Hemodynamic determinants of mortality after Fontan operation

Ohuchi, Hideo; Miyazaki, Aya; Negishi, Jun; Hayama, Yosuke; Nakai, Michikazu; Nishimura, Kunihiro; Ichikawa, Hajime; Shiraishi, Isao; Yamada, Osamu

doi:10.1016/j.ahj.2017.03.020

Cited by 60 publications

(52 citation statements)

References 26 publications

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“…The normal CI/high FP profile was associated with the worst long‐term survival in our cohort. Our findings are in agreement with the observations of Ohuchi et al . who reported the combination of a central venous pressure ≥ 14 mmHg and a CI ≥3.0 L/min/m 2 to be associated with reduced survival among adults post‐Fontan.…”

Section: Discussioncontrasting

confidence: 48%

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Haemodynamic profiles in adult Fontan patients: associated haemodynamics and prognosis

Miranda

Borlaug

Hagler

et al. 2019

European J of Heart Fail

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Aims The Fontan operation is associated with significant long‐term morbidity and mortality, posing challenges in management. No haemodynamic classification has been established to aid management and prognostication of patients with Fontan palliation. Our aim was to assess haemodynamic profiles for Fontan patients and evaluate their relationships to prognosis. Methods and results Eighty‐four adult Fontan patients without obstruction or significant valve disease undergoing cardiac catheterization for Fontan‐related co‐morbidities were divided into four different haemodynamic profiles based on normal/high Fontan pressures (FP) (< 15 or ≥ 15 mmHg) and low/normal cardiac index (CI) (< 2.5 or ≥ 2.5 L/min/m2). Fourteen patients had low CI/high FP (17%), 24 low CI/normal FP (29%), 20 normal CI/high FP (24%), and 26 normal CI/normal FP (31%). Demographic and clinical data were similar among groups except for a trend towards a higher prevalence of cirrhosis in the normal CI/high FP (50%) group. Systemic vascular afterload, assessed by systemic vascular resistance and effective arterial elastance, was higher in patients with low CI/high FP and low CI/normal FP as compared to normal CI profiles. Pulmonary vascular resistance indices were higher in patients with low CI/high FP than all other profiles. The normal CI/high FP haemodynamic profile was an independent predictor of mortality (hazard ratio 4.1, 95% confidence interval 1.2–11.5). Heterotaxy and protein‐losing enteropathy were also predictors of mortality on the multivariate model. Conclusion The proposed haemodynamic classification provides prognostic information in adults post‐Fontan with patients in the normal CI/high FP profile having worse survival. Whether this classification can guide management in these patients deserves further investigation.

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Section: Discussioncontrasting

confidence: 48%

“…The presence of right‐to‐left shunt via VVC or pulmonary arteriovenous fistulae has also been proposed as an additional mechanism for the increased CI in failing Fontan patients . Although CI was inversely proportional to arterial oxygen saturation in our patients, their correlation was not statistically significant.…”

Section: Discussionmentioning

confidence: 99%

Haemodynamic profiles in adult Fontan patients: associated haemodynamics and prognosis

Miranda

Borlaug

Hagler

et al. 2019

European J of Heart Fail

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“…Although classification based on haemodynamic parameters in Fontan patients is not completely new and has been reported in other variants, the finding in this cohort that a ‘normal CI/high FP’ profile appeared to be an independent predictor for mortality seems counterintuitive. A high CVP, high pulmonary vascular resistance (PVR) and low CI are all known to predict adverse outcome as reported in previous reports, including one of the current authors . However, the finding that not a reduced but a normal CI (> 2.5 L/min/m 2 , in combination with high CVP) predicts mortality is more difficult to digest.…”

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confidence: 51%

The Janus‐faced Fontan circulation: unravelling its elusive pathophysiology

Ridderbos

Hoendermis

Berger

et al. 2019

European J of Heart Fail

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This article refers to 'Haemodynamic profiles in adultFontan patients: associated haemodynamics and prognosis' by W.R. Miranda et al., published in this issue on pages 803-809.The Fontan procedure is considered the treatment of choice for patients with a univentricular heart defect for whom biventricular repair is not possible. The Fontan procedure entails the surgical rerouting of the systemic venous return directly to the pulmonary arteries and thereby renouncing a subpulmonary ventricle. This results in a restored balance between pulmonary and systemic blood flow, maintaining oxygenation and perfusion. However, due to the absence of a subpulmonary ventricle, the Fontan circulation is characterized by a paradoxal chronically increased systemic venous pressure and non-pulsatile pulmonary blood flow. Furthermore, the single ventricle needs to drive the cardiac output through both the systemic and pulmonary vascular beds.Short-term outcomes of the Fontan procedure have been considered successful. However, long-term outcomes are characterized by an impaired functional clinical status with gradual attrition of the Fontan circulation over time.

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“…The main complications after the Fontan procedure have been described as multi-end-organ congestion due to high venous pressure, low cardiac output leading to chronic heart failure, and mild but significant hypoxia [21]. In addition, elevated CVP and low arterial blood oxygen saturation have correlated with mortality in both early and late Fontan patients, while a greater end-diastolic volume index, along with a lower ejection fraction, has also been associated with increased mortality in early term patients [22]. With EDP and LAP pressures increasing, it is possible that this operation could lead to cardiac dysfunction, lowered cardiac output, and death, and so these values should be monitored on a long-term scale.…”

Section: Hemodynamics and Morbiditiesmentioning

confidence: 99%