Imperial College London

Professor Konstantinos Dimopoulos

Faculty of MedicineNational Heart & Lung Institute

Professor of Practice (Adult Congenital Heart Disease)
 
 
 
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Contact

 

+44 (0)20 7352 8121 ext 82771k.dimopoulos02

 
 
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Location

 

Chelsea WingRoyal Brompton Campus

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Summary

 

Publications

Publication Type
Year
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475 results found

Constantine A, Dimopoulos K, Haworth SG, Muthurangu V, Moledina Set al., 2022, Twenty-year experience and outcomes in a National Pediatric Pulmonary Hypertension Service, American Journal of Respiratory and Critical Care Medicine, ISSN: 1073-449X

Journal article

Arvanitaki A, Gatzoulis MA, Opotowsky AR, Khairy P, Dimopoulos K, Diller G-P, Giannakoulas G, Brida M, Griselli M, Grünig E, Montanaro C, Alexander PD, Ameduri R, Mulder BJM, D'Alto Met al., 2022, Eisenmenger Syndrome: JACC State-of-the-Art Review., J Am Coll Cardiol, Vol: 79, Pages: 1183-1198

Although major breakthroughs in the field of pediatric cardiology, cardiac surgery, intervention, and overall care improved the outlook of congenital heart disease, Eisenmenger syndrome (ES) is still encountered and remains a complex clinical entity with multisystem involvement, including secondary erythrocytosis, increased thrombotic and bleeding diathesis, high arrhythmogenic risk, progressive heart failure, and premature death. Clearly, care for ES is best delivered in multidisciplinary expert centers. In this review, we discuss the considerable recent progress in understanding the complex pathophysiology of ES, means of prognostication, and improvement in clinical outcomes achieved with pulmonary arterial hypertension-targeted therapies. Additionally, we delineate areas of uncertainty in various aspects of care, discuss gaps in current evidence, and review current status in less privileged countries and propose initiatives to reduce disease burden. Finally, we propose the application of emerging technologies to enhance the delivery and quality of health care related to ES and beyond.

Journal article

Constantine A, Barradas-Pires A, Dimopoulos K, 2022, Cardiopulmonary exercise testing in congenital heart disease: Towards serial testing as part of long-term follow-up, European Journal of Preventive Cardiology, Vol: 29, Pages: 510-512, ISSN: 2047-4873

Journal article

Ghonim S, Gatzoulis M, Ernst S, Li W, Moon J, Smith G, Heng EL, Keegan J, Ho SY, McCarthy KP, Shore D, Uebing A, Kempny A, Alpendurada F, Dimopoulos K, Pennell DJ, Babu-Narayan Set al., 2022, Predicting survival in repaired tetralogy of Fallot- a lesion specific and personalised approach, JACC: Cardiovascular Imaging, Vol: 15, Pages: 257-268, ISSN: 1876-7591

Objective: We sought to identifyrepaired tetralogy of Fallot (rTOF) patients at high-risk of death and malignant ventricular arrhythmia (VA). Background: To date there is no robust risk stratification scheme to predict outcomes in adults with rTOF. Methods: Consecutive patients were prospectively recruited for late gadolinium enhancement cardiovascular magnetic resonance (LGE CMR) to define right and left ventricular (RV,LV) fibrosisin addition to proven risk markers. Results: The primary end-point was all-cause mortality. Of the 550 patients, (median age 32 years, 56% male), 27 died (mean follow-up 6.4 (±5.8); total 3512 years). Mortality was independently predicted by RVLGE extent, presence of LVLGE, RV ejection fraction (EF) ≤47%, LVEF ≤55%, B-type natriuretic peptide (BNP) ≥127ng/L, peak exercise oxygen uptake (V02) ≤17ml/kg/min, prior sustained atrial arrhythmia and age ≥50 years. The weighted scores for each of the above independent predictors differentiated a high-risk sub-group patients with a 4.4%, annual risk of mortality (AUC 0.87,P<0.001). The secondary end-point (VA), a composite of life-threatening sustained ventricular tachycardia/resuscitated ventricular fibrillation/sudden cardiac death occurred in 29. Weighted scores that included several predictors of mortality and RV outflow tractakinetic length ≥55mm and RVsystolic pressure ≥47mm Hg identified high-risk patients with a 3.7%, annual risk ofVA (AUC 0.79,P<0.001) RVLGE was heavily weighted in both risk scores due to its strong relative prognostic value. Conclusion: We present ascore integrating multiple appropriately weighted risk factors to identify the sub-group of rTOF patients that are at highannual risk of death who may benefit from targeted therapy.

