Predictors of survival in patients with precapillary pulmonary hypertension

2021 ◽  
Vol 22 (Supplement_1) ◽  
Author(s):  
M Fontes Oliveira ◽  
MI Oliveira ◽  
R Costa ◽  
A Dias Frias ◽  
I Silveira ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Pericardial Effusion ◽  
Tricuspid Regurgitation ◽  
Independent Predictor ◽  
Functional Class ◽  
Walking Distance ◽  
Severe Tricuspid Regurgitation ◽  
Acceleration Time ◽  
Nyha Functional Class ◽  
Eccentricity Index

Abstract Funding Acknowledgements Type of funding sources: None. Introduction Although the perceived prognosis of patients with precapillary pulmonary hypertension (PH) is poor, the natural history of this condition is very heterogeneous. In this study we sought to identify predictors of poor outcomes which could help refine prognosis. Methods We studied consecutive patients referred to our centre from 12/2016 to 11/2018 with confirmed precapillary PH. A range of clinical, laboratory, echocardiographic and right heart catheterization (RHC) data variables were collected to assess predictors of survival. Outcome was defined as mortality from any cause. Results Of the 80 included patients, 51 (64%) were female and mean age was 60.5 ± 16.0 years. The majority of patients (45%) had pulmonary arterial hypertension (group 1) and 41% were chronic thromboembolic pulmonary hypertensive disease patients (group 4). During a median follow-up of 18.7 [IQR 12.3 – 26.7] months, 10 patients (12.5%) died. New York Heart Association (NYHA) functional class (HR 19.4 [95% CI 2.56 - 147.5], p = 0.004) was the strongest predictor of mortality, whereas higher haemoglobin (HR 0.70 [0.49 - 0.99], p= 0.047) and 6-minute walking distance (6MWD) expressed as percentage of predicted (HR 0.96 [0.93 - 0.99], p = 0.004) were associated with better survival overall. Echocardiographic parameters such as eccentricity index (HR 3.35 (95% CI 1.11 - 10.0), p = 0.031), short pulmonary acceleration time (HR 0.98 [95% CI 0.96 - 0.99], p = 0.008), the presence of moderate to severe tricuspid regurgitation (HR 6.46 [95% CI 1.67 - 25.0], p = 0.007) and pericardial effusion (HR 3.86 [95% CI 1.12 - 13.4], p = 0.033) were also associated with death. Traditional right ventricular function parameters such as fractional area change, tricuspid annular plane systolic excursion (TAPSE) and S velocity of the lateral annular tricuspid annulus did not predict mortality in these patients. Invasive pressures and pulmonary vascular resistance measured by RHC were also not associated with mortality. In multivariable analysis, NYHA functional class was the only independent predictor of mortality in patients with precapillary PH (HR 14.5 [95% CI 2.3 - 146.8], p = 0.006). Conclusion Eccentricity index, short pulmonary acceleration time, moderate to severe tricuspid regurgitation and pericardial effusion were associated with poor survival. Functional class was the strongest independent predictor of mortality in precapillary PH patients. These parameters may help stratify the risk of death in this heterogenous population.

Tricuspid valvuloplasty for isolated tricuspid regurgitation in children

2020 ◽  
Vol 30 (8) ◽  
pp. 1076-1080
Author(s):  
Guanxi Wang ◽  
Kai Ma ◽  
Kunjing Pang ◽  
Sen Zhang ◽  
Lei Qi ◽  
...  
Keyword(s):  
Drug Therapy ◽  
Tricuspid Regurgitation ◽  
Excellent Result ◽  
Functional Class ◽  
Tricuspid Valve Repair ◽  
Valve Repair ◽  
Severe Tricuspid Regurgitation ◽  
Nyha Functional Class ◽  
Hospital Patients

