Sacubitril/Valsartan for heart failure in adults with systemic right ventricle: A single center safety and efficacy experience

Aim: MicroRNAs (miRNAs) have been shown to play an important role in the progression of heart failure (HF). The aim of our study was to analyze miRNAs in the blood of patients with transposition of the great arteries and a systemic right ventricle (TGA-RV) in order to identify those that predict worsening HF.Materials and Methods: In 36 patients with TGA-RV, SurePrint™ 8 × 60K Human v21 miRNA microarrays were used to determine the miRNA abundance profiles and compared to 35 age- and gender-matched healthy volunteers (HVs). MiRNAs that were most significantly abundant or best related to worsening HF were further validated by RT-qPCR.Results: Using miRNA array analysis, a total of 50 down-regulated and 56 up-regulated miRNAs were found to be differentially abundant in TGA-RV patients compared to HVs. Six of these 106 miRNAs were significantly related to worsening HF. After validation by RT-qPCR, four miRNAs turned out to be significantly associated with worsening HF, namely miR-150-5p, miR-1255b-5p, miR-423-3p, and miR-183-3p. In the stepwise multivariable Cox regression analysis, ejection fraction of the systemic RV, high sensitive TNT and miR-183-3p were found to be independent predictors of worsening HF (P = 0.001, P = 0.002, and P = 0.001, respectively).Conclusions: In patients with TGA-RV, miR-183-3p is an independent predictor of worsening HF and thus may be used as additional biomarker in the risk assessment of these patients.

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Adverse remodeling of the subpulmonary left ventricle in patient with systemic right ventricle is associated with clinical outcome

European Heart Journal - Cardiovascular Imaging ◽

10.1093/ehjci/jeab090.118 ◽

2021 ◽

Vol 22 (Supplement_2) ◽

Author(s):

B Santens ◽

F Helsen ◽

A Van De Bruaene ◽

P De Meester ◽

A Budts ◽

...

Keyword(s):

Heart Failure ◽

Left Ventricle ◽

Clinical Outcome ◽

Right Ventricle ◽

Prognostic Markers ◽

Volume Index ◽

Short Term ◽

Systemic Right Ventricle ◽

Adverse Remodeling

Abstract Funding Acknowledgements Type of funding sources: Other. Main funding source(s): This research received project funding by KU Leuven Background – Early recognition of adverse remodeling is important since outcome is unfavorable once patients with a systemic right ventricle (sRV) become symptomatic. We aimed assessing prognostic markers linked to short-term clinical evolution in this population. Purpose - We aimed assessing short-term clinical evolution and early prognostic markers of cardiac complications in adults with sRV (atrial switch repair for D-transposition of the great arteries (D-TGA) and congenitally corrected transposition of the great arteries (ccTGA)) based on detailed phenotyping. Methods– Thirty-three patients with sRV underwent detailed phenotyping including exercise CMR. Adverse outcome was a composite of heart failure episode and tachyarrhythmia. Descriptive statistics and univariate cox regression analyses were performed. Results - Thirty-three patients (76% male) with sRV were followed over mean follow-up time of 3 years. Mean age was 40 ± 8 (range 26-57) years at latest follow-up. When compared to baseline, (I) most patients remained in NYHA functional class I (76%), (II) the degree of severity of the SAVV regurgitation rose and (III) more electrical instability was documented at latest follow-up. Six (18%) of a total of nine events were counted as first cardiovascular events (9% heart failure, 9% arrhythmia). NTproBNP (HR 11.02 (95%CI 1.296-93.662), p= 0.028), oxygen pulse (HR 1.202 (95% CI 1.012-1.428), p = 0.037), left ventricle end diastolic volume index (LVEDVi) in rest (HR 1.046 (95% CI 1.002-1.092), p = 0.041) and during exercise (HR 1.035 (95% CI 1.002-1.069), p = 0.038), stroke volume index (SVi) of the subpulmonary left ventricle (LV) in rest (HR 1.154 (95% CI 1.005-1.322), p = 0.038) and at peak exercise (HR 1.065 (95% CI 1.007-1.125), p = 0.026) were significantly associated with the first cardiovascular event (Figure 1A and B). Conclusion – NTproBNP was by far the best prognostic marker for clinical outcome. Adverse remodelling with increase of LVEDVi and SVi of the subpulmonary LV at rest and during exercise were associated with worse clinical outcome. We theorize that remodeling of the subpulmonary ventricle might be an early sign of a failing sRV circulation (Figure 2).

