Meta-analysis: Incidence of cirrhosis and hepatocellular carcinoma in patients with Fontan palliation.

BACKGROUND AND AIMS: The Fontan palliation is the final stage of surgery for many children born with univentricular physiology. Almost all Fontan patients develop liver fibrosis which may eventually lead to cirrhosis and hepatocellular carcinoma (HCC). These are important causes of morbidity and mortality in these patients. We performed a systematic review and meta-analysis to assess the incidence of cirrhosis and HCC in Fontan patients and stratify it based on time since surgery. METHODS: A literature search of seven databases identified 1158 records. Studies reporting the number of cirrhosis and HCC cases in Fontan patients and time since Fontan surgery were included. In the cirrhosis cohort, we included only those studies where all patients underwent liver biopsy. RESULTS: A total of 23 studies were included: 12 and 13 studies in the cirrhosis and HCC cohorts, respectively, with two studies included in both cohorts. The incidence of cirrhosis was 0.97 per 100 patient-years (95% CI 0.57-1.63), with the incidence and cumulative incidence ≥20 years post Fontan surgery being 1.61 per 100 patient-years (95% CI 1.24-2.08) and 32.2% (95% CI 25.8%-39.4%), respectively. The incidence of HCC was 0.12 per 100 patient-years (95% CI 0.07-0.21), with the incidence and cumulative incidence ≥20 years post Fontan surgery being 0.20 per 100 patient-years (95% CI 0.12-0.35) and 3.9% (95% CI 2.2%-6.8%), respectively. Only about 70% of patients with HCC (20/28) had underlying cirrhosis. CONCLUSION: The incidence of cirrhosis and HCC increases over time, especially at ≥20 years post Fontan surgery. Studies are needed to further identify at-risk patients in order to streamline surveillance for these highly morbid conditions.

Comparison of Wound Debridement Pressures With Updated Tests.

BACKGROUND: Wound debridement is one of the key treatment methods for chronic and acute wounds. Various tools are used to perform debridement, but the force applied to the tissue by these different instruments has been poorly documented in a limited number of past research efforts. PURPOSE: The aim of this study was to provide insight into the actual amount of pressure exerted on wound tissue. METHODS: We used a digital force transducer to measure the pressure applied by multiple combinations of angiocatheter needles (catheters), syringes, and other common debridement tools. The data obtained were compared with the pressure measurements reported by previous studies. The common standard used in research is a 35-mL syringe with a 19-gauge catheter with a pressure of 7 to 8 pounds per square inch (psi), which is regarded as the most effective for wound care. RESULTS: Many of the instruments measured in this experiment closely reflected the pressure measurements previously published in the research literature and are safe to use to properly irrigate wounds. However, some discrepancies were also found, ranging from a small psi variability to several psi. Further studies and testing may be beneficial to confirm the results of this experiment. CONCLUSION: Certain tools produced higher pressures that were not suitable for routine wound care. Findings from this study can also be used by clinicians to choose appropriate tools and to monitor pressure when they use various common irrigation tools.

Anatomical Differences Between Human and Pig Hearts and Their Relevance for Cardiac Xenotransplantation Surgical Technique.

Cardiac xenotransplantation has been proposed to bridge the gap between supply and demand for patients with end-stage heart failure requiring transplantation. However, differences in pig anatomy compared with human anatomy require modification of the surgical approach. In addition, careful consideration should be given to size matching before transplantation. (Level of Difficulty: Advanced.).

Heritable FLNA Gene Mutation in a Patient With Thoracic Aortic Aneurysm.

A 47-year-old woman with systemic lupus erythematosus and previously repaired ascending aortic aneurysm presented with a new ascending aortic aneurysm. Genetic testing revealed a FLNA gene mutation. Her mother subsequently tested positive and was found to have an aneurysm on screening, illustrating the utility of genetic screening for aortopathies. (Level of Difficulty: Intermediate.).

Pulmonary Valve Replacement in an Adult Jehovah's Witness with Tetralogy of Fallot.

The refusal of Jehovah's Witnesses to use blood products can limit access to cardiac surgery, as patients may not be offered surgery for complex disease, especially revision surgery. We report a successful, complex adult congenital heart disease (ACHD) surgery with intraoperative and perioperative optimization. We have tried to highlight through this case that complex ACHD surgeries can be performed in Jehovah's Witness patients with skilled perioperative and intraoperative management. The role of bovine hemoglobin in this population is being defined and was found helpful in this case.

Adherence to quality indicators and hospital outcomes for patients with decompensated cirrhosis: An observational study.

