Caso Clínico: Tetralogía de Fallot Extrema / Case Report: Extreme Tetralogy of Fallot

INTRODUCCIÓN: La tetralogía de Fallot representa la cardiopatía congénita cianógena más frecuente después del primer año de vida, ocurre aproximadamente en 1 de cada 3 500 nacimientos y representa el 7 al 10% de todas las Enfermedades Cardiacas Congénitas. Su variante extrema que se acompaña de atresia pulmonar representa el 1 a 2% de las cardiopatías congénitas. Presentamos una Tetralogía de Fallot Extrema Ductus Dependiente de manejo complejo. CASO CLÍNICO: Se trata de un neonato a término de 12 días de vida, adecuado para la edad gestacional, sin factores de riesgo prenatales, ni antecedentes patológicos familiares, el cual es diagnosticado de Tetralogía de Fallot Extrema por ecocardiograma. EVOLUCIÓN: Paciente inicialmente tratado con infusión de prostaglandina E1. Posteriormente mediante fístula Blalock-Taussig izquierda. CONCLUSIONES: Para mejorar el pronóstico y sobrevida de un paciente con Tetralogía de Fallot Extrema el diagnóstico prenatal oportuno y el tratamiento apropiado es de vital importancia, sin embargo el tratamiento definitivo con derivaciones pulmonares sistémicas representan complicaciones con riesgo de mortalidad. El presente caso tuvo un desenlace desfavorable.

BACKGROUND: The Tetralogy of Fallot represents the most common cyanogenic heart disease after the first year of life. It occurs in 1 of 3 500 live births and accounts as 7 to 10% of all Congenital Heart Diseases. Its extreme variant is accompanied by pulmonary atresia and accounts 1 to 2% of the Congenital Heart Diseases. We present an Extreme Tetralogy of Fallot­ Ductus Dependent of complex management. CLINICAL CASE: A full term 12 day neonate, adequate for gestational age, without prenatal risk factors or pathologic family antecedents, is diagnosed with Extreme Tetralogy of Fallot by echocardiogram. EVOLUTION: The patient was treated initially with an infusion of prostaglandin E1. Then, a left Blalock-Taussig shunt was performed. CONCLUSIONS: To improve the prognosis and survival rates of patients with Extreme Tetralogy of Fallot , a timely diagnosis and treatment are of vital importance. Definitive treatment with systemic pulmonary shunts carries high risk complications. The present case had an unfavorable evolution.

Caso Clínico: Coartación de Aorta Torácica / Case Report: Thoracic Aortic Coarctation

INTRODUCCIÓN: Las enfermedades cardíacas congénitas son relativamente frecuentes, afectando a 8 de cada 1000 recién nacidos en Ecuador. El 30% de niños que tienen anormalidades genéticas son portadores de cardiopatías congénitas que aparecen en las primeras semanas del embarazo, destacándose como causas principales los factores genéticos, infecciosos y químicos. La importancia de presentar el tema radica en el hecho de ser el primer lactante intervenido por cardiopatía congénita en el hospital “José CarrascoArteaga”, con la participación multidisciplinaria de especialistas en el tema. CASO CLÍNICO: El presente caso trata sobre un paciente lactante de 7 meses de edad, con antecedentes de distrés respiratorio luego del nacimiento; a la exploración física lossignos que más se destacaron fueron la presencia de un soplo holosistólico en barra en todos los focos de auscultación, la disminución de pulsos femorales y tensión arterial baja en miembros inferiores. EVOLUCIÓN: Se realizaron estudios complementarios concluyéndose el diagnóstico decoartación de aorta torácica descendente, se inició tratamiento a base de betabloqueantes durante cuatro meses y se decidió tratamiento quirúrgico mediante coartectomía deaorta con anastomosis término-terminal, el paciente evolucionó favorablemente despuésdel procedimiento. CONCLUSIÓN: El presente caso demuestra la necesidad de una sospecha clínica paradeterminar el diagnóstico de enfermedades congénitas graves, resalta el procedimiento diagnóstico con el apoyo de los métodos complementarios y respalda la evidencia disponible en cuanto al tratamiento de la patología con el procedimiento quirúrgico adecuado, el mismo que fue seleccionado de acuerdo a la condición clínica del paciente, con buenosresultado(AU)

