Pediatr Nephrol (2004) 19:408-412 DOl 10.1007 /s00467 -003-1396-6 ORIGINAL ARTICLE Olanrewaju Adedoyin . Rachel Frank • Suzanne Vento . Marcela Vergara . Bernard Gauthier . Howard Trachtman Cardiac disease in children with primary glomerular disorders- role of focal segmental glomerulosclerosis Received: 7 July 2003 / Revised: 2 December 2003 / Accepted: 3 December 2003 / Published online: 10 February 2004 © IPNA 2004 Abstract Anecdotal reports suggest a higher frequency of serious cardiac complications, particularly cardiomy- opathy and congestive heart failure (CHF), in children with focal segmental glomerulosclerosis (FSGS). We report the occurrence of cardiac disease in children with FSGS compared with other glomerular causes of pri- mary nephrotic syndrome (NS). A chart review was performed on all patients evaluated at the Schneider Children's Hospital between 1985 and 2003 with a diagnosis of membranoproliferative glomerulonephritis (MPGN), membranous nephropathy (MN), focal global glomerulosclerosis (FGGS), and FSGS. Clinical and demographic data were compiled, specifically whether or not the patient had clinically evident cardiac disease. The blood pressure (BP) and hematocrit in patients with FSGS and chronic renal failure (CRF) (glomerular filtra- tion rate <30 ml/min per 1.73 m2) in the 3 months prior to the development of cardiac complications were compared with the values in FSGS patients with CRF but no cardiac complications, and in patients with the other causes of primary NS in whom CRF developed. There were 48 patients with FSGS, 22 with MPGN, 19 with MN, and 4 with FGGS. Cardiac disease occurred in 6 children (mean age 11 years), all with FSGS. Four of these patients were black and 5 were female. CHF occurred in all patients, cardiomyopathy in 4, and left ventricular hypertrophy in 5 O. Adedoyin . R. Frank· S. Vento· M. Vergara· B. Gauthier· H. Trachtman Division of Nephrology, Schneider Children's Hospital of the North Shore-Long Island Jewish Health System, Long Island Campus for the Albert Einstein College of Medicine, New Hyd_e Park, New York, USA H. Trachtman (~) Division of Nephrology, Schneider Children's Hospital, 269-01 76th Avenue, New Hyde Park, New York 11040-1432, USA e-mail: trachtma@lij.edu Tel.: +1-718-4703491 Fax: +1-718-4700887 patients. There was no significant difference in the BP and the hematocrit levels between the 6 patients with both FSGS and cardiac disease, 3 patients with FSGS and CRF but no cardiac disease, and the 5 patients withthe other glomerulopathies in whom CRF occurred (P>O.I). Our findings suggest that there is a clinical association between FSGS and cardiac disease in pediatric patients. We speculate that the immune mechanism responsible for the development of FSGS may also affect the heart. Keywords Cardiac disease . Focal segmental glomerulosclerosis . Primary nephrotic syndrome Introduction Patients with chronic renal failure (CRF) manifest in- creased cardiac morbidity and mortality compared with age-matched controls. This is mainly due to hypertension, electrolyte disorders, volume overload, and anemia [1]. Indices of altered renal function such as micro albumin- uria, overt proteinuria, increased serum creatinine con- centration, or decrease in estimated glomerular filtration rate (GFR) are independent predictors of cardiac morbid- ity and mortality [2, 3]. Most of the clinical observations of cardiac complica- tions of CRF have been made in adults, especially those with diabetes mellitus and hypertension. Cardiac disease in these patients mostly takes the form of myocardial infarction. The emerging importance of cardiac disease in children and young adults with end-stage renal disease (ESRD) is highlighted by a recent report indicating that cardiovascular deaths account for 23% of the mortality in this population [4]. However, the relationship between cardiac disease and CRF and the various types of primary. nephrotic syndrome (NS), in particular, has not been sufficiently examined in pediatric patients. In this report, we describe our experience with serious cardiac compli- cations that developed in children with focal segmental glomerulosclerosis (FSGS) and compare this frequency with the occurrence of cardiac disease in children with