Background: Quality indicators (QIs) exist for the care of patients with cirrhosis. We retrospectively examined the records of patients admitted to a large academic tertiary care centre for adherence to QIs and examined for an association between QI adherence and hospital outcomes. Methods: We conducted a cross-sectional study of all patients with decompensated liver cirrhosis admitted to a large academic tertiary care centre over a 2-year period (2014-2016). Medical records of 522 patients were examined for 17 QIs related to inpatient cirrhosis care and adherence-judged using three different standards: 100% adherence, 70% adherence, or the QI score as a continuous variable. Linear and logistic regression was used to evaluate the association between QI score and length of stay (LOS), 30-day readmissions, and inpatient mortality, respectively. Results: Adherence to QIs was variable (range 20%-95%). Overall, adherence to QIs relating to variceal bleeding was higher than adherence to indicators related to hepatic encephalopathy and spontaneous bacterial peritonitis. There was weak evidence for a decreased odds of 30-day readmission when more QIs were met, regardless of the method used to quantify adherence (100% standard OR 0.53 [95% CI 0.26-1.09, p = .09], 70% standard OR 0.58 [95% CI 0.32-1.06, p = .08], continuous method OR 0.90 [95% CI 0.81-1.01, p = .07]). There was no observed relationship between mortality and QI adherence and equivocal evidence for an association between QI adherence and LOS. Conclusions: Adherence to QIs related to inpatient care of decompensated cirrhosis may be associated with decreased 30-day readmissions.

Transmitral Septal Myectomy for Hypertrophic Obstructive Cardiomyopathy.

BACKGROUND: Intrinsic abnormalities of the mitral valve are common in patients with hypertrophic cardiomyopathy and may need to be addressed at operation. METHODS: Consecutive patients undergoing transmitral septal myectomy were retrospectively reviewed. The ventricular septum was exposed through a left atriotomy, and the anterior leaflet of the mitral valve was detached from its annulus. An extended myectomy was performed to the base of the papillary muscles. After myectomy, the anterior leaflet was reattached and concomitant mitral valve repair or replacement was performed. In some cases, we performed a modified anterolateral commissural closure suture, which served to reposition the lateral aspect of the anterior leaflet out of the left ventricular outflow tract ("curtain stitch"). RESULTS: Twenty patients who underwent this procedure were identified (70% women; mean age 63 years). Mitral regurgitation was moderate in 55% and severe in 40%. Preoperative peak left ventricular outflow tract gradient was 92 ± 43 mm Hg. Mitral valve repair (n = 11) or replacement (n = 9) was performed. Predischarge transthoracic echocardiography demonstrated a left ventricular outflow tract gradient of 10 ± 5 mm Hg. There was no operative mortality. Follow-up was 100% complete and averaged 22 ± 25 months. No patient required reoperation, and there was no recurrence of left ventricular outflow tract obstruction or mitral regurgitation greater than mild. CONCLUSIONS: Potential advantages of transmitral myectomy include a panoramic view of the septum and mitral subvalvular apparatus and the ability to simultaneously address mitral valve pathology. Consideration should be given to using the transmitral approach to septal myectomy as the preferred approach for the surgical treatment of hypertrophic cardiomyopathy.

Energy drink-induced near-fatal ventricular arrhythmia prevented by an intracardiac defibrillator decades after operative "repair" of tetralogy of Fallot.

We describe a 45-year old man who experienced a potentially fatal arrhythmia after consumption of multiple energy drinks. At 5 years old, he underwent "repair" of tetralogy of Fallot using a patch in the right ventricular outflow tract, and at age 40 had an automatic implantable cardiac defibrillator (AICD) placed. His first AICD shock occurred within 30 minutes after he finished the third energy drink and was preceded by feelings of lightheadedness and severe dizziness. Without the AICD, he likely would have died. The risk of consuming energy drinks in those with underlying structural heart disease and the general population should be determined. Warning labels should be required to inform consumers of the risks posed by these drinks and of appropriate limits for consumption.

Adults with congenital heart disease and heart transplantation: optimizing outcomes.

The growing population of adults with congenital heart disease (ACHD) present a distinct set of challenges in the evaluation, listing, and safe performance of heart transplantation. We review existing literature to (1) describe the complex anatomic and physiologic features that characterize this group of patients, (2) discuss factors contributing to high waiting list times and waiting list morbidity and mortality experienced by this population, and finally, (3) identify risk factors for the elevated post-transplant morbidity and mortality reported in ACHD patients. We conclude with a discussion of critical areas in need of further investigation as well as ways to amend the current listing criteria to better accommodate ACHD patients in need of heart transplantation.

Aggregate risk of cardiovascular disease among adolescents perinatally infected with the human immunodeficiency virus.

