Cases reported "Tetralogy of Fallot"

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1/31. Decreased left ventricular filling pressure 8 months after corrective surgery in a 55-year-old man with tetralogy of fallot: adaptation for increased preload.

    A 55-year-old man with tetralogy of fallot underwent corrective surgery. Left ventricular filling pressure increased markedly with increased left ventricular volume one month after surgery, then decreased over the next 7 months, presumably due to increased left ventricular compliance.
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2/31. cyanosis due to diastolic right-to-left shunting across a ventricular septal defect in a patient with repaired tetralogy of fallot and pulmonary atresia.

    cyanosis as a result of right-to-left shunting across a ventricular septal defect is commonly encountered in patients with congenital heart disease when systolic pressure in the right ventricle exceeds that in the left ventricle. Reported is the case of a child who remained cyanosed after surgical correction of pulmonary atresia despite right ventricular systolic pressure being lower than left ventricular pressure. Colour-flow Doppler showed a residual ventricular septal defect, with right-to-left shunting in diastole alone.
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3/31. Rastelli type repair using Freestyle valved conduit for a 69-year-old woman with tetralogy of fallot.

    A 69-year-old woman visited our hospital with general fatigue and shortness of breath on effort as the chief complaints. She was diagnosed as having tetralogy of fallot, using cardiac ultrasonography. The cardiac catheterization findings showed that right venticular hypertension was at 114/5 mmHg, which was parallel to the left ventricular pressure, and a pressure gradient of about 100 mmHg was observed between the right ventricle and the pulmonary artery. Coronary artery angiography revealed that the left coronary artery was intact, but the right had an abnormal origin from the left valsalva sinus and was estimated at nearly equal to the single coronary. Therefore, we performed a Rastelli type operation with a valved conduit which we made using a composite Hemashield artificial graft (diameter 20 mm) and Freestyle valve (diameter 21 mm). The postoperative course was uneventful and she was discharged with hemodynamic conditions mostly normalized.
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4/31. Reconstruction of the right ventricular outflow tract with a conduit in congenital heart disease.

    A series of five patients with complex cyanotic congenital cardiac malformations underwent surgical reconstruction of the right ventricular outflow tract using a Dacron conduit with a porcine aortic valve. All patients survived and all have shown clinical improvement. At cardiac catheterization postoperatively, a pressure gradient of between 20 and 50 mm Hg across the conduit was found in all patients. This surgical approach to patients necessitating reconstruction of the right ventricular outflow tract is effective and appears to have the best long-term results.
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5/31. Nasal mask bilevel positive airway pressure ventilation for diaphragmatic paralysis after pediatric open-heart surgery.

    A 2-year-old boy underwent surgical repair of tetralogy of fallot. Topical cooling of the heart with ice slush was used during the operation. Diaphragmatic paralysis occurred after the operation, inducing severe respiratory distress. To avoid repeated intubation and tracheostomy, the patient was placed on nasal mask bilevel positive airway pressure (BiPAP) ventilation. After ventilatory support with BiPAP for 40 days, the patient recovered spontaneously from the paralysis. No sedation was required during this time. This report illustrates the usefulness of BiPAP for a pediatric patient with diaphragmatic paralysis after cardiac surgery.
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6/31. Necrotizing arteritis in uncorrected tetralogy of fallot with pulmonary artery.

    A 10-year-old girl with uncorrected tetralogy of fallot with pulmonary atresia presented with fevers of unknown origin and left lung infiltrates. At autopsy, necrotizing vascular changes resembling those of severe pulmonary hypertension (grade VI in the health-Edwards classification) were confined to the left lung. Pulmonary blood flow and pressure were greater in the left lung and were provided by an enlarged collateral artery arising directly from the descending thoracic aorta. To our knowledge, this is the first report of necrotizing arteritis of the pulmonary arteries in uncorrected tetralogy of fallot with pulmonary atresia.
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7/31. Total correction of tetralogy of fallot. How to deal with the complicated ascending aorta-right pulmonary artery anastomosis.

    Ascending aorta-right pulmonary artery anastomosis may be complicated by deformity at the anastomotic site leading to discontinuity between pulmonary artery branches. Simple closure of such shunts through the ascending aorta is associated with residual stenosis and pressure gradients. An alternative approach which allows reconstruction of the entire intrapericardial pulmonary arterial system at the initial corrective operation is described. The transection of the ascending aorta facilitates the exposure necessary for this extensive repair. Two illustrative cases are presented.
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8/31. pulmonary artery stenting without angiographic imaging.

    pulmonary artery stenosis is a frequent complication seen after surgical repair of tetralogy of fallot. In this setting, endovascular stent implantation is now accepted as the first-choice therapeutic option. However, angiographic imaging still being held as mandatory to check the stent position before final deployment, this procedure is not considered suitable for patients who cannot be submitted to angiography. In this paper, we report a novel method for the correct implantation of an endovascular stent without angiographic imaging. A 9-year-old boy underwent cardiac catheterization to relieve a severe left pulmonary artery stenosis. A previous attempt had been aborted due to a life-threatening anaphylactic reaction to the contrast medium. To avoid angiography, a contrast medium-filled compliant atrial septal defect sizing balloon (Amplatzer Sizing Balloon, AGA Medical Corporation) was used to image the vessel stenosis and successfully guide stent deployment. After the procedure, the transstenotic pressure gradient disappeared and the left-to-right pulmonary perfusion imbalance almost completely reverted.
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9/31. A critical increase in right-to-left shunt after acute myocardial infarction in a 68-year-old male with tetralogy of fallot.

    A male patient with tetralogy of fallot accompanied by aortic regurgitation had maintained sufficient exercise capacity for a number of decades with the status of acyanotic tetralogy of fallot. When he was 67 years old, he suffered a posterior wall acute myocardial infarction and direct percutaneous coronary angioplasty successfully revascularised the target lesion in the left circumflex artery. However, a few months after the onset of the myocardial infarction, his shortness of breath became clinically significant and was associated with increased right-to-left shunt and increased right ventricular end-diastolic pressure, as well as hypoxia. At 68 years old, therefore, total corrective repair of the tetralogy with replacement of the aortic and pulmonary valves was performed. The patient was asymptomatic after the successful operation. This report suggests that coronary artery disease can be one of the potential factors in inducing critical hemodynamic changes in aging patients with congenital heart disease, especially those who have a shunt between the right and left chambers. The unique clinical course is described with some discussion of the repair of tetralogy in adults.
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10/31. Prolonged survival in a female with untreated tetralogy of fallot.

    It has been reported that most patients with untreated tetralogy of fallot (TOF) die by the time they reach adulthood. We report the case of a 72-year-old female diagnosed by echocardiography and cardiac cathetherization as having TOF and diagnosed at birth with a ventricular septal defect (VSD). During childhood, she was very thin and lacking in physical strength. On first consultation at our hospital, she was suffering from mild dyspnea, classified as NYHA functional class III, and her fingers were clubbed and cyanotic. Her PaO2 was 48.0 mmHg under room air, and hypoxia was recognized. An echocardiography and cardiac cathetherization showed a VSD, hypertrophy of the right ventricle, over-riding of the aorta and stenosis of the right ventricular outflow tract with a pressure gradient of 84 mmHg. There was a bidirectional shunt with 24% flow from the left to right and 43% from the right to left ventricle. Her Qp/Qs was 0.75. Surgical treatment was recommended. However, the patient refused, because her symptoms were alleviated with home oxygen therapy. This report shows the prolonged survival of this 72-year-old female with untreated TOF.
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