Median Mean Pulmonary Artery Pressure Research Articles

Fontan Completion in Adult Patients with Functionally Univentricular Hearts.

Although there are considerable amounts of data on the outcomes of pediatric patients who have undergone Fontan repair, little is known about having Fontan completed in adulthood. The study presented the midterm results of our unit's experience with the Fontan completion procedure in adult patients with functionally univentricular hearts. Between 2014 and 2023, 16 adult patients underwent total cavopulmonary connection (TCPC) completion. Relevant information was retrospectively collected. Sixteen patients with a median age of 19 years (18-21 years) were included. Median arterial oxygen saturation was 76% (70-80.75%), and 62.5% of the patients were New York Heart Association (NYHA) Class III. The median mean pulmonary artery pressure was 14 mm Hg (9.5-14.5 mm Hg). Nine patients (56%) had heterotaxy syndrome, and the median time between the last operation and TCPC was 15.5 years (6.75-17.5 years). The median durations for bypass and cross-clamp were 160 minutes (130-201 minutes) and 120 minutes (84.5-137.5 minutes), consecutively. The postoperative course was straightforward in all. The median arterial oxygen saturation before discharge was 89.5% (85-90%), and 68.75% of the patients were NYHA Class II. Follow-up was complete for all patients with a median of 24 months. There was no early or late mortality or significant morbidity during the study period. We concluded that the intra-extracardiac Fontan technique was feasible for meticulously selected adults undergoing TCPC completion, as evidenced by an acceptable mortality rate and a satisfactory midterm outcome, including improvements in their NYHA functional class. However, the long-term consequences must be monitored.

Characteristics and outcomes of patients developing pulmonary hypertension associated with proteasome inhibitors.

Pulmonary arterial hypertension (PAH) has been described in patients treated with proteasome inhibitors (PIs). Our objective was to evaluate the association between PIs and PAH. Characteristics of incident PAH cases previously treated with carfilzomib or bortezomib were analysed from the French pulmonary hypertension registry and the VIGIAPATH programme from 2004 to 2023, concurrently with a pharmacovigilance disproportionality analysis using the World Health Organization (WHO) global database (VigiBase) and a meta-analysis of randomised controlled trials. 11 incident cases of PI-associated PAH were identified (six with carfilzomib and five with bortezomib) with a female:male ratio of 2.7:1, a median age of 61 years, and a median delay between PI first exposure and PAH of 6 months. Four patients died (two from right heart failure, one from respiratory distress and one from an unknown cause). At diagnosis, six were in New York Heart Association Functional Class III/IV with severe haemodynamic impairment (median mean pulmonary arterial pressure 39 mmHg, cardiac index 2.45 L·min-1·m-2 and pulmonary vascular resistance 7.2 WU). In the WHO pharmacovigilance database, 169 cases of PH associated with PI were reported since 2013 with significant signals of disproportionate reporting (SDR) for carfilzomib, regardless of the definition of cases or control group. However, SDR for bortezomib were inconsistent. The systematic review identified 17 clinical trials, and carfilzomib was associated with a significantly higher risk of dyspnoea, severe dyspnoea and PH compared with bortezomib. PIs may induce PAH in patients undergoing treatment, with carfilzomib emitting a stronger signal than bortezomib, and these patients should be monitored closely.

Characteristics and outcomes of gemcitabine-associated pulmonary hypertension.

Despite its known cardiac and lung toxicities, the chemotherapy drug gemcitabine has only rarely been associated with pulmonary hypertension (PH), and the underlying mechanism remains unclear. The objective of the present study was to assess the association between gemcitabine and PH. We identified incident cases of precapillary PH confirmed by right heart catheterisation in patients treated with gemcitabine from the French PH Registry between January 2007 and December 2022. The aetiology, clinical, functional, radiological and haemodynamic characteristics of PH were reviewed at baseline and during follow-up. A pharmacovigilance disproportionality analysis was conducted using the World Health Organization (WHO) pharmacovigilance database. We identified nine cases of pulmonary arterial hypertension, either induced (in eight patients) or exacerbated (in one patient) by gemcitabine. Patients exhibited severe precapillary PH, with a median mean pulmonary arterial pressure of 40 (range 26-47) mmHg, a cardiac index of 2.4 (1.6-3.9) L·min-1·m-2 and a pulmonary vascular resistance of 6.3 (3.1-12.6) Wood units. The median time from the initiation of gemcitabine to the onset of PH was 7 (4-50) months, with patients receiving a median of 16 (6-24) gemcitabine injections. Six patients showed clinical improvement upon discontinuation of gemcitabine. In the WHO pharmacovigilance database, we identified a significant signal with 109 cases reporting at least one adverse event related to PH with gemcitabine. Both clinical cases and pharmacovigilance data substantiate a significant association between gemcitabine use and the onset or worsening of precapillary PH. The observed improvement following the discontinuation of treatment underscores the importance of PH screening in gemcitabine-exposed patients experiencing unexplained dyspnoea.

