Letter to the Editor
Saime Paydas
Esmeray Acarturk
Cukurova University Medical Faculty,
Departments of Nephrology and Cardiology,
Balcali Hospital, Adana. Turkey
Dear Sir,
In this study we describe a case of horse
shoe kidney in association with the endocar
dial cushion defect.
A 33-year-old male was admitted to hos
pital for hypertension. Six years ago he had
been hospitalized for lead intoxication. He
was working in a storage battery. At that time
bradycardia was detected. Cardiac investiga
tion showed a congenital endocardial
cushion defect. He had an Autime 2 model
dual chamber pace maker implanted. He was
treated 4 times for lead intoxication after
wards. Finally he stopped actively working
with lead. Two years ago at his latest ad
mission hypertension was found but he did
not regularly take medicine for hypertension.
Physical examination revealed a pulse of 76/
min, blood pressure of 190/130 mm Hg, 3/6
holosystolic murmur in the mesocardiac and
apical region. Laboratory examinations were
as follows: hematocrit 48%, WBC 4,800/mm3,
platelet count 200.000/mm'. BUN 20 mg/dl,
glucose90 mg/dl, Na 139 mEq/I, K4.7 mEq/
I, alkaline phosphatase 88 IU/I, AST 54 IU/1,
ALT 27 IU/I, uric acid 5.2 mg/dl, Ca 8.8
mg/dl, P 3.2 mg/dl, creatinine clearance 98
ml/min : proteinuria was negative, urine sedi
ment was normal. Telecardiography dis
closed cardiomegaly and pacemaker image.
Left ventricular hypertrophy and pacemaker
rhythm were determined on electrocardio
graphy. With the échocardiographie examin
ation a perimembranous ventricular defect
and a primum atrial septal defect were found
(fig. I). A rapid-sequence intravenous pyelo-
gram and renal computerized tomography
disclosed a horseshoe kidney. Enalapril ma-
leate (10 mg/day) was started. Follow-up
hypertension was controlled with enalapril
maleate.
Nephron 1993:63:479
A Case with Endocardial Cushion
Defect and Horseshoe Kidney
Fig. 1 . Apical
four-chamber view
of the patient with
cushion defect
showing the peri
membranous ventri
cular septal defect
(VSD) and primum
atrial septal defect
(ASD).
He had been followed for 2 years and
blood pressure and cardiac and renal func
tion were stable.
Horseshoe kidney is a relatively common
condition (1 in 400 of the population). Two
kidneys fuse across the midline, most com
monly at the lower poles. Other developmen
tal abnormalities such as Turner’s syndrome
or Trisomy 18 accompany the horseshoe kid
ney. [1]. No chromosomal abnormality was
found in our patient. In the literature renal and
cardiac abnormalities together such as Down
syndrome and renal dysplasia, multiple renal
cysts and Ehlers-Danlos syndrome and Kauf
man syndrome with heart disease and bifur
cation of renal pelvis or ureteral ectasia have
been reported [3]. Huber et al. [2] have found
three cases of abdominal aortic aneurysm in
association with a horseshoe kidney. We pre
sented here a case of a horseshoe kidney in
association with an endocardial defect.
References
1 Warkany J. Passarge E. Smith LB: Congenital
malformations in autosomal trisomy syn
dromes. Am J Dis Child 1966:112:502.
2 Huber D, Griffin A, Niesche J, Frawley J, Gray
L: Aortic aneurysm surgery in the presence of a
horseshoe kidney. Aust NZ J Surg 1990:60:
963-966.
3 Moffat, DB: Development abnormalities of the
urogenital systems; in Chisholm GD, Williams
D1 (eds): Scientific Foundations of Urology.
Chicago, Year Book. 1982, pp 357-374.
Dr. Saime Paydas © 1993 S. Karger AG. Basel
Cukurova University Medical Faculty 0028-2766/93/
Departments of Nephrology and Cardiology 0634-0479S2.75/0
Balcali Hospital
TR-01330 Adana (Turkey)