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1.
Strahlenther Onkol ; 200(2): 159-174, 2024 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-37272996

RESUMO

PURPOSE: Spinal metastases (SM) are a common radiotherapy (RT) indication. There is limited level I data to drive decision making regarding dose regimen (DR) and target volume definition (TVD). We aim to depict the patterns of care for RT of SM among German Society for Radiation Oncology (DEGRO) members. METHODS: An online survey on conventional RT and Stereotactic Body Radiation Therapy (SBRT) for SM, distributed via e­mail to all DEGRO members, was completed by 80 radiation oncologists between February 24 and April 29, 2022. Participation was voluntary and anonymous. RESULTS: A variety of DR was frequently used for conventional RT (primary: n = 15, adjuvant: n = 14). 30 Gy/10 fractions was reported most frequently. TVD in adjuvant RT was heterogenous, with a trend towards larger volumes. SBRT was offered in 65% (primary) and 21% (adjuvant) of participants' institutions. A variety of DR was reported (primary: n = 40, adjuvant: n = 27), most commonly 27 Gy/3 fractions and 30 Gy/5 fractions. 59% followed International Consensus Guidelines (ICG) for TVD. CONCLUSION: We provide a representative depiction of RT practice for SM among DEGRO members. DR and TVD are heterogeneous. SBRT is not comprehensively practiced, especially in the adjuvant setting. Further research is needed to provide a solid data basis for detailed recommendations.


Assuntos
Radioterapia (Especialidade) , Radiocirurgia , Neoplasias da Coluna Vertebral , Humanos , Neoplasias da Coluna Vertebral/radioterapia , Neoplasias da Coluna Vertebral/secundário , Radio-Oncologistas , Inquéritos e Questionários , Radiocirurgia/métodos
2.
Artigo em Inglês | MEDLINE | ID: mdl-39419848

RESUMO

PURPOSE: Bisphosphonates are pivotal in managing bone tumors by inhibiting bone resorption. This study investigates the therapeutic potential of [177Lu]Lu-P15-073, a novel bisphosphonate, for radioligand therapy (RLT) in bone metastases. METHODS: Ten patients (age 35 to 75) with confirmed bone metastases underwent therapy with a single dose of [177Lu]Lu-P15-073 (1,225 ± 84 MBq, or 33 ± 2 mCi). Prior to treatment, bone metastases were verified via [99mTc]Tc-MDP bone scans. Serial planar whole-body scans monitored biodistribution over a 14-day period. Dosimetry was assessed for major organs and tumor lesions, while safety was evaluated through blood biomarkers and pain scores. RESULTS: Serial planar whole-body scans demonstrated rapid and substantial accumulation of [177Lu]Lu-P15-073 in bone metastases, with minimal uptake in blood and other organs. The absorbed dose in the critical organ, red marrow, was measured at (0.034 ± 0.010 mSv/MBq), with a notably low normalized effective dose (0.013 ± 0.005 mSv/MBq) compared to other 177Lu-labeled bisphosphonates. Persistent high uptake in bone metastases was observed, resulting in elevated tumor doses (median 3.12 Gy/GBq). Patients exhibited favorable tolerance to [177Lu]Lu-P15-073 therapy, with no new instances of side effects. Additionally, 87.5% (7/8) of patients experienced a significant reduction in pain scale (numerical rating scale, NRS, from 5.1 ± 2.3 to 3.0 ± 1.8). The tumor-background ratio (TBRmean) of [99mTc]Tc-MDP correlated significantly with [177Lu]Lu-P15-073 uptake (P < 0.01), indicating its potential for prediction of absorbed dose. CONCLUSIONS: This study demonstrates the safety, dosimetry, and efficacy of a single therapeutic dose of [177Lu]Lu-P15-073 in bone metastases. The treatment was well-tolerated with no severe adverse events. These findings suggest that [177Lu]Lu-P15-073 holds promise as a novel RLT agent for bone metastases.