Invasive haemodynamics predict outcomes in paediatric pulmonary artery hypertension.

Invasive haemodynamics are often performed for initiating and guiding pulmonary artery hypertension therapy. Little is known about the predictive value of invasive haemodynamic indices for long-term outcomes in children with pulmonary artery hypertension. We aimed to evaluate invasive haemodynamic data to help predict outcomes in paediatric pulmonary artery hypertension. Patients with pulmonary artery hypertension who underwent cardiac catheterisation (2006-2019) at a single centre were included. Invasive haemodynamic data from the first cardiac catheterisation and clinical outcomes were reviewed. The combined adverse outcome was defined as pericardial effusion (due to right ventricle failure), creation of a shunt for pulmonary artery hypertension (atrial septal defect or reverse Pott's shunt), lung transplant, or death. Among 46 patients with a median [interquartile range (IQR)] age of 13.2 [4.1-44.7] months, 76% had CHD. Median mean pulmonary artery pressure was 37 [28-52] mmHg and indexed pulmonary vascular resistance was 6.2 [3.6-10] Woods units × m2. Median pulmonary artery pulsatility index was 4.0 [3.0-4.7] and right ventricular stroke work index was 915 [715-1734] mmHg mL/m2. After a median follow-up of 2.4 years, nine patients had a combined adverse outcome (two had a pericardial effusion, one underwent atrial level shunt, one underwent reverse Pott's shunt, and six died). Patients with an adverse outcome had higher systolic and mean pulmonary artery pressures, higher diastolic and transpulmonary pressure gradients, higher indexed pulmonary vascular resistance, higher pulmonary artery elastance, and higher right ventricular stroke work index (p < 0.05 each). Invasive haemodynamics (especially mean pulmonary artery pressure and diastolic pressure gradient) obtained at first cardiac catheterisation in children with pulmonary artery hypertension predicts outcomes.

Lung Transplantation in Pulmonary Arterial Hypertension: The Portuguese Experience

In patients with pulmonary arterial hypertension (PAH), refractory to medical therapy, lung transplantation emerges as an option. This study describes the outcomes of 8 PAH patients who underwent lung transplantation. A retrospective, single-center study was conducted among patients with PAH who underwent lung transplantation in our center. Patients had a median age of 46 years, with female sex predominance (75%). Causes of HAP were pulmonary veno-occlusive disease (n = 5, 62.5%), idiopathic PAH (n = 2, 25%), and heritable PAH (n = 1, 12.5%). Pre-transplant hemodynamics revealed a median mean pulmonary artery pressure of 58.5 mm Hg (48-86). All patients received bilateral lung transplants with extracorporeal membrane oxygenation support, displaying immediate post-transplant hemodynamic improvement. Primary graft dysfunction grade 3 (PGD 3) was observed in 75% of patients. Five patients (62.5%) died, with a 72.9% survival at 12 months and 29.2% at 24 months post-transplantation. Our study reveals the complexity and challenges of lung transplants in patients with PAH. Despite notable immediate hemodynamic improvements, high rates of PGD 3 and the survival rate remain a concern. Further research to define optimal peri and post-transplant management to improve survival is required.

Initial experience and outcomes with a hybrid extracorporeal membrane oxygenation and cardiopulmonary bypass circuit for lung transplantation