Journal article

Constantine A, Dimopoulos K, Jenkins P, Tulloh RMR, Condliffe R, Jansen K, Chung NAY, Oliver J, Parry H, Fitzsimmons S, Walker N, Wort S, Papaioannou V, von Klemperer K, Clift Pet al., 2021, Use of pulmonary arterial hypertension therapies in Fontan patients: current practice across the United Kingdom, Journal of the American Heart Association, Vol: 11, Pages: 1-19, ISSN: 2047-9980

Background: The Fontan circulation is a successful operative strategy for abolishing cyanosis and chronic volume overload in congenital heart disease (CHD) patients with single ventricle physiology. ‘Fontan failure’ is a major cause of poor quality of life and mortality in these patients. We assessed the number and clinical characteristics of adult Fontan patients receiving pulmonary arterial hypertension (PAH) therapies across specialist centers in the UK.Methods and Results: We identified all adult patients with a Fontan-type circulation under active follow-up in 10 specialist CHD centers in England and Scotland between 2009 and 2019. Patients on PAH therapies were matched to untreated patients. A survey of experts was also performed. Of 1538 Fontan patients followed in specialist centers, only 76 (4.9%) received PAH therapies during follow-up. The vast majority (90.8%) were treated with a phosphodiesterase-5 inhibitor. In 33% of patients, PAH therapies were started after surgery or during hospital admission. In the matched cohort, treated patients were more likely to be significantly limited, have ascites, history of protein losing enteropathy, or receive loop diuretics (p<0.0001 for all), also reflecting survey responses indicating that failing Fontan is an important treatment target. After a median 12[11-15] months, functional class was more likely to improve in the treated group (p=0.01), with no other changes in clinical parameters or safety issues. Conclusions: PAH therapies are used in adult Fontan patients followed in specialist centers, targeting individuals with very advanced disease or complications. Follow-up suggests stabilization of the clinical status after 12 months of therapy.

Journal article

Constantine A, Condliffe R, Clift P, Tulloh R, Jansen K, Dimopoulos Ket al., 2021, Cardiac disease in Down Syndrome: literature review and international expert consensus in collaboration with Down Syndrome International (DSi), Journal of Congenital Cardiology, Vol: 5, Pages: 1-3, ISSN: 2056-7251

BackgroundCongenital heart disease is common in patients with Down syndrome, yet clinical recommendations relating to its diagnosis and management in this patient group are lacking.Main bodyWe discuss the ongoing collaboration between an international panel of cardiovascular experts and expert stakeholders from Down Syndrome International, an international disabled people's organisation with membership of organisations and individuals from 136 countries worldwide. The aim of this collaboration is to describe best clinical practice, focusing on 10 key areas relating to Down syndrome and cardiac disease, from prenatal diagnosis to the care of patients in areas of differing resource availability.ConclusionsThe planned expert consensus statement on cardiac disease in people with Down syndrome aims to foster communication between experts, direct future research and inform future practice guidelines for the diagnosis and management of cardiovascular disease in people with Down syndrome.