BACKGROUND: Perinatally HIV-infected adolescents may be susceptible to aggregate atherosclerotic cardiovascular disease risk, as measured by the Pathobiological Determinants of Atherosclerosis in Youth (PDAY) coronary arteries and abdominal aorta risk scores, as a result of prolonged exposure to HIV and antiretroviral therapy. METHODS AND RESULTS: Coronary arteries and abdominal aorta PDAY scores were calculated for 165 perinatally HIV-infected adolescents, using a weighted combination of modifiable risk factors: dyslipidemia, cigarette smoking, hypertension, obesity, and hyperglycemia. Demographic and HIV-specific predictors of scores ≥1 were identified, and trends in scores over time were assessed. Forty-eight percent and 24% of the perinatally HIV-infected adolescents had coronary arteries and abdominal aorta scores ≥1, representing increased cardiovascular disease risk factor burden. Significant predictors of coronary arteries scores ≥1 included male sex, history of an AIDS-defining condition, longer duration of use of a ritonavir-boosted protease inhibitor, and no prior use of tenofovir. Significant predictors of abdominal aorta scores ≥1 included suppressed viral load, history of an AIDS-defining condition, and longer duration of boosted protease inhibitor use. No significant changes in coronary arteries and abdominal aorta risk scores were observed over the 4-year study period. CONCLUSIONS: A substantial proportion of perinatally HIV-infected youth have high PDAY scores, reflecting increased aggregate atherosclerotic cardiovascular disease risk factor burden. High scores were predicted by HIV disease severity and boosted protease inhibitor use. PDAY scores may be useful in identifying high-risk youth who may benefit from early lifestyle or clinical interventions.

Cardiac effects in perinatally HIV-infected and HIV-exposed but uninfected children and adolescents: a view from the United States of America.

INTRODUCTION: Human immunodeficiency virus (HIV) infection is a primary cause of acquired heart disease, particularly of accelerated atherosclerosis, symptomatic heart failure, and pulmonary arterial hypertension. Cardiac complications often occur in late-stage HIV infections as prolonged viral infection is becoming more relevant as longevity improves. Thus, multi-agent HIV therapies that help sustain life may also increase the risk of cardiovascular events and accelerated atherosclerosis. DISCUSSION: Before highly active antiretroviral therapy (HAART), the two-to-five-year incidence of symptomatic heart failure ranged from 4 to 28% in HIV patients. Patients both before and after HAART also frequently have asymptomatic abnormalities in cardiovascular structure. Echocardiographic measurements indicate left ventricular (LV) systolic dysfunction in 18%, LV hypertrophy in 6.5%, and left atrial dilation in 40% of patients followed on HAART therapy. Diastolic dysfunction is also common in long-term survivors of HIV infection. Accelerated atherosclerosis has been found in HIV-infected young adults and children without traditional coronary risk factors. Infective endocarditis, although rare in children, has high mortality in late-stage AIDS patients with poor nutritional status and severely compromised immune systems. Although lymphomas have been found in HIV-infected children, the incidence is low and cardiac malignancy is rare. Rates of congenital cardiovascular malformations range from 5.6 to 8.9% in cohorts of HIV-uninfected and HIV-infected children with HIV-infected mothers. In non-HIV-infected infants born to HIV-infected mothers, foetal exposure to ART is associated with reduced LV dimension, LV mass, and septal wall thickness and with higher LV fractional shortening and contractility during the first two years of life. CONCLUSIONS: Routine, systematic, and comprehensive cardiac evaluation, including a thorough history and directed laboratory assays, is essential for the care of HIV-infected adults and children as cardiovascular illness has become a part of care for long-term survivors of HIV infection. The history should include traditional risk factors for atherosclerosis, prior opportunistic infections, environmental exposures, and therapeutic and illicit drug use. Laboratory tests should include a lipid profile, fasting glucose, and HIV viral load. Asymptomatic cardiac disease related to HIV can be fatal, and secondary effects of HIV infection often disguise cardiac symptoms, so systematic echocardiographic monitoring is warranted.

HAART to heart: highly active antiretroviral therapy and the risk of cardiovascular disease in HIV-infected or exposed children and adults.

Infection with HIV is independently associated with an increased risk for clinical heart failure, cardiomyopathies and premature atherosclerosis, including stroke and myocardial infarction in both the pre-HAART and HAART eras. HAART is also associated with clinical cardiovascular concerns. In HIV-infected individuals, HAART may cause adverse lipid profiles and increased risk for cardiovascular events. Its effects on the developing heart remain unclear. Although in utero HAART exposure may improve cardiac function in the first 2 years of life, it may also inhibit myocardial growth. Additional potentially damaging cardiovascular effects of HAART are present, and continuing cardiovascular risk evaluations, screening and follow-up of treated patients is necessary. Here, we review available research in this field and highlight the importance of understanding known complications and their mechanisms.

