Chronic thromboembolic pulmonary hypertension secondary to implantable cardioverter defibrillator lead thrombus in a patient with Brugada syndrome: a rare complication requiring a multidisciplinary approach

2020 ◽  
Vol 13 (5) ◽  
pp. e234549
Author(s):  
Sofia Alegria ◽  
Filipa Ferreira ◽  
Débora Repolho ◽  
Maria José Loureiro
Keyword(s):  
Pulmonary Hypertension ◽  
Implantable Cardioverter Defibrillator ◽  
Brugada Syndrome ◽  
Rare Complication ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Functional Class ◽  
Heart Team ◽  
Cardioverter Defibrillator ◽  
Thromboembolic Pulmonary Hypertension ◽  
Appropriate Shocks

We report the case of a 57-year-old male patient with prior syncope associated with sustained ventricular tachycardia in the setting of Brugada syndrome, who was submitted to implantation of a cardioverter defibrillator for secondary prevention. During follow-up, he presented a significant increase in lead impedance, and a transthoracic echocardiogram showed a mass attached to the lead. He was started on oral anticoagulation after infective endocarditis was excluded but nevertheless suffered repeated episodes of pulmonary embolism that led to severe chronic thromboembolic pulmonary hypertension. After heart team discussion, he was referred to pulmonary endarterectomy and replacement of the implantable cardioverter defibrillator with a subcutaneous device. This led to significant improvement of functional class and normalisation of pulmonary haemodynamics. More recently, he suffered syncope in the setting of ventricular fibrillation with appropriate shocks and was started on quinidine without further recurrence of arrhythmic episodes.

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.

scholarly journals Balloon pulmonary angioplasty for inoperable chronic thromboembolic pulmonary hypertension: the UK experience

Open Heart ◽  
2020 ◽  
Vol 7 (1) ◽  
pp. e001144 ◽  
Author(s):  
Stephen P Hoole ◽  
John G Coghlan ◽  
John E Cannon ◽  
Dolores Taboada ◽  
Mark Toshner ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Functional Status ◽  
Minute Ventilation ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Carbon Dioxide Production ◽  
Functional Class ◽  
Right Heart Catheterisation ◽  
Balloon Pulmonary Angioplasty ◽  
Thromboembolic Pulmonary Hypertension ◽  
The Uk

ObjectiveInoperable chronic thromboembolic pulmonary hypertension (CTEPH) managed medically has a poor prognosis. Balloon pulmonary angioplasty (BPA) offers a new treatment for inoperable patients. The national BPA service for the UK opened in October 2015 and we now describe the treatment of our initial patient cohort.MethodsThirty consecutive, inoperable, anatomically suitable, symptomatic patients on stable medical therapy for CTEPH were identified and offered BPA. They initially underwent baseline investigations including Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR) quality of life (QoL) questionnaire, cardiopulmonary exercise test, 6 min walk distance (6MWD), transthoracic echocardiography, N-terminal probrain natriuretic peptide (NT pro-BNP) and right heart catheterisation. Serial BPA sessions were then performed and after completion, the treatment effect was gauged by comparing the same investigations at 3 months follow-up.ResultsA median of 3 (IQR 1–6) BPA sessions per patient resulted in a significant improvement in functional status (WHO functional class ≥3: 24 vs 4, p<0.0001) and QoL (CAMPHOR symptom score: 8.7±5.4 vs 5.6±6.1, p=0.0005) with reductions in pulmonary pressures (mean pulmonary artery pressure: 44.7±11.0 vs 34.4±8.3 mm Hg, p<0.0001) and resistance (pulmonary vascular resistance: 663±281 vs 436±196 dyn.s.cm-5, p<0.0001). Exercise capacity improved (minute ventilation/carbon dioxide production: 55.3±12.2 vs 45.0±7.8, p=0.03 and 6MWD: 366±107 vs 440±94 m, p<0.0001) and there was reduction in right ventricular (RV) stretch (NT pro-BNP: 442 (IQR 168–1607) vs 202 (IQR 105–447) pg/mL, p<0.0001) and dimensions (mid RV diameter: 4.4±1.0 vs 3.8±0.7 cm, p=0.002). There were no deaths or life-threatening complications and the mild-moderate per-procedure complication rate was 10.5%.ConclusionsBPA is safe and improves the functional status, QoL, pulmonary haemodynamics and RV dimensions of patients with inoperable CTEPH.

scholarly journals The role of the heart team in the diagnosis and treatment of chronic thromboembolic pulmonary hypertension

