Mid-term Outcome of 100 Consecutive Ross Procedures: Excellent Survival, But Yet to Be a Cure.

The Ross procedure offers excellent short-term outcome but the long-term durability is under debate. Reinterventions and follow-up of 100 consecutive patients undergoing Ross Procedure at our centre (1993-2011) were analysed. Follow-up was available for 96 patients (97%) with a median duration of 5.3 (0.1-17.1) years. Median age of the patient cohort was 15.2 (0.04-58.4) years with 76 males. 93% had underlying congenital aortic stenosis. Root replacement technique was applied in all. The most common valved conduits used for reconstruction of the right ventricular outflow tract were homografts (66 patients) and bovine jugular vein (ContegraR) graft (31 patients). Additional procedures included Ross-Konno procedure (14%), resection of subaortic stenosis/myectomy (11%) and reduction plasty of the ascending aorta (25%). One patient died within the first 30 days (1%). Late deaths occurred in 4 patients (4%) 0.5-4.5 years postoperatively: causes included pulmonary hypertension due to endocardial fibroelastosis (2), subarachnoid haemorrhage (1) and sudden cardiac death (1). Five-year survival was 93.6 (95% CI 88.1-99.1)%. Moderate or severe aortic (autograft) regurgitation needing reoperation occurred in 8 patients with a 5-year freedom from autograft reoperation of 98.5 (95.6-100)%. Five-year freedom from reintervention (surgery or catheter based) on the right ventricular outflow tract conduit was 91.5 (85.5-96.5)%. Univariate predictors of this reinterventions were smaller graft size (p = 0.03) and use of a ContegraR graft (p = 0.04). Ross procedure can be performed with low mortality and good survival in the long term. Most of the reinterventions are related to the neo-right ventricular outflow tract and may be partly attributed to the lack of growth. While the Ross Procedure remains an invaluable option for aortic valve disease in children, new solutions for the neo-pulmonary valve as well as for the less often occurring problems on the autograft are needed.

Signal Thresholding Segmentation of Ventricular Volumes in Young Patients with Various Diseases-Can We Trust the Numbers?

In many cardiac diseases, right and left ventricular volumes in systole and diastole are diagnostically and prognostically relevant. Measurements are made by segmentation of the myocardial borders on cardiac magnetic resonance (CMR) images. Automatic detection of myocardial contours is possible by signal thresholding techniques, but must be validated before use in clinical settings. Biventricular volumes were measured in end-diastole (EDVi) and in end-systole (ESVi) both manually and with the MassK application, with signal thresholds at 30%, 50%, and 70%. Stroke volumes (SV) and cardiac indices (CI) were calculated from volumetric measurements and from flow measured in the ascending aorta and the main pulmonary artery, and both methods were compared. Reproducibility of volumetric measurements was tested in 20 patients. Measurements were acquired in 94 patients aged 15 ± 9 years referred for various conditions. EDVi and ESVi of both ventricles were largest with manual segmentation and inversely proportional to the MassK threshold. Manual and k30 SV and CI corresponded best to flow measurements. Interobserver variability was low for all volumes manually and with MassK. In conclusion, manual and 30% threshold-based biventricular volume segmentation agree best with two-dimensional, phantom-corrected phase contrast flow measurements in a young cardiac referral population and are well reproducible.

Possible effects of left pulmonary artery stenting in single ventricle patients on bronchial area, lung volume and lung function.

Background: Left pulmonary artery (LPA) stenting is often required in single ventricle (SV) patients. Due to their close anatomical relationship an LPA stent could potentially compress the left main bronchus (LMB). We assessed the impact of LPA stenting on bronchial size, pulmonary volumes, and lung function in a cohort of SV patients. Methods: Forty-nine patients underwent cardiovascular magnetic resonance (CMR) and 36 spirometry 11 (8-15) years after Fontan. All patients were free of respiratory symptoms. LPA stents were inserted in 17 (35%) patients at 8.8 (3.4-12.6) years. Area/shape of the main bronchi (n = 46) and lung volumes (n = 47) were calculated from CMR-ZTE images for each lung and transformed in right-to-left (r/l) ratio and indexed for BSA. The effect of early stent insertion (prior to stage III) was analyzed. Results: Patients with LPA stent had larger r/l ratio for main bronchus area (p < 0.001) and r/l ratio difference for lung volumes was slightly larger in patients with early stenting. A trend toward a deformation of LMB shape in patients with LPA stent and toward a higher prevalence of abnormal spirometry in patients with early stent implantation was observed. Conclusions: In this cohort of patients, early insertion of LPA stents seems to relate with smaller LMB sizes and a trend toward smaller left lung volume and higher prevalence of impaired lung function. Whether these findings are caused by the stent or, at least to a certain degree, present prior to the implantation needs to be verified.