Cardiovascular disease and therapeutic drug-related cardiovascular consequences in HIV-infected patients.

The incidence of cardiovascular disease is greatly increased in the HIV-infected population compared with people of the same age without HIV. Cardiovascular manifestations of HIV/AIDS include, but are not limited to, accelerated atherosclerosis, pulmonary arterial hypertension, vasculitis, myocarditis, cardiomyopathy, pericardial diseases, malignancy (myocardial Kaposi sarcoma and B-cell immunoblastic lymphoma), and endocarditis. Drug effects and interactions that challenge the cardiovascular system are even more prevalent in this population, and careful review and surveillance of medication effects is crucial as is careful selection of highly active antiretroviral therapy. A focused assessment and understanding of disease prevalence and presentation is needed as symptoms may be non-specific and cardiovascular physical examination findings indeterminate due to co-morbid conditions in the patient population now living with chronic HIV infection.

Mid-ventricular obstructive hypertrophic cardiomyopathy during pregnancy complicated by preeclampsia and acute myocardial infarction: a case report.

Hypertrophic cardiomyopathy (HCM) with mid-ventricular obstruction (MVO) is a rare condition occurring in 1% of HCM patients. It is characterized by asymmetric left ventricular hypertrophy with MVO and elevated intraventricular pressure gradients. Myocardial infarction has been associated with mid-ventricular obstructive HCM. Briefly, this case presents an unusual clinical scenario where a young pregnant woman complicated by preeclampsia presents with myocardial injury and hemodynamic compromise related to undiagnosed HCM with MVO illustrating hemodynamic challenges created by pregnancy and surgery.

Heart failure and "inoperable" aortic stenosis: staged balloon aortic valvuloplasty and aortic valve bypass.

The prognosis for patients with symptomatic aortic stenosis is poor but is improved significantly by surgical aortic valve replacement. Unfortunately, many patients are refused surgery because of age, comorbidities, and hemodynamic instability. This report describes the successful use of balloon aortic valvuloplasty as a bridge to aortic valve bypass surgery (apicoaortic conduit) in an elderly patient with class IV congestive heart failure and severe left ventricular systolic dysfunction as a consequence of aortic stenosis who was not a candidate for traditional surgical valve replacement.

Accelerated cardiovascular disease and myocardial infarction risk in patients with the human immunodeficiency virus.

Highly active antiretroviral therapy has greatly reduced mortality among human immunodeficiency virus (HIV)-infected patients by delaying, and possibly preventing, progression to AIDS. The risk of cardiovascular disease (CVD) is now an important consideration in these patients and may increase as they live longer. Risk factors for CVD, the inflammatory effects of HIV, and the metabolic complications of antiretroviral therapy may accelerate the onset of CVD. Death from myocardial infarction, however, is still rare compared with death from progression of HIV disease, and the benefits of antiretroviral therapy clearly outweigh any associated risk of CVD. In this review, the authors describe the risk of accelerated CVD in HIV-infected individuals, the proposed viral and therapy-related mechanisms of CVD, the clinical features of CVD in these patients, and monitoring and management guidelines to reduce CVD risk. Identifying, monitoring, and treating CVD risk factors in HIV-positive patients is vital to improving their lives and should become standard practice.

Impact of HIV and highly active antiretroviral therapy on leukocyte adhesion molecules, arterial inflammation, dyslipidemia, and atherosclerosis.

Highly active antiretroviral therapy (HAART) has greatly extended the lives of people infected with the human immunodeficiency virus (HIV). This reduced risk of early death from opportunistic infections or other sequelae of HIV infection, however, means that other possible causes of death emerge. Myocardial infarction has become a matter of particular concern. Two of the main sources of cardiovascular disease in this population are believed to be vascular inflammation and dyslipidemia. We review the evidence for this hypothesis and discuss the relationship of HIV to vascular inflammation. Current treatment guidelines do not recommend the immediate initiation of HAART unless warranted, potentially allowing long-term, unchecked viral impact on the development of atherosclerosis. Finally, we consider the protease inhibitors traditionally included in HAART regimens and their relationship to the development of dyslipidemia, as well as other classes of antiretrovirals, such as the non-nucleoside reverse transcriptase inhibitors, which might be a better choice for patients with cardiovascular risks. Other strategies, such as pharmacologic, nutritional, and physical activity interventions are discussed. The patients who might benefit most are those in whom the precursors of vascular plaques, such as fatty streak, smooth muscle cell, macrophage, and T-lymphocyte aggregation not yet identified by echocardiographic and biopsy findings have already developed as a result of unchecked viral inflammation and replication.