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1.
Neumol. pediátr. (En línea) ; 19(2): 67-70, jun. 2024. ilus
Article in Spanish | LILACS | ID: biblio-1566999

ABSTRACT

Se presenta un caso poco frecuente de hemoptisis en un paciente adolescente con cirugía de Fontan, causada por el sangrado de una malformación arteriovenosa pulmonar (MAVP), tras la práctica de un instrumento de viento (saxofón). El paciente se estudió con angiografía y se realizó cierre percutáneo, consiguiendo posteriormente mejoría clínica y evitando la aparición de nuevos episodios de hemoptisis. Actualmente existe escasa literatura que reporte casos de hemoptisis en pacientes con cirugía de Fontan y ningún caso publicado en relación a gatillantes relacionados a las maniobras de valsalva; como la generada al practicar instrumentos de viento. Por esta razón, se decide publicar este caso clínico en pro de generar mayores conocimientos en este grupo de pacientes con cardiopatías congénitas operadas con procedimientos paliativos como es la cirugía de Fontan.


A rare case of hemoptysis is presented in an adolescent patient with Fontan surgery, caused by bleeding from a pulmonary arteriovenous malformation (PAVM), after playing a wind instrument (saxophone). The patient was studied with angiography and percutaneous closure was performed, subsequently achieving clinical improvement and preventing the appearance of new episodes of hemoptysis. Currently, there is little literature on hemoptysis in patients with Fontan surgery and no case published in relation to triggers related to valsalva maneuvers; like that generated when practicing wind instruments. For this reason, it was decided to publish this clinical case in order to generate greater knowledge in this group of patients with congenital heart disease operated on with palliative procedures such as Fontan surgery.


Subject(s)
Arteriovenous Malformations/complications , Pulmonary Artery/abnormalities , Pulmonary Veins/abnormalities , Fontan Procedure/adverse effects , Hemoptysis/etiology , Hemoptysis/therapy , Arteriovenous Malformations/diagnostic imaging , Pulmonary Artery/diagnostic imaging , Pulmonary Veins/diagnostic imaging , Angiography , Radiography, Thoracic , Valsalva Maneuver , Embolization, Therapeutic
2.
Med. infant ; 31(1): 31-36, Marzo 2024. Ilus, Tab
Article in Spanish | LILACS, UNISALUD, BINACIS | ID: biblio-1552833

ABSTRACT

Introducción: Se ha postulado que el uso de vasopresina tendría efectos beneficiosos en el postoperatorio de cirugía cardiovascular. Objetivo: Evaluar la respuesta a la vasopresina en el postoperatorio (POP) de cirugía de Fontan de nuestra población. Métodos: Estudio de casos y controles anidados en una cohorte retrospectiva. Se incluyeron pacientes con cirugía de Fontan entre 2014 y 2019. Se registraron variables demográficas, datos del cateterismo pre-Fontan, días de asistencia respiratoria mecánica (ARM), necesidad de inotrópicos, diuréticos, diálisis, dieta hipograsa, octreotide, sildenafil y nutrición parenteral total (NPT); balance de fluidos al primer y segundo día POP, necesidad de cateterismo en el POP, días de permanencia de tubo pleural, días de internación, necesidad de reinternación y mortalidad. Se compararon los grupos con y sin vasopresina utilizando la prueba de Mann- Whitney-Wilcoxon test. Se consideró significativa una p < 0.05. Resultados: Del total analizado, 35 pacientes recibieron vasopresina. En el grupo control fueron 58 pacientes con características similares de gravedad sin vasopresina. No se encontraron diferencias en la evolución postoperatoria entre ambos grupos. El grupo con vasopresina recibió en mayor proporción dieta hipograsa. Conclusiones: En nuestra serie el uso de vasopresina no marcó diferencias significativas en términos de morbimortalidad con relación al grupo control (AU)


Introduction: The use of vasopressin has been suggested to have beneficial effects in the postoperative period after cardiovascular surgery. Objective: To evaluate the response to vasopressin in the postoperative period (POP) of Fontan surgery in our population. Methods: Nested case-control study in a retrospective cohort. Patients who underwent Fontan surgery between 2014 and 2019 were included. Demographic variables, pre-Fontan catheterization data, days of mechanical ventilation (MRA), need for inotropics, diuretics, dialysis, low-fat diet, octreotide, sildenafil and total parenteral nutrition (TPN); fluid balance at first and second day POP, need for catheterization at POP, duration of chest tube drainage, days of hospitalization, need for readmission, and mortality were recorded. Groups with and without vasopressin were compared using the Mann-Whitney- Wilcoxon test. A p < 0.05 was considered significant. Results: Of all patients analyzed, 35 received vasopressin. The control group consisted of 58 patients with similar severity characteristics who did not receive vasopressin. No differences were found in the postoperative outcome between the two groups. The vasopressin group received a higher proportion of low-fat diet. Conclusions: In our series the use of vasopressin did not show significant differences in terms of morbidity and mortality compared to the control group (AU)


