Importance of arrhythmia management for sinus rhythm maintenance in the Fontan circulation

DOI Open Access
  • Umei Masahiko
    Department of Cardiovascular Medicine, The University of Tokyo Hospital
  • Soma Katsura
    Department of Cardiovascular Medicine, The University of Tokyo Hospital
  • Saito Akihito
    Department of Cardiovascular Medicine, The University of Tokyo Hospital
  • Kojima Toshiya
    Department of Cardiovascular Medicine, The University of Tokyo Hospital
  • Tokiwa Hiroyuki
    Department of Cardiovascular Medicine, The University of Tokyo Hospital
  • Goto Kohsaku
    Department of Cardiovascular Medicine, The University of Tokyo Hospital
  • Komuro Issei
    Department of Cardiovascular Medicine, The University of Tokyo Hospital
  • Yao Atsushi
    Division for Health Service Promotion, The University of Tokyo

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Other Title
  • フォンタン循環における洞調律維持を基本とする不整脈管理の重要性

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Abstract

After the Fontan procedure, there is a high risk of developing supraventricular tachyarrhythmias such as atrial flutter and atrial fibrillation in the chronic phase, leading to worsening heart failure and/or sudden death. Therefore, specific care for Fontan circulation is required.The patient was a 29-year-old man having undergone extracardiac total cavo-pulmonary connection for a single right ventricle. Although he had persistent atrial flutter, his heart rate was well controlled around 70 beats per minute under the treatment with sotalol. However, his hypoxia and leg edema was worsened, suggesting congestive heart failure. The ventricular diastolic dysfunction under the atrial flutter was thought to be the primary reason, causing the elevation of atrial and pulmonary venous pressures (as indicated by mean pulmonary arterial wedge pressure of 16 mmHg). In addition, the pulmonary vascular resistance was found to be increased to be 3.3 WU, causing a marked elevation in the systemic venous pressure and a so-called out-of-proportion pulmonary hypertension as indicated by mean pulmonary arterial pressure of 26 mmHg. In this case, it was confirmed that atrial and venous pressures and pulmonary vascular resistance so much improved after conversion of atrial flutter to sinus rhythm. We believe that this is an educational case to show that sinus rhythm maintenance rather than the heart rate control under the atrial flutter is vital to keep stable the Fontan circulation.

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