Caracterización de la fisiología restrictiva del ventrículo derecho mediante flujo 4D / 4-D flow characterization of right ventricular restrictive physiology

Abstract: Right ventricular restrictive physiology (RVRP) occurs in diverse clinical scenarios, most frequently after repair of Tetralogy of Fallot (TOF). Cardiac magnetic resonance (CMR) can comprehensively evaluate RVRP using 4D flow along with anatomical and fibrosis characterization. Also, RVRP is associated with less pulmonary regurgitation and fewer right ventricle enlargement; its long term protective role is debated. RVRP is a challenging and relevant diagnosis, which hallmark is the presence of antegrade pulmonary arterial Flow in late diastole throughout the respiratory cycle. Also, other hemodynamic findings could aid such us flow in; caval veins, suprahepatic, coronary sinus and tricuspid valve. Obtaining all these flow curves is virtually impossible by echocardiography. CMR with 4DF is a unique and powerful technique enabling this comprehensive hemodynamic evaluation as depicted in this case.

Asociación entre el síndrome de Wolfram y la tetralogía de Fallot en una niña / Association of Wolfram syndrome with Fallot tetralogy in a girl

El síndrome de Wolfram (DIDMAOS: diabetes insípida, diabetes mellitus, atrofia óptica y sordera) esuntrastorno neurodegenerativo raro. Las mutaciones del gen WFS1 (wolframina) en el cromosoma 4 son responsables de las manifestaciones clínicas en la mayoría de los pacientes con síndrome de Wolfram. El síndrome de Wolfram también está acompanado por trastornos psiquiátricos, anomalías urodinámicas, movilidad articular limitada, neuropatía autónoma cardiovascular y gastrointestinal, hipogonadismo hipogonadotrópico en los varones y complicaciones microvasculares de la diabetes. Los datos acerca de las malformaciones cardíacas asociadas en los niños con síndrome de Wolfram disponibles en las publicaciones científicas son muy limitados. En este artículo presentamos el caso de una niña de 5 años con síndrome de Wolfram y tetralogía de Fallot.

Wolfram syndrome (DIDMOAD: diabetes insipidus, diabetes mellitus, optic atrophy and deafness) is a rare neurodegenerative disorder. Mutations of the WFS1 (wolframin) on chromosome 4 are responsible for the clinical manifestations in majority of patients with Wolfram syndrome. Wolfram syndrome is also accompanied by neurologic and psychiatric disorders, urodynamic abnormalities, restricted joint motility, cardiovascular and gastrointestinal autonomic neuropathy, hypergonadotrophic hypogonadism in males and diabetic microvascular disorders. There are very limited data in the literature regarding cardiac malformations associated in children with Wolfram syndrome. A 5-year-old girl with Wolfram syndrome and tetralogy of Fallot is presented herein.

Oral and systemic manifestations, and dental management of a pediatric patient with tetralogy of fallot: a case report / Características sistémicas, orales y manejo dental de paciente pediátrico con tetralogía de fallot: reporte de caso

Abstract: introduction: tetralogy of Fallot is a congenital heart disease and the most common cyanotic heart defect in children. It is clinically characterized by a ventricular septal defect, pulmonary stenosis, overriding aorta over ventricular septal defect and right ventricular hypertrophy. There is little or no information about the oral manifestations in patients with this pathology. A report and discussion of a pediatric patient diagnosed with Tetralogy of Fallot, its clinical manifestations, oral findings and dental management are presented. Case Report: A four-year-old male patient diagnosed with Tetralogy of Fallot and epileptic attacks. The patient has deciduous teeth with many severe early childhood caries, stomatitis and cyanotic mucous membranes, root fragments, periapical abscess and noticeable enamel hypoplasia. Conclusions: In agreement with other authors, children with systemic diseases such as Tetralogy of Fallot have a higher rate of caries, poor oral hygiene, high susceptibility to other infections and bacterial endocarditis, cyanotic mucous membranes and enamel hypoplasia. Primary prevention is critical, proper dental hygiene, regular dental check-ups and the use of antibiotic prophylaxis are particularly important, especially in high-risk patients.