Subject(s)
Humans , Infant , Child, Preschool , Postoperative Complications/drug therapy , Arginine Vasopressin/administration & dosage , Arginine Vasopressin/therapeutic use , Fontan Procedure/adverse effects , Antidiuretic Agents/administration & dosage , Antidiuretic Agents/therapeutic use , Indicators of Morbidity and Mortality , Retrospective Studies , Treatment Outcome , Hemodynamics
3.
Chinese Journal of Hepatology ; (12): 109-112, 2023.
Article in Chinese | WPRIM | ID: wpr-970961

ABSTRACT

Fontan-associated liver disease (FALD) is one of the main complications after the Fontan procedure, manifesting mostly as liver fibrosis and even cirrhosis, with a high incidence rate and a lack of typical clinical symptoms that seriously affect patient prognosis. The specific cause is unknown, although it is considered to be associated with long-term elevated central venous pressure, impaired hepatic artery blood flow, and other relevant factors. The absence of association between laboratory tests, imaging data, and the severity of liver fibrosis makes clinical diagnosis and monitoring difficult. A liver biopsy is the gold standard for diagnosing and staging liver fibrosis. The most important risk factor for FALD is time following the Fontan procedure; therefore, it is recommended to do a liver biopsy 10 years after the Fontan procedure and to be cautious for the presence of hepatocellular carcinoma. Combined heart-liver transplantation is a recommended choice with favorable outcomes for patients with Fontan circulatory failure and severe hepatic fibrosis.


Subject(s)
Humans , Liver Diseases/pathology , Liver Cirrhosis/pathology , Liver/pathology , Carcinoma, Hepatocellular/pathology , Liver Transplantation/adverse effects , Fontan Procedure/adverse effects , Postoperative Complications/pathology , Liver Neoplasms/pathology
4.
Int. j. cardiovasc. sci. (Impr.) ; 34(5): 523-530, Sept.-Oct. 2021. tab, graf
Article in English | LILACS | ID: biblio-1340048

ABSTRACT

Abstract Background: Fontan circulation can be associated with significant morbidity, especially Protein-Losing Enteropathy (PLE). Echocardiographic parameters can provide valuable diagnostic information about a patient's risk of developing PLE after Fontan surgery. Objectives: To describe echocardiographic/ultrasonographic parameters associated with PLE in patients after Fontan surgery through a systematic review with meta-analysis. Methods: A literature search was performed in electronic databases to identify relevant studies about echocardiographic parameters and PLE prediction in children after Fontan surgery. The search terms used were: "echocardiography", "ultrasonography", "Fontan," and "protein-losing enteropathy". A p < 0.05 was considered statistically significant. Results: A total of 653 abstracts were obtained from electronic databases and bibliographic references. From these, six articles met criteria to be included in the qualitative analysis and three in the quantitative (meta-analysis). The resistance index in the superior mesenteric artery was described in three studies, and the quantitative analysis showed statistical significance (p < 0.001). Other echocardiographic and ultrasonographic parameters were also described, albeit in single studies not allowing a meta-analysis. Conclusion: This systematic review with meta-analysis identified echocardiographic and ultrasonographic parameters related to PLE in patients with Fontan physiology. Vascular ultrasonography seems to play a prominent role in this aspect, but additional studies are needed to increase the degree of evidence.


Subject(s)
Humans , Male , Female , Protein-Losing Enteropathies/diagnostic imaging , Fontan Procedure/methods , Echocardiography/methods , Ultrasonography/methods , Fontan Procedure/adverse effects
5.
Autops. Case Rep ; 9(3): e2019118, July-Sept. 2019. ilus
Article in English | LILACS | ID: biblio-1017367

ABSTRACT

Acute obstruction of superior vena cava anastomosis right after the Glenn procedure may lead to tragic consequences. We describe the case of a one-year-old child with tricuspid atresia and a previous Blalock-Taussig shunt procedure, who presented severe low cardiac output syndrome right after the Glenn procedure and died forty-four hours after the procedure. The autopsy showed obstruction of the superior vena cava anastomosis. Patients that present superior vena cava syndrome and low cardiac output right after the Glenn procedure should have the surgical anastomosis revised immediately.


Subject(s)
Humans , Female , Infant, Newborn , Heart Bypass, Right , Fontan Procedure/adverse effects , Heart Defects, Congenital/pathology , Autopsy , Fatal Outcome , Tricuspid Atresia/complications , Blalock-Taussig Procedure/adverse effects
6.
Arq. bras. cardiol ; 112(2): 130-135, Feb. 2019. tab, graf
Article in English | LILACS | ID: biblio-983830

