Michael McConnell

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.

Public Health Science Agenda for Congenital Heart Defects: Report From a Centers for Disease Control and Prevention Experts Meeting

Congenital heart defects (CHDs) are the most common type of birth defect, affecting ≈1% of births per year.[1][1] Although survival has been improving over time, there remain numerous gaps in the understanding of the public health impact of CHDs across the lifespan. Recognizing that there was “a

Interactive Telecardiology for the Evaluation of Heart Murmurs in Children

OBJECTIVE To study the accuracy, patient satisfaction, and cost of telecardiographic evaluations of pediatric patients. MATERIALS AND METHODS Patients referred to a rural pediatric cardiology outreach clinic were examined in person by a pediatric cardiologist. A second pediatric cardiologist who had no knowledge of the findings of face-to-face examination reevaluated the same patients utilizing a 768-Kbps telemedicine system. Any additional testing was performed by personnel who had no knowledge of the face-to-face evaluation. The main outcome measures included the final cardiac diagnosis, frequency of additional tests such as electrocardiography, (ECG) echocardiography (ECHO), and patient satisfaction. RESULTS The diagnosis was agreed upon in 19 of the 21 patients studied. Two patients with small ventricular septal defects were missed during the telemedicine evaluation. The utilization rates of additional studies for both the face-to-face cardiologist and the telemedicine cardiologist were not significantly different. Patient satisfaction with the telemedicine encounter was good. CONCLUSIONS Telemedicine appears to be effective and useful for the cardiac evaluation of pediatric patients. In spite of high data-transfer rates, differences between telemedicine and face-to-face patient encounters were observed.

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.

Closure of Symptomatic Ventricular Septal Defects: How Early Is Too Early?

With improvements in technology and surgical technique, pediatric cardiologists are challenging surgeons to close symptomatic ventricular septal defects (VSDs) in ever smaller patients. Although delaying surgery may facilitate operative repair, early intervention decreases the period of time these patients require therapy to prevent heart failure, maintains growth, and minimizes exposure to increased pulmonary pressures. To evaluate early intervention, we compare the outcomes of VSD closure in different-sized children. From December 2002 to July 2005, 225 patients underwent closure of a VSD. These patients were divided into four weight-based groups: <4 kg (group 1, n = 28), 4 to 6 kg (group 2, n = 93), 6 to 10 kg (group 3, n = 47), and >10 kg (group 4, n = 57). We reviewed operative and postoperative data, and comparisons were made between the groups. Median weights and ages at the time of surgery were 3.5 kg and 77 days (group 1), 4.9 kg and 128 days (group 2), 7.1 kg and 309 days (group 3), and 18.2 kg and 190 days (group 4). Operative data included cardiopulmonary bypass (CPB), aortic cross-clamp, and procedure times. CPB (p = 0.064), cross-clamp (p = 0.665), and procedure (p = 0.187) times were not significantly affected by decreasing weight. Postoperative continuous data included duration of ventilation and length of intensive care unit (ICU) and hospital stay. Ventilation (p = 0.667) and ICU (p = 0.976) times and length of hospital stay (p = 0.905) were also unaffected by decreasing weight. Postoperative catagoric data included complications and presence of a residual VSD. There was no significant difference in complications (p = 0.763) or residual VSD (p = 0.696) between groups. There was no mortality and no persistent heart block requiring placement of a permanent pacemaker. With improvements in technology and surgical technique, safe and effective VSD closure can be performed in increasingly smaller children. Earlier repair decreases the period of time these patients require aggressive medical therapy to prevent heart failure and maintain growth. It also decreases the period of time for which they are exposed to increased pulmonary pressures and are at risk for infectious respiratory complications. It does not appear to affect operative or postoperative outcomes.

Impact of Spironolactone on Endothelial Function in Patients with Single Ventricle Heart

BACKGROUND Mid-term survivors of the Fontan procedure are at risk for progressive heart failure, and endothelial dysfunction is thought to contribute to this process. Aldosterone antagonism has been shown to improve survival in adults with heart failure and the effects are mediated in part by changes in endothelial function. In the present study, we sought to determine if a short course of spironolactone improves endothelial function and alters serum cytokine profiles in adolescents and adults with single ventricle heart. METHODS Subjects had baseline assessment of flow-mediated dilation and cytokine profiles (C-reactive protein, interleukin-6, interleukin-1b, interleukin-10, tumor necrosis factor-alpha). They were started on spironolactone 25 mg once a day and uptitrated to 50 mg once daily. After 4 weeks, flow-mediated dilation and cytokine profiles were re-evaluated. RESULTS Ten subjects (median age 28 years) were enrolled and completed the protocol. The median flow-mediated dilation at baseline was 9.1% and did not change significantly after 4 weeks of spironolactone 7.6%, P = .46. There was mild elevation in serum cytokine profiles and only interleukin-1b decreased significantly with therapy, 0.39 to 0.23 pg/mL, P = .04. CONCLUSIONS In this small study, a short course of spironolactone did not improve endothelial function or alter the majority of serum cytokine levels. Whether single ventricle patients might realize other potential benefits of aldosterone antagonism such as reduced cardiac fibrosis remains to be determined.

Hemodynamic response to exercise in patients with sickle cell anemia

SummaryIn order to evaluate potential electrocardiographic (ECG) correlates of ST-segment depression during exercise in patients with sickle cell anemia (SS), 43 subjects, aged 5–23 years, underwent submaximal exericse testing. Eight (19%) had ST-segment depression on the exercise ECG during submaximal exercise. These eight patients had significantly lower hemoglobin levels than the 35 subjects without evidence of ST-segment depression. These eight subjects also had significantly higher peak blood pressures and peak heart rates than the 35 subjects without ST-segment depression. The product of peak heart rate and peak systolic blood pressure, the maximal double product, is a correlate of myocardial oxygen consumption and was significantly higher in the patients with ST-segment depression than in the patients without ST-segment depression. These results indicate that patients with SS and evidence of exercise-induced ST-segment depression may have decreased myocardial oxygen supply due to low hemoglobin levels and increased myocardial oxygen demand (elevated double products) when compared to subjects with SS who do not have exercise-induced ST-segment depression.

Fontan conversion with hepatic vein exclusion: a means for hepatic preservation in single ventricle heart disease.

Patients with single ventricle heart disease often undergo staged surgical palliation, ultimately resulting in Fontan anatomy and physiology. Long-term consequences include cirrhosis of the liver, protein-losing enteropathy, and premature death. Elevated central venous pressure and venous congestion transmitted to the abdominal viscera have been implicated in the aetiology of many of these complications. We present a novel operation directed at protecting the liver and intestines by excluding the splanchnic venous return from the Fontan pathway. Instead of exposure to elevated Fontan pressures, the liver and intestines will be exposed to lower common atrial pressures. We hope that this modification will minimise the abdominal complications of Fontan anatomy and physiology.

Cyanosis Produced By Superior Vena Caval Stenosis

Superior vena cava syndrome is a rare complication of pacemaker implantation. Narrowing and occlusion of the superior vena cava is often asymptomatic due to the formation of decompressive collateral pathways to the right atrium. We present a case in which an anomalous venous pathway allowed decompression of the systemic venous return into the left atrium, resulting in arterial desaturation, cyanosis, and fatigue.