BackgroundThe utilization of extracorporeal life support (ECLS) for intraoperative support during lung transplantation has increased over the past decade. Although veno-arterial extracorporeal membrane oxygenation (VA-ECMO) has recently emerged as the preferred modality over cardiopulmonary bypass (CPB), many centers continue to use both forms of ECLS during lung transplantation. Our novel hybrid VA-ECMO/CPB circuit allows for seamless transition from VA-ECMO to CPB at a significant cost savings compared to a standalone VA-ECMO circuit. This study describes our initial experience and outcomes in the first 100 bilateral lung transplantations using this novel hybrid VA-ECMO/CPB circuit. MethodsMedical records from September 2017 to May 2021 of the first 100 consecutive patients undergoing bilateral lung transplantation with intraoperative hybrid VA-ECMO support were examined retrospectively. We excluded patients with single lung transplants, retransplantations, preoperative ECLS bridging, and veno-venous (VV) ECMO and those supported with CPB only. Perioperative recipient, anesthetic, perfusion variables, and outcomes were assessed. ResultsOf the 100 patients supported with VA-ECMO, 19 were converted intraoperatively to CPB. Right ventricular dysfunction was seen in 37% of patients, and the median mean pulmonary artery pressure was 28 mm Hg. No oxygenator clotting was observed with a median heparin dose of 13,000 units in the VA-ECMO group. Primary graft dysfunction grade 3 at 72 hours was observed in 10.1% of all patients and observed 1-year mortality was 4%. ConclusionsThe use of a hybrid VA-ECMO/CPB circuit in our institution allows for rapid conversion to CPB with acceptable outcomes across a diverse recipient group at a significantly reduced cost compared to standalone VA-ECMO circuits.

CT-derived lung vessel morphology correlates with prognostic markers in precapillary pulmonary hypertension

While computed tomography pulmonary angiography (CTPA) is an integral part of the work-up in patients with suspected pulmonary hypertension (PH), there is no established CTPA-derived prognostic marker. We aimed to assess whether quantitative readouts of lung vessel morphology correlate with established prognostic indicators in PH. We applied a fully-automatic in-house developed algorithm for segmentation of arteries and veins to determine lung vessel morphology in patients with precapillary PH who underwent right heart catheterization and CTPA between May 2016 and May 2019. Primary endpoint of this retrospective study was the calculation of receiver operating characteristics for identifying low and high mortality risk according to the 3-strata risk assessment model presented in the current guidelines. We analyzed 73 patients, median age 65years (interquartile range (IQR): 54-76), female/male ratio 35/38, median mean pulmonary arterial pressure 37mm Hg (IQR: 30-46), and found significant correlations with important prognostic factors in pulmonary arterial hypertension. N-terminal pro-brain natriuretic peptide, cardiac index, mixed venous oxygen saturation, and 6-minute walking distance were correlated with the ratio of the number of arteries over veins with vessel diameters of 6-10mm (Spearman correlation coefficients ρ=0.64, p<0.001; ρ=-0.60, p<0.001; ρ=-0.47, p=0.005; ρ=-0.45, p=0.001, respectively). This ratio predicted a low- and high-risk score with an area under the curve of 0.73 (95% confidence interval (CI): 0.56-0.90) and 0.86 (95% CI: 0.74-0.97), respectively. The ratio of the number of arteries over veins with diameters between 6 and 10mm is significantly correlated with prognostic markers in pulmonary hypertension and predicts low and high mortality risk.

The clinical profile of pulmonary arterial hypertension in newly diagnosed patients with connective tissue disease from the point of view of the cardiologist

Aim: to evaluate demographic and disease characteristics in pulmonary arterial hypertension associated with connective tissue disease (PAH-CTD) patients.Materials and methods: the study enrolls 68 patients with PAH-CTD. These patients were diagnosed in department of pulmonary hypertension and heart disease of the E.I. Chazov National Medical Research Centre of cardiology. Clinical, functional and hemodynamic characteristics of PAH patients were examined. The diagnosis was confirmed according to Eurasian (2019) and Russian (2020) guidelines for the diagnosis and treatment of pulmonary hypertension.Results: Of 68 patients with CTD 35 (51,5%) had systemic sclerosis, 11 (16,2%) - mixed CTD, 22 (32,3%) - other CTD. The median time from PAH onset to first visit to health care worker was 4 months, the median time from first visit to PAH diagnosis was 7,5 months. The median age was 59,5 years, 94,1% patients were women. The median 6-minute walking distance (6MWD) was 345 (259,25-400,00) m, which was corresponding to WHO functional class III, the median Borg dyspnea index was 5 (3,0-6,0). 12 (17,7%) patients did not undergo 6MWD due to severity of their condition. According to right heart catheterization data the median mean pulmonary arterial pressure was 51,0 (35,5-62,0) mmHg, the median cardiac index was 2,1 (1,7-2,5) liter/min/m2, the median pulmonary vascular resistance was 11,48 (5,99-15,71) Wood units. According to several risk stratification calculators, the vast majority of patients were at high risk of 1-year mortality: due to risk status, PAH-specific therapy was initiated to 88,3% patients: 42,7% received double and 8,8% - triple PAH-specific therapy.Conclusions: In Russian clinical practice, PAH-CTD is characterized by late- onset diagnosis. Newly diagnosed patients have significant functional and hemodynamic impairment, high risk of 1-year mortality, which results in combined PAH-specific therapy initiation.

