Cases reported "Pulmonary Heart Disease"

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1/20. Cor pulmonale presenting in a patient with congenital kyphoscoliosis following intercontinental air travel.

    We present the case of a 59-year-old man with congenital kyphoscoliosis who developed cor pulmonale for the first time following intercontinental air travel. Prolonged exposure to the low partial pressure of oxygen in the cabin of the aircraft led to pulmonary hypertension and right heart failure. The case highlights the potential for long-haul air travel to cause decompensation in patients with thoracic deformity and apparently stable cardiorespiratory function. It also emphasises the need for patients and their medical attendants to carefully consider the potential health implications of the hypoxic atmosphere in pressurised aircraft.
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2/20. Hurler's syndrome with cor pulmonale secondary to obstructive sleep apnoea treated by continuous positive airway pressure.

    A 6-year-old boy with Hurler's syndrome presented with right heart failure and pulmonary hypertension secondary to severe obstructive sleep apnoea. Both his sleep apnoea and cor pulmonale were effectively controlled with continuous positive airway pressure therapy.
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3/20. Neuromuscular disease, respiratory failure and cor pulmonale.

    Respiratory muscle weakness is an uncommon cause of chronic respiratory failure and a rare cause of cor pulmonale. The problem may not be apparent unless specific physical signs are sought or appropriate investigations performed. We present three patients who presented diagnostic difficulty for prolonged periods until the presence of respiratory muscle weakness was considered. Once the diagnosis was established treatment with nocturnal nasal intermittent positive pressure ventilation produced a dramatic improvement in symptoms and allowed a return to a near normal lifestyle.
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4/20. Reconstruction of bilateral branch pulmonary artery stenosis caused by Takayasu's aortitis.

    A 63 year-old female presented with dyspnea on exertion. Her chest X-ray showed cardiomegaly, and right ventricular overload and tricuspid regurgitation were detected. Her pulmonary ventilation and blood flow scintigraphy findings were suspicious of pulmonary vascular disease; the diagnosis was pulmonary hypertension and bilateral branch pulmonary artery stenosis. After the inflammation settled, the stenotic bilateral branch pulmonary artery was reconstructed with a prosthetic vessel and the pulmonary pressure normalized immediately. A resected specimen revealed that the stenotic changes were from Takayasu's disease. The patient's postoperative course was uneventful, and pulmonary ventilation and blood scintigraphy returned to an almost normal range. At follow-up 5 years and 6 months after the operation, there was no evidence of pulmonary artery disease (eg, stenosis and/or ischemia) or of any change in the central vessels of the retina, the so-called Takayasu's retinopathy.
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5/20. Left ventricular cardiac tamponade in the setting of cor pulmonale and circumferential pericardial effusion. Case report and review of the literature.

    Circumferential pericardial effusion typically results in biventricular tamponade and equalization of intracardiac and pericardial pressure during diastole. However, tamponade may involve the right or left ventricle. While isolated left ventricular cardiac tamponade (LVCT) can occur as a postoperative complication from localized posterior pericardial effusions, circumferential pericardial effusions leading to LVCT are rare. We report a case of a patient with severe pulmonary hypertension, a large nonloculated pericardial effusion, and LVCT, which was probably due to a chronic undifferentiated connective tissue disorder. This case illustrates that when evaluating patients with circumferential pericardial effusions and associated pulmonary hypertension, the typical findings of cardiac tamponade (pulsus paradoxus, right ventricular diastolic compression and hypotension) may be masked. The echocardiogram must be reviewed carefully as it may reveal left ventricular diastolic compression, the hallmark of LVCT, which may significantly compromise left ventricular filling and cardiac output.
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6/20. Case report: intermittent, positive pressure ventilation-dependent right bundle branch block.

