Abstract
BACKGROUND: The association of right ventricular dysfunction, atrial tachyarrhythmias, and cardiac conduction system disease is rarely reported in literature. Previous case reports include right ventricular dysfunction associated with sudden cardiac death and ventricular arrhythmia. Other case reports with similar clinical presentation triad differ by either left ventricular involvement or having an abnormal presentation of systemic diseases. CASE PRESENTATION: We report a case series of right ventricular dysfunction, atrial tachyarrhythmias, and cardiac conduction system disease in a single family. The proband in this case series is a 70-year-old Egyptian male who presented to our care for dual-chamber pacemaker generator replacement. He had the pacemaker for 10 years because of complete heart block. Device interrogation showed recorded paroxysmal atrial fibrillation episodes. Transthoracic echocardiography showed normal left ventricle internal dimensions and ejection fraction, dilated right ventricle with preserved systolic function, severe tricuspid regurgitation, and systolic pulmonary artery pressure of 40 mmHg. On reviewing his old records, there was no right ventricular affection 10 years ago; progressive right ventricular dysfunction occurred over the last decade. Despite guideline-directed medical therapy, the patient's clinical status deteriorated, and surgical tricuspid valve replacement was performed with a tissue valve. Since the surgical intervention, his clinical status and functional capacity improved, and repeated echocardiography showed no tricuspid regurgitation with persistence of the right ventricular dilatation but with normal right ventricular function. On reviewing his family history, the triad of right ventricular dysfunction, atrial tachyarrhythmias, and cardiac conduction system disease occurred in affected family members with various presentations. There was no left ventricular involvement, no history of ventricular arrhythmias in any of the affected members, and there was no history of sudden cardiac death across all three generations. CONCLUSION: Familial clustering of right ventricular dysfunction, atrial tachyarrhythmias, and cardiac conduction system disease do exist. This constellation of findings is not well described in literature. Further reporting of similar cases and investigating of genetic bases are required.