3.
Catheter Cardiovasc Interv ; 104(1): 71-81, 2024 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-38822744

RESUMO

BACKGROUND: Hybrid stage I palliation (HS1P) is an alternative approach for initial palliation in hypoplastic left heart syndrome (HLHS) patients. Unlike surgical stage I palliation where atrial septectomy is routinely performed, atrial septal intervention (ASI) during HS1P is variable. In this study, we described our experience with ASI in single ventricle (SV) patients who underwent HS1P and identified factors associated with need for ASI after HS1P. METHODS: Data were retrospectively collected for all HLHS patients who underwent HS1P at our center over the past 12 years. We evaluated ASIs performed during the HS1P (intra-HS1P ASI) and ASIs performed during the period from HS1P to the subsequent surgical stage, either interval Norwood stage I or comprehensive stage II (post-HS1P ASI). Patient factors and procedural data were compared to identify factors associated with undergoing post-HS1P ASI and the impact of ASI on patient outcomes was evaluated. RESULTS: Of 50 SV patients included, 23 (46%) underwent intra-HS1P ASI and 26 (52%) underwent post-HS1P ASI. Need for post-HS1P ASI was lower among patients who had an intra-HS1P ASI as compared to those who did not (30% vs. 70%; p = 0.005). There were no significant differences in short or Midterm outcomes between patients who underwent intra-HS1P ASI or post-HS1P ASI and their counterparts. CONCLUSIONS: ASI is common both during and after HS1P but is generally well tolerated and type of ASI does not significantly impact overall patient outcomes. Our findings suggest that the current approach of individualizing management of ASI in the HS1P population is effective and safe.


Assuntos
Cateterismo Cardíaco , Síndrome do Coração Esquerdo Hipoplásico , Procedimentos de Norwood , Cuidados Paliativos , Humanos , Síndrome do Coração Esquerdo Hipoplásico/cirurgia , Síndrome do Coração Esquerdo Hipoplásico/fisiopatologia , Estudos Retrospectivos , Resultado do Tratamento , Feminino , Masculino , Fatores de Tempo , Fatores de Risco , Procedimentos de Norwood/efeitos adversos , Recém-Nascido , Cateterismo Cardíaco/efeitos adversos , Cateterismo Cardíaco/instrumentação , Septo Interatrial/diagnóstico por imagem , Septo Interatrial/fisiopatologia , Septo Interatrial/cirurgia , Lactente , Coração Univentricular/cirurgia , Coração Univentricular/fisiopatologia , Coração Univentricular/diagnóstico por imagem
4.
Curr Oncol Rep ; 26(4): 400-408, 2024 04.
Artigo em Inglês | MEDLINE | ID: mdl-38539021

RESUMO

PURPOSE OF REVIEW: Increasing life expectancy among patients with advanced cancer has placed a greater emphasis on optimizing pain control and quality of life. Concurrently, significant advancements in radiotherapy for bone metastases have permitted for dose escalation strategies such as stereotactic radiotherapy. This review aims to provide updated information on the management of bone metastases in light of these developments. RECENT FINDINGS: We reviewed recent studies regarding the role and details of external beam radiotherapy for bone metastases, with emphasis on differences by treatment site as well as intention (palliative versus ablative for oligometastases). Conventional palliative radiotherapy remains a mainstay of management. While stereotactic radiotherapy may augment durability of pain relief and even survival time, there are significant questions remaining regarding optimal dosing and patient selection. Radiotherapy for bone metastases continues to evolve, particularly with increasing use of stereotactic radiotherapy. Future studies are needed to clarify optimal dose, fractionation, modality, and patient selection criteria among different radiotherapy approaches.


Assuntos
Neoplasias Ósseas , Radiocirurgia , Humanos , Qualidade de Vida , Cuidados Paliativos , Dor
5.
Artigo em Inglês | MEDLINE | ID: mdl-38522869

RESUMO

Neonates with single ventricle physiology and ductal-dependent systemic circulation, such as those with hypoplastic left heart syndrome, undergo palliation in the first days of life. Over the past decades, variations on the traditional Stage 1 palliation, also known as Norwood operation, have emerged. These include the hybrid palliation and the total transcatheter approach. Here, we review the current evidence and data on different Stage 1 approaches, with a focus on their advantages, challenges, and future perspectives. Overall, although controversy remains regarding the superiority or inferiority of one approach to another, outcomes after the Norwood and the hybrid palliation have improved over time. However, both procedures still represent high-risk approaches that entail exposure to sternotomy, surgery, and potential cardiopulmonary bypass. The total transcatheter Stage 1 palliation spares patients the surgical and cardiopulmonary bypass insults and has proven to be an effective strategy to bridge even high-risk infants to a later palliative surgery, complete repair, or transplant. As the most recently proposed approach, data are still limited but promising. Future studies will be needed to better define the advantages, challenges, outcomes, and overall potential of this novel approach.


