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1.
NPJ Genom Med ; 9(1): 34, 2024 May 30.
Artículo en Inglés | MEDLINE | ID: mdl-38816462

RESUMEN

Kawasaki disease (KD) is a multisystem inflammatory illness of infants and young children that can result in acute vasculitis. The mechanism of coronary artery aneurysms (CAA) in KD despite intravenous gamma globulin (IVIG) treatment is not known. We performed a Whole Genome Sequencing (WGS) association analysis in a racially diverse cohort of KD patients treated with IVIG, both using AHA guidelines. We defined coronary aneurysm (CAA) (N = 234) as coronary z ≥ 2.5 and large coronary aneurysm (CAA/L) (N = 92) as z ≥ 5.0. We conducted logistic regression models to examine the association of genetic variants with CAA/L during acute KD and with persistence >6 weeks using an additive model between cases and 238 controls with no CAA. We adjusted for age, gender and three principal components of genetic ancestry. The top significant variants associated with CAA/L were in the intergenic regions (rs62154092 p < 6.32E-08 most significant). Variants in SMAT4, LOC100127, PTPRD, TCAF2 and KLRC2 were the most significant non-intergenic SNPs. Functional mapping and annotation (FUMA) analysis identified 12 genomic risk loci with eQTL or chromatin interactions mapped to 48 genes. Of these NDUFA5 has been implicated in KD CAA and MICU and ZMAT4 has potential functional implications. Genetic risk score using these 12 genomic risk loci yielded an area under the receiver operating characteristic curve (AUC) of 0.86. This pharmacogenomics study provides insights into the pathogenesis of CAA/L in IVIG-treated KD and shows that genomics can help define the cause of CAA/L to guide management and improve risk stratification of KD patients.

2.
medRxiv ; 2024 Feb 01.
Artículo en Inglés | MEDLINE | ID: mdl-38352371

RESUMEN

Background: Kawasaki disease (KD) is a multisystem inflammatory illness of infants and young children that can result in acute vasculitis. The pathological walls of afflicted coronary arteries show propensity for forming thrombosis and aneurysms. The mechanism of coronary artery aneurysms (CAA) despite intravenous gamma globulin (IVIG) treatment is not known. Methods: We performed a Whole Genome Sequencing (WGS) association analysis in a racially diverse cohort of KD patients treated with IVIG, both using AHA guidelines. We defined coronary aneurysm (CAA) (N = 234) as coronary z>2.5 and large coronary aneurysm (CAA/L) (N = 92) as z>5.0. We conducted logistic regression models to examine the association of genetic variants with CAA/L during acute KD and with persistence >6 weeks using an additive model between cases and 238 controls with no CAA. We adjusted for age, gender and three principal components of genetic ancestry. We performed functional mapping and annotation (FUMA) analysis and further assessed the predictive risk score of genomic risk loci using the area under the receiver operating characteristic curve (AUC). Results: The top significant variants associated with CAA/L were in the intergenic regions (rs62154092 p<6.32E-08 most significant). Variants in SMAT4, LOC100127 , PTPRD, TCAF2 and KLRC2 were the most significant non-intergenic SNPs. FUMA identified 12 genomic risk loci with eQTL or chromatin interactions mapped to 48 genes. Of these NDUFA5 has been implicated in KD CAA and MICU and ZMAT4 has potential functional implications. Genetic risk score using these 12 genomic risk loci yielded an AUC of 0.86. Conclusions: This pharmacogenomics study provides insights into the pathogenesis of CAA/L in IVIG-treated KD patients. We have identified multiple novel SNPs associated with CAA/L and related genes with potential functional implications. The study shows that genomics can help define the cause of CAA/L to guide management and improve risk stratification of KD patients.

