scholarly journals Primary alveolar hypoventilation and XXXXY chromosopathy

2016 ◽  
Vol 65 (3) ◽  
Author(s):  
D. Samolski ◽  
A. Antón ◽  
M. Mayos ◽  
M. Subirana ◽  
R. Güell
Keyword(s):  
Respiratory Failure ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Positive Pressure Ventilation ◽  
Cor Pulmonale ◽  
Positive Pressure ◽  
Hypercapnic Respiratory Failure ◽  
Non Invasive ◽  
Alveolar Hypoventilation ◽  
Pulmonary Arterial

The association of primary alveolar hypoventilation (PAH) and chromosomic diseases has not been described previously. A 19 year-old man with Fraccaro’s syndrome (XXXXY karyotype) was admitted to evaluate chronic hypercapnic respiratory failure, pulmonary arterial hypertension and cor pulmonale. PAH was diagnosed. As effective treatment, such as non-invasive positive pressure ventilation (NIPPV), is available for this disorder we should intensify the search for PAH in patients with chromosome disease.

scholarly journals Non-invasive positive pressure ventilation in acute hypercapnic respiratory failure: clinical experience of a respiratory ward

2004 ◽  
Vol 61 (2) ◽  
Author(s):  
R. Scala ◽  
M. Naldi ◽  
I. Archinucci ◽  
G. Coniglio
Keyword(s):  
Clinical Practice ◽  
Respiratory Failure ◽  
Standard Therapy ◽  
Positive Pressure Ventilation ◽  
Controlled Trial ◽  
Positive Pressure ◽  
Hypercapnic Respiratory Failure ◽  
Non Invasive ◽  
Arterial Blood ◽  
Acute Hypercapnic Respiratory Failure

Background: Although a controlled trial demonstrated that non-invasive positive pressure ventilation (NIV) can be successfully applied to a respiratory ward (RW) for selected cases of acute hypercapnic respiratory failure (AHRF), clinical practice data about NIV use in this setting are limited. The aim of this observational study is to assess the feasibility and efficacy of NIV applied to AHRF in a RW in everyday practice. Methods: Twenty-two percent (216/984) of patients consecutively admitted for AHRF to our RW in Arezzo (years: 1996-2003) received NIV in addition to standard therapy, according to pre-defined routinely used criteria. Tolerance, effects upon arterial blood gases (ABG), success rate (avoidance a priori criteria for intubation) and predictors of failure of NIV were analysed. Results: Nine patients (4.2%) were found to be intolerant to NIV, while the remaining 207 (M: 157, F: 50; mean (SD) age: 73.2 (8.9) yrs; COPD: 71.5%) were ventilated for >1 hour. ABG significantly improved after two hours of NIV (pH: 7.32 (0.06) versus median (Interquartiles) 7.28 (7.24-7.31), p<0.0001; PaCO2: 71.9 (13.5) mmHg versus 80.0 (15.2) mmHg, p<0.0001; PaO2/FiO2: 212 (66) versus 184 (150-221), p<0.0001). NIV succeeded in avoiding intubation in 169/207 patients (81.6%) with hospital mortality of 15.5%. NIV failure was independently predicted by Activity of Daily Living score, pneumonia as cause of AHRF and Acute Physiology and Chronic Health Evaluation III score. Conclusions: In clinical practice NIV is feasible, effective in improving ABG and useful in avoiding intubation in most AHRF episodes that do not respond to the standard therapy managed in an RW adequately trained in NIV.

scholarly journals Right ventricular stroke work index by echocardiography in adult patients with pulmonary arterial hypertension

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Raluca Jumatate ◽  
Annika Ingvarsson ◽  
Gustav Jan Smith ◽  
Anders Roijer ◽  
Ellen Ostenfeld ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Right Ventricular ◽  
Adult Patients ◽  
Stroke Work ◽  
Non Invasive ◽  
Work Index ◽  
Stroke Work Index ◽  
Pulmonary Arterial ◽  
Rv Function

Abstract Background In adult patients with pulmonary arterial hypertension (PAH), right ventricular (RV) failure may worsen rapidly, resulting in a poor prognosis. In this population, non-invasive assessment of RV function is challenging. RV stroke work index (RVSWI) measured by right heart catheterization (RHC) represents a promising index for RV function. The aim of the present study was to comprehensively evaluate non-invasive measures to calculate RVSWI derived by echocardiography (RVSWIECHO) using RHC (RVSWIRHC) as a reference in adult PAH patients. Methods Retrospectively, 54 consecutive treatment naïve patients with PAH (65 ± 13 years, 36 women) were analyzed. Echocardiography and RHC were performed within a median of 1 day [IQR 0–1 days]. RVSWIRHC was calculated as: (mean pulmonary arterial pressure (mPAP)—mean right atrial pressure (mRAP)) x stroke volume index (SVI)RHC. Four methods for RVSWIECHO were evaluated: RVSWIECHO-1 = Tricuspid regurgitant maximum pressure gradient (TRmaxPG) x SVIECHO, RVSWIECHO-2 = (TRmaxPG-mRAPECHO) x SVIECHO, RVSWIECHO-3 = TR mean gradient (TRmeanPG) x SVIECHO and RVSWIECHO-4 = (TRmeanPG–mRAPECHO) x SVIECHO. Estimation of mRAPECHO was derived from inferior vena cava diameter. Results RVSWIRHC was 1132 ± 352 mmHg*mL*m−2. In comparison with RVSWIRHC in absolute values, RVSWIECHO-1 and RVSWIECHO-2 was significantly higher (p < 0.001), whereas RVSWIECHO-4 was lower (p < 0.001). No difference was shown for RVSWIECHO-3 (p = 0.304). The strongest correlation, with RVSWIRHC, was demonstrated for RVSWIECHO-2 (r = 0.78, p < 0.001) and RVSWIECHO-1 ( r = 0.75, p < 0.001). RVSWIECHO-3 and RVSWIECHO-4 had moderate correlation (r = 0.66 and r = 0.69, p < 0.001 for all). A good agreement (ICC) was demonstrated for RVSWIECHO-3 (ICC = 0.80, 95% CI 0.64–0.88, p < 0.001), a moderate for RVSWIECHO-4 (ICC = 0.73, 95% CI 0.27–0.87, p < 0.001) and RVSWIECHO-2 (ICC = 0.55, 95% CI − 0.21–0.83, p < 0.001). A poor ICC was demonstrated for RVSWIECHO-1 (ICC = 0.45, 95% CI − 0.18–0.77, p < 0.001). Agreement of absolute values for RVSWIECHO-1 was − 772 ± 385 (− 50 ± 20%) mmHg*mL*m−2, RVSWIECHO-2 − 600 ± 339 (-41 ± 20%) mmHg*mL*m−2, RVSWIECHO-3 42 ± 286 (5 ± 25%) mmHg*mL*m−2 and for RVSWIECHO-4 214 ± 273 (23 ± 27%) mmHg*mL*m−2. Conclusion The correlation with RVSWIRHC was moderate to strong for all echocardiographic measures, whereas only RVSWIECHO-3 displayed high concordance of absolute values. The results, however, suggest that RVSWIECHO-1 or RVSWIECHO-3 could be the preferable echocardiographic methods. Prospective studies are warranted to evaluate the clinical utility of such measures in relation to treatment response, risk stratification and prognosis in patients with PAH.

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