Assuntos
Síndrome do Coração Esquerdo Hipoplásico , Procedimentos de Norwood , Coração Univentricular , Recém-Nascido , Lactente , Humanos , Síndrome do Coração Esquerdo Hipoplásico/cirurgia , Procedimentos de Norwood/métodos , Cuidados Paliativos/métodos , Ventrículos do Coração , Resultado do Tratamento , Estudos Retrospectivos
6.
Pediatr Cardiol ; 45(2): 221-227, 2024 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-38153546

RESUMO

BACKGROUND: Turner syndrome (TS) is associated with left-sided cardiac lesions, including hypoplastic left heart syndrome (HLHS). Mortality as high as 80-90% has been reported following stage I single-ventricle palliation (S1P) in patients with TS and HLHS (TS + HLHS). The specific factors that relate to poor outcomes are not well understood. METHODS: This is a single-center, retrospective cohort study that includes 197 patients with HLHS who underwent S1P between 2008 and 2022. The clinical outcomes and interstage hemodynamics of TS + HLHS patients (N = 11) were compared with HLHS without TS (TS-HLHS), (N = 186). RESULTS: Of the 11 TS + HLHS patients, 10 underwent S1P; 4 underwent Glenn and 1 had hemodynamics considered prohibitive for Glenn; only 1 survived to Fontan palliation. Post-S1P mortality was higher in TS + HLHS (60 v 25%, p = 0.017). Following S1P, TS + HLHS had higher rates of postoperative ECMO (70 v 28%, p = 0.006), surgical necrotizing enterocolitis (20 v 3%, p = 0.007), peritoneal drain placement (70 v 31%, p = 0.012), urinary tract infection (30 v 9%, p = 0.035), and ICU readmissions (median 5 v 1, p = 0.035). Interstage hemodynamics demonstrated higher right ventricular end diastolic, (11 v 8mmHg, p = 0.033), mean pulmonary artery (20 v 13mmHg) (p = 0.002), and left atrial pressures (9 v 6mmHg, p = 0.047) in TS + HLHS. CONCLUSION: High mortality rates are described in TS + HLHS patients following S1P. In our cohort, despite most surviving more than 30 days post-S1P, long-term survival remained poor. Interstage catheterization data suggest poor physiologic candidacy for subsequent stages of single-ventricle palliation. Understanding the clinical and hemodynamic factors related to poor outcomes in TS + HLHS will help inform management for this population.


Assuntos
Síndrome do Coração Esquerdo Hipoplásico , Síndrome de Turner , Recém-Nascido , Humanos , Síndrome de Turner/complicações , Resultado do Tratamento , Estudos Retrospectivos , Hemodinâmica , Morbidade , Cuidados Paliativos
7.
Pediatr Cardiol ; 45(3): 623-631, 2024 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-38159143

RESUMO

Completing 3-stage palliation for hypoplastic left heart syndrome requires significant resources. An analysis of recent data has not been performed. We aimed to determine total charges necessary to complete all 3 stages of single-ventricle palliation, including interstage encounters. We also aimed to determine overall resource utilization, including hospital days, interstage admissions, and interstage procedures. We performed a retrospective cohort study using data from the Pediatric Health Information System database between 2016 and 2021, including all patients who completed 3-stage palliation for hypoplastic left heart syndrome. We identified 199 patients who underwent 3-stage palliation of hypoplastic left heart syndrome between 2016 and 2021. Median total adjusted charges (interquartile range, IQR) over the course of 3-stage palliation were $1,475,800 ($1,028,900-2,191,700). Median adjusted charges (IQR) for stage 1, 2, and 3 hospitalizations were $604,300 ($419,000-891,400), $234,000 ($164,300-370,800), and $256,260 ($178,300-345,900), respectively. Median hospital length of stay (IQR) for stages 1, 2, and 3 was 36 (26,53), 9 (6,17), and 10 (7,14) days, respectively. Pulmonary artery stenosis was the most common admitting diagnosis for interstage hospitalizations (3.4% of hospitalizations). Cardiac catheterization (24.1% of procedures) and feeding tube placement (10.0% of procedures) were the most common principal procedures during interstage hospitalizations. Total inpatient charges incurred throughout 3-stage palliation of hypoplastic left heart syndrome are substantial and have risen since prior studies. Gastrointestinal comorbidities and feeding optimization contribute considerably to this resource utilization.


