Serial right ventricular assessment in patients with hypoplastic left heart syndrome: a multiparametric cardiovascular magnetic resonance study

Sobh, Mohamed; Freitag‐Wolf, Sandra; Scheewe, Jens; Kanngiesser, Luca Mitch; Uebing, Anselm; Gabbert, Dominik Daniel; Voges, Inga

doi:10.1093/ejcts/ezab232

Cited by 9 publications

(9 citation statements)

References 20 publications

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“…In a recent study, we were able to show that in patients with HLHS RVEF remains largely unchanged over a period of 10 years after TCPC completions but that indexed RV volumes significantly increase in patients older than 10 years of age. 8 …”

Section: Discussionmentioning

confidence: 99%

“…That there is an increase of indexed RV volumes during longitudinal follow in older patients with HLHS (>10 years) was already recently shown by this group. 8 Potential reasons might be a higher degree of tricuspid regurgitation but also cardiovascular and metabolic changes during puberty with a raise in blood pressure, increased RV afterload and insulin resistance should be taken into account. 38 , 39 …”

Section: Discussionmentioning

confidence: 99%

“…In a recent study, our group showed a significant increase in RV volumes with only mild reduction in RV ejection in patients with HLHS during serial follow‐up. 8 …”

mentioning

confidence: 99%

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Serial Assessment of Right Ventricular Deformation in Patients With Hypoplastic Left Heart Syndrome: A Cardiovascular Magnetic Resonance Feature Tracking Study

Kanngiesser

Freitag‐Wolf

Grazioli

et al. 2022

JAHA

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Background As right ventricular dysfunction is a major cause of adverse outcome in patients with hypoplastic left heart syndrome, the aim was to assess right ventricular function and deformation after Fontan completion by performing 2‐dimensional cardiovascular magnetic resonance feature tracking in serial cardiovascular magnetic resonance studies. Methods and Results Cardiovascular magnetic resonance examinations of 108 patients with hypoplastic left heart syndrome (female: 31) were analyzed. Short‐axis cine images were used for right ventricular volumetry. Two‐dimensional cardiovascular magnetic resonance feature tracking was performed using long‐axis and short‐axis cine images to measure myocardial global longitudinal, circumferential, and radial strain. All patients had at least 2 cardiovascular magnetic resonance examinations after Fontan completion and 41 patients had 3 examinations. Global strain values and right ventricular ejection fraction decreased from the first to the third examination with a significant decline in global longitudinal strain from the first examination to the second examination (median, first, and third quartile: −18.8%, [−20.5;−16.5] versus −16.9%, [−19.3;−14.7]) and from the first to the third examination in 41 patients (−18.6%, [−20.9;−15.7] versus −15.8%, [−18.7;−12.6]; P ‐values <0.004). Right ventricular ejection fraction decreased significantly from the first to the third examination (55.4%, [49.8;59.3] versus 50.2%, [45.0;55.9]; P <0.002) and from the second to the third examination (53.8%, [47.2;58.7] versus 50.2%, [45.0;55.9]; P <0.0002). Conclusions Serial assessment of cardiovascular magnetic resonance studies in patients with hypoplastic left heart syndrome after Fontan completion demonstrates a significant reduction in global strain values and right ventricular ejection fraction at follow‐up. The significant reduction in global longitudinal strain between the first 2 examinations with non‐significant changes in right ventricular ejection fraction suggest that global longitudinal strain measured by 2‐dimensional cardiovascular magnetic resonance feature tracking might be a superior technique for the detection of changes in myocardial function.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Serial Assessment of Right Ventricular Deformation in Patients With Hypoplastic Left Heart Syndrome: A Cardiovascular Magnetic Resonance Feature Tracking Study

Kanngiesser

Freitag‐Wolf

Grazioli

et al. 2022

JAHA

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“…Longitudinal studies using transthoracic echocardiography have shown progressive ventricular dilation and dysfunction after the Fontan operation; however, the clinical importance of these changes are not well known [16][17][18][19]. Prior publications from our institution using cross-sectional cohorts that overlap with the current study have demonstrated relationship between ventricular dilation and dysfunction with transplantfree survival [10,12].…”

Section: Discussionmentioning

confidence: 61%

Longitudinal changes in ventricular size and function are associated with death and transplantation late after the Fontan operation

Ghelani

Lü

Sleeper

et al. 2022

Journal of Cardiovascular Magnetic Resonance

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Background Cross-sectional studies have reported that ventricular dilation and dysfunction are associated with adverse clinical outcome in Fontan patients; however, longitudinal changes and their relationship with outcome are not known. Methods This was a single-center retrospective analysis of Fontan patients with at least 2 cardiovascular magnetic resonance (CMR) scans without intervening interventions. Serial measures of end-diastolic volume index (EDVI), end-systolic volume index (ESVI), ejection fraction (EF), indexed mass (massi), mass-to-volume ratio, and end-systolic wall stress (ESWS) were used to estimate within-patient change over time. Changes were compared for those with and without a composite outcome (death, heart transplant, or transplant listing) as well as between patients with left (LV) and right ventricular (RV) dominance. Results Data from 156 patients were analyzed with a mean age at 1st CMR of 17.8 ± 9.6 years. 490 CMRs were included with median of 3 CMRs/patient (range 2–9). On regression analysis with mixed effects models, volumes and ESWS increased, while mass, mass-to-volume ratio, and EF decreased over time. With a median follow-up of 10.2 years, 14% met the composite outcome. Those with the composite outcome had a greater increase in EDVI compared to those without (4.7 vs. 0.8 ml/BSA1.3/year). Compared with LV dominance, RV dominance was associated with a greater increase in ESVI (1.4 vs. 0.5 ml/BSA1.3/year), a greater decrease in EF (− 0.61%/year vs. − 0.24%/year), and a higher rate of the composite outcome (21% vs. 8%). Conclusions Ventricles in the Fontan circulation exhibit a steady decline in performance with an increase in EDVI, ESVI, and ESWS, and decrease in EF, mass index, and mass-to-volume ratio. Those with death or need for heart transplantation have a faster increase in EDVI. Patients with rapid increase in EDVI (> 5 ml/BSA1.3/year) may be at a higher risk of adverse outcomes and may benefit from closer surveillance. RV dominance is associated with worse clinical outcomes and remodeling compared to LV dominance.

