Matthew J. Lewis

Procedural Success and Adverse Events in Pulmonary Artery Stenting: Insights From the NCDR.

BACKGROUND Risk factors associated with outcomes for pulmonary artery (PA) stenting remain poorly defined. OBJECTIVES The goal of this study was to determine the effect of patient and procedural characteristics on rates of adverse events and procedural success. METHODS Registry data were collected, and 2 definitions of procedural success were pre-specified for patients with biventricular circulation: 1) 20% reduction in right ventricular pressure or 50% increase in PA diameter; and 2) 25% reduction in right ventricular pressure or 50% decrease in PA gradient or post-procedure ratio of in-stent minimum to pre-stent distal diameter >80%. A separate definition of procedural success based on normalization of PA diameter was pre-specified for patients with single ventricle palliation. RESULTS Between January 2011 and January 2014, a total of 1,183 PA stenting procedures were performed at 59 institutions across 1,001 admissions; 262 (22%) procedures were performed in patients with a single ventricle. The rate of procedural success was 76% for definition 1, 86% for definition 2, and 75% for single ventricle patients. In the multivariate analysis, ostial stenosis was significantly associated with procedural success for biventricular patients according to both definitions. The overall complication rate was 14%, with 9% of patients experiencing death or a major adverse event (MAE). According to multivariate analysis, weight <4 kg, having a single ventricle, and emergency status were significantly associated with death or MAEs. CONCLUSIONS In our analysis, success was >75% across all definitions, and adverse events were relatively common. Biventricular patients with an ostial stenosis had a higher probability of a successful outcome. Patients who had a single ventricle, weight <4 kg, or who underwent an emergency procedure had a higher risk of death or MAE. These findings may help inform patient selection for PA stenting.

Is systemic right ventricular function by cardiac MRI related to the degree of tricuspid regurgitation in congenitally corrected transposition of the great arteries

BACKGROUND AND METHODS Systemic right ventricular dysfunction and tricuspid regurgitation (TR) are frequently encountered in patients with congenitally corrected transposition of the great arteries (CCTGA). Studies using echocardiography have suggested a relationship between the degree of TR and systemic right ventricular dysfunction; however, assessment of systemic right ventricular function by echocardiography is limited. Cardiac MRI (CMR) is the gold standard for volumetric assessment of the systemic right ventricle. We performed a retrospective cohort study at our center evaluating all adult patients with CCTGA who underwent a CMR between 1/1999 and 1/2013 to determine the relationship between the degree of TR and systemic right ventricular function. RESULTS Of the 33 patients identified, 12 had ≤ mild TR (37%), 13 had moderate TR (40%), and 8 had severe TR (24%). Mean age at CMR was 38 years (23-64). Mean right ventricular ejection fraction (45% vs. 41% vs. 42%, p=0.68) and mean indexed right ventricular end diastolic volume (122 ml/m(2) vs. 136 ml/m(2) vs. 138 ml/m(2)p=0.36) were not significantly different for patients with ≤ mild TR, moderate TR or severe TR. The degree of TR was not associated with additional congenital lesions, prior procedures, presence of an intraventricular conduction delay, or decreased left ventricular function. CONCLUSION No association between the degree of TR and right ventricular volume or ejection fraction by CMR was identified. Failure to show worsening function or increased volume with greater degrees of TR suggests that the degree of regurgitation alone may not fully explain the heterogeneity in right ventricular size and function.

Can Physicians Identify Inappropriate Nuclear Stress Tests? An Examination of Inter-Rater Reliability for the 2009 Appropriate Use Criteria for Radionuclide Imaging

