate.