A Rare Associated Anomaly in Tetralogy of Fallot: Scimitar Syndrome in an Infant

Abstract Pulmonary venous connections may be infrequently abnormal in patients with tetralogy of Fallot (TOF). A special subgroup of partial anomalous pulmonary venous return,"scimitar cyndrome", and its coexistence with TOF is less frequently reported. It may proceed unnoticed, as cyanosis already predominates in the clinical picture. This uncommon association must be kept in mind for patients with TOF who have an accessory flow in the inferior vena cava, especially when all pulmonary venous return to the left atrium is not clearly seen.

Tetralogía de Fallot y ecocardiografía / Case report: echocardiography in Fallot tetralogy

Tetralogy of Fallot is the most frequent cyanotic congenital heart disease. This is a case report of a pediatric patient who underwent her surgery with the monitorization of tranesopageal echocardiography

La tetralogía de Fallot es la cardiopatía congénita cianosante más frecuente. Se presenta un caso clínico de un paciente pediátrico que se monitorizó con ecocardiografía transesofágica intraoperatoria y se muestran las imágenes y los cambios en el manejo debido a la monitorización ecocardiográfica.

Potts Anastomosis: Transthoracic Echocardiographic Features - Tetralogy of Fallot, Doubly Committed Sub-arterial Ventricular Septal Defect, Anomalous Left Anterior Descending Coronary Artery Crossing Right Ventricular Outflow Tract / Anastomosis de Potts: características ecocardiográficas transtorácicas - tetralogía de Fallot, defecto septal con compromiso doble subarterial ventricular, y arteria coronaria descendente anterior izquierda anómala a través del tracto de salida ventricular derecho

ABSTRACT Potts anastomosis is a central systemic-pulmonary surgical shunt between the descending aorta and the left pulmonary artery, developed and subsequently disbanded in the 1950s to provide pulmonary blood flow in patients with tetralogy of Fallot. Blalock-Taussig shunt is a peripheral systemic pulmonary communication which was varied to make the modified Blalock-Taussig shunt, which is now the standard of surgical care for temporary or permanent blood flow to the right or left pulmonary artery from the subclavian artery. The central shunts were disbanded in the 1950s as early development of pulmonary hypertension and its sequelae were the major prohibitive complications. This is a case report of a patient with tetralogy of Fallot with a rare combination of doubly committed sub-arterial ventricular septal defect, anomalous left anterior descending coronary artery crossing the right ventricular outflow tract and patent ductus arteriosus, who developed pulmonary hypertension within four years of Potts anastomosis and then required cardiac and lung transplantation. The transthoracic echocardiographic images are the focussed feature in this paper, confirming clearly defined structural anatomy in complex structural congenital heart disease.

RESUMEN La anastomosis de Potts - desarrollada y disuelta posteriormente en los años 50 - es una derivación quirúrgica sistémico-pulmonar central entre la aorta descendente y la arteria pulmonar izquierda, cuyo fin es proporcionar flujo de sangre pulmonar en pacientes con la tetralogía de Fallot. La derivación de Blalock-Taussig es una comunicación pulmonar sistémica periférica. Este procedimiento sufrió cambios que condujeron a la derivación modificada de Blalock-Taussig, que es ahora el procedimiento quirúrgico estándar para tratar el flujo de sangre temporal o permanente hacia la arteria pulmonar derecha o izquierda desde la arteria subclavia. Las derivaciones centrales fueron disueltas en los años 50, cuando el desarrollo temprano de la hipertensión pulmonar y sus secuelas eran las complicaciones prohibitivas principales. Éste es un reporte de caso de un paciente con la tetralogía de Fallot con una rara combinación de defecto septal con compromiso doble subarterial ventricular, arteria coronaria descendente anterior izquierda anómala a través del tracto de salida ventricular derecho, y conducto arterioso persistente. El paciente desarrollo hipertensión pulmonar a los cuatro años de una anastomosis de Potts, y requirió entonces trasplante cardíaco y pulmonar. Las imágenes ecocardiografias transtorácicas constituyen el aspecto central de este trabajo, que confirma la anatomía estructural claramente definida de la enfermedad cardíaca congénita, estructuralmente compleja.