2020 ◽  
Vol 27 (5) ◽  
pp. 9-21
Author(s):  
L. V. Kulyk ◽  
Yu. M. Sirenko ◽  
G. D. Radchenko ◽  
L. I. Vasylyeva ◽  
I. O. Zhyvylo ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Surgical Treatment ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Diagnosis And Treatment ◽  
Long Term Results ◽  
High Tech ◽  
Heart Team ◽  
Thromboembolic Pulmonary Hypertension ◽  
Team Concept

The aim – to present to the medical community the functioning algorithm of the heart team concept in the diagnosis and surgical treatment of chronic thromboembolic pulmonary hypertension (CTEPH) in Ukraine. The concept of the CTEPH team implies a multi-disciplinary approach to the diagnosis and, consequently, surgical treatment of CTEPH with the participation of a radiologist, a pulmonologist, a cardiologist, a hematologist, a neurologist, and a cardiac surgeon. Considering the low incidence of the disease, the need for high-tech diagnostic tools, including CT angiography and angiopulmonography, as well as challenges of evaluating the operability of patients and the technical complexity of operations, patients with CTEPH are get together in the so-called reference centers. Within the framework of the CTEPH team concept, specific protocols and «road maps» have been developed for both diagnosis and treatment of the disease. In order to eliminate subjectivity in determining the operability of a patient with CTEPH, a special algorithm has been developed, which is applied to a clinical case for illustration. Monitoring patients with pulmonary embolism who are qualifed for a newly coined definition – postembolic pulmonary syndrome, has become a new task of the reference centers. The new syndrome is suggested to include CTEPH, as well as a similar, but not identical pathological condition, named chronic thromboembolic lung disease. The treatment of choice for CTEPH is pulmonary thrombendarterectomy. Mandatory elements of the surgical protocol include the creation of a «dry» operating field by means of a temporary circulatory arrest under deep hypothermia of 18 °C. Operations for distal lesions of the pulmonary arteries have become a recent achievement. The success of the operation depends on the anatomical type of the lesions, the degree of distal arteriopathy, the extent of the intervention, and the comorbid factors. Immediate and long-term results of the operation are evaluated as good and very good. The long-term survival of patients after surgery is significantly higher than of those who were administered medical treatment.Conclusions. Patients with suspected CTEPH should be referred to a reference expert center for diagnosis verification and operability determining. The standard diagnosis of CTEPH is angiopulmonography with simultaneous measurement of pressure in the right heart; the standard treatment is pulmonary thrombendarterectomy.

Abstract 16122: Percutaneous Transluminal Balloon Angioplasty Ameliorates Metabolic and Renal Dysfunctions Associated With Hemodynamic Improvement in Patients With Chronic Thromboembolic Pulmonary Hypertension

Circulation ◽  
2014 ◽  
Vol 130 (suppl_2) ◽  
Author(s):  
Shunsuke Tatebe ◽  
Koichiro Sugimura ◽  
Kotaro Nochioka ◽  
Masanobu Miura ◽  
Saori Yamamoto ◽  
...  
Keyword(s):  
Insulin Resistance ◽  
Pulmonary Hypertension ◽  
Renal Function ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Functional Class ◽  
Walk Distance ◽  
Pulmonary Hemodynamics ◽  
Nyha Functional Class ◽  
Thromboembolic Pulmonary Hypertension ◽  
Percutaneous Transluminal

