Maan Jokhadar

Features of portal hypertension are associated with major adverse events in Fontan patients: The VAST study

BACKGROUND Chronic congestive hepatopathy is known to cause hepatic fibrosis and portal hypertension in patients post-Fontan operation for single ventricle palliation. The clinical significance of these findings is not clear. We hypothesized that features of portal hypertension would be significantly related to major adverse events. METHODS A retrospective review of 73 adult and pediatric post-Fontan patients referred for a liver evaluation from 2001 to 2011 was performed. The relationship between features of portal hypertension (VAST score ≥2, 1 point each for Varices, Ascites, Splenomegaly or Thrombocytopenia) and a major adverse event (death, need for transplant, or hepatocellular carcinoma) was examined using logistic regression. RESULTS 73 post-Fontan patients (30% female, 73% Caucasian, 66% systemic left ventricle (SLV), mean age 24±11 years, mean interval from Fontan 17±6 years) were included in analysis. Features of portal hypertension (VAST score ≥2) were present in 26 (36%), and there were 19 major adverse events: death (n=12), transplant (n=6), and HCC (n=1). A significant relationship was found between VAST score ≥2 and major adverse events (OR=9.8, 95% CI [2.9-32.7]). After adjusting for time since Fontan, SLV, age, hemoglobin and type of failure, VAST score ≥2 remained significant (OR=9.1, 95% CI [1.4-57.6]). CONCLUSION Fontan patients with features of portal hypertension have a 9-fold increased risk for a major adverse event. Therapies targeted to manage clinical manifestations of portal hypertension, and early referral to heart transplant may help delay major adverse events. Future prospective studies are needed to confirm these findings.

Risk Factors for Major Adverse Events Late after Fontan Palliation

OBJECTIVE Risk factors for major adverse events late after Fontan palliation are unknown. Prior studies have suggested ventricular function and morphology as important risk factors. The aim of this study is to (1) characterize the late major adverse event profile in adult Fontan patients and (2) identify additional risk factors that may contribute to adverse outcomes. DESIGN AND SETTING A retrospective review of all adult patients >15 years post-Fontan seen at a tertiary academic center was conducted. Clinical, laboratory, cardiac data, and abdominal imaging were collected via chart review. Major adverse events (death, cardiac transplantation, or listing) were identified, and timing of events was plotted using Kaplan-Meier methods. Univariate and multivariate logistic regression was used to determine independent predictors of late-term events. RESULTS A total of 123 adult Fontan patients were identified (mean time post-Fontan 22.4 years [±4.4]). Major adverse events occurred in 19/123 patients (15%). In this 15-year survivor cohort, transplant-free survival rates were 94.6%, 82.9%, and 59.8% at 20, 25, and 30 years postoperation, respectively. Modes of death were Fontan failure with preserved function (4), congestive heart failure with decreased function (2), sudden death (2), thromboembolic event (1), post-Fontan conversion (2), and posttransplant (2). No differences in adverse outcomes were found based on morphology of the systemic ventricle, Fontan type, or systolic ventricular function. On the other hand, features of portal hypertension (OR 19.0, CI 4.7-77.3, P < .0001), presence of a pacemaker (OR 13.4, CI 2.6-69.8, P = .002), and systemic oxygen desaturation (OR 0.86, CI 0.75-0.98, P = .02) were risk factors for major adverse events in the multivariate analysis. CONCLUSIONS In adult Fontan patients surviving >15 years post-Fontan, portal hypertension, oxygen desaturation, and need for pacemaker were predictive of adverse events. Traditional measures may not predict late-term outcomes in adult survivors; further study of the livers role in late outcomes is warranted.

