Dataset Information

Longitudinal changes in echocardiographic measures of ventricular function after Fontan operation.

ABSTRACT:

Background

While numerous indices have been developed in an attempt to quantify ventricular function in patients with single ventricle heart disease after Fontan, there are little data on how these parameters change over time.

Methods

A retrospective observational study was performed of individuals who underwent Fontan operation at Children's Hospital of Philadelphia (CHOP) in 2006 and 2007. Measurements of fractional area change (FAC), tricuspid annular planar systolic excursion (TAPSE), myocardial performance index (MPI), systolic to diastolic (s/d) ratio, and myocardial strain and strain rate were made offline. A composite outcome of protein-losing enteropathy (PLE), plastic bronchitis, transplant, or death was created, and change in function was compared between those who did and those who did not meet the outcome.

Results

There were 312 echocardiograms from 40 unique patients (75% male, 55% dominant right ventricle). The aggregate mean values for most assessed parameters were worse than what would be expected for a healthy age-matched population. The global longitudinal strain rate increased (worsened) by 0.014 (1/s) per year (P = .02), and the global circumferential strain rate increased (worsened) by 0.011 (1/s) per year (P = .01). There was no difference in the rate of change of ventricular function in the 6 patients who met the composite endpoint vs those who did not.

Conclusion

This study demonstrates that global longitudinal strain rate and global circumferential strain rate decrease over 10 years following Fontan operation. These measures of ventricular performance may be early signs of cardiac dysfunction that predate more obvious echocardiographic signs of deterioration.

SUBMITTER: Campbell MJ

PROVIDER: S-EPMC7721979 | biostudies-literature |

REPOSITORIES: biostudies-literature

ACCESS DATA

Similar Datasets

Project description:ObjectivePhysical activity at high altitude is expected to pose risks for patients with Fontan circulation and to impair systemic ventricular function. This study aims to determine the effect of high-altitude hypoxia on ventricular function in Fontan patients at rest and after exercise. We hypothesize that systemic ventricular function deteriorates under hypoxic conditions in Fontan patients.MethodsIn this prospective study, 21 Fontan patients (NYHA class I-II) and 21 age-, gender- and body mass index-matched healthy controls were enrolled (median age 17.9 and 16.9 years). Transthoracic echocardiography was performed at rest, after peak (PE) and after continuous exercise (CE) in normoxia and hypoxia at simulated altitude (2,500 m above sea level). The effect of hypoxia on echocardiographic parameters was quantified by linear mixed-effects models and the difference between normoxia and hypoxia (Δ= hypoxia-normoxia).ResultsAt rest, cardiac output (CO) estimated by outflow tract velocity time integral × heart rate and annular plane systolic excursion (APSE) were lower in hypoxia compared to normoxia in Fontan patients (CO: Δ = -12.0%, n.s.; APSE: Δ = -9.6%, p < 0.001), an increase was observed in controls (CO: Δ = 8.5%, n.s.; APSE: Δ = 2.5%, n.s.). Other parameters of systolic and diastolic function did not show relevant changes. After exercise under hypoxic conditions, Fontan patients did not show relevant deterioration of systolic function compared to normoxia. Late, active diastolic filling reflected by A-wave velocity remained unchanged in Fontan patients, but increased in controls. Under hypoxic conditions, CO and workload were higher after CE than PE in Fontan patients (CO: PE Δ = 1,530 vs. CE 1630), whereas controls showed higher work load and CO estimates after PE than CE as expected (CO: PE Δ = 2,302 vs. CE 2149).ConclusionFontan patients clinically tolerated short-term altitude exposure up to two hours and exercise and showed no consistent deterioration of systolic systemic ventricular function, but parameters of myocardial contractility, heart rate and cardiac output did not increase as observed in controls. This is likely to be multifactorial and may include intrinsic cardiac dysfunction as well as preload inadequacy and the lack of augmented atrial contraction. CE may be better tolerated than PE.

Project description:Background Prenatal diagnosis of single ventricle-type congenital heart disease is associated with improved clinical courses. Prenatal counseling allows for optimal delivery preparations and opportunity for prenatal intervention. Expectant parents frequently ask what the likelihood of survival through staged palliation is and the factors that influence outcome. Our goal was specifically to quantify peri- and postnatal outcomes in this population. Methods and Results We identified all patients with a prenatal diagnosis of single ventricle-type congenital heart disease presenting between July 2004 and December 2011 at our institution. Maternal data, fetal characteristics, and data from the postnatal clinical course were collected for each patient. Kaplan-Meier curves and multivariate analysis with logistic regression were used to evaluate variables associated with decreased transplant-free survival. Five hundred two patients were identified, consisting of 381 (76%) right ventricle- and 121 left ventricle-dominant lesions. After prenatal diagnosis, 42 patients did not follow up at our center; 79 (16%) chose termination of pregnancy, and 11 had intrauterine demise with 370 (74%) surviving to birth. Twenty-two (6%) underwent palliative care at birth. Among 348 surviving to birth with intention to treat, 234 (67%) survived to at least 6 months post-Fontan palliation. Presence of fetal hydrops, right ventricle dominance, presence of extracardiac anomalies, and low birthweight were significantly associated with decreased transplant-free survival. Conclusions In patients with a prenatal diagnosis of single ventricle-type congenital heart disease and intention to treat, 67% survive transplant-free to at least 6 months beyond Fontan operation. An additional 5% survive to 4 years of age without transplant or Fontan completion. Fetuses with right ventricle-dominant lesions, extracardiac anomalies, hydrops, or low birthweights have decreased transplant-free survival.