Introduction We present a case of diffuse distal congenital pulmonary artery hypoplasia with elevated right ventricular systolic pressures, successfully treated with pulmonary artery stenting. Case Report A 35-year-old female presented with increasing exertional dyspnoea, on a background of diffuse distal congenital pulmonary artery hypoplasia due to an elastin gene mutation and morbid obesity.There was evidence of mild right heart failure on examination and oxygen saturations were normal. Her transthoracic echocardiogram showed right ventricular (RV) hypertrophy with moderately impaired systolic function, moderate tricuspid regurgitation and an estimated RV systolic pressure (RVSP) of 120 mmHg. Her right atrial pressure was 8 mmHg on right heart catheterisation, RVSP was 138 mmHg, mean pulmonary artery pressure (mPAP) was 84 mmHg, with a normal cardiac output and pulmonary capillary wedge pressure. A decision was made for an experienced interventional radiologist to proceed initially with balloon pulmonary angioplasty (BPA). Her obesity made her an unsuitable candidate for lung transplantation. BPA to the left and right lower lobe pulmonary artery basal segments was performed, without clinical improvement. One week later, cutting BPA was performed to the left lower lobe pulmonary artery and its A7/8 and A9/10 trunks, without improvement. Finally, PA stenting was undertaken to the left lower lobe main branch and the proximal A7/8 and A9/10 trunks, right lower lobe PA and the right middle lobe A4/5 trunk. Completion angiography showed improved flow to all stented segments, except A4. The mPAP was 36 mmHg and there were no complications.The patient experienced a marked improvement in exercise tolerance at six months follow-up with normal RV contractility. Summary We present a rare case of congenital pulmonary artery hypoplasia treated with BPA and subsequent successful PA stenting. Treatments for this condition not well characterised, highlighting the importance of this case.

Abstract Introduction COPD/OSA overlap syndrome (OVS) is associated with poor outcomes in studies conducted in ambulatory settings. However, little is known about the prognosis of patients hospitalised with acute hypercapnic respiratory failure (ARF) requiring NIV. The aim of this study was to compare the long-term prognosis of OVS patients compared to patients with COPD. Methods In this retrospective cohort study, 129 patients with COPD and 52 OVS patients were treated with NIV for ARF and followed up for a median of 1.8 years (IQR 3.8). We compared patient characteristics and overall survival. Results Compared to patients with COPD alone, OVS patients had higher prevalence of hypertension and type-2 diabetes mellitus. There was no difference in arterial pH, PaCO2 or serum bicarbonate at hospital presentation. Mortality was lower in OVS (HR 0.57, 95% CI 0.38-0.85) and remained lower after adjustment for age, gender, BMI, FEV1%predicted and comorbid cardiovascular disease. Median survival in OVS patients discharged home on NIV was significantly higher compared to OVS not discharged on therapy, as well as COPD patients, irrespective of home therapy prescription (p<0.01). Discussion OVS patients discharged on NIV had lower mortality compared to OVS patients not discharged on NIV. Patients with COPD and ARF requiring NIV have overall higher mortality rates compared to OVS patients. These findings suggest that following hospital admission with ARF, OVS patients may benefit from ongoing home NIV.

The experience of outpatient care may differ for selected patient groups. This prospective, observational study evaluates the patient experience of multidisciplinary outpatient Cystic Fibrosis (CF) care via telehealth compared with face-to-face care the year prior, by place of residence and presence of multi-resistant microbiota.

In normal conditions, impedance of the pulmonary circulation (PA-Zc) is one-third lower than the systemic circulation (Ao-Zc) with ventricular function coupled to the arterial circulation by relative matching between contractility and afterload. Simultaneous measurement of Ao-Zc and PA-Zc using a cardiac magnetic resonance (CMR) technique has not previously been performed.

The relationship between initial treatment strategy and survival in pulmonary arterial hypertension (PAH) is uncertain, although early combination therapy is proposed by current guidelines for majority of patients.

