Circulation derived from 4D flow MRI correlates with right ventricular dysfunction in patients with tetralogy of Fallot

Tsuchiya, Nanae; Nagao, Michinobu; Shiina, Yumi; Miyazaki, Shohei; Inai, Kei; Murayama, Sadayuki; Sakai, Shuji

doi:10.1038/s41598-021-91125-2

Cited by 14 publications

(10 citation statements)

References 18 publications

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“…Previous studies have shown the utility of 4D flow-derived vorticity metrics which has been associated with inefficient blood flow in patients with pulmonary [ 12 , 14 , 15 , 20 – 23 ] and aortic disease [ 13 , 16 ]. Although qualitative vortices and swirling has been associated with diseased states [ 13 , 22 ], our study shows global quantitative metrics of vorticity increasing post-PEA in patients with CTEPH.…”

Section: Discussionmentioning

confidence: 99%

“…Other methods focus on the balance between clockwise and counterclockwise helicity and normalized helicity intensity in aortic valvular disease [18,19]. 4D flow CMR in the PAs has shown promise for characterizing RV-PA dynamics in healthy subjects and patients with PH through fluid dynamics derived metrics of secondary flow profiles, such as vorticity and helicity, specifically in severe stages of PH and large dilatation of the pulmonary trunk [12,14,[20][21][22][23]. However, comprehensive analyses of velocity flow profiles and secondary flow profiles including vorticity and helicity have yet to be investigated in patients with CTEPH following a PEA to determine their utility in evaluating recovery and reverse remodeling of the RV.…”

Section: Introductionmentioning

confidence: 99%

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4D flow cardiovascular magnetic resonance recovery profiles following pulmonary endarterectomy in chronic thromboembolic pulmonary hypertension

Dong

Azarine

Haddad

et al. 2022

Journal of Cardiovascular Magnetic Resonance

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Background Four-dimensional flow cardiovascular magnetic resonance imaging (4D flow CMR) allows comprehensive assessment of pulmonary artery (PA) flow dynamics. Few studies have characterized longitudinal changes in pulmonary flow dynamics and right ventricular (RV) recovery following a pulmonary endarterectomy (PEA) for patients with chronic thromboembolic pulmonary hypertension (CTEPH). This can provide novel insights of RV and PA dynamics during recovery. We investigated the longitudinal trajectory of 4D flow metrics following a PEA including velocity, vorticity, helicity, and PA vessel wall stiffness. Methods Twenty patients with CTEPH underwent pre-PEA and > 6 months post-PEA CMR imaging including 4D flow CMR; right heart catheter measurements were performed in 18 of these patients. We developed a semi-automated pipeline to extract integrated 4D flow-derived main, left, and right PA (MPA, LPA, RPA) volumes, velocity flow profiles, and secondary flow profiles. We focused on secondary flow metrics of vorticity, volume fraction of positive helicity (clockwise rotation), and the helical flow index (HFI) that measures helicity intensity. Results Mean PA pressures (mPAP), total pulmonary resistance (TPR), and normalized RV end-systolic volume (RVESV) decreased significantly post-PEA (P < 0.002). 4D flow-derived PA volumes decreased (P < 0.001) and stiffness, velocity, and vorticity increased (P < 0.01) post-PEA. Longitudinal improvements from pre- to post-PEA in mPAP were associated with longitudinal decreases in MPA area (r = 0.68, P = 0.002). Longitudinal improvements in TPR were associated with longitudinal increases in the maximum RPA HFI (r=-0.85, P < 0.001). Longitudinal improvements in RVESV were associated with longitudinal decreases in MPA fraction of positive helicity (r = 0.75, P = 0.003) and minimum MPA HFI (r=-0.72, P = 0.005). Conclusion We developed a semi-automated pipeline for analyzing 4D flow metrics of vessel stiffness and flow profiles. PEA was associated with changes in 4D flow metrics of PA flow profiles and vessel stiffness. Longitudinal analysis revealed that PA helicity was associated with pulmonary remodeling and RV reverse remodeling following a PEA.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

4D flow cardiovascular magnetic resonance recovery profiles following pulmonary endarterectomy in chronic thromboembolic pulmonary hypertension

Dong

Azarine

Haddad

et al. 2022

Journal of Cardiovascular Magnetic Resonance

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“…In our study cohort, MPA diameter in patients with RV moderate-severe dilatation was larger than that in patients with RV mild dilatation. Other studies paid more attention to hemodynamic factors in pulmonary artery, with some suggesting that increased longitudinal circulation of MPA was associated with RV dysfunction (35). In addition, it has been shown that end-diastolic anterior blood ow in the MPA is associated with poor outcomes in patients with rTOF(36).…”

Section: Preoperative Clinical Featuresmentioning

confidence: 99%

Right ventricular dilatation score: a new assessment to right ventricular dilatation in adult patients with repaired tetralogy of Fallot

