Pregnancy in women with hypertrophic cardiomyopathy: data from the European Society of Cardiology initiated Registry of Pregnancy and Cardiac disease (ROPAC).

AIMS: We report the maternal and foetal outcomes at birth and after 6 months in a cohort of pregnant women with hypertrophic cardiomyopathy (HCM). Although most women with HCM tolerate pregnancy well, there is an increased risk of obstetric and cardiovascular complications. METHODS AND RESULTS: All pregnant women with HCM entered into the prospective worldwide Registry of Pregnancy and Cardiac disease (ROPAC) were included in this analysis. The primary endpoint was a major adverse cardiovascular event (MACE), which included death, heart failure (HF), thrombo-embolic event, and arrhythmia. Baseline and outcome data were analysed and compared for patients with MACE vs. without MACE and for patients with obstructive HCM vs. non-obstructive HCM. Sixty pregnant women (mean age 30.4 ± 6.0 years) with HCM (41.7% obstructive) were included. No maternal mortality occurred in this cohort. In 14 (23%) patients at least one MACE occurred: 9 (15.0%) HF and 7 (12%) an arrhythmia (6 ventricular and 1 atrial fibrillation). MACE occurred most commonly during the 3rd trimester and postpartum period. In total, 3 (5.0%) women experienced foetal loss. Women with MACE had a higher rate of emergency Caesarean delivery for cardiac reasons (21.4% vs. 0%, P = 0.01). No significant differences in pregnancy outcome were found between women with obstructive and non-obstructive HCM. NYHA functional class of ≥II and signs of HF before pregnancy, were associated with MACE. CONCLUSION: Although most women with HCM tolerated pregnancy well, cardiovascular complications were not uncommon and predicted by pre-pregnancy status facilitating pre-pregnancy counselling and targeted antenatal care.

Variation in the CYP2D6 genotype is not associated with carvedilol dose changes in patients with heart failure.

WHAT IS KNOWN AND OBJECTIVE: Carvedilol is the standard of care for heart failure (HF) patients. Carvedilol is partially metabolized by the highly polymorphic enzyme, CYP2D6. To reach an effective dose while avoiding adverse drug reactions (ADRs), testing of CYP2D6 genotype prior to carvedilol initiation may be considered. The objectives of this study were to determine CYP2D6 metabolic genotypes in an Israeli cohort of HF patients and to investigate the relationship between genotype, carvedilol dose and number of ADRs to determine the importance of CYP2D6 genotyping prior to treatment initiation. METHODS: Ninety-three patients with HF on carvedilol were CYP2D6 genotyped and classified as poor (PM), intermediate (IM), extensive (EM) or ultrarapid (UM) metabolizers. Carvedilol dose and ADRs were calculated and correlated with genotype using linear regression statistic analysis. RESULTS AND DISCUSSION: The distribution of the CYP2D6 phenotype in the Israeli population with HF is similar to the European general population. There were no significant differences of carvedilol dose and number of ADRs among genotype groups. Genotype group affiliation and number of adverse drug reactions were not predictive of carvedilol dose changes. WHAT IS NEW AND CONCLUSION: Genotype group affiliation and number of adverse drug reactions were not predictive of carvedilol dose during therapy for patients with HF. The Israeli CYP2D6 phenotype distribution in HF patients was consistent with the frequency in the general European population.

Aortic valve replacement or heart transplantation in patients with aortic stenosis and severe left ventricular dysfunction.