AbstractBackground:Isolated congenital tricuspid regurgitation other than Ebstein’s anomaly was rare especially for children. The objective of this study was to investigate the clinical characteristics and to assess the results of tricuspid valvuloplasty for children with isolated tricuspid regurgitation.Methods:From January 2010 to June 2019, 10 consecutive patients with isolated tricuspid regurgitation who were unresponsive to drug therapy underwent tricuspid valvuloplasty in our hospital. Patients’ clinical data were analysed retrospectively.Results:Mean age at operation was 48.5 ± 31.0 (range: 9–106) months and mean weight at operation was 16.1 ± 6.9 (range: 8.6–33.0) kg. All patients presented severe isolated tricuspid regurgitation. According to pathological lesions, the main causes accounted for chordae tendinea rupture (3/10), leaflet cleft (2/10), mal-connected chordal tendinea to leaflets (2/10), elongated chordae (1/10) and chordae absent (1/10), and severe anterior leaflet dysplasia (1/10). Individualised tricuspid valvuloplasty was adapted to all of them successfully. Post-operative echocardiography showed no tricuspid regurgitation in two patients and mild regurgitation in eight patients. The cardiothoracic ratios on their chest roentgenograms decreased from 0.59 ± 0.05 to 0.54 ± 0.05. At the latest follow-up (50.4 ± 47.2 months), echocardiography showed that mild to moderate tricuspid regurgitation in seven patients, moderate tricuspid regurgitation in three patients, and no patient with severe tricuspid regurgitation. All patients were in NYHA functional class I.Conclusions:For patients with isolated tricuspid regurgitation who were not well responsive to drug therapy, individualised tricuspid valve repair can achieve an excellent result.

scholarly journals Standardized exercise training is feasible, safe, and effective in pulmonary arterial and chronic thromboembolic pulmonary hypertension: results from a large European multicentre randomized controlled trial

2020 ◽  
Author(s):  
Ekkehard Grünig ◽  
Alison MacKenzie ◽  
Andrew J Peacock ◽  
Christina A Eichstaedt ◽  
Nicola Benjamin ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Exercise Training ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Functional Class ◽  
European Countries ◽  
Walking Distance ◽  
Control Group ◽  
Randomized Controlled ◽  
Thromboembolic Pulmonary Hypertension ◽  
Pulmonary Arterial

Abstract Aims This prospective, randomized, controlled, multicentre study aimed to evaluate efficacy and safety of exercise training in patients with pulmonary arterial (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH). Methods and results For the first time a specialized PAH/CTEPH rehabilitation programme was implemented in 11 centres across 10 European countries. Out of 129 enrolled patients, 116 patients (58 vs. 58 randomized into a training or usual care control group) on disease-targeted medication completed the study [85 female; mean age 53.6 ± 12.5 years; mean pulmonary arterial pressure 46.6 ± 15.1 mmHg; World Health Organization (WHO) functional class II 53%, III 46%; PAH n = 98; CTEPH n = 18]. Patients of the training group performed a standardized in-hospital rehabilitation with mean duration of 25 days [95% confidence interval (CI) 17–33 days], which was continued at home. The primary endpoint, change of 6-min walking distance, significantly improved by 34.1 ± 8.3 m in the training compared with the control group (95% CI, 18–51 m; P < 0.0001). Exercise training was feasible, safe, and well-tolerated. Secondary endpoints showed improvements in quality of life (short-form health survey 36 mental health 7.3 ± 2.5, P = 0.004), WHO-functional class (training vs. control: improvement 9:1, worsening 4:3; χ2  P = 0.027) and peak oxygen consumption (0.9 ± 0.5 mL/min/kg, P = 0.048) compared with the control group. Conclusion This is the first multicentre and so far the largest randomized, controlled study on feasibility, safety, and efficacy of exercise training as add-on to medical therapy in PAH and CTEPH. Within this study, a standardized specialized training programme with in-hospital start was successfully established in 10 European countries.

