Extracorporeal membrane oxygenation with heparin-coated systems in a 13-month-old infant with acute hypoxic respiratory failure after correction of tetralogy of Fallot

Hemorrhagic disorders due to systemic heparinization are frequent during extracorporeal lung support (veno-venous extracorporeal membrane oxygenation: vv-ECMO). The development of heparin-coated systems has reduced the need for high-dose heparinization. Whereas the use of these heparin-coated membrane lungs and tubings has been described in former studies in adults, only few reports exist in children. This case report describes the application of a heparin-coated extracorporeal system for long-term vv-ECMO in a 13-month-old infant suffering from acute hypoxic respiratory failure after correction of tetralogy of Fallot. Only moderately elevated levels of activated clotting time (ACT, 120-160 s) and activated partial thromboplastin time (aPTT, 40-60 s) were necessary to avoid thrombotic events in the extracorporeal system. Thoracotomies were performed twice without bleeding complications by discontinuation of the systemic heparinization. We conclude that the use of heparin-coated membrane lungs in infants may improve the safety of extracorporeal lung support and permits surgical intervention without major risk of bleeding.     

Surgical treatment of ventricular tachycardia after surgical repair of tetralogy of Fallot. Relation between intraoperative mapping and histological findings

BACKGROUND: The mechanism of ventricular tachycardia (VT) after correction of tetralogy of Fallot (TF) is poorly understood. The purpose of this study was to examine the histopathology of the arrhythmogenic area detected by intraoperative mapping. METHODS AND RESULTS: The patients were three men who underwent radical surgery for TF at age 3, 3, or 5 years, respectively. VT developed at 8, 9, or 11 years, respectively, after surgery, and shock developed during VT in every case. The ECG revealed monomorphic VT in two cases and polymorphic VT in one case. Induction of VT resulted in a wide left-axis deviation-pattern QRS with cycle lengths varying between 260 and 330 milliseconds. The VT origin was identified at the right ventricular outflow tract (RVOT). A radical operation was performed with the patient under cardiopulmonary bypass. On epicardial mapping, delayed activation of the RVOT was recorded during sinus rhythm, and clockwise circus movement of the macroreentry current during VT on the right ventricular free wall was documented in each case. The VTs were treated successfully by surgical resection and cryoablation of the myocardium. In every patient, histology of the myocardial specimens showed degeneration, adiposis, fibrosis, inflammatory cell infiltration, and scattered myocyte islets. These lesions corresponded anatomically to the area of myocardium in which delayed activation was evident during epicardial mapping. CONCLUSIONS: The results of this study indicate that patients with VT after radical correction of the TF have abnormal histopathological findings at the site of the prior right ventriculotomy scar. These lesions were noted within the region of delayed activation found during epicardial mapping and were found to be a part of the reentrant circuit.     

Open-heart correction of tetralogy of Fallot in an acyanotic dog

Tetralogy of Fallot was diagnosed in an acyanotic 11-month-old dog. Predicted pressure gradient across the pulmonic valve, as assessed by use of continuous wave Doppler echocardiography, was 94.5 mm Hg. Bidirectional shunting was identified by means of selective angiography. Open-heart correction was performed, using a transatrial approach with limited ventriculotomy and cardiopulmonary bypass. The hypertrophied infundibulum was resected, the ventricular septal defect was closed primarily, and a transannular pericardial patch graft was applied. Pressure gradients across the pulmonic valve were 52.9 and 22.8 mm Hg 2 weeks and 4 months after surgery, respectively. Advances in cardiopulmonary bypass, anesthetic management, and use of the transatrial approach may improve the success of open-heart correction of tetralogy of Fallot in dogs.     

Long-term results following surgical operation of tetralogy of Fallot

(1) The ECGs of 84 patients who survived corrective surgery for tetralogy of Fallot were reviewed. The overall incidence of sudden death during at least 5 years period of follow-up was 4%, mortality being highest in those with CRBBB and LAH (40%). (2) The ratio of leftward to rightward QRS forces projected on the X axis (Rs/Sx) is significantly decreased in postoperative T/F patients. (3) Twenty-seven patients who had corrective surgery for tetralogy of Fallot underwent cardiac catheterization postoperatively. LVEF was mildly, but significantly diminished and LVEDV was increased significantly, while peak dp/dt, Vpm and Vmax were significantly depressed. These findings indicate that patients who had corrective surgery for tetralogy of Fallot have impaired left ventricular function.     

