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

Abstract

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 ...

Open heart surgery in an infant with hereditary spherocytosis and a review of literature

“…A literature review gives only a few congenital heart disease cases with hereditary spherocytosis undergoing open‐heart surgery (Table 2). 2–6 Our case is the only reported infant with hereditary spherocytosis to undergo open‐heart surgery.…”

“…Kawahira et al 3 reported a 15‐month‐old child who underwent open‐heart surgery without a previous splenectomy, and they conclude that splenectomy before cardiac operations in children with HS may not always be necessary. Use of non‐ionic antihemolytic detergent poloxamer 188 and haptoglobin has been reported 2,3,5 . Poloxamer 188 protects the red cell membrane and prevents the increase of serum‐free hemoglobin.…”

Orthotopic Heart Transplantation in a Child With Hereditary Spherocytosis

“…Although HS occurs as often as in 1 in 2000 people (1), there is surprisingly little published about HS management during open heart surgery. We found only 21 reported cases of which only 7 (Table 2) were less than 18 years of age at the time of surgery (2–8). There were no reports of patients with HS undergoing heart transplantation.…”

“…The risk for hemolysis during CPB in patients with HS is largely theoretical. A variety of approaches have been advocated for HS patients requiring cardiac operation including avoidance of cardiopulmonary bypass, pre‐emptive splenectomy (9), administration of haptoglobin to reduce plasma free hemoglobin (3,4,8), using poloxamer 188 (a non‐ionic antihemolytic detergent that protects RBC membranes during CPB) (8), or simply proceeding with surgery employing CPB without any special measures (2,5,7). No significant hemolysis or renal failure occurred in any of these reports.…”

Activation of Endothelin-1 Receptor Signaling Pathways Is Associated With Neointima Formation, Neoangiogenesis and Irreversible Pulmonary Artery Hypertension in Patients With Congenital Heart Disease

“…There is much argument and uncertainty about the benefits or risks of corrective surgery of the underlying anatomical defect. 3, 4 It is unclear why some patients undergoing complete repair or palliative surgery of the CHD still develop irreversible PAH. The pathological mechanisms of pulmonary vasculopathy are poorly understood in patients with irreversible PAH secondary to CHD.…”