Assuntos
Síndrome do Coração Esquerdo Hipoplásico , Procedimentos de Norwood , Humanos , Criança , Lactente , Estudos Retrospectivos , Pacientes Internados , Síndrome do Coração Esquerdo Hipoplásico/cirurgia , Síndrome do Coração Esquerdo Hipoplásico/diagnóstico , Tempo de Internação , Hospitalização , Cuidados Paliativos/métodos , Resultado do Tratamento , Procedimentos de Norwood/métodos
8.
Pediatr Cardiol ; 45(3): 673-680, 2024 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-36809419

RESUMO

There are no published studies that examine the safety and tolerability of medication to treat attention-deficit/hyperactivity disorder (ADHD) in children with histories of Fontan palliation (Fontan) or heart transplant (HT), despite the high prevalence of ADHD in these populations. To address this gap, we examined the cardiac course, somatic growth, and incidence of side effects for one year after medication initiation amongst children with Fontan or HT and comorbid ADHD. The final sample comprised 24 children with Fontan (12 medication-treated, 12 control) and 20 children with HT (10 medication-treated, 10 control). Demographic, somatic growth (height and weight percentile-for age), and cardiac data (blood pressure, heart rate, results of 24 h Holter monitoring, electrocardiograms) were extracted from electronic medical records. Medication-treated and control subjects were matched by cardiac diagnosis (Fontan or HT), age, and sex. Nonparametric statistical tests were utilized to compare between- and within-group differences prior to, and one year post, medication initiation. There were no differences in somatic growth or cardiac data when comparing medication-treated participants to matched controls, regardless of cardiac diagnosis. Within the medication group, a statistically significant increase in blood pressure was observed, though the group average remained within clinically acceptable limits. While results are preliminary in nature due to our very limited sample size, our findings suggest that ADHD medications can be tolerated with minimal cardiac or somatic growth effects amongst complex cardiac patients. Our preliminary results favor treating ADHD with medication, which has considerable implications for long-term academic/employment outcomes and quality of life for this population. Close collaboration between pediatricians, psychologists, and cardiologists is essential to individualizing and optimizing interventions and outcomes for children with Fontan or HT.


Assuntos
Transtorno do Deficit de Atenção com Hiperatividade , Técnica de Fontan , Transplante de Coração , Criança , Humanos , Adolescente , Técnica de Fontan/efeitos adversos , Transtorno do Deficit de Atenção com Hiperatividade/tratamento farmacológico , Qualidade de Vida , Transplante de Coração/efeitos adversos , Coração
9.
Pediatr Cardiol ; 45(5): 998-1006, 2024 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-38519622

RESUMO

We sought to evaluate the potential clinical role of 4D-flow cardiac magnetic resonance (CMR)-derived energetics and flow parameters in a cohort of patients' post-Fontan palliation. In patients with Fontan circulation who underwent 4D-Flow CMR, streamlines distribution was evaluated, as well a 4D-flow CMR-derived energetics parameters as kinetic energy (KE) and energy loss (EL) normalized by volume. EL/KE index as a marker of flow efficiency was also calculated. Cardiopulmonary exercise test (CPET) was also performed in a subgroup of patients. The population study included 55 patients (mean age 22 ± 11 years). The analysis of the streamlines revealed a preferential distribution of the right superior vena cava flow for the right pulmonary artery (62.5 ± 35.4%) and a mild preferential flow for the left pulmonary artery (52.3 ± 40.6%) of the inferior vena cave-pulmonary arteries (IVC-PA) conduit. Patients with heart failure (HF) presented lower IVC/PA-conduit flow (0.75 ± 0.5 vs 1.3 ± 0.5 l/min/m2, p = 0.004) and a higher mean flow-jet angle of the IVC-PA conduit (39.2 ± 22.8 vs 15.2 ± 8.9, p < 0.001) than the remaining patients. EL/KE index correlates inversely with VO2/kg/min: R: - 0.45, p = 0.01 peak, minute ventilation (VE) R: - 0.466, p < 0.01, maximal voluntary ventilation: R:0.44, p = 0.001 and positively with the physiological dead space to the tidal volume ratio (VD/VT) peak: R: 0.58, p < 0.01. From our data, lower blood flow in IVC/PA conduit and eccentric flow was associated with HF whereas higher EL/KE index was associated with reduced functional capacity and impaired lung function. Larger studies are needed to confirm our results and to further improve the prognostic role of the 4D-Flow CMR in this challenging population.


