Cases reported "Tetralogy Of Fallot"

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11/619. Two brothers with varying combinations of severe developmental delay, epilepsy, microcephaly, tetralogy of fallot and hydronephrosis.

    We report on a sib pair who manifest a pattern of anomalies which appears to be unique and for which we are unable to provide a cytogenetic or molecular genetic explanation. While a number of their physical features are distinct, their overall appearance and pattern of neurological impairment suggest they suffer from the same genetic disorder. ( info)

12/619. A case of lateral facial clefts with Fallot tetralogy, duodenal stenosis and intestinal malrotation: a new multiple congenital anomaly syndrome?

    Multiple congenital malformations in a Caucasian female infant are described which include lateral facial clefts, malformed external ears, cleft palate, Fallot tetralogy, duodenal stenosis and intestinal malrotation. There were no associated limb or spinal anomalies. This case appears to be an example of a new multiple congenital anomaly syndrome. ( info)

13/619. Inverted left atrial appendage presenting as a left atrial mass after cardiac surgery.

    Inversion of the left atrial appendage can masquerade as a new left atrial mass. Failure to be aware of this entity can result in unnecessary diagnostic and therapeutic procedures. If the entity is diagnosed intraoperatively, treatment is simple with external reduction and ligation. ( info)

14/619. Extra-anatomical bypass with EPTFE graft for residual pulmonary artery stenosis in tetralogy of fallot.

    We describe the case of 35-year old woman who had undergone radical surgery to correct tetralogy of fallot malformation at the age of nine admitted to our hospital because of palpitation and exertional dyspnea. angiography revealed 90% re-stenosis of the right pulmonary artery and grade IV tricuspid regurgitation. Right lung was perfused only 16.7% as shown by pulmonary perfusion scintigraphy. Interposition between the pulmonary trunk and right pulmonary artery using an EPTFE graft and tricuspid annuloplasty using a Duran ring was performed. blood flow to the right lung improved markedly from 16.7% to 37.0% and her symptom disappeared completely. ( info)

15/619. One autopsy case of an elderly traffic accident victim with tetralogy of fallot.

    The case of a 61-year-old male traffic accident victim with tetralogy of fallot (TOF) is reported. The autopsy revealed massive hemorrhages in the subcutaneous tissue, muscle, and subarachnoidal space. Furthermore, multiple fractures of ribs, sternum and thoracic vertebrae were observed. Histopathological examination revealed changes characteristic of trauma, such as acute lung congestion, acute renal cortical necrosis, and embolization in the lungs and kidney. These autopsy and histological observations indicated that traumatic shock was cause of his death. Moreover, histologically, we observed changes due to his congenital heart disease, such as right ventricular hypertrophy, heart failure cells in the lungs, sclerosis of the liver, and hyaline degeneration in the kidney. Furthermore, ischemic changes, shrinkage or loss of neurons, were seen in hippocampus, and swelling of astrocytes in both cortex and hippocampus were also observed. These observations lead us to speculate that a hypoxic episode may have caused his accidental death while driving. ( info)

16/619. tetralogy of fallot in the fetus: findings at targeted sonography.

    OBJECTIVES: To evaluate the findings of tetralogy of fallot in various fetal sonographic views. methods: We reviewed the fetal sonograms and medical records of 20 fetuses with prenatal diagnosis of tetralogy of fallot. We analyzed the indications for targeted sonography, the abnormalities seen in various sonographic views, the postnatal echocardiographic and angiographic findings and autopsy findings. RESULTS: The most common indication for targeted sonography was an abnormal (n = 12) or inadequate (n = 3) finding on sonographic screening in which the abnormality was most frequently found on the three-vessel view (n = 9). The key pathological features of tetralogy of fallot were uniformly demonstrated in the ventricular outflow tract, three-vessel and short-axis views. The ductus arteriosus was small in 70% of cases and not identifiable in the remaining fetuses. In three of six fetuses with no identifiable ductus, the ductus was shown to be absent at autopsy. The direction of ductal flow was variable. CONCLUSION: The key features of tetralogy of fallot were always demonstrable in the ventricular outflow tract, three-vessel and short-axis views. The most common reason for referral was the abnormal three-vessel view. ( info)

17/619. Laparoscopic cholecystectomy for the adult with unrepaired tetralogy of fallot: a case report.

    A 24-year-old woman with a history of unrepaired tetralogy of fallot was scheduled to undergo laparoscopic cholecystectomy. Her significant history included tetralogy of fallot with pulmonary atresia, hypoplastic left pulmonary artery, pulmonary vascular obstructive disease, a functioning right subclavian artery to right pulmonary artery shunt (modified Blalock-Taussig palliative procedure) with a similar shunt on the left side that is occluded. The patient underwent general endotracheal anesthesia for laparoscopic cholecystectomy for cholelithiasis and pancreatitis. Anesthetic induction, intraoperative course, and the postoperative period proceeded uneventfully, and the patient quickly progressed to the preoperative level of functioning. The careful application of pharmacological and physiological principles guided the anesthetic plan and produced a successful outcome. Principles for the anesthetic management of the patient with cyanotic congenital heart disease undergoing noncardiac surgery are reviewed. ( info)

18/619. Transaortic approach for a closure of a residual ventricular septal defect of the tetralogy of fallot.

    BACKGROUND: A residual ventricular septal defect as part of the tetralogy of fallot (TOF) is often difficult to close when a long interval has elapsed after a radical operation or the position of the defect is problematic. methods: When an accurate diagnosis and closure of a residual ventricular septal defect (VSD) were not obtainable through right atriotomy and right ventriculotomy, the ascending aorta was opened. The smooth surface of the left ventricular septum makes it possible to find the position of the defect, and close it accurately and safely. RESULTS: Three consecutive patients were operated upon using this method. They had undergone repair of tetralogy of fallot anomalies 8 years, 26 years, and 21 years prior to this surgery, respectively. All patients survived the operation and were discharged from the hospital. None of the patients had residual defects, aortic regurgitation, or new atrioventricular block. CONCLUSIONS: This transaortic approach for closing residual ventricular septal defects related to the tetralogy of fallot offers an alternate method when either the right atrial or right ventricular approach cannot be performed successfully. This approach may make it possible to close such defects directly. ( info)

19/619. Preventing sudden death after repair of tetralogy of fallot: complex therapy for complex patients.

    Sudden arrhythmic death in patients with repaired tetralogy of fallot or its variants has a variety of causes. Consequently, it can serve as a paradigm for management of potentially malignant arrhythmias in all pediatric patients, particularly with regard to the use of nonpharmacologic therapy for management. Five cases are presented as touchpoints for discussion and demonstrate a number of important issues concerning the assessment and reduction of sudden cardiac death risk in these patients. First, there are no clinical parameters that can be used to accurately assess risk. Second, pharmacologic agents alone rarely are adequate therapy. Third, catheter ablation and antitachycardia devices continue to play an ever increasing role in management of these patients, and, finally, additional data are necessary to establish clear management guidelines in patients with congenital heart disease at risk for arrhythmic death. ( info)

20/619. Serous fluid leakage after a modified Blalock-Taussig shunt: a cause of hypercyanotic episodes.

    We report a case of a 10-week-old girl, with tetralogy of fallot and a Blalock-Taussig shunt, who presented with hypercyanotic episodes. She was found to have serous fluid leakage around her shunt, causing compression of her trachea. Hypercyanotic episodes resulting from shunt leakage have not previously been reported. An awareness of this possible complication of a Blalock-Taussig shunt will allow the emergency physician to consider it in the differential diagnosis of hypercyanotic episodes. ( info)
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