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1/63. Successful treatment with methylprednisolone pulse therapy for a life-threatening pulmonary insufficiency in a patient with chronic granulomatous disease following pulmonary invasive aspergillosis and burkholderia cepacia infection.

    A 14-year-old boy with X-linked chronic granulomatous disease developed severe invasive pulmonary aspergillosis. He was treated with itraconazole and amphotericin b. However, he deteriorated with progressive pulmonary lesions. burkholderia cepacia was isolated from his bronchoalveolar lavage. Finally, he was given granulocyte transfusions. Following this procedure, his condition rapidly worsened leading to respiratory failure. His lung biopsy demonstrated organizing pneumonia at his right middle lobe. Then, a methylprednisolone pulse therapy was initiated together with the administration of appropriate antibiotics and adequate amounts of amphotericin b. Dramatically, his condition improved. Therefore, a methylprednisolone pulse therapy with appropriate antimicrobial drugs seems to be beneficial for severe pulmonary insufficiency in this type of patients. copyright copyright 1999 S. Karger AG, Basel ( info)

2/63. diagnosis and natural history of isolated congenital pulmonary regurgitation in fetal life.

    We describe a rare instance of isolated pulmonary regurgitation caused by a dysplastic pulmonary valve which was detected prenatally. Fetal echocardiography demonstrated severe pulmonary regurgitation, and progressive cardiomegaly because of right ventricular volume overload. After birth, conservative therapy was successful in alleviating the pulmonary vascular resistance, and the pulmonary regurgitation gradually decreased. ( info)

3/63. Imaging of an aneurysm of the sinus of valsalva with transesophageal echocardiography, contrast angiography and MRI.

    A sinus of valsalva aneurysm is an uncommon congenital defect, which requires appropriate diagnosis with either echocardiography, magnetic resonance imaging or contrast angiography. Treatment consists of aortic valve repair. We describe a young woman with an aneurysm of the non-coronary sinus of valsalva, an atrial septal defect and pulmonary insufficiency. The different imaging techniques and possibilities of surgical correction are described. ( info)

4/63. Isolated congenital absence of a single pulmonary valve cusp.

    Isolated absence of a single pulmonary valve is extremely unusual. We present a 16-year-old male with the absence of one cusp resulting in significant pulmonary regurgitation, right ventricular enlargement, and dilatation of the main pulmonary artery and its branches. Surgery consisted of creating a valve cusp from the posterior wall of the pulmonary artery at the base of the pulmonary trunk. Follow-up at 3.5 years revealed good results with only mild pulmonary stenosis and regurgitation. ( info)

5/63. Isolated pulmonic valve endocarditis in healthy hearts: a case report and review of the literature.

    The case of a 53-year-old man with isolated pulmonic valve endocarditis in a structurally normal heart is presented. The patient had a history of chronic obstructive pulmonary disease and was admitted to hospital with an apparent exacerbation with pneumonia. blood cultures grew staphylococcus aureus, and an echocardiogram identified a large vegetation on the pulmonic valve in a structurally normal heart. He was unsuccessfully treated with antibiotics and eventually required pulmonic valve replacement. The literature from 1960 to 1999 identified only 36 reported cases of pulmonic valve endocarditis in structurally normal hearts. The present report underscores the importance of suspecting pulmonic valve endocarditis in patients with multiple pulmonary lesions, and discusses the predisposing factors, clinical features, diagnostic role of echocardiography and the potential benefits of early surgical treatment. ( info)

6/63. Fetal diagnosis of lethal dysfunction of the right heart in three siblings.

    A woman, having already delivered one child, underwent fetal echocardiography during three subsequent pregnancies. All three showed enlargement and poor function of the right-sided chambers. The first was still-born, the second died as a neonate, while the third pregnancy was terminated. Pathological examination revealed the same findings in each fetus, possibly representing a variation of Uhl's anomaly, or alternatively a hitherto unrecognised cardiomyopathic process. ( info)

7/63. Isolated pulmonic valve endocarditis caused by group B streprococcus (streptococcus agalactiae)--a case report and literature review.

    The pulmonic valve is the least commonly involved valve in infective endocarditis. Pulmonic valve endocarditis is usually associated with tricuspid valve endocarditis, and isolated pulmonic valve endocarditis is exceedingly rare. The predisposing factors for developing pulmonic valve endocarditis include a congenitally anomalous pulmonic valve, intravenous drug abuse, and the presence of indwelling intravenous or flow-directed pulmonary artery catheters. More cases of group B streptococcus endocarditis are being reported. The risk factors for group B streptococcus endocarditis include diabetes mellitus, cancer, alcoholism, malnutrition, immunocompromised status, intravenous drug abuse, postpartum and postabortion states, and underlying valvular disease. The vegetations of this type of endocarditis are usually large and have a higher tendency to result in embolism. The presentation of group B streptococcus endocarditis is usually acute and may result in rapid valve destruction if not treated promptly. A case of isolated pulmonic valve endocarditis caused by group B streptococcus, streptococcus agalactiae, is presented that was diagnosed with multiplane transesophageal echocardiography in a 40-year old, alcoholic, malnourished man, who was successfully treated with intravenous penicillin g. The literature on the isolated pulmonic valve endocarditis caused by group B streptococcus is reviewed. ( info)

8/63. pulmonary valve injury: Swan-Ganz or surgery.

    pulmonary artery catheter (PAC) is a commonly used monitor in cardiac surgery. pulmonary valve injury from the balloon of the pulmonary artery catheter is exceedingly rare; its description by transesophageal echocardiography is nonexistent in the literature. A patient is reported who developed a flail anterior pulmonary leaflet several days after successful myectomy for hypertrophic cardiomyopathy. The temporal sequence of PAC use and four transesophageal echocardiography studies over 13 days, with the first three showing normal pulmonary valves, suggested pulmonary valve trauma, most likely from a Swan-Ganz catheter, as the etiology. Partial damage to the pulmonary valve from the original surgery may have predisposed the patient to further damage when the PAC was reintroduced in the intensive care unit. ( info)

9/63. Ventilatory insufficiency due to asbestos-related diffuse pleural fibrosis successfully treated with noninvasive home mechanical ventilation.

    A 54-year-old man with diffuse pleural fibrosis due to previous asbestos exposure developed hypercapnic respiratory failure. Noninvasive mechanical ventilation (NIMV) was started at the hospital and maintained at the patient's home, achieving reversal of the respiratory failure. During a 2-year follow-up, NIMV has been well tolerated by the patient and no relapse in ventilatory failure has occurred. Home NIMV can be considered as an alternative to pleural decortication in asbestos-related diffuse pleural fibrosis with ventilatory insufficiency. ( info)

10/63. Serial stent implantation to relieve in-stent stenosis in obstructed total anomalous pulmonary venous return.

    A nine-year-old girl with complex cyanotic heart disease associated with supracardiac total anomalous venous return and obstruction at the drainage site of the vertical vein into the left innominate vein had stent placement at the age of seven, with immediate increase of oxygen saturation and improvement of physical activity. Significant in-stent stenosis occurred that was successfully treated by concentrical placement of a second stent. This case report demonstrates the transluminal approach to be effective in the treatment of stenoses in congenital heart defects that are not eligible for corrective surgery. ( info)
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