Patient and disease characteristics of pulmonary arterial hypertension patients for prostacyclin receptor agonist selexipag treatment initiation

Aim: to evaluate demographic and disease characteristics in pulmonary arterial hypertension (PAH) patients, for which selexipag is prescribed as PAH-specific treatment.Materials and methods: the study enrolls 73 patients with PAH, where there were 49 patients with idiopathic PAH and 24 patients with associated conditions. These patients were diagnosed in department of pulmonary hypertension and heart disease of the National Medical Research Centre of cardiology named after academician E.I. Chazov of Ministry of Health. Clinical, functional and hemodynamic characteristics of PAH patients were examined. The diagnosis was confirmed according to Eurasian (2019) and Russian (2020) guidelines for the diagnosis and treatment of pulmonary hypertension.Results: At selexipag initiation, median of patient`s age was 43 years, 86,3% were female. Etiological analysis revealed idiopathic PAH in 49 (67,1%) patients, 24 (32,9%) had associated conditions: 14 (19,2%) had connective tissue disease‒associated PAH, 6 (8,2%) had PAH after correction of the initial heart defect, 4 (5,5%) had HIV-associated PAH. The median 6-minute walking distance (6MWD) was 370 (300,0-443,75) m, which was corresponding to WHO functional class III, the median Borg dyspnea index was 5 (3,0-6,0). 7 (9,6%) patients did not undergo 6MWD due to severity of their condition. According to right heart catheterization data the median mean pulmonary arterial pressure was 58,5 (48,25-65,0) mmHg, the median right atrium pressure was 7,5 (5,0-10,0) mmHg, the median venous oxygen saturation 58,5% (56,0-66,0), the median cardiac index was 2,0 (1,6-2,5) liter/min/ m2, the median pulmonary vascular resistance was 15,0 (10,3-19,1) Wood units. At selexipag initiation, according to Eurasian (2019) and Russian (2020) guidelines 1 (1,3%) was at low risk, 21 (28,8%) were at intermediate risk and 51 (69,9%) were at high risk of 1-year mortality. Due to risk status, selexipag was initiated in double (50,7%) and triple (49,3%) PAH-specific therapy.Conclusions: At selexipag initiation, PAH-patients typically have WHO FC III and are at high risk, despite receiving PAH-specific treatment. Selexipag was prescribed as part of a combination regimen in most patients.

The angiogenic gene profile of pulmonary endarterectomy specimens: Initial study

ObjectivesThe underlying mechanisms for the development of chronic thromboembolic pulmonary hypertension and prognostic biomarkers are not clear yet. Thus, our aim is to assess and identify new biomarkers for the expression of 84 key genes linked to angiogenesis. MethodsPatients who had levels more than 1000 dynes·sec·cm−5 were included in the test group, and the other patients were included in the control group. Twelve specimens were taken from the patients. RT2 Profiler PCR Array (Qiagen) was used to quantify the expression of the 84 key genes. ResultsEight patients (6 male, 2 female, median age 54.4 ± 13.1 years) who underwent pulmonary endarterectomy were included. Pulmonary vascular resistance improved significantly from 811 ± 390 dyn/s/cm−5 to 413.3 ± 144.9 dyn/s/cm−5 (P < .005). A difference was also detected in median mean pulmonary arterial pressure, which decreased from 49.8 ± 9 mm Hg to 32.62 ± 2.50 mm Hg (P > .005) after surgery. Median length of hospital stay was 11.62 ± 2.97 days. The test group had a distinct pattern of impaired angiogenic and antiangiogenic genes. The expression levels of TGFA, TGFB1, THBS2, THBS1, TGFBR1, SERPINE1, SERPINF1, TGFB2, TIMP2, VEGFC, IFNA1, TNF, CXCL10, NOS3, IGF1, and MMP14 were downregulated in the specimens from the patients who had higher pulmonary vascular resistance values, whereas some genes, including PDGFA, showed upregulation that was statistically nonsignificant in the same group. ConclusionsThese results can lead to the development of new markers that could predict adverse outcomes of patients with CTEPH. Identification of new markers that are related to worse outcomes would enable screening patients for early diagnosis and treatment.