3.
Respir Med Case Rep ; 46: 101939, 2023.
Artículo en Inglés | MEDLINE | ID: mdl-37942172

RESUMEN

Sarcoidosis is a multisystem granulomatous disorder of unknown etiology, manifesting with bilateral hilar adenopathy, pulmonary reticular opacities, skin, joint or eye lesions. Heerfordt-Waldenström Syndrome - a constellation of facial palsy, fever, uveitis and parotitis - is a rare presentation of this disorder. A 47-year-old Chinese woman presented with unintentional weight loss, lethargy with mediastinal and hilar lymphadenopathy. Biopsy of the right paratracheal lymph node via mediastinoscopy showed mycobacterial granulomatous lymphadenitis consistent with tuberculosis with several acid-fast bacilli identified. Lymphoproliferative disorder was ruled out. She was started on treatment for tuberculosis. Eleven weeks into treatment, she developed a right facial palsy accompanied with fever, uveitis and occipital headache. At this juncture, further history revealed a background of recurrent alternating facial palsy and parotid gland enlargement which was treated for Bell's palsy by three different doctors. New nodules appeared in the left lobe of the thyroid gland. Biopsy of a palpable thyroid nodule and a right supraclavicular lymph nodule showed histological features suggestive of sarcoidosis. Fungal and mycobacterial infections were ruled out. In addition, examination of her cerebral spinal fluid showed lymphocytic inflammation. The serum ACE level was not raised. A diagnosis of sarcoidosis with incomplete features of Heerfordt-Waldenström Syndrome along with thyroid and meningeal involvement was made. The patient was commenced on prednisolone and azathioprine and her symptoms responded shortly after. We present a rare case of Heerfordt-Waldenström Syndrome with thyroid and meningeal involvement in a Chinese woman.

4.
Front Pediatr ; 11: 1203431, 2023.
Artículo en Inglés | MEDLINE | ID: mdl-37441576

RESUMEN

Introduction: Kawasaki Disease (KD) is a leading cause of pediatric acquired heart disease in the United States, affecting up to 7,000 children annually. Seasonal variation, an epidemiological characteristic of KD, has previously been reported predominantly among Asian children; however, little is known about the epidemiology and seasonality of KD of Black children within the U.S. Methods: Electronic medical records were abstracted from 529 hospitalized KD patients admitted to a single tertiary center in Alabama between 2005 and 2019. Medical charts were reviewed to confirm KD diagnosis following American Heart Association criteria. Cases were stratified by the month of diagnosis date to assess seasonality, and statewide distribution of incidence is reported at county level using geographical spatial analysis. Comparisons were performed between Black patients and White patients with KD. Results: The average number of KD cases per year was 35. Approximately, 60% were males and 44% were White children (N = 234), 45% were Black children (N = 240) and 11% were other races (N = 55). Black children were younger than White children at KD admission (median age 32 vs. 41 months respectively, p = 0.02). Overall, the highest rates of cases occurred between January and April. When stratifying by race, cases started to rise in December among White children with the highest rates between February and April with a peak in March. Among Black children cases were high during the winter season (January-April) with a peak in April. Similarly high rates also occurred in June, July and November. There were no differences in geographical distribution of cases by race. Conclusion: KD incidence among White children in Alabama follows a seasonal cycle similar to other regions in the U.S. However, sustained incidence and additional peaks outside of the usual KD seasonality were seen among Black children with KD. Further studies are needed to investigate differential triggers between races.

5.
Disabil Health J ; 16(2): 101427, 2023 04.
Artículo en Inglés | MEDLINE | ID: mdl-36621354

RESUMEN

BACKGROUND: Advances in medicine and technology, have enabled greater numbers of children with complex illness to survive into adulthood. Adolescents with these conditions are at high risk for adverse outcomes when transitioning to adult health care. The "Staging Transition for Every Patient" (STEP) Program was developed to systematically improve the transition from pediatric to adult healthcare. OBJECTIVE: This article details the development of the STEP program and the novel use of "Individualized Transition Plans" (ITP) in the clinic setting. METHODS: A provider needs' assessment of the existing transition services among youth with specific diagnoses was performed, a steering committee was developed that created a transition policy, and a medical home within the adult system was established with an interdisciplinary approach. The ITP focuses on 5 individualized goals, it was developed and tested with the first-year cohort of patients. RESULTS: In the initial needs assessment, 7 of 35 diagnoses were found to have an effective transition plan. The STEP program partnered with departments across the adult facility to conduct 267 interdisciplinary patient visits. In the first year, 169 new patients were seen in the clinic. The average age was 23.0 ± 4.1 years old. The ITP goals included referrals to adult specialists, advanced care planning, career and education, transition readiness, caregiver burden, and an emergency sick plan. CONCLUSION: There is a need for organized transition care for medically complex youth. The STEP program answers that need by addressing the unique needs of each patient. Individualized transition planning builds trust and addresses multiple domains of health.