INTRODUCTION: The decision to perform aortic valve replacement (AVR) or heart transplantation (HTx) for aortic stenosis (AS) with severe left ventricular dysfunction is difficult and may be affected by prior myocardial infarction (MI) and coronary artery disease (CAD). METHODS: Patients who underwent AVR from 1988 to 2001 with left ventricular ejection fraction (LVEF) <30% and severe AS (aortic valve area [AVA] < 1.0 cm(2); n = 51) were assessed for operative mortality, late survival, and predictors of outcome, and were compared with HTx. Subsequently, 131 patients with LVEF ≤ 35% who underwent AVR for critical AS (AVA < 0.8 cm(2)) were evaluated. RESULTS: In the first 51 patients, 3-year survival was 100% ± 0% with no CAD, and 45% ± 10% with CAD (P < .05); 3-year survival was 88% ± 12% with no bypass, 73% ± 12% with one to two grafts, and 18% ± 11% with three grafts (P < .01). Survival with HTx was 78% at 3 years. In the subsequent analysis of 131 patients, 90-day survivors were followed for a mean 4.6 ± 3.5 years. Advanced age (P = .001) was the only predictor of long-term mortality. LVEF improved from 28.5% ± 5.2% before AVR to 45.4% ± 13.2% at 1-month postoperatively (P < .0001). New York Heart Association (NYHA) class III/IV decreased from 94.2% pre-AVR to 12.8% at 1 year (P < .0001). Predictors of LVEF recovery were no previous MI (P = .007) and higher AS gradient (P = .03). CONCLUSIONS: In severe AS and LVEF <30% with no concomitant CAD or with CAD requiring one to two bypass grafts, AVR has a survival equal to or exceeding that of HTx. In patients with CAD requiring more than two bypass grafts, survival is significantly reduced, and HTx can be considered.

Diabetes, but not the metabolic syndrome, predicts the severity and extent of coronary artery disease in women.

BACKGROUND: Previous studies have suggested that diabetes and metabolic syndrome are significant risk factors for coronary artery disease (CAD). However, in women, their relative importance remains controversial. AIM: To evaluate risk factors for CAD in women and their association with the severity and extent of coronary angiographic findings. METHODS: We clinically evaluated 243 consecutive female patients with chest pain who underwent coronary angiography. The location and extent of coronary artery occlusions were assessed using the modified Gensini index. RESULTS: Compared with women with normal coronary arteries (n = 90), those with CAD (n = 153) reported less physical activity (p = 0.001), and had higher prevalences of diabetes (p = 0.046), hypertension (p = 0.002), and the metabolic syndrome (p = 0.001). They also had lower HDL cholesterol levels (p = 0.017), higher levels of triglycerides (p = 0.005), and higher fasting plasma glucose (FPG) (p < 0.001). Physical activity, FPG, serum triglycerides and HDL-cholesterol, but not the metabolic syndrome, were independent predictors of CAD. A score combining the extent and severity of angiographic findings was significantly higher in women with diabetes (p = 0.007), hypertension (p = 0.010) and FPG >or=100 mg/dl (p = 0.031), but showed no association with the metabolic syndrome. In a multivariate linear regression analysis, diabetes was an independent predictor of the extent and severity of angiographic score (p = 0.013). DISCUSSION: Diabetes, fasting plasma glucose and hypertension, but not the metabolic syndrome, were associated with severity of coronary angiographic findings in these women.

Cardiac tamponade as a manifestation of tuberculosis.

Tuberculosis has been increasing in incidence in recent years. Pericardial involvement and pericardial effusions are well-documented and may result in pericardial tamponade. Despite this, large pericardial effusions are uncommon, and manifestation as cardiac tamponade is rare. We report two cases of tuberculous pericarditis in which the initial feature was tamponade. Since the diagnosis of tuberculosis may be delayed due to the slow-growing nature of the bacterium, physicians need to be aware of this possibility and consider the use of modern diagnostic techniques that may permit an earlier diagnosis.

Primary multifocal lymphoma of bone presenting as hypercalcemic crisis: report of a rare manifestation of extranodal lymphoma.

