Prevalence, predictors, and prognostic value of renal dysfunction in adults with congenital heart disease.

BACKGROUND: Renal insufficiency in patients with ischemic heart disease and acquired heart failure is associated with higher mortality and morbidity. We studied the prevalence of renal dysfunction in adult patients with congenital heart disease (ACHD) and its relation to outcome. METHODS AND RESULTS: A total of 1102 adult patients with congenital heart disease (age 36.0+/-14.2 years) attending our institution between 1999 and 2006 had creatinine concentration measured. Glomerular filtration rate (GFR) was calculated with the Modification of Diet in Renal Disease equation. Patients were divided into groups of normal GFR (> or =90 mL . min(-1) . 1.73 m(-2)), mildly impaired GFR (60 to 89 mL . min(-1) . 1.73 m(-2)), and moderately/severely impaired GFR (<60 mL . min(-1) . 1.73 m(-2)). Survival was compared between GFR groups by Cox regression. Median follow-up was 4.1 years, during which 103 patients died. Renal dysfunction was mild in 41% of patients and moderate or severe in 9%. A decrease in GFR was more common among patients with Eisenmenger physiology, of whom 72% had reduced GFR (<90 mL . min(-1) . 1.73 m(-2), P<0.0001 compared with the remainder), and in 18%, this was moderate or severe (P=0.007). Renal dysfunction had a substantial impact on mortality (propensity score-weighted hazard ratio 3.25, 95% CI 1.54 to 6.86, P=0.002 for moderately or severely impaired versus normal GFR). CONCLUSIONS: Deranged physiology in adult patients with congenital heart disease is not limited to the heart but also affects the kidney. Mortality is 3-fold higher than normal in the 1 in 11 patients who have moderate or severe GFR reduction.

Afro-Caribbean Heart Failure in the United Kingdom: Cause, Outcomes, and ATTR V122I Cardiac Amyloidosis.

BACKGROUND: It has been reported that subjects of African descent present with heart failure at a younger age and because of different causes than whites. We present contemporary data from UK Afro-Caribbean patients in London. METHODS AND RESULTS: All patients with heart failure presenting to St George's Hospital Heart Failure clinic between 2005 and 2012 were included (n=1392). Patients were predominantly white (71%) and male (67%), and median age at presentation was 73 years (range, 18-100 years). In 211 Afro-Caribbean patients, the most common cause of heart failure was nonischemic dilated cardiomyopathy in 27.5% (whites, 19.9%; P<0.001). Lower rates of ischemic cardiomyopathy were observed (13% versus 41%; P<0.001). The fourth most common cause of heart failure in Afro-Caribbeans was cardiac amyloidosis (11.4%). The prevalence may have been even higher as not all patients were tested for amyloidosis. Patients with ATTR V122I had the worst prognosis compared with other causes of Afro-Caribbean heart failure and white patients. To better understand this condition, we analyzed data from the largest international cohort of ATTR V122I patients, followed up at the UK National Amyloidosis Center (n=72). Patients presented with cardiac failure (median age, 75 [range, 59-90] years). Median survival was 2.6 years from diagnosis. CONCLUSIONS: In London, the cause of heart failure varies depending on ethnicity and affects age of presentation and outcomes. In Afro-Caribbean patients, ATTR V122I is an underappreciated cause of heart failure, and cardiomyopathy is often misattributed to hypertension. As promising TTR therapies are in development, increased awareness and proactive detection are needed.

Ischaemic heart disease in the ageing woman.

Cardiovascular disease is one of the leading causes of death worldwide. Since 1984, the total number of deaths from cardiovascular disease has been greater for women compared with men. This might be, in part, related to gender-specific differences in the presentation of people with chest pain but, importantly, the risk of heart disease in women is often underestimated owing to the misperception that women are 'protected' against cardiovascular disease. Studies have shown that medical services are underused, delays occur in health-care seeking behaviour, resource use patterns are less intensive, and it takes longer to diagnose women compared with men. This often leads to less aggressive treatment strategies which, in turn, may translate into poorer outcomes compared with those in men. In this chapter, we review the gender-specific differences in epidemiology, diagnosis, and management of people with ischaemic heart disease, and focus particularly on the effect of ageing and menopause in ischaemic heart disease.

Near miss sudden cardiac death on a young patient with repaired atrioventricular septal defect.

Patients with congenital heart disease often face the prospect of long-term haemodynamic or arrhythmic complications for which lifelong follow-up in specialist adult congenital heart disease (ACHD) centres is required. We describe the case of a 25-year-old man with repaired atrioventricular septal defect who was referred to our centre after a ventricular fibrillation arrest. Serial echocardiograms in previous years had shown progressive severe left ventricular outflow obstruction, but the patient had not been operated on as he was deemed asymptomatic and reluctant to consider surgery. Management and criteria for further intervention in ACHD patients often differ from those of patients with acquired heart disease and reliance on symptoms alone is not good practice and may prove catastrophic.