Asunto(s)
Personas con Discapacidad , Transición a la Atención de Adultos , Adulto , Adolescente , Humanos , Niño , Adulto Joven
6.
Heart Rhythm ; 18(11): 1825-1832, 2021 11.
Artículo en Inglés | MEDLINE | ID: mdl-34333088

RESUMEN

BACKGROUND: Children with catecholaminergic polymorphic ventricular tachycardia (CPVT) are at risk for sudden death, and a risk stratification tool does not exist. OBJECTIVE: The purpose of this study was to determine whether proband status, age at symptom onset, and/or sex are independent predictors of cardiac events. METHODS: A multicenter, ambispective, cohort of pediatric CPVT patients was categorized by sex, proband status, and age at symptom onset (D1: first decade of life [symptom onset <10 years] or D2: second decade of life [symptom onset 10-18 years, inclusive]). Demographics, therapy, genetics, and outcomes were compared between groups. RESULTS: A total of 133 patients were included and stratified into 58 D1 and 75 D2 patients (68 female and 65 male; 106 probands and 27 relatives). Localization of RYR2 variants to hotspots differed based on proband status and age at symptom onset. The cardiac event rate was 33% (n = 44/133), inclusive of a 3% (n = 4/133) mortality rate, over a median of 6 years (interquartile range 3-11) after time of symptom onset. Proband status, rather than age at of symptom onset or sex, was an independent predictor of time to first cardiac event (P = .008; hazard ratio = 4.4). The 5-, 10- and 15-year event-free survival rates for probands were 77%, 56%, and 46%, respectively, and for relatives were 96%, 91%, and 86%, respectively. Event risk after diagnosis was 48% (32/67) in patients on ß-blocker or flecainide alone vs 10% (5/48) in patients on ß-blocker plus flecainide and/or left cardiac sympathetic denervation (P <.001). CONCLUSION: Proband status, but not age at symptom onset or male sex, independently predicted an earlier onset of cardiac events. A larger sample size would enable a comprehensive investigation of other risk factors.


Asunto(s)
Taquicardia Ventricular/epidemiología , Adolescente , Edad de Inicio , Canadá/epidemiología , Niño , Femenino , Humanos , Masculino , Factores de Riesgo , Índice de Severidad de la Enfermedad , Factores Sexuales , Taquicardia Ventricular/terapia , Estados Unidos/epidemiología
7.
J Pediatr ; 229: 54-60.e2, 2021 02.
Artículo en Inglés | MEDLINE | ID: mdl-32980379

RESUMEN

OBJECTIVE: To determine whether Black children with Kawasaki disease exhibit disparities in prevalence, sequelae, and response to intravenous gamma globulin (IVIG) treatment. STUDY DESIGN: International Classification of Diseases codes were used to identify children with Kawasaki disease admitted to a tertiary center in the southeastern US. Subjects diagnosed and treated according to American Heart Association criteria were included. Demographic, laboratory, clinical, and echocardiographic data from the electronic medical record (2000-2015) were compared between Blacks and Whites. RESULTS: Data from 369 subjects (52% Whites and 48% Blacks) were included in our analysis. No significant differences related to timely admission, IVIG treatment, or coronary artery (CA) abnormalities during hospitalization were observed. Blacks showed lower IVIG response rates than Whites for patients administered IVIG within 10 days of fever onset (86.6% vs 95.6%; P = .007). Blacks received more ancillary drugs (9.6% vs 2.6%; P = .003), and endured longer hospitalizations (mean, 5 ± 3.9 days vs 3.4 ± 2.2 days; P = .001). Blacks presented with higher C-reactive protein level and erythrocyte sedimentation rate and lower hemoglobin, albumin, and sodium levels. Blacks had a higher proportion of persistent CA abnormalities than Whites at second follow-up echocardiogram (14.5% vs 6.3%; P = .03), and at third follow-up echocardiogram (21.2% vs 6.9%; P = .01). CONCLUSIONS: Compared with White children, Black children with Kawasaki disease had higher IVIG refractory prevalence, more severe inflammation, more ancillary treatments, and longer hospitalizations. Despite no racial differences in time to diagnosis or initial treatment, there was greater CA abnormality persistence among Black children at follow-up.


