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Query: UMLS:C0392680 (shortness of breath)
 5,217 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A report is given on a 59 yr old man with hereditary spherocytosis and progressive shortness of breath on exertion, due to severe pulmonary hypertension and cor pulmonale. An open lung biopsy was performed in order to exclude all known aetiologies of secondary pulmonary hypertension. Pathological examination revealed in situ thrombosis and asymmetric fibromuscular hyperplasia of small- and medium-sized pulmonary arteries. Both primary pulmonary hypertension and hereditary spherocytosis have a low incidence in the general population and their simultaneous occurrence has not been reported previously; the possibility that this was due to a causal relationship and not to coincidence cannot be ruled out, in view of some similarities with pulmonary hypertension complicating sickle cell anaemia.
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PMID:Spherocytosis and pulmonary hypertension coincidental occurrence or causal relationship? 193 35

We report the clinical features, electrocardiographic and chest X-ray findings, and Doppler-measured pulmonary artery pressures in 22 children admitted to Goroka Base Hospital with primary pulmonary hypertension and compare them with findings in 10 controls. Symptoms frequently reported by patients were recurrent cough (95%) and shortness of breath (77%). 82% of the patients had increased intensity of the pulmonary component of the second heart sound. 45% of the patients had clinical evidence of right heart failure. Chest X-rays were readable in 14 patients: the mean cardio-thoracic ratio was 65% and in the controls 58%; the mean diameter of the descending branch of the right pulmonary artery was 9 mm and in the controls 6.4 mm. 13 patients and 8 controls had electrocardiograms: 11 patients had right axis deviation, 2 a normal axis, and all showed evidence of right ventricular hypertrophy; 7 controls had a normal axis, 1 had right axis deviation, and 3 controls had right ventricular hypertrophy. Pulmonary artery pressures in the patients ranged from 41 to 137 mmHg with a mean of 79 mmHg. The control group had a mean pulmonary artery pressure of 28 mmHg, but 5 of the 10 controls had pulmonary artery pressures greater than the accepted norm (peak systolic pressure less than 30 mmHg). Altitude and chest infection may be playing a role in the pathogenesis of pulmonary hypertension in patients in the highlands but further studies need to be done to define the causes and the pathological changes in the pulmonary vasculature, as well as to determine local norms and the natural history of pulmonary hypertension in highland children.
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PMID:Unexplained pulmonary hypertension in children in the highlands of Papua New Guinea. 857 77

An obese 23-year-old man with sleep-disordered breathing and primary pulmonary hypertension (PPH) had been administered oral beraprost sodium, anticoagulant warfarin, and home oxygen therapy, at another hospital as treatment for the PPH, but he had not experienced any symptomatic improvement. The patient had a body mass index of 32.4kg/m2, and complained of fatigue, shortness of breath on exertion, excessive daytime sleepiness, and snoring. Arterial blood gas analysis showed a PaO2 and a PaCO2 of 70.9 and 31.2mmHg, respectively. A polysomnographic study revealed central sleep apnea with an apnea-hypopnea index (AHI) of 29.7episodes/h. The patient showed improvement of daytime sleepiness after starting nocturnal nasal bilevel positive airway pressure (BiPAP) therapy for the central sleep apnea, but his pulmonary hypertension, measured in the daytime, worsened. The patient died suddenly while walking to the bathroom in the morning 1 month after initiation of BiPAP therapy. It is necessary to consider the possibility of sudden death when nasal BiPAP therapy is given to a PPH patient with central sleep apnea.
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PMID:Primary pulmonary hypertension with central sleep apnea: sudden death after bilevel positive airway pressure therapy. 1098 61

Idiopathic pulmonary hypertension is a rare disease characterized by sustained elevation of the pulmonary artery pressure and pulmonary vascular resistance, normal pulmonary artery wedge pressure, in the absence of a known cause. Prior reports suggest a very high maternal mortality in patients with idiopathic pulmonary hypertension undergoing pregnancy, and for that the recommendation has been avoidance of pregnancy (or termination if the patient is already pregnant). On the other hand, there have been multiple reports of patients with idiopathic pulmonary hypertension sustaining pregnancy and labor without major complications. This case report illustrates the course of pregnancy and labor in a patient diagnosed with idiopathic pulmonary hypertension. At age 24, the patient started with symptoms of shortness of breath and chest pain, and upon evaluation she was found with moderately severe idiopathic pulmonary hypertension. One year and 8 months later the patient becomes pregnant, and begins follow up with gynecology and cardiology. During this time the patient was asymptomatic, and did not have any clinical evidence of pulmonary hypertension. The risks of pregnancy were discussed with the patient, and she decided to continue pregnancy. She had an uneventful pregnancy, complicated only by preterm labor at 34 weeks and 5 days of gestation. She had spontaneous labor and delivered vaginally a healthy baby boy, weighting 4 pounds and 12 ounces. No invasive monitoring was used. The mother and the baby were discharged home 48 hours postpartum. Seven months later the patient returned for evaluation, presenting evidence of severe pulmonary hypertension. She has been followed up ever since by a cardiologist and currently is stable but symptomatic. This report adds to the amount of evidence that suggests that pregnancy and labor in a patient with idiopathic pulmonary hypertension may have a better outcome than previously reported. The decision of undertaking and/or continuing pregnancy in a patient with idiopathic pulmonary hypertension relies ultimately on the patient's choice, but should be done on an individual basis after careful evaluation of the risks. Finally, the need of close follow up with a multidisciplinary team is mandatory in the patient with idiopathic pulmonary hypertension that wishes to undergo pregnancy.
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PMID:Uneventful pregnancy and delivery in a patient with idiopathic pulmonary hypertension: a case report. 1720 1

Chest pain and shortness of breath are chief complaints frequently evaluated in the emergency department. ACS, pulmonary embolism, and disorders involving the lung parenchyma are some of the disease processes commonly screened for. Occasionally, patients presenting with histories and clinical exams consistent with these common illnesses may end up having more rare pathology. We present the case of a young patient who presented with chest pain and dyspnea with ECG changes and history concerning for pulmonary embolism who was ultimately diagnosed with idiopathic primary pulmonary hypertension. The importance of a prompt diagnosis of this condition along with emergency department management of complications related to the disease is discussed in this report.
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PMID:An uncommon cause of dyspnea in the emergency department. 3003 34

A 31-year old woman presented to the acute medical unit 9 days post-partum with shortness of breath and peripheral oedema. Initially suspected to have either a pulmonary embolism or post-partum cardiomyopathy, she proceeded to have imaging including a CT Pulmonary angiogram and echocardiogram, which were suggestive of pulmonary hypertension and severe right heart failure. Her history and other investigations did not reveal any obvious cause for this. She was transferred to a specialist centre where she was diagnosed with Idiopathic Pulmonary Arterial Hypertension (IPAH), previously known as primary pulmonary hypertension. Shortness of breath during pregnancy and in the postpartum period is a relatively common acute medical presentation. Whilst IPAH is a rare diagnosis, it carries a high mortality rate, particularly in pregnancy, and requires prompt specialist investigation, diagnosis and management.
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PMID:Idiopathic Pulmonary Arterial Hypertension Unmasked by Pregnancy. 3321 78