Left heart hypoplasia in the fetus: Echocardiographic predictors of outcome.

INTRODUCTION: Fetal left heart hypoplasia (LHH) with an apex-forming left ventricle may require neonatal intervention but it is difficult to predict. METHODS: We performed a retrospective study of fetuses with LHH defined as normal segmental anatomy, apex-/near-apex forming left ventricle, and ≥1 left-sided z-score ≤ -2 between 1997 and 2014. Fetuses with mitral or aortic atresia, critical aortic stenosis, extracardiac anomalies, or fetal intervention were excluded. Classification and regression tree analyses (CART) were performed to construct algorithms to predict postnatal circulation: no surgery versus biventricular surgery versus single ventricle (SV) palliation. RESULTS: Among 138 included fetuses, 52 (37%) underwent neonatal surgery, with 10 (7%) undergoing SV palliation. The strongest single outcome discriminator was exclusively left-to-right flow foramen ovale (FO) flow ≥32 weeks gestational age (GA) (seen in 0% with no surgery, 22% with biventricular surgery, 88% with SV palliation). On CART analysis >32 weeks GA, fetuses with right-to-left FO flow and aortopulmonary ratio >0.76 had 0% probability of neonatal surgery, while those with left-to-right FO flow and mitral valve z-score < -3.6 had a 70% probability of SV palliation. CONCLUSION: SV palliation is an uncommon outcome of fetal LHH. Fetal FO flow and other echocardiographic measures can help determine risk and type of postnatal intervention.

Echocardiographic Parameters and Outcomes in Primary Fetal Cardiomyopathy.

OBJECTIVES: Primary fetal cardiomyopathy is a rare entity, with a poor prognosis. We sought to describe its echocardiographic characteristics and outcomes. METHODS: We performed a 12-year retrospective review of fetuses with primary cardiomyopathy. RESULTS: Of more than 6000 fetuses evaluated, 25 met criteria for primary cardiomyopathy, and 18 had sufficient echocardiographic and pregnancy outcome data for inclusion. At echocardiography, the median gestational age was 29.6 weeks (range, 21.0-36.4 weeks); median cardiovascular profile score was 6 (range, 1-9); median right ventricular Tei index was 0.52 (range, 0.32-0.94); and median left ventricular Tei index was 0.40 (range, 0.15-0.88). Two had fetal demise, and 16 survived to delivery. The median cardiovascular profile score in those with fetal demise was 3.0 and in those who survived to delivery was 6.5 (range, 3-9; P = .14). The median right ventricular Tei index in those with fetal demise was 0.39 and in those surviving to delivery was 0.53 (range, 0.38-0.94; P = .49). The median left ventricular Tei index in those with fetal demise was 0.29 and in those surviving to delivery was 0.42 (range, 0.15-0.88; P = .50). Sixty-day survival was available in 11 of 16 fetuses. In addition to the 2 with fetal demise, 4 had postnatal demise, and 5 were alive at a median follow-up of 39 months. Hydrops (P = .01), skin edema (P = .01), and mild or greater mitral regurgitation (P = .02) were associated with fetal or postnatal demise, with a trend toward an association between moderate or greater tricuspid regurgitation (P = .07) and fetal or postnatal demise. CONCLUSIONS: Hydrops and atrioventricular valvar regurgitation are ominous signs in primary fetal cardiomyopathy. Although other commonly used methods for assessing cardiovascular performance may help in diagnosing primary cardiomyopathy, these data suggest limited predictive value.

Diastolic velocity half time is associated with aortic coarctation gradient at catheterization independent of echocardiographic and clinical blood pressure gradients.

