Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0729233 (Thoracic)
 6,478 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Primary pulmonary hypertension (PPH) is a progressive disease characterised by raised pulmonary vascular resistance, which results in diminished right-heart function due to increased right ventricular afterload. PPH occurs most commonly in young and middle-aged women; mean survival from onset of symptoms is 2-3 years. The aetiology of PPH is unknown, although familial disease accounts for roughly 10% of cases, which suggests a genetic predisposition. Current theories on pathogenesis focus on abnormalities in interaction between endothelial and smooth-muscle cells. Endothelia-cell injury may result in an imbalance in endothelium-derived mediators, favouring vasoconstriction. Defects in ion-channel activity in smooth-muscle cells in the pulmonary artery may contribute to vasoconstriction and vascular proliferation. Diagnostic testing primarily excludes secondary causes. Catheterisation is necessary to assess haemodynamics and to evaluate vasoreactivity during acute drug challenge. Decrease in pulmonary vascular resistance in response to acute vasodilator challenge occurs in about 30% of patients, and predicts a good response to chronic therapy with oral calcium-channel blockers. For patients unresponsive during acute testing, continuous intravenous epoprostenol (prostacyclin, PGI2) improves haemodynamics and exercise tolerance, and prolongs survival in severe PPH (NYHA functional class III-IV). Thoracic transplantation is reserved for patients who fail medical therapy. We review the progress made in diagnosis and treatment of PPH over the past 20 years.
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PMID:Primary pulmonary hypertension. 972 4

Based on data reported to the UNOS/ISHLT International Registry for Thoracic Organ Transplantation, we showed that: 1. The number of heart transplant operations performed in the United States has decreased by 164 procedures between 1998 (2,346) and 1999 (2,182). The number of lung transplants increased by 13 in 1999 to 877. 2. The most frequently reported indication for heart transplantation in the US is coronary artery disease (44.8%). For other thoracic transplants, the most frequently reported indications include cystic fibrosis (35.5%) for double lung, emphysema/COPD (49.7%) for single lung and congenital heart disease (46.6%) for heart-lung. The most frequently reported diagnoses for thoracic transplantation outside the US include cardiomyopathy (43.8%) for heart, cystic fibrosis (33.4%) for double-lung, emphysema/COPD (26.6%) for single-lung and primary pulmonary hypertension (24.8%) for heart-lung transplants. 3. US heart transplant recipients are predominately male (76.7%), between 50 and 64 years of age (51.3%) and white (81.4%). US lung transplant recipients are also predominately between 50 and 64 years of age (44.7%) and white (89.9%), but unlike heart recipients are more likely to be female (51.2%). No meaningful variance from the US recipient demographic profile is noted for the non-US recipients during the same time period. 4. Pediatric recipients (< 18 years of age) received 10.9% of the reported heart transplants and 6.2% of reported lung transplants. 5. One-year survival for thoracic transplants performed in the US is 82.4% for heart, 74.1% for lung and 62.0% for heart-lung. Five-year survival for US thoracic transplants is 66.8% for heart and 43.2% for lung. 6. Long-term patient survival rates are: 22.5% at 17 years for heart, 20.8% at 10 years for lung and 24.3% at 13 years for heart-lung recipients. 7. The most important risk factor for mortality of US heart recipients at one month, one year and conditionally at 5 years after transplantation was receipt of a previous heart transplant. Significant short-term risk factors include donor age, recipient age and ischemic time. Substantial long-term risk factors include older donor age, recipient age, recipient race and diagnosis. 8. The factors having the most significant impact on lung mortality at all time points are related to either the patient's medical condition (e.g., in the ICU prior to transplant, requiring mechanical ventilation) or diagnosis. 9. Mechanical ventilation, recipient race and recipient age have the largest impact on heart-lung mortality. 10. For heart and lung recipients, the major cause of hospitalization during the first year after transplantation is infection alone.
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PMID:Worldwide thoracic organ transplantation: a report from the UNOS/ISHLT International Registry for Thoracic Organ Transplantation. 1151 24

Based on data reported to the UNOS/ISHLT Thoracic and International Registry for Thoracic Organ Transplantation: 1. The number of heart transplant operations performed in the United States decreased between 1998-1999 and 17 (1%) more procedures were performed in 1999 (2,181) than in 2000 (2,198). Sixty-nine more lung transplants (an 8% increase) were reported in 2000 than in 1999. 2. Coronary artery disease and cardiomyopathy were the most frequently cited indications for heart transplantation in the US and have been reported at similar rates during the past 10 years. Combined, these diagnoses account for approximately 85% of all heart transplants. In 2000, half of all lung transplants were performed for emphysema/COPD or alpha-1 antitrypsin deficiency. The most frequently reported diagnoses for thoracic transplantation outside the US were: cardiomyopathy (49%) for heart, cystic fibrosis (30%) for double lung, emphysema/COPD (34%) for single lung and primary pulmonary hypertension (21%) for heart-lung transplants. 3. US heart transplant recipients were predominately male (76%), aged 50-64 (51%) and white (81%). US lung transplant recipients were also predominately between ages 50-64 (47%) and white (90%), but unlike heart recipients were more likely to be female (51%). No meaningful variance from the US recipient demographic profile was noted for the non-US recipients during the same time period. 4. Pediatric recipients (< 18 years of age) received 11% of the reported heart transplants and 6% of the reported lung transplants in the US. 5. Among US thoracic transplant recipients during 1999, the one-year survival rates were 84% for heart, 59% for heart-lung and 77% for lung. The 5-year survival rates for transplants performed during 1995 were: 71% for heart, 56% for heart-lung and 44% for lung transplants. 6. The long-term patient survival rates were: 23% at 19 years for heart, 16% at 11 years for lung and 23% at 14 years for heart-lung recipients. 7. During the first year after transplantation, 66% of heart recipients and 44% of lung recipients did not require rehospitalization. Among those recipients who were rehospitalized, the major cause was infection.
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PMID:Worldwide thoracic organ transplantation: a report from the UNOS/ISHLT international registry for thoracic organ transplantation. 1221 89

Thoracic organ transplantation made a fresh start in Hungary with the first double lung transplant in December 2015. This major leap in Hungarian transplantation was preceded by almost 10 years of preparation, new infrastructure development, and structural changes not only at the organizational level but in human resources as well. In the following years, until recently, altogether 47 lung transplants were performed on 24 men and 23 women. The underlying pathologies were as follows: chronic obstructive pulmonary disease, 25; cystic fibrosis, 11; idiopathic pulmonary fibrosis, 7; as well as other diseases, including bronchiectasis, eosinophilic granuloma, lymphangioleiomyomatosis, and primary pulmonary hypertension in 4 cases. The youngest recipient was 13 and the oldest was 65 years old. Overall survival rates at 30 days and at 1 year were 96% and 82%, respectively. No patients were lost in the cystic fibrosis and other diseases group, whereas the 1-year survival rates of the chronic obstructive pulmonary disease and idiopathic pulmonary fibrosis groups were 73% and 71%, respectively. The results show the robustness and viability of the program, although there is still opportunity for further improvement. In this short paper, we summarize the fields of possible further cooperation of thoracic and cardiac teams as well as future challenges facing the new Hungarian lung transplant program.
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PMID:Lung Transplantation in Hungary From Cardiac Surgeons' Perspective. 3110 Dec 11