Mid-Systolic Notching of the Pulmonary Valve Doppler Signal is Highly Associated with Pulmonary Hypertension.

Mid-systolic notching (MSN) of the pulmonary valve Doppler signal represents a reflected systolic pressure wave from the pulmonary vasculature and is often seen in pulmonary hypertension (PH). We hypothesize that MSN is associated with a higher pulmonary vascular resistance (PVR) and mean pulmonary artery pressure (mPAP), and a diagnosis of PH in pediatric patients. This was a retrospective study of patients ≤ 18 years who had an echocardiogram obtained ≤ 30 days before catheterization for suspected PH. MSN was defined as an indentation in the initial two thirds of the systolic Doppler signal. PH was defined as mPAP > 20 mmHg and PVR ≥ 3.0 Wu  m2. Subgroups (MSN vs. normal) were compared. Receiver operator characteristic determined a continuous variable's discriminatory ability for a diagnosis of PH. Reproducibility of MSN was assessed. In total, 90 patients (73 with congenital heart disease) were included, of which 36 had MSN and 54 were normal. MSN patients were more likely to have PH, and had significantly higher mPAP, PVR, and lower pulmonary stroke volume. The presence of MSN had good discriminatory ability for PH diagnosis. The presence of MSN had high specificity (96%) for PH, whereas sensitivity was lower (54%). Reproducibility was 100% for MSN. MSN is a simple, highly reproducible echocardiographic metric associated with higher mPAP and PVR. When present, there is a high likelihood a diagnosis of PH confirmed by catheterization. Incorporation of MSN into imaging protocols may be useful. MSN appears worthy of further investigation in pediatric patients with suspected PH.

Aortic Root Distortion During Balloon Angioplasty of Right Ventricular Outflow Tract Prior to Transcatheter Pulmonary Valve Implantation.

We describe our experience with aortic root distortion in transcatheter pulmonary valve implantation (TPVI). Aortic root distortion (AD) can be observed with balloon angioplasty of the right ventricular outflow tract (RVOT), but its long-term significance is unknown. This has been a common finding in our institution, though not fully appreciated in our early experience. Retrospective review of procedural angiograms prior to TPVI and follow up imaging was performed. Between June 2012 and October 2017, 47 patients underwent catheterization to attempt TPVI. Five patients had coronary compression which precluded TPVI (one with significant AD as well). Four patients had significant AD and did not receive TPVI. Of the remaining 38 successful TPVI, 20 had adequate imaging to assess the aortic root. Four patients had severe AD, 7 had mild AD, and 9 with no AD. Severity of AI did not correlate with degree of AD. Median follow up after TPVI was 46 months (IQR 21-67). Of the 4 patients with severe AD who received TPVI, 1 has new mild AI with 78 months follow up. Of the 18 patients who received TPVI without adequate arch imaging, 2 patients have new mild AI with 86 and 75 months follow up. AD during RVOT angioplasty is a relatively common finding. In our early experience, some patients who were retrospectively identified to have severe AD received TPVI. These patients have done well, though further data is needed before considering severe AD a benign finding.

Melody valve implantation within freestyle stentless porcine aortic heterograft.

BACKGROUND: The Melody transcatheter pulmonary valve has been implanted successfully worldwide since its first implant in 2000. The vast majority of these valves have been implanted in pulmonary homografts. In our institution, the most common valve used for pulmonary valve replacement is the Medtronic Freestyle stentless porcine aortic heterograft. OBJECTIVE: We describe our experience implanting the Melody valve within the Freestyle heterograft. METHODS: Retrospective chart review was performed. RESULTS: Between June 2012 and June 2015, 19 Melody valves were placed within Freestyle heterografts. The most common indication for intervention was pulmonary stenosis. Following pre-stent and Melody valve implantation, right ventricle-to-pulmonary artery gradient decreased from 38.1 ± 12.1 to 10 ± 4.7 mm Hg (P < 0.001), and right ventricular pressure decreased from 61.7 ± 17.8 to 35.6 ± 10.2 mm Hg (P < 0.001). Two procedural adverse events occurred. At median follow-up of 24 months (range 2-48 months), no patients had mean right ventricular outflow tract gradients >30 mm Hg or worse than mild insufficiency. No valve reinterventions have been necessary and no episodes of endocarditis have been observed. CONCLUSIONS: The Melody valve can be implanted successfully within a stentless aortic bioprosthesis with good short- and intermediate-term longevity. © 2016 Wiley Periodicals, Inc.

Care of children who have had surgery for congenital heart disease.

Children who have had surgical correction for congenital heart disease can present to the ED with an acute illness that could be associated with their cardiac lesion. There is no data available to summarize complications that could be associated with surgically corrected congenital heart disease. This work was undertaken to describe the common procedures used, list known complications of these procedures, and review general management principles in caring for the acutely ill child who has had heart surgery.