ABSTRACT

Abstract Background: The Fontan-Kreutzer procedure (FK) was widely performed in the past, but in the long-term generated many complications resulting in univentricular circulation failure. The conversion to total cavopulmonary connection (TCPC) is one of the options for treatment. Objective: To evaluate the results of conversion from FK to TCPC. Methods: A retrospective review of medical records for patients who underwent the conversion of FK to TCPC in the period of 1985 to 2016. Significance p < 0,05. Results: Fontan-type operations were performed in 420 patients during this period: TCPC was performed in 320, lateral tunnel technique in 82, and FK in 18. Ten cases from the FK group were elected to conversion to TCPC. All patients submitted to Fontan Conversion were included in this study. In nine patients the indication was due to uncontrolled arrhythmia and in one, due to protein-losing enteropathy. Death was observed in the first two cases. The average intensive care unit (ICU) length of stay (LOS) was 13 days, and the average hospital LOS was 37 days. A functional class by New York Heart Association (NYHA) improvement was observed in 80% of the patients in NYHA I or II. Fifty-seven percent of conversions due to arrhythmias had improvement of arrhythmias; four cases are cured. Conclusions: The conversion is a complex procedure and requires an experienced tertiary hospital to be performed. The conversion has improved the NYHA functional class despite an unsatisfactory resolution of the arrhythmia.


Resumo Fundamento: O procedimento de Fontan-Kreutzer (FK) foi amplamente realizado no passado, mas a longo prazo gerou muitas complicações, resultando em falha na circulação univentricular. A conversão para conexão cavopulmonar total (CCPT) é uma das opções de tratamento. Objetivo: Avaliar os resultados da conversão de FK para CCPT. Métodos: Revisão retrospectiva de prontuários de pacientes submetidos à conversão de FK para CCPT no período de 1985 a 2016. Significância p < 0,05. Resultados: Operações do tipo Fontan foram realizadas em 420 pacientes durante este período: CCPT foi realizada em 320, técnica de túnel lateral em 82 e FK em 18. Dez casos do grupo FK foram eleitos para conversão em CCPT. Todos os pacientes submetidos à conversão de Fontan foram incluídos neste estudo. Em nove pacientes, a indicação deveu-se a arritmia não controlada e em um devido à enteropatia perdedora de proteínas. A morte foi observada nos dois primeiros casos. O tempo médio de internação na unidade de terapia intensiva (UTI) foi de 13 dias e o tempo médio de internação hospitalar foi de 37 dias. Uma classe funcional pela melhora da New York Heart Association (NYHA) foi observada em 80% dos pacientes em NYHA I ou II. Cinquenta e sete por cento das conversões devido a arritmias tiveram melhora das arritmias; quatro casos foram curados. Conclusões: A conversão é um procedimento complexo e requer que um hospital terciário experiente seja realizado. A conversão melhorou a classe funcional da NYHA, apesar de uma resolução insatisfatória da arritmia.


Subject(s)
Humans , Male , Female , Child , Adolescent , Adult , Young Adult , Heart Bypass, Right/methods , Fontan Procedure/methods , Univentricular Heart/surgery , Heart Failure/surgery , Arrhythmias, Cardiac/surgery , Arrhythmias, Cardiac/mortality , Time Factors , Retrospective Studies , Treatment Outcome , Statistics, Nonparametric , Heart Bypass, Right/mortality , Fontan Procedure/adverse effects , Fontan Procedure/mortality , Coronary Circulation , Kaplan-Meier Estimate , Univentricular Heart/mortality , Heart Failure/mortality , Length of Stay
7.
Rev. bras. cir. cardiovasc ; 31(2): 174-177, Mar.-Apr. 2016. tab, graf
Article in English | LILACS | ID: lil-792661

ABSTRACT

Abstract Six months after undergoing a Fontan operation, a 7-year-old boy with right atrial isomerism and a single functional ventricle was admitted to our emergency department with cyanosis. Emergency cardiac catheterization revealed a large veno-venous fistula that began in a left hepatic vein, connected to the left accessory hepatic veins, and drained into the common atrium, resulting in desaturation. The fistula was occluded proximally with an Amplatzer septal occluder, with satisfying results; the patient's systemic arterial saturation decreased during his hospital stay. Three weeks after the first intervention, a second procedure was performed to retrieve the first device and to close the fistula distally. Multiple attempts with different types of gooseneck snares and a bioptome catheter failed to retrieve the first device, so a telescopic method was used to re-screw it. Using a Mullins long sheath and delivery sheath, the delivery cable was manipulated to fit into the slot of the end screw, and the cable was rotated gently in a clockwise direction to re-screw the device. Then, another Amplatzer septal occluder was placed at the distal end of the fistula. In conclusion, distal transcatheter occlusion of intrahepatic veno-venous fistulas might lead to better clinical outcomes in selected patients. Amplatzer septal occluder device can be retrieve without any complication within three weeks.