Background: Insulin resistance, dyslipidemia and renal dysfunction have been regarded as poor prognostic factors for pulmonary hypertension. We and others have recently demonstrated that percutaneous transluminal pulmonary angioplasty (PTPA) markedly improves pulmonary hemodynamics in patients with chronic thromboembolic pulmonary hypertension (CTEPH). In this study, we examined whether PTPA also improves metabolic and renal impairments in CTEPH patients. Methods and Results: From April 2012 to May 2013, we examined serum levels of lipids and fatty acids fractions and plasma levels of glucose and immunoreactive insulin in 68 consecutive patients with CTEPH (64±14[SD] years, M/F 13/55) and calculated the homeostatic model assessment of insulin resistance (HOMA-IR). Renal function was assessed by estimated GFR (eGFR) and urinary albumin-to-creatinine (U-A/C) ratio. Vascular stiffness was evaluated by cardio-ankle vascular index (CAVI). The measurements were repeated after PTPA in 49 patients. Among the 68 patients, we noted NYHA functional class ≥III in 17, 6 min-walk distance <300m in 15, and cardiac index <2.0l/min/m 2 in 18, and regarding metabolic disorders, hypertension in 41, diabetes in 6 and dyslipidemia in 24. Insulin resistance (defined as HOMA-IR >2.0) was noted in 29 out of 63 (58%). Regarding renal function, eGFR was 61.6±17.8 ml/min/m 2 , U-A/C ratio 80.8±214.7mg/gCre, and chronic kidney disease in stage ≥3 was noted in 32 patients (47%). We performed PTPA in 49 patients (mean 3.3 essions/patient), which markedly improved NYHA functional class (P=0.008), 6min-walk distance (102±25 m, P<0.0001) and mean pulmonary arterial pressure (-9.9±1.3 mmHg, P<0.0001). Furthermore, PTPA significantly improved metabolic profiles such as HDL-chol (6.2 ±2.2 mg/dl, P=0.01), EPA (18.3±6.2 mg/dl, P=0.006), fasting blood sugar (-8.0±3.5 mg/dl, P=0.04), HbA1c (-0.3±0.1 %, P<0.0001) and CAVI (-0.44±0.23, P=0.02) . PTPA also significantly improved eGFR (5.2±1.2 ml/min/m 2 , P <0.001) and U-A/C ratio (-47.1±18.9 mg/gCre, P=0.004). Conclusions: These results indicate that metabolic and renal dysfunctions are commonly present in CTEPH patients and that PTPA markedly improves those disorders in addition to pulmonary hemodynamics.

scholarly journals Sequential treatment with riociguat and balloon pulmonary angioplasty for patients with inoperable chronic thromboembolic pulmonary hypertension

2018 ◽  
Vol 8 (3) ◽  
pp. 204589401878399 ◽  
Author(s):  
Christoph B. Wiedenroth ◽  
H. Ardeschir Ghofrani ◽  
Miriam S.D. Adameit ◽  
Andreas Breithecker ◽  
Moritz Haas ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Functional Class ◽  
World Health ◽  
Pulmonary Hemodynamics ◽  
Mean Pulmonary Arterial Pressure ◽  
Pulmonary Hemodynamic ◽  
Hemodynamic Assessment ◽  
Balloon Pulmonary Angioplasty ◽  
Thromboembolic Pulmonary Hypertension

Riociguat is the treatment of choice for inoperable patients with chronic thromboembolic pulmonary hypertension (CTEPH). We addressed here whether additional balloon pulmonary angioplasty (BPA) provides further benefits. A prospective series of 36 consecutive patients with inoperable CTEPH were treated with riociguat at least three months before BPA. All patients underwent diagnostic workup at baseline, before BPA treatments, and six months after final intervention. The main outcome measures were pulmonary hemodynamic parameters and World Health Organization (WHO) functional class (FC). Significant improvements in pulmonary hemodynamics and physical capacity were observed for riociguat treatment, and subsequent BPA interventions yielded further benefits. With targeted medication, WHO FC improved by at least one class in 13 (36.1%) patients ( P = 0.01). Hemodynamic assessment showed significant improvements in mean pulmonary arterial pressure (mPAP) (49 ± 12 mmHg vs. 43 ± 12 mmHg; P = 0.003) and PVR (956 ± 501 dyn·s·cm–5 vs. 517 ± 279 dyn·s·cm–5; P = 0.0001). Treatment with a combination of targeted medication and BPA resulted in WHO FC improvement in 34 (94.4%) patients. Hemodynamic assessment showed significant improvement in mPAP (43 ± 12 mmHg vs. 34 ± 14 mmHg; P = 0.0001) and PVR (517 ± 279 dyn·s·cm–5 vs. 360 ± 175 dyn·s·cm–5; P = 0.0001). These findings provide, for the first time, support for the therapeutic strategy recommended by current guidelines.

scholarly journals Balloon Pulmonary Angioplasty in Patients with Chronic Thromboembolic Pulmonary Hypertension: Impact on Clinical and Hemodynamic Parameters, Quality of Life and Risk Profile

2020 ◽  
Vol 9 (11) ◽  
pp. 3608
Author(s):  
Pavel Jansa ◽  
Samuel Heller ◽  
Michal Svoboda ◽  
Michal Pad’our ◽  
David Ambrož ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Pulmonary Hypertension ◽  
Real Life ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Heart Association ◽  
Risk Profile ◽  
Functional Class ◽  
Balloon Pulmonary Angioplasty ◽  
Thromboembolic Pulmonary Hypertension