The functional status of arrhythmia patients in the late period after the radical correction of tetralogy of Fallot

OBJECTIVE: To assess the cognitive performance of elderly patients referred to the memory clinic. DESIGN: The patients were interviewed using the computerised mental state programme, GMS-AGECAT, and assessed on the Cognitive Assessment Scale (CAS) which includes the Elderly Cognitive Assessment Questionnaire (ECAQ) and the Cambridge Memory Test (CMT), modified for Chinese elderly. SUBJECTS: There were 72 Chinese elderly subjects aged 65 years and above, referred to the NUH Memory Clinic in the first year. RESULTS: Only 45 (62.5%) of the 72 subjects were diagnosed to have dementia using DSM III R criteria. There were 25 cases of Alzheimer's Disease and 20 multi-infarct dementia. In the non-demented group, 15 (20.8%) had depression or anxiety disorders and 12 (16.7%), had no mental disorders but had physical illness which could affect memory. There was a highly significant difference in the ECAQ, CMT and CAS scores of demented and non-demented elderly. There was also a significant difference in the cognitive performance of elderly with mild dementia and those with no mental disorder. CONCLUSION: The memory clinic is a useful facility for the diagnosis and management of dementia. The Cognitive Assessment Scale provides a valid and reliable battery of tests for dementia.     

Sinus node dysfunction after complete correction of tetralogy of Fallot. Report of a case

Atrioventricular conduction defects after complete correction of tetralogy of Fallot are well known and their prevalence is decreasing with improved operative techniques. On the other hand, sinus node dysfunction has not been described after this type of surgery. The authors report the case of a 24 year old patient operated at the age of 2 for a favourable form of tetralogy of Fallot. In the long-term, this patient had a minimal anatomical sequel (mild pulmonary insufficiency) and first degree AVB with complete right bundle branch block with sinus bradycardia on the surface ECG. A recent aggravation of the patient's functional status led to rhythmological investigation which showed chronotropic atrial incompetence with nodal atrioventricular delay. The patient was implanted with a permanent DDD cardiac pacemaker programmed in the DDDR mode with a good outcome. Atrial chronotropic incompetence is one of the modes of expression of sinus node dysfunction. This has not been previously described in this context. This form of sinus node dysfunction should be systematically excluded after surgical cure of tetralogy of Fallot by exercise stress testing and 24 hour Holter ECG. Symptomatic patients are greatly improved by permanent cardiac pacing with hysteresis.     

Optimal size of outflow patch in total correction of tetralogy of Fallot

Hemodynamic and angiocardiographic studies were performed in postoperative patients with tetralogy of Fallot. Pressure gradient between the right ventricle and pulmonary artery was correlated with the narrowest area in the pulmonary arterial pathway. Regurgitant fraction was also correlated with regurgitant area which was determined by preoperative area of the pulmonic annulus and width of the outflow patch. Follow-up study of postoperative patients with tetralogy indicated that those with pressure gradient less than 20 mmHg and regurgitant fraction less than 15% could be considered ideally corrected. A table was constructed for determining the most appropriate width of the outflow patch for the ideal correction.     

Left and right ventricular volume characteristics in tetralogy of Fallot and their relationship to arterial oxygen saturation and age

Left (LV) and right ventricular (RV) volume characteristics in 43 patients with tetralogy of Fallot (TOF) undergoing no prior surgical intervention, aged 3-50 months, were evaluated. The control group consisted of 45 patients with Kawasaki disease without cardiac lesions, aged 12-82 months. The TOF patients were divided into four groups: those having arterial oxygen saturation < 80% with an age at the time of study < 18 months (group 1a) or with that > or = 18 months (group 1b), and those with arterial oxygen saturation > or = 80% with an age < 18 months (group 2a) or with that > or = 18 months (group 2b). The results were compared with those in control subjects. In group 1a, each of LV end-diastolic volume (EDV), LV ejection fraction (EF), RVEDV and RVEF was reduced. In group 1b, LVEDV, LVEF and RVEF were decreased. In groups 2a and 2b, RVEF alone was depressed. From these results, the severity of hypoxemia was an important risk factor for ventricular dysfunctions. No influence of age on the volume characteristics was found. The investigations suggested that patients with TOF having an arterial oxygen saturation < 80% are probably candidates for early surgical intervention.     

