Incidence and predictors for the development of significant supradiaphragmatic decompressing venous collateral channels following creation of Fontan physiology

2001 ◽  
Vol 11 (3) ◽  
pp. 289-294 ◽  
Author(s):  
Howard S. Weber
Keyword(s):  
Fontan Operation ◽  
Cardiac Physiology ◽  
Number Of Patients ◽  
Coil Occlusion ◽  
Angiographic Data ◽  
Fontan Physiology ◽  
Cavopulmonary Connection ◽  
Cavopulmonary Shunt ◽  
Bidirectional Cavopulmonary Connection

The occurrence of supradiaphragmatic decompressing venous collateral channels following construction of a bidirectional cavopulmonary connection or completion of the Fontan operation resulting in abnormal systemic hypoxemia has been infrequently described. In addition, the incidence and predictors of these channels have not been well delineated, especially in those patients without formation of such structures preoperatively. I evaluated, retrospectively, 40 patients who had undergone either construction of a bidirectional cavopulmonary shunt or completion of the Fontan operation, and who had complete pre and postoperative hemodynamic and angiographic data. Of the patients, 17 (43%) had developed a total of 21 decompressing venous collateral channels, of which 7 (18%) were considered to be hemodynamically significant requiring transcatheter coil occlusion. Of all variables examined, seven patients with significant decompressing collaterals had a greater transpulmonary gradient at follow-up catheterization (8 + /− 2 vs 5 + /− 2 mmHg, p= .01) and lower systemic saturations at routine clinical follow-up visits (82 + /− 5 vs 89 + /− 5 mmHg, p= .007) in comparison to the 33 others. When not evident preoperatively, decompressing venous collateral channels develop in a significant number of patients following conversion to Fontan physiology. If sufficiently large, they may produce lower than expected systemic saturations for the observed cardiac physiology. The larger decompressing channels are more likely to occur when a greater transpulmonary gradient exists postoperatively, which may require cardiac catheterization and transcatheter coil occlusion.

scholarly journals Outcomes following modified extracardiac Fontan procedure of direct total cavopulmonary connection with autologous vessels: a single-centre 10-year experience

2019 ◽  
Author(s):  
Xiaobing Liu ◽  
Haiyun Yuan ◽  
Jimei Chen ◽  
Jianzheng Cen ◽  
Zhiqiang Nie ◽  
...  
Keyword(s):  
Fontan Operation ◽  
Growth Potential ◽  
Total Cavopulmonary Connection ◽  
Long Term Outcomes ◽  
Protein Losing Enteropathy ◽  
Significant Difference ◽  
Cavopulmonary Connection ◽  
Extracardiac Fontan

AbstractOBJECTIVESThe modified extracardiac Fontan of direct total cavopulmonary connection (dTCPC) with entirely autologous vessels is a better solution in selected patients with functionally univentricular hearts because it combines the advantages of a tubular connection and of full growth potential. We investigated the mid-term to long-term outcomes of the physical condition of patients who had the dTCPC and assessed the growth potential of the autologous dTCPC pathway.METHODSFrom July 2005 to June 2014, 31 patients, aged 1.8–14.0 years, underwent a modified extracardiac Fontan with dTCPC at our institution. Twenty-two patients underwent a 1-stage dTCPC and 9 patients underwent a 2-stage dTCPC. The preoperative and postoperative data were reviewed retrospectively.RESULTSThere were 17 (54.8%) boys and 14 (45.2%) girls, with a median age of 6.4 years (range 1.8–14.0 years) and a mean weight of 17.8 kg (range 9–41 kg). The mean follow-up period was 6.0 years (range 2.1–10.2 years). There was 1 early death and 2 late deaths. The event-free survival rate for the 31 patients was 88.9% at 10 years during the follow-up period. Late protein-losing enteropathy, thromboembolism, arrhythmia or heart failure were not observed. There was a significant difference between the preoperative and postoperative data for body mass index and the diameters of the autologous dTCPC pathway and pulmonary artery branches, confirming that the growth potential of the autologous dTCPC pathway was realized.CONCLUSIONSThe dTCPC procedure could be performed with satisfactory midterm to long-term outcomes in selected patients. It has the potential of retaining the advantage of the extracardiac Fontan operation together with the potential for growth and the avoidance of prosthetic materials.

scholarly journals Outcomes of Single Ventricle Patients Undergoing the Kawashima Procedure: Can We Do Better?