Subject(s)
Humans , Male , Child , Vascular Fistula/surgery , Fontan Procedure/adverse effects , Septal Occluder Device , Hepatic Veins/surgery , Scimitar Syndrome/surgery , Venae Cavae/diagnostic imaging , Angiography , Hepatic Veins/diagnostic imaging , Jugular Veins/surgery
9.
Rev. bras. cir. cardiovasc ; 29(4): 595-605, Oct-Dec/2014. tab, graf
Article in Portuguese | LILACS | ID: lil-741722

ABSTRACT

Objetivo: Comparar a eficácia do AAS e da Varfarina na profilaxia da trombose em pacientes submetidos a operação cavopulmonar total. Avaliar se fatores de coagulação (VII, VIII e Proteína C), dados clínicos, fenestração ou fatores hemodinâmicos, interferem na trombose no pós-operatório. Métodos: Estudo prospectivo e randomizado de 30 pacientes, randomizados em Grupo I (Varfarina) e Grupo II (AAS), submetidos à derivação cavopulmonar total com tubo extracardíaco, entre 2008 e 2011, com seguimento de dois anos. Foram realizadas consultas clínicas que avaliavam efeitos colaterais e aderência. Realizado ecocardiograma transesofágico no pós-operatório imediato, 3, 6,12 e 24 meses; angiotomografia aos 6, 12 e 24 meses de pós-operatório para avaliação de alterações na parede interna do tubo ou trombos e cintilografia pulmonar, para avaliar possível TEP. Resultados: Dois óbitos no grupo I; 33,3% dos pacientes apresentaram trombo (46,7% no Grupo II). A ocorrência prévia de trombo e baixos níveis de proteína C da coagulação foram os únicos fatores que influenciaram no tempo livre de trombo (P=0,035 e 0,047). Avaliação angiotomográfica: 35,7% dos pacientes do grupo II tinham atapeteamento maior que 2 mm (P=0,082). Cintilografia: dois pacientes apresentaram TEP no grupo II. Cinco pacientes tiveram dificuldade de aderência, 4 no grupo I com INR variando de 1 a 6,4. Conclusão: A ocorrência prévia de trombo é um fator de risco para trombose no pós-operatório. Pacientes em uso de AAS tendem a depósito de material na parede do tubo. O número reduzido da amostra não permitiu concluir qual a droga mais eficaz na prevenção da trombose na população estudada. .


Objective: To compare the efficacy of aspirin and warfarin for prophylaxis of thrombosis in patients undergoing total cavopulmonary anastomosis. Evaluate whether coagulation factors (VII, VIII and protein C), clinical data, fenestration or hemodynamic factors, interfere with postoperative thrombosis. Methods: A prospective, randomized study of 30 patients, randomized into Group I (Warfarin) and Group II (AAS), underwent total cavopulmonary shunt with extracardiac conduit, between 2008 and 2011, with follow-up by clinical visits to evaluate side effects and adhesion. Performed transesophageal echocardiography in post operatory time, 3, 6,12 and 24 months; angiotomography at 6, 12 and 24 months to evaluate changes in the internal tube wall or thrombi and pulmonary scintigraphy to evaluate possible PTE. Results: Two deaths in group I; 33.3% of patients had thrombus (46.7% in Group II). The previous occurrence of thrombus and low levels of coagulation protein C were the only factors that influenced the time free of thrombus (P=0.035 and 0.047). Angiotomographic evaluation: 35.7% in group II presented material accumulation greater than 2 mm (P=0.082). Scintigraphy: two patients had PTE in group II. Five patients had difficulty to comply with the treatment, 4 in group I with INR ranging from 1 to 6.4. Conclusion: The previous occurrence of thrombus is a risk factor for thrombosis in the postoperative period. Patients using AAS tend to deposit material in the tube wall. The small sample size did not allow to conclude which is the most effective drug in the prevention of thrombosis in this population. .


Subject(s)
Adolescent , Child , Child, Preschool , Female , Humans , Male , Anticoagulants/therapeutic use , Aspirin/therapeutic use , Fontan Procedure/methods , Platelet Aggregation Inhibitors/therapeutic use , Thrombosis/prevention & control , Warfarin/therapeutic use , Echocardiography , Fontan Procedure/adverse effects , Fontan Procedure/mortality , Kaplan-Meier Estimate , Prospective Studies , Postoperative Complications/drug therapy , Postoperative Complications/prevention & control , Pulmonary Embolism/prevention & control , Pulmonary Embolism , Radionuclide Imaging , Statistics, Nonparametric , Time Factors , Tomography, X-Ray Computed , Treatment Outcome , Thrombosis/drug therapy , Thrombosis/mortality , Ventricular Dysfunction/physiopathology
10.
Rev. cuba. pediatr ; 86(4): 413-422, oct.-dic. 2014. tab, ilus
Article in Spanish | LILACS, CUMED | ID: lil-730316

ABSTRACT

INTRODU: la enteropatía perdedora de proteínas puede aparecer en la evolución de los pacientes con corazón univentricular que sobreviven a la derivación cavopulmonar total. Una vez que se diagnostica, la mortalidad es alta. OBJETIVO: identificar los posibles factores de riesgo de esta complicación. MÉTODOS: se realizó un estudio de cohorte prospectivo de la evolución en 74 pacientes con derivación cavopulmonar total, intervenidos en el Cardiocentro Pediátrico "William Soler", desde enero de 1992 hasta enero de 2011. RESULTADOS: el tiempo promedio de evolución fue de 8 años. Sufrió enteropatía perdedora de proteínas 8,1 % de los pacientes. Se presentó con mayor frecuencia en los operados con la técnica intratrial, en los operados con más de 6 años de edad, y en quienes sufrieron derrames pleurales persistentes en el posoperatorio inmediato. Se encontró relación significativa entre la enteropatía y la disfunción ventricular posoperatoria, con RR= 11,45 (IC: 95 %: 2,37 a 55,16). El análisis multivariado identificó a la disfunción ventricular como factor de riesgo. CONCLUSIÓN: la detección de disfunción ventricular en la evolución del paciente con derivación cavopulmonar debe orientar el tratamiento, en aras de evitar la aparición de enteropatía perdedora de proteínas.


