Effects of Regional Dysfunction and Late Gadolinium Enhancement on Global Right Ventricular Function and Exercise Capacity in Patients With Repaired Tetralogy of Fallot

Wald, Rachel M.; Haber, Idith; Wald, Ron; Valente, Anne Marie; Powell, A.; Geva, Tal

doi:10.1161/circulationaha.108.816546

Cited by 245 publications

(137 citation statements)

References 35 publications

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“…These changes highlight the extensive adverse remodeling that occurs even during the asymptomatic stages of PI+PS, prior to the development of systolic dysfunction and clinical heart failure. While the profibrotic remodeling alone is unlikely to fully explain the progression to systolic heart failure, both animal and human studies (29,(31)(32)(33) show that it is a significant contributing factor. RV volume and pressure overload can also occur in the setting of flow-induced pulmonary hypertension (PAH) due to systemic to pulmonary artery shunts in CHD.…”

Section: Discussionmentioning

confidence: 99%

“…RV fibrosis demonstrated by cardiac MRI using late Gadolinium enhancement is a unique feature following RVOT reconstruction; it is seen in >98% of children and adults with repaired tetralogy of Fallot and is a precursor to late arrhythmias, exercise intolerance and ventricular dysfunction (29,31,32). Although the mechanisms mediating fibrosis have been described in the postloaded LV (33), whether these mechanisms are similar in the RV subjected to combined volume and pressure load has not previously been evaluated.…”

Section: Discussionmentioning

confidence: 99%

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miR-21 is associated with fibrosis and right ventricular failure

Reddy

Zhao

et al. 2017

JCI Insight

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

miR-21 is associated with fibrosis and right ventricular failure

Reddy

Zhao

et al. 2017

JCI Insight

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“…Second, though CMR imaging is well accepted as the gold standard for evaluating RV function and volume, 4,38, 39 we did not use it to assess patients in this study. It was difficult to have healthy controls undergo CMR examination, because of the high cost, the requirement for sedation in young children, and low availability.…”

Section: Study Limitationsmentioning

confidence: 99%

Mechanisms of Left Ventricular Dysfunction Assessed by Layer-Specific Strain Analysis in Patients With Repaired Tetralogy of Fallot

Yamada

Takahashi

Kobayashi

et al. 2017

Circ J

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importance of LV-RV interaction in patients who have undergone TOF repair. 8 Geva et al showed that lower LVEF and older age at repair were the strongest independent factors associated with impaired clinical status in long-term survivors of rTOF. 13 Recently, Diller et al showed that LV longitudinal dysfunction, such as impairment in longitudinal global strain and decreased mitral annular plane systolic excursion, were associated with an increased risk of sudden cardiac death and/or life threatening ventricular arrhythmias in patients with rTOF. 12 Therefore, the assessment of LV function in patients with TOF is crucial because it is the strongest determinant of a poor clinical outcome in these patients. Despite the importance of LV dysfunction, the mechanisms and time course of the development of LV dysfunction remain poorly understood. 14 Its mechanisms and time course in patients with rTOF are important because it has the potential to become T etralogy of Fallot (TOF) is the most prevalent form of cyanotic congenital heart disease. 1 Significant advances in the early management of TOF have dramatically improved the survival of TOF patients, 2 with current 30-year survival rates reaching 89%. 3 However, late ventricular dysfunction related to residual hemodynamic and electrophysiological abnormalities remains a problem, contributing to increasing morbidity and mortality rates beginning in the third decade of life. 2,4-6 Previous investigators assessing ventricular function in patients with repaired TOF (rTOF) have primarily focused on right ventricular (RV) mechanics and their interaction with pulmonary regurgitation (PR). 7-9 Moreover, the importance of left ventricular (LV) dysfunction as a risk factor for long-term adverse outcomes is increasingly acknowledged. (rTOF) is an important risk factor for adverse outcomes. The aim of this study was to assess the details and time course of such LV dysfunction using layer-specific strain analysis by echocardiography.

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“…44 This is clinically important as a risk factor for increased propensity to arrhythmias, exercise intolerance, and RV failure. 44,45 It has been suggested that these differences in response between the RV and LV to volume loading may stem from the different embryological origin of the 2 ventricles. 27 There has been increasing interest in the role of microRNAs (miRNA) as regulators of a wide range of cardiovascular processes and as possible therapeutic targets.…”

Section: March 4 2014mentioning

confidence: 99%

Right Versus Left Ventricular Failure

2014

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1033V entricular failure manifests in many forms, its underlying physiology ranging from overt left ventricular (LV) systolic dysfunction to isolated right ventricular (RV) diastolic dysfunction, and the wide portfolio of resulting symptoms vary from chronic fluid retention to acute multiorgan dysfunction and death. In this review, we discuss the morphological, functional, and molecular similarities and differences in RV and LV responses to adverse loading and failure. We further discuss whether LV and RV function and failure can truly be discussed as separate entities and thereby examine interactions between the ventricles that on one hand contribute to ventricular dysfunction but on the other may be harnessed for therapeutic benefit. Embryological and Physiological Differences Between the RV and LVThe RV and LV have different embryological origins.1 The LV originates from the primary heart field; the RV, from the secondary heart field. Consequently, several genes specifically control RV formation, including, among others, Hand2 and Tbx20.2 During gestation, the RV functions as the systemic ventricle ( Figure 1A). During fetal life, in addition to supplying the modest amount of pulmonary blood flow, the RV pumps blood to the lower body and placenta and contributes more than half of the combined cardiac output.3 With the transition from fetal to postnatal physiology and with the reduction in pulmonary vascular resistance, the subpulmonary RV transforms its morphology and geometry, becoming a thin-walled chamber to adopt its postnatal physiological characteristics. 4 Because it faces a low impedance pulmonary circulation, the normal postnatal RV maintains a cardiac output equal to that of the LV at approximately a fifth of the energy cost. The trapezoidal RV pressure-volume loop reflects this difference, with few if any isovolumic periods. Consequently, RV output starts early during pressure generation and is later maintained by a "hangout period" when antegrade flow continues into the pulmonary artery despite the onset of RV relaxation.5 In contrast, the rectangular LV pressure-volume loops reflect the LV square-wave pump function with distinct and well-developed isovolumic contraction and relaxation periods.6 Likewise, RV myocytes display faster twitch velocities than LV myocytes. 7The physiological differences are reflected in morphological differences between the ventricles (Table 1). The low-pressure RV is triangular in the sagittal plane and crescent-shaped in cross section as a result of the concave RV free wall and convex interventricular septum wrapping around the high-pressured, thick-walled, bullet-shaped LV. Consequently, although the normal RV has a lower ratio of volume to surface area and a thinner wall than the LV, 8 the low cavity pressure determines a lower wall stress and lower oxygen demands.Anatomic differences are also apparent in myocardial architecture. LV subepicardial and subendocardial fibers are oblique and helical with fiber angles ranging from 30° to 80° with a mean of ≈60°, whereas ...

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Effects of Regional Dysfunction and Late Gadolinium Enhancement on Global Right Ventricular Function and Exercise Capacity in Patients With Repaired Tetralogy of Fallot

Cited by 245 publications

References 35 publications

miR-21 is associated with fibrosis and right ventricular failure

miR-21 is associated with fibrosis and right ventricular failure

Mechanisms of Left Ventricular Dysfunction Assessed by Layer-Specific Strain Analysis in Patients With Repaired Tetralogy of Fallot

Right Versus Left Ventricular Failure

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