Year : 2012  |  Volume : 15  |  Issue : 4  |  Page : 305--308

Anesthetic management of a child with nephrotic syndrome undergoing open heart surgery: Report of a rare case

Vishnu Datt, Deepak K Tempe, Kapil Arora, Sanjula Virmani, Chander S Joshi, Saket Agarwal 
 Department of Anaesthesiology, Intensive Care and Cardiothoracic and Vascular Surgery, G.B. Pant Hospital, New Delhi, India

Correspondence Address:
Vishnu Datt
Department of Anesthesiology and Intensive Care, Academic Block, Room no. 619, G.B. Pant Hospital, New Delhi - 110 002


The congenital nephrotic syndrome (NS) in infancy and childhood is an important entity but combination with acyanotic congenital heart disease is uncommon. Anesthesia in such cases is challenging because of associated problems like hypo-protienemia, anti-thrombin III deficiency, edema, hyperlipidemia, coagulopathy, cardiomyopathy, immunodeficiency, increased lung water etc. We describe anesthetic management of a patient with childhood NS and sinus venosus atrial septal defect (ASD) undergoing open heart surgery. We also suggest guidelines for safe conduct of anesthesia and CPB in such patients.

How to cite this article:
Datt V, Tempe DK, Arora K, Virmani S, Joshi CS, Agarwal S. Anesthetic management of a child with nephrotic syndrome undergoing open heart surgery: Report of a rare case.Ann Card Anaesth 2012;15:305-308

How to cite this URL:
Datt V, Tempe DK, Arora K, Virmani S, Joshi CS, Agarwal S. Anesthetic management of a child with nephrotic syndrome undergoing open heart surgery: Report of a rare case. Ann Card Anaesth [serial online] 2012 [cited 2021 Jan 23 ];15:305-308
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Full Text


Nephrotic syndrome (NS) has been recognized in pediatric patients. Sequelae of NS have many important perioperative implications due to hypo-proteinemia, anti-thrombin III deficiency, edema, hyperlipidemia, coagulopathy and cardiomyopathy, which pose a great challenge to the anesthesiologist. To the best of our knowledge, this is the first reported case of sinus venosus type of atrial septal defect (ASD) with childhood NS undergoing open heart surgery wherein the peri-operative anesthetic management is discussed. Authors also suggest various strategies during cardiopulmonary bypass (CPB) to achieve favorable outcome after cardiac surgery.

 Case Report

An 8-year-old- boy weighing 21 kg presented with childhood NS and sinus venosus ASD. He had generalized swelling and abdominal distention. The heart rate was 84/min and blood pressure was 126/70 mmHg. Airway assessment was normal. Air entry was reduced in both lower zones. Blood biochemistry revealed hypo-albuminemia (serum albumin - 2 g/dl) and urine examination was suggestive of severe proteinuria exceeding 4 g/L. Other hematological and biochemical investigations were within normal limits. Cardiovascular examination revealed ejection systolic murmur at the left upper sternal border. Electrocardiogram (ECG) showed normal sinus rhythm and right ventricular (RV) hypertrophy. Chest radiograph showed cardiomegaly and bilateral pleural effusion. Ultrasonography of abdomen showed free fluid in the peritoneal cavity. Transthoracic echocardiography revealed 1.2 cm sinus venosus ASD with left to right shunt and normal pulmonary venous drainage. The patient was treated with oral prednisolone (10 mg three times daily), amoxicillin with clavulanic acid (325 mg twice daily) and cough expectorant (ambroxolol, guaiphenesin). There was a gradual improvement in his clinical status. The patient was scheduled for closure of the sinus venosus ASD under CPB. Premedication consisted of intramuscular morphine (4 mg) and promethazine (10 mg). In the operating room, ECG leads and pulse oximetry probe were attached and a venous cannula and radial artery catheter were inserted under local anesthesia. Anesthesia was induced with fentanyl (200 μg) and thiopentone (25 mg) and atracurium (12 mg) was administered to facilitate tracheal intubation with a 6 mm endotracheal tube. Lungs were ventilated with 50% O2 in air using tidal volume 200 ml and a respiratory rate of 15 per minute. A 5.5 Fr triple lumen central venous catheter was inserted via right internal jugular vein. Anesthesia was maintained with intermittent midazolam (0.05 mg/kg), fentanyl (2.5 μg/ kg/hr), atracurium (5 mg/ hr) and isoflurane (0.5-2%) before CPB and propofol (6 mg/kg/ hr) infusion during CPB. The CPB circuit was primed with Hartman's solution 400 ml, Blood 300 ml and 50 ml 25% albumin; mannitol 10 gm, furosemide 10 mg and methylprednisolone 500 mg were also added to the prime. Heparin (3 mg/kg) was administered to achieve activated clotting time (ACT) >480 sec. The sinus venosus ASD was closed with a pericardial patch under standard mild hypothermic CPB with potassium enriched cardioplegic myocardial protection. During CPB the arterial flow was kept at 2.4 L/m 2 and mean arterial pressure (MAP) was maintained between 50-70 mmHg. After completition of the surgical procedure, the patient was weaned off CPB without any difficulty. Total CPB time and ischemia time were 38 min and 22 min, respectively. Heparin was neutralized with protamine (70 mg) and after securing hemostasis the chest was closed. Total urine output during surgery was 800 ml and he received two units of fresh frozen plasma (FFP) perioperatively. Post-operatively, patient was put on intravenous hydrocortisone 50 mg, 8 hourly for 48 hrs and then switched over to oral prednisolone. Trachea was extubated after 12 hrs in the cardiac intensive care unit (ICU). Rest of the postoperative course was uneventful. Ascites and peripheral edema subsided with steroid therapy and the patient was discharged from the hospital on 12 th postoperative day.


