Open heart operation in a young child with spherocytosis

Kawahira, Youichi; Kishimoto, Hiroyuki; Lio, Masahiko; Ikawa, Seiichiro; Ueda, Hiroyuki; Kayatani, Futoshi; Nakada, Taka‐aki

doi:10.1016/0003-4975(94)90481-2

Cited by 10 publications

(28 citation statements)

References 4 publications

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“…7 Administration of haptoglobin decreases the serum free hemoglobin concentration and prevents secondary organ failure. 7 Hara et al report a 10-year-old girl with HS who underwent successful atrial septal defect closure, using poloxamer 188, haptoglobin, and a centrifugal pump during CPB, which they concluded was effective for preventing intraoperative hemolysis. 5 Because splenectomy is a very effective treatment for reducing hemolysis, thus leading to a significant prolongation of the red cell lifespan, 1 it has been advocated before open heart surgery in patients with HS to prevent hemolysis during and after surgery.…”

Section: Discussionmentioning

confidence: 99%

“…Previous reports recommend the use a non-ionic antihemolytic detergent, poloxamer 188, or haptoglobin. 5,7,8 Poloxamer 188 protects the red blood cell membranes while not increasing the serum free hemoglobin concentration during CPB. 7 Administration of haptoglobin decreases the serum free hemoglobin concentration and prevents secondary organ failure.…”

Section: Discussionmentioning

confidence: 99%

“…5,7,8 Poloxamer 188 protects the red blood cell membranes while not increasing the serum free hemoglobin concentration during CPB. 7 Administration of haptoglobin decreases the serum free hemoglobin concentration and prevents secondary organ failure. 7 Hara et al report a 10-year-old girl with HS who underwent successful atrial septal defect closure, using poloxamer 188, haptoglobin, and a centrifugal pump during CPB, which they concluded was effective for preventing intraoperative hemolysis.…”

Section: Discussionmentioning

confidence: 99%

“…The systolic pressure gradient between the right ventricle and distant main pulmonary trunk was 49 mmHg. Although open heart surgery using CPB has the risk of hemolytic crisis, the most recent reports 5,7 suggest that the results for patients with HS without splenectomy are acceptable. Therefore, the patient was scheduled for open heart surgery at the age of 18 months.…”

mentioning

confidence: 99%

“…1 There are only a few case reports of patients with HS who have undergone open heart surgery. [2][3][4][5][6][7][8][9] Theoretically, these patients have a high risk of perioperative hemolysis and secondary renal dysfunction attributable to the deleterious effects of cardiopulmonary bypass (CPB). Because limited information is available concerning such operations for patients with HS, we report the findings of an 18-month-old child with congenital heart disease and HS.…”

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confidence: 99%

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Open Heart Operation in a Child With Congenital Heart Disease and Hereditary Spherocytosis

Yoshimura

Murakami

Otaka

et al. 2006

Circ J

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ereditary spherocytosis (HS) is a genetically determined red blood cell membrane disorder that results in hemolytic anemia. 1 There are only a few case reports of patients with HS who have undergone open heart surgery. [2][3][4][5][6][7][8][9] Theoretically, these patients have a high risk of perioperative hemolysis and secondary renal dysfunction attributable to the deleterious effects of cardiopulmonary bypass (CPB). Because limited information is available concerning such operations for patients with HS, we report the findings of an 18-month-old child with congenital heart disease and HS. Case ReportA 5-month-old girl was referred to hospital after a diagnosis of ventricular septal defect (VSD) and pulmonary stenosis (PS). She was suspected to have HS because her father and grandmother were known to have suffered from HS. Her red blood cells showed increased osmotic fragility, and a diagnosis of HS was made. At the ages of 7 and 17 months, 10 ml/kg of packed red blood cells were transfused in order to treat the progressive anemia. Gradually, the pressure gradient between the right ventricle and pulmonary artery decreased, and left ventricular volume increased (Table 1). On admission, the patient was anemic and icteric. She weighed 11.9 kg. Physical examination revealed a systolic ejection murmur at the left second intercostal space and splenomegaly. Hematologic investigation revealed a hemoglobin level of 7.2 g/dl, and reticulocyte count of 36.8%. The total bilirubin level was 2.8 mg/dl (Table 2). Two-dimensional echocardiography showed a large perimembranous VSD with left-to-right shunt, and valvular and supravalvular PS. The pulmonary annulus measured 12 mm and the main pulmonary trunk measured 6 mm. Circulation Journal Vol.70, December 2006Echocardiography also showed non-compaction of the left ventricular muscle. Cardiac catheterization confirmed the left-to-right shunt, with a ratio of pulmonary blood to systemic blood flow (Qp/Qs) of 1.78:1. The left ventricle was dilated to 195% of the normal volume, but overall left ventricular function was normal. Central venous pressure and pulmonary capillary wedge pressure was 9 mmHg and 13 mmHg, respectively. The systolic pressure gradient between the right ventricle and distant main pulmonary trunk was 49 mmHg. Although open heart surgery using CPB has the risk of hemolytic crisis, the most recent reports 5,7 suggest that the results for patients with HS without splenectomy are acceptable. Therefore, the patient was scheduled for open heart surgery at the age of 18 months. Her parents were informed of the purpose and risk of the surgical treatment, and they gave written informed consent.She underwent closure of the VSD, commissurotomy of the pulmonary valve, and patch angioplasty of the pulmonary trunk using autologous pericardium. The CPB circuit consisted of a roller pump and a membrane oxygenator, and the CPB time was 150 min. In order to maintain the hematocrit level and osmotic pressure of the priming solution, 150 ml of packed red blood cells and 20 ml of ...