Journal article

Vidal MLB, Kempny A, Li W, Dimopoulos K, Wort J, Fischer A, Orwat S, Baumgartner H, Gatzoulis MA, Diller Get al., 2021, Utility of a Novel Ensemble Based Deep Learning Network for the Automatic Detection of Pulmonary Hypertension and Right Ventricular Dilatation: A Study Based on Data From 783 Individuals From Two Tertiary Centres, Publisher: LIPPINCOTT WILLIAMS & WILKINS, ISSN: 0009-7322

Conference paper

Vidal MLB, Kempny A, Li W, Dimopoulos K, Wort J, Gatzoulis MA, Diller Get al., 2021, Deep Learning Networks Trained on Routine Echocardiography Images Provide Expert Level Prognostication in Patients With Pulmonary Hypertension: A Study on 408 Patients From an Expert Centre, Publisher: LIPPINCOTT WILLIAMS & WILKINS, ISSN: 0009-7322

Conference paper

Assenza GE, Krieger E, Baumgartner H, Cupido B, Dimopoulos K, Louis C, Lubert AM, Stout KK, Valente AM, Zeppenfeld K, Opotowsky ARet al., 2021, AHA/ACC vs ESC Guidelines for Management of Adults With Congenital Heart Disease JACC Guideline Comparison, JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY, Vol: 78, Pages: 1904-1918, ISSN: 0735-1097

Journal article

Khan U, Shaw T, Kempny A, Gatzoulis MA, Dimopoulos K, Li Wet al., 2021, The clinical presentation and outcome of aortic coarctation associated with left ventricular inflow and outflow tract lesion in adult patients: Shone syndrome and beyond, INTERNATIONAL JOURNAL OF CARDIOLOGY, Vol: 343, ISSN: 0167-5273

Journal article

Gillesen MG, Fedchenko MF, Giang WGK, Dimopoulos KD, Eriksson PE, Dellborg MD, Mandalenakis Zet al., 2021, Chronic kidney disease in patients with congenital heart disease - a nationwide cohort study, Publisher: OXFORD UNIV PRESS, Pages: 1846-1846, ISSN: 0195-668X

Conference paper

Nashat H, Rocha L, Constantine A, Pires A, Patel R, Swan L, Alexander D, Gatzoulis MA, Johnson M, Dimopoulos K, Rafiq Iet al., 2021, Cardiovascular outcomes in women with the highest classification of maternal cardiovascular risk in pregnancy, Publisher: OXFORD UNIV PRESS, Pages: 2903-2903, ISSN: 0195-668X

Conference paper

Constantine A, Dimopoulos K, Muthurangu V, Haworth S, Moledina Set al., 2021, Twenty-year experience and outcomes in a national paediatric pulmonary hypertension service, Publisher: OXFORD UNIV PRESS, Pages: 1972-1972, ISSN: 0195-668X

Conference paper

Constantine A, Dimopoulos K, 2021, Integrating patient-reported outcome measures in congenital heart disease care, European Heart Journal - Quality of Care and Clinical Outcomes, Vol: 7, Pages: 325-326, ISSN: 2058-5225

Journal article

Krishnathasan K, Constantine A, Kempny A, Rafiq I, Gatzoulis MA, Heng EL, Montanaro C, Babu-Narayan SV, Li W, Dimopoulos Ket al., 2021, Predictors of adverse clinical outcome after hospitalisation for decompensated heart failure in ACHD patients, Publisher: OXFORD UNIV PRESS, Pages: 1854-1854, ISSN: 0195-668X

Conference paper

Constantine A, Dimopoulos K, Condliffe R, Clift P, Chaplin G, Muthurangu V, Haworth SG, Moledina Set al., 2021, Pulmonary arterial hypertension associated with congenital heart disease in children: clinical characterisation, outcomes and changes in demographics over time, Publisher: OXFORD UNIV PRESS, Pages: 1857-1857, ISSN: 0195-668X

