Anurag Sahu

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.

Noninvasive assessment of left ventricular assist devices with cardiovascular computed tomography and impact on management

BACKGROUND Left ventricular assist devices (LVADs) provide a bridge to recovery or heart transplantation but require serial assessment. Echocardiographic approaches may be limited by device artifact and acoustic window. Cardiovascular computed tomography (CCT) may provide improved non-invasive imaging of LVADs. We evaluated the diagnostic findings and clinical impact of CCT for non-invasive assessment of patients with LVADs. METHODS CCT examinations performed between 2005 and 2008 in patients with LVADs were identified. Acquisitions were completed on the identical 64-detector-row scanner with intravenous contrast administration. Electrocardiographic gating was used in patients with pulsatile devices, and peripheral pulse gating was used in patients with continuous-flow devices. Comparison was made between CCT results and 30-day outcomes, including echocardiographic and intraoperative findings. RESULTS We reviewed 32 CCT examinations from 28 patients. Indications included evaluation of low cardiac output symptoms, assessment of cannula position, low flow reading on the LVAD, and surgical planning. CCT identified critical findings in 6 patients, including thrombosis and inlet cannula malposition, all confirmed intraoperatively. CCT missed 1 case of intra-LVAD thrombus. Using intraoperative findings as the gold standard, CCTs sensitivity was 85% and specificity was 100%. Echocardiographic LVAD evaluation did not correlate with findings on CCT (kappa = -0.29, 95% confidence interval, -0.73 to 0.13). CONCLUSIONS This preliminary observational cohort study indicates that non-invasive imaging using CCT of LVADs is feasible and accurate. CCT warrants consideration in the initial evaluation of symptomatic patients with LVADs.

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.

Abdominal Imaging Surveillance in Adult Patients After Fontan Procedure: Risk of Chronic Liver Disease and Hepatocellular Carcinoma

PURPOSE To assess the prevalence of chronic liver disease (CLD) and hepatocellular carcinoma (HCC) in adult patients who had surveillance imaging after Fontan procedure. METHODS Institutional review board-approved, Health Insurance Portability and Accountability Act-compliant retrospective study evaluated electronic medical records including radiology reports and clinical notes for adult patients after Fontan procedure between January 1993 and January 2016. Abdominal ultrasound, computed tomography, and magnetic resonance imaging reports were reviewed for changes of CLD and HCC. Existence of concomitant viral hepatitis was also recorded. RESULTS A total of 145 patients (male: 78 and female: 67) had surveillance imaging after Fontan procedure. In all, 78% (113/145) had ongoing imaging surveillance (median follow-up 3.05 years, IQR: 0.75-5.3 years); 19% (21/113) had an initial normal study and remained normal throughout follow-up; 19% (21/113) had an initial normal study with subsequent imaging reporting changes of CLD; and 62% (71/113) had existing changes of CLD on initial study. HCC was identified in 5 patients (median 22 years post-Fontan, IQR: 10-29 years), 4 of which had a normal initial study. Only 1 patient with HCC had concomitant viral hepatitis C infection. CONCLUSION Radiologists should be aware that CLD is exceedingly common in post-Fontan cardiac physiology, and surveillance imaging is warranted given the risk of HCC.

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.

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

Pregnancy in a Previously Conjoined Thoracopagus Twin with a Crisscross Heart

Background. Crisscross heart (CCH) is a complex, rare, congenital, rotational, cardiac abnormality that accounts for <0.1% of congenital heart defects (CHD). CCH is characterized by the crossing of the inflow streams of the two ventricles due to an abnormal twisting of the heart. A case of maternal CCH has not been previously reported. Case. We report a case of a primigravida with a CCH, who was separated at birth from her thoracopagus conjoined twin. Pregnancy was managed by congenital cardiology, maternal-fetal medicine, anesthesiology, and obstetrics. She underwent a 39-week vaginal delivery without maternal or neonatal complication. Conclusion. A successful term pregnancy outcome was achieved in a patient with CCH using a multidisciplinary approach to address her cardiac condition.

Konno Aortoventriculoplasty Following Arterial Switch Operation Requires Pulmonary Valve Disruption and Right Ventricular Outflow Tract Reconstruction

In the presence of a small aortic annulus, aortic valve replacement can be challenging. Several annular enlarging techniques have been described to allow placement of an adequately sized prosthesis. In patients who have undergone prior cardiac surgery, particularly for congenital heart defects, standard techniques may require modification. This case describes the challenges of enlarging the aortic annulus after previous arterial switch operation for transposition of the great arteries. Specifically, the Konno aortoventriculoplasty following arterial switch operation requires pulmonary valve disruption and right ventricular outflow tract reconstruction.

Double whammy: anomalous pulmonary and systemic venous drainage in a patient with scimitar syndrome.

Anomalously draining right pulmonary veins are expected with scimitar syndrome, but systemic venous abnormalities are rare. We present an unusual case of a female patient with scimitar and an interrupted inferior vena cava.

Nonanatomical Mitral Valve Replacement in the Pulmonary Venous Confluence for Heavily Calcified Aortic or Mitral Annulus

Mitral valve replacement surgery after previous aortic valve surgery can be extremely challenging. The presence of heavy calcification and scarring in the aortic and/or mitral annulus can make suture placement nearly impossible. Although novel techniques have been described, even these alternatives might not be feasible. We present a case of nonanatomical mitral valve replacement in the pulmonary venous confluence as a salvage operation. This can provide adequate hemodynamics and survival until a subsequent care plan can be established.