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Hemodynamics of Fontan Failure: The Role of Pulmonary Vascular Disease.

ABSTRACT: Nonpulsatile pulmonary blood flow in Fontan circulation results in pulmonary vascular disease, but the potential relationships between pulmonary vascular resistance index (PVRI) and Fontan failure have not been studied. The objective was to determine whether the absence of subpulmonary ventricle in the Fontan circulation would make patients more vulnerable to even low-level elevations in PVRI, and when coupled with low cardiac index, this would identify patients at increased risk of Fontan failure.Two hundred sixty-one adult Fontan patients underwent cardiac catheterization; age 26±3 years, men 146 (56%), atriopulmonary Fontan 144 (55%). Patients were divided into 2 groups: those with high PVRI (>2 WU·m2) and low cardiac index <2.5 L min-1 m-2 (group 1, n=70, 30%), and those with normal PVRI and normal cardiac index (group 2, n=182, 70%). Fontan failure was defined by the composite of all-cause mortality, listing for heart transplantation, or initiation of palliative care. There were 68 (26%) cases of Fontan failure during a mean follow-up of 8.6±2.4 years. When compared with group 2, freedom from Fontan failure was significantly lower in group 1: 66% versus 89% at 5 years. The combination of high PVRI and low cardiac index was an independent risk factor for Fontan failure (hazard ratio, 1.84; 95% confidence interval, 1.09-2.85).When coupled with low cardiac index, even mild elevations in PVRI identify patients at high risk of Fontan failure. This suggests that pulmonary vascular disease is a key mechanism underlying Fontan failure and supports further studies to understand the pathophysiology and target treatments to pulmonary vascular tone in this population.

SUBMITTER: Egbe AC

PROVIDER: S-EPMC5739063 | biostudies-literature | 2017 Dec

REPOSITORIES: biostudies-literature

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Hemodynamics of Fontan Failure: The Role of Pulmonary Vascular Disease.

Egbe Alexander C AC Connolly Heidi M HM Miranda William R WR Ammash Naser M NM Hagler Donald J DJ Veldtman Gruschen R GR Borlaug Barry A BA

Circulation. Heart failure 20171201 12

<h4>Background</h4>Nonpulsatile pulmonary blood flow in Fontan circulation results in pulmonary vascular disease, but the potential relationships between pulmonary vascular resistance index (PVRI) and Fontan failure have not been studied. The objective was to determine whether the absence of subpulmonary ventricle in the Fontan circulation would make patients more vulnerable to even low-level elevations in PVRI, and when coupled with low cardiac index, this would identify patients at increased r ...[more]

PMID: 29246897

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Project description:BackgroundPulmonary vascular disease, pulmonary endothelial dysfunction, liver fibrosis, renal disease, and exercise intolerance are common in adults with Fontan physiology. Although the pathophysiologic mechanisms linking these phenomena have been studied, certain aspects are not well understood.ObjectivesThis study hypothesized that impaired pulmonary vascular reserve (VR) plays a central role linking these abnormalities, and that patients with abnormal pulmonary VR with exercise, compared with patients with normal VR, would display poorer pulmonary endothelial function, greater liver stiffness, more renal dysfunction, and poorer exercise capacity.MethodsSymptomatic adults with the Fontan palliation (n = 29) underwent invasive cardiopulmonary exercise testing, echocardiography, and assessment of microvascular function. Abnormal pulmonary VR was defined by the slope of increase in pulmonary pressure relative to cardiac output with exercise >3 mm Hg/l/min. Pulmonary endothelial function was assessed using reactive hyperemia index. End-organ function was assessed using magnetic resonance elastography-derived liver stiffness, glomerular filtration rate, N-terminal pro-B-type natriuretic peptide, and peak oxygen consumption (Vo2).ResultsCompared with individuals with normal VR (n = 8), those with abnormal VR (n = 21) displayed higher central and pulmonary venous pressures, and more severely impaired cardiac output and stroke volume responses to exertion, but similar pulmonary vascular resistance at rest. Patients with abnormal VR displayed more severely impaired reactive hyperemia index, increased liver stiffness, lower glomerular filtration rate, higher N-terminal pro-B-type natriuretic peptide, and lower peak Vo2. As compared to pulmonary vascular resistance at rest, slope of increase in pulmonary pressure relative to cardiac output displayed stronger correlations with reactive hyperemia index (r = -0.63 vs. r = -0.31; Meng test p = 0.009), magnetic resonance elastography-derived liver stiffness (r = 0.47 vs. r = 0.29; Meng test p = 0.07), glomerular filtration rate (r = -0.52 vs. r = -0.24; Meng test p = 0.03), N-terminal pro-B-type natriuretic peptide (r = 0.56 vs. r = 0.17; Meng test p = 0.02), and peak Vo2 (r = -0.63 vs. r = -0.26; Meng test p = 0.02).ConclusionsPulmonary vascular limitations in Fontan physiology are related to pulmonary endothelial and end-organ dysfunction, suggesting a mechanistic link between these commonly observed findings, and these abnormalities are more apparent during exercise testing, with little relationship at rest.

Project description:Objective:The one and a half ventricle repair (1.5VR) is a common clinical choice for patients with right heart dysfunction. Considering the influence of blood circulation failure and reoperation in urgent need, this essay aims to explore the hemodynamic effects of different pulmonary vascular resistance (PVR) values on reoperation after 1.5VR failure. Methods:The lumped parameter model (LPM) was used to simulate the reoperation, including the return biventricular repair (2VR), ligation of azygos vein (1.5VR') and return single ventricular repair (1.0VR). Firstly, the debugging parameters were used to simulate the hemodynamics of 2VR. Secondly, the value of PVR was changed from one to four times while the other parameters remained unchanged. Finally, 15 cardiac cycles were simulated and the 15th result was obtained. In this work, the left and right ventricular stroke work and their sum (Plv, Prv, Ptotal), the left and right ventricular ejection fraction (LVEF, RVEF), the mean Cardiac Output (mCO) and the mean pressure and flow-rate ratio of superior and inferior vena cava (mPsvc\mPivc and mQsvc\mQivc), respectively, were used to describe the hemodynamics of reoperation. Results:With the change of PVR from one to four times, the values of Plv, Prv, Ptotal, LVEF, and RVEF gradually decreased. The change rate of Plv, Ptotal and LVEF of 1.0VR were the largest in the three kinds of reoperation. The change rate of Prv of 1.5VR' was larger than that of 2VR, but it was the opposite for their EF change rate. The mCO of 2VR, 1.5VR', and 1.0VR decreased by 18.53%, 37.58%, and 48.07%, respectively. The mPsvc\mPivc of 1.5VR' increased from 3.76 to 6.77 and the mQsvc\mQivc decreased from 0.55 to 0.36, while the mPsvc\mPivc and mQsvc\mQivc of 2VR and 1.0VR remained 1 and 0.67, respectively. The peak value of the tricuspid flow-rate (Qti) waveform of 2VR and 1.5VR' changed from "E peak" to "A peak." Conclusion:The numerical results demonstrate the highly reoperation-dependent hemodynamic consequences and their responses to variations in PVR. Comprehensive analysis of EF, mCO and ventricular stroke work indicates that PVR has a greater impact on 1.5VR' and 1.0VR. Therefore, we suggest that the selection strategy of reoperation should focus on PVR.

Dataset Information

Hemodynamics of Fontan Failure: The Role of Pulmonary Vascular Disease.

Publications

Hemodynamics of Fontan Failure: The Role of Pulmonary Vascular Disease.

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