Pulmonary hypertension and mitral regurgitation in an infant with an anatomically normal mitral valve.

A full-term, female presented on her date of birth with severe pulmonary hypertension (PH) and mitral regurgitation (MR), requiring veno-arterial extracorporeal membrane oxygenation. After the treatment, her PH and MR were resolved with no anatomic abnormality present. We propose a positive feedback loop of PH causing right ventricular dilation and interventricular septal shifts, worsening MR, and elevated left atrial, and potentially pulmonary, pressures.

Comparison of Semi-Automated Versus Manual Quantitative Right Ventricular Assessment in Hypoplastic Left Heart Syndrome.

Quantitative echocardiographic evaluation is important for systemic right ventricles, but its asymmetric shape makes this challenging and time consuming when performed manually. Semi-automated software could make these quantitative measures easier to accomplish in the clinical setting. We hypothesized that semi-automated software would approximate manual measures of right ventricular size and function. Children with hypoplastic left heart who had echocardiograms were prospectively identified. These measurements were performed using manual and semi-automated techniques: end-diastolic and end-systolic area, fractional area change (FAC), dimensions (longitudinal, basal and mid-cavitary diameters), and tricuspid annular plane systolic excursion (TAPSE). Agreement between measures was evaluated. Sixty-three echocardiograms were analyzed. Intra- and inter-observer reliability was acceptable and similar between methods except that inter-observer reliability for the manual method was superior for TAPSE. Correlation between methods was high (r > 0.9, p < 0.001) for most of the measures. Correlation for FAC was r = 0.79, and for TAPSE the correlation was r = 0.61 (both p < 0.001). The percent relative difference between manual and semi-automated methods was less than 6% for most measures. End-systolic area and FAC had a relative difference of 10% and 11% respectively. The only measure with substantial bias between the manual and semi-automated methods was TAPSE which had a relative difference of 52%. EchoInsight® semi-automated software provides similar measures of right ventricular dimensions and FAC in patients with hypoplastic left heart compared to manual measures. Measures of TAPSE do not correlate well between manual and semi-automated methods. Further research is warranted on the use of semi-automated analyses in this patient population.

Subaortic Stenosis Resection in Children: Emphasis on Recurrence and the Fate of the Aortic Valve.

BACKGROUND: Recurrence after surgical resection of discrete subvalvar aortic stenosis in children often requires repeat operation. Risk factors for recurrence are poorly understood. We sought to determine potential risk factors for recurrence and postoperative comorbidities in the long term. METHODS: Retrospective chart review was performed on all pediatric patients who underwent surgical resection of discrete subaortic stenosis at our institution. Demographics, perioperative findings, and clinical data were analyzed for predisposing factors. RESULTS: From 1991 to 2015, a total of 104 patients underwent primary surgical resection of discrete subaortic stenosis. There were no postoperative deaths. Three (2.9%) patients required pacemaker implantation. Nine (8.4%) patients required repeat resection for recurrence of subaortic membrane over a median follow-up of 8.5 years (interquartile range: 5.9-13.5 years). Actuarial freedom from repeat resection was 100%, 94%, and 82% at one, five, and ten years, respectively. Repeat resection occurred more frequently in patients with genetic disease (37.5% vs 10.7%; P = .033) and preoperative mitral regurgitation (MR; 25% vs 1.2%; P < .001). Postoperative aortic insufficiency (AI) that was moderate or worse was associated with older age at the time of first resection (relative risk [RR]: 1.54, P < .05), moderate or severe preoperative AI (RR: 1.84, P = .002), and repeat resection of subaortic stenosis (RR: 1.90, P < .001). CONCLUSION: The majority of children who undergo surgical resection of subaortic stenosis will not experience recurrence in childhood and those who do require repeat resection may have a higher incidence of genetic disease and preoperative MR. Postoperative AI is associated with repeat resection, older age at the time of surgery, and degree of preoperative AI.