Assuntos
Teste de Esforço , Técnica de Fontan , Cardiopatias Congênitas , Artéria Pulmonar , Humanos , Feminino , Masculino , Cardiopatias Congênitas/cirurgia , Cardiopatias Congênitas/fisiopatologia , Adulto Jovem , Teste de Esforço/métodos , Artéria Pulmonar/diagnóstico por imagem , Artéria Pulmonar/fisiopatologia , Adolescente , Adulto , Imagem Cinética por Ressonância Magnética/métodos , Criança , Veia Cava Superior/diagnóstico por imagem , Velocidade do Fluxo Sanguíneo/fisiologia , Insuficiência Cardíaca/fisiopatologia , Insuficiência Cardíaca/cirurgia , Veia Cava Inferior/diagnóstico por imagem
10.
Pediatr Cardiol ; 2024 Sep 17.
Artigo em Inglês | MEDLINE | ID: mdl-39287667

RESUMO

Despite the clinical results of the Fontan operation have certainly improved, it still presents with an inherent surgical risk of death and early morbidities. This is a retrospective clinical study of children undergoing Fontan operation in 9 congenital cardiac centers in Italy between 1990 and 2023. Clinical and surgical data were collected via a dedicated RedCap database. Primary outcome was cohort's mortality, also considering different decades, while secondary outcomes were postoperative complications and reintervention. In the last 3 decades, there were 897 patients undergoing Fontan operation, M/F 512/384, median age: 4.5 years (IQR 3.3-6.4), median weight 16 kg (IQR 14-22). A first palliation was deemed necessary in 710 patients (80%), and most patients underwent a staged Fontan (93%); an extracardiac conduit was used in 790 patients (88%). Postoperative complications (mild to severe) occurred in 410 patients (46%), and early reinterventions were required in 66 patients (7.5%). Overall operative mortality was 1.7% (15 patients). Age at Fontan greater than 4 years was associated with an early need for transcatheter reintervention (adj p value = 0.037) and a higher incidence of postoperative complications (adj p value = 0.017). The Fontan operation has seen significant improvements in immediate outcomes, notably a remarkable reduction in overall mortality to just 1.35% in the last decade. While minor complications have remained steady, there has been a substantial decrease in major early complications, deaths, and the need for reinterventions. Notably, patients aged over 4 years seem to face a higher risk of postoperative morbidity, underscoring the critical role of age in preoperative assessment and management strategies for Fontan patients.

11.
Pediatr Cardiol ; 2024 Sep 26.
Artigo em Inglês | MEDLINE | ID: mdl-39325157

RESUMO

We describe the perioperative BNP trends in patients with HLHS from S1P until interstage discharge to home, death, or S2P. This prospective cohort study includes all newborns with hypoplastic left heart syndrome (HLHS) who underwent Norwood procedures (S1P) at Texas Children's Hospital from April 2018 through April 2019. Our study included 19 newborns with HLHS. There was no interstage mortality; 47% were discharged home prior to the S2P procedure. Nine patients (50%) had higher BNP levels immediately after arrival to the cardiac intensive care unit (ICU) after S1P compared to preoperative levels. BNP levels were higher in those with a shorter duration of mechanical intubation (P = 0.02) and those with moderately depressed right ventricular systolic function in the immediate postoperative period (P = 0.02). RVPAs patients had higher BNP levels (median 3357 pg/mL) than mBTTs (median 2862 pg/mL), that was not statistically significant (P = 0.4). Despite higher BNP levels in RVPAs in the early postoperative period, these subjects had shorter mechanical ventilation, ICU, and hospital length of stay duration. BNP trends for HLHS patients vary in the postoperative period after S1P. RVPAs had higher BNP levels than mBTTs in the early postoperative period after S1P; however, this was not associated with worse outcomes.