2019 ◽  
Vol 10 (1) ◽  
pp. 20-27 ◽  
Author(s):  
Bahaaldin Alsoufi ◽  
Joshua Rosenblum ◽  
Curtis Travers ◽  
Kirk Kanter ◽  
Philip M. Trusty ◽  
...  
Keyword(s):  
Single Ventricle ◽  
Fontan Operation ◽  
Pulmonary Arteries ◽  
Vena Cava ◽  
Virtual Surgery ◽  
Number Of Patients ◽  
Computer Based ◽  
Atrial Isomerism ◽  
Kawashima Operation

Objectives: Current technology advances in virtual surgery modeling and computational flow dynamics allow preoperative individualized computer-based design of Fontan operation. To determine potential role of those innovations in patients undergoing hepatic vein incorporation (HVI) following Kawashima operation, we retrospectively examined historic cohort of patients who underwent HVI following Kawashima with focus on regression of pulmonary arteriovenous malformations (PAVMs). Methods: Twenty-two children with single ventricle and interrupted inferior vena cava underwent Kawashima operation (2002-12). Twenty-one (96%) patients had left atrial isomerism and 21 (96%) had undergone prior first-stage palliation. Clinical outcomes were examined. Results: Mean O2 saturation (SaO2) increased from 77% ± 8% to 85% ± 6% ( P = .002) after Kawashima. Fifteen (68%) patients developed PAVMs. Eighteen patients underwent HVI (median age and interval from Kawashima: 4.4 and 3.7 years, respectively). Mean SaO2 prior to HVI was 77% ± 8% and increased to 81% ± 10% at the time of hospital discharge ( P = .250), with five patients requiring home oxygen. On follow-up, mean SaO2 increased to 95% ± 4% ( P < .001). Overall ten-year survival following Kawashima was 94%. Conclusions: A large number of patients develop PAVMs and subsequent cyanosis after Kawashima operation. Early following HVI, SaO2 is commonly low and insignificantly different from that prior to HVI. Although SaO2 will improve on follow-up in most patients, a number of patients continue to have low saturations, indicating incomplete resolution of PAVMs. Given the heterogeneity of those patients and lack of preoperative predictors for complete PAVM regression, our findings suggest a role for virtual surgery to determine optimal individual procedure design that would provide even distribution of hepatic blood flow to both pulmonary arteries.

STRUCTURAL AND DYNAMIC FEATURES OF EAR-LY DISABILITY DUE TO MENTAL DISEASES

2020 ◽  
pp. 62-67
Author(s):  
Shmakova O.P.
Keyword(s):  
People With Disabilities ◽  
Child And Adolescent Psychiatry ◽  
Child Psychiatrist ◽  
Childhood And Adolescence ◽  
Disabled Children ◽  
Dynamic Features ◽  
Factors Affecting ◽  
Number Of Children ◽  
Number Of Patients

Prevention of disability is one of the most significant tasks of child and adolescent psychiatry. Obtaining data on the dynamics of the number of people with disabilities and the factors affecting this indicator seems to be one of the relevant aspects. Aim: to trace the dynamics of the number of children with disabili-ties and to assess the change in the structure of early disability over the past decades. Materials and Meth-ods. A comparative analysis of two cohorts of patients was carried out: 1st - patients born in 1990-1992. (1203 patients (men - 914, 76%; women - 289, 24%)) who applied to the district neuropsychiatric dispensa-ry for outpatient care in childhood and adolescence; II - children and adolescents born in 2005 - 2018 (602 patients (male - 410, 68%; female - 192, 32%), ob-served at the time of the study by a child psychiatrist in the neuropsychiatric dispensary. Research methods: clinical and psychopathological; follow-up; statisti-cal. Results. Comparison of the number and nosologi-cal distribution of disabled children in two cohorts showed that over the 15th year there has been a shift towards an increase in the proportion of disabled children among patients observed by child and ado-lescent psychiatrists. The increase in the number of children with disabilities was due to those suffering from childhood autism and other disorders of general development. There were no statistically significant differences in the number of people with disabilities who received benefits before the age of 7, as well as differences in gender ratios among disabled people in the two cohorts. Conclusion. Early disability is a mul-tifactorial phenomenon, prevalence, dynamics, the structure of which depends not only on clinical, but also on socio-administrative realities. Children with autism require increased attention, since there has been a multiple increase in the number of patients with this diagnosis.

