Unilateral absence of the pulmonary artery: a report of two cases that presented different clinical courses.

We recently diagnosed two cases of isolated unilateral absence of the pulmonary artery just after birth. Through the therapy, we could not prevent obstruction of the pulmonary artery and lead to complications. There have been no symptoms attributed to isolated unilateral absence of the pulmonary artery so far. We should carefully consider the strategy for therapeutic intervention for asymptomatic cases.

Advanced cuff pressure control ventilation (ACPCV); a bench study of a new concept of mechanical ventilation.

The concept of advanced cuff pressure control ventilation (ACPCV) is that the endotracheal tube (ETT) cuff volume could be controlled and allowed to exhale the gas through the vocal cords. The potential advantages of ACPCV are reduction of dead space, reduction of expiratory airway resistance, and preservation of vocal cord function. We developed the ACPCV system and investigated its performance in bench studies. The ETT cuff volume was regulated via four steps, depending on airway pressure and tracheal pressure. Two ventilatory settings were examined under several rates of spontaneous breathing efforts. Imposed expiratory resistance (RE), imposed expiratory work of breathing (WOB), and auto-PEEP of ACPCV were compared with continuous mandatory ventilation (CMV). RE of ACPCV (2.6 ± 0.5 cm H2O/l/s) was significantly lower than that of CMV (11.6 ± 1.6 cm H2O/l/s) (p < 0.001). Expiratory WOB of ACPCV (0.25 ± 0.02 J/l) was significantly lower than that of CMV (0.54 ± 0.10 J/l) (p < 0.001). Auto-PEEP of ACPCV (-0.6 ± 0.2 cm H2O) was significantly lower than that of CMV (1.1 ± 0.7 cm H2O) (p < 0.001). ACPCV can significantly reduce RE and expiratory WOB by controlling the ETT cuff volume in synchronisation with mechanical ventilation.

Predictors of post-extubation stridor in patients on mechanical ventilation: a prospective observational study.

The cuff leak test (CLT) has been widely accepted as a simple and noninvasive method for predicting post-extubation stridor (PES). However, its accuracy and clinical impact remain uncertain. We aimed to evaluate the reliability of CLT and to assess the impact of pre-extubation variables on the incidence of PES. A prospective observational study was performed on adult critically ill patients who required mechanical ventilation for more than 24 h. Patients were extubated after the successful spontaneous breathing trial, and CLT was conducted before extubation. Of the 191 patients studied, 26 (13.6%) were deemed positive through CLT. PES developed in 19 patients (9.9%) and resulted in a higher reintubation rate (8.1% vs. 52.6%, p < 0.001) and longer intensive care unit stay (8 [4.5-14] vs. 12 [8-30.5] days, p = 0.01) than patients without PES. The incidence of PES and post-extubation outcomes were similar in patients with both positive and negative CLT results. Compared with patients without PES, patients with PES had longer durations of endotracheal intubation and required endotracheal suctioning more frequently during the 24-h period prior to extubation. After adjusting for confounding factors, frequent endotracheal suctioning more than 15 times per day was associated with an adjusted odds ratio of 2.97 (95% confidence interval, 1.01-8.77) for PES. In conclusion, frequent endotracheal suctioning before extubation was a significant PES predictor in critically ill patients. Further investigations of its impact on the incidence of PES and patient outcomes are warranted.

Constitutively active form of natriuretic peptide receptor 2 ameliorates experimental pulmonary arterial hypertension.

We recently found a constitutively active mutant of natriuretic peptide receptor 2 (caNPR2; V883M), which synthesizes larger amounts of cyclic guanosine monophosphate (cGMP) intracellularly without any ligand stimulation than existing drugs. The aim of this study was to investigate the therapeutic effects of gene transduction using caNPR2 for pulmonary arterial hypertension (PAH). In vitro gene transduction into human pulmonary arterial smooth muscle cells using Sendai virus (SeV) vectors carrying caNPR2 induced 10,000-fold increases in the synthesis of cGMP without ligand stimulation, and the proliferation of caNPR2-expressing cells was significantly attenuated. The PAH model rats generated by hypoxia and the administration of SU5416 were then treated with SeV vectors through a direct injection into the left pulmonary artery. Right ventricular systolic pressure was significantly decreased 2 weeks after the treatment, while systemic blood pressure remained unchanged. Histological analyses revealed that the medial wall thickness and occlusion rate of pulmonary arterioles were significantly improved in caNPR2-treated lungs. Neither the systemic integration of virus vectors nor side effects were observed. The massive stimulation of cGMP synthesis by gene therapy with caNPR2 was safe and effective in a PAH rat model and, thus, has potential as a novel therapy for patients with severe progressive PAH.