Hemodynamic markers of pulmonary vasculopathy for prediction of early right heart failure and mortality after heart transplantation

Elevated pulmonary vascular resistance (PVR) is broadly accepted as an imminent risk factor for mortality after heart transplantation (HTx). However, no current HTx recipient risk score includes PVR or other hemodynamic parameters. This study examined the utility of various hemodynamic parameters for risk stratification in a contemporary HTx population. Patients from seven German HTx centers undergoing HTx between 2011 and 2015 were included retrospectively. Established risk factors and complete hemodynamic datasets before HTx were analyzed. Outcome measures were overall all-cause mortality, 12-month mortality, and right heart failure (RHF) after HTx. The final analysis included 333 patients (28% female) with a median age of 54 (IQR 46-60) years. The median mean pulmonary artery pressure was 30 (IQR 23-38) mm Hg, transpulmonary gradient 8 (IQR 5-10) mm Hg, and PVR 2.1 (IQR 1.5-2.9) Wood units. Overall mortality was 35.7%, 12-month mortality was 23.7%, and the incidence of early RHF was 22.8%, which was significantly associated with overall mortality (log-rank HR 4.11, 95% CI 2.47-6.84; log-rank p < .0001). Pulmonary arterial elastance (Ea) was associated with overall mortality (HR 1.74, 95% CI 1.25-2.30; p < .001) independent of other non-hemodynamic risk factors. Ea values below a calculated cutoff represented a significantly reduced mortality risk (HR 0.38, 95% CI 0.19-0.76; p < .0001). PVR with the established cutoff of 3.0 WU was not significant. Ea was also significantly associated with 12-month mortality and RHF. Ea showed a strong impact on post-transplant mortality and RHF and should become part of the routine hemodynamic evaluation in HTx candidates.

20 years of experience with the Fontan procedure: characteristics and clinical outcomes of children in a tertiary referral hospital.

Without participating in a contractile chamber, the Fontan procedure seeks to create a separation of oxygenated and deoxygenated blood in patients with univentricular heart, reducing the risks of long-term hypoxemia and improving their survival. This study describes the clinical outcomes of children undergoing the Fontan procedure between 2000 and 2020 in a tertiary referral hospital care centre in southwestern Colombia. A retrospective observational descriptive study. The 81 patients who underwent the Fontan procedure were included. Categorical variables were presented with percentages and continuous variables with measures of central tendency according to the distribution of the data evaluated through the Shapiro-Wilk test. Sociodemographic, clinical, surgical variables, complications, and mortality were described. Between 2000 and 2020, 81 patients underwent the Fontan procedure: 43 (53.1%) males and a median age of 5.3years (interquartile range 4.3-6.6). The most common diagnosis was tricuspid atresia (49.4%). The median mean pulmonary arterial pressure was 12mmHg (interquartile range 10-15), the Nakata index 272mm2/m2 (interquartile range 204-327), and the McGoon index (interquartile range 1.86-2.3). Seventy-two (88.9%) patients underwent extracardiac Fontan and 44 (54.3%) patients underwent fenestration. The median hospitalisation days were 19days. The main complication was coagulopathy (19.8%), mortality in the first month between 2000 and 2010 was 8.6%, and after 2010 was 1.2%. The Fontan procedure is a palliative surgery for children with complex heart disease. According to anatomical and physiological variables, the proper choice of patients determines the short- and long-term results.

Routine Coronary Angiography is Still the Key Test for Patients Eligible for Lung Transplantation Also for Those With No Symptoms and With High Risk of Coronary Artery Disease

Coronary artery disease (CAD) has a considerable morbidity and mortality effect on the outcomes of a lung transplant. Currently, coronary angiography is performed as part of the pretransplant evaluation process. Unfortunately, there are no clear guidelines about performing cardiac angiography in lung transplant candidates. The aim of our work is to find a correlation between cardiovascular risk and coronary arterial status to optimize the selection of patients for coronary angiography prior transplantation. We retrospectively analyzed 48 patients in whom coronary angiography and cardiac catheterization was performed during assessment for bilateral lung transplantation at the Medical University of Gdańsk from 2018 to 2021. The coronary artery disease status was classified into 2 categories: without any stenosis and with stenosis. For each patient, the 10-year cardiovascular risk was estimated by using a Systematic COronary Risk Evaluation calculator modified for the Polish population. Coronary stenosis was detected in 15 patients during angiography (31%). The group with coronary stenosis had a median SCORE risk of 8%, which is considered as high risk, and in patients without stenosis it was 5%, which is also considered a high risk. Median mean pulmonary artery pressure in patients with stenosis was the same as that in patients without stenosis (23 mm Hg). CAD among lung transplant candidates cannot be predicted by risk factors, so coronary angiography is very important as a part of the evaluation process. Because pulmonary hypertension has a big impact on surveillance after transplantation, performing heart catheterization during the qualification process is crucial.