Balloon pulmonary angioplasty (BPA) is a novel treatment option for patients with chronic thromboembolic pulmonary hypertension (CTEPH) who are not eligible for pulmonary endarterectomy (PEA) or suffer from persistent pulmonary hypertension after PEA. The aim of this study was to evaluate the real-life efficacy and safety of BPA in a consecutive group of patients who were diagnosed and treated in the national referral center for CTEPH in the Czech Republic. Here we report data from 160 BPA procedures performed in 64 patients. Efficacy analysis was performed in the subgroup of 25 patients who completed BPA series. Significant improvements were observed in New York Heart Association functional class (4% to 79% in I/II, p < 0.001), 6 min walking test distance (+54.3 m, p < 0.001), risk profile (15.8% to 68.5% with presence of 2/3 low risk criteria, p < 0.001), pulmonary artery mean pressure (−18%, p < 0.001), pulmonary vascular resistance (−32%, p < 0.001), stroke volume (+17%, p = 0.011) and quality of life (+37% in assessment of overall health status by a patient, p < 0.001). We observed 1 fatal periprocedural complication (1.6% of all 64 patients) and 19 BPA-related non-fatal complications (11.9% of all 160 interventions) that predominantly included hemoptysis (10.0% of all sessions). Overall survival at 12 months was 94.6%.

scholarly journals Effect of domiciliary oxygen therapy on exercise capacity and quality of life in patients with pulmonary arterial or chronic thromboembolic pulmonary hypertension: a randomised, placebo-controlled trial

2019 ◽  
Vol 54 (2) ◽  
pp. 1900276 ◽  
Author(s):  
Silvia Ulrich ◽  
Stéphanie Saxer ◽  
Elisabeth D. Hasler ◽  
Esther I. Schwarz ◽  
Simon R. Schneider ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Pulmonary Hypertension ◽  
Exercise Capacity ◽  
Physical Functioning ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Ambient Air ◽  
Functional Class ◽  
Thromboembolic Pulmonary Hypertension ◽  
Exercise Induced

Study questionWe investigated whether domiciliary oxygen therapy (DOXT) increases exercise capacity and quality of life in patients with pulmonary arterial or distal chronic thromboembolic pulmonary hypertension (PAH/CTEPH) presenting with mild resting hypoxaemia and exercise-induced oxygen desaturation.Materials and methods30 patients with PAH/CTEPH, mean±sdage 60±15 years, pulmonary artery pressure 39±11 mmHg, resting arterial oxygen saturation measured by pulse oximetry (SpO2) ≥90%,SpO2drop during a 6-min walk test ≥4%, on pulmonary hypertension-targeted medication, were randomised in a double-blind crossover protocol to DOXT and placebo (ambient air) treatment, each over 5 weeks, at 3 L·min−1vianasal cannula overnight and when resting during the day. Treatment periods were separated by 2 weeks of washout. Co-primary outcomes were changes in 6-min walk distance (6MWD, breathing ambient air) and physical functioning scale of the 36-item short-form medical outcome questionnaire during treatment periods.ResultsDOXT increased the 6MWD from baseline 478±113 m by a mean (95% CI) of 19 (6–32) m, and physical functioning from 52±29 by 4 (0–8) points. Corresponding changes with placebo were 1 (−11–13) m in 6MWD and −2 (−6–2) points in physical functioning. Between-treatment differences in changes were 6MWD 18 (1–35) m (p=0.042) and physical functioning 6 (1–11) points (p=0.029). DOXT significantly improved the New York Heart Association functional classversusplacebo.Answer to the questionThis first randomised trial in PAH/CTEPH patients with exercise-induced hypoxaemia demonstrates that DOXT improves exercise capacity, quality of life and functional class. The results support large long-term randomised trials of DOXT in PAH/CTEPH.

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

2019 ◽  
Vol 91 (4) ◽  
pp. 43-47
Author(s):  
N M Danilov ◽  
Yu G Matchin ◽  
A M Chernyavsky ◽  
A G Edemsky ◽  
D S Grankin ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Artery ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Functional Class ◽  
Reverse Remodeling ◽  
Systolic Pulmonary Artery Pressure ◽  
Right Heart ◽  
Balloon Pulmonary Angioplasty ◽  
Thromboembolic Pulmonary Hypertension ◽  
The Right

Aim. To evaluate the effectiveness of balloon pulmonary angioplasty (BPA) in patients with inoperable chronic thromboembolic pulmonary hypertension (CTEPH). Materials and methods. Forty patients with inoperable CTEPH were enrolled in this study. The indications were determined by multidisciplinary team. The average age of patients was 