Conference paper

D'Alto M, Chessa M, Santoro G, Giordano M, Gaio G, Romeo E, Argiento P, Wacker J, D'Aiello F, Sarubbi B, Russo MG, Golino P, Costantine A, Naeije R, Dimopoulos Ket al., 2021, The adding value of fluid challenge and balloon occlusion tests in patients with atrial septal defect, Publisher: OXFORD UNIV PRESS, Pages: 1840-1840, ISSN: 0195-668X

Conference paper

Wustmann K, Constantine A, Davies JE, Li W, Pennell D, Wort S, Kempny A, Price L, McCabe C, Mohiaddin R, Francis D, Gatzoulis M, Dimopoulos Ket al., 2021, Prognostic implications of pulmonary wave reflection and reservoir pressure in patients with pulmonary hypertension, International Journal of Cardiology: Congenital Heart Disease, Vol: 5, Pages: 1-8, ISSN: 2666-6685

BackgroundRight ventricular (RV) coupling to the pulmonary circulation influences the response of the RV to the increased afterload caused by pulmonary hypertension (PH), which ultimately determines prognosis. A methodology that accounts for pulsatile flow is required when assessing ventriculo-arterial coupling. We applied wave intensity analysis (WIA) methods to assess the compliance of the main pulmonary artery (PA) in patients with or without PH and compared this to PA distensibility, RV function and clinical outcomes.MethodsHigh-fidelity blood pressure and Doppler flow velocity tracings were obtained simultaneously during cardiac catheterisation for suspected PH. RV volumes, main PA distensibility and ventriculo-arterial coupling (Emax/Ea) were analysed using cardiovascular magnetic resonance.ResultsThe study included 17 ​PH patients and 6 controls. Wave speed, reservoir and excess pressure were higher in PH patients compared to controls (p ​< ​0.01 for all). Waveforms relating to RV ejection, microvascular wave reflection and late systolic proximal deceleration were higher in PH patients compared to controls (p ​< ​0.01 for all) and related to echocardiographic findings, including PA Doppler notching and shortened acceleration time. Wave speed, reservoir pressure and excess pressure were strongly correlated to main PA distensibility, RV function and Emax/Ea. A higher total pressure integral was associated with an increased risk of death (all-cause mortality).ConclusionThe reservoir-excess pressure model, in combination with conventional clinical imaging, provides valuable information on the pathophysiology of PH that standard haemodynamic parameters do not. Future studies should further investigate the prognostic implications of WIA in PH, and its potential role in clinical practice.

Journal article

Jansen K, Constantine A, Condliffe R, Tulloh R, Clift P, Moledina S, Wort SJ, Dimopoulos Ket al., 2021, Pulmonary arterial hypertension in adults with congenital heart disease: markers of disease severity, management of advanced heart failure and transplantation, Expert Review of Cardiovascular Therapy, Vol: 19, Pages: 837-855, ISSN: 1477-9072

Introduction:Pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD) is a progressive, life-limiting disease. Areas covered:In this paper, we review the classification and pathophysiology of PAH-CHD, including the mechanisms of disease progression and multisystem effects of disease. We evaluate current strategies of risk stratification and the use of biological markers of disease severity, and review principles of management of PAH-CHD. The indications, timing and the content of advanced heart failure assessment and transplant listing are discussed, along with a review of the types of transplant and other forms of available circulatory support in this group of patients. Finally, the integral role of advance care planning and palliative care is discussed. Expert opinion/commentary:All patients with PAH-CHD should be followed up in expert centers, where they can receive appropriate risk assessment, PAH therapy and supportive care. Referral for transplant assessment should be considered if there continue to be clinical high-risk features, persistent symptoms or acute heart failure decompensation despite appropriate PAH specific therapy. Expert management of PAH-CHD patients, therefore, requires vigilance for these features, along with a close relationship with local advanced heart failure services and a working knowledge of listing criteria, which may disadvantage congenital heart disease patients.