Ebstein anomaly combined with unique pulmonary venous abnormality in a 9-month-old child

2020 ◽  
Vol 30 (7) ◽  
pp. 1026-1028
Author(s):  
Emily N. Sanders ◽  
Dala Zakaria
Keyword(s):  
Pulmonary Hypertension ◽  
Tricuspid Regurgitation ◽  
Wide Spectrum ◽  
Delayed Diagnosis ◽  
Medical Attention ◽  
Ebstein Anomaly ◽  
Severe Tricuspid Regurgitation ◽  
Age Of Diagnosis

AbstractEbstein anomaly is a rare CHD known for its wide spectrum of presentation with the age of diagnosis dependent on the malformation’s severity. Here, the authors describe a case of delayed diagnosis of Ebstein anomaly, secondary to lack of medical attention, which resulted in severe tricuspid regurgitation and pulmonary hypertension. Furthermore, the case was complicated by a unique pulmonary venous abnormality.

scholarly journals Balloon pulmonary angioplasty for inoperable patients with chronic thromboembolic disease

2017 ◽  
Vol 8 (1) ◽  
pp. 204589321775312 ◽  
Author(s):  
Christoph B. Wiedenroth ◽  
Karen M. Olsson ◽  
Stefan Guth ◽  
Andreas Breithecker ◽  
Moritz Haas ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Arterial Compliance ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Functional Class ◽  
Thromboembolic Disease ◽  
World Health ◽  
Vascular Lesions ◽  
Walking Distance ◽  
Balloon Pulmonary Angioplasty ◽  
Chronic Thromboembolic Disease

Symptomatic patients with residual pulmonary perfusion defects or vascular lesions but no pulmonary hypertension at rest are diagnosed with chronic thromboembolic disease (CTED). Balloon pulmonary angioplasty (BPA) is an emerging treatment for patients with inoperable chronic thromboembolic pulmonary hypertension (CTEPH), but data regarding the safety and efficacy of BPA in patients with CTED are lacking. We report a prospective series of ten consecutive patients with CTED who underwent 35 BPA interventions (median of four per patient) at two German institutions. All patients underwent a comprehensive diagnostic workup at baseline and 24 weeks after their last intervention. BPA was safe, with one pulmonary vascular injury and subsequent self-limiting pulmonary bleeding as the only complication (2.9% of the interventions, 10% of the patients). After the procedures, World Health Organization functional class, 6-min walking distance, pulmonary vascular resistance, and pulmonary arterial compliance improved, and NT-proBNP concentrations declined in 9/10 patients. BPA may be a new treatment option for carefully selected patients with CTED. A larger, prospective, international registry is required to confirm these results.

scholarly journals Outcomes of Tricuspid Regurgitation after Percutaneous Mitral Commissurotomy

2021 ◽  
Vol 30 (2) ◽  
pp. 9-19
Author(s):  
Bryan Rene F. Toledano ◽  
Maria Johanna Jaluage-Villanueva ◽  
Sharon Marisse Lacson
Keyword(s):  
Tricuspid Regurgitation ◽  
Numerical Data ◽  
Functional Class ◽  
Test Results ◽  
Chi Square ◽  
Severe Tricuspid Regurgitation ◽  
Chi Square Test ◽  
Echocardiographic Parameters ◽  
Mitral Commissurotomy

