Myocardial Fibrosis Identified by Cardiac Magnetic Resonance Late Gadolinium Enhancement Is Associated With Adverse Ventricular Mechanics and Ventricular Tachycardia Late After Fontan Operation

Q: What are the key findings of this study?

Positive late gadolinium enhancement on CMR was present in 28% of late Fontan survivors and was associated with higher odds of non-sustained ventricular tachycardia.

Q: What is the clinical evidence from this study?

Study design: Cohort. Population: Functional single ventricle late after Fontan operation (n=90). Intervention: Positive late gadolinium enhancement (LGE) / percent LGE of total myocardial mass on CMR vs. Absence of LGE in the ventricular myocardium (negative LGE). Primary outcome: Non-sustained ventricular tachycardia (NSVT) (OR 1.2, 95% CI 1.1-1.4, p=0.006).

Q: What does this research mean for the field?

Myocardial fibrosis detected by late gadolinium enhancement on cardiac MRI is associated with impaired ventricular mechanics and a higher prevalence of non-sustained ventricular tachycardia late after the Fontan operation. Novelty: ClaimNovelty.INCREMENTAL. Consensus alignment: ConsensusAlignment.NEUTRAL.

Rahul Hardas Rathod; Ashwin Prakash; Andrew J. Powell; Tal Geva

doi:10.1016/j.jacc.2009.12.036

April 1, 2010Journal of the American College of CardiologyOpen Access

Myocardial Fibrosis Identified by Cardiac Magnetic Resonance Late Gadolinium Enhancement Is Associated With Adverse Ventricular Mechanics and Ventricular Tachycardia Late After Fontan Operation

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Key result

Positive LGE on CMR linked to ~20% higher odds of NSVT in Fontan survivors.

OR 1.2
95% CI 1.1-1.4
P=0.006
n=90

Why the study?

Patients who have undergone the Fontan palliation may develop ventricular dysfunction and arrhythmias, but the underlying mechanisms and risk factors are poorly defined. The relationship between myocardial fibrosis identified by CMR and ventricular performance and arrhythmias late after the Fontan operation needed evaluation.

Is myocardial fibrosis identified by cardiac magnetic resonance late gadolinium enhancement associated with adverse ventricular mechanics and ventricular tachycardia in patients late after the Fontan operation?

Key Points

To evaluate the relationship between myocardial fibrosis identified by cardiac magnetic resonance and adverse ventricular performance and arrhythmias in patients late after Fontan palliation.
Retrospective cohort study of 90 patients (mean age at study 23.1 ± 10.9 years) who had undergone a Fontan operation and technically successful cardiac magnetic resonance with myocardial delayed enhancement between January 2002 and November 2008.
Ventricular volumes, mass, and ejection fraction were quantified from cine acquisitions, and myocardial fibrosis was identified and measured as percent late gadolinium enhancement (LGE) of total ventricular mass using the full-width at half-maximum technique.
Multivariate regression analysis with forward stepwise selection evaluated independent associations between LGE and ventricular mechanics or arrhythmia outcomes.
Ventricular late gadolinium enhancement was identified in 25 of 90 patients (28%).
Compared with patients without LGE, those with positive LGE demonstrated lower mean ejection fraction (45% v. 56%, P<0.001), higher median end-diastolic volume (100 mL/BSA1.3 v. 82 mL/BSA1.3, P=0.004), higher median ventricular mass index (63 g/BSA1.3 v. 45 g/BSA1.3, P<0.001), and more regional wall motion abnormalities (52% v. 28%, P=0.05).
Patients with positive LGE had a higher frequency of non-sustained ventricular tachycardia (36% v. 11%, P=0.01), and higher percent LGE remained independently associated with non-sustained ventricular tachycardia on multivariate regression (OR 1.2, 95% CI 1.1 to 1.4, P=0.006).

Study Design

Type

Cohort (n=90)

Multicenter

No

Structured PICO

Is myocardial fibrosis identified by cardiac magnetic resonance late gadolinium enhancement associated with adverse ventricular mechanics and ventricular tachycardia in patients late after the Fontan operation?

P

Population

90 patients late after the Fontan operation (mean age 23.1 ± 10.9 years, mean 15.7 ± 6.4 years since Fontan) who underwent cardiac magnetic resonance with myocardial delayed enhancement at a single US center.

E

Exposure

Presence and extent of myocardial fibrosis identified by cardiac magnetic resonance (CMR) late gadolinium enhancement (LGE), acquired 15-20 minutes after injection of 0.2 mmol/kg gadopentetate dimeglumine and quantified as percent LGE of total ventricular mass using the full-width at half-maximum (FWHM) technique.

C

Comparator

Patients late after the Fontan operation without positive late gadolinium enhancement (negative LGE) on CMR (n=65).