Journal article

Samaranayake CB, Warren C, Siewers K, Craig S, Price LC, Kempny A, Dimopoulos K, Gatzoulis M, Hopkinson NS, Wort SJ, Hull JH, McCabe Cet al., 2021, Impact of cyanosis on ventilatory responses during stair climb exercise in Eisenmenger syndrome and idiopathic pulmonary arterial hypertension, INTERNATIONAL JOURNAL OF CARDIOLOGY, Vol: 341, Pages: 84-87, ISSN: 0167-5273

Journal article

Dawes T, Dimopoulos K, Mccabe C, Bax S, Kempny A, Molyneaux P, George P, Kouranos V, Chua F, Renzoni E, Kokosi M, Wells AU, Wort SJ, Price LCet al., 2021, Survival effects of pulmonary vasodilators in group 3 pulmonary hypertension, Publisher: EUROPEAN RESPIRATORY SOC JOURNALS LTD, ISSN: 0903-1936

Conference paper

Samaranayake C, Luo Y, Siewers K, Warren C, Craig S, Harries C, Price L, Kempny A, Gatzoulis M, Dimopoulos K, Hopkinson N, Wort SJ, Hull J, Mccabe Cet al., 2021, Impact of cyanosis on ventilatory kinetics during stairclimbing in pulmonary arterial hypertension, Publisher: EUROPEAN RESPIRATORY SOC JOURNALS LTD, ISSN: 0903-1936

Conference paper

Ladouceur M, Segura de la Cal T, Gaye B, Valentin E, Ly R, Iserin L, Legendre A, Mousseaux E, Li W, Rafiq I, Kempny A, Barradas-Pires A, Babu-Narayan S, Gatzoulis MA, Dimopoulos Ket al., 2021, Effect of medical treatment on heart failure incidence in patients with a systemic right ventricle, HEART, Vol: 107, Pages: 1384-1389, ISSN: 1355-6037

Journal article

Klok FA, Toenges G, Mavromanoli AC, Barco S, Ageno W, Bouvaist H, Brodmann M, Cuccia C, Couturaud F, Dellas C, Dimopoulos K, Duerschmied D, Empen K, Faggiano P, Ferrari E, Galie N, Galvani M, Ghuysen A, Giannakoulas G, Huisman M, Jimenez D, Kozak M, Lang IM, Lankeit M, Meneveau N, Munzel T, Palazzini M, Petris AO, Piovaccari G, Salvi A, Schellong S, Schmidt K-H, Verschuren F, Schmidtmann I, Meyer G, Konstantinides Set al., 2021, Early switch to oral anticoagulation in patients with acute intermediate-risk pulmonary embolism (PEITHO-2) : a multinational, multicentre, single-arm, phase 4 trial, LANCET HAEMATOLOGY, Vol: 8, Pages: E627-E636, ISSN: 2352-3026

Journal article

Assenza GE, Dimopoulos K, Budts W, Donti A, Economy KE, Gargiulo GD, Gatzoulis M, Landzberg MJ, Valente AM, Roos-Hesselink Jet al., 2021, Management of acute cardiovascular complications in pregnancy, EUROPEAN HEART JOURNAL, Vol: 42, Pages: 4224-4240, ISSN: 0195-668X

Journal article

Barradas-Pires A, de la Cal TS, Constantine A, Dimopoulos Ket al., 2021, Systemic microangiopathy in Eisenmenger syndrome – The missing link?, International Journal of Cardiology, Vol: 337, Pages: 62-63, ISSN: 0167-5273

Journal article

D'Alto M, Constantine A, Chessa M, Santoro G, Gaio G, Giordano M, Romeo E, Argiento P, Wacker J, D'Aiello F, Sarubbi B, Russo MG, Naeije R, Golino P, Dimopoulos Ket al., 2021, Fluid challenge and balloon occlusion testing in patients with atrial septal defects, Heart, ISSN: 1355-6037