Successful treatment of ventricular arrhythmia in tetralogy of fallot repair using catheter ablation

Abstract Tetralogy of Fallot (ToF) is one of the most prevalent congenital heart disease. Its surgical corrections may haemodinamically correct a disease, but the incisions may create scars that will originate ventricular arrhythmias. Even though life threatening arrhythmias are not common, some patients present unstable ventricular tachycardia (VT) of ectopic ventricular beats triggering heart failure and symptoms. We describe the treatment of a 16-years-old woman with late ToF repair and drug refractory Implantable cardioverter defibrillator (ICD) shocks. The patient underwent successful ablation of VT using X-ray and anatomic landmarks without the use of electroanatomical mapping. We were able to reduce drugs after one month of ablation and improve quality of life and symptoms. In this paper we describe the indications and perform a brief review of the key points for successful radiofrequency catheter ablation of VT in ToF patients.

Resonancia magnética cardíaca en el seguimiento alejado de pacientes con tetralogía de Fallot / Cardiac Magnetic Resonance in long term follow-up of Tetralogy of Fallot

INTRODUCCIÓN: La tetralogía de Fallot (TF) es la cardiopatía congénita cianótica más frecuente. La insuficiencia pulmonar (IP) y dilatación del ventrículo derecho (VD) son las complicaciones más frecuentes a largo plazo. La resonancia magnética cardiaca (RMC) es el "gold standard" para la evaluación del VD. OBJETIVO: Analizar la información obtenida de las RMC en el seguimiento de pacientes con TF. PACIENTES Y MÉTODO: Se incluyeron RMC realizadas entre 2007 y 2012 a pacientes con TF, reparados con parche transanular (PTA) o ampliación infundibular (AInf) y sin recambio valvular pulmonar (RVP). La fracción de regurgitación pulmonar (FRP), el volumen y función ventricular fueron evaluados. RESULTADOS: Se realizaron 122 RMC a 114 pacientes. Edad promedio al examen 15,4 ± 7,4 años. 53,3% presentó IP severa (> 40%). La media del volumen de fin de diástole del VD (VFDVD) fue 157,3 ± 38,6 ml/m2, fin de sístole (VFSVD) de 85,3 ± 27 ml/m2 y fracción de eyección (FEVD) 46,4 ± 7,1%. 48,4% presentaba un VFDVD mayor de 150 ml/m2 y el 32,8% mayor a 170 ml/ m2. El PTA se relacionó con mayores volúmenes de VD que la AInf. VFDVD mayor a 170 ml/m2 mostraron peor FEVD (FEVD 47,9 ± 7% vs 43,2 ± 6,4%, p < 0,01). DISCUSIÓN: Casi la mitad mostró una significativa dilatación del VD demostrando que la indicación de RMC es tardía en el seguimiento. El PTA se asoció con mayores VFDVD y VFSVD pero no a peor FEVD.

INTRODUCCIÓN: Tetralogy of Fallot (TOF) is the most frequent cyanotic congenital heart disease. Pulmonary regurgitation (PR) and right ventricle (RV) enlargement and dysfunction are the most common long-term complications. Cardiac magnetic resonance (CMR) is the gold standard for RV evaluation. OBJECTIVE: To analyze CMR results in the follow-up of TOF patients. PATIENTS AND METHOD: All CMR performed between 2007 and 2012 in TOF patients with transannular patch (TAP) repair or infundibular widening, and without pulmonary valve replacement (PVR) were included. Pulmonary regurgitant fraction (PRF), ventricular end-diastolic (EDV) and end-systolic volume (ESV), and ejection fraction (EF) were examined. RESULTS: 122 CMR were performed in 114 patients. Average age at CMR was 15.4±7.4 years. 53.3% of them presented severe PR (> 40%). RVEDV was 157.3 ± 38.6 ml/m2, RVESV was 85.3 ± 27 ml/m2 and RVEF was 46.4 ± 7.1%. RVEDV was > 150 ml/ m2 in 48.4% and > 170 ml/m2 in 32.8% of patients. Patients with TAP showed larger RV volumes compared with those with infundibular widening. RVEDV > 170 ml/m2 showed worse RVEF that those with lower RVEDV (47.9 ± 7% vs 43.2 ± 6.4%, p < 0.01). CONCLUSION: Almost half of the pa tients showed significant RV enlargement, demonstrating that the indication of CMR is late in their follow-up. TAP was associated with higher RVEDV and RVESV, but no worse RVEF.