Resumen: introducción: la tetralogía de Fallot es la patología cardíaca congénita y defecto cardiaco cianótico más común en niños. Clínicamente se caracteriza por una comunicación interventricular, estenosis pulmonar, cabalgamiento de la aorta sobre la comunicación interventricular e hipertrofia del ventrículo derecho. Existe poca o nula información sobre las manifestaciones orales de pacientes con esta patología. Se relata el informe y discusión de un paciente pediátrico diagnosticado con tetralogía de Fallot, sus manifestaciones clínicas, hallazgos orales y manejo dental. Refiriendo además la condición de salud oral del paciente antes y después de los procedimientos dentales. Reporte del Caso. Paciente masculino de 4 años, diagnosticado con Tetralogía de Fallot y crisis epilépticas. Presenta dentición temporal con múltiples caries de la infancia temprana severa, estomatitis y mucosas cianóticas, restos radiculares, absceso periapical y una evidente hipoplasia del esmalte. Conclusiones. En concordancia con otros autores, los niños con enfermedades sistémicas como tetralogía de Fallot tienen un mayor índice de caries, higiene oral deficiente, alta susceptibilidad a otras infecciones y a endocarditis bacteriana, mucosas cianóticas e hipoplasia del esmalte. La prevención primaria es decisiva, higiene dental apropiada, revisiones odontológicas habituales y uso de antibioprofilaxis son significativos sobre todo en pacientes de alto riego.

Absceso cerebral en un paciente con tetralogía de Fallot / Brain abscess in a patient with tetralogy of Fallot

Fundamento: Las crisis de hipoxia constituye la principal complicación de pacientes con tetralogía de Fallot, sin embargo pueden existir otras formas clínicas graves con compromiso vital. Los fenómenos embolígenos a distancia y los eventos neurológicos de causa infecciosa constituyen complicaciones que no se encuentran reportadas con frecuencia. Presentación de caso: Se presenta el caso de un preescolar que ingresa con signos de focalización motora y fiebre en el Servicio de Pediatría del Hospital Central de Huambo. Al realizar exámenes complementarios se evidencian estigmas de cardiopatía congénita cianótica e imagen tomográfica compatible con absceso de la región talámica derecha. Se describe la evolución inmediata al ingreso y se expone la experiencia particular de este caso donde se asocia una complicación neurológica de una de las cardiopatías congénitas más frecuentes en la práctica pediátrica. Conclusiones: Las complicaciones infecciosas del sistema nervioso central no son descartables en un paciente con cardiopatía congénita cianótica, debido a fenómenos embolígenos y al propio estado de hipoxemia, que crea las condiciones favorables para la invasión de gérmenes anaeróbicos en el cerebro.

Background: Hypoxia crises are the major complication of patients with tetralogy of Fallot, however there may be other serious life-threatening clinical forms. Embolic phenomena events at distance and neurological events of infectious cause are complications not widely reported in the literature. Case presentation: The case of a preschooler who was admitted with signs of motor targeting and fever in the Department of Pediatrics in the Central Hospital of Huambo is presented. When further examination is made cyanotic congenital heart disease stigmata and tomographic compatible image with abscess in the right thalamic region is evidenced. Immediate evolution at admission is described and the particular experience of this case where a neurological complication of one of the most common congenital heart diseases in pediatric practice is exposed. Conclusions: Infectious complications of the central nervous system are not disposable in a patient with cyanotic congenital heart disease, due to embolic phenomena and the state of hypoxemia itself, which creates favorable conditions for the invasion of anaerobic bacteria in the brain.

