Radiofrequency catheter ablation of supraventricular tachycardia in an adult with corrected tetralogy of Fallot

An 18 year old female with Fallot's tetralogy had undergone complete repair at thirteen years of age. Two years later she first presented a wide complex right bundle branch block tachycardia at a rate of 220 beats/min which could not be controlled on intravenous verapamil. Electrical shock successfully converted tachycardia to sinus rhythm, which showed typical Wolff-Parkinson-White syndrome. On electrophysiological study, the ortodromic tachycardia was found to be due to left lateral atrioventricular accessory pathway, which was ablated by radiofrequency catheter ablation. One year later she was symptom-free without antiarrhythmic medication.     

A case of an adult patient of tetralogy of Fallot having malignant hyperthermia during surgery

An episode of malignant hyperthermia occurring in a 42-year-old man undergoing hypothermic cardiopulmonary bypass is reported. Malignant hyperthermia is a syndrome initiated by a hypermetabolic state of skeletal muscle. A patient presented for correction of an acyanotic tetralogy of Fallot. The coincidental usage of hypothermic cardiopulmonary bypass obscured the classical presenting sings and symptoms of the malignant hyperthermia. And the disease of tetralogy of Fallot made the syndrome difficult to manage. Although the clinical diagnosis of malignant hyperthermia is difficult to be confirmed, when it is suspected, it is prudent for the case to be initially treated as malignant hyperthermia.     

Study of tetralogy of Fallot by echocardiography

Twelve patients with a Fallot's tetralogy proved by a haemodynamic and angiographic examination were studied by echography, a non invasive technique. The diameters of the right and left ventricles, of the left atrium and of the aorta were measured. The movements of the interventricular septum and the position of the aorta in relation to it were analysed. Moreover, by scanning of the left ventricle an opening was looked for in the septum. The ventricular septal defect together with a dilatation of the right ventricle and a more or less intense dilatation of the root of the aorta were found in all cases. In 10 cases, an aorta overriding the septum was observed, and this was the more obvious the older the child. In the two post-operative cases studied, the septum was found to be in line with the aorta. There are therefore echocardiographic criteria making it possible to diagnose Fallot's tetralogy. These criteria are the more obvious the older the child.     

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.     

Monocusp homograft VSD patch in atrioventricular canal with tetralogy of Fallot

A monocusp aortic homograft was used to compensate for deficient right atrioventricular valve tissue during repair of complete atrioventricular canal defect with tetralogy of Fallot. The homograft was used to produce a comma-shaped ventricular septal defect patch together with the septal leaflet of the right atrioventricular valve, thus committing native leaflet tissue to left atrioventricular valve reconstruction. One year postoperatively the child is in New York Heart Association class I with no tricuspid regurgitation.     

Balloon pulmonary valvuloplasty for infants with severe tetralogy of Fallot

Balloon pulmonary valvuloplasty was performed in 3 infants with severe tetralogy of Fallot at days 24, 54 and 86 because of progressive hypoxemia. In two patients, the balloon catheter (4 cm long, 5-8 mm diameter) could not pass through the pulmonary valve. This necessitated a smaller balloon and required a two-step procedure. Initially, a coronary artery balloon (2 cm long, 3.5 mm diameter) was used. Following balloon valvuloplasty, arterial oxygen saturation increased from 63 to 83% in case 1, from 69 to 85% in case 2 and 63 to 86% in case 3. Immediate postvalvuloplasty right ventricular cineangiography revealed that the maximal opening diameter of the pulmonary valve leaflets increased from 1-2 mm to 3-4 mm in cases 1 and 3, and from 2-3 mm to 4-5 mm in case 2. No significant complications occurred. Echocardiographic follow-up data showed that the diameter of the right ventricular outflow tract and pulmonary arteries increased with age. The present results show that the pulmonary valvuloplasty is an effective procedure for relief of pulmonary valve stenosis in tetralogy of Fallot and to improve oxygenation and growth of the pulmonary arteries and right ventricular outflow tract without the need of an immediate aortopulmonary shunt.     

Anomalous coronary artery with tetralogy of Fallot and aortopulmonary window

Anomalous origin of the left main coronary artery from the pulmonary artery is rarely associated with other conditions. We report the case of an infant born with tetralogy of Fallot and aortopulmonary window who at the time of surgical repair was found to have an anomalous left main coronary artery originating from the right pulmonary artery.     

Comparison of five methods for the determination of arginine hydrolysis by viridans streptococci

The authors describe a rare case of cystic nephroma treated by partial surgical excision. Because there is concurrence in the association of multilocular cysts with Wilms tumors and others tumors of the kidney, it is underlined the importance of a differential diagnosis to avoid nephrectomy for treatment of this benign neoplasm.     

Primary thrombosis of pulmonary artery in a child with tetralogy of Fallot

A 34-month-old child with tetralogy of Fallot developed an acute, primary thrombosis of the pulmonary trunk. A greatly increased haematocrit was most probably a predisposing factor. An unusual postmortem finding was the large number of extremely dilated myocardial capillaries. In a review of a series of 394 consecutive necropsy cases with many kinds of cardiac anomalies, there was only one case (a 28-year-old man) with occluding thrombus formation in the pulmonary artery. This case also belonged to the Fallot group but a subacute glomerulonephritis seemed to be the major cause of death.     