Surgery in Adults With Congenital Heart Disease: Risk Factors for Morbidity and Mortality

BACKGROUND Patients with congenital heart disease are frequently surviving into adulthood, and many of them will require surgery. Unfortunately, outcome data in this patient population are limited. We aimed to identify risk factors associated with adverse events in adults with congenital heart disease undergoing cardiac surgery and establish long-term survival data. METHODS We retrospectively studied 458 adult patients who underwent cardiac surgery for congenital heart disease between 2000 and 2010. We constructed logistic models to assess risk factors for mortality, major adverse event (stroke, renal failure, prolonged ventilation, prolonged coma, deep sternal infection, reoperation, and operative mortality), and prolonged length of stay (>7 days). Long-term, all-cause mortality was also measured. RESULTS Sixteen patients died (3.49%). Risk factors for mortality included a history of cerebrovascular disease (odds ratio [OR]: 4.51), New York Heart Association (NYHA) class 3 or 4 (OR: 8.88), and surgery on the aorta or the aortic valve (OR: 5.74). Ninety-four patients suffered a major adverse event (20.5%). Significant risk factors were male gender (OR: 2.28), NYHA class of 3 or 4 (OR 2.58), 2 concomitant major operations (OR: 2.15), and cardiopulmonary bypass time of greater than 100 minutes (OR: 3.18). Last, 90 patients (19.7%) remained in the hospital longer than 7 days. Significant risk factors for a prolonged length of stay included chronic lung disease (OR: 3.05), NYHA class of 3 or 4 (OR: 3.69), surgery by an adult cardiac surgeon (OR 2.58), 2 concomitant major operations (OR: 3.28), and cardiopulmonary bypass time of greater than 100 minutes (OR: 2.41). Survival at 1, 5, and 10 years was 97.6%, 95.2%, and 93.4%, respectively. CONCLUSIONS Surgery in adults with congenital heart disease can be performed with low morbidity and mortality. Nonetheless, there remain important risk factors for adverse events. Awareness and modification of risk factors may help improve outcomes.

Catheter-measured Hemodynamics of Adult Fontan Circulation: Associations with Adverse Event and End-organ Dysfunctions.

BACKGROUND In heart failure, a high systemic vascular resistance index (SVRI), high central venous pressure (CVP), and low cardiac index (CI) predict poor outcomes. Conversely, late hemodynamic manifestations of failing Fontan circulation and associations with end-organ dysfunction are not well understood. METHODS A retrospective review of right-heart catheterization data of adult Fontan patients between 2002 and 2014 was conducted. Relationships between hemodynamic variables and serious adverse events (death or heart transplant) were examined using the Cox proportional hazard analysis. Correlations between the hemodynamic measurements and signs of end-organ dysfunction (MELD-XI, Child-Pugh, VAST score, estimated glomerular filtration rate [eGFR]) were analyzed. RESULTS Sixty post-Fontan patients (85% systemic left ventricle, 40% atriopulmonary Fontan, mean age of 28 years, and mean time since Fontan operation of 21.9 years) were included. At baseline, those with an event were statistically younger, had lower transcutaneous oxygen saturations, were more likely to have an atriopulmonary Fontan, and were more likely to have a pacemaker. Eighteen experienced a cardiovascularly significant event. Using univariate analysis to compare the event and nonevent groups, mean CI was 2.8 ± 0.9 vs. 2.4 ± 0.5 L/min/m2 (P = .004), and CVP was 18.6 ± 6.5 vs. 16.1 ± 4.3 mmHg (P = .03). However, the statistical significances did not persist in the multivariate model. Higher CVP and pulmonary capillary wedge pressure (PCWP) were associated with higher MELD-XI and Child-Pugh scores, and the VAST score was only associated with PCWP. CONCLUSIONS Symptomatic adult Fontan patients who experienced an event manifested with a higher CI and CVP, although the multivariate Cox proportional hazard analysis did not yield any significant associations. The presences of hepatic dysfunction and portal venous outflow obstruction were associated with a higher CVP and PCWP. Renal dysfunction was prevalent but no statistically significant association between the hemodynamic measurements was identified, although trends toward a higher CVP and transpulmonary gradient were identified.

