Surgical Management of Marfan Syndrome in Children

Between August 1983 and January 1991, seven patients with Marfan syndrome underwent surgery for severe cardiovascular complications. The mean age at presentation was 5.7 months (range 4 to 9 months) in the infant group (n = 3), and 13.3 years (range 10 to 16 years) in a group of older children (n = 4). The primary indications for surgery in the infant group (performed at a mean of 3 years after diagnosis) were ascending aortic aneurysm with valvar regurgitation in one patient, and severe mitral valve prolapse with regurgitation in two. In the older group, surgical indications (performed at a mean of 2.8 years after diagnosis) were ascending aortic aneurysm with valvar regurgitation in three patients and acute aortic dissection in one. For aortic surgery, a composite valved conduit was used in four patients, and an aortic homograft in one. For mitral valve surgery, mechanical prostheses were used. Ail patients survived the primary operation. Over a mean follow-up of 17.5 patient-years (range 1 to 9 years), two patients in the infant Marfan group went on to further successful surgery (prosthetic mitral valve replacement and aortic root repair with aortic homograft) at a mean interval of 4.3 years after the Initial surgery. Our results suggest that the major cardiovascular risk factors of Marfan syndrome in the young, even in those diagnosed during infancy, have been favorably changed by surgery with an encouraging medium-term outlook. The correct timing of surgery is aided by echocardiography. (J Card Surg 1994;9:50–54)     

Lipoprotein(a) and Oxidized Phospholipids Promote Valve Calcification in Patients With Aortic Stenosis

Background

Lipoprotein(a) [Lp(a)], a major carrier of oxidized phospholipids (OxPL), is associated with an increased incidence of aortic stenosis (AS). However, it remains unclear whether elevated Lp(a) and OxPL drive disease progression and are therefore targets for therapeutic intervention.

Venous thromboembolism in patients with liver diseases

Essentials

• Emerging evidence shows that patients with liver disease are not protected from thrombotic events.
• We assessed the risk of venous thromboembolism (VTE) in patients with liver disease.
• The presence of VTE resulted in an increase in mortality for patients with liver disease.
• Hospitalized patients with moderate‐severe liver disease had low risk of VTE during admission.

Summary

Background and Aims

Patients with liver disease were traditionally believed to be protected against development of blood clots, but some studies have shown a potential increased risk of venous thrombotic complications. We assessed the risk of venous thromboembolism (VTE) in patients with liver disease.

Methods

Data in discharge reports of patients with liver disease and control patients without liver disease were analyzed from the national inpatient sample. Incidence of VTE was compared in patients with mild, moderate‐severe or no liver disease, and the impact on in‐hospital mortality and length of stay was calculated.

Results

The overall incidence of VTE for patients with no liver disease, mild liver disease and moderate‐severe liver disease was 2.7, 2.4 and 0.9 per 100 patient discharges, respectively. In the presence of VTE, in‐hospital mortality was 10.8%, 5.8%, and 21.7% for the no liver disease, mild disease and moderate‐severe liver disease, respectively. The presence of VTE resulted in an increase in mortality for patients with no liver disease (OR, 1.16; 95% CI, 1.14–1.18) and moderate‐severe liver disease (OR, 1.63; CI 95%, 1.42–1.88).

Conclusions

Patients with moderate‐severe liver disease have a lower risk of VTE than those without liver disease. Development of thrombosis during admission increased the risk of in‐hospital mortality.

     

Long-term follow-up of chronic hepatitis C in patients diagnosed at a tertiary-care center

