Predictors of Valve Failure Following Surgical Atrioventricular Valve Replacement with a Melody Valve in Infants and Children.

Options for atrioventricular (AV) valve replacement in small pediatric patients are very limited. The Melody valve has shown reasonable short-term outcomes. This study was aimed at identifying predictors of valve failure following AV valve replacement with a Melody valve at a single-center. 26 patients underwent 37 AV valve replacements with 31/37 (84%) of valves placed in the systemic AV valve position. Median age at procedure was 17 months (IQR 4-33) and weight was 8.5 kg (IQR 6.25-12.85). Median balloon size for valve implant was 20 mm (IQR 18-22). Repeat intervention occurred in 21 cases (57%) with repeat surgery in all but one. Median freedom from re-intervention was 31 months; 19% were free from re-intervention at 60 months. Age < 12 months weight < 10 kg and BSA < 0.4 m2 were all significant risk factors for early valve failure (p = 0.003, p 0.017, p 0.025, respectively). Valve longevity was greatest with balloon inflation to diameter 1.20-1.35 times the patient's expected annular diameter (Z0), relative to both smaller or larger balloons (p = 0.038). In patients less than 12 months of age, patients with single ventricle physiology had an increased risk of early valve failure (p = 0.004).

Usefulness of TI-scout images in the assessment of late gadolinium enhancement in children.

BACKGROUND: Cardiovascular magnetic resonance (CMR) late gadolinium enhancement (LGE) requires identification of the normal myocardial nulling time using inversion time (TI)-scout imaging sequence. Although TI-scout images are not primarily used for myocardial assessment, they provide information regarding different signal recovery patterns of normal and abnormal myocardium facilitating identification of LGE in instances where standard LGE images alone are not diagnostic. We aimed to assess the diagnostic performance of TI-scout as compared to that of standard LGE images. METHODS: CMR studies with LGE imaging in 519 patients (345 males, 1-17 years) were reviewed to assess the diagnostic performance of LGE imaging in terms of the location of LGE and the pathologic entities. The diagnostic performance of the TI-scout and standard LGE imaging was classified into four categories: (1) equally diagnostic, (2) TI-scout superior to standard LGE, (3) standard LGE superior to TI-scout, and (4) complementary, by the consensus of the two observers. RESULTS: The study cohort consisted of 440 patients with negative LGE and 79 with evidence for LGE. For a negative diagnosis of LGE, TI-scout and standard LGE images were equally diagnostic in 75% of the cases and were complementary in 12%. For patients with LGE, TI-scout images were superior to standard LGE images in 52% of the cases and were complementary in 19%. The diagnostic performance of TI-scout images was superior to that of standard LGE images in all locations. TI-scout images were superior to standard LGE images in 11 of 12 (92%) cases with LGE involving the papillary muscles, in 7 /12 (58%) cases with subendocardial LGE, and in 4/7 (57%) cases with transmural LGE. TI-scout images were particularly useful assessing the presence and extent of LGE in hypertrophic cardiomyopathy (HCM). TI-scout was superior to standard LGE in 6/10 (60%) and was complementary in 3/10 (30%) of the positive cases with HCM. CONCLUSIONS: TI-scout images enhance the diagnostic performance of LGE imaging in children.

Can Abbreviated Cardiac Magnetic Resonance Imaging Adequately Support Clinical Decision Making After Repair of Tetralogy of Fallot?

Quantification of pulmonary regurgitation (PR), pulmonary flow distribution, and ventricular function is important for clinical surveillance in repaired Tetralogy of Fallot (TOF). Cardiovascular magnetic resonance (CMR) is the established reference, but cost, test duration, and patient discomfort are potential limitations to its serial use. We investigated whether an Abbreviated CMR protocol would alter clinical decisions in TOF from those that would have been made using a full protocol. Patients > 7 years with repaired TOF were identified. CMR was performed according to standard complete imaging protocol. CMRs were prepared in two ways, Full and Abbreviated and submitted for review by two imaging specialists. In conjunction with clinical information and case-specific quantitative CMR data (PR fraction, ventricular volumes, ejection fraction, branch pulmonary artery flow), Full and Abbreviated image sets were anonymized and uploaded for review. For the first half, Imager 1 received Abbreviated, and Imager 2 Full and for the remaining, Imager 1 received Full and Imager 2 received Abbreviated. Blinded to the other's choices, Imagers provided clinical decisions. Inter-rater agreement for each decision was measured. In all, 124 studies from 80 patients (mean 17.8 years) were analyzed. For 'intervention versus no-intervention' decision, the inter-rater agreement was strong [κ 0.75, p < 0.0001, 95% CI (0.630, 0.869)]. Agreement for recommended timing of follow-up imaging was good (κ 0.64, p < 0.0001, 95% CI (0.474, 0.811)] in the 'no-intervention' group. When raters were asked whether or not further imaging was necessary, agreement was modest [κ 0.363 (p < 0.0001), 95% CI (0.038, 0.687)]. In conclusion, Abbreviated CMR yield decisions for clinical care similar to those made using the standard full protocol. These results suggest a potential enhancement of clinical practice in which efficiency and cost saving might be achieved using Abbreviated CMR for routine follow-up surveillance of TOF.

Impact of clinical follow-up and diagnostic testing on intervention for tetralogy of Fallot.

OBJECTIVE: Our purpose was to evaluate yield of tools commonly advocated for surveillance of tetralogy of Fallot (TOF). METHODS: All patients (pts) with TOF, seen at any time from 1/2008 to 9/2013 in an academic cardiology practice were studied. At the first and each subsequent outpatient visit, the use of tools including history and physical (H&P), ECG, Holter (HOL), echocardiogram (Echo), MR or CT (MR-CT) and stress testing (STR) were noted. Recommendations for intervention (INT) and for time to next follow-up were recorded; rationale for each INT with attribution to one or more tools was identified. RESULTS: There were 213 pts (mean 11.5 years, 130 male) who had 916 visits, 123 of which (13.4%) were associated with 138 INTs (47 surgical, 54 catheter-mediated, 37 other medical). Recommended follow-up interval was 9.44±6.5 months, actual mean follow-up interval was 11.7 months. All 916 (100%) patient visits had a H&P which contributed to 47.2% of INT decisions. Echo was performed in 652 (71.2%) of visits, and contributed to 53.7% of INTs. MR-CT was obtained in 129 (14.1%) of visits, and contributed to 30.1% of INTs. ECG was applied in 137 (15%) visits, and contributed to 1.6% of INTs. HOL was obtained in 188 (20.5%) visits, and contributed to 11.3% of INTs. STR was performed at 101 (11%) of visits, and contributed to 8.9% of INTs. CONCLUSIONS: INTs are common in repaired TOF, but when visits average every 11-12 months, most visits do not result in INT. H&P, Echo and HOL were the most frequently applied screens, and all frequently yielded relevant information to guide INT decisions. STR and MR/CT were applied as targeted testing and in this limited, non-screening role had high relevance for INT. There was low utilisation of ECG and major impact on INT was not demonstrated. Risk stratification in TOF may be possible, and could result in more efficient surveillance and targeted testing.

Staged Left Ventricular Recruitment and Biventricular Conversion in Hypoplastic Left Heart Syndrome.

We describe a relatively long left ventricular recruitment pathway consisting of early and serial aortic valvuloplasties and multiple endocardial fibroelastosis resections resulting in successful biventricular conversion of hypoplastic left heart syndrome.