Results From the United States Chronic Thromboembolic Pulmonary Hypertension Registry: Enrollment Characteristics and 1-Year Follow-up

BackgroundThe United States Chronic Thromboembolic Pulmonary Hypertension Registry (US-CTEPH-R) was designed to characterize the demographic characteristics, evaluation, clinical course, and outcomes of surgical and nonsurgical therapies for patients with chronic thromboembolic pulmonary hypertension.Research QuestionWhat are the differences in baseline characteristics and 1-year outcomes between operated and nonoperated subjects?Study Design and MethodsThis study describes a multicenter, prospective, longitudinal, observational registry of patients newly diagnosed (< 6 months) with CTEPH. Inclusion criteria required a mean pulmonary artery pressure ≥ 25 mm Hg documented by right heart catheterization and radiologic confirmation of CTEPH. Between 2015 and 2018, a total of 750 patients were enrolled and followed up biannually until 2019.ResultsMost patients with CTEPH (87.9%) reported a history of acute pulmonary embolism. CTEPH diagnosis delays were frequent (median, 10 months), and most patients reported World Health Organization functional class 3 status at enrollment with a median mean pulmonary artery pressure of 44 mm Hg. The registry cohort was subdivided into Operable patients undergoing pulmonary thromboendarterectomy (PTE) surgery (n = 566), Operable patients who did not undergo surgery (n = 88), and those who were Inoperable (n = 96). Inoperable patients were older than Operated patients; less likely to be obese; have a DVT history, non-type O blood group, or thrombophilia; and more likely to have COPD or a history of cancer. PTE resulted in a median pulmonary vascular resistance decline from 6.9 to 2.6 Wood units (P < .001) with a 3.9% in-hospital mortality. At 1-year follow-up, Operated patients were less likely treated with oxygen, diuretics, or pulmonary hypertension-targeted therapy compared with Inoperable patients. A larger percentage of Operated patients were World Health Organization functional class 1 or 2 at 1 year (82.9%) compared with the Inoperable (48.2%) and Operable/No Surgery (56%) groups (P < .001).InterpretationDifferences exist in the clinical characteristics between patients who exhibited operable CTEPH and those who were inoperable, with the most favorable 1-year outcomes in those who underwent PTE surgery.Clinical Trial RegistrationClinicalTrials.gov; No.: NCT02429284; URL: www.clinicaltrials.gov.

Oxy-right Ventricular Assist Device for Bridging of Right Heart Failure to Lung Transplantation.

Right heart failure develops in lung transplantation candidates on extracorporeal membrane oxygenation (ECMO) support and increases mortality. The safety and feasibility of the oxy-right ventricular assist device (oxyRVAD) as a bridge to lung transplantation in severe right heart failure caused by terminal lung disease have not been evaluated. We retrospectively reviewed 14 patients who used oxyRVAD for bridging of right heart failure to lung transplantation. The major cause of lung transplantation was acute exacerbation of interstitial lung disease (78.6%), and the median venovenous ECMO duration was 7 d. Before oxyRVAD, median mean pulmonary artery pressure was 60.5 mm Hg (interquartile range [IQR], 54-68), and the median peak tricuspid regurgitation velocity was 3.9 m/s (IQR, 3.7-4.1). After oxyRVAD conversion, median mean pulmonary artery pressure was 60.5 mm Hg (IQR, 57.3-65), and the median peak tricuspid regurgitation velocity was 2.9 (IQR, 2.6-3.2). All patients were hemodynamically stable (median arterial blood pressure, 83 mm Hg; median heart rate, 79 bpm). Three patients developed pulmonary congestion (21.4%), and all patients stabilized within 24 h. Active rehabilitation during ECMO was possible in all patients, and the median duration of awake state during ECMO was 14 d. A total of 10 patients were bridged successfully to lung transplantation, and hospital survival rates were 90%. OxyRVAD stabilized hemodynamic parameters without fatal complications, permitted the discontinuation of sedation, and allowed active rehabilitation in patients with severe right heart failure. OxyRVAD may be a feasible option for bridging of right heart failure to lung transplantation.