Background— We sought to determine inter-rater reliability of the 2009 Appropriate Use Criteria for radionuclide imaging and whether physicians at various levels of training can effectively identify nuclear stress tests with inappropriate indications. Methods and Results— Four hundred patients were randomly selected from a consecutive cohort of patients undergoing nuclear stress testing at an academic medical center. Raters with different levels of training (including cardiology attending physicians, cardiology fellows, internal medicine hospitalists, and internal medicine interns) classified individual nuclear stress tests using the 2009 Appropriate Use Criteria. Consensus classification by 2 cardiologists was considered the operational gold standard, and sensitivity and specificity of individual raters for identifying inappropriate tests were calculated. Inter-rater reliability of the Appropriate Use Criteria was assessed using Cohen &kgr; statistics for pairs of different raters. The mean age of patients was 61.5 years; 214 (54%) were female. The cardiologists rated 256 (64%) of 400 nuclear stress tests as appropriate, 68 (18%) as uncertain, 55 (14%) as inappropriate; 21 (5%) tests were unable to be classified. Inter-rater reliability for noncardiologist raters was modest (unweighted Cohen &kgr;, 0.51, 95% confidence interval, 0.45–0.55). Sensitivity of individual raters for identifying inappropriate tests ranged from 47% to 82%, while specificity ranged from 85% to 97%. Conclusions— Inter-rater reliability for the 2009 Appropriate Use Criteria for radionuclide imaging is modest, and there is considerable variation in the ability of raters at different levels of training to identify inappropriate tests.

Postoperative tricuspid regurgitation after adult congenital heart surgery is associated with adverse clinical outcomes

OBJECTIVE Many patients with adult congenital heart disease will require cardiac surgery during their lifetime, and some will have concomitant tricuspid regurgitation. However, the optimal management of significant tricuspid regurgitation at the time of cardiac surgery remains unclear. We assessed the determinants of adverse outcomes in patients with adult congenital heart disease and moderate or greater tricuspid regurgitation undergoing cardiac surgery for non-tricuspid regurgitation-related indications. METHODS All adult patients with congenital heart disease and greater than moderate tricuspid regurgitation who underwent cardiac surgery for non-tricuspid regurgitation-related indications were included in a retrospective study at the Schneeweiss Adult Congenital Heart Center. Cohorts were defined by the type of tricuspid valve intervention at the time of surgery. The primary end point of interest was a composite of death, heart transplantation, and reoperation on the tricuspid valve. RESULTS A total of 107 patients met inclusion criteria, and 17 patients (17%) reached the primary end point. A total of 68 patients (64%) underwent tricuspid valve repair, 8 patients (7%) underwent tricuspid valve replacement, and 31 patients (29%) did not have a tricuspid valve intervention. By multivariate analysis, moderate or greater postoperative tricuspid regurgitation was associated with a hazard ratio of 6.12 (1.84-20.3) for the primary end point (P = .003). In addition, failure to perform a tricuspid valve intervention at the time of surgery was associated with an odds ratio of 4.17 (1.26-14.3) for moderate or greater postoperative tricuspid regurgitation (P = .02). CONCLUSIONS Moderate or greater postoperative tricuspid regurgitation was associated with an increased risk of death, transplant, or reoperation in adult patients with congenital heart disease undergoing cardiac surgery for non-tricuspid regurgitation-related indications. Concomitant tricuspid valve intervention at the time of cardiac surgery should be considered in patients with adult congenital heart disease with moderate or greater preoperative tricuspid regurgitation.

The Miracle Baby Grows Up: Hypoplastic Left Heart Syndrome in the Adult

Purpose of ReviewHypoplastic left heart syndrome (HLHS) is characterized by underdevelopment of the mitral valve, left ventricle, and aorta and is ultimately palliated with a single-ventricle repair. Universally fatal in infancy prior to the advent of modern surgical techniques, the majority of HLHS patients will now reach adulthood. However, despite improvements in early survival, the HLHS population continues to face significant morbidity and early mortality. This review delineates common sources of patient morbidity and highlights areas in need of additional research for this growing segment of the adult congenital heart disease population.Recent FindingsIt has become increasingly clear that palliated adult single ventricle patients, like those with HLHS, face significant life-long morbidity from elevated systemic venous pressures as a consequence of the Fontan procedure. Downstream organ dysfunction secondary to elevated Fontan pressures has the potential to significantly impact long-term management decisions, including strategies of organ allocation. Because of the presence of a morphologic systemic right ventricle, HLHS patients may be at even higher risk than other adult patients with a Fontan.SummaryBecause the adult HLHS population continues to grow, recognition of common sources of patient morbidity and mortality is becoming increasingly important. A coordinated effort between patients and providers is necessary to address the many remaining areas of clinical uncertainty to help ensure continued improvement in patient prognosis and quality of life.