Asunto(s)
Negro o Afroamericano , Disparidades en el Estado de Salud , Síndrome Mucocutáneo Linfonodular/etnología , Sedimentación Sanguínea , Proteína C-Reactiva/análisis , Preescolar , Aneurisma Coronario/diagnóstico por imagen , Ecocardiografía , Femenino , Hemoglobinas/análisis , Humanos , Inmunoglobulinas Intravenosas/uso terapéutico , Tiempo de Internación/estadística & datos numéricos , Masculino , Síndrome Mucocutáneo Linfonodular/terapia , Estudios Retrospectivos , Albúmina Sérica , Sodio/sangre , Sudeste de Estados Unidos/epidemiología , Población Blanca
11.
PLoS One ; 13(11): e0205925, 2018.
Artículo en Inglés | MEDLINE | ID: mdl-30403697

RESUMEN

BACKGROUND: Catecholaminergic polymorphic ventricular tachycardia (CPVT) is often a life-threatening arrhythmia disorder with variable penetrance and expressivity. Little is known about the incidence or outcomes of CPVT patients with ≥2 variants. METHODS: The phenotypes, genotypes and outcomes of patients in the Pediatric and Congenital Electrophysiology Society CPVT Registry with ≥2 variants in genes linked to CPVT were ascertained. The American College of Medical Genetics & Genomics (ACMG) criteria and structural mapping were used to predict the pathogenicity of variants (3D model of pig RyR2 in open-state). RESULTS: Among 237 CPVT subjects, 193 (81%) had genetic testing. Fifteen patients (8%) with a median age of 9 years (IQR 5-12) had ≥2 variants. Sudden cardiac arrest occurred in 11 children (73%), although none died during a median follow-up of 4.3 years (IQR 2.5-6.1). Thirteen patients (80%) had at least two RYR2 variants, while the remaining two patients had RYR2 variants plus variants in other CPVT-linked genes. Among all variants identified, re-classification of the commercial laboratory interpretation using ACMG criteria led to the upgrade from variant of unknown significance (VUS) to pathogenic/likely pathogenic (P/LP) for 5 variants, and downgrade from P/LP to VUS for 6 variants. For RYR2 variants, 3D mapping using the RyR2 model suggested that 2 VUS by ACMG criteria were P/LP, while 2 variants were downgraded to likely benign. CONCLUSIONS: This severely affected cohort demonstrates that a minority of CPVT cases are related to ≥2 variants, which may have implications on family-based genetic counselling. While multi-variant CPVT patients were at high-risk for sudden cardiac arrest, there are insufficient data to conclude that this genetic phenomenon has prognostic implications at present. Further research is needed to determine the significance and generalizability of this observation. This study also shows that a rigorous approach to variant re-classification using the ACMG criteria and 3D mapping is important in reaching an accurate diagnosis, especially in the multi-variant population.


Asunto(s)
Predisposición Genética a la Enfermedad , Sistema de Registros , Taquicardia Ventricular/genética , Adolescente , Niño , Preescolar , Femenino , Humanos , Recién Nacido , Masculino , Miocardio/patología , Dominios Proteicos , Canal Liberador de Calcio Receptor de Rianodina/química , Canal Liberador de Calcio Receptor de Rianodina/genética , Taquicardia Ventricular/terapia , Resultado del Tratamiento
14.
Europace ; 20(3): 541-547, 2018 03 01.
Artículo en Inglés | MEDLINE | ID: mdl-28158428

RESUMEN

Aims: Catecholaminergic polymorphic ventricular tachycardia (CPVT) is an ion channelopathy characterized by ventricular arrhythmia during exertion or stress. Mutations in RYR2-coded Ryanodine Receptor-2 (RyR2) and CASQ2-coded Calsequestrin-2 (CASQ2) genes underlie CPVT1 and CPVT2, respectively. However, prognostic markers are scarce. We sought to better characterize the phenotypic and genotypic spectrum of CPVT, and utilize molecular modelling to help account for clinical phenotypes. Methods and results: This is a Pediatric and Congenital Electrophysiology Society multicentre, retrospective cohort study of CPVT patients diagnosed at <19 years of age and their first-degree relatives. Genetic testing was undertaken in 194 of 236 subjects (82%) during 3.5 (1.4-5.3) years of follow-up. The majority (60%) had RyR2-associated CPVT1. Variant locations were predicted based on a 3D structural model of RyR2. Specific residues appear to have key structural importance, supported by an association between cardiac arrest and mutations in the intersubunit interface of the N-terminus, and the S4-S5 linker and helices S5 and S6 of the RyR2 C-terminus. In approximately one quarter of symptomatic patients, cardiac events were precipitated by only normal wakeful activities. Conclusion: This large, multicentre study identifies contemporary challenges related to the diagnosis and prognostication of CPVT patients. Structural modelling of RyR2 can improve our understanding severe CPVT phenotypes. Wakeful rest, rather than exertion, often precipitated life-threatening cardiac events.