BACKGROUND/AIMS: The natural history of chronic hepatitis C (HCV) is not completely understood. This study was aimed to evaluate the long-term outcome of the disease over a prolonged period of time and to identify factors associated with progression. METHODS: One hundred and sixteen patients with non-cirrhotic chronic non-A, non-B hepatitis consecutively diagnosed at a tertiary hospital between 1971 and 1977 were followed until December 1998 or until death. Patients with significant alcohol intake were excluded from the study. Variables obtained at the time of diagnosis, including epidemiological, clinical, laboratory, and histological data were recorded to determine risk factors associated with the development of liver cirrhosis and hepatic decompensation. RESULTS: Based on complete follow-up data, the development of liver cirrhosis and hepatic decompensation was evaluated in 94 and 114 of the 116 patients, respectively. Thirty-seven (39.3%) of 94 patients developed liver cirrhosis; an aspartate aminotransferase (AST) value higher than 70 IU/L was associated with development of cirrhosis (odds ratio (OR) 4.22, 95% CI 1.3-13.8). Hepatic decompensation occurred in 12 (10.5%) of 114 patients, its cumulative probability being 2.8% at 10 years, 5.2% at 15 years and 19.8% at 20 years. The only factor independently associated to the development of hepatic decompensation was the presence of fibrosis (stage 2 or 3) in the initial liver biopsy (OR 4.1, IC 95% 1.22-13.9). Liver-related death occurred only in seven (6%) of 114 patients. In comparison with the 116 patients diagnosed in the 1970's, patients with chronic hepatitis C diagnosed in 1999 were younger, more often asymptomatic, had lower AST and alanine aminotransferase (ALT) values and had significantly lower grade and stage histological scores. CONCLUSIONS: In summary, chronic hepatitis C had a high rate of progression to liver cirrhosis over a prolonged follow-up. However, this might be related to the fact that two decades ago the diagnosis was made at a significantly more advanced stage of the disease. Patients at high risk of progression can be identified by biochemical and histological variables at the time of diagnosis.     

Improving HIV Screening and Receipt of Results by Nurse-Initiated Streamlined Counseling and Rapid Testing

Background  HIV testing is cost-effective in unselected general medical populations, yet testing rates among those at risk remain low, even among those with regular primary care. HIV rapid testing is effective in many healthcare settings, but scant research has been done within primary care settings or within the US Department of Veteran’s Affairs Healthcare System. Objectives  We evaluated three methods proven effective in other diseases/settings: nurse standing orders for testing, streamlined counseling, and HIV rapid testing. Design  Randomized, controlled trial with three intervention models: model A (traditional counseling/testing); model B (nurse-initiated screening, traditional counseling/testing); model C (nurse-initiated screening, streamlined counseling/rapid testing). Participants  Two hundred fifty-one patients with primary/urgent care appointments in two VA clinics in the same city (one large urban hospital, one freestanding outpatient clinic in a high HIV prevalence area). Measurements  Rates of HIV testing and receipt of results; sexual risk reduction; HIV knowledge improvement. Results  Testing rates were 40.2% (model A), 84.5% (model B), and 89.3% (model C; p = <.01). Test result receipt rates were 14.6% (model A), 31.0% (model B), 79.8% (model C; all p = <.01). Sexual risk reduction and knowledge improvement did not differ significantly between counseling methods. Conclusions  Streamlined counseling with rapid testing significantly increased testing and receipt rates over current practice without changes in risk behavior or posttest knowledge. Increased testing and receipt of results could lead to earlier disease identification, increased treatment, and reduced morbidity/mortality. Policymakers should consider streamlined counseling/rapid testing when implementing routine HIV testing into primary/urgent care.     

Cardiac troponin I in neonates undergoing the arterial switch operation

BACKGROUND: Cardiac troponin I (TnI) is a sensitive and specific marker of myocardial injury, but little is known about its release after complex congenital heart surgery. We investigated whether TnI correlates with early clinical outcome in neonates undergoing the arterial switch operation (ASO) for transposition of the great arteries (TGA). METHODS: Troponin I was measured serially up to 48 hours postoperatively in 31 neonates undergoing the ASO alone (simple TGA) and 9 neonates undergoing the ASO combined with other procedures (complex TGA) (eg, closure of a ventricular septal defect) and correlated with intraoperative and postoperative clinical parameters. RESULTS: There was no mortality. Troponin I peaked at either 4 or 12 hours postoperatively in all patients (median for simple TGA = 3.4 ng/mL, interquartile range 2.4 to 4.6; median for complex TGA = 4.7 ng/mL, interquartile range 3.2 to 6.8, p = 0.20). Peak TnI correlated with the durations of inotropic support (r = 0.54, p < 0.001), ventilation (r = 0.51, p < 0.01), and intensive care unit stay (r = 0.50, p < 0.01). The duration of cardiopulmonary bypass, aortic cross-clamping, and circulatory arrest did not correlate with the peak or total TnI release. The duration of aortic cross-clamping correlated poorly with the duration of inotropic support (r = 0.40, p < 0.05). The complex TGA group had longer aortic cross-clamp times, required more postoperative inotropic support, and had significantly higher total TnI release compared with the simple TGA group. CONCLUSIONS: There are weak but statistically significant correlations between peak TnI and clinical outcome. Complexity of the defect and ischemic times may be as useful to predict outcome in this group of patients.