Valvuloplasty in 103 fetuses with critical aortic stenosis: outcome and new predictors for postnatal circulation

Tulzer, Andreas; Arzt, W; Gitter, Roland; Sames-Dolzer, Eva; Kreuzer, Michaela; Mair, Rudolf; Tulzer, Gerald

doi:10.1002/uog.24792

Cited by 33 publications

(63 citation statements)

References 25 publications

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“…All cases with a prenatal diagnosis of SAS, defined as a combination of valvular AS ( Figure 1 ), maximum velocity of mitral insufficiency (MI) >250 cm/s, monophasic inflow in the left ventricle ( Figure 2 ), reversal of flow in the transverse aortic arch ( Figure 3 ) and left ventricular length Z-score >−1, detected in a 10 years period (2010 to 2020) in a tertiary referral center (University of Bonn, Bonn, Germany) were retrospectively reviewed for course and outcome. The cut-off value for left ventricular length Z-score >−1, was used, which is in accordance with the recently published data of Tulzer et al (2022), as using these inclusion criteria seems to be of better prognostic significance for biventricular outcome [ 5 ].…”

Section: Methodsmentioning

confidence: 64%

“…Fetal aortic valvuloplasty (FAV) was introduced in the 1990s in order to preserve biventricular circulation in fetuses at risk [ 4 ]. Since then, albeit still low, the success rate of the interventions improved considerably with 37–43% biventricular (BV) outcome and less than 15% procedure related mortality in high volume centers [ 5 , 6 ].…”

Section: Introductionmentioning

confidence: 99%

“…Till now recent literature lists selection criteria based mainly on the clinical experience of the two most experienced centers (Boston vs. Linz group). Selection criteria such as a combination of LV pressure >47 mmHg and ascending aorta Z-score ≥0.57 defined by the Boston working group are confronting selection criteria such as a combination RV/LV length ratio <1.09, or alternatively a RV/LV length ratio of 1.09–1.3 combined with MR Vmax >314 cm/s, established by the Linz working group [ 5 , 6 ].…”

Section: Introductionmentioning

confidence: 99%

“…Although the criteria identifying fetuses with SAS that will progress to HLHS are recognized, the criteria [ 2 ] identifying those left ventricles that will most likely recover to their full biventricular potential are less well defined. Further, even though the long-term outcome of patients with critical aortic stenosis and biventricular circulation is justifiably assumed to be better than that with univentricular palliation, the risks of biventricular correction are achieved by multiple interventions with increased morbidity and mortality must be considered [ 5 ].…”

Section: Introductionmentioning

confidence: 99%

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Intrauterine Valvuloplasty in Severe Aortic Stenosis—A Ten Years Single Center Experience

Walter

Strizek

Weber

et al. 2022

JCM

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Objective: To assess the course and outcome of fetal aortic valvuloplasty (FAV) in fetuses with severe aortic stenosis (SAS) in a single center. Methods: All fetuses with a prenatal diagnosis of SAS with subsequent FAV were retrospectively collected in one tertiary center for fetal medicine over a period of 10 years. In the study, period fetuses with SAS were considered suitable for FAV in the presence of markedly elevated left ventricular pressures (maximum velocity of mitral regurgitation (MR Vmax) >250 cm/s and/or maximum velocity of aortic stenosis (AS Vmax) >250 cm/s), retrograde flow in the transverse aortic arch and a left ventricular length Z-score >−1. Results: In the study period 29 fetuses with AS were treated with 38 FAV. If reinterventions are included 82.7% of fetuses received a technically successful FAV. Procedure related death occurred in three (10.3%) cases, spontaneous fetal death in 2 (6.9%), and termination of pregnancy was performed in 3 cases (10.3%). Among the 21 live births (72.4%), four died in infancy. Among the remaining survivors, 8/17 (47.1%) had a biventricular outcome at the age of one year, 8/17 (47.1%) were univentricular and one infant (5.9%) is biventricular at the age of eight months. Fetuses with biventricular outcome had significantly greater left ventricular (LV) length Z-scores (p = 0.031), and lower tricuspid to mitral valve (TV/MV) ratios (p = 0.003). Conclusions: FAV has a high technical success rate and a low rate of procedure related mortality if performed in experienced hands. The success rate of biventricular circulation at the age of one year is moderate and seems to depend rather on the center’s experience and postnatal surgical strategies than solely on prenatal selection criteria. In the absence of randomized controlled trials, FAV remains an experimental intervention.