12.
Pediatr Cardiol ; 2024 Jul 19.
Artigo em Inglês | MEDLINE | ID: mdl-39030348

RESUMO

Controlling pulmonary blood flow in patients who have undergone Norwood palliation, especially early postoperatively, is challenging due to a change in the balance of systemic and pulmonary vascular resistance. We applied a combination therapy of clipping and balloon angioplasty for right ventricle-pulmonary artery (RV-PA) shunt to control pulmonary blood flow, but the influence of the combination therapy on the PA condition is uncertain. Retrospectively analysis was conducted of all infants with hypoplastic left heart syndrome who had undergone Norwood palliation with RV-PA shunt at Okayama University Hospital from January 2008 to September 2022. A total of 50 consecutive patients underwent Norwood palliation with RV-PA shunt in this study period. Of them, 29 patients underwent RV-PA shunt flow clipping, and the remaining 21 had unclipped RV-PA shunt. Twenty-three patients underwent balloon angioplasty for RV-PA shunt with clips. After balloon angioplasty, oxygen saturation significantly increased from 69 (59-76)% to 80 (72-86)% (p < 0.001), and the narrowest portion of the clipped conduit significantly improved from 2.8 (1.8-3.4) to 3.8 (2.9-4.6) mm (p < 0.001). In cardiac catheterizations prior to Bidirectional cavo-pulmonary shunt (BCPS), there were no significant differences in pulmonary-to-systemic flow ratio (Qp/Qs), ventricular end-diastolic pressure, Nakata index, arterial saturation, mean pulmonary artery pressure and pulmonary vascular resistance index. On the other hand, in Cardiac catheterizations prior to Fontan, Nakata index was larger in the clipped group (p = 0.02). There was no statistically significant difference in the 5-year survival between the two groups (clipped group 96%, unclipped group 74%, log-rank test: p = 0.13). At least, our combination therapy of clipping and balloon angioplasty for RV-PA shunt did not negatively impact PA growth. Although there is a trend toward better but not statistically significant difference in outcomes in the clipped group compared to the non-clipped group, this treatment strategy may play an important role in improving outcomes in hypoplastic left heart syndrome.

13.
Cardiol Young ; : 1-7, 2024 Oct 18.
Artigo em Inglês | MEDLINE | ID: mdl-39422085

RESUMO

OBJECTIVE: Our objective was to assess the predictive value of physiologic dead space fraction for mortality in patients undergoing the comprehensive stage 2 operation. METHODS: This was a single-centre retrospective observational study conducted at a quaternary free-standing children's hospital specialising in hybrid palliation of single ventricle cardiac disease. 180 patients underwent the comprehensive stage 2 operation. 76 patients (42%) underwent early extubation, 59 (33%) standard extubation, and 45 (25%) delayed extubation. We measured time to extubation, post-operative outcomes, length of stay and utilised Fine gray models, Youden's J statistic, cumulative incidence function, and logistic regression to analyse outcomes. RESULTS: Delayed extubation group suffered significantly higher rates of mortality (31.1% vs. 6.8%), cardiac arrest (40.0% vs. 10.2%), stroke (37.8% vs. 11.9%), and need for catheter (28.9% vs. 5.1%) and surgical intervention (24.4% vs. 8.5%) (P < 0.001). Physiologic dead space fraction was significantly higher in the delayed extubation group and in non-survivors with a value of 0.3, which was found to be the discriminatory point by Youden's J statistic. For a 0.1 unit increase in physiologic dead space fraction on post-operative day 1, the odds of a patient expiring increase by a factor of 2.26 (95% CI 1.41-3.97, p < 0.001) and by a factor of 3.79 (95% CI 1.65-11.7, p 0.01) on post-operative day 3. CONCLUSIONS: Delayed extubation impacts morbidity and mortality in patients undergoing the comprehensive stage 2 operation. Increased physiologic dead space fraction in the first 60 hours after arrival to the ICU is associated with higher mortality.

14.
Cardiol Young ; 34(6): 1312-1324, 2024 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-38287708

RESUMO

BACKGROUND: Complex CHDs are life threatening, and surgical treatment is needed for survival. Fontan palliation led to a significant increase in survival rates during the last decades. Consequently, quality of life became more essential. While a reduced quality of life compared to healthy children has been reported, detailed knowledge about individual quality of life and particular areas is lacking. Furthermore, the effect of different risk factors on quality of life is only rarely evaluated. METHOD AND RESULTS: Database of the department for pediatric cardiology, Heart Center Leipzig, was screened for children after total cavopulmonary connection palliation. n = 39 patients were included in the study, the outcome after total cavopulmonary connection was analysed in detail and quality of life data were collected and analysed using the standardised questionnaire "Pediatric quality of life inventory", version 4.0. We compared the total health score of our patients to the mean score of healthy children in the literature. The mean follow-up time was 6.4 ± 3.2 years, the overall survival was 100% after maximal follow-up time of 11.1 years. We could not find any age or gender dependence, nor an influence of age at total cavopulmonary connection on the later quality of life. Yet, patients with three-staged surgery exhibited a worse quality of life than patients with two-staged palliation. Late complications might influence quality of life, but patient number is too small, to find universal results. CONCLUSION: The total cavopulmonary connection palliation affects physical and psychological quality of life as well as cardiac health independently from age and gender. More patients and longer observation should be examined to confirm the results.