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“…Right ventricular function was measured with CMR as well as with 2-dimensional transthoracic echocardiography. Participants who underwent CMR were 10.9 years of age (interquartile range, 10.4-11.5) at the time of CMR, and, compared with those who did not have CMR, they had a significantly shorter hospital length of stay after the Norwood procedure (22 [interquartile range, [16][17][18][19][20][21][22][23][24][25][26][27][28][29][30][31][32][33] versus 28 days [interquartile range, 20-39]; P=0.02) and were more likely to be male (66% versus 47%; P=0.01; Table S2). Among the 177 participants who underwent CMR, RVEF was measurable in 168 (95%); the mean±SD for the overall cohort was 51.5±8.6% (RVPAS 51±9.…”

Section: Right Ventricular Functionmentioning

confidence: 99%

Longitudinal Follow-Up of Children With HLHS and Association Between Norwood Shunt Type and Long-Term Outcomes: The SVR III Study

Goldberg,

Trachtenberg,

William Gaynor

et al. 2023

Circulation

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OBJECTIVE: In the SVR trial (Single Ventricle Reconstruction), newborns with hypoplastic left heart syndrome were randomly assigned to receive a modified Blalock-Taussig-Thomas shunt (mBTTS) or a right ventricle-to-pulmonary artery shunt (RVPAS) at Norwood operation. Transplant-free survival was superior in the RVPAS group at 1 year, but no longer differed by treatment group at 6 years; both treatment groups had accumulated important morbidities. In the third follow-up of this cohort (SVRIII [Long-Term Outcomes of Children With Hypoplastic Left Heart Syndrome and the Impact of Norwood Shunt Type]), we measured longitudinal outcomes and their risk factors through 12 years of age. METHODS: Annual medical history was collected through record review and telephone interviews. Cardiac magnetic resonance imaging (CMR), echocardiogram, and cycle ergometry cardiopulmonary exercise tests were performed at 10 through 14 years of age among participants with Fontan physiology. Differences in transplant-free survival and complication rates (eg, arrhythmias or protein-losing enteropathy) were identified through 12 years of age. The primary study outcome was right ventricular ejection fraction (RVEF) by CMR, and primary analyses were according to shunt type received. Multivariable linear and Cox regression models were created for RVEF by CMR and post-Fontan transplant-free survival. RESULTS: Among 549 participants enrolled in SVR, 237 of 313 (76%; 60.7% male) transplant-free survivors (mBTTS, 105 of 147; RVPAS, 129 of 161; both, 3 of 5) participated in SVRIII. RVEF by CMR was similar in the shunt groups (RVPAS, 51±9.6 [n=90], and mBTTS, 52±7.4 [n=75]; P =0.43). The RVPAS and mBTTS groups did not differ in transplant-free survival by 12 years of age (163 of 277 [59%] versus 144 of 267 [54%], respectively; P =0.11), percentage predicted peak V o 2 for age and sex (74±18% [n=91] versus 72±18% [n=84]; P =0.71), or percentage predicted work rate for size and sex (65±20% versus 64±19%; P =0.65). The RVPAS versus mBTTS group had a higher cumulative incidence of protein-losing enteropathy (5% versus 2%; P =0.04) and of catheter interventions (14 versus 10 per 100 patient-years; P =0.01), but had similar rates of other complications. CONCLUSIONS: By 12 years after the Norwood operation, shunt type has minimal association with RVEF, peak V o 2 , complication rates, and transplant-free survival. RVEF is preserved among the subgroup of survivors who underwent CMR assessment. Low transplant-free survival, poor exercise performance, and accruing morbidities highlight the need for innovative strategies to improve long-term outcomes in patients with hypoplastic left heart syndrome. REGISTRATION: URL: https://www.clinicaltrials.gov ; Unique identifier: NCT00115934.

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Serial right ventricular assessment in patients with hypoplastic left heart syndrome: a multiparametric cardiovascular magnetic resonance study

Cited by 9 publications

References 20 publications

Serial Assessment of Right Ventricular Deformation in Patients With Hypoplastic Left Heart Syndrome: A Cardiovascular Magnetic Resonance Feature Tracking Study

Serial Assessment of Right Ventricular Deformation in Patients With Hypoplastic Left Heart Syndrome: A Cardiovascular Magnetic Resonance Feature Tracking Study

Longitudinal changes in ventricular size and function are associated with death and transplantation late after the Fontan operation

Longitudinal Follow-Up of Children With HLHS and Association Between Norwood Shunt Type and Long-Term Outcomes: The SVR III Study

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