Left Atrial Deformation Analysis in Patients with Corrected Tetralogy of Fallot by 3D Speckle-Tracking Echocardiography (from the MAGYAR-Path Study) / Análise por Speckle-Tracking com Ecocardiografia 3D da Deformação de Átrio Esquerdo em Pacientes com Tetralogia de Fallot Corrigida (do MAGYAR-Path Study)

Abstract Background: Three-dimensional (3D) echocardiography coupled with speckle-tracking echocardiographic (STE) capability is a novel methodology which has been demontrated to be useful for the assessment of left atrial (LA) volumes and functional properties. There is increased scientific interest on myocardial deformation analysis in adult patients with corrected tetralogy of Fallot (cTOF). Objectives: To compare LA volumes, volume-based functional properties and strain parameters between cTOF patients and age- and gender-matched healthy controls. Methods: The study population consisted of 19 consecutive adult patients with cTOF in sinus rhythm nursing at the University of Szeged, Hungary (mean age: 37.9 ± 11.3 years, 8 men, who had repair at the age of 4.1 ± 2.5 years). They all had undergone standard transthoracic two-dimensional Doppler echocardiographic study extended with 3DSTE. Their results were compared to 23 age- and gender-matched healthy controls (mean age: 39.2 ± 10.6 years, 14 men). Results: Increased LA volumes and reduced LA emptying fractions respecting cardiac cycle could be demonstrated in cTOF patients compared to controls. LA stroke volumes featuring all LA functions showed no differences between the 2 groups examined. LA global and mean segmental uni- and multidirectional peak strains featuring LA reservoir function were found to be diminished in adult patients with cTOF as compared to controls. Similarly to peak strains reduced global and mean segmental LA strains at atrial contraction characterizing atrial booster pump function could be demonstrated in cTOF patients as compared to controls. Conclusions: Significant deterioration of all LA functions could be demonstrated in adult patients with cTOF late after repair.

Resumo Fundamento: Ecocardiografia tridimensional (3D) acoplada à técnica de speckle-tracking (3DSTE) é uma nova metodologia útil para a avaliação de volumes e propriedades funcionais do átrio esquerdo (AE). Há crescente interesse científico na análise da deformação miocárdica em adultos com tetralogia de Fallot corrigida (cTOF). Objetivos: Comparar os volumes de AE, propriedades funcionais baseadas no volume e parâmetros de strain entre pacientes com cTOF e controles saudáveis pareados por idade e sexo. Métodos: A população do estudo consistiu em 19 adultos com cTOF consecutivos, em ritmo sinusal, acompanhados na Universidade Szeged, Hungria (idade média: 37,9 ± 11,3 anos; 8 homens; com correção cirúrgica aos 4,1 ± 2,5 anos de idade). Todos foram submetidos a ecocardiografia transtorácica bidimensional com Doppler padrão e 3DSTE. Os resultados foram comparados aos de 23 controles saudáveis pareados por idade e sexo (idade média: 39,2 ± 10,6 anos; 14 homens). Resultados: Aumento dos volumes de AE e redução das frações de esvaziamento de AE em relação ao ciclo cardíaco foram demonstrados em pacientes com cTOF em comparação aos dos controles. Os volumes de ejeção de AE caracterizando todas as funções do AE não diferiram entre os dois grupos. Strains de AE global e segmentar médio uni- e multidimensional, caracterizando função de reservatório de AE, estavam diminuídos em adultos com cTOF em comparação aos de controles. À semelhança dos strains de pico, reduzidos strains de AE global e segmentar médio na contração atrial, caracterizando função de bomba atrial, foram demonstrados em pacientes com cTOF em comparação aos de controles. Conclusões: Demonstrou-se significativa deterioração das funções de AE em adultos com cTOF em fase tardia após correção.

Tetralogy of Fallot in a Case of Dextrocardia with Situs Solitus.

Dextrocardia associated with situs solitus (so called ‘dextroversion’) is a cardiac positional anomaly in which the heart is located in the right hemithorax with its base-toapex axis directed to the right and caudad along with normal anatomical position of other intrathoracic and abdominal viscera. The malposition is intrinsic to the heart and not caused by extracardiac abnormalities. Dextroversion is the second most common type of dextrocardia. In dextroversion, there is a 90% incidence of additional cardiac malformations, including anomalous pulmonary venous return, Tetralogy of Fallot, septal defects, pulmonic stenosis, coarctation of the aorta, and corrected TGA.Here we report a rare case of Tetralogy of Fallot in a 17 year old male with dextroversion.