THE EFFECT OF ANGIOTENSIN-CONVERTING-ENZYME INHIBITORS ON THE RATE OF ASCENDING AORTA DILATION IN PATIENTS WITH BICUSPID AORTIC VALVES

BACKGROUND Bicuspid aortic valve (BAV), the most common congenital heart defect, is associated with progressive aortic dilation and increased risk of dissection and rupture. The use of an angiotensin-converting enzyme (ACE) inhibitor has recently proven effective in slowing the rate of aortic dilation in other aortopathies, suggesting it may also be useful in managing BAV. We sought to determine whether ACE inhibitors effectively reduce the rate of aortic dilation in adolescents and young adults with BAV. METHODS A retrospective cohort study was undertaken. Subjects receiving ACE inhibitor therapy were compared with controls with BAV and matched for the degree of aortic regurgitation. The rate of change in ascending aorta diameter was compared between the two groups. RESULTS Among the 141 subjects identified in the patient records, 103 were receiving no medication and 38 were taking ACE inhibitors. Over a mean follow-up of 37 months, the ascending aorta Z-score increased slightly among those receiving ACE inhibitor +4.5 ± 1.6 to +4.7 ± 1.6. The rate of change was no different than those subjects not receiving ACE inhibitors, P = .64. CONCLUSION In an adolescent and young adult population with an isolated BAV, there is no proven benefit to ACE inhibitor use with regard to slowing rate of ascending aortic dilation.

De novo DQ donor‐specific antibodies are associated with worse outcomes compared to non‐DQ dnDSA following heart transplantation

Antibody‐mediated rejection (AMR) resulting from de novo donor‐specific antibodies (dnDSA) leads to adverse outcomes following heart transplantation (HTx). It remains unclear what role dnDSA to specific HLA antigens play in adverse outcomes. This study compares outcomes in patients developing dnDSA to DQ antigens with those developing non‐DQ dnDSA and those free from dnDSA.

Cor triatriatum sinister: a patient, a review, and some unique findings.

Introduction: Cor triatriatum sinister (CTS) is an uncommon clinical entity in pediatric and adult congenital clinics and is even rarer in adult cardiology practices. Our patient illustrates the historical and physiologic aspects of this entity and its unique echocardiographic features. This document features our patient and her imaging and goes on to review the important features of CTS, some of which are not commonly recognized.

REDUCED HEPATIC VENOUS FLOW IS A MARKER OF ADVERSE OUTCOMES IN PATIENTS WITH FONTAN CIRCULATION

background: Liver injury and portal hypertension are significant complications of the Fontan circulation and are thought to result from chronic venous hypertension. The flow characteristics of the involved veins, specifically the hepatic vein (HV), are unknown in this population, and their association with clinical outcomes has not been studied. We hypothesize that decreased HV flow is associated with adverse clinical outcomes.

Relief of right ventricle to pulmonary artery conduit compression by chest wall resection.

Many congenital heart defects require reconstruction of the right ventricular outflow tract utilizing a right ventricle to pulmonary artery conduit. One of the challenges with these conduits is the development of conduit stenosis. This phenomenon is quite common and typically results from a combination of progressive calcification, fibrosis, and/or the relative size mismatch that occurs with patient growth. However, extrinsic compression is much less common and a much more difficult problem to address. Chest wall resection and reconstruction is an option for alleviating external conduit compression that provides good results.

REPEAT PULMONARY VALVE REPLACEMENT: SIMILAR INTERMEDIATE-TERM OUTCOMES BETWEEN SURGICAL AND TRANSCATHETER PROCEDURES

There is no previous report comparing clinical or echocardiographic outcomes in patients with a history of prior surgical pulmonary valve replacement (SPVR) who undergo repeat SPVR or transcatheter pulmonary valve replacement (TPVR). We retrospectively identified patients who had previous SPVR and