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Section: Methodsmentioning

confidence: 64%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Intrauterine Valvuloplasty in Severe Aortic Stenosis—A Ten Years Single Center Experience

Walter

Strizek

Weber

et al. 2022

JCM

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“…This procedure is performed at mid-gestation with the goal of relieving fetal aortic stenosis and preventing progression to HLHS. Current era results from the two largest-volume centers show procedural success of 94–96% and biventricular circulation in 55–59% of liveborns after fetal aortic valvuloplasty [ 26 , 47 ]. However, biventricular circulation after a recent-era fetal aortic valvuloplasty has a probability of transplant-free survival to 6 years (0.82; 95% confidence interval 0.73–0.89) similar to that for patients managed as univentricular hearts (0.72; 95% confidence interval 0.61–0.82) in cases of HLHS due to aortic stenosis and mitral stenosis as reported in the Single Ventricle Reconstruction trial [ 48 ].…”

Section: Surgical Interventions To Treat Hypoplastic Left Heart Syndromementioning

confidence: 99%

Flow-Mediated Factors in the Pathogenesis of Hypoplastic Left Heart Syndrome

Rahman

Chaturvedi

Sled

2022

JCDD

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Hypoplastic left heart syndrome (HLHS) is a life-threatening congenital heart disease that is characterized by severe underdevelopment of left heart structures. Currently, there is no cure, and affected individuals require surgical palliation or cardiac transplantation to survive. Despite these resource-intensive measures, only about half of individuals reach adulthood, often with significant comorbidities such as liver disease and neurodevelopmental disorders. A major barrier in developing effective treatments is that the etiology of HLHS is largely unknown. Here, we discuss how intracardiac blood flow disturbances are an important causal factor in the pathogenesis of impaired left heart growth. Specifically, we highlight results from a recently developed mouse model in which surgically reducing blood flow through the mitral valve after cardiogenesis led to the development of HLHS. In addition, we discuss the role of interventional procedures that are based on improving blood flow through the left heart, such as fetal aortic valvuloplasty. Lastly, using the surgically-induced mouse model, we suggest investigations that can be undertaken to identify the currently unknown biological pathways in left heart growth failure and their associated therapeutic targets.

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Hemodynamic and anatomic changes after fetal aortic valvuloplasty are associated with procedural success and postnatal biventricular circulation

et al. 2022

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Background There are minimal data characterizing the trajectory of left heart growth and hemodynamics following fetal aortic valvuloplasty (FAV). Methods This retrospective study included patients who underwent FAV between 2000 and 2019, with echocardiograms performed pre‐FAV, immediately post‐FAV, and in late gestation. Results Of 118 fetuses undergoing FAV, 106 (90%) underwent technically successful FAV, of which 55 (52%) had biventricular circulation. Technically successful FAV was associated with improved aortic valve growth (p < 0.001), sustained antegrade aortic arch (AoA) flow (p = 0.02), improved mitral valve (MV) inflow pattern (p = 0.002), and favorable patent foramen ovale (PFO) flow pattern (p = 0.004) from pre‐FAV to late gestation. Compared to patients with univentricular outcome, patients with biventricular outcome had less decrement in size of the left ventricle (LV) (p < 0.001) and aortic valve (p = 0.005), as well as more physiologic PFO flow (p < 0.001) and antegrade AoA flow (p < 0.001) from pre‐FAV to late gestation. In multivariable analysis, echocardiographic predictors of biventricular outcome were less decline in LV end diastolic dimension (p < 0.001), improved PFO flow (p = 0.004), and sustained antegrade AoA flow (p = 0.002) from pre‐FAV to late gestation. Conclusion Stabilization of left heart growth and improved hemodynamics following successful FAV through late gestation are associated with postnatal biventricular circulation.

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Valvuloplasty in 103 fetuses with critical aortic stenosis: outcome and new predictors for postnatal circulation

Cited by 33 publications

References 25 publications

Intrauterine Valvuloplasty in Severe Aortic Stenosis—A Ten Years Single Center Experience

Intrauterine Valvuloplasty in Severe Aortic Stenosis—A Ten Years Single Center Experience

Flow-Mediated Factors in the Pathogenesis of Hypoplastic Left Heart Syndrome

Hemodynamic and anatomic changes after fetal aortic valvuloplasty are associated with procedural success and postnatal biventricular circulation

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