Impact of the Fontan Operation on Organ Systems

2019 ◽  
Vol 19 (3) ◽  
pp. 205-214
Author(s):  
Miriam Michel ◽  
Manuela Zlamy ◽  
Andreas Entenmann ◽  
Karin Pichler ◽  
Sabine Scholl-Bürgi ◽  
...  
Keyword(s):  
Systematic Study ◽  
Fontan Operation ◽  
Gastrointestinal System ◽  
Adjunct Treatment ◽  
Organ Systems ◽  
Fontan Surgery ◽  
Fontan Patients

: In patients having undergone the Fontan operation, besides the well discussed changes in the cardiac, pulmonary and gastrointestinal system, alterations of further organ systems including the hematologic, immunologic, endocrinological and metabolic are reported. As a medical adjunct to Fontan surgery, the systematic study of the central role of the liver as a metabolizing and synthesizing organ should allow for a better understanding of the pathomechanism underlying the typical problems in Fontan patients, and in this context, the profiling of endocrinological and metabolic patterns might offer a tool for the optimization of Fontan follow-up, targeted monitoring and specific adjunct treatment.

Cerebellotrigeminal Dermal Dysplasia (Gómez-López-Hernández Syndrome)

2018 ◽  
Vol 16 (05) ◽  
pp. 362-368 ◽  
Author(s):  
Federica Sullo ◽  
Agata Polizzi ◽  
Stefano Catanzaro ◽  
Selene Mantegna ◽  
Francesco Lacarrubba ◽  
...  
Keyword(s):  
De Novo ◽  
Chromosomal Rearrangements ◽  
Number Of Patients ◽  
Wide Range ◽  
Visual Problems ◽  
Neurodevelopmental Abnormalities ◽  
Clinical Triad ◽  
High Degree ◽  
Motor Handicap

Cerebellotrigeminal dermal (CTD) dysplasia is a rare neurocutaneous disorder characterized by a triad of symptoms: bilateral parieto-occipital alopecia, facial anesthesia in the trigeminal area, and rhombencephalosynapsis (RES), confirmed by cranial magnetic resonance imaging. CTD dysplasia is also known as Gómez-López-Hernández syndrome. So far, only 35 cases have been described with varying symptomatology. The etiology remains unknown. Either spontaneous dominant mutations or de novo chromosomal rearrangements have been proposed as possible explanations. In addition to its clinical triad of RES, parietal alopecia, and trigeminal anesthesia, CTD dysplasia is associated with a wide range of phenotypic and neurodevelopmental abnormalities.Treatment is symptomatic and includes physical rehabilitation, special education, dental care, and ocular protection against self-induced corneal trauma that causes ulcers and, later, corneal opacification. The prognosis is correlated to the mental development, motor handicap, corneal–facial anesthesia, and visual problems. Follow-up on a large number of patients with CTD dysplasia has never been reported and experience is limited to few cases to date. High degree of suspicion in a child presenting with characteristic alopecia and RES has a great importance in diagnosis of this syndrome.

scholarly journals Biomarkers-based personalized follow-up in chronic heart failure improves patient’s outcomes and reduces care associate cost

2021 ◽  
Vol 19 (1) ◽  
Author(s):  
Antonio Leon-Justel ◽  
Jose I. Morgado Garcia-Polavieja ◽  
Ana Isabel Alvarez-Rios ◽  
Francisco Jose Caro Fernandez ◽  
Pedro Agustin Pajaro Merino ◽  
...  
Keyword(s):  
Heart Failure ◽  
Hospital Admissions ◽  
Left Ventricular ◽  
Left Ventricular Ejection ◽  
Secondary Outcome ◽  
Ventricular Ejection Fraction ◽  
Number Of Patients ◽  
Ed Visits ◽  
The Impact