    A 73-year-old man with severe chronic obstructive pulmonary disease and cor pulmonale developed an intermittent right bundle branch block (RBBB) during mechanical ventilation. The appearance and disappearance of his RBBB was temporally associated with each positive pressure breath. Intermittent RBBB is a previously undescribed complication of positive pressure ventilation.
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7/20. recurrence of sleep apnea syndrome following tracheostomy. A shift from obstructive to central apnea.

    This report describes an unusual case of severe obstructive sleep apnea and alveolar hypoventilation leading to hypersomnolence and cor pulmonale, which were corrected by tracheostomy. Four years later, after a 22.5-kg weight gain, nocturnal apneas of similar frequency, duration, and depth of desaturation reappeared but were totally central in origin. The central apneas were eliminated with home nocturnal positive-pressure ventilation via cuffed tracheostomy tube. Each time the patient's apneas were corrected (obstructive: tracheostomy; central: mechanical ventilation), daytime alveolar hypoventilation disappeared rapidly. Yearly right heart catheterizations and radionuclide ejection fractions documented pulmonary hypertension and right heart failure, with resolution following tracheostomy and recurrence after appearance of central apneas. The changes in hemodynamic status corresponded to the patient's weight, presence of apnea, daytime alveolar hypoventilation, and treatment of nocturnal oxyhemoglobin desaturation. This case illustrates the theory of a common etiology of both central and obstructive apnea through abnormal respiratory controller gain and points to several roles obesity may play in apnea.
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8/20. Treatment of pulmonary hypertension with diltiazem in a child with bronchopulmonary dysplasia.

    A two-year-old child dying of pulmonary hypertension and cor pulmonale secondary to bronchopulmonary dysplasia, was demonstrated to have reactive pulmonary hypertension in response to 100% oxygen and isoproterenol infusion. In an attempt to find an oral medication to maintain pulmonary vasodilatation, experimental trials were done using hydralazine, salbutamol, nifedipine and diltiazem. Cardiac index, pulmonary and systemic vascular resistances and intrapulmonary shunts were monitored during the trials. hydralazine, salbutamol and nifedipine were ineffective. diltiazem 2.0 mg given every 6 h resulted in a profound and sustained decrease in pulmonary pressures and resistance, and a reversal of the cor pulmonale.
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9/20. pierre robin syndrome and pulmonary hypertension.

    Five infants with pierre robin syndrome developed evidence of carbon dioxide retention and congestive cardiac failure despite measures to alleviate upper airway obstruction. Investigations included chest radiography, electrocardiography, echocardiography, and cardiac catheterization; pulmonary hypertension was diagnosed. In two cases raised main pulmonary artery pressures of 40 mm Hg and 120 mm Hg were recorded. Relief of upper airway obstruction was achieved by tracheostomy in three cases and nasopharyngeal intubation in two cases, with reversal of signs of cor pulmonale in each. Four patients progressed well with no recurrence of cardiac problems but one died suddenly one month after apparently successful management by tracheostomy.
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10/20. nifedipine inhibits hypoxic pulmonary vasoconstriction during rest and exercise in patients with cystic fibrosis and cor pulmonale.

    nifedipine has recently been reported to reduce pulmonary artery pressure and pulmonary vascular resistance during rest and exercise in adult patients with hypoxic pulmonary hypertension from chronic obstructive pulmonary disease. To determine whether nifedipine reduces pulmonary vascular resistance in patients with cor pulmonale from cystic fibrosis (CF), we studied 2 patients with severe CF lung disease during rest and exercise while breathing room air or receiving low-flow oxygen therapy. nifedipine markedly lowered pulmonary vascular resistance and improved cardiac index and pulmonary pressure-flow relationships during all treatment conditions. nifedipine did not substantially change arterial PO2, except for a slight decrease during exercise while receiving low-flow oxygen. nifedipine, however, markedly increased oxygen delivery during rest and exercise. Both patients noted improved exercise tolerance with chronic nifedipine therapy. nifedipine may be a useful adjuvant to supplemental oxygen in the treatment of patients with CF and cor pulmonale.
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