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

mentioning

confidence: 99%

mentioning

confidence: 99%

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Open Heart Operation in a Child With Congenital Heart Disease and Hereditary Spherocytosis

Yoshimura

Murakami

Otaka

et al. 2006

Circ J

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Hereditary spherocytosis and elliptocytosis associated with prosthetic heart valve replacement: rheological study of erythrocyte modifications

et al. 2009

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The implantation of a prosthetic heart valve (HVP) in patients with hereditary spherocytosis (HS) and hereditary elliptocytosis (HE) is rare, and the changes in the structure and deformability of erythrocytes that follow implantation in these patients have been poorly described. In the present study, the erythrocytes in HS and HE patients with mechanical HVP were compared to the erythrocytes in patients with only congenital membrane defects, in terms of biochemical modifications and rheological behaviour. Integral and cytoskeletal erythrocyte membrane proteins were studied, and blood viscosity (shear rate/shear stress ratio), aggregation ratio [eta(1 s(-1))/eta(200 s(-1))], and red cell visco-elasticity were determined. Valve replacement with a mechanical prosthesis worsened anaemia and resulted in a change in haemolysis, from sub-clinical to evident. The rheological investigation of erythrocytes from HS patients confirmed the characteristic increased viscosity and aggregation ratio and the decreased deformability. The rheological behaviour of erythrocytes from patients with HVP showed a decrease in viscosity and an increase in elastic modulus. In these patients, the prosthesis seems to have induced traumatic damage to the erythrocyte membrane, leading to fragmentation and lysis, which in turn modified rheological parameters. The biochemical and rheological investigation allowed us to understand the clinical and haematological pictures of the patients and to describe the role played by different factors in haemolytic anaemia.

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The Successful Use of an Exchange Transfusion in a Child with Hereditary Spherocytosis Undergoing Congenital Cardiac Surgery

Stevens,

Buckley,

Schwartz

et al. 2021

J Extra Corpor Technol

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Little is reported in the literature regarding hereditary spherocytosis (HS) and cardiopulmonary bypass (CPB). We present a case of a 19-month-old girl child who was referred for an atrioventricular septal defect (AVSD) and HS. The patient underwent surgical repair, and an exchange transfusion was performed at the initiation of CPB. No significant hemolysis or events attributed to HS were observed during or after CPB. The surgical repair of an AVSD in a pediatric patient with HS and total volume exchange transfusion is herein reported.

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Open heart operation in a young child with spherocytosis

Cited by 10 publications

References 4 publications

Open Heart Operation in a Child With Congenital Heart Disease and Hereditary Spherocytosis

Open Heart Operation in a Child With Congenital Heart Disease and Hereditary Spherocytosis

Hereditary spherocytosis and elliptocytosis associated with prosthetic heart valve replacement: rheological study of erythrocyte modifications

The Successful Use of an Exchange Transfusion in a Child with Hereditary Spherocytosis Undergoing Congenital Cardiac Surgery

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