Association between serum soluble ST2 level and right ventricle systolic function on pulmonary hypertension due to atrial septal defect

Pulmonary hypertension (PH) due to atrial septal defect (ASD) may cause a decline in right ventricle (RV) function. Soluble ST2 isa prognostic biomarker for left ventricle dysfunction. However, its role in RV function has not been investigated. This study aimed to investigate the association between serumsoluble ST2 with RV systolic function in patients withASD–associatedPH. This was a cross sectional study. Subjects were patients participated in the COHARD-PH registry performed in Dr. Sardjito General Hospital, Yogyakarta Indonesia. The patients with ASD and PH confirmed by right heart catheterization (RHC) were enrolled in this study. The soluble ST2 level was measured in the serum collected from pulmonary artery during RHC. Right ventricle systolic function was determined by transthoracic echocardiography using peak systolic velocity of tricuspid annulus (S’) parameter. This study was performed in 32 adults with uncorrected ASD. They predominantly females [n=29 (90.6%)] with median age of 31(22.5-44.0) years old. Mean ASD diameter was 2.69±0.53 cm. Median mean pulmonary artery pressure (mPAP) 45.0 (36.25-70.0) mmHg. Median soluble ST2 level was 23.28 ng/mL. There were no significant correlations between soluble ST2 level with S’value (r=0.071; p=0.35), with mPAP (r=0.043; p=0.815), with pulmonary vasculer resistance (PVR) (r=0.025; p=0.893) and with right ventricle (RV) diameter (r=0.200; p=0.273). Soluble ST2 level was found higher in subject with RV dysfunctionbut not statistically significant. In conclusion, serum soluble ST2 level did not associate withRV systolic function, measured by S’, in adult ASD-associated PH.

Safety and Outcomes of Transcatheter Closure of Patent Ductus Arteriosus in Children With Pulmonary Artery Hypertension.

To investigate whether transcatheter device closure of patent ductus arteriosus (PDA) is safe in children with pulmonary artery hypertension, we retrospectively analyzed our experience with 33 patients who underwent the procedure from January 2000 through August 2015. Pulmonary artery hypertension was defined as a pulmonary vascular resistance index (PVRI) >3 WU · m2. All 33 children (median age, 14.5 mo; median weight, 8.1 kg) underwent successful closure device implantation and were followed up for a median of 17.2 months (interquartile range [IQR], 1.0-63.4 mo). During catheterization, the median PVRI was 4.1 WU · m2 (IQR, 3.6-5.3 WU · m2), and the median mean pulmonary artery pressure was 38.0 mmHg (IQR, 25.5-46.0 mmHg). Premature birth was associated with pulmonary vasodilator therapy at time of PDA closure ( P=0.001) but not with baseline PVRI (P=0.986). Three patients (9.1%) had device-related complications (one immediate embolization and 2 malpositions). Two of these complications involved embolization coils. Baseline pulmonary vasodilator therapy before closure was significantly associated with intensive care unit admission after closure (10/12 [83.3%] with baseline therapy vs 3/21 [14.3%] without; P <0.001). Of 11 patients receiving pulmonary vasodilators before closure and having a device in place long-term, 8 (72.7%) were weaned after closure (median, 24.0 mo [IQR, 11.0-25.0 mo]). We conclude that transcatheter PDA closure can be performed safely in many children with pulmonary artery hypertension and improve symptoms, particularly in patients born prematurely. Risk factors for adverse outcomes are multifactorial, including coil use and disease severity. Multicenter studies in larger patient populations are warranted.

Combined lung transplantation and percutaneous septal defect closure for end-stage atrial septal defect associated pulmonary arterial hypertension

Pulmonary arterial hypertension (PAH) is a rare but severe complication of unrepaired atrial septal defect (ASD). In patients with ASD, PAH and right ventricle failure, heart-lung transplantation (HLT) is currently viewed as the best ultimate procedure but is limited by the low number of grafts. We aim to report our preliminary experience of double lung transplantation (DLT) followed by percutaneous ostium secundum ASD closure in patients with end-stage ASD associated PAH. We report 6 patients with end-stage ASD associated PAH treated with DLT followed by percutaneous ASD closure. Median ages at ASD diagnosis and PAH diagnosis were respectively 22 y.o (min 0, max 37) and 16 (min 2 max 40). Median mean pulmonary artery pressure (mPAP) and pulmonary vascular resistance before DLT were 78 mmHg (min 49, max108) and 14.0WU.m 2 (min 12.5, max 17.4) respectively. Median age at DLT was 29 y.o (min 20, max 44). Severe primary graft dysfunction occurred in 3 (50%) patients and was successfully managed medically. Post operative extracorporeal membrane oxygenation was required in 5 (83%) patients. Median ventilation time was 12 days (min 2, max 20). Median delay between DLT and percutaneous ASD closure occlusion was 4.4 months (min 3.9 max 18.7). Percutaneous closure was achieved using Amplatzer septal occluder, size running from n°16 to 40. mPAP was within normal range in all patient before ASD closure. All patient had improvement of their respiratory functional tests. All patients had improvement of right ventricular function ( Fig. 1 ) and none of them developed any chronic lung allograft dysfunction. Median follow up after lung transplant was 397 months (min: 265; max: 562). One patient died of pulmonary infection complication at 14.5 months after lung transplant. DLT followed by percutaneous ASD closure is an efficient therapeutic approach in patients with end-stage ASD associated PAH that may offer an alternative option to HLT.

