Cavopulmonary Anastomosis in a Patient With Arrhythmogenic Right Ventricular Cardiomyopathy With Severe Right Ventricular Dysfunction.

Vaidyanathan, Swaminathan; Kothandam, Sivakumar; Kumar, Rajesh; Indrajith, Sujatha Desai; Agarwal, Ravi

Vaidyanathan, Swaminathan; Kothandam, Sivakumar; Kumar, Rajesh; Indrajith, Sujatha Desai; Agarwal, Ravi.

Afiliación

Vaidyanathan S; Department of Cardiac Surgery, Madras Medical Mission, Chennai, India.
Kothandam S; Department of Paediatric Cardiology, Madras Medical Mission, Chennai, India.
Kumar R; Department of Paediatric Cardiology, Madras Medical Mission, Chennai, India.
Indrajith SD; Department of Anesthesiology, Madras Medical Mission, Chennai, India.
Agarwal R; Department of Cardiac Surgery, Madras Medical Mission, Chennai, India.

World J Pediatr Congenit Heart Surg ; 11(4): NP60-NP62, 2020 Jul.

Article en En | MEDLINE | ID: mdl-28825387

RESUMEN

A 26-year-old lady presented with exertional dyspnea, palpitations, central cyanosis, and oxygen saturations of 80% in room air. Her electrocardiogram, echocardiogram, and cardiac magnetic resonance were diagnostic of arrhythmogenic right ventricular dysplasia. There was no documented ventricular arrhythmia or syncopal episodes and Holter recordings were repeatedly normal. Cardiac hemodynamics showed right to left shunt through atrial septal defect, low pulmonary blood flow, normal atrial pressures, and minimally elevated right ventricular end-diastolic pressures. Since her presenting symptoms and cyanosis were attributed to reduced pulmonary blood flow, she underwent off-pump cavopulmonary anastomosis between right superior vena cava and right pulmonary artery. As we intended to avoid the adverse effect of extracorporeal circulation on the myocardial function and pulmonary vasculature, we did not attempt to reduce the size of the atrial septal defect. Her postoperative period was uneventful; oxygen saturation improved to 89% with significant improvement in effort tolerance. At 18-month follow-up, there were no ventricular arrhythmias on surveillance. The clinical presentation of this disease may vary from serious arrhythmias warranting defibrillators and electrical ablations at one end to right ventricular pump failure warranting cardiomyoplasty or right ventricular exclusion procedures at the other end. However, when the presentation was unusual with severe cyanosis through a stretched foramen ovale leading to reduced pulmonary blood flows, Glenn shunt served as a good palliation and should be considered as one of the options in such patients.

Asunto(s)

Displasia Ventricular Derecha Arritmogénica/cirugía; Puente Cardíaco Derecho/métodos; Disfunción Ventricular Derecha/cirugía; Función Ventricular Derecha/fisiología; Adulto; Displasia Ventricular Derecha Arritmogénica/complicaciones; Displasia Ventricular Derecha Arritmogénica/fisiopatología; Femenino; Ventrículos Cardíacos/cirugía; Humanos; Periodo Posoperatorio; Arteria Pulmonar/cirugía; Circulación Pulmonar; Vena Cava Superior/cirugía; Disfunción Ventricular Derecha/etiología; Disfunción Ventricular Derecha/fisiopatología

Palabras clave

arrhythmia; atrial septal defect (ASD); cardiomyopathy; cavopulmonary anastomosis; cyanosis; magnetic resonance imaging (MRI); off pump surgery; right; sudden cardiac death; ventricle

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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Función Ventricular Derecha / Disfunción Ventricular Derecha / Puente Cardíaco Derecho / Displasia Ventricular Derecha Arritmogénica Tipo de estudio: Etiology_studies Límite: Adult / Female / Humans Idioma: En Revista: World J Pediatr Congenit Heart Surg Año: 2020 Tipo del documento: Article País de afiliación: India Pais de publicación: Estados Unidos

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