OBJECTIVE: The most accurate noninvasive parameter to predict whether a patient with aortic coarctation will meet interventional criteria at catheterization remains elusive. We aim to determine the best independent echocardiographic predictors of a coarctation peak-to-peak pressure gradient ≥20 mm Hg at catheterization, the accepted threshold for intervention. DESIGN: Retrospective query of our catheterization database from 1/2007 to 7/2016 for the diagnostic code of aortic coarctation was performed. Multiple echocardiographic measurements and blood pressure gradients prior to cardiac catheterization were collected. Univariate correlation of variables with the continuous catheterization peak were calculated using Spearman's rho. Univariate association with peak-to-peak gradient at catheterization ≥20 mm Hg was tested using Mann-Whitney U test and the Pearson chi-square test or Fisher's exact test. Multivariable logistic regression assessed the independent association of the clinically relevant metrics with gradient at catheterization ≥20 mm Hg. RESULTS: Sixty-eight patients met study criteria (median age 9.25 years), of whom 84% underwent intervention at catheterization. Echocardiographic peak and mean coarctation velocity, indexed systolic and diastolic velocity half times (SVHTi, DVHTi), and blood pressure gradient all had moderate correlation (Spearman's rho = 0.529-0.617, P < .001) with the continuous catheterization gradient and were significantly associated with the binary outcome of catheterization peak ≥20 mm Hg (P < .001). Logistic regression found echocardiographic mean systolic gradient (OR 1.213 [95% CI 1.041-1.414]) and DVHTi (OR 1.039 [95% CI 1.004-1.074]) independently associate with catheterization peak ≥20 mm Hg after controlling for blood pressure gradient (OR 1.066 [0.987-1.150]). CONCLUSIONS: Most echocardiographic estimates show moderate correlation with arch gradient at catheterization. Noninvasive four extremity blood pressure gradient is significantly associated with peak-to-peak gradient ≥20 mm Hg. DVHTi may provide a unique independently associated echocardiographic estimate of coarctation severity. Further study of these variables with larger cohorts may allow for development of predictive models to direct catheterization.

Tissue Doppler Imaging Measures Correlate Poorly with Left Ventricular Filling Pressures in Pediatric Cardiomyopathy.

SETTING: In adults with cardiomyopathy, tissue Doppler imaging (TDI) has been shown to correlate with left ventricular filling pressures (LVFPs) and has been advocated as a surrogate to catheterization. However, this has not been validated in children. DESIGN: This is a retrospective review of children ≤18 years old with dilated, hypertrophic, restrictive, and left ventricular noncompaction cardiomyopathy who underwent cardiac catheterization within 3 months of an echocardiogram. Spearman's correlation coefficients were calculated to assess a correlation between LVFP and mitral inflow E/A ratio, lateral mitral E/E', and septal E/E'. RESULTS: Thirty-eight patients were included in the study; median age was 8.6 years old. The median LVFP was 19 mm Hg, median mean pulmonary artery pressure was 25 mm Hg, and median pulmonary vascular resistance index (PVRi) was 2.4 Wu. There was no significant correlation between LVFP or PVRi with lateral mitral E/E' or septal E/E'. There was a positive correlation between LVFP and mitral inflow E/A ratio (rs = 0.59, P < .01). In a subgroup analysis of patients with hypertrophic or restrictive cardiomyopathy, there was a negative correlation (rs = 0.56, P = .02) between the mean pulmonary artery pressure and septal A'. CONCLUSIONS: TDI measures of diastolic function are not reliable surrogates for LVFP, mean pulmonary artery pressures, and PVRi at catheterization in children.

Pediatric ventricular assist device use as a bridge to transplantation does not affect long-term quality of life.

OBJECTIVE: The present study sought to determine the long-term quality of life (QOL) of children who required long-term ventricular assist device (VAD) support as a bridge to transplantation (BTT) compared with children who underwent heart transplantation without VAD support. Currently, 20% of children undergoing heart transplantation have required a VAD as a BTT. Few data have been published assessing how children requiring a VAD as a BTT will fair in terms of their long-term QOL. METHODS: The present study used a cross-sectional design, using the Core and Cardiac modules of the Pediatric Quality of Life Inventory survey. In a secondary analysis, the factors associated with worse QOL outcomes among the VAD patients were also investigated. RESULTS: At follow-up (median, 4.2 years), between the 21 children who required a VAD as a BTT and 42 who went straight to transplantation, no significant differences were found in the QOL as measured using the Psychosocial Health Summary Score, Physical Health Summary Score, or Total Score in the survey's Core Module, nor were any differences found in the outcomes assessed using the survey's Cardiac Module. Of the patients who required a VAD, only the presence of a neurologic complication was associated with worse QOL, which was demonstrated by decreased Physical Health Summary and Cardiac Communication scores. CONCLUSIONS: Over the long term, surviving children who required a long-term VAD as a BTT experience a similar QOL as those who went straight to transplantation.

Fixed subaortic stenosis: a clinical dilemma for clinicians and patients.

Subaortic stenosis carries considerable morbidity and mortality. In most cases, patients have an underlying left ventricular outflow tract morphology that promotes turbulence at the outflow tract, which induces the development of subaortic fibromuscular tissue. A subset of patients will progress to develop severe stenosis and aortic regurgitation, but it has been difficult to determine which patients are at risk. While resection of the subaortic tissue improves immediate outcome, many patients have recurrence of both stenosis and regurgitation, questioning the efficacy of surgical intervention in asymptomatic patients. This review article describes the current understanding of the etiology, treatment, and prognosis of subaortic stenosis.