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Methods CMR scans of patients with Noonan’s syndrome referred for imaging were analyzed. All patients were imaged on a 1.5T MRI. RV volumes were generated from SSFP cines and normalized to gender, body surface area and age. Phase contrast velocity mapping was used to assess pulmonary and aortic flow. The prevalence of concomitant cardiovascular abnormalities was also assessed. Results Thirty two consecutive patients (age 29 (20 - 50), (median (IQR); male 69%) were studied. Prior intervention for MPA or PV stenosis was performed in 15 (47%) patients and data was compared to 17 (53%) Noonan’ sp atients without MPA or PV intervention. Pulmonary regurgitation was more prevalent (73% vs. 33%, c 2 = 8.4; p = 0.037) and severe (regurgitant fraction 26.4±5% (mean ± SEM) vs. 6.5 ± 3%; p<0.01) in the prior intervention group. Although indexed RV end-diastolic volumes were also greater (128 ±16 mL/m 2 vs. 91 ± 10 mL/m 2 ; p=0.04), no difference was observed in RVESV, RVEF or wall thickness, nor right atrial area (all, p=0.3). Mild to moderate PV stenosis was seen in 5 (33%) with prior intervention and 4 (24%) without. Other abnormalities included tricuspid dysplasia (n=1, 3%), anomalous pulmonary venous drainage (n=2, 6%), aortic coarctation (n=1, 3%), aortic root dilatation (n=1, 3%), myxomatous mitral valve (n=2, 6%), evidence of previous ASD repair (n=8, 25%), VSD repair (n=3, 9%) and AVSD repair (n=1,3%). Conclusions CMR-determined moderate and severe pulmonary regurgitation is frequent in patients with Noonan’ ss yndrome after pulmonary intervention. RV function remains well preserved despite increased volume load. CMR, an ideal imaging modality for the assessment of pulmonary valve and RV function also allows detection of associated cardiac abnormalities in Noonan’s syndrome.

Right ventricular morphology and function in patients with Noonan’s syndrome after pulmonary intervention