[Peripheral small airway dysfunction differences between idiopathic pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension].
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Objective: To investigate the peripheral small airway dysfunction differences between idiopathic pulmonary arterial hypertension (IPAH) and chronic thromboembolic pulmonary hypertension (CTEPH). Methods: Impulse oscillmetory system testing (IOS) and pulmonary function testing (PFT) were performed in IPAH and CTEPH patients and 30 healthy control group. We also carried out a subgroup analysis depending on their medical history of airway diseases. Results: We included 42 IPAH and 47 CTEPH patients (with or without airways disease: 8 vs. 34 and 17 vs. 34, respectively). Compared with CTEPH patients, IPAH patients were younger but had more serious pulmonary vessel resistance and mean pulmonary arterial resistance. Compared with IPAH patients, CTEPH patients had significant impaired peripheral small airway dysfunction with decreased of MEF(50) (% pred), MMEF(75/25) evaluated by PFT and R5-R20, Δ R5-R20 and AX measured by IOS [10.6(2.0, 33.0) vs. 2.5(-5.0, 16.5); 22.1(14.0, 32.6) vs. 15.5 (7.0, 23.2); 7.64(4, 18.6) vs. 6(3, 11) respectively, all P<0.05]. Subgroup analysis revealed there were no significant peripheral small dysfunction differences in IPAH patients with or without airway diseases. CTEPH patients had a higher proportion of airway diseases and more serious peripheral dysfunction than IPAH patients with airway diseases. Compared with control healthy group, peripheral airway dysfunction was more obvious even in IPAH and CTEPH patients without airway diseases. Conclusion: Compared with IPAH, CTEPH patients were older, but had better hemodynamics and a higher proportion of airway diseases. The peripheral airway dysfunction were more serious in CTEPH patients without airway diseases than IPAH patients without airway diseases and healthy controls group.目的： 比较特发性肺动脉高压（IPAH）及慢性血栓栓塞性肺动脉高压（CTEPH）患者的小气道功能差异。 方法： 回顾性纳入2015年11月至2017年11月上海市肺科医院肺循环科确诊为肺动脉高压的患者89例，其中IPAH患者42例（IPAH组），其中男18例，女29例，年龄26~73岁，平均（46±16）岁；CTEPH患者47例（CTEPH组），其中男13例，女29例，年龄22~80岁，平均（61±13）岁；同期30名健康体检者作为对照组，其中男12名，女18名，年龄24~59岁，平均（54±6）岁。所有患者均进行常规肺功能及脉冲振荡肺功能（IOS）检查，分析IPAH与CTEPH患者肺功能小气道变化特点，并比较合并其他气道疾病对小气道功能的影响。 结果： 与IPAH组比较，CTEPH组患者小气道功能受累更严重，肺功能呼出50%肺活量时最大呼气流量占预计值%（MEF(50)占预计值%）在IPAH组和CTEPH组分别为（74±22）%和（59±27）%。IOS检查CTEPH组振荡频率为5和20 Hz时气道阻力差值（R(5)-R(20)）为10.6（2.0, 33.0），R(5)-R(20)与振荡频率为20 Hz时的气道阻力比值（ΔR(5)-R(20)）为22.1（14.0, 32.6），电抗面积为7.6（4.0, 18.6），均高于IPAH组[2.5（-5.0, 16.5）、15.5（7.0, 23.2）、6.0（3.0, 11.0），均P<0.05]。IPAH组中合并其他气道病变的患者与不合并气道病变的患者相比，小气道功能无明显统计学差异（P>0.05）；合并气道疾病的CTEPH患者小气道功能比合并气道疾病的IPAH患者更差；不合并气道疾病的CTEPH患者小气道功能比不合并气道疾病的IPAH患者及对照组差。 结论： 与IPAH患者相比，CTEPH患者平均确诊年龄高，确诊时血流动力学参数较好，合并气道疾病比例高，小气道功能受损更明显；不合并气道疾病的CTEPH患者的小气道功能差于不合并气道疾病的IPAH患者及健康人群。.Keywords:
Endothelial Dysfunction
Pulmonary wedge pressure
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We evaluated 74 patients with pulmonary hypertension secondary to chronic obstructive pulmonary disease and compared pulmonary function tests as well as capillary blood gas values with pulmonary hemodynamic parameters. Thirty-four patients demonstrated pulmonary hypertension only at exercise, fourty patients had pulmonary hypertension at rest (mean pulmonary artery pressure greater than 20 mm Hg). There was a significant correlation between capillary oxygen tension and mean pulmonary artery pressure as well as pulmonary vascular resistance, both at rest and during exercise. The relation between capillary PCO2 and pulmonary hemodynamics were statistically less significant. The correlation between criteria of pulmonary obstruction and/or increased lung volumes and mean pulmonary artery pressure and pulmonary vascular resistance were not as significant. The 34 patients with pulmonary hypertension only at exercise differed significantly from the other groups of patients with pulmonary hypertension at rest as seen in all pulmonary function parameters. Furthermore there was also a difference in the capillary oxygen tension during exercise, but not in the capillary carbon dioxide tension. In conclusion, of all pulmonary function tests the most efficient criteria for pulmonary hypertension are reduced FEV1/VC and low oxygen tension at rest respectively a fall during exercise.
