Search

Background: Critical aortic stenosis (AS) in fetuses may progress to hypoplastic left heart syndrome (HLHS) with need for postnatal single ventricular (SV) palliation. Fetal aortic valvuloplasty (FAV) is performed to achieve postnatal biventricular (BV) circulation. However, the impact of FAV on fetal myocardial function is difficult to measure. Prediction of postnatal circulatory status and, therefore, counseling is challenging. Methods: Retrospective study of fetuses with critical AS who underwent FAV. Global Longitudinal Peak Systolic Strain (GLPSS) of the left ventricle (LV) and right ventricle (RV) were retrospectively analyzed before and after intervention. Fisher's Exact Test and Mann–Whitney-U Test were used for univariant statistical analysis. Results: 23 fetuses with critical AS were included. After intervention fetuses demonstrated more negative LV-GLPSS mean values post- vs. pre-intervention (− 5.36% vs. − 1.57%; p < 0.05). RV-GLPSS was decreased in all fetuses, there was no peri-interventional change. 20 fetuses were born alive. Postnatally, 10 had BV and 10 SV circulation. Improved post-interventional LV-GLPSS strain values correlated with BV outcome (p < 0.05). Pre-interventional continuous LV-GLPSS values correlated with postnatal SV vs. BV outcome (p < 0.05). Conclusion: In some fetuses, LV myocardial function assessed by speckle tracking echocardiography (STE) improves after FAV. Improved post-interventional LV-GLPSS correlates with biventricular postnatal outcome. Furthermore, pre-interventional LV- and RV-GLPSS correlate with postnatal outcome. Further studies are needed to asses, if pre-interventional STE parameters might predict which fetuses will benefit from FAV with postnatal BV circulation. [ABSTRACT FROM AUTHOR]

Critical aortic stenosis (AS) of the fetal heart causes a drastic change in the cardiac biomechanical environment. Consequently, a substantial proportion of such cases will lead to a single-ventricular birth outcome. However, the biomechanics of the disease is not well understood. To address this, we performed Finite Element (FE) modelling of the healthy fetal left ventricle (LV) based on patient-specific 4D ultrasound imaging, and simulated various disease features observed in clinical fetal AS to understand their biomechanical impact. These features included aortic stenosis, mitral regurgitation (MR) and LV hypertrophy, reduced contractility, and increased myocardial stiffness. AS was found to elevate LV pressures and myocardial stresses, and depending on severity, can drastically decrease stroke volume and myocardial strains. These effects are moderated by MR. AS alone did not lead to MR velocities above 3 m/s unless LV hypertrophy was included, suggesting that hypertrophy may be involved in clinical cases with high MR velocities. LV hypertrophy substantially elevated LV pressure, valve flow velocities and stroke volume, while reducing LV contractility resulted in diminished LV pressure, stroke volume and wall strains. Typical extent of hypertrophy during fetal AS in the clinic, however, led to excessive LV pressure and valve velocity in the FE model, suggesting that reduced contractility is typically associated with hypertrophy. Increased LV passive stiffness, which might represent fibroelastosis, was found to have minimal impact on LV pressures, stroke volume, and wall strain. This suggested that fibroelastosis could be a by-product of the disease progression and does not significantly impede cardiac function. Our study demonstrates that FE modelling is a valuable tool for elucidating the biomechanics of congenital heart disease and can calculate parameters which are difficult to measure, such as intraventricular pressure and myocardial stresses. [ABSTRACT FROM AUTHOR]

Objective Valvuloplasty of the fetal aortic valve has the potential to prevent progression of critical aortic stenosis (AS) to hypoplastic left heart syndrome (HLHS). The aim of the study was to assess 24 aortic valvuloplasties regarding indications, success rate, procedure-related risks and outcome. Methods Between January 2001 and December 2009 we performed 24 aortic valvuloplasties in 23 fetuses with critical AS at a median gestational age of 26 + 4 (range, 21 + 3 to 32 + 5) weeks by a transabdominal ultrasoundguided approach. Four fetuses had hydrops as a late sign of heart failure. Results In 16/24 procedures (66.7%) corresponding to 16/23 fetuses (69.6%) the procedures were technically successful, with one intrauterine death in this group. After an initial learning curve, success rate improved to 78.6% (11 of the last 14 interventions were successful). In 10 out of the 15 (66.7%) successfully-treated and liveborn fetuses a biventricular circulation could be achieved postnatally. All four fetuses with hydrops had successful interventions, hydrops disappearing within 5 weeks. In 8/24 interventions (33.3%) the aortic valve could not be treated successfully, with intrauterine fetal death in two of these cases. In one fetus a repeat procedure was successful. All surviving fetuses with unsuccessful (n = 5) or no (n = 5) procedure performed developed HLHS until delivery. Conclusions Fetal aortic valvuloplasty could be performed successfully in selected fetuses with critical AS and evolving HLHS, with a biventricular outcome in two thirds of the patients. Safety and success rate were dependent on patient selection and the level of experience of the whole interventional team. In fetuses with AS and hydrops, aortic valvuloplasty could reverse end-stage heart failure and hydrops and ensure fetal survival. [ABSTRACT FROM AUTHOR]

Fetal hydrops of various etiologies can be associated with maternal mirror syndrome. The exact pathophysiology of this rare disorder remains unclear, yet it can result in significant maternal and fetal morbidity. The recent advances in prenatal diagnosis and therapeutic interventions have focused on altering the course of pathologies associated with an expected poor prognosis. Severe fetal aortic valve stenosis with its potential for hypoplastic left heart fit this category. We report a case of maternal mirror syndrome in a parturient following intrauterine therapy for severe fetal aortic stenosis and hydrops. Despite a technically successful intervention, the mother required emergency cesarean section. We review the few reports of mirror syndrome as well as the current approaches to fetal aortic stenosis. This case reinforces the need for in depth risk analysis prior to fetal interventions. It also emphasizes the advantage of earlier detection and therapy with fetal aortic stenosis since fetal hydrops carries a somber prognosis. Copyright © 2006 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]