IntroductionCareful, stepwise assessment is required in all atrial septal defect (ASD) patients to exclude pulmonary vascular or left ventricular (LV) disease. Fluid challenge and balloon occlusion may unmask LV disease and post-capillary pulmonary hypertension, but their role in the evaluation of patients with ‘operable’ ASDs is not well established. MethodsWe conducted a prospective study in 3 Italian specialist centres between 2018 and 2020. Patients selected for percutaneous ASD closure underwent assessment at baseline and after fluid challenge, balloon occlusion and both. ResultsFifty patients (46[38.2,57.8] years, 72% female) were included. All had a shunt fraction >1.5, pulmonary vascular resistance (PVR) <5 WU and pulmonary arterial wedge pressure (PAWP) <15 mmHg. Individuals with a PVR ≥2 WU at baseline (higher PVR group) were older, more symptomatic, with a higher baseline systemic vascular resistance (SVR) than the lower PVR group (all p<0.0001). Individuals with a higher PVR experienced smaller increases in pulmonary blood flow following fluid challenge (0.3[-0.1,0.5] vs. 2.0[1.5,2.8] L/min, p<0.0001). Balloon occlusion led to a more marked fall in SVR (p<0.0001) and a larger increase in systemic blood flow (p=0.024) in the higher PVR group. No difference was observed in PAWP following fluid challenge and/or balloon occlusion between groups; 4(8%) patients reached a PAWP≥18 mmHg following the addition of fluid challenge to balloon occlusion testing.ConclusionsIn adults with ASD without overt LV disease, even small rises in PVR may have significant implications on cardiovascular haemodynamics. Fluid challenge may provide additional information to balloon occlusion in this setting.

Journal article

Barradas-Pires A, Constantine A, Dimopoulos K, 2021, Safety of physical sports and exercise in ACHD, International Journal of Cardiology Congenital Heart Disease, Vol: 4, Pages: 1-10, ISSN: 2666-6685

An active lifestyle, including regular physical activity, is an important component of long-term health and wellbeing, including in patients with adult congenital heart disease (ACHD). In the past, however, safety concerns regarding ACHD patients engaging in exercise have led to overly restrictive advice from specialists. Over the past few decades, it has become clear that the vast majority of patients with ACHD can exercise safely within their own limits; moreover, exercise capacity is highly variable between patients and should be measured objectively when possible.Cardiopulmonary exercise testing (CPET) is an essential part of ACHD surveillance and provides an integrative assessment of the cardiovascular, respiratory and muscular response to exercise. It allows an objective assessment of exercise tolerance, identification of the extent and mechanisms of exercise limitations, monitoring of disease progression and forms the basis of exercise prescription.Exercise prescription in ACHD patients ideally follows evaluation in an expert center, incorporating clinical assessment and exercise testing, to identify specific risk factors for adverse events during exercise and allow individualized recommendations of safe limits for exercise. In cases where specific risks exist, the intensity of exercise should be adjusted with respect to its dynamic and static components. The exercise prescription should then be reviewed regularly and amended as risk factors emerge or are successfully treated.This review highlights the benefits of physical activity in ACHD patients and discusses the main parameters that influence the risk of certain forms of exercise in some patients. CPET and exercise prescription for non-competitive sports are also discussed.

Journal article

Constantine A, Costola G, Bianchi P, Chessa M, Giamberti A, Kempny A, Rafiq I, Babu-Narayan S, Gatzoulis M, Hoschtitzky A, Shore D, Aw T-C, Ranucci M, Dimopoulos Ket al., 2021, Enhanced assessment of perioperative mortality risk in adults with congenital heart disease, Journal of the American College of Cardiology, Vol: 78, Pages: 234-242, ISSN: 0735-1097