Zhou¹,

Zhang²,

Han³

et al. 2023

Preprint

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Background: Patients with repaired tetralogy of Fallot (rTOF) experience long-term chronic pulmonary valve regurgitation resulting in right ventricular (RV) dilatation. According to current guidelines, the evaluation of patients with rTOF for RV dilatation should be based on cardiac magnetic resonance (CMR). However, for many asymptomatic patients, routine CMR is not practical. Our study aims to identify screening methods for CMR based on echocardiographic data, with the goal of establishing a more practical and cheap method of screening for severity of RV dilatation in patients with asymptomatic rTOF. Methods: 32 rTOF patients (mean age, 21(10.5) y, 21 males) with moderate to severe pulmonary regurgitation (PR) were prospectively recruited. Each patient received CMR and echocardiogram examination within 1 month prior to operation and collected clinical data, and then received echocardiogram examination at discharge and 3-6 months post-surgery. Results: RV moderate-severe dilatation was defined as right ventricular end-diastolic volume index (RVEDVI)≥160 ml/m2or right ventricular end-systolic volume index (RVESVI)≥80 ml/m2in 15 of 32 patients (RVEDVI, 202.15[171.51, 252.56] ml/m2, RVESVI, 111.99 [96.28, 171.74] ml/m2). The other 17 (RVESDI, 130.19 [117.91, 139.35] ml/m2, RVESVI= 67.91 [63.35, 73.11] ml/m2) were defined as right ventricle mild dilatation, i.e., RVEDVI<160ml/m2 and RVESVI<80 ml/m2, and the two parameters were higher than normal values. Compared with the RV mild dilatation group, patients of RV moderate-severe dilatation have worse cardiac function before surgery (right ventricular ejection fraction, 38.92(9.19) % versus 48.31(5.53) %, p<0.001; Left ventricular ejection fraction, 59.80(10.26) versus 66.41(4.15), p=0.021).Patients with RV moderate-severe dilatation faced longer operation time and more blood transfusion during operation (operation time, 271.53(08.33) min versus 170.53(72.36) min, p<0.01; Intraoperative blood transfusion, 200(175) ml versus 100(50) ml, p=0.001). Postoperative RV moderate-severe dilatation patients have poor short-term prognosis, which was reflected in a longer postoperative hospital stay (6.59 [2.12] days versus 9.80 [5.10] days, p=0.024) and a higher incidence of hypohepatia (0[0] % versus 4[26.7] %, p=0.023). Patients with RV dilatation score >2.35 were diagnosed with RV moderate-severe dilatation (AUC=0,882; Sensitivity =94.1%; Specificity =77.3%). Conclusions: RV moderate-severe dilatation is associated with worse preoperative cardiac function and short-term prognosis after PVR in rTOF patients with moderate to severe PR. The RV dilatation score is an effective screening method. When RV dilatation score>2.35, the patient is indicated for further CMR examination and treatment.

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“…[ 13 ] Tetralogy of Fallot (TOF) is the most common cyanotic heart condition in children that have survived beyond the neonatal period. [ 14 ] As described by Joseph et al, [ 15 ] they generated induced pluripotent stem cell (iPSC) lines from 5 patients with single ventricle disease (SVD), 5 patients with TOF, and 5 healthy individuals (non-CHD). Then, they differentiated all iPSC lines into cardiomyocytes (iPSC-CMs) and used highly enriched iPSC-CMs for RNA sequencing.…”

Section: Introductionmentioning

confidence: 99%

Alternative splicing signatures of congenital heart disease and induced pluripotent stem cell-derived cardiomyocytes from congenital heart disease patients

Zou

Liu

et al. 2022

Medicine

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Congenital heart disease (CHD) is the most serious congenital defect in newborns with higher mortality. Alternative splicing (AS) plays an essential role in numerous heart diseases. However, our understanding of the link between mRNA splicing and CHD in humans is limited. Here, we try to investigate the genome-wide AS events in CHD using bioinformatics methods. We collected available RNA-seq datasets of CHD-induced pluripotent stem cell-cardiomyocytes (iPSC-CMs) (including single ventricle disease [SVD] and tetralogy of Fallot [TOF]) and non-CHD from the Gene Expression Omnibus database. Then, we unprecedentedly performed AS profiles in CHD-iPSC-CMs and non-CHD-iPSC-CMs. The rMAPS was used to generate RNA-maps for the analysis of RNA-binding proteins’ (RBPs) binding sites. We used StringTie to identify and quantify the transcripts from aligned RNA-Seq reads. A quantification matrix was generated with respect to different groups by extracting the transcripts per million values from StringTie outputs. Then, this matrix was used for correlation analysis between the expression level of RBP and AS level. Finally, we validated our AS results using RNA-seq data from CHD and non-CHD patient tissue samples. We identified CHD-related AS events using CHD-iPSC-CMs and CHD samples from patients. The results showed that functional enrichment of abnormal AS in SVD and TOF was transcription factor-related. Using rMAPS, RNA-binding proteins which regulated these AS were also determined, and RBP-AS regulatory network was constructed. Overall, we identified abnormal AS in CHD-iPSC-CMs and CHD samples from patients. We predicted AS regulators in SVD and TOF, respectively. At last, we concluded that AS played a key role in the pathogenesis of CHD.

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Circulation derived from 4D flow MRI correlates with right ventricular dysfunction in patients with tetralogy of Fallot

Cited by 14 publications

References 18 publications

4D flow cardiovascular magnetic resonance recovery profiles following pulmonary endarterectomy in chronic thromboembolic pulmonary hypertension

4D flow cardiovascular magnetic resonance recovery profiles following pulmonary endarterectomy in chronic thromboembolic pulmonary hypertension

Right ventricular dilatation score: a new assessment to right ventricular dilatation in adult patients with repaired tetralogy of Fallot

Alternative splicing signatures of congenital heart disease and induced pluripotent stem cell-derived cardiomyocytes from congenital heart disease patients

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