Prognostic value of troponin in infants with hypoplastic left heart syndrome between Stage I and II of palliation.

BACKGROUND: The period between stage I and II procedure for treatment of hypoplastic left heart syndrome (HLHS) bears high mortality and morbidity. METHODS: We sought to analyze the prognostic value of Troponin T/I (Trop), a well-recognized marker for myocardial damage and heart failure, for predicting outcome in a retrospective analysis of 70 infants with HLHS at our institution between March 2001 and October 2014. RESULTS: Stage I procedure consisted of Norwood I operation in 35 (50%) and Hybrid-approach in 22 (31%) patients. Palliative care was chosen for 13 (19%) patients. Trop values were collected from clinical charts and were analyzed in relation to the overall outcome. Trop was significantly higher after Norwood I operation in comparison to Hybrid-approach (median 7.1 µg/l (0.7-20.9), vs 1.2 µg/l (0.3-17.9), P < 0.001). Overall mortality of treated patients was 39% (22 patients). Survival was 54% (19 patients) after Norwood and 73% (16 patients) after Hybrid-approach. Independently from the procedure used, maximal Trop and initial lactate values were significantly higher in non-survivors than in survivors, with median Trop of 9 µg/l (0.6-18.8) vs. 3.4 µg/l (0.4-20.9), P 0.007, and median lactate of 3.7 mmol/L (1.6-25) vs. 2.9 mmol/L (0.3-14.6), p 0.03. Reinterventions were required in 17 (30%) patients, 4 (11%) after Norwood and 13 (59%) after Hybrid procedure. No correlation was found between the need for reintervention and Trop levels in the interstage period. CONCLUSIONS: Patients with HLHS have significantly higher Trop levels after Norwood procedure than after Hybrid-approach. Maximal Trop values were related to mortality, but did not correlate with the need for reinterventions.

Contrast-enhanced magnetic resonance angiography of the great arteries in patients with congenital heart disease: an accurate tool for planning catheter-guided interventions.

BACKGROUND: Catheter-guided interventions are increasingly used for relief of lesions in patients with congenital heart disease. Exact anatomical imaging with measurement of the vascular structures is crucial in the planning of such interventions. This can be provided non-invasively and without radiation by contrast-enhanced MR angiography (CE-MRA). AIM: To evaluate the accuracy of the measurements of the vessels obtained by CE-MRA in comparison to those obtained by conventional X-ray angiography (XRA). METHODS: Measurements of the diameters of aorta and pulmonary arteries were performed retrospectively and blinded on the CE-MRA and XRA images, in comparable locations. The limits of agreement between the two methods were calculated. RESULTS: Twenty-one CE-MRA and XRA were performed in 20 children with congenital heart disease, median age 4 years (1 day-13 years), weight 18 kg (3.2-74 kg). The time interval between CE-MRA and XRA was 2.6 +/- 2.3 months. A total of 98 measurements, 38 of the aorta and 60 of the pulmonary arteries were performed on the images obtained by each technique. The correlation between CE-MRA and XRA measurements was excellent, r = 0.97, p < 0.0001. The mean difference between the two techniques was 0.018 +/- 1.1 mm; the limits of agreement -2.14 and + 2.18 mm. Similar agreement was found for measures of the aorta (r = 0.97, mean difference 0.20 +/- 1.08 mm) and of the pulmonary arteries (r = 0.97, mean difference 0.048 +/- 0.89 mm). CONCLUSIONS: CE-MRA provides accurate quantitative anatomical information, which highly agrees with XRA data, and can therefore be used for planning catheter-guided procedures.