Purpose Impairment of left ventricular (LV) relaxation is associated with increased mortality after surgical treatment of pulmonary hypertension (PH). We sought to describe the pathophysiology of LV diastolic dysfunction in a porcine model of chronic thromboembolic pulmonary hypertension (CTEPH). The reversibility of LV diastolic dysfunction was investigated in patients with CTEPH after pulmonary endarterectomy (PEA). Methods CTEPH was induced in 2-month-old Large White piglets (PH group, n=6) by ligation of the left pulmonary artery (PA) followed by weekly embolization of right lower lobe for 5 weeks using a strong tissue glue (N-acetyl cyanoacrylate). These animals were compared to sham-operated animals (controls, n=6). LV diastolic function was assessed using echocardiography and conductance catheter measurements. LV fibrosis was investigated at 6 weeks using red Sirius staining of myocardial tissues. Echocardiographic measurements for LV diastolic function were retrospectively analyzed in 102 patients, before and after PEA. Results Mean PA pressure was higher at 6 weeks in PH animals compared to controls (28.5 [28.0; 34.2] vs. 14.0 [12.5; 14.0] mmHg, p<0.01). Increased end-diastolic LV pressure was observed in PH group (21.9 [18.1; 22.7] vs. 12.2 [11.7; 13.8] mmHg, p=0.013), along with a marked decrease in the curve-fitting constant (c) and the maximum rate of LV filling (dV/dtmax), respectively by 49% (p=0.03) and 74% (p=0.014). Stiffness constant ß was strongly correlated with Doppler imaging index E/A (r=-0.94, p=0.015). Mean LV fibrosis score was significantly higher in PH group at 6 weeks (5.11±0.89% vs. 3.29±1.14%, p<0.01). Pre-operative impairment of LV filling pattern was remarkable in patients with CTEPH (E/A = 0.81±0.32; E/E'=6.42±2.84), and significantly improved at 7 days post PEA (+30%, p<0.001). At 6 months, E/A ratio was significantly higher (0.91 [0.75; 1.20] vs. 0.76 [0.66; 0.94], p=0.05) but remained abnormal, despite significant decrease in mean pulmonary vascular resistance (7.3 ±3.1WU vs. 3.8±1.5WU, p<0.001). Conclusion Impaired myocardial stiffness was associated with LV fibrosis in our piglet model of CTEPH. Mild LV diastolic dysfunction was observed at 6 months in patient with CTEPH despite significant decrease in RV pressure overload after PEA. Myocardial fibrosis may be responsible for persistent abnormal LV relaxation.

Paediatric pulmonary arterial hypertension is a rare disease but causes significant morbidity and mortality where it is found. Literature on survival and response to therapy is scant.

RATIONALE: In Chronic Obstructive Pulmonary Disease (COPD), expiratory flow limitation and gas trapping cause an increase in FRC, known as hyperinflation. Hyperinflation impedes diaphragm function, causes breathlessness and reduces exercise capacity. In healthy people, elastic chest compression reduced FRC. Therefore, we investigated whether elastic chest compression could reduce hyperinflation in patients with COPD. METHODS: Eight patients with COPD and gas trapping (RV > 120% predicted) performed body plethysmography at baseline and with elastic chest compression placed at the Xiphisternum and over the abdomen. Participants then underwent two magnetic resonance imaging scans of the thorax without and with elastic chest compression to measure the height, length and angle of the diaphragm at FRC. Data are presented as mean ± SD. RESULTS: COPD participants were 66±7.9 years old with moderate to severe airflow obstruction (FEV 1 = 48.2±19% predicted). Elastic chest compression reduced FRC (148.7±25 vs 138.9±22% predicted, p = 0.002) but did not alter TLC or RV (p = 0.25 and 0.58, respectively). This led to an increase in inspiratory capacity with elastic