P1‐46: A case of bronchial obstruction due to pulmonary hypertension

Background. Pulmonary capillary hemangiomatosis (PCH) is a rare disease, especially in infancy. Four infants have been reported up to the age of 12 months. So far, no familial patients are observed at this age.Patients. We report three siblings, two female newborns and a foetus of 15-week gestation of unrelated, healthy parents suffering from histologically proven PCH. The first girl presented with increased O2requirements shortly after birth and patentductus arteriosus(PDA). She subsequently developed progressive respiratory failure and pulmonary hypertension and died at the age of five months. The second girl presented with clinical signs of bronchial obstruction at the age of three months. The work-up showed a PDA—which was surgically closed—pulmonary hypertension, and bronchial wall instability with stenosis of the left main bronchus. Transient oxygen therapy was required with viral infections. The girl is now six years old and clinically stable without additional O2requirements. Failure to thrive during infancy and a somewhat delayed development may be the consequence of the disease itself but also could be attributed to repeated episodes of respiratory failure and a long-term systemic steroid therapy. The third pregnancy ended as spontaneous abortion. The foetus showed histological signs of PCH.Conclusion. Despite the differences in clinical course, the trias of PCH, PDA, and pulmonary hypertension in the two life born girls suggests a genetic background.

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Mechanisms for compensation of pulmonary hypertension in chronic obstructive pulmonary disease

Arterial’naya Gipertenziya (Arterial Hypertension) ◽

10.18705/1607-419x-2018-24-3-339-350 ◽

2018 ◽

Vol 24 (3) ◽

pp. 339-350

Author(s):

V. P. Zolotnitskaya ◽

T. D. Vlasov ◽

V. I. Amosov ◽

O. V. Lukina ◽

A. O. Agafonov

Keyword(s):

Blood Flow ◽

Pulmonary Hypertension ◽

Pulmonary Artery ◽

Photon Emission ◽

Diagnostic Methods ◽

Bronchial Obstruction ◽

External Respiration ◽

True Incidence ◽

Pulmonary Parenchyma ◽

Increased Blood Flow

Despite multiple risk factors of pulmonary hypertension (PH) are known, the true incidence of PH remains undefined in patients with severe bronchial obstruction. Objective. The aim of the study was to identify mechanisms of compensation of the increase in pulmonary artery pressure and to determine their prognostic role. Design and methods. The results of a complex clinical and radiological studies of 200 patients with COPD II (B), and 207 patients with COPD III (D), and COPD IV (D), (GOLD 2017) were analyzed. A comparative analysis of changesin pulmonary circulation in patients (n = 8) with primary PH was performed. Male subjects predominated among the examined patients with COPD (90,6 %), the average age of patients with COPD II was 54,0 ± 6,3 years, in patients with COPD III–IV stages — 63,9 ± 5,8 years. The average age of patients with primary PH was 38,7 ± 7,9 years. The following diagnostic methods were performed: single-photon emission computer tomography, multidetector computed tomography, external respiration function, echocardiography. Results. In patients with COPD II (B), both ventilation and perfusion disorders developed, the diffusion capacity of the lungs decreased and the intrapulmonary shunting of the blood occurred. Despite overall decrease in pulmonary perfusion, local zones of increased blood flow appeared in the unchanged pulmonary parenchyma. This compensation mechanism was observed in 54,6 % of patients and was accompanied by partial decrease of pressure in the pulmonary artery to values close to normal. In severe COPD, 44,4 % patients developed hyperperfusion zones, but without any compensatory effect. In such cases, pressure reduction in the pulmonary artery system was promoted by the development of blood bypass, which was observed only in 10 % cases. Conclusions. We can speculate that the mechanism of compensation of increased pressure in the pulmonary artery and reduction of PaO2 results from the development of local zones of increased blood flow in the unchanged pulmonary parenchyma, but in severe bronchial obstructive pathology it causes the formation of blood shunting.

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Impaired Pulmonary Vascular Adaptation to Exercise in Emphysema without Pulmonary Hypertension – A Proof-of-Concept Study

10.21203/rs.3.rs-1074821/v1 ◽

2021 ◽

Author(s):

Thibaut Capron ◽

Axel Bartoli ◽

Stanislas Rappacchi ◽

Clarisse Gautier ◽

Faiza Bettayeb ◽

...

Keyword(s):

Magnetic Resonance Imaging ◽

Pulmonary Hypertension ◽

Cardiac Output ◽

Magnetic Resonance ◽

Bronchial Obstruction ◽

Resonance Imaging ◽

Pulmonary Hemodynamics ◽

Ventricular Ejection Fraction ◽

Vascular Adaptation ◽

Adaptation To Exercise

Abstract Background: Chronic obstructive pulmonary disease with emphysema lead to respiratory disability beyond bronchial obstruction. The functional impact of pulmonary vascular lesions in emphysema remains unknown. We investigated pulmonary vascular adaptation to exercise in patients with extended emphysema.Methods: Chest magnetic resonance imaging was used to quantitatively assess right-heart function, pulmonary artery and distal pulmonary blood flow. This was performed at rest and during cycling exercise with a magnetic resonance imaging-compatible cyclo-ergometer. Seven emphysematous patients without pulmonary hypertension were compared to 7 healthy non-smokers matched in gender and age.Results: At rest, cardio-pulmonary hemodynamics and distal pulmonary vascular parameters were similar in both groups. Intrasubject adaptation to exercise in emphysematous patients was characterized by a higher increase in right-ventricular ejection fraction (ΔRVEF +8.1 vs. -2.4 %, P=0.046) though a lower right-cardiac output (4.41 vs. 5.79 L/min, P=0.04) at exercise. Accounting for right-cardiac output variation, the distal pulmonary vascular yield index trended to be decreased in patients (ΔPBF/ΔQf -0.78 vs. +18.83 %, P=0.18).Conclusions: Pulmonary vascular adaptation to exercise is impaired in emphysematous patients without identified pulmonary hypertension.Clinical trial registration NCT 04126616.

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