Assuntos
Técnica de Fontan , Cardiopatias Congênitas , Cuidados Paliativos , Qualidade de Vida , Humanos , Masculino , Feminino , Técnica de Fontan/métodos , Seguimentos , Criança , Pré-Escolar , Cardiopatias Congênitas/cirurgia , Cardiopatias Congênitas/psicologia , Cuidados Paliativos/métodos , Inquéritos e Questionários , Adolescente , Estudos Retrospectivos , Lactente , Alemanha/epidemiologia , Taxa de Sobrevida/tendências , Resultado do Tratamento , Fatores de Risco
15.
Cardiol Young ; 34(5): 1145-1147, 2024 May.
Artigo em Inglês | MEDLINE | ID: mdl-38477599

RESUMO

The coexistence of aortic valve atresia and interrupted aortic arch are an extremely rare condition. In this pathology, blood flow to the ascending aorta and coronary arteries should be provided through the ductus arteriosus or collaterals originating from the descending aorta. In rare cases where bilateral ductus arteriosus is present, they can provide circulation. Here, we report two cases in which coronary arteries and ascending aorta were supplied by one ductus arteriosus and distal systemic circulation is supplied by a second ductus arteriosus in one patient and a collateral artery in the other. Initial palliation was successfully performed by bilateral pulmonary artery banding and transcatheter ductal stent implantation in both cases.


Assuntos
Aorta Torácica , Cuidados Paliativos , Stents , Humanos , Aorta Torácica/anormalidades , Aorta Torácica/cirurgia , Aorta Torácica/diagnóstico por imagem , Cuidados Paliativos/métodos , Masculino , Recém-Nascido , Feminino , Valva Aórtica/cirurgia , Valva Aórtica/anormalidades , Valva Aórtica/diagnóstico por imagem , Cateterismo Cardíaco/métodos , Anormalidades Múltiplas
16.
Cardiol Young ; : 1-3, 2024 Oct 18.
Artigo em Inglês | MEDLINE | ID: mdl-39422101

RESUMO

A newborn with unbalanced atrioventricular septal defect and heterotaxy syndrome underwent early surgeries for single-ventricular palliation due to a small left ventricle. Following procedures, including the modified Damus-Kaye-Stansel, there was a notable increase in left ventricular size. This progression allowed successful biventricular repair at 7 months. This case highlights potential ventricular development after palliative procedures in patients with borderline ventricular size.

17.
Dig Endosc ; 36(4): 473-480, 2024 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-37612129

RESUMO

OBJECTIVES: We aimed to evaluate the efficacy and safety of metal stents compared with plastic stents when bilateral side-by-side stents were deployed for malignant hilar biliary obstruction (MHBO). METHODS: Fifty patients with unresectable advanced MHBO were randomly assigned to the metal stent (MS, n = 25) or plastic stent group (PS, n = 25). Fully covered self-expandable metal stents with 6 mm diameter and plastic stents with either 7F straight or double pigtail were used for MS and PS groups, respectively. Time to recurrent biliary obstruction (TRBO) was evaluated as the primary outcome. RESULTS: Both groups had 100% technical success rates; 88% and 76% of clinical success rates were obtained in MS and PS, respectively. Although stent migrations were more frequent in MS than PS (48% vs. 16%, P = 0.02), the mean TRBO was significantly longer in MS (190 days; 95% confidence interval [CI] 121-260 days vs. 96 days; 95% CI 50-141 days, P = 0.02). The placement of plastic stents (hazard ratio 2.42; 95% CI 1.24-4.73; P = 0.01) was the only significant risk factor associated with TRBO in multivariable analysis. The rates of adverse events were similar between the two groups (difference 0%; 95% CI -25% to 25%; P > 0.99). CONCLUSIONS: During bilateral side-by-side deployment in MHBO, the use of metal stents appears to be preferable to plastic stents in terms of TRBO, despite a higher frequency of stent migration.