INTRODUCTION: protein-losing enteropathy may occur in the progression of patients with univentricular heart, who survived total cavopulmonary shunt. Once diagnosed, the mortality rate of the condition is high. ONJECTIVE: to identify the possible risk factor of this complication. METHODS: a prospective cohort study of the progression of 74 patients with total cavopulmonary shunt was conducted from January 1992 through January 2011. They had been operated on at "William Soler" pediatric cardiac center. RESULTS: the average time of progression was 8 years. In this group, 8.1 % of patients suffered protein-losing enteropathy that was more frequently seen in patients operated on by the intraatrial technique, aged over 6 years and in those suffering persistent pleural effusion in the immediate postoperative period. Significant statistical relation was found between enteropathy and postoperative ventricular dysfunction with RR= 11.45 (CI: 95 %: 2.37 to 55.16). The multivariate analysis showed that the ventricular dysfunction was a risk factor. CONCLUSIONS: Detection of the ventricular dysfunction in the progression of a patient with cavopulmonary shunt should guide the treatment to avoid occurrence of protein-losing enteropathy.


Subject(s)
Humans , Protein-Losing Enteropathies/complications , Protein-Losing Enteropathies/etiology , Protein-Losing Enteropathies/prevention & control , Ventricular Dysfunction/prevention & control , Fontan Procedure/adverse effects , Fontan Procedure/methods , Prospective Studies , Cohort Studies
11.
Ann Card Anaesth ; 2014 Jul; 17(3): 229-231
Article in English | IMSEAR | ID: sea-153676

ABSTRACT

In patients with a right to left intracardiac shunt, air embolism results in an obligatory systemic embolization. Nonembolization of entrained air is described in a child with a single ventricle physiology who had earlier undergone bidirectional Glenn shunt construction and Damus–Kaye–Stansel anastomosis. The air entrainment was detected by intra-operative transesophageal echocardiography. The combined effect of a “diving bell” phenomenon and mild aortic valve regurgitation are suggested as the reasons for the confi nement of air into the ventricle preventing catastrophic systemic embolization.


Subject(s)
Child , Echocardiography, Transesophageal/methods , Embolism, Air/etiology , Fontan Procedure/adverse effects , Humans , Male , Monitoring, Intraoperative , Sternotomy/methods
12.
Arch. pediatr. Urug ; 85(2): 91-94, jun. 2014. ilus
Article in Spanish | LILACS | ID: lil-768426

ABSTRACT

El manejo del corazón univentricular ha mejorado laexpectativa de vida de los pacientes con la cirugía deFontan.Objetivo: identificar las complicacionesposquirúrgicas más frecuentes de la cirugía de Fontanen pacientes de una institución privada de la ciudadde Medellín.Metodología: estudio descriptivo del tipo de seriesclínicas. La población estuvo compuesta porpacientes sometidos a la cirugía de Fontan. Lainformación sobre las variables sociodemográficas yclínicas fue tomada directamente de las historiasclínicas. La investigación contó con la aprobación delComité de ética. Se empleó el programa SPSS®versión 17.0 (SPSS Inc; Chicago, Illinois, EE.UU.) parael procesamiento y análisis de la información. Para elmanejo estadístico de los datos se empleó laestadística descriptiva.Resultados: participaron 21 pacientes, la mediana deedad fue de 5 años (mínimo 3 y máximo 10); el 66,7%fueron de sexo masculino. El 100% de los niños tenían antecedentes de cirugía paliativa previa, la másfrecuente fue el Glenn bidireccional. En cuanto a lascomplicaciones más frecuentes fueron derramepleural (52,4%), disfunción miocárdica e infección enherida (19%).Conclusiones: las complicaciones que sepresentaron coinciden con lo reportado en la literatura, cabe resaltar que ningún paciente refirió estenosissubaórtica ni hemorragias.


Univentricular heart management has improved thelife expectancy of patients with Fontan surgery.Objective: identify the most common postoperative complications of the Fontan surgery in patientsfrom a private institution from Medellin.Methodology: descriptive clinical series. Thepopulation was patients undergoing the Fontansurgery. Information about sociodemographic andclinical variables was taken directly from themedical records. The research was approved bythe Ethics Committee. We used SPSS ® version17.0 (SPSS Inc, Chicago, Illinois, USA) forprocessing and analysis of information. For statistical handling of data is used for descriptiv estatistics. Results: 21 patients participated, the median agewas 5 years (minimum 3, maximum 10), 66.7% were male. 100% of the children had a history of priorpalliative surgery, the most common was the bidirectional Glenn. As for the most common complications were pleural effusion (52.4%), myocardial dysfunction and wound infection (19%). Conclusions: the complications presented agree with those reported in the literature, it is notable that no patient reported sub aortic stenosis and bleeding.