Pulmonary wedge pressure
Oxygen tension
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Chronic obstructive pulmonary disease (COPD) is a common entity in clinical practice. Development of right ventricular hypertrophy and eventual right side heart failure is also common in such patients. However, some disturbance in left ventricular (LV) function has been observed among such patients. The aim of this study was to evaluate LV function in patients with chronic obstructive pulmonary disease (COPD) with or without pulmonary hypertension. Thirty-six patients with COPD without additional cardiac diseases and 12 age and sex-matched healthy subjects were enrolled into the study. All patients underwent spirometry, standard and tissue Doppler echocardiography. 20 COPD patients (55.6%) had pulmonary hypertension. Left ventricular systolic function did not differ between patient and control groups. However the difference between both groups was significant regarding left ventricular diastolic function and left ventricular global function. Left ventricular diastolic function and global function differed significantly between different COPD grades. Patients with pulmonary hypertension had significantly higher heart rate, less E wave peak velocity (measured by DTI) (P ⩽ 0.05), less E/A ratio (measured by DTI) (P ⩽ 0.01) and E/A ratio (measured by flow) and higher myocardial performance index (P ⩽ 0.05) than normal pulmonary pressure patients. Left ventricular diastolic function and LV global function are affected in COPD patients especially with progression of the disease. COPD patients with pulmonary hypertension are more liable to LV diastolic and global dysfunction than normal pulmonary pressure COPD patients. Doppler tissue echocardiography is a better tool in the assessment of left ventricular function.
Right ventricular hypertrophy
Ventricular pressure
Tissue Doppler echocardiography
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Attenuation of endothelial-dependent coronary vasodilation has been reported in idiopathic dilated cardiomyopathy and anatomically normal coronaries; however, data are insufficient for understanding the incidence and extent of this finding. The response of conductance and resistance coronary arteries to endothelial stimulation with acetylcholine was examined in 25 patients. Coronary blood flow had a variable response to acetylcholine and suggested coronary endothelial dysfunction in approximately half of the patients. Abnormal endothelial dysfunction involved the large conductance epicardial coronary arteries and the small resistance vessels. Abnormal endothelial response of coronary blood flow to acetylcholine could not be predicted by demographic and hemodynamic data. Conclusions: Coronary artery endothelial function is heterogeneous in patients with idiopathic dilated cardiomyopathy. Endothelial dysfunction is present in approximately half of the cases and involves both resistance as well as conductance coronary blood vessels. Furthermore, coronary endothelial function cannot be predicted by demographic and hemo-dynamic parameters or left ventricular ejection fraction.
Endothelial Dysfunction
Coronary arteries
Dilated Cardiomyopathy
Coronary circulation
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Background: Iron deficiency promotes pulmonary vascular remodeling in pre-clinical models, and is associated with worse outcomes in pulmonary arterial hypertension. However, the consequences of iron deficiency in patients with pulmonary hypertension due to chronic lung disease (Group 3 PH) are unexplored. Methods: We studied 122 consecutive Group 3 PH patients from the University of Minnesota Pulmonary Hypertension Repository. Serum soluble transferrin receptor (sTR) levels quantified iron deficiency. We evaluated the relationship between iron deficiency and pulmonary vascular disease, right ventricular (RV) function, exercise capacity, and survival. Results: The iron deficient group (<4.8mg/L sTR) had significantly higher mean pulmonary arterial pressure (40±9 mmHg, n=59 vs. 44±13 mmHg, n=61; p =.02) and lower pulmonary arterial compliance (2.2±1.2 mL/mmHg, n=52 vs. 1.7±0.8 mL/mmHg, n=55; p =.01), but there was no difference in pulmonary vascular resistance. Moreover, there were trends for higher right atrial pressure (7±4 mmHg, n=58 vs. 9±6 mmHg, n=61; p =0.08) in iron deficient patients. However, iron deficiency did not significantly alter RV function by echocardiography, 6-minute walk distance, or survival. Conclusions: Iron deficiency in Group 3 PH is associated with worse pulmonary vascular disease. This suggests iron deficiency could contribute to pulmonary vascular disease in Group 3 PH, and future studies are needed to determine if iron replacement could be a therapy for this deadly type of PH.
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