Balloon pulmonary valvuloplasty for pulmonary valve stenosis with atrial septal defect

We performed successful balloon pulmonary valvuloplasty (BPV) in 6 patients with pulmonary valve stenosis and atrial septal defect (ASD) accompanied by atrial left-to-right shunt without an increase of shunting immediately after BPV. It suggests that such patients should be treated by BPV initially and the need for ASD repair can be assessed during long-term follow-up.     

Microangiopathic hemolytic anemia and thrombocytopenia in a child with atrial septal defect and pulmonary hypertension

We found that exposure to room temperature (RT/21 degrees C) causes apoptosis in HL-60 cells. Here we characterized RT-induced apoptosis in HL-60. After exposure to RT, apoptosis starts within 6 h and more than 80% of the cells underwent apoptosis within 20 h. All cells, however, were committed to apoptosis after 16 h and no viable cells could be recovered. The caspase-1 inhibitor (YVAD-CHO) effectively blocked apoptosis, whereas the caspase-3 inhibitor (DEVD-CHO) did not. About 20% of newly obtained early passage HL-60 cells (passage 10) also underwent apoptosis by RT treatment. These data suggest that some population in HL-60 which responds to RT with apoptosis became dominant during passaging.     

Pure epicarditis after surgical repair of atrial septal defect: a rare form of constrictive pericarditis. Apropos of a case

The authors report a case of constrictive pericarditis occurring two years after surgical repair of an atrial septal defect in a 37 year-old-man. The diagnosis was made by right heart catheterisation and magnetic resonance imaging. The special feature of this clinical case was the exclusive localisation of the fibrosis on the visceral pericardium or epicarditis. The outcome was favourable after resection of the visceral pericardium respecting the parietal pericardium. Magnetic resonance imaging and right heart catheterisation performed 6 months after surgery were normal. Constrictive pericarditis is a classical complication of cardiac surgery but relatively rare after repair of an atrial septal defect. Isolated involvement of the visceral pericardium is rare and allows surgical correction by exclusive epicardectomy respecting the parietal pericardium.     

A case of congenital tricuspid regurgitation associated with atrial septal defect and peripheral pulmonary stenosis

This report describes a 5-year-old girl with congenital tricuspid regurgitation associated with an atrial septal defect and peripheral pulmonary stenosis. The girl was diagnosed with the heart murmur at birth and recently developed the cardiomegaly. Cardiac echocardiography and catheterization showed severe tricuspid regurgitation, an atrial septal defect of the secundum type and peripheral pulmonary stenosis. In the operative findings, the tricuspid annulus was dilated to 33 mm in diameter, and leaflets were attached normally to the antomic annulus. There was a large cleft of the anterior leaflet of the tricuspid valve. Suture of the cleft and annuloplasty of the tricuspid valve, suture closure of the atrial septal defect and patch dilatation of peripheral pulmonary stenosis were successfully performed. Including this case, 19 other cases with congenital tricuspid regurgitation undergoing surgery were reported to date.     

Impact of ultrafiltration on blood use for atrial septal defect closure in infants and children

BACKGROUND: Infants and children undergoing open cardiac operations have a high incidence of blood product transfusion. Ultrafiltration has been shown to reverse hemodilution and improve myocardial function and hemodynamics after cardiopulmonary bypass (CPB). METHODS: The effect of ultrafiltration on the amount of blood transfusion and hospital charge in 39 consecutive patients who underwent elective atrial septal defect repair was examined. Patients in group I (n=26) had a conventional cardiopulmonary circuit prime with blood, whereas 13 patients had bloodless prime (group II). Ultrafiltration was used immediately after weaning from CPB in group II. The patients in group I received blood products after discontinuation of CPB to achieve a hematocrit of 30%. The amount of blood product used, hematocrit immediately after CPB and on arrival in intensive care unit, postoperative hemodynamics and saturations, total operating room charge, blood charge, hospital stay, and hospital charge were compared. RESULTS: Mean body weight (15.8 kg in group I versus 17.5 kg in group II) and preoperative hematocrit values (35.6% in group I versus 34.2% in group II) were similar. Mean hematocrit immediately after CPB was 22% and 14% in group I and II, respectively (p < 0.0001). The mean hematocrit upon arrival to the intensive care unit was 34% in group I and 22% in group II (p < 0.0001). The amount of blood product transfusion was 32 mL/kg in group I and 3 mL/kg in group II patients (p < 0.0001). The patients in group II had significantly less blood bank charges; however, operating room charges and total hospital charges were similar between the two groups. CONCLUSIONS: Elective atrial septal defect repair was performed with no blood product transfusion without increased morbidity or hospital stay. Ultrafiltration can be used to reverse hemodilution resulting from a bloodless CPB prime without an increase in hospital charge.     

Diversion of the inferior vena cava into the left atrium after repair of atrial septal defect with partial anomalous pulmonary venous drainage

An 11-year-old female had operative repair of atrial septal defect associated with partial anomalous pulmonary venous drainage by direct suture at 6-year-old. Postoperatively, cyanosis and nodal bradycardia were noted by physical exercise. During 5 years thereafter, the symptoms were gradually aggravated accompanying. Cardiac catheterization revealed diversion of the inferior vena cava into the left atrium. Oxygen sampling showed right to left shunt of 22% at the atrial level. At reoperation, orifice of the inferior vena cava into the right atrium became narrow 5 mm in diameter. After enlargement of the orifice, the atrial septum was created with a Gore-Tex patch to redirect the inferior vena cava to the right atrium. Post operative course was uneventful with disappearance of the cyanosis. The patient is up and well now 9 years after reoperation.     