Subject(s)
Humans , Male , Female , Child, Preschool , Child , Postoperative Complications , Fontan Procedure/adverse effects , Tricuspid Atresia/surgery , Colombia , Hypoplastic Left Heart Syndrome/surgery
13.
J. bras. pneumol ; 40(1): 82-85, jan-feb/2014. graf
Article in English | LILACS | ID: lil-703608

ABSTRACT

Plastic bronchitis (PB), although a rare cause of airway obstruction, has mortality rates up to 50% in children after Fontan-type cardiac surgery. We present the case of an 18-month-old female patient with PB following pneumonia. At 6 months of age, the patient underwent the Glenn procedure due to functionally univentricular heart. Fiberoptic bronchoscopy revealed complete blockage of the left bronchus by mucoid casts. Pharmacotherapy consisted of glucocorticosteroids, azithromycin, and enalapril maleate. The child also received nebulized 3% NaCl solution, which proved to be beneficial. In children submitted to Fontan-type procedures, physicians must be alert for PB, which can be triggered by respiratory tract infection.


A bronquite plástica (BP), embora uma causa rara de obstrução de vias aéreas, apresenta taxas de mortalidade de até 50% em crianças submetidas a cirurgia cardíaca do tipo Fontan. Apresentamos o caso de uma menina de 18 meses de idade com BP secundária a pneumonia. Aos 6 meses de idade, a paciente havia sido submetida à operação de Glenn devido a coração funcionalmente univentricular. A fibrobroncoscopia revelou obstrução completa do bronco esquerdo por moldes mucoides. A farmacoterapia consistiu em glicocorticosteroides, azitromicina e maleate de enalapril. Adicionalmente, a criança recebeu nebulização de solução de NaCl a 3%, que provou ser benéfica. Em crianças submetidas a operações do tipo Fontan, devemos nos manter alerta quanto à BP, que pode ser desencadeada por infecção do trato respiratório.


Subject(s)
Female , Humans , Infant , Bronchitis/drug therapy , Bronchitis/etiology , Fontan Procedure/adverse effects , Saline Solution, Hypertonic/administration & dosage , Azithromycin/administration & dosage , Drug Therapy, Combination , Enalapril/administration & dosage , Glucocorticoids/administration & dosage , Heart Defects, Congenital/surgery , Nebulizers and Vaporizers
14.
Arq. bras. cardiol ; 97(1): e4-e7, jul. 2011. ilus
Article in Portuguese | LILACS | ID: lil-597673

ABSTRACT

Apesar dos avanços na cirurgia de Fontan, obstruções nos condutos extracardíacos podem ocorrer e causar deterioração clínica. Relatamos dois casos em que foram realizados implante de stent para correção de estenose na cirurgia de Fontan. Ascite era o sinal clínico comum; um paciente tinha enteropatia perdedora de proteínas.Todos os procedimentos obtiveram sucesso angiográfico e clínico.


Despite advances in Fontan surgery, obstruction in extracardiac ducts may occur and cause clinical deterioration. We report two cases of stent implantation for correction of stenosis in the Fontan surgery. Ascites was a common clinical sign; a patient was protein losing enteropathy. All procedures were angiographically and clinically successful.


Pese a los avances en la cirugía de Fontan, obstrucciones en los conductos extracardíacos pueden producirse y ocasionar deterioro clínico. Relatamos dos casos en que se llevaron a cabo implante de stent para la corrección de estenosis en la cirugía de Fontan. Ascitis era o el signo clínico común; un paciente tenía enteropatía perdedora de proteínas. Todos los procedimientos obtuvieron éxito angiográfico y clínico.


Subject(s)
Child , Female , Humans , Male , Fontan Procedure/adverse effects , Pulmonary Artery/surgery , Stents , Ascites/etiology , Constriction, Pathologic/surgery , Fontan Procedure/methods , Protein-Losing Enteropathies/etiology , Treatment Outcome , Vena Cava, Inferior/surgery
16.
Arq. bras. cardiol ; 94(4): e109-e112, abr. 2010. ilus
Article in Portuguese | LILACS | ID: lil-546706

ABSTRACT

Relatamos um caso incomum de associação de bronquite plástica (BP) com enteropatia perdedora de proteínas (EPP) em menina de 4 anos e 9 meses de idade. com dupla via de entrada de ventrículo único tipo esquerdo e concordância ventrículo-arterial submetida à cirurgia cavopulmonar total. com túnel lateral intracardíaco aos três anos. Tornaram-se chamativas a eliminação de molde brônquico de fibrina de 10 cm (BP) e a elevação de alfa-1-antitripsina de 52 mg/g de fezes. Em uso de sildenafila. programou-se. em caso de continuidade do processo. a ligadura do ducto torácico e transplante cardíaco.