Pentalogía de Cantrell: un caso en embarazo gemelar / Cantrell`s Pentalogy: A case in twin pregnancy

La pentalogía de Cantrell es una enfermedad muy poco frecuente sobre todo en embarazos múltiples. Está caracterizado por presentar malformaciones integradas por defectos de cinco anomalías: tercio inferior del esternón, defecto epigástrico de la línea media abdominal, alteración del segmento anterior del diafragma, defectos pericárdicos y malformaciones cardiacas. Se reporta un caso en una paciente primigesta de 22 años de edad y embarazo gemelar que acude para su control ecográfico a las 27,3 semanas de gestación, en donde se encuentran los siguientes hallazgos ecográficos: anencefalia, anormalidad facial y corazón fuera de la cavidad torácica. Se da el nacimiento de los gemelos por vía cesárea a las 35 semanas: el primero de aspecto normal y el segundo con Pentalogía de Cantrell y otras malformaciones asociadas.

Pentalogy of Cantrell is a low frequency disease within multiple pregnancy, characterized with malformations with five anomalies defects: lower sternal defect, midline supraumbilical abdominal wall defect, diaphragmatic pericardial defect, anterior diaphragmatic defect and various intracardiac malformations. We report a case in a patient in her first pregnancy of 22 year old, twin pregnancy. She is 27, 3 weeks pregnant, comes for ultrasound control of the gestation, where the following ultrasound results are found: anencephaly, facial abnormalities and the heart out of the chest cavity. The birth of the twins is given via caesarian section at 35 weeks; the first of normal appearance and the second with Pentalogy of Cantrell and other associated malformations

Comparative study to evaluate the efficacy of transthoracic echocardiography versus cardiac catheterization for pre operative assessment of tetralogy of fallot

To determine the results of transthoracic echocardiography and cardiac catheterization for pre operative assessment of Tetralogy of Fallot and operative outcomes for the patients. Case Series; prospective descriptive study. Armed Forces Institute of Cardiology/National Institute of Heart Diseases, Rawalpindi, Jan 2006 to December 2010. All patients admitted in AFIC/NIHD for surgical repair of Tetralogy of Fallot. A total of 746 patients were included in study, male [33%], female [67%], mean age was 4.7 years. Group I was evaluated for total correction with combined transthoracic two dimensional echocardiography and cardiac catheterization, and group II was subjected to transthoracic two dimensional echocardiography only for pre operative assessment. Overall mortality was 8.4% in group I and 10.8% in group II. In group II, 84% had total correction and 16% had closed heart, while in group II 73% had open heart and 27% had closed heart surgery. Tetralogy of Fallot is the leading cause of cyanotic congenital cardiac heart disease. The pre operative evaluation with help of Transthoracic two dimensional echocardiography requires oral sedation, is cost effective and minimally invasive. Cardiac catheterization being reserved for the patients having inadequate information on Transthoracic echocardiography. Early surgery during childhood has long term benefits in terms of morbidity and lifestyle adjustment

Intra-operative trans-esophageal echocardiography in congenital heart disease.

Intra-operative trans-esophageal echocardiography (TEE) is an important monitoring and diagnostic tool used during surgery for repair of congenital heart disease. In several studies,TEE has been shown to provide additional intra-cardiac anatomic information. Its ability to be used intra- operatively before and after cardiac repair makes it a unique tool. Before TEE was available for intra-operative use, significant residual abnormalities were frequently not detected. The result was often substantial post-operative morbidity and mortality and sometimes the need for re-operation. According to practice guidelines established by the Society of Cardiovascular Anesthesiologists and the American Society of Anesthesiologists, there is strong evidence for the usefulness of TEE in surgery for congenital heart disease because it significantly improves the clinical outcome of these patients. Before surgical correction, TEE helps confirm diagnosis and spot any additional lesion, while after the surgical correction, it provides baseline parameters for comparison after the surgical correction.

Diagnosis and management of cyanotic congenital heart disease: part II.

In this review, the clinical features and management of less commonly encountered cyanotic cardiac lesions are reviewed. Pathophysiology, clinical features, laboratory studies and management are discussed. The clinical and non-invasive laboratory features of these cardiac defects are sufficiently characteristic for the diagnosis and invasive cardiac catheterization and angiographic studies are not routinely required. Such studies may be needed either to define features that could not be clearly defined by non-invasive studies or prior to performing trans-catheter interventions. Surgical correction or effective palliation is possible at relatively low risk. But, residual defects, some requiring repeat catheter or surgical intervention, may be seen in a significant percentage of patients and consequently, continued follow-up after surgery is recommended.