Successful repair of total anomalous pulmonary venous connection and incomplete endocardial cushion defect associated with left isomerism

Successful repair of a 8-month-old girl with polysplenia was reported. The cardiovascular anomalies were TAPVC (II b), incomplete ECD, interruption of inferior vena cava with hemiazygos continuation, bilateral superior vena cava, and left superior vena cava draining into the coronary sinus. Cardiopulmonary bypass was established with ascending aortic perfusion and caval cannulation. A left superior vena cava was directly cannulated after establishing partial bypass. In this case the left pulmonary vein drained into the right atrium near the orifice of the coronary sinus, so the atrial septal flap was made and sutured between the orifice of the left pulmonary vein and the coronary sinus in order to avoid late pulmonary vein obstruction. Then, atrium was separated by an intraatrial baffle which was sutured to the atrial septal flap. Recently, it becomes possible to surgical repair of polysplenia syndrome according to the advancements of the diagnostic methods, cardiopulmonary bypass, and the technique of the open heart surgery.     

Repair of complete atrioventricular septal defect with severe pulmonary hypertension--effect of re-pulmonary artery banding and analysis of lung biopsy: a case report

HLA-B18 is a well defined Bw6-associated serologic specificity. Up to now, four different sequences have been characterised in Caucasian populations (B*1801,3,4,5), and one in Orientals (B*1802). We report a new HLA-B18 subtype (B*1806) which was serologically detected in a Spanish Caucasian individual as a B18 Bw4-associated antigen. Complete coding region sequencing showed that B*1806 differs from B*1801 in a unique nucleotide at position 299 (A to T), giving rise to an amino acid replacement in residue 76 (glutamic acid to valine) placed at the alpha1 domain. Therefore, in contrast to the serologic results, B*1806 possesses the canonical Bw6 motif at position 77-83. Subsequent flow cytometric assays proved that B*1806 evidences neither Bw4 nor Bw6 epitopes. Only three additional HLA-B alleles encode valine at codon 76, B*4601, B*7301 and B*5503, and like B*1806, all of them would include a Bw6 motif associated to the negative recognition by Bw6 antibodies. These findings support that valine at position 76 will modify the Bw6 epitope drastically, and suggest that this group of HLA-B alleles would define a third, Bw4 and Bw6-negative, lineage of molecules. Furthermore, valine 76 will also prevent the binding of Bw6 antibodies to those HLA-C antigens with the canonical Bw6 epitope (Cw*1,3,7,8,12,13,14,16).     

Survival of a patient with postinfarction ventricular septal defect following venoarterial bypass with centrifugal pump and reoperation for residual shunt

A 61-year-old man was hospitalized because of circulatory collapse due to postinfarction ventricular septal defect. As his hemodynamic condition deteriorated despite intraaortic counterpulsation, he underwent patch closure of VSP and patch reconstruction of the anterior left ventricular wall concomitant with coronary artery bypass grafting to the circumflex lesion immediately after admission. Femorofemoral circulatory assist with centrifugal pump was necessitated to wean from cardiopulmonary bypass because of severe left ventricular dysfunction. Circulatory assist was controlled to maintain mixed venous oxygen saturation of more than 70% under mild hypothermia. On the second postoperative day (POD), increased oxygen saturation from right atrium to pulmonary artery developed (Qp/Qs = 2.1). Further surgery was performed on an emergency basis for additional patch closure of VSP. Then he was successfully weaned from cardiopulmonary bypass successfully. The patient was extubated on the 14th POD and was ambulatory when he discharged on the 56th POD. Immediate surgical intervention should be performed for the patient with postinfarction ventricular septal defect when the hemodynamic state deteriorates under intraaortic counterpulsation.     

Pulmonary vascular disease in a child with atrial septal defect of the secundum type and type I glycogen storage disease

Suppression of cell-mediated immunity (CM) and lowering of survival rate follow major thermal injury are associated with absorption of burn toxin. Wistar rats received 30% full-thickness burn, and were then treated with early burn excision or wet dressing of cerium nitrate for the wound. At day 14 postburn, both survival rates and peripheral blood Th/Ts rate were monitored. It was found that both excision and cerium nitrate could prevent marked lowering of survival rate and Th/Ts ratio were monitored. It was found that both excision and cerium nitrate could prevent markly lowering of survival rates and Th/Ts ratio induced by severe burn. This indicated that CE, in the early postburn days, may replace early burn excision to prevent the suppression of CMI following severe burn.     

Secondary pulmonary hypertension in a patient with severe chronic restrictive pulmonary insufficiency--normalized during treatment with non-invasive intranasal ventilation and oxygen

The present case report describes a young woman with severe restrictive lung failure, who developed pulmonary hypertension. She was treated successfully with nocturnal noninvasive nasal ventilation and oxygen, which removed symptoms and signs of cardiac incompensation. The improvement has persisted for five years.