Left ventricular (LV) diastolic dysfunction is associated with poor outcomes after tetralogy of Fallot (TOF) repair, although its cause is not known, and its relation to right ventricular (RV) performance has never been examined. The aim of this study was to test the hypothesis that RV dilation leads to LV diastolic dysfunction after TOF repair. Patients with repaired TOF who underwent cardiac catheterization and cardiac magnetic resonance imaging within 6 months from January 2003 and April 2011 were reviewed to assess the relation of LV end-diastolic pressure (LVEDP) and indexed RV end-diastolic volume (RVEDVi). Thirty-eight patients were included at a median age of 10.1 years (range 0.6 to 54.7). There was a significant linear association between RVEDVi and LVEDP (p = 0.05). RV end-diastolic pressure (p <0.001), right pulmonary artery systolic pressure (p = 0.009), left pulmonary artery systolic pressure (p = 0.02), and total cardiopulmonary support time (p = 0.04) during TOF repair were also significantly associated with LVEDP. Compared to patients with LVEDP <12 mm Hg, those with LVEDP ≥12 mm Hg had significantly higher mean RVEDVi (135.2 ± 47.8 vs 98.6 ± 28 ml/m(2), p = 0.007) and mean RV end-diastolic pressure (11.7 ± 1.6 vs 8.5 ± 2.8 mm Hg, p = 0.0003). In conclusion, after TOF repair, LVEDP is significantly associated with RVEDVi. Furthermore, mean RVEDVi is significantly higher in patients with LVEDP ≥12 mm Hg. These findings support the theory that RV dilation may impair LV diastolic function and that LV parameters may also be important to consider in determining timing of pulmonary valve replacement.
[Show abstract][Hide abstract] ABSTRACT: Tetralogy of Fallot (TOF) is an important lesion for all pediatric and congenital heart surgeons. In designing the most appropriate operation for children with TOF, the postoperative physiology should be taken into account, both in the short and long term. The balance between pulmonary stenosis (PS) and pulmonary insufficiency (PI) may be critical for preservation of ventricular function. A unified repair strategy that limits both residual PS and PI is presented, along with supporting experimental evidence, a strategy for dealing with coronary anomalies, and comments regarding best timing of operation.
Annals of Pediatric Cardiology 07/2008; 1(2):93-100. DOI:10.4103/0974-2069.43873
[Show abstract][Hide abstract] ABSTRACT: Tetralogy of Fallot (TOF) is the most common form of cyanotic congenital heart disease. If left untreated, it carries a 33% mortality in the first year of life and a 50% mortality in the first 3 years of life. Since the introduction of the first open-heart repair by Lillehei and Varco in 1954, surgical management of TOF has evolved to be the primary repair during infancy in the majority of patients. Surgical management of TOF results in anatomic and functional abnormalities in the majority of patients, such as chronic pulmonary valve regurgitation and right ventricular (RV) dysfunction. Long-standing chronic pulmonary valve regurgitation can result in RV dilatation and failure, increasing tricuspid regurgitation, impaired exercise performance and supraventricular or ventricular arrhythmias. A timely reoperation may prevent these consequences, with a complete RV-function recovery. This article provides insight into the questions of when to perform a pulmonary valve implantation and in whom.
Expert Review of Cardiovascular Therapy 07/2012; 10(7):917-23. DOI:10.1586/erc.12.67
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