Serial cardiac MRIs in adult Fontan patients detect progressive hepatic enlargement and congestion

BACKGROUND The progression of hepatic disease in adult Fontan patients is not well understood. They reviewed the experience with serial cardiac MRIs (CMR) in adult Fontan patients to determine if hepatic anatomic markers of prolonged Fontan exposure were present and if clinical predictors of progressive hepatic congestion could be identified. METHODS AND RESULTS A retrospective cohort study of all adult Fontan patients who had undergone at least two CMRs was performed. Hepatic dimensions, inferior vena cava (IVC) size, right hepatic vein (RHV) size and spleen diameter were determined from images acquired at the time of clinically guided CMR. Two radiologists with expertise in hepatic imaging graded congestion and liver size independently using post-gadolinium contrast sequences. Twenty-seven patients met inclusion criteria. Over a mean time of 5.1 years between CMRs, there was a significant increase in mean lateral-medial hepatic dimension (P = .005), mean RHV diameter (P = .004), and mean splenic diameter (P = .001). Serial post-gadolinium imaging was available in 25/27 (93%) patients of which 15/27 (55%) showed evidence of progressive hepatic congestion across serial studies. Progressive hepatic congestion was associated with single ventricle ejection fraction (SVEF) less than 50% (P = .008), and larger indexed end-diastolic (EDVI) and end-systolic volume (ESVI). RHV diameter was the only anatomic variable significantly correlated with time from Fontan completion (P = .004). CONCLUSIONS Serial CMRs detected progressive liver and hepatic vein enlargement in our cohort of adult Fontan patients over a mean time of 5.2 years. Progressive hepatic congestion occurs in a significant number of adult Fontan patients and may be associated with ventricular enlargement and decreased ventricular function by CMR.

RATE OF ARRHYTHMIA RECURRENCE POST-ABLATION IN ADULT CONGENITAL HEART DISEASE

Catheter ablation is commonly performed to treat persistent tachyarrhythmias in adult congenital heart disease (ACHD). Despite the frequency of ablations in the ACHD population, recurrence rates remain unknown. We sought to determine the risk factors and recurrence rate for tachyarrhythmias post

When Should Adult Congenital Heart Disease Patients be Considered for Transplant and Deciding Which Organs to Transplant

The number of adult congenital heart disease (ACHD) patients continues to increase. Because of multiple related factors such as aging, residual cardiac lesions and prior palliative procedures, advanced heart failure (HF) is increasingly prevalent in this population. Consequently, there is an emerging need to determine which patients are best suited for advanced cardiac therapies, including heart transplantation (HT) and mechanical circulatory support. Unfortunately, optimizing patient selection for these therapies is complicated by patient heterogeneity, variable HF presentation across lesion-type, and a paucity of outcome data. The lack of patient specific data and the increasing number of ACHD patients with end-stage HF identifies a need to more precisely stratify risk and determine appropriate timing for referral. As such, this article will discuss the clinical recognition and classification of advanced HF in the ACHD patient populations and review current data regarding HT outcomes. Following, key considerations regarding the timing of HT in specific forms of ACHD, the role of device therapy, and when dual organ transplantation should be considered will be reviewed. Finally, existing knowledge gaps and key research needs will be highlighted.

THE SYSTEMIC RIGHT VENTRICLE IN TRANSPOSITION OF THE GREAT ARTERIES: WHAT CAN WE LEARN FROM SERIAL IMAGING?

Background: Adult patients with congenitally corrected transposition of the great arteries (ccTGA) and D-transposition of the arteries (D-TGA) after a Mustard or Senning repair develop systemic right ventricular (SRV) dilation. However, the time frame and risk factors for progressive SRV enlargement

TIMING OF PULMONARY VALVE REPLACEMENT: CAN WE USE THE SAME VOLUMETRIC THRESHOLDS IN REPAIRED TETRALOGY OF FALLOT AND PULMONARY STENOSIS?

Valvar pulmonary stenosis (PS) is a common form of congenital heart disease in which relief of PS can result in pulmonary regurgitation and right ventricular dilation. While similarities with repaired tetralogy of Fallot (TOF) exist, the RV outflow patch in TOF patients complicates use of TOF-based