Insuficiencia valvular pulmonar en el posoperatorio alejado de tetralogía de Fallot. Aporte del ecocardiograma transtorácico convencional para la toma de decisiones / Pulmonary valve insufficiency in the postoperative tetralogy of Fallot away. Contribution of conventional transthoracic echocardiography for decision making

La insuficiencia valvular pulmonar constituye uno de los principales problemas en el posoperatorio alejado de tetralogía de Fallot, siendo su incidencia muy frecuente y dependiente de múltiples aspectos. En este trabajo se efectúa una revisión relacionada con la utilidad de la ecocardiografía transtorácica convencional como instrumento para la evaluación delos pacientes con insuficiencia valvular pulmonar en el posoperatorio alejado de la tetralogía de Fallot. Se exponen los principales conceptos fisiopatológicos de esta entidad y, en función de ellos, se presentan los parámetros ecocardiográficos más relevantes a tener en cuenta en la valoración de estos pacientes. Finalmente se consideran las pautas y la oportunidad para el reemplazo valvular pulmonar.

Pulmonary valve regurgitation is one of the main problems in the late post-op of Tetrallogy of Fallot, having a great incidence and depending on many aspects . In these paper a review is done considering the usefulness of conventional transtoracic ecocardiography as a tool to evaluate patients with pulmonary regurgitation.in the late post-op of Tetralogy of Fallot. Main physiopathologic concepts about these entity are presented, and as a function of that, the most important ecocardiographic parameters to be considered evaluating these patients are also described. Finally guidelines on the opportunity for pulmonary valve replacement are considered.

Aorto-cavitary fistula: a complication of infective endocarditis / La fístula aorto-cavitaria: una complicación de la endocarditis infecciosa

Aorto-cavitary fistulae are rare complications of infective endocarditis. The diagnosis, in the absence of concomitant aortic valve disease, replacement of aortic valve with homograft or prosthetic valve, periannular abscess and negative blood culture, requires a high index of suspicion and has important prognostic and management significance. The sensitivity of the Modified Duke Criteria is challenged in this case report with a documented right sinus of valsalva fistula to the right ventricle seen on transthoracic echocardiography.

Los fístulas aorto-cavitarias son complicaciones raras de la endocarditis infecciosa. El diagnóstico - en ausencia de la enfermedad concomitante de la válvula aórtica, el reemplazo de válvula aórtica con homoinjertos o válvulas protésicas, absceso perianular, y cultivo de sangre negativo - requiere un alto índice de sospecha, y reviste gran importancia para la prognosis y el tratamiento. En este reporte de caso, se cuestiona la sensibilidad de los Criterios de Duke Modificados, con la documentación de una fístula del seno de valsalva derecho hacia el ventrículo derecho, observada en una ecocardiografía transtorácica.

Subaortic Membrane Late after Surgical Correction of Tetralogy of Fallot

We herein report a rare case of subaortic stenosis in association with a previous tetralogy of Fallot (TOF) surgical repair, which was not taken into account as a differential diagnosis. Echocardiography plays a pivotal role in identification of this rare combination. Therefore, echocardiography should be performed periodically during follow-up of patients with surgically corrected TOF. Given the clinical complications that can result from subaortic stenosis (i.e., aortic regurgitation and infective endocarditis), early and aggressive management of this rare combination should be performed.

Implante de stent na via de saída de ventrículo direito: alternativa à paliação cirúrgica convencional? / Stenting the right ventricle outflow tract: is it an alternative to palliative surgery?

O uso de stents intravasculares em crianças com cardiopatia congênita é procedimento bem estabelecido, porém a indicação de paliação por meio de procedimentos percutâneos ao invés da cirurgia convencional permanece incerta. Neste relato são apresentados três casos de obstrução crítica de via de saída do ventrículo direito (VSVD), submetidos a ampliação da VSVD com implante de stent. O implante foi realizado com sucesso nos três casos, permitindo restabelecer o fluxo pulmonar para a árvore pulmonar e melhorar a saturação de oxigênio.