Asunto(s)
Calsecuestrina/genética , Mutación , Canal Liberador de Calcio Receptor de Rianodina/genética , Taquicardia Ventricular/genética , Adolescente , Niño , Análisis Mutacional de ADN , Muerte Súbita Cardíaca/epidemiología , Femenino , Marcadores Genéticos , Predisposición Genética a la Enfermedad , Herencia , Humanos , Masculino , Modelos Moleculares , Linaje , Fenotipo , Pronóstico , Conformación Proteica , Sistema de Registros , Estudios Retrospectivos , Factores de Riesgo , Canal Liberador de Calcio Receptor de Rianodina/química , Canal Liberador de Calcio Receptor de Rianodina/metabolismo , Relación Estructura-Actividad , Taquicardia Ventricular/diagnóstico , Taquicardia Ventricular/mortalidad , Taquicardia Ventricular/fisiopatología
16.
Circ Cardiovasc Genet ; 9(2): 136-146, 2016 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-26969752

RESUMEN

BACKGROUND: Calmodulin (CaM) is encoded by 3 genes, CALM1, CALM2, and CALM3, all of which harbor pathogenic variants linked to long QT syndrome (LQTS) with early and severe expressivity. These LQTS-causative variants reduce CaM affinity to Ca(2+) and alter the properties of the cardiac L-type calcium channel (CaV1.2). CaM also modulates NaV1.5 and the ryanodine receptor, RyR2. All these interactions may play a role in disease pathogenesis. Here, we determine the spectrum and prevalence of pathogenic CaM variants in a cohort of genetically elusive LQTS, and functionally characterize the novel variants. METHODS AND RESULTS: Thirty-eight genetically elusive LQTS cases underwent whole-exome sequencing to identify CaM variants. Nonsynonymous CaM variants were over-represented significantly in this heretofore LQTS cohort (13.2%) compared with exome aggregation consortium (0.04%; P<0.0001). When the clinical sequelae of these 5 CaM-positive cases were compared with the 33 CaM-negative cases, CaM-positive cases had a more severe phenotype with an average age of onset of 10 months, an average corrected QT interval of 676 ms, and a high prevalence of cardiac arrest. Functional characterization of 1 novel variant, E141G-CaM, revealed an 11-fold reduction in Ca(2+)-binding affinity and a functionally dominant loss of inactivation in CaV1.2, mild accentuation in NaV1.5 late current, but no effect on intracellular RyR2-mediated calcium release. CONCLUSIONS: Overall, 13% of our genetically elusive LQTS cohort harbored nonsynonymous variants in CaM. Genetic testing of CALM1-3 should be pursued for individuals with LQTS, especially those with early childhood cardiac arrest, extreme QT prolongation, and a negative family history.


Asunto(s)
Calmodulina/genética , Síndrome de QT Prolongado/genética , Mutación Missense/genética , Secuencia de Aminoácidos , Animales , Calmodulina/química , Demografía , Femenino , Humanos , Masculino , Ratones Endogámicos C57BL , Prevalencia , Adulto Joven
17.
Circ Arrhythm Electrophysiol ; 8(3): 633-42, 2015 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-25713214

RESUMEN

BACKGROUND: Catecholaminergic polymorphic ventricular tachycardia is an uncommon, potentially lethal, ion channelopathy. Standard therapies have high failure rates and little is known about treatment in children. Newer options such as flecainide and left cardiac sympathetic denervation are not well validated. We sought to define treatment outcomes in children with catecholaminergic polymorphic ventricular tachycardia. METHODS AND RESULTS: This is a Pediatric and Congenital Electrophysiology Society multicenter, retrospective cohort study of catecholaminergic polymorphic ventricular tachycardia patients diagnosed before 19 years of age. The cohort included 226 patients, including 170 probands and 56 relatives. Symptomatic presentation was reported in 176 (78%). Symptom onset occurred at 10.8 (interquartile range, 6.8-13.2) years with a delay to diagnosis of 0.5 (0-2.6) years. Syncope (P<0.001), cardiac arrest (P<0.001), and treatment failure (P=0.008) occurred more often in probands. ß-Blockers were prescribed in 205 of 211 patients (97%) on medication, and 25% experienced at least 1 treatment failure event. Implantable cardioverter defibrillators were placed in 121 (54%) and was associated with electrical storm in 22 (18%). Flecainide was used in 24% and left cardiac sympathetic denervation in 8%. Six deaths (3%) occurred during a cumulative follow-up of 788 patient-years. CONCLUSIONS: This study demonstrates a malignant phenotype and lengthy delay to diagnosis in catecholaminergic polymorphic ventricular tachycardia. Probands were typically severely affected. ß-Blockers were almost universally initiated; however, treatment failure, noncompliance and subtherapeutic dosing were often reported. Implantable cardioverter defibrillators were common despite numerous device-related complications. Treatment failure was rare in the quarter of patients on flecainide. Left cardiac sympathetic denervation was not uncommon although the indication was variable.