BACKGROUND: Congenital cardiac diseases are relatively frequent, affecting 8 of 1000 newborns in Ecuador. 30% of children with congenital abnormalities have also any congenital cardiopathy which appear at early pregnancy stages; genetic, infectious and chemical factorsoutstand as main causes of this. Importance of this topic lies in the fact of the first surgical treatment performed in a patient diagnosed with a congenital cardiopathy by a multidisciplinaryparticipation of specialists at “José Carrasco Arteaga” hospital.CASE REPORT: This case is about a 7-months old nursing infant who had precedents of respiratory distress after birth; bar holosystolic murmur at every auscultation foci, femoral pulses decrease and low arterial tension in lower limbs were outstanding findings in physical examination. EVOLUTION: Complementary studies were performed, a descendant thoracic aortic coarctation diagnosis was concluded, a betablocker based therapy was used during 4 months and surgical treatment by aortic coarctectomy with end-to-end anastomosis was decided, the patient had a favourably evolution after procedure. CONCLUSION: This case demonstrates the need of clinic suspicion to determine the diagnosisof severe congenital diseases, it highlights the diagnostic process with complementary studies and supports available evidence about the treatment of this pathology with the appropriatetreatment, which was selected according to patient’s condition and had good results(AU)

Cierre percutáneo de la orejuela izquierda: Descripción del primer caso intervenido en el país / LEFT ATRIAL APPENDAGE’S PERCUTANEOUS CLOSURE: DESCRIPTION OF THE FIRST CASE IN THE COUNTRY INTERVENED

La fibrilación auricular no valvular (FANV) es responsable del 20% de los eventos cerebrovasculares (ECV), asociada a una morbilidad y mortalidad elevadas. La anticoagulación oral (ACO) representa la terapia de primera línea en la prevención de la embolia sistémica; sin embargo, hasta la mitad de los pacientes con clara indicación para su utilización no la reciben, ya sea por contraindicación, riesgo de sangrado, o múltiples limitaciones inherentes a la medicación. En los últimos años, el cierre percutáneo de la orejuela izquierda (OI) ha emergido como una alternativa eficaz y segura para los pacientes con alto riesgo de cardioembolia y contraindicación a la terapia de ACO.A continuación se describe el primer caso a nivel nacional de oclusión percutánea de la OI mediante el implante transvenoso del dispositivo WatchmanTM (Boston Scientific, USA), que hizo parte de una serie de pacientes intervenidos en la Unidad de Hemodinámica del Hospital José Carrasco Arteaga en la ciudad de Cuenca.(AU)

Non-valvular atrial fibrillation is reponsible for 20% of strokes associated with high morbility and mortality. Oral anticoagulation is considered the cornerstone therapy to prevent a systemic embolism. Nevertheless, half of the eligible patients for oral anticoagulation therapy are not prescribed these due to contraindications, risk of bleeding, or because of multiple factors related to medication pitfalls. New anticoagulants have been evaluated in several clinical trials that show similar efficacy compared with warfarin, however, risk of major hemorrage over lifetime remains equivalent. In recent years, left atrial appendage closure has emerged as an effective and safe alternative to prevent systemic embolism in patients with contraindication to oral anticoagulation. We present the first case of percutaneous left atrial appendage closure in our country using the Watchman device in a group of patients examined at the José Carrasco Arteaga Hospital. (AU)

Oclusão Percutânea do Apêndice Atrial Esquerdo com Prótese Watchman® / Left Atrial Appendage Closure With the WatchmanTM Device