The use of intravascular stents in children with congenital heart disease is well established, however the use of palliative percutaneous therapy instead of a conventional surgery remains uncertain. We present three cases of critical right ventricular outflow tract (RVOT) obstruction treated with stenting to expand the RVOT. Stenting was successful in all three cases, reestablishing pulmonary flow into the pulmonary arteries and improving oxygen saturation.

Tetralogía de Fallot con atresia pulmonar: Morfopatología y anatomía quirúrgica / Tetralogy of Fallot with pulmonary atresia: Morphopathology and surgical anatomy

Se detalla la morfopatología de la tetralogía de Fallot con atresia pulmonar como un espectro de variaciones que sirve de fundamento para destacar la anatomía quirúrgica de esta cardiopatía y se muestra la base embriológica que determina su estructura. Se estudiaron 35 corazones con el sistema secuencial segmentario. Se determinó: situs atrial, conexiones entre los segmentos cardíacos y entre el ventrículo derecho y la vasculatura arterial pulmonar. El situs atrial fue solitus, predominó la conexión atrioventricular concordante (33), las conexiones ventriculoarteriales fueron concordantes (17), doble salida de ventrículo derecho (9) y única vía de salida (9). Todos presentaron atresia valvular pulmonar; el tronco pulmonar fue hipoplásico (20), atrésico proximal (6) y ausente (9), ramas pulmonares confluentes e hipoplásicas (25), ausencia de confluencia (5), ausencia total de ramas y de conductos arteriosos (5) y colaterales aortopulmonares (10). Los corazones mostraron un espectro morfopatológico de severidad que expresa la tendencia a la desaparición de la conexión entre el ventrículo derecho y la circulación arterial intrapulmonar. Se resaltan las formas de la irrigación arterial pulmonar como fundamento para unifocalizar el flujo hacia los pulmones y se hace hincapié en la utilidad de la clasificación anatomoquirúrgica de Barbero Marcial. El conocimiento embriológico es útil para entender las conexiones vasculares del ventrículo derecho con los derivados de los sextos arcos aórticos, los vasos arteriales intrapulmonares y las colaterales aortopulmonares.

The morphopathology of tetralogy of Fallot with pulmonary atresia is detailed as a spectrum of variations which is the foundation to highlight the surgical anatomy of this cardiopathy and it is shown the embryological basis which determines its structure. Thirty five hearts were studied with the methodology of the segmental sequential system. The atrial situs, the connections between the cardiac chambers and between the right ventricle and the arterial pulmonary vasculature were determined. The atrial situs was solitus, the concordant atrioventricular connection was the most frequent (33), the ventriculoarterial connections were concordant (17), double outlet right ventricle (9) and single outlet (9). All hearts had atresia of the pulmonary valve; the pulmonary trunk was hypoplastic (20), atretic proximally (6) and completely absent (9), confluent and hypoplastic pulmonary branches (25), absence of confluence (5), complete absence of pulmonary branches and arterial ducts (5) and presence of aortopulmonary collaterals (10). The hearts show a morphopathologic spectrum of severity which documents the tendency in disappearing the connection between the right ventricle and the intrapulmonary arterial circulation. The determination of the arterial supply to the lungs is highlighted to unifocalize the blood flow toward the lungs. The usefulness of Barbero Marcial's surgical classification is emphasized. The embryologic knowledge is basic in understanding the vascular connections between right ventricle and the derivatives of embryonic sixths aortic arches, the intrapulmonary arterial vessels and the aortopulmonary collaterals.

Tetralogy of Fallot: a case report and dental considerations / Tetralogia de Fallot: relato de caso com considerações dentárias

OBJETIVO: Apresentar um caso de Tetralogia de Fallot, uma doença cardíaca congênita grave, que pode causar um impacto nos tecidos dentários, os quais necessitam de tratamento. DISCUSSÃO E CONCLUSÃO: O tratamento adequado de pacientes com problemas cardíacos depende do conhecimento do clínico a respeito da condição. Este relato enfatiza o manejo odontológico de pacientes com Tetralogia de Fallot.

