Cases reported "Dyspnea"

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1/90. Treatment of a large congenital coronary fistula with coil embolization.

    A 77-year-old woman suffering from progressive dyspnea and chest pain for 2 1/2 years was admitted to hospital. There were no ECG changes at exercise test and a dobutamine stress echocardiography was normal. At catheterization, right-sided pressures were within normal limits. coronary angiography revealed a congenital coronary fistula, 3-4 mm in diameter, from the left anterior descending artery to the proximal pulmonary artery. There was no significant rise in blood oxygen saturation in the pulmonary artery. Transcatheter coil embolization was performed in the distal part of the tortuous fistula. Flow ceased within minutes, demonstrating the feasibility and efficacy of this technique for treating large fistulas.
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2/90. Syphilitic aortic regurgitation. An appraisal of surgical treatment.

    During the 10 years from 1964 to 1973, fifteen patients with severe syphilitic aortic regurgitation were treated surgically at the National Heart Hospital. In thirteen the valve was replaced and in two it was repaired. In addition four had replacement of an aneurysmal ascending aorta with a Dacron graft and seven some form of plastic repair to the coronary ostia. Three patients died within 1 month of surgery and a further six during the follow-up period which varied from 1 to 55 months (mean 25-5). The six survivors have been followed-up for an average of 33 months. Factors contributing to this high mortality were analysed and it was found that the mean duration of effort dyspnoea was 22 months in the survivors compared with 48 months in those who had died. Similarly the average duration of nocturnal dyspnoea was 4 months in the survivors compared with a mean of 8 months in those who had died. Only six out of the fifteen patients had angina; this was present in two of the survivors and in four of the fatalities. The pulse pressure, heart size, and haemodynamic findings were similar in the two groups. The prognostic value of an elevated erythocyte sedimentation rate was also examined. It was concluded that preoperative investigations should include aortography, coronary arteriography, an assessment of left ventricular function, and whenever possible myocardial biopsy. These data were interpreted as suggesting that patients should be referred for surgery at an earlier stage in the disease--certainly before the onset of cardiac failure and--and that if this more aggresive attitude was adopted, as it has been in non-syphilitic cases of aortic valve disease, the present high mortality in this group would be reduced.
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3/90. Intravenous injection of talc-containing drugs intended for oral use. A cause of pulmonary granulomatosis and pulmonary hypertension.

    Clinical and morphologic features are described in two patients known to have repeatedly injected intravenously talc-containing drugs intended for oral use. In one patient severe pulmonary hypertension developed; the talc granulomas in him were located predominantly within the pulmonary arteries. The second patient had normal pulmonary arterial pressures, and the talc granulomas in him were located predominantly in the pulmonary interstitium. Of 19 previously described patients with pulmonary talc granulomas, 12 had morphologic evidence of pulmonary hypertension (in three of severe degree); in each, talc granulomas were located predominantly within the pulmonary arteries. In those without signs of pulmonary hypertension, granulomas were located predominantly in the pulmonary interstitium. Why there are differences in the distribution of the talc granulomas is unclear. It is clear, however, as demonstrated by one of our patients, that severe pulmonary hypertension may be a consequence of intravenous injection of drugs intended for oral use.
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4/90. A case of diffuse pulmonary arteriovenous fistula.

    A 30-year-old Japanese woman was admitted to hospital for dyspnea. She had a history of corrective surgery for a large atrial septal defect and partial anomalous pulmonary venous drainage, which had produced cyanosis in her infancy. However, her cyanosis continued postoperatively. Angiography revealed a double inferior vena cava (IVC), with the left IVC connected with the hemiazygos vein and the right IVC with the left atrium through a very small orifice. Most of the blood from the 2 IVCs flowed into the superior vena cava via the distended azygos and hemiazygos veins. Pulmonary arteriography revealed no abnormal structures. Pulmonary arterial pressure was normal. There was marked pulmonary venous oxygen desaturation. perfusion lung scintigraphy revealed multiple segmental perfusion defects. These findings suggested the presence of diffuse microscopic pulmonary arteriovenous fistulas bilaterally in the lungs. The patient appears to be the first reported adult case of microscopic and diffuse arteriovenous fistulas. Neither resection of the arteriovenous fistulas nor corrective surgery for the diversion was indicated, and heart-lung transplantation might be the only treatment able to relieve her dyspnea.
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5/90. Intrathoracic stomach presenting as acute tension gastrothorax.

    Total intrathoracic stomach creating pulmonary and hemodynamic compromise is a rare life-threatening complication in patients with hiatal hernia. The presentation and clinical course of this condition are discussed. physicians should consider this entity in patients presenting with apparent tension pneumothorax without history or other evidence of trauma or positive pressure ventilation who do not respond to standard interventions.
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6/90. Inspiratory pressure threshold training for glottal airway limitation in laryngeal papilloma.