Usefulness of color kinesis imaging for evaluation of regional right ventricular wall motion in patients with surgically repaired tetralogy of Fallot

We evaluated regional right ventricular wall motion during systole in patients with surgically repaired tetralogy of Fallot (TOF) using color kinesis imaging. Color kinesis images were obtained in a subcostal sagittal view from 19 patients with repaired TOF (TOF group), aged 3 to 5 years, and 20 age-matched normal subjects (control group). For regional wall motion analysis, the endocardial motion distance and the fractional area change (FAC) were calculated for 6 segments obtained from color kinesis images. The endocardial inward excursion distances and the FACs in the upper, middle, and lower posterior segments of the TOF group were significantly greater than those of the control group (p <0.01 for each segment). The upper anterior segment showed significantly reduced inward excursion distance and FAC in the TOF group than in the control group (p <0.01, both). The dyskinetic outward excursion distances of the middle and lower posterior segments were significantly lower in the TOF group than in the control group (p <0.01, both). In the upper and middle anterior segments, the endocardial outward excursion distances were significantly higher in the TOF group than in the control group (p <0.01 and 0.05). Correlation between right ventriculographic and color kinesis measurements was excellent (y = 1.14x - 1.30, r = 0.87 for the endocardial inward excursion, and y = 1.03x +/- 0.56, r = 0.91 for the FAC). In conclusion, color kinesis is a useful method for assessing regional right ventricular wall motion. Segmental analysis of color kinesis images provides accurate, automated, and quantitative diagnosis of regional right ventricular wall motion abnormalities in patients with surgically repaired TOF.     

Response to exercise in patients after repair of tetralogy of Fallot

Cardiac catheterization and submaximal exercise testing was performed in 38 patients after repair of tetralogy of Fallot (TF), and compared to 6 control patients who had functional murmurs. Cardiac index, heart rate, and stroke volume index were significantly lower in the TF group than in the control group. Right and left ventricular end-diastolic pressure increased significantly during exercise, which was not found in the control group. Total pulmonary vascular resistance (TPVR), which decreased significantly with exercise in the control group, did not change remarkably during exercise. TPVR was significantly higher in the TF group than in the control group both at rest and during exercise. Several factors were compared between patients with good cardiac index (> 5.0 l/min/m2; Group 1) and poor cardiac index (< 5.0 l/min/m2; Group 2) during exercise. Stroke volume index, right ventricular ejection fraction at rest were significantly higher in Group 1 than Group 2. TPVR, right and left ventricular end-diastolic and end-systolic volume index were significantly lower in Group 1 than in Group 2. There was no significant difference in heart rate, left ventricular ejection fraction, residual pulmonary stenosis, right to left ventricular systolic pressure ratio, and severity of pulmonary regurgitation between two groups. These findings indicate that abnormalities of exercise tolerance in patients after repair of TF were related to poor response of heart rate, pulmonary vascular resistance, and systolic and diastolic ventricular function.     

Study of the cardiorespiratory response and chronotropic function after correction of tetralogy of Fallot. Important role of residual pulmonary regurgitation

The pathogenic O1 Amazonia variant of Vibrio cholerae has been shown previously to have a cytotoxin acting on cultured Vero and Y-1 cells, and to lack important virulence factors such as the cholera toxin (Coelho et al. 1995a). This study extends the molecular analysis of the Amazonia strains, detecting the presence of the toxR gene, with a very similar sequence to that of the E1 Tor and classical biotypes. The outer membrane proteins are analyzed, detecting a variation among the group of Amazonia strains, with three different patterns found. As a by-product of this work a polymerase chain reaction fragment was sequenced, reading part of the sequence of the Lon protease of the Amazonia strains. This gene was not previously described in V. cholerae, but its sequence is present in the TIGR database specific for this species.     

Early age at repair prevents restrictive right ventricular (RV) physiology after surgery for tetralogy of Fallot (TOF): diastolic RV function after TOF repair in infancy

Single-unit spike trains recorded from parietal cortex of monkeys performing a tactile short-term memory task show characteristic fluctuations (transitions) in their firing frequency that are related to memory. Spike trains recorded during the memory period, when the animal must retain information for the short term, show a higher rate of such transitions than spike trains recorded during intertrial baseline periods. In the present study, an analysis of multiple temporal resolutions over which these transitions are observed reveals that the memory-related transitions occur most prominently in the 25-50 Hz range. The results of this study suggest that, in the monkey, high frequency fluctuations of neuronal discharge in the parietal cortex are correlated with haptic short-term memory. The presence of such fluctuations are also consistent with theoretical models of short-term memory.     

Nitric oxide: a greenhouse gas is used in the treatment of respiratory failure.

Medical science has long made the improbable probable, saving lives and improving quality of life. Upon the introduction of medical devices that can deliver safe quantities of the poisonous gas nitric oxide to help patients with respiratory and other illnesses, the FDA requested ASTM Committee F29 on Anesthetic and Respiratory Equipment to provide standards for these devices. Dr. Daniel Supkis and Mark Graber explain the delicate process of delivering NO to patients and how ASTM standards now in development will increase the safety of this procedure.