O

Outcome

Ventricular performance parameters (ejection fraction [EF], indexed end-diastolic volume [EDVi], indexed end-systolic volume [ESVi], indexed ventricular mass [massi], regional wall motion abnormalities [RWMA]) and arrhythmias (specifically non-sustained ventricular tachycardia [NSVT]).surrogate

Main Result

Odds Ratio: 1.2 (95% CI 1.1–1.4)

Absolute Event Rate: 36% vs 11%

p-value: p=0.006

Myocardial fibrosis detected by CMR late gadolinium enhancement is present in 28% of late Fontan survivors and correlates with ventricular dilation, hypertrophy, systolic dysfunction, and non-sustained ventricular tachycardia.

Limitations

Single-center, retrospective study design
Insufficient follow-up duration to assess hard clinical outcomes such as cardiac transplantation, ICD implantation, or death
Not designed to identify the root causes of myocardial fibrosis
MDE technique was not applied uniformly to all Fontan patients undergoing CMR and evolved over the study period
Selection bias due to exclusion of patients with pacemakers or defibrillators and potential over-representation of patients with ventricular dysfunction
Lack of long-term follow-up to evaluate hard clinical outcomes such as cardiac transplantation, ICD implantation, or death
Study was not designed to identify the root causes of myocardial fibrosis
MDE technique was not applied uniformly to all patients undergoing CMR and evolved during the study period
Selection bias from exclusion of patients with pacemakers and defibrillators and potential over-representation of symptomatic patients with ventricular dysfunction

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Implication

Supports LGE as arrhythmia risk marker in Fontan survivors; leaves open need for prospective validation before clinical adoption.

Abstract

Objectives To evaluate the relationship between myocardial fibrosis identified by cardiac magnetic resonance (CMR) and ventricular performance and arrhythmias in patients late after the Fontan operation. Background Patients who have undergone the Fontan palliation may develop ventricular dysfunction and arrhythmias, but the mechanisms and risk factors are poorly defined. Methods All patients who have had a Fontan operation and a CMR study with the myocardial delayed enhancement technique from January 2002 to November 2008 were retrospectively identified. Results Of 90 patients (mean age at study 23.1 ± 10.9 years), 25 (28%) had positive late gadolinium enhancement (LGE) in the ventricular myocardium. Patients with positive LGE had lower mean ejection fraction (EF) (45% v. 56%, P<0.001), increased median end-diastolic volume (EDVi) (100 mL/BSA1.3 v. 82 mL/BSA1.3, P=0.004), increased median ventricular massi (63 g/BSA1.3 v. 45 g/BSA1.3, P<0.001), higher frequency of regional wall motion abnormalities (52% v. 28%, P=0.05), and higher frequency of non-sustained ventricular tachycardia (NSVT) (36% v. 11%, P=0.01). Multivariate regression analysis demonstrated that more extensive positive LGE, expressed as percent LGE of total myocardial mass, was associated with lower EF (P=0.002), increased EDVi (P<0.001), increased massi (P<0.001), and a higher frequency of NSVT (OR 1.2, 95% CI 1.1 to 1.4, P=0.006). Conclusions In this cohort of late Fontan survivors, myocardial fibrosis was common and associated with adverse ventricular mechanics and higher prevalence of NSVT. Further studies are warranted to examine the utility of LGE for risk stratification and treatment of ventricular arrhythmia and dysfunction in Fontan patients.

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Cite This Study

Rathod et al. (2010) conducted a cohort in Functional single ventricle late after Fontan operation (n=90). Positive late gadolinium enhancement (LGE) / percent LGE of total myocardial mass on CMR vs. Absence of LGE in the ventricular myocardium (negative LGE) was evaluated on Non-sustained ventricular tachycardia (NSVT) (OR 1.2, 95% CI 1.1-1.4, p=0.006). Positive late gadolinium enhancement on CMR was present in 28% of late Fontan survivors and was associated with higher odds of non-sustained ventricular tachycardia.

synapsesocial.com/papers/6af5d8201e5d5ce4576e3443 https://doi.org/10.1016/j.jacc.2009.12.036

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Myocardial Fibrosis Identified by Cardiac Magnetic Resonance Late Gadolinium Enhancement Is Associated With Adverse Ventricular Mechanics and Ventricular Tachycardia Late After Fontan Operation

Key Points

Study Design

Structured PICO

Main Result

Limitations

Ask about this paper

Implication

Abstract

Discussion

Cite This Study

Myocardial Fibrosis Identified by Cardiac Magnetic Resonance Late Gadolinium Enhancement Is Associated With Adverse Ventricular Mechanics and Ventricular Tachycardia Late After Fontan Operation

Key Points

Study Design

Structured PICO

Main Result

Limitations

Ask about this paper

Implication

Abstract

Discussion

Cite This Study

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