Bilateral perirenal fluid accumulation associated with Eisenmenger's syndrome

Gerhard Pölzl1, Karl Lhotta2, Martin Tiefenthaler2, Alexander Smekal3 and Silvana Müller1

1 Division of Cardiology and 2 Division of Clinical Nephrology, Department of Internal Medicine and 3 Institute of Radiology II, Innsbruck University Hospital, Innsbruck, Austria

Correspondence and offprint requests to: Dr G. Pölzl, Division of Cardiology, Department of Internal Medicine, Innsbruck University Hospital, Anichstrasse 35, 6020 Innsbruck, Austria. Email: gerhard.poelzl{at}uklibk.ac.at

Keywords: Eisenmenger's syndrome; pulmonary hypertension; renal capsule; renal compression

Introduction

Eisenmenger's syndrome results from congenital heart disease with a large left to right shunt causing systemic blood pressure in the pulmonary circulation. In addition to severe pulmonary hypertension, these patients have reduced arterial oxygen saturation and polycythaemia. Kidney function may be impaired and proteinuria is frequently found [1]. Renal histology shows glomerulomegaly, mesangial expansion and focal segmental glomerulosclerosis [2].

Case

A 40-year-old man with Eisenmenger's syndrome due to ventricular septal defect presented with a solid painless mass in the left abdomen. A computed tomography scan demonstrated bilateral perirenal fluid in the subcapsular space (Figure 1A). The inferior aspect of the left kidney appeared compressed by a massive anterior cystic formation (Figure 1B). A 24 h urine collection showed 1.3 g proteinuria and a creatinine clearance of 64 ml/min. Haematocrit was 73.7%, PO2 was 41.6 mmHg and blood pressure was 140/100 mmHg.




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Fig. 1. Computed tomography scan of the abdomen. Frontal (A) and sagittal (B) views.

 
Perirenal fluid accumulation has been reported previously in Eisenmenger's syndrome [3]. We speculate that pulmonary hypertension is transmitted to the renal venous system and causes transsudation of fluid out of superficial stellate veins into the subcapsular space. Perirenal fluid in our patient probably caused a renal compression syndrome with reduced glomerular filtration rate, increased intrarenal vascular resistance and hypertension. Whether and how this condition should be treated is unknown, but repeated phlebotomies have been reported to be beneficial [3].

Conflict of interest statement. None declared.

References

  1. Flanagan MF, Hourihan M, Keane JF. Incidence of renal dysfunction in adults with cyanotic congenital heart disease. Am J Cardiol 1991; 68: 403–406[CrossRef][ISI][Medline]
  2. Hida K, Wada J, Yamasaki H et al. Cyanotic congenital heart disease associated with glomerulomegaly and focal segmental glomerulosclerosis: remission of nephrotic syndrome with angiotensin-converting enzyme inhibitor. Nephrol Dial Transplant 2002; 17: 144–147[Free Full Text]
  3. Pentimone F, Meola M, Del Corso L. Bilateral perinephric fluid accumulation: an unusual manifestation of pulmonary hypertension – a case report. Angiology 1993; 44: 500–505[ISI][Medline]




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