Asunto(s)
Antiarrítmicos/uso terapéutico , Muerte Súbita Cardíaca/prevención & control , Cardioversión Eléctrica , Simpatectomía , Taquicardia Ventricular/terapia , Adolescente , Factores de Edad , Antiarrítmicos/efectos adversos , Niño , Muerte Súbita Cardíaca/etiología , Desfibriladores Implantables , Cardioversión Eléctrica/efectos adversos , Cardioversión Eléctrica/instrumentación , Cardioversión Eléctrica/mortalidad , Femenino , Humanos , Masculino , Selección de Paciente , Fenotipo , Sistema de Registros , Estudios Retrospectivos , Factores de Riesgo , Índice de Severidad de la Enfermedad , Simpatectomía/efectos adversos , Simpatectomía/mortalidad , Taquicardia Ventricular/complicaciones , Taquicardia Ventricular/diagnóstico , Taquicardia Ventricular/mortalidad , Taquicardia Ventricular/fisiopatología , Factores de Tiempo , Resultado del Tratamiento
19.
J Thorac Cardiovasc Surg ; 148(6): 2517-23.e1, 2014 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-25277471

RESUMEN

OBJECTIVE: Dating back to the first published report of the Fontan circulation in 1971, multiple studies have examined the long-term results of this standard procedure for palliation of single-ventricle heart disease in children. Although the technique has evolved over the last 4 decades to include a polytetrafluorethylene (PTFE) conduit for a large percentage of patients, the long-term outcome has not yet been established. The aim of the current study was to investigate the possibility of a late increasing risk for death after 15 years among patients with a modern Fontan operation and to evaluate late morbidity. METHODS: Between January 1, 1988, and December 31, 2011, 207 patients underwent the Fontan procedure using an internal or external PTFE conduit plus a bidirectional cavopulmonary connection. Survival and late adverse events were analyzed. Risk factors for early and late mortality were examined using hazard function methodology. RESULTS: At 1, 10, and 20 years, survival for the entire cohort was 95%, 88%, and 76%, respectively, with no deaths in the last 6 years of the study. Hazard modeling showed a 1.3% risk of death per year 24 years after the Fontan procedure, with no late increasing hazard phase. Freedom from reoperations was greater than 90% at 20 years and freedom from thrombotic complications was 98% at 20 years (with greater than 80% of patients on aspirin alone). Survival curves were superimposable for 16- to 20-mm conduits, and the freedom from any reoperation including transplantation was greater than 90% after 20 years. Multivariable risk factor analysis identified only earlier date of operation as a predictor of early and late mortality. By era of surgery, the 10-year predicated survival is 89% for patients undergoing surgery in 2000 and 94% for patients in 2010. CONCLUSIONS: Early and late survival after a Fontan operation with a PTFE conduit is excellent, with no late phase of increasing death risk after 20 years. Late functional status is good, the need for late reoperation is rare, and thrombotic complications are uncommon on a standard medical regimen including aspirin as the only anticoagulation medication.


Asunto(s)
Procedimiento de Fontan/mortalidad , Cardiopatías Congénitas/cirugía , Anticoagulantes/uso terapéutico , Procedimiento de Fontan/efectos adversos , Procedimiento de Fontan/instrumentación , Cardiopatías Congénitas/diagnóstico , Cardiopatías Congénitas/mortalidad , Humanos , Estimación de Kaplan-Meier , Análisis Multivariante , Cuidados Paliativos , Complicaciones Posoperatorias/mortalidad , Complicaciones Posoperatorias/cirugía , Modelos de Riesgos Proporcionales , Reoperación , Estudios Retrospectivos , Medición de Riesgo , Factores de Riesgo , Sobrevivientes , Factores de Tiempo , Resultado del Tratamiento
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