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Systolic dysfunction of the subpulmonary left ventricle is associated with the severity of heart failure in patients with a systemic right ventricle

International Journal of Cardiology ◽

10.1016/j.ijcard.2020.09.051 ◽

2020 ◽

Author(s):

Elena Surkova ◽

Teresa Segura ◽

Konstantinos Dimopoulos ◽

Daniela Bispo ◽

Caroline Flick ◽

...

Keyword(s):

Heart Failure ◽

Left Ventricle ◽

Right Ventricle ◽

Systolic Dysfunction ◽

Systemic Right Ventricle

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Levosimendan as a bridge to heart transplant in a 16-year-old patient with univentricular heart

Cardiology in the Young ◽

10.1017/s104795112000219x ◽

2020 ◽

Vol 30 (10) ◽

pp. 1504-1506

Author(s):

Magdalena Mazurak ◽

Paweł Cześniewicz ◽

Jacek Kusa

Keyword(s):

Heart Failure ◽

Right Ventricle ◽

Effective Treatment ◽

Heart Transplant ◽

Univentricular Heart ◽

Systemic Right Ventricle

AbstractWe report the successful use of levosimendan in the treatment of heart failure in a patient with the univentricular heart. The presented case was atypical because our patient had systemic right ventricle. To our knowledge, it is the first reported such case with intermittent levosimendan administration as an effective treatment and bridge to successful heart transplant in a patient with functionally univentricular heart.

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Pregnancy outcomes in women with a systemic right ventricle and transposition of the great arteries results from the ESC-EORP Registry of Pregnancy and Cardiac disease (ROPAC)

Heart ◽

10.1136/heartjnl-2020-318685 ◽

2021 ◽

pp. heartjnl-2020-318685

Author(s):

Oktay Tutarel ◽

Lucia Baris ◽

Werner Budts ◽

Mohamad Gamal Abd-El Aziz ◽

Csilla Liptai ◽

...

Keyword(s):

Heart Failure ◽

Right Ventricle ◽

Maternal Mortality ◽

Cardiac Disease ◽

Pregnancy Outcomes ◽

Fetal Loss ◽

Coronary Event ◽

Fetal Outcome ◽

Mortality Data ◽

Systemic Right Ventricle

ObjectiveCardiac disease is a major cause of maternal mortality. Data regarding pregnancy outcomes in women with a systemic right ventricle (sRV) are scarce. We studied pregnancy outcomes in women with an sRV after the atrial switch procedure for transposition of the great arteries (TGA) or congenitally corrected TGA (CCTGA).MethodsThe ESC EORP Registry of Pregnancy and Cardiac Disease is an international prospective registry of pregnant women with cardiac disease. Pregnancy outcomes (maternal/fetal) in all women with an sRV are described. The primary end point was a major adverse cardiac event (MACE) defined as maternal death, supraventricular or ventricular arrhythmias requiring treatment, heart failure, aortic dissection, endocarditis, ischaemic coronary event and other thromboembolic events.ResultsAltogether, 162 women with an sRV (TGA n=121, CCTGA n=41, mean age 28.8±4.6 years) were included. No maternal mortality occurred. In 26 women, at least one MACE occurred, heart failure in 16 (9.8%), arrhythmias (atrial 5, ventricular 6) in 11 (6.7%) and others in 4 (2.5%). Prepregnancy signs of heart failure as well as an sRV ejection fraction <40% were predictors of MACE. One woman experienced fetal loss, while no neonatal mortality was observed. No significant differences were found between women with CCTGA and TGA. In the subset of women who had an echocardiogram before and after pregnancy, no clear deterioration in sRV was observed.ConclusionThe majority of women with an sRV tolerated pregnancy well with a favourable maternal and fetal outcome. Heart failure and arrhythmias were the most common MACE.

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