We report an unusual case of association of plastic bronchitis (PB) to protein-losing enteropathy (PLE) in a girl of 4 years and 9 months of age with double inlet single left ventricle and ventriculoarterial concordance. submitted to total cavopulmonary surgery. with an intracardiac lateral tunnel at the age of three. The elimination of the 10 cm fibrin bronchial mold (PB) and the alpha-1-antitrypsin elevation of 52 mg/g in feces had both become outstanding. Using sildenafil. the thoracic duct ligature and the cardiac transplant were programmed in case of continuity of the process.


Subject(s)
Child, Preschool , Female , Humans , Bronchitis/etiology , Fontan Procedure/adverse effects , Protein-Losing Enteropathies/etiology , Heart Ventricles/abnormalities
17.
Rev. bras. cir. cardiovasc ; 24(4): 463-469, out.-dez. 2009. ilus, tab
Article in English, Portuguese | LILACS | ID: lil-540747

ABSTRACT

Objetivo: Estudos recentes de fluxo com modelos experimentais de anastomoses cavopulmonares totais (ACPTs) baseados em ressonância magnética e angiografia demonstram que este é um procedimento bem estabelecido para o tratamento de várias cardiopatias, mas o melhor arranjo espacial continua controverso. Nosso intuito é apresentar os resultados imediatos com três diferentes técnicas de ACPTs. Métodos: Ensaio clínico de ACPTs realizadas no período de janeiro de 2005 a julho de 2008 com 40 pacientes, com idade média de 6,4 ± 3,2 anos, com Glenn prévio. Os pacientes foram divididos em três grupos, dependendo da técnica cirúrgica empregada: Grupo 1 (G1) - túnel lateral; Grupo 2 (G2) - conduto extracardíaco; Grupo 3 (G3) - conduto intracardíaco dirigido para o ramo esquerdo de artéria pulmonar, todos com fenestração. Foram avaliadas variáveis pré e pós-operatórias. Resultados: Foram incluídos 11 pacientes no G1, 10 no G2 e 19 no G3. As variáveis pré-operatórias foram semelhantes nos três grupos (P>0,05). A mortalidade foi maior nos Grupos 1 e 2 (9,1 por cento e 10,0 por cento, respectivamente), comparadas ao Grupo 3 (zero), porém sem significância estatística (P=0,3841). Efusão pleural foi ausente no Grupo 3, diferença significativa (P=0,0128) em relação aos outros grupos (40,0 por cento e 33,3 por cento). A mediana do tempo de hospitalização pós-operatória foi menor no Grupo 3 (8 dias), em relação aos grupos 1 e 2 (18 e 13 dias, respectivamente) (P=0,0164). Conclusão: A técnica de conduto intracardíaco foi associada a menor morbidade pós-operatória, sendo a opção atual do nosso serviço na anastomose cavopulmonar total.


Objectives: Recent experimental flow studies based on angiography and magnetic resonance have shown that total cavopulmonary anastomosis (TCPA) is a valid concept for surgical treatment of many congenital heart defects, but there is not agreement of the best surgical arrangement. Our purpose is to analyze the immediate results with three different techniques of TCPA. Methods: We sought to analyze retrospectively all TCPA realized from January 2005 to July 2008; there were 40 patients, all with previous Glenn anastomosis, with mean age of 6.4 ± 3.2 years. Three different techniques were employed: Group 1 (G1) lateral tunnel, Group 2 (G2) extracardiac conduits, Group 3 (G3) intracardiac conduit directed to the left pulmonary artery. All patients had a fenestration done. Results: G1 had 11 patients, G2 10 patients and G3 19 patients. Preoperative data were similar in the 3 groups (P>0.05). Surgical mortality was higher in Groups 1 and 2 (9.1 percent and 10 percent) compared to Group 3 (0 percent) but there was no statistical significance (P=0.3841). Pleural effusion was absent in Group 3 (0 percent), which was statistically significant in relation to the other groups (P=0.0128). The hospitalization time was also significantly lower in G3 (8 days) in relation to G1 (18 days) and G2 (13 days) (P=0.0164). Conclusion Intracardiac TCPA was associated with lower postoperative morbidity and is currently our preferred technique.