PURPOSE The gap in evidence in the management of multivalvular lesions can be addressed by providing more data on clinical and echocardiographic outcomes after Percutaneous Mitral commissurotomy (PMC). METHODS Participants were Filipinos aged >/= 19 years old, admitted due to severe mitral stenosis with moderate to severe tricuspid regurgitation (TR). The outcome of PMC was divided into 2 groups: Significant TR which included the progression of moderate to severe TR or persistence of severe TR and Insignificant TR group which included those with mild TR, regression to moderate to mild TR, severe to moderate, or persistence of moderate TR. These groups were compared from baseline, 24th hour, 1st month, and 6th month using the same echocardiographic parameters. The numerical data between significant and nonsignificant tricuspid regurgitation were compared using non-parametric Mann Whitney U test and categorical data using the Chi-Square test. RESULTS A total of 38 participants were analyzed. On the 24th-hour post- PTMC, the Significant TR group had significantly higher RAVI (42.3 vs 26.1, p=.004), RVD mid (3.81 vs 2.92, p=.001), SPAP (60.5 vs 38.5, p=.003), and RVOT (2.8 vs 2.2, p=.001) and lower MV planimetry (1.25 vs 1.58, p=.009); On the 1st-month RVD mid (3.4 vs 2.8, p=.02) and TV annulus (3.35 vs 2.76, p=0.10) were significantly higher in the Significant TR group; On the 6th month RAVI (59 vs 24.7, p=.001), RVD mid (4 vs 2.73, p=.006), and TV annulus (4.5 vs 2.67 p=.001) were significantly higher in the Significant TR group when compared to Insignificant TR group. CONCLUSION PMC improved baseline parameters of SPAP, MV planimetry, MV gradient, and functional class on short-term follow-up on both groups of TR. Majority of outcomes after the procedure had insignificant TR. However, those with significant TR had higher RVD mid and TV annulus from the 24th hour to 6 months when compared to the insignificant TR group.

scholarly journals Perioperative Management of Patients with Ventricular Septal Defect, Severe Tricuspid Regurgitation and Gerbode Defects

2021 ◽  
Vol 1 (1) ◽  
pp. 27
Author(s):  
Purwoko Purwoko ◽  
Ardhana Surya Aji
Keyword(s):  
Pulmonary Hypertension ◽  
Ventricular Septal Defect ◽  
Tricuspid Valve ◽  
Tricuspid Regurgitation ◽  
Perioperative Management ◽  
Septal Defect ◽  
The Body ◽  
Severe Pulmonary Hypertension ◽  
Severe Tricuspid Regurgitation ◽  
Gerbode Defect

<p>Ventricular Septal Defect (VSD) is a congenital heart disease that causes the connection between left and right ventricles called a Gerbode defect. Manifestation of a Gerbode defect is damage to the opening tricuspid valve caused regurgitation of the tricuspid valve. Delay in diagnosis and intervention will affect pre-operative nutritional status and malnutrition.</p><p>We reported a boy aged 2 months, weighing 3100 grams with biliary atresia followed by VSD, severe TR, and Gerbode defect who will undergo the Kasai procedure. Preoperative physical examination showed GCS E4V5M6, SpO2 100%. The skin gets icteric all over the body and conjunctiva. The cardiovascular system has a regular I-II heart sound, 2/3 mid clavicular S noise as high as 2 ICS and a pansystolic murmur. The examination of the abdomen is slight distended. Child pug score 8. Hemoglobin value 6.7gr%, hematocrite 37%, APTT 44.8 seconds, SGOT 443 U / L, SGPT 560 U / L, total bilirubin 23.89 mg / dl, direct bilirubin 13.92 mg / dl, and indirect bilirubin 9.97 mg / dl.</p><p>The goal of anesthesia in VSD, Severe Tricuspid Regurgitation (TR) with Gerbode Defect is preventing excessive ventilation to avoid severe pulmonary hypertension. The choice of anesthetic agent is based on the patient's physiology and balancing pulmonary and systemic blood flow. Perioperative management of cases of VSD, TR Severe with Gerbode defect in the following report describes the importance of understanding the pathophysiology of VSD and Gerbode defects to obtain a good outcome.</p><p>Perioperative management of VSD patients, severe tricuspid regurgitation with Gerbode defect requires more supervision, especially to minimize the increase in PVR, maintain systemic vascular resistance (SVR) and avoid excessive ventilation to prevent severe pulmonary hypertension.</p>

scholarly journals Outcomes of Isolated Tricuspid Valve Surgery

2020 ◽  
Vol 23 (6) ◽  
pp. E763-E769
Author(s):  
Gemma Sánchez-Espín ◽  
Jorge Rodríguez-Capitán ◽  
Juan José Otero Forero ◽  
Víctor Manuel Becerra Muñoz ◽  
Emiliano Andrés Rodríguez Caulo ◽  
...  
Keyword(s):  
Tricuspid Valve ◽  
Tricuspid Regurgitation ◽  
Functional Class ◽  
Valve Surgery ◽  
Perioperative Mortality ◽  
Severe Tricuspid Regurgitation ◽  
Tricuspid Valve Surgery ◽  
Long Term Mortality

