Sinus node dysfunction (SND) with junctional rhythm (JR) is common after the Fontan operation. Atrial pacing (AP) restores atrioventricular (AV) synchrony, but the placement of a pacemaker carries significant morbidity. To study the impact of AP on echocardiographic parameters of function in Fontan patients with SND and JR. Nine Fontan patients with AP for SND and JR were prospectively studied with echocardiography in the following conditions-baseline paced rhythm, underlying JR and, if possible, slow-paced rhythm below their baseline paced rate (~ 10 bpm faster than their JR rate). Cardiac index was significantly lower in JR (3 ± 1.1 L/min/m) vs AP (4.2 ± 1.4 L/min/m; p = 0.002). Diastolic function also significantly worsened with increased ratio of early diastolic systemic AV valve inflow velocity to early diastolic systemic AV valve annulus velocity (E/e' ratio) by tissue Doppler imaging (TDI) in JR (11.6 ± 4.6) vs AP (8.8 ± 2.2, p = 0.016). Pulmonary venous flow reversal was present in 7/9 patients in JR vs 0/9 in AP (p = 0.016). There were no significant differences in these echocardiographic measurements between the paced and slow-paced conditions. When compared to AP, JR was associated with a significant reduction in cardiac output and diastolic function, and an increased prevalence of pulmonary vein flow reversal. There were no differences between paced and slow-paced conditions, suggesting that AV synchrony rather than heart rate was primarily contributing to cardiac output. Further studies are needed to understand the chronic impact of JR on Fontan outcomes.
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http://dx.doi.org/10.1007/s00246-023-03345-0 | DOI Listing |
JACC Adv
January 2025
Department of Cardiology, Boston Children's Hospital, Boston, Massachusetts, USA.
Background: The Fontan operation is associated with chronic venous hypertension, liver and renal disease, and several other sequelae. The alterative surgical approach, when feasible, a biventricular conversion (BiV), may diminish some of these long-term risks.
Objectives: The aim of this study was to compare long-term outcomes of patients undergoing BiV with those undergoing a destination Fontan operation.
J Thorac Cardiovasc Surg
January 2025
Department of Pediatrics, National Taiwan University Children's Hospital, Taipei, Taiwan. Electronic address:
Objective: The modified Fontan operation, a palliative approach for single ventricular circulation patients, often incorporates a fenestration to facilitate postoperative management. Postoperative fenestration closure is sometimes performed to mitigate potential risks such as low oxygen saturation. However, the benefits and potential risks of this procedure remain under investigation.
View Article and Find Full Text PDFJ Clin Med
December 2024
Department of Pediatric Cardiology, Saarland University Medical Center, D-66421 Homburg, Germany.
Systemic-to-pulmonary collaterals (SPCs) are common in congenital heart disease (CHD). Particularly in single ventricle anatomy and Fontan circulation, SPC can both complicate the postoperative course and lead to clinical deterioration in the long term. The treatment of SPC is controversial.
View Article and Find Full Text PDFAnn Thorac Surg Short Rep
December 2024
Department of Cardiovascular Surgery, Nagano Children's Hospital, Azumino, Japan.
Background: The discussion of Fontan fenestration is difficult because many institutions have different strategies over time. In our institute, we performed a non-fenestrated Fontan procedure for single-ventricular physiology as our definitive strategy.
Methods: Between August 1999 and December 2007, 72 consecutive patients with single-ventricle physiology underwent extracardiac total cavopulmonary connection without fenestration as our definitive strategy.
Ann Thorac Surg Short Rep
September 2024
Biostatistics Unit, Department of Data Science, National Center for Child Health and Development, Tokyo, Japan.
Background: The primary treatment for hypoplastic left heart syndrome (HLHS) is the Fontan pathway, which entails performing the Glenn procedure. We hypothesized that the superior vena cava in patients with HLHS was short. As the length of the superior vena cava influences the Glenn procedure, we compared its length between patients with HLHS and those with other congenital heart diseases.
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