Mechanical tricuspid valve replacement in hypoplastic left heart syndrome: An institutional experience.

Objective: Atrioventricular valvar regurgitation in patients with single ventricles is associated with worse outcomes. Valve repair or replacement has been undertaken in an attempt to reduce mortality and morbidity. Current data on valve replacement in single ventricle patients are limited and derived from heterogenous populations. We describe our experience with repair and replacement of the tricuspid valve in children undergoing single ventricle palliation for hypoplastic left heart syndrome. Methods: We included 27 patients with hypoplastic left heart syndrome with at least moderate tricuspid regurgitation who underwent valve intervention between 2007 and 2021 at our institution; charts were retrospectively reviewed for data. Results: Eleven patients (73% male) underwent valve replacement (median age, 3 years). Preoperative ventricular systolic function was normal in 10 patients (91%). Median follow-up postoperatively was 4 years with no early mortality, 1 (9%) late mortality, and 1 heart transplant (9%). Morbidity consisted of complete heart block in 1 patient (9%), with no important hemorrhagic or thrombotic events. Among survivors of replacement (n = 9), ventricular function was preserved in all (n = 8) who had normal function preoperatively and improved in the remaining patient. Sixteen patients underwent valve repair (median age, 4 months) with no early mortality, 8 (50%) midterm mortalities, and 2 heart transplants (12%). Conclusions: Tricuspid valve replacement is a feasible option in hypoplastic left heart syndrome with significant tricuspid regurgitation, with favorable outcomes in the intermediate follow-up. When undertaken in the setting of normal function, ventricular function may be preserved in up to 80% of patients. Long-term follow-up is needed.

Height Versus Body Surface Area to Normalize Cardiovascular Measurements in Children Using the Pediatric Heart Network Echocardiographic Z-Score Database.

Normalizing cardiovascular measurements for body size allows for comparison among children of different ages and for distinguishing pathologic changes from normal physiologic growth. Because of growing interest to use height for normalization, the aim of this study was to develop height-based normalization models and compare them to body surface area (BSA)-based normalization for aortic and left ventricular (LV) measurements. The study population consisted of healthy, non-obese children between 2 and 18 years of age enrolled in the Pediatric Heart Network Echo Z-Score Project. The echocardiographic study parameters included proximal aortic diameters at 3 locations, LV end-diastolic volume, and LV mass. Using the statistical methodology described in the original project, Z-scores based on height and BSA were determined for the study parameters and tested for any clinically significant relationships with age, sex, race, ethnicity, and body mass index (BMI). Normalization models based on height versus BSA were compared among underweight, normal weight, and overweight (but not obese) children in the study population. Z-scores based on height and BSA were calculated for the 5 study parameters and revealed no clinically significant relationships with age, sex, race, and ethnicity. Normalization based on height resulted in lower Z-scores in the underweight group compared to the overweight group, whereas normalization based on BSA resulted in higher Z-scores in the underweight group compared to the overweight group. In other words, increasing BMI had an opposite effect on height-based Z-scores compared to BSA-based Z-scores. Allometric normalization based on height and BSA for aortic and LV sizes is feasible. However, height-based normalization results in higher cardiovascular Z-scores in heavier children, and BSA-based normalization results in higher cardiovascular Z-scores in lighter children. Further studies are needed to assess the performance of these approaches in obese children with or without cardiac disease.

Unruptured sinus of valsalva aneurysm in a neonate with hypoplastic left heart syndrome: echocardiographic diagnosis and features.

An unruptured sinus of Valsalva aneurysm is usually asymptomatic, and a rare, often incidental echocardiographic diagnosis. Its presentation in the neonatal period is extremely rare. We describe this finding in a neonate with hypoplastic left heart syndrome, which has not been previously reported in the literature. This constellation of findings may have important clinical implications in regards to medical management and surgical options.

Anomalous left main coronary artery origin from the right sinus of Valsalva: a novel echocardiographic screening method.

Anomalous origin of the left main coronary artery from the right sinus (ALMCA) is a rare entity associated with an increased risk for sudden cardiac death. Although transthoracic echocardiography often is the primary screening tool for the diagnosis, its accuracy remains undefined, resulting in the use of more expensive methods such as cardiac catheterization and magnetic resonance imaging when clinical suspicion exists. This report aims to describe a novel echocardiographic screening method for detecting ALMCA and to apply it retrospectively for patients with confirmed findings intraoperatively and/or on autopsy. All patients with a confirmed diagnosis of ALMCA who presented to our institution from 2004 to 2012 were included in the study. Eight patients had adequate images, including two patients with an initial diagnosis of normal coronary origins. A control group consisted of 35 patients with normal coronaries shown by echocardiogram. Five of these patients had normal coronary artery origins confirmed by magnetic resonance imaging (MRI). Echocardiographic images were reviewed, and the left main coronary was identified in the parasternal long-axis view between the aorta and pulmonary trunk. The angle of the left main coronary course was measured. In patients with ALMCA, the proximal course of the vessel was steeper as it coursed posteriorly. In contrast, the course was almost horizontal in patients with normal coronary origins. Based on these findings, a cutoff angle of 28° is proposed. In the parasternal long-axis view, ALMCA can be identified by its anomalous proximal course. This screening method is reliable and increases the accuracy of transthoracic echocardiograms.