Assuntos
Neoplasias dos Ductos Biliares , Colestase , Stents Metálicos Autoexpansíveis , Humanos , Estudos Prospectivos , Stents/efeitos adversos , Colestase/etiologia , Colestase/cirurgia , Stents Metálicos Autoexpansíveis/efeitos adversos , Implantação de Prótese , Resultado do Tratamento , Estudos Retrospectivos , Neoplasias dos Ductos Biliares/complicações , Neoplasias dos Ductos Biliares/cirurgia
18.
Heart Lung Circ ; 33(8): 1193-1200, 2024 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-38871532

RESUMO

AIM: Heart transplantation (HT) in patients with failing univentricular circulation is often challenging. This is compounded by the ever-increasing number of patients with prior Norwood-type reconstruction of the aorta, large aortic root, and often dense adhesions from multiple prior operations. We aimed to elucidate differences in outcomes of HT in patients with prior univentricular palliations, with and without prior Norwood-type aortic arch reconstruction (ArchRec). METHODS: All patients who underwent HT for failed univentricular palliation during the 1990-2022 period were included in the study. RESULTS: Of 45 patients, 18 had undergone ArchRec. Hospital mortality improved in the recent era (17.4% before 2006 vs 0% after 2006; p=0.11), despite a higher proportion of patients with ArchRec (17.4% before 2006 vs 60.8% after 2006, p=0.002). Patients with ArchRec had a higher number of prior cardiac surgeries (4.1±1.5 vs 3.2±1.3, p=0.04), longer cardiopulmonary bypass time (320±23 vs 242±21 min, p=0.02), more concomitant arch reconstruction (33.3% vs 0%, p=0.02), greater need for post-HT extracorporeal membrane oxygenation (33.3% vs 3.7%; p=0.01) and longer hospital stay (37.1±30.5 days vs 23.6±11.8 days, p=0.04). Freedom from death or retransplantation for all patients was 91%, 73%, 67%, and 53% at 1, 5, 10, and 15-years, respectively. Prior ArchRec, Fontan procedure, and earlier eras were not risk factors for death. CONCLUSIONS: The outcomes of HT after univentricular palliation have improved in recent times and low operative mortality can be achieved. Despite increased complexity, good similar outcomes can be achieved in patients with and without prior arch reconstruction regardless of the palliation stage.


Assuntos
Transplante de Coração , Humanos , Transplante de Coração/métodos , Masculino , Feminino , Mortalidade Hospitalar/tendências , Lactente , Estudos Retrospectivos , Cuidados Paliativos/métodos , Coração Univentricular/cirurgia , Procedimentos de Norwood/métodos , Taxa de Sobrevida/tendências , Recém-Nascido , Seguimentos
19.
Med Anthropol Q ; 38(3): 271-284, 2024 09.
Artigo em Inglês | MEDLINE | ID: mdl-38860724

RESUMO

This article examines how militarized regimes of narcotics and price control sustain unpalliated cancer pain in Pakistan. It shows how these regimes of control-reimagined as "regimes of pain"-render morphine, a cheap, effective opiate analgesic, scarce in hospitals. Meanwhile, heroin, morphine's illegal derivative, proliferates in illicit circuits. The article highlights a devastating consequence of the global wars against drugs and "terror": the consignment of cancer patients to agonizing end-of-life pain. Widening the analytic lens upon palliation beyond bodies and their clinical encounters, the article offers a geopolitics of palliation. It shows how narcovigilance targeting illicit drugs has the perverse effect of throttling morphine's licit supply. It shows further how unviably low price ceilings, purported to ensure a poor population's access to morphine, render it scarce on the official market. These mutually reinforcing regimes of control thus thwart their own purported objectives, consigning cancer patients to preventable, yet unpalliated, pain.


Assuntos
Analgésicos Opioides , Antropologia Médica , Dor do Câncer , Morfina , Cuidados Paliativos , Humanos , Paquistão , Dor do Câncer/tratamento farmacológico , Morfina/uso terapêutico , Analgésicos Opioides/uso terapêutico , Neoplasias , Masculino
20.
Br J Haematol ; 203(5): 710-711, 2023 12.
Artigo em Inglês | MEDLINE | ID: mdl-37909166

RESUMO

In this issue, we publish the last instalment in our series 'Global View' within the 'Wider Perspective' umbrella. In it we query experts from a variety of countries-deliberately trying to encompass both those with strained economies as well as more affluent ones-as to how patients are handled within such widely varying health systems. Commentary on: Hokland et al. AML in the elderly-A global view. Br J Haematol 2023;203:760-773.

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