4d cardiac magnetic resonance flow in patients with pulmonary arterial hypertension associated with congenital heart disease

Right heart catheterization is an invasive exam that is currently the gold standard to assess pulmonary hemodynamics for diagnosis and follow-up of pulmonary arterial hypertension (PAH) associated with congenital heart diseases (CHD). Cardiac magnetic resonance 4D flow (4D CMR flow) emerges as a promising non-invasive imaging. We assess the accuracy of 4D CMR flow to measure pulmonary cardiac output (Qp). We prospectively included 28 patients (median age was 42 years old [35–52] with PAH and CHD (pre-tricuspid shunt n = 23, 82.1%, median mean pulmonary artery pressure 46 mmHg [40–58]; median pulmonary vascular resistance 15.0 WU.m 2 [7.5–25.6]). Qp was measured invasively using Fick principle (direct oxygen consumption measure) during a right heart catheterization (QpF) and compared to Qp measured by 2D (Qp2D) and 4D flow CMR (Qp4D) on the same day. 4D CMR flow analysis was feasible in all patients. Qp4D and QpF were strongly correlated (rho = 0.87, P < 0.0001; r 2 = 0.68, P < 0.0001). Using Bland Altman analysis, mean difference was 0.0 ± 1.0 L/min. Mean difference within two observers (interobserver variability) was 0.3 ± 0.4 L/min ( Fig. 1 ). Mean difference within one observer (intra-observer variability) was 0.1 ± 0.3 L/min. Qp2D and QpF were moderately correlated (rho = 0.54, P = 0.008; r 2 = 0.41, P = 0.0007). Using Bland Altman analysis, mean difference was 0.6 ± 2.3 L/min ( Fig. 1 ). Qp measured by 4D CMR flow is well correlated to QpF. Further studies are needed to explore other potential interests of using 4D CMR flow to assess PAH, including the derivation of additional hemodynamic parameters such as pulmonary artery compliance, wall shear stress and pulse wave velocities, which could provide further insights into pulmonary artery remodelling and interactions between pulmonary arterial stiffening and right ventricular dysfunction.

P1739 Diameter of the pulmonary artery in relation to the ascending aorta: a promising parameter for the diagnosis of pulmonary hypertension

Abstract Background Pulmonary hypertension (PH) is a disease with significant morbidity and mortality. The first imaging modality in the diagnostic algorithm is transthoracic echocardiography (TTE). Despite significant technological advances in recent years, there are still patients with PH who are missed by TTE. The pulmonary artery to ascending aorta ratio (PA/Ao-R) has been evaluated in cardiac magnetic resonance (CMR) and computed tomography (CT) studies. The ratio was able to detect patients with PH, it correlated with hemodynamics, and it was predictive for mortality. We sought to evaluate the diagnostic accuracy of PA/Ao-R measured by TTE in the detection of PH. Methods We evaluated consecutive patients who received quasi-simultaneous transthoracic echocardiography and right heart catheterization (RHC) for evaluation of pulmonary hypertension between July 2015 and July 2016. Results A total of 84 patients fulfilled the inclusion criteria, 47 were female, mean age was 65 years. Median PA/Ao-R was 0.8 (Q1;Q3 0.69;0.94). Seventy patients had PH (median mean pulmonary artery pressure (mPAP) was 35.5 mmHg, Q1;Q3 27;47). The area under the curve for the diagnosis of PH via PA/Ao-R was 0.853. A cut-off of 1 was chosen for further analysis. Sensitivity for the diagnosis of PH was 100%, specificity was 23%. PA/Ao-R correlated with mPAP (r = 0.65, p &amp;lt; 0.001), and pulmonary vascular resistance (r = 0.68, p &amp;lt; 0.001). In the subgroup of patients with PH, those patients with a PA/Ao-R of ≥1 had significantly (p &amp;lt; 0.001) higher pulmonary pressures than those below, with a mean mPAP of 53 mmHg as compared to 38.5 mmHg. Conclusion PA/Ao-R correlates with mPAP. A ratio of ≥1 should prompt suspicion for the presence of PH. PA/Ao-R should be reported in every patient who is evaluated for PH.