Virus-associated haemophagocytic syndrome caused by rubella in an adult

Haemophagocytic syndrome is a systemic clinicopathological entity characterized by systemic proliferation of benign haemophagocytic histiocytes, fever, cytopenia, abnormal liver function and, frequently, coagulopathy and hepatosplenomegaly. Its occurrence has been documented in association with viral, bacterial, fungal and parasitic infections, a wide spectrum of malignant neoplasms, autoimmune diseases and drugs. We report a case of rubella virus-associated haemophagocytic syndrome in a previously healthy 29-year-old woman. Blood tests showed cytopenia, especially severe thrombocytopenia, liver dysfunction, hyperferritinaemia and hypercytokinaemia. Bone marrow examination showed many mature histiocytes with active haemophagocytosis. A skin biopsy from the rash revealed perivascular lymphohistiocytic infiltrates with haemophagocytic histiocytes in the upper and mid-dermis. The patient was treated with antibiotics and immunoglobulin, and by supportive measures including platelet transfusion, and recovered completely.     

The Blalock-Taussig operation: the procedure of choice in the hypoxic infant with tetralogy of Fallot

An experimental model is presented enabling an analysis of the healing process of completely cut and re-sutured free segments of rabbit flexor tendons, kept avascular in a synovial milieu and completely isolated from adhesion formation. Under these conditions the cut tendons heal within a few weeks. It can be shown that this healing process is a result of intrinsic tendon cell activity only.     

End-on aortogram: improved identification of important coronary artery anomalies in tetralogy of Fallot

OBJECTIVE: To identify coronary artery anomalies in patients with tetralogy of Fallot with an aortogram taken with steep caudal and left oblique angulation ("end-on" aortogram). DESIGN: Prospective evaluation of end-on aortogram in the preoperative angiographic assessment of consecutive patients with tetralogy of Fallot. SETTING: Regional paediatric cardiology centre. PATIENTS: 34 patients, aged 3 months to 12 years (median age 9 months). METHODS: An aortogram was performed with steep caudal (38 degrees-45 degrees) and left oblique (0 degrees-30 degrees) angulation under general anaesthetic as part of routine preoperative angiographic assessment. RESULTS: The origins and courses of the coronary arteries were visualised in all patients and important coronary artery anomalies were identified in four patients: single left coronary artery; single right coronary artery (two patients); separate high origin of left anterior descending. These anomalous coronary vessels crossed the right ventricular outflow tract. CONCLUSIONS: It is important to identify preoperatively coronary arteries that may interfere with right ventricular outflow tract reconstruction. An aortogram with steep caudal and left oblique angulation is useful in identifying anomalous coronary arteries and more importantly it defines the relation of these vessels to the right ventricular outflow tract.     

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.     

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.     

Increased prevalence of penicillin-resistant viridans group streptococci in Japanese children with upper respiratory infection treated by beta-lactam agents and in those with oncohematologic diseases

BACKGROUND: Viridans group streptococci, especially penicillin-resistant strains, have been emerging as pathogens of bacteremia in neutropenic patients with hematologic malignancies. OBJECTIVES: To survey the penicillin susceptibilities of viridans group streptococci in Japanese children with and without oncohematologic diseases and to evaluate the effect of the short term administration of beta-lactam agents on the antibiotic susceptibility. METHODS: We tested 113 isolates of viridans group streptococci by the microdilution method for the minimal inhibitory concentrations (MICs) to 10 antibiotics. We isolated 40 isolates from the throats of children with an upper respiratory infection (URI) before beta-lactam antibiotic treatment, 32 isolates after the treatment, 33 isolates in hospitalized children with oncohematologic diseases and 8 isolates from blood. RESULTS: Twenty-five isolates (62.5%) from the children with URI before treatment were penicillin-intermediate or -high level resistant (MIC > or = 0.25 microg/ml). The prevalence of those isolates after antibiotic treatment (87.5%) was significantly increased compared with that before treatment (P = 0.03). The prevalences of the penicillin-high level resistant isolates (MIC > or = 4 microg/ml) in the children with oncohematologic diseases (39.4%) and in the isolates from blood (62.5%) were significantly higher than that in the children with URI before treatment (12.5%) (P < 0.01). Decreased susceptibilities to other beta-lactam agents were observed in the penicillin-high level resistant strains. CONCLUSIONS: The high prevalence of penicillin-intermediate or -high level resistant viridans group streptococci in healthy Japanese children was documented. The administration of beta-lactam agents decreased the prevalence of penicillin-susceptible isolates in the children with URI. High prevalences of penicillin-high level resistant isolates were observed in the oncohematologic patients and in the isolates from blood.     

Dispersion of ventricular recovery time following surgery for tetralogy of Fallot: correlation with negative prognostic factors

By use of gene targeting and/or transgenesis, it is now possible to make defined changes in genes whose functions underlie mammalian cardiovascular function. Because of technical and economic considerations, these experiments are largely confined to the mouse. Genetic modification of the loci responsible for aspects of cardiac development, differentiation, and function via gene targeting, as well as modulation of the cardiac protein complement using transgenesis, has begun to provide mouse models of cardiac hypertrophy, dilated cardiomyopathy, and hypertrophic cardiomyopathies. In order to use these animal models fully and explore their phenotypes at the whole organ and whole animal levels, the extension of cardiovascular physiological methodologies to the mouse is imperative. Techniques for exploring aspects of cardiovascular function are well developed for larger animal models, but their modification for the small size of the mouse heart and for the animal's rapid cardiac cycle has proven to be a formidable challenge, requiring the combined efforts of the molecular biology, physiology, and cardiology communities. We review here the ability of present-day technology to obtain reproducible data on murine cardiac function at the whole organ and animal levels.