Primary non-Hodgkin's lymphoma of bone is uncommon and usually manifests clinically as localized bone pain. Here we report a woman who presented with hypercalcemic crisis and extensive investigation revealed a primary multifocal lymphoma of bone. The course of the disease was very aggressive and despite intensive supportive care and urgent chemotherapy the patient died within 1 month. Since her blood PTH and calcitriol levels were suppressed and her parathyroid-hormone-related peptide (PTHrp) was mildly elevated, we believe that release of cytokines combined with PTHrp, as well as extensive osteolytic lesions, were the causes of the hypercalcemia. This is an unusual presenting symptom of lymphomas and to the best of our knowledge severe symptomatic hypercalcemia and crisis has never been reported in primary lymphoma of bone before.

Evaluation of carotid arterial plaques after endarterectomy for Helicobacter pylori infection.

Chronic inflammation is linked to atherosclerosis and Helicobacter pylori has been suggested to be an etiologic agent, although the evidence is equivocal. In this report, H. pylori was not detected by the polymerase chain reaction in atherosclerotic plaque from carotid endarterectomy samples.

Idiopathic calcitriol-induced hypercalcemia. A new disease entity?

We describe 3 patients with a prolonged history of hypercalcemia. The patients did not take any vitamin supplements. All patients had low parathyroid hormone (PTH), high calcitriol (1,25(OH)2D3), and high angiotensin-converting enzyme (ACE) blood levels. There was no evidence of sarcoidosis of any other underlying disease in the results of an extensive workup. Treatment with prednisone resulted in normalization of calcium levels in all patients. They remained dependent on low-dose prednisone on a subsequent prolonged follow-up.

[Budd-Chiari syndrome].

Budd Chiari syndrome is a rare disorder resulting from occlusion of hepatic venous drainage by hepatic vein thrombosis or by a membranous web in the inferior vena cava. In western countries the commonest causes are myeloproliferative disorders and hypercoagulable states. Presentation may be acute with rapid accumulation of ascites and hepatic failure, or subacute with symptoms developing over a few months. A chronic progressive form has also been described. On presentation there is usually abdominal pain, ascites, and hepatosplenomegaly; hepatic encephalopathy is found in about a third. Noninvasive, ultrasound-Doppler is recommended in diagnosis, and has a high correlation with hepatic venography. Liver biopsy is required for therapeutic decisions. Those with advanced hepatic failure or severe fibrosis on liver biopsy are referred for hepatic transplantation. When biopsy shows only hepatic congestion and inflammatory infiltrates, portosystemic shunting is recommended. We present a 61-year-old woman with ascites and hepatosplenomegaly that had developed over the courses of a few months. Budd-Chiari syndrome with chronic myelofibrosis and congenital protein C deficiency were diagnosed. Portosystemic shunt was performed but death from sepsis followed shortly.

[Purulent pericarditis].

Purulent pericarditis is diagnosed when pus is drained from the pericardial space or when bacteria are cultured from the pericardial fluid. This rare disease is often diagnosed late, when severe hemodynamic compromise develops due to pericardial tamponade. It is usually a complication of pneumonia, especially if there is empyema as well, and often follows chest surgery or chest wall infections. It sometimes appears in patients with septicemia, especially when they are debilitated or immuno-compromised. Diagnosis is aided by echocardiography. Pericardiocentesis and drainage of the pus, as well as prolonged antibiotic treatment, are mandatory. Delay in diagnosis and treatment often results in death. Some surviving patients may develop constrictive pericarditis and require pericardiectomy. We report a 73-year-old man with pulmonary lymphoma who suffered from purulent pericarditis secondary to sepsis with methicillin-resistant Staphylococcus aureus. Pericardial drainage and appropriate antibiotic treatment eventually resulted in complete recovery.

Enterococcal endocarditis after extracorporeal shock wave lithotripsy for nephrolithiasis.

We report a case of enterococcal endocarditis following extracorporeal shock wave lithotripsy (ESWL) for ureteral stone. Although endocarditis following ESWL is very rare, transient bacteraemia occurs during ESWL. This case is a reminder that enterococcal endocarditis may follow innovative genitourinary procedures without appropriate prophylaxis.