Abstract Background Heart failure (HF) is a major and growing medical and economic problem, with high prevalence and incidence rates worldwide. Cardiac Biomarker is emerging as a novel tool for improving management of patients with HF with a reduced left ventricular ejection fraction (HFrEF). Methods This is a before and after interventional study, that assesses the impact of a personalized follow-up procedure for HF on patient’s outcomes and care associated cost, based on a clinical model of risk stratification and personalized management according to that risk. A total of 192 patients were enrolled and studied before the intervention and again after the intervention. The primary objective was the rate of readmissions, due to a HF. Secondary outcome compared the rate of ED visits and quality of life improvement assessed by the number of patients who had reduced NYHA score. A cost-analysis was also performed on these data. Results Admission rates significantly decreased by 19.8% after the intervention (from 30.2 to 10.4), the total hospital admissions were reduced by 32 (from 78 to 46) and the total length of stay was reduced by 7 days (from 15 to 9 days). The rate of ED visits was reduced by 44% (from 64 to 20). Thirty-one percent of patients had an improved functional class score after the intervention, whereas only 7.8% got worse. The overall cost saving associated with the intervention was € 72,769 per patient (from € 201,189 to € 128,420) and €139,717.65 for the whole group over 1 year. Conclusions A personalized follow-up of HF patients led to important outcome benefits and resulted in cost savings, mainly due to the reduction of patient hospitalization readmissions and a significant reduction of care-associated costs, suggesting that greater attention should be given to this high-risk cohort to minimize the risk of hospitalization readmissions.

scholarly journals 288. Follow-Up Blood Cultures in Gram-Negative Bacteremia: How Do They Impact Outcomes

2020 ◽  
Vol 7 (Supplement_1) ◽  
pp. S144-S144
Author(s):  
Azza Elamin ◽  
Faisal Khan ◽  
Ali Abunayla ◽  
Rajasekhar Jagarlamudi ◽  
aditee Dash
Keyword(s):  
Clinical Outcomes ◽  
Antibiotic Treatment ◽  
Readmission Rate ◽  
Blood Cultures ◽  
Categorical Variables ◽  
Gram Negative ◽  
Number Of Patients ◽  
Significant Difference ◽  
The Impact

Abstract Background As opposed to Staphylococcus. aureus bacteremia, there are no guidelines to recommend repeating blood cultures in Gram-negative bacilli bacteremia (GNB). Several studies have questioned the utility of follow-up blood cultures (FUBCs) in GNB, but the impact of this practice on clinical outcomes is not fully understood. Our aim was to study the practice of obtaining FUBCs in GNB at our institution and to assess it’s impact on clinical outcomes. Methods We conducted a retrospective, single-center study of adult patients, ≥ 18 years of age admitted with GNB between January 2017 and December 2018. We aimed to compare clinical outcomes in those with and without FUBCs. Data collected included demographics, comorbidities, presumed source of bacteremia and need for intensive care unit (ICU) admission. Presence of fever, hypotension /shock and white blood cell (WBC) count on the day of FUBC was recorded. The primary objective was to compare 30-day mortality between the two groups. Secondary objectives were to compare differences in 30-day readmission rate, hospital length of stay (LOS) and duration of antibiotic treatment. Mean and standard deviation were used for continuous variables, frequency and proportion were used for categorical variables. P-value &lt; 0.05 was defined as statistically significant. Results 482 patients were included, and of these, 321 (67%) had FUBCs. 96% of FUBCs were negative and 2.8% had persistent bacteremia. There was no significant difference in 30-day mortality between those with and without FUBCs (2.9% and 2.7% respectively), or in 30-day readmission rate (21.4% and 23.4% respectively). In patients with FUBCs compared to those without FUBCs, hospital LOS was longer (7 days vs 5 days, P &lt; 0.001), and mean duration of antibiotic treatment was longer (14 days vs 11 days, P &lt; 0.001). A higher number of patients with FUBCs needed ICU care compared to those without FUBCs (41.4% and 25.5% respectively, P &lt; 0.001) Microbiology of index blood culture in those with and without FUBCs Outcomes in those with and without FUBCs FUBCs characteristics Conclusion Obtaining FUBCs in GNB had no impact on 30-day mortality or 30-day readmission rate. It was associated with longer LOS and antibiotic duration. Our findings suggest that FUBCs in GNB are low yield and may not be recommended in all patients. Prospective studies are needed to further examine the utility of this practice in GNB. Disclosures All Authors: No reported disclosures

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