Introdução: O acidente vascular cerebral secundário à fibrilação atrial tem sido associado a taxas de mortalidade e de incapacidade permanente elevadas, porquanto sua prevenção eficaz é importante. O tratamento com varfarina diminui em 60% o risco de acidente vascular cerebral; todavia, até metade dos pacientes com fibrilação atrial não faz uso da anticoagulação. A oclusão do apêndice atrial esquerdo surgiu como estratégia alternativa para prevenção do acidente vascular cerebral. Métodos: Foram selecionados pacientes com fibrilação atrial, escore de CHA2DS2-VASc ≥ 2, não elegíveis para anticoagulação, para se submeterem ao fechamento percutâneo do apêndice atrial esquerdo com a prótese Watchman®. O procedimento foi realizado sob anestesia geral e guiado por ecocardiografia transesofágica. Resultados: Dos 11 pacientes selecionados, 2 não foram tratados por apresentarem trombo pré ou durante o procedimento, antes do implante do dispositivo. A idade foi de 74 ± 5,1 anos, 66,6% eram do sexo masculino, com escores CHA2DS2-VASc de 4 ± 1,4 e HAS-BLED de 3,4 ± 1,1, 77% tinham contraindicação ou condições sociais desfavoráveis para utilizarem a anticoagulação. O sucesso técnico foi de 100%, sendo alcançada a oclusão completa em todos os casos, com tempo médio de fluoroscopia de 22,1 ± 10,8 minutos e ausência de complicações hospitalares. No seguimento de 78,3 ± 41,5 dias, não ocorreram desfechos clínicos, mas um paciente apresentou trombo no dispositivo e recebeu anticoagulação por 3 meses. Conclusões: A oclusão percutânea do apêndice atrial esquerdo com dispositivo Watchman® é factível e pode ser uma alternativa atrativa na prevenção de acidente vascular cerebral nos pacientes com fibrilação atrial e limitação para anticoagulação.

Background: Stroke secondary to atrial fibrillation has been associated to a high risk of permanent, severe disability, and high early mortality, and therefore its effective prevention is of paramount importance. Warfarin therapy reduces the risk of stroke by 60%, however, half of the patients with atrial fibrillation do not receive anticoagulation. Left atrial appendage closure has emerged as an alternative strategy for stroke prevention. Methods: Patients with atrial fibrillation and CHADSVASc score ≥ 2, not eligible for anticoagulation, were submitted to left atrial appendage closure using the WatchmanTM device. The procedure was performed under general anesthesia and was guided by transesophageal echocardiography. Results: Of the 11 selected patients, 2 were not treated due to thrombi presented prior or during the procedure and before device implantation. Mean age was 74 ± 5.1 years, 66% were male, CHA2DS2-VASc score was 4 ± 1.4, HASBLED score was 3.4 ± 1.1, 77% had contraindications or had unfavorable social conditions for anticoagulation. Technical success was 100% and complete occlusion was obtained in all of the cases, with a mean fluoroscopic time of 22.1 ± 10.8 minutes, and no hospital complications. At a follow-up of 78.3 ± 41.5 days, there were no clinical events but one patient had thrombus formation on the device and received anticoagulation for 3 months. Conclusions: Left atrial appendage closure with the WatchmanTM device is feasible and may be a good alternative therapy for stroke prevention in patients with atrial fibrillation and restrictions for anticoagulation.

Intervenção percutânea em lesão de tronco de coronária esquerda não protegido / Percutaneous coronary intervention for unprotected left main coronary artery lesions