OBJECTIVE: To present a case of Tetralogy of Fallot, a serious congenital heart disease, which can have an impact upon the dental tissue and which needs dental care. DISCUSSION AND CONCLUSION: Successful management of cardiac patients depends upon, knowledge about the condition. This paper highlights on dental management of Tetralogy of Fallot patients.

A new association: Dandy-Walker's variant and tatralogy of Fallot with atrial septal defect and patent ductus arteriosus and primary hypothyroidy.

An 11-month-old girl who has Dandy-Walker's variant (DWV) associated with tetralogy of Fallot (TOF), atrial septal defect (ASD), patent ductus arteriosus (PDA), and primary hypothyroidy is presented. There has been no report describing a case of DWV associated with TOF, ASD and PDA and primary hypothyroidy. The first case of Dandy-Walker malformation associated with TOF was reported by Kohyama et al in 1988, since then, a few cases were reported in the literature. Our patient is the first reported case.

O uso de dexmedetomidina na sedação de crises hipercianóticas em um recém-nascido com tetralogia de Fallot / Sedation of hypercyanotic spells in a neonate with tetralogy of Fallot using dexmedetomidine

OBJETIVO: A sedação é um passo importante para aliviar e prevenir a recorrência de cianose no manejo de pacientes com crises hipercianóticas associadas à tetralogia de Fallot (T4F). Este relato de caso ilustra a eficácia da sedação induzida por dexmedetomidina no manejo de crises hipercianóticas em um recém-nascido com T4F. DESCRIÇÃO: Um paciente recém-nascido a termo de 8 dias de idade com T4F apresentava crises hipercianóticas, indicadas por quedas abruptas no nível de saturação arterial (SpO2), medido por um oxímetro de pulso, de 80 por cento até 50 por cento, quando o paciente ficava agitado ou irritável. Nós começamos a infusão contínua de dexmedetomidina em uma dosagem de 0,2 µg/kg/min sem injeção de ataque em bolus. Cerca de meia hora depois do início da infusão de dexmedetomidina, o paciente atingiu um nível aceitável de sedação, e sua freqüência cardíaca diminuiu aproximadamente 20 batidas por minuto. Não houve nenhuma depressão respiratória aparente ou mudança acentuada em sua pressão arterial. A SpO2 também continuou estável durante a infusão de dexmedetomidina. No dia seguinte à sua hospitalização, o paciente passou com sucesso por uma operação de anastomose de Blalock-Taussig. COMENTÁRIOS: A dexmedetomidina pode ser útil no manejo de crises hipercianóticas em pacientes pediátricos com T4F.

OBJECTIVE:Sedation is an important step in the management of patients with hypercyanotic spells associated with tetralogy of Fallot (TOF) to ameliorate and prevent recurrence of cyanosis. This case report illustrates the effectiveness of dexmedetomidine-induced sedation in the management of hypercyanotic spells in a neonate with TOF. DESCRIPTION: An 8-day-old term newborn patient with TOF showed hypercyanotic spells, as indicated by an abrupt decrease in arterial saturation (SpO2) level measured by a pulse oximeter from 80 percent to as low as 50 percent, when the patient became irritable and agitated. We started continuous infusion of dexmedetomidine at a dose of 0.2 µg/kg/min without a loading bolus injection. About half an hour after commencement of dexmedetomidine infusion, the patient reached an acceptable level of sedation, together with a drop in heart rate by approximately 20 beats/min. There was no apparent respiratory depression or marked change in blood pressure. SpO2 was also stable during dexmedetomidine infusion. The patient underwent a successful Blalock-Taussig shunt operation on the next day of admission. COMMENTS: Dexmedetomidine may be useful for the management of hypercyanotic spells in pediatric patients with TOF.