BackgroundIn-hospital mortality is a rare, yet feared complication following cardiac surgery in adult congenital heart disease (ACHD). A risk score, developed and validated in ACHD, can be helpful to optimize risk assessment.ObjectivesTo assess the performance of EuroSCORE II components and procedure-related Adult Congenital Heart Surgery (ACHS) score, identify additional risk factors, and develop a novel risk score for predicting in-hospital mortality after ACHD surgery.MethodsWe assessed perioperative survival in patients >16 years undergoing congenital heart surgery in a large tertiary center between 2003 and 2019. A risk variable derived PErioperative ACHd (PEACH) score was calculated for each patient. Internal and external validation of the model was undertaken, including testing in a validation cohort of patients operated in a second European ACHD center. ResultsThe development cohort comprised 1782 procedures performed during the study period. Re-sternotomy was undertaken in 897(50.3%). There were 31(1.7%) in-hospital deaths. The PErioperative ACHd (PEACH) score showed excellent discrimination ability (AUC 0.88, 95%CI:0.83-0.94), and performed better than the ACHS score in our population (ACHS AUC 0.69, 95%CI:0.6-0.78, p=0.0003). A simple 3-tiered risk stratification was formed: PEACH score 0 (in-hospital mortality 0.2%), 1-2 (3.6%), ≥3 (17.2%). In a validation cohort of 975 procedures, the PEACH score retained its discriminative ability (AUC 0.75, 95%CI:0.72-0.77) and was well calibrated (Hosmer Lemishow 2 goodness-of-fit p=0.55). There was agreement in expected and observed perioperative mortality between cohorts.ConclusionsThe PEACH score is a simple, novel peri-operative risk score developed and validated specifically for ACHD patients undergoing cardiac surgery. Condensed abstract:The PEACH score is a simple, novel peri-operative risk score developed and validated specifically for ACHD patients undergoing cardiac surgery. In this study, we assesse

Journal article

Xu Z, Gatzoulis MA, Dimopoulos K, Li Q, Zhang C, Keller BB, Gu Het al., 2021, Better Outcomes in Pulmonary Arterial Hypertension After Repair of Congenital Heart Disease, Compared With Idiopathic Pulmonary Arterial Hypertension., CJC Open, Vol: 3, Pages: 872-879

BACKGROUND: Pulmonary vascular changes in postoperative pulmonary artery hypertension (PAH) are similar to those seen in idiopathic PAH. Data are sparse on direct comparative midterm outcomes for these 2 high-risk populations. METHODS: Patients with idiopathic or postoperative PAH referred to a large tertiary hospital between June 2005 and July 2019 were retrospectively evaluated. RESULTS: A total of 364 consecutive patients were studied, including 201 postoperative PAH patients and 163 patients with idiopathic PAH, with a median age of 18.7 (interquartile range 10.0, 31.5) and 7.3 (IQR: 2.9, 18.3) years, respectively. PAH-specific drugs were used in 77.7% of patients; 31.4% received combination therapy. Patients with idiopathic PAH had a shorter 6-mintue walk distance, lower percutaneous oxygen saturation, and higher B-type natriuretic peptide levels than those with postoperative PAH at diagnosis (all P < 0.001), During a median follow-up time of 3.4 (interquartile range: 2.1, 5.8) years, 56 patients (15.4%) died, and one underwent bilateral lung transplantation. Patients with postoperative PAH had better survival than those with idiopathic PAH, according to age (hazard ratio [HR] 0.128, 95% confidence interval [CI]: 0.07-0.22, P < 0.0001); Kaplan-Meier survival estimates at 5 years for idiopathic and postoperative PAH patients were 74.3% and 92.6%, respectively. Patients in New York Heart Association functional class III-IV had an over 4-fold increased risk of death (HR 4.85, 95% CI: 2.61-9.00, P < 0.0001). Patients < 18 years of age at idiopathic PAH diagnosis had a worse survival compared to adult patients (HR 6.90, 95% CI: 4.19-15.56, P = 0.040). CONCLUSIONS: Postoperative-PAH patients had better midterm survival compared to patients with idiopathic PAH. Mortality was significant in both PAH groups, reinforcing the need for early diagnosis and optimal individualized management to improve outcomes.

Journal article

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