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Background The prognosis of left ventricular noncompaction (LVNC) remains elusive despite its recognition as a clinical entity for >30 years. We sought to identify clinical and imaging characteristics and risk factors for mortality in patients with LVNC. Methods and Results 339 adults with LVNC seen between 2000 and 2016 were identified. LVNC was defined as end‐systolic noncompacted to compacted myocardial ratio >2 (Jenni criteria) and end‐diastolic trough of trabeculation‐to‐epicardium (X):peak of trabeculation‐to‐epicardium (Y) ratio <0.5 (Chin criteria) by echocardiography; and end‐diastolic noncompacted:compacted ratio >2.3 (Petersen criteria) by magnetic resonance imaging. Median age was 47.4 years, and 46% of patients were female. Left ventricular ejection fraction <50% was present in 57% of patients and isolated apical noncompaction in 48%. During a median follow‐up of 6.3 years, 59 patients died. On multivariable Cox regression analysis, age (hazard ratio [HR] 1.04; 95% CI, 1.02–1.06), left ventricular ejection fraction <50% (HR, 2.37; 95% CI, 1.17–4.80), and noncompaction extending from the apex to the mid or basal segments (HR, 2.11; 95% CI, 1.21–3.68) were associated with all‐cause mortality. Compared with the expected survival for age‐ and sex‐matched US population, patients with LVNC had reduced overall survival ( P <0.001). However, patients with LVNC with preserved left ventricular ejection fraction and patients with isolated apical noncompaction had similar survival to the general population. Conclusions Overall survival is reduced in patients with LVNC compared with the expected survival of age‐ and sex‐matched US population. However, survival rate in those with preserved left ventricular ejection fraction and isolated apical noncompaction was comparable with that of the general population.

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BackgroundStenosis of the venous connections and conduits is a well‐known late complication of the Fontan procedure. Currently, data on the outcomes of percutaneous intervention for the treatment of extra‐ or intracardiac conduits and lateral tunnel baffles obstruction are limited. In an attempt to better define the nature and severity of the stenosis and the results of catheter interventional management, we reviewed Fontan patients with obstructed extra‐ or intracardiac conduits and lateral tunnel baffles.MethodsRetrospective review of all Fontan patients who had cardiac catheterization from January 2002 to October 2018 was performed. Hemodynamic and angiographic data that assessed extra‐ or intracardiac conduit, or lateral tunnel baffle obstruction/stenosis were evaluated.ResultsTwenty patients underwent catheter intervention because of conduit stenosis, including calcified homografts, stenotic Gore‐Tex conduits and obstructed lateral tunnels. Six other patients had Fontan obstruction but were referred for surgical revision. After stenting, there was a significant reduction in the connection gradient [2.0 mm Hg (IQR 2; 3) vs 0 mm Hg (IQR 0; 1), P < .0001]. Fontan conduit/connection diameter increased [10.5 mm (IQR 9; 12) vs 18 mm (IQR 14.9; 18); P < .0001] and New York Heart Association class [III (IQR II; III) vs I (IQR II; III); P = .03) with stent placement.ConclusionsWe demonstrated the hemodynamics and angiographic subtypes of conduit stenosis in patients after Fontan, We showed that calcified homografts, stenotic Gore‐Tex conduits and lateral tunnels pathways can be safely and effectively stented to eliminate obstruction. Percutaneous stenting is associated with a decrease in connection gradients and improvement in functional capacity.

PPV/RVPAC IE is associated with significant morbidity, mortality and high risk of requiring operative intervention. TTE and TEE are marginal diagnostic tools when used independently; they should be used as complementary techniques in the evaluation of those patients. Severe PPV/RVPAC stenosis was more common than regurgitation in patients with IE; thus IE should be considered in patients presenting with new PPV/RVPAC obstruction.

BackgroundNational prevalence and outcomes of Fontan operation in the USA is unknown. Study objective was to determine trends (temporal change) in the annual volume of Fontan operations, in-hospital mortality, postoperative complications and type of hospital discharge.MethodsReview of the Nationwide Inpatient Sample for patients that underwent Fontan operation from 2001 to 2014 using the International Classification of Diseases-Ninth Revision, Clinical Modification procedure code for Fontan operation, that is, 35.94. To evaluate for change in patients’ demographics over the years, we divided the patient population into four groups based on procedure year (2001–2004, 2005–2008, 2009–2011 and 2012–2014).ResultsAn estimated 15 934 Fontan operations were performed in the USA from 2001 to 2014. Median (Q1–Q3) age was 3 (2–4) years and 39.8% were female. Hypoplastic left heart syndrome was the most common (29%) congenital heart disease diagnosis. An estimated 1175 procedures were performed in 2001 and 1340 in 2014, but there was no significant change in the number of procedures per year (p=0.47). There was significant decline in in-hospital mortality from 4.5% (53/1175) in 2001 to 1.1% (15/1340) in 2014 (p=0.009). When we compared event rates between 2001–2004 and 2012–2014 periods, there was significant decline in postoperative cardiac complications (12.6% (459/3640) to 8% (378/4706), p=0.007) and respiratory complications (17.1% (623/3640) to 10.2% (481/4706), p<0.001). However, there was increase in the number of patients discharged to home with healthcare assistance or transferred to another acute care facility (5.8% (211/3640) to 9.4% (443/4706), p=0.01) and inflation-adjusted hospitalisation cost (US$46 978 to US$60 383, p<0.001), but no significant change in length of stay (p=0.73).ConclusionOn the average, 1062 Fontan operations are performed annually in the USA with no change in volume of procedures but a decrease in in-hospital mortality and postoperative complications over a 15-year period.