NS in infancy and childhood is an important entity. The incidence of NS ranges from 2-7 per 100,000 children and a preponderance in boys is established. [1],[2] NS comprises of proteinuria, hypo-albuminemia (<2.5 g/ dl) and edema. Nephrotic-range proteinuria in children is protein excretion of more than 40 mg/m 2 /h. Heavy proteinuria (0.1 g/kg body weight/24 hr) is the basic abnormality leading to hypo-protinemia. A spot (random) urinary protein to creatinine ratio exceeding 2 (2 mg/1 mg) indicates nephrotic range proteinuria and correlates with results from 24-hr urine collection. [3]

Hypo-proteinemia affect the balance between hydrostatic pressure and colloid osmotic pressure leading to increased loss of fluid from the capillaries, increasing the likelihood of pulmonary edema formation. Edema is the presenting symptom in about 95% of children with NS. Complications associated with NS in children include increased incidence of renal failure, hypercoagulability, thromboembolism, restricted cardiomyopathy, infection, hyperlipidemia, hematuria (microscopic or gross) and others like biventricular hypertrophy and systolic anterior motion of the mitral valve due to high dose steroid (prednisone >20 mg/day) and immunosuppressive therapy. [4],[5],[6],[7] Thus, the anesthetic management should consider the following issues; renal protection, hypo-proteinemia, heparin resistance, fluid and electrolyte balance, and corticosteroid therapy.

There are multiple risk factors for developing post-operative renal dysfunctions such as low cardiac output, low renal blood flow, renal athero-embolism, use of nephrotoxic drugs, hypo-calcemia, hyper-uricemia, hemoglobinuria, myoglobinuria, oxygen free radical generation, local leukocyte activation, exogenous catecholamine administration, and nuclear factor- kappa B (NF-kB) activation following prolonged CPB. [8] A low hematocrit during CPB may also precipitate acute renal failure mainly due to low oxygen delivery. However, it can be avoided by increasing the oxygen delivery with an adequately increased pump flow. [9] The renal functions can be preserved during cardiac surgery with the use of mannitol, furosemide and by maintaining adequate hydration and avoiding over hydration, nephrotoxic agents, antibiotics and non steroidal anti-inflammatory drugs. [10] Diuretics have reno-protective effect by increasing tubular solute flow, urine generation, maintaining tubular patency and keeping water balance in the body, thereby avoid oliguria, anuria and need for dialysis. [11] However, other clinical trials have shown no benefit or possibly even harm and increased post operative serum creatinine levels in such patients with post operative diuretic therapy. [12] Several authors have used fenoldopam (0.1-0.3 μg/kg/min) during CPB and early post operative period in patients with serum creatinine >1.5 mg/dl and observed an improvement in post operative renal functions. [13],[14]

The authors utilized a balanced general anesthesia technique and a short CPB wherein mannitol, furosemide, methylprednisolone were administered and MAP was maintained between 50-70 mmHg to maintain the renal blood flow. Atracurium and cisatracurium are the muscle relaxants of choice as these undergo spontaneous Hoffman degradation at body temperature. All the modern inhalational anesthetics like isoflurane, desflurane, enflurane and halothane are suitable in patients with mild to moderate renal disease. It has also been observed that renal blood flow is maintained with isoflurane, desflurane and halothane. [15]