INTRODUÇÃO: Refinamentos da intervenção coronária percutânea (ICP), entre eles a utilização do ultrassom intravascular (IVUS) e da reserva de fluxo fraccionada (FFR), têm permitido o tratamento de lesões complexas com bons resultados. Como consequência, a abordagem percutânea das lesões de TCE se difundiu, incluindo hospitais de menor volume de procedimentos. Nosso objetivo foi apresentar os resultados iniciais e tardios da ICP de lesões de TCE não protegido. MÉTODOS: Foram incluídos pacientes consecutivos tratados em dois centros hospitalares, de agosto do 2009 a julho do 2013. A indicação da estratégia percutânea baseou-se em avaliação clínica, cálculo do escore Syntax, EuroScore e no desejo do paciente. RESULTADOS: Foram tratados 28 pacientes, com idade de 69,2 ± 10,1 anos, 39% eram diabéticos e 39% eram portadores síndrome coronária aguda. Metade dos pacientes tinha EuroScore ≥ 6; o escore Syntax foi de 26,0 ± 8,4 e 82% tinham lesões localizadas na bifurcação. As intervenções foram guiadas por IVUS e/ou FFR em 71,4% dos pacientes, 93% foram tratados com stents farmacológicos, predominantemente pela técnica de 1 stent, e o sucesso angiográfico foi alcançado em 100% dos casos. O acompanhamento foi de 19,2 ± 13,7 meses. A taxa de desfechos clínicos maiores foi de 21,4% no seguimento maior a 4 anos, óbito cardíaco de 14,2%, infarto do miocárdio não fatal de 3,5%, e revascularização da lesão-alvo em 3,5%. CONCLUSÕES: A ICP de TCE não protegido guiada, sempre que possível, por IVUS e/ou FFR é segura e eficaz no curto e longo prazos, na experiência em um hospital com moderado volume de procedimentos.

BACKGROUND: Refinements in percutaneous coronary intervention (PCI), including the use of intravascular ultrasound (IVUS) and fractional flow reserve (FFR), have allowed the treatment of complex lesions with good results. As a result, the percutaneous approach for left main coronary artery (LM) lesions has spread, including centers with a lower volume of PCI procedures. Our objective was to report the early and late outcomes of PCI in unprotected LM lesions. METHODS: Consecutive patients treated at two different hospitals from August 2009 to July 2013 were included. The indication for the percutaneous approach was based on the clinical assessment and the calculation of Syntax score, EuroScore and on the patient's wishes. RESULTS: Twenty-eight patients with mean age of 69.2 ± 10.1 years were treated, 39% were diabetic and 39% had acute coronary syndromes. Half of the patients had EuroScore ≥ 6; the Syntax score was 26.0 ± 8.4 and 82% had LM bifurcation lesions. Interventions were guided by IVUS and/or FFR in 71.4% of the patients, 93% were treated with drug-eluting stents, and the 1-stent technique was used in most occasions. Angiographic success was achieved in 100% of the cases. At the 19.2 ± 13.7 month follow-up, the rate of major adverse cardiac events was 21.4% in the follow-up longer than 4 years, cardiac death 14.2%, non-fatal myocardial infarction 3.5% and target-lesion revascularization 3.5%. CONCLUSIONS: PCI in unprotected LM lesions, guided by IVUS and/or FFR whenever possible, is safe and effective in the short and long-term, in the experience of a hospital with a moderate number of PCI procedures.

Avaliação ultrassonográfica intracoronária de imagem angiográfica duvidosa durante intervenção percutânea em bifurcação / Intravascular ultrasound evaluation of equivocal angiographic imaging during percutaneous coronary intervention of bifurcation lesions

O uso do ultrassom intracoronário (USIC) para guiar o implante do stent em bifurcações, especialmente na detecção de complicações relacionadas ao procedimento, tem diminuído as taxas de desfechos clínicos maiores. Neste artigo, reportamos o caso de um paciente submetido a intervenção em bifurcação, em que o fio-guia ultrapassou inadvertidamente por trás das hastes proximais do stent, levando à deformação de sua borda. A complicação foi suspeitada na angiografia e confirmada com o USIC. O USIC foi fundamental no diagnóstico, na confirmação do reposicionamento correto do fio-guia, e na avaliação da ótima expansão e da aposição completa das hastes do stent, prevenindo possível desfecho trombótico precoce e garantindo o resultado a longo prazo.

The use of intravascular ultrasound (IVUS) to guide stent implantation in bifurcation lesions, especially for the detection of procedure-related complications, has decreased the rates of major clinical outcomes. We report a case of a patient undergoing bifurcation intervention, where the guidewire inadvertently was passed behind the proximal stent struts, deforming the stent edge. This complication was suspected at the angiography and was confirmed by IVUS. IVUS was crucial for the diagnosis, to confirm the correct repositioning of the guidewire, and to assess the optimal expansion and complete apposition of stent struts, thus preventing a possible early thrombotic event and ensuring a good long-term outcome.