Subject(s)
Child , Female , Humans , Male , Fontan Procedure , Fontan Procedure/adverse effects , Fontan Procedure/methods , Fontan Procedure/mortality , Pleural Effusion/etiology , Statistics, Nonparametric , Time Factors
19.
Arch. cardiol. Méx ; 75(4): 425-434, oct.-dic. 2005. ilus, tab
Article in Spanish | LILACS | ID: lil-631922

ABSTRACT

Se presenta un análisis retrospectivo de pacientes sometidos a cirugía de Fontan, de enero de 1989 a diciembre 2003, con el fin de evaluar los resultados, así como las variables clínicas y hemodinámicas que inciden en su morbilidad y mortalidad. Se estudiaron un total de 81 pacientes con los siguientes diagnósticos: 53 con atresia tricuspídea, 14 con atresia pulmonar con septum intacto, 11 con conexión atriouniventricular y 3 con anomalía de Ebstein. A 61 pacientes se les realizó Fontan con túnel intra-atrial y en 20 se utilizó un tubo extracardíaco, fenestrados en 53 pacientes. La mortalidad operatoria (< 30 días) fue 13% para atresia tricuspídea y 25% en el resto, siendo básicamente ésta por choque cardiogénico y arritmias graves. La sobrevida global fue de 71% en 55 meses de seguimiento. Hubo que desmantelar el Fontan en 5 casos. De las diversas variables analizadas se encontró 2.8 veces más riesgo de morir en pacientes sin fenestración y 3.6 veces cuando a las 72 hrs postquirúrgicas la presión de atrio izquierdo fue > 10 mmHg y la presión media de la arteria pulmonar > 20 mmHg. Las complicaciones más relevantes fueron: arritmias (38%), enteropatía perdedora de proteínas (8%) y eventos trombóticos cerebrales (1 %). El 52% de los pacientes han reingresado siendo las principales causas falla hemodinámica e infecciones.


The purpose of this retrospective study was to determine the outcome of patients who underwent a Fontan procedure at National Heart Institute "Ignacio Chavez", Mexico, from January 1989 to December 2003. We had 81 patients with a mean age of 7 years old: 53 with tricuspid atresia, 14 with pulmonary atresia and intact septum, 11 with univentricular atrioventricular connection and 3 with Ebstein's anomaly. An intra-atrial tunnel was performed on 61 patients and an extracardiac conduit on the rest. Of all, 53 underwent a fenestration. The surgical mortality was 13% fortricuspid atresia and 25% for the other diseases. We had an overall mortality of 28.4%, being the main causes cardiogenic shock and arrhythmia. The global survival was 71% in 55 months of following. Takedown was indicated on 5 patients. The patients without fenestration showed to have 2.8 times more risk of death. The mean pulmonary artery pressure > 20 mm Hg plus the left atrium pressure > 10 mmHg increased the death risk 3.6 times. Of the 52% who required readmission, the main causes were hemodynamical failure and infections. The more relevant complications were: arrhythmia (38%), protein-losing enteropathy (8%) and thrombotic cerebral events (1.4%).


Subject(s)
Adolescent , Adult , Child , Child, Preschool , Female , Humans , Infant , Male , Fontan Procedure , Heart Defects, Congenital/surgery , Fontan Procedure/adverse effects , Postoperative Complications/epidemiology , Postoperative Complications/etiology , Retrospective Studies , Risk Factors , Time Factors
20.
Rev. chil. cardiol ; 24(2): 157-167, abr.-jun. 2005. ilus, tab
Article in Spanish | LILACS | ID: lil-423532

ABSTRACT

Introducción: La incidencia de taquicardias auriculares (TA) luego de la operación de Fontan es alta. El acceso a la aurícula pulmonar (AuP) requiere de una punción transeptal o acceso aórtico retrógrado. Se presentan resultados iniciales con una técnica percutánea que permite el acceso a la AuP para mapeo y ablación por radiofrecuencia (ARF). Métodos: Seis EEF con mapeo 3D electroanatómico (CARTO) y ARF se efectuaron en 5 pacientes (1.2 a 17 años). Todos tenían un Fontan (túnel lateral) y TA. Vía transtoráxica se posicionó en la AuP un catéter Navistar para mapeo, estimulación y ARF. Se indujo TA con estimulación programada e isoproterenol. La secuencia de activación, áreas de bloqueo eléctrico y anatómico, y la ubicación del electrograma de His fueron definidas con mapeo 3D electroanatómico. Las zonas de interés fueron estudiadas con técnicas de “entrainment”. Luego de la ARF, el éxito se definió como la imposibilidad de reinducir TA. Resultados: Se encontró taquicardia por reentrada auricular en 5 casos y taquicardia ectópica en uno. La ARF fue exitosa en todos ellos. Hubo 1 pneumotórax y hemotórax en dos casos. En un paciente se observó recurrencia de la TA a los 3 meses, que requirió una segunda ARF, sin nueva recidiva. Ningún paciente esta recibiendo tratamiento antiarrítmico. Resumen: El acceso transtorácico percutáneo a la AuP parece apropiado para mapear y tratar las taquicardias auriculares en pacientes con cirugía de Fontan.


Subject(s)
Adolescent , Humans , Infant , Child, Preschool , Child , Catheter Ablation/methods , Electrophysiology , Fontan Procedure/adverse effects , Tachycardia, Ectopic Atrial , Tachycardia, Atrioventricular Nodal Reentry/surgery , Heart Defects, Congenital/complications , Catheterization, Peripheral/methods , Fluoroscopy , Follow-Up Studies , Postoperative Complications , Treatment Outcome , Tachycardia/etiology
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