    A single-subject design was used to determine if inspiratory pressure threshold training increases inspiratory muscle strength and reduces the sensation of dyspnea during exercise and speech. The subject was a 23-year-old female with congenital juvenile papilloma which has been in remission for 10 years. A 4-week inspiratory muscle training program was implemented using an inspiratory pressure threshold trainer. The pressure threshold of the trainer was set by the experimenter. The pressure threshold setting of the trainer was based on a percentage of the subject's maximum inspiratory pressure measured prior to training. The average range of the pressure threshold was 40 to 70 cmH2O. In order for inspiratory air to flow, the subject generated inspiratory pressure, independent of airflow rate. Maximum inspiratory pressure (MIP) was the dependent variable used as the index of inspiratory muscle strength. Exercise dyspnea was a dependent variable rated by the subject during a progressive treadmill test. dyspnea associated with speech was rated following production of a comfortable and loud speech task. MIP increased by 57% following the training program with a 2-scale point reduction in the perception of dyspnea during exercise. dyspnea during loud speech decreased from moderate to mild. The changes in dyspnea, both during exercise and speech, are directly related to inspiratory muscle strengthening. The results suggest that inspiratory muscle training may improve respiratory related function in patients with restrictive upper airway disorders.
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7/90. Reversible platypnoea and orthodeoxia after surgical removal of an hydatid cyst from the liver.

    A patient with a large hydatid cyst of the liver developed a positionally symptomatic right to left shunting across a patent foramen ovale with both platypnoea and orthodeoxia, despite normal pulmonary arterial pressures and normal pulmonary function tests. When the patient was in the supine position the calculated right to left shunt was 15.1% and 29.5% when seated. The shunt was attributed to the compression of the right atrium and ventricle by the cyst. Surgical evacuation of the cyst relieved the symptoms and the positionally induced shunting.
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8/90. nitric oxide successfully used to treat acute chest syndrome of sickle cell disease in a young adolescent.

    OBJECTIVES: To report a case of acute chest syndrome (ACS) of sickle cell disease treated successfully with nitric oxide and to review the physiologic effects of nitric oxide and its potential ability to improve outcome in ACS. DESIGN: Descriptive case report. SETTING: Eighteen-bed pediatric intensive care unit in a university children's hospital. PATIENT: A 15-yr-old black male with sickle cell disease, bilateral pulmonary infiltrates, refractory hypoxemia, and unstable hemodynamics. INTERVENTION: In addition to exchange transfusion, invasive hemodynamic monitoring, and aggressive ventilatory support, inhaled nitric oxide was administered in the gas mixture in a concentration of 20 ppm for 72 hrs. MEASUREMENTS AND MAIN RESULTS: cardiac output, pulmonary arterial pressure, pulmonary artery occlusion pressure, systemic vascular resistance, pulmonary vascular resistance, shunt fraction, and alveolar-arterial oxygen gradient were compared with and without inhaled nitric oxide. Marked reductions in pulmonary arterial pressure and pulmonary vascular resistance were noted. cardiac output improved, and shunt fraction and alveolar-arterial oxygen gradient were markedly reduced. The patient required decreased ventilator and hemodynamic support and rapidly made a complete recovery. CONCLUSIONS: nitric oxide may be beneficial for patients with ACS because of its ability to ameliorate pulmonary hypertension and ventilation/perfusion mismatch. nitric oxide may confer some protection against polymerization of sickle hemoglobin and exert a reversible antiplatelet effect that may be beneficial in ACS. Further study is necessary to determine the safety and efficacy of inhaled nitric oxide as a treatment for ACS.
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9/90. Platypnoea-orthodeoxia syndrome.

    PLATYPNOEA: orthodeoxia is a rare syndrome of postural hypoxaemia accompanied by breathlessness. The predominant symptom, dyspnoea induced by upright posture, can be debilitating and difficult to discern without thorough evaluation of the patient's pattern of dyspnoea. The precise cause of the syndrome is unclear but patients develop right to left intracardiac shunting in the presence of normal right sided cardiac pressures. Initially, patients should have confirmation of orthostatic desaturation by erect and supine pulse oximetry. However, definitive diagnosis of an orthostatic intracardiac shunt is most readily established by echocardiography. The use of echocontrast with postural manoeuvres may facilitate the diagnosis. The treatment of choice is surgical closure of the intracardiac (usually interatrial) communication, which may result in dramatic symptomatic and haemodynamic improvement. Three cases (a 27 year old man and two women aged 63 and 72 years) are described that exemplify the presentation of this syndrome, and reflect the varied management strategies and outcomes of this condition.
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10/90. The invaluable pressure-volume curve.

    We present a case in which the pressure-volume (P-V) curve proved invaluable in the diagnostic workup of a patient. The patient was a 43-year-old man who presented with progressive dyspnea on exertion, restrictive spirometry, exercise desaturation, and an unremarkable CT scan. Because of the unexpected finding of an unremarkable CT scan, we wanted more data assuring the presence of an indication for lung biopsy. Detailed pulmonary function tests, including a P-V curve, were administered. The P-V curve was abnormal, thus prompting a biopsy, which revealed hypersensitivity pneumonitis. In this report, we discuss the use of P-V curves and the clinical presentation of hypersensitivity pneumonitis.
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