Hypo-proteinemia is the major concern during anesthetic management in NS, as the available binding sites for intravenous anesthetics are decreased in hypo-albuminemia. As thiopentone and benzodiazepines extensively bind to albumin, their free fraction is increased and a greater proportion of the administered drug reaches to the receptor sites. [16] Their metabolites are also pharmacologically active and so potentiate their clinical effect. However, dose recommendations are not available in the literature. Although, free fraction of fentanyl, alfentanil, propofol and etomidate is increased, no delay in recovery after administration is expected, due to lack of free metabolites and short redistribution of these agents. Therefore, these agents appear to be a better choice in patients with hypo-albuminemia., [13],[14],[17] Anti-fibrinolytic agents (aprotinin, and epsolin-aminocaproic acid) have a tendency to precipitate tubular proteinuria, and should be avoided in such patients. [9] Some patients with NS can develop hypo-proteinemia induced myocardial edema which is associated with left ventricular diastolic dysfunction. [18] Therefore, addition of albumin and fresh blood in the prime to maintain hematocrit (>26%) and albumin levels (>2.5 g/dl) is advisable. In conjunction with diuretic therapy, a short-term use of 25 percent albumin is appropriate for patients with acute severe peripheral or pulmonary edema who have failed diuretic therapy. Albumin administration also appears to exert protective effects on the kidney by inhibition of apoptosis and scavenging of reactive oxygen species. [19] Fritzand et al. have reported that post-operative serum albumin values <18 g/l and procalcitonin >2.5 ng/l are predictive for a higher 28-day mortality rate in cardiac surgical patients. [18] In fact it has been considered a better outcome predictor than the Euro score, which represents the pre-operative condition of the patient. [20] Davari and colleagues have also reported that after pediatric cardiac surgery low albumin level is associated with an increased risk of pericardial effusion, renal failure, seizures and death. [21] In the present patient albumin and whole blood were added to the prime solution and FFP and fresh blood were administered in the perioperative period to maintain the serum protein levels.

Sodium retention by kidney precedes proteinuria induced generalized edema. Sodium reabsorption by distal renal tubules is increased due to inappropriately low natriuretic response to atrial natriuretic peptides. Therefore the use of loop diuretics and/or thiazide diuretics or potassium sparing diuretics to decrease sodium reabsorption in the distal nephrons is expected to be beneficial. Additionally, sodium containing fluids should be restricted and albumin solution be administered along with plasma ultrafiltration during CPB to control serum sodium levels and generalized edema. [22] In such patients, heparin resistance may also be encountered due to deficiency of Anti-thrombin III and plasminogen and may require FFP or whole fresh blood transfusion to achieve a target ACT (>480 sec). [23]

Prolonged corticosteroid therapy may precipitate sodium and fluid retention, potassium loss, increase in blood pressure and blood sugar levels, and also increases the susceptibility for infections, thromboembolism and impaired wound healing. [15] Therefore, perioperative monitoring should include blood pressure, blood sugar, body weight, and serum electrolytes. Dosage of steroids needs to be reduced if patient develops the described complications. Patients on chronic steroid therapy may require prolonged postoperative mechanical ventilation and likely to suffer from atrial arrhythmias. [24] Perioperatively steroid therapy should be continued to avoid the hemodynamic instability. [25] In such patients thromboembolic complications occur between 1.8% to 6.6% that justifies prophylactic administration of oral anticoagulants. [26]

The recommendations regarding CPB management in these patients are not available in the literature. Based on reported clinical experiences and pathophysiological concepts various strategies to achieve favorable outcome after cardiac surgery can be recommended and include the use of balanced anesthetic technique (fentanyl, propofol, isoflurane, nitrous oxide, midazolam, and atracurium with moderate hyperventilation),whole blood and albumin in prime solution, a pump flow of 2.4 L/m 2 to maintain MAP of 60-65 mmHg, steroid therapy perioperatively, and avoidance of acidosis and maintaining electrolyte balance (Na + ,K + ,Mg 2+ , Ca 2+ ). [13],[18],[19],[27]

In conclusion, sequelae of NS have many implications due to hypo-proteinemia, anti-thrombin III deficiency and cardiomyopathy. The case report demonstrates that cardiac surgery using CPB can be safely performed under balanced general anesthesia in patients with NS.


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