Background: Isolated tricuspid valve surgery is a rarely performed procedure and traditionally is associated with a bad prognosis, although its clinical outcomes still are little known. The aim of this study was to assess the short- and long-term clinical outcomes obtained at our center after isolated tricuspid valve surgery as treatment for severe tricuspid regurgitation. Methods: This retrospective study included 71 consecutive patients with severe tricuspid regurgitation who underwent isolated tricuspid valve surgery between December 1996 and December 2017. Perioperative and long-term mortality, tricuspid valve reoperation, and functional class were analyzed after follow up. Results: Regarding surgery, 7% of patients received a De Vega annuloplasty, 14.1% an annuloplasty ring, 11.3% a mechanical prosthesis, and 67.6% a biological prosthesis. Perioperative mortality was 12.7% and no variable was shown to be predictive of this event. After a median follow up of 45.5 months, long-term mortality was 36.6%, and the multivariate analysis identified atrial fibrillation as the only predictor (Hazard Ratio 3.014, 95% confidence interval 1.06-8.566; P = 0.038). At the end of follow up, 63.6% of survivors had functional class I. Conclusions: Isolated tricuspid valve surgery was infrequent in our center. Perioperative mortality was high, as was long-term mortality. However, a high percentage of survivors were barely symptomatic after follow up.

Echocardiographic probability of pulmonary hypertension: a validation study

2022 ◽  
pp. 2102548
Author(s):  
Michele D'Alto ◽  
Marco Di Maio ◽  
Emanuele Romeo ◽  
Paola Argiento ◽  
Ettore Blasi ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Right Heart Catheterization ◽  
Right Atrial ◽  
Pulmonary Vascular Disease ◽  
Heart Catheterization ◽  
Right Heart ◽  
Basal Diameter ◽  
Acceleration Time ◽  
Eccentricity Index ◽  
Current Guidelines

BackgroundAccording to current guidelines, the diagnosis of pulmonary hypertension (PH) relies on echocardiographic probability followed by right heart catheterization. How echocardiography predicts PH recently re-defined by a mean pulmonary artery pressure (mPAP) >20 mmHg instead of ≥25 mmHg and pulmonary vascular disease defined by a pulmonary vascular resistance (PVR) >3 or >2 Wood units has not been established.MethodsA total of 278 patients referred for PH underwent a comprehensive echocardiography followed by a right heart catheterization. Fifteen patients (5.4%) were excluded because of insufficient quality echocardiography.ResultsWith PH defined by a mPAP >20 mmHg, 23 patients had no PH, 146 had pre-capillary and 94 post-capillary PH. At univariate analysis, maximum velocity of tricuspid regurgitation (TRV) ≥2.9 and ≤3.4 m s−1, left ventricle (LV) eccentricity index >1.1, right ventricle (RV) outflow tract (OT) notching or acceleration time <105 ms, RV-LV basal diameter >1 and PA diameter predicted PH, whereas inferior vena cava diameter and right atrial area did not. At multivariable analysis, only TRV ≥2.9 m s−1 independently predicted PH. Additional independent prediction of PVR >3 Wood units was offered by LV eccentricity index >1.1 and RVOT acceleration time <105 ms and/or notching, but with no improvement of optimal combination of specificity and sensibility or positive prediction.ConclusionsEchocardiography as recommended in current guidelines can be used to assess the probability of re-defined PH in a referral center. However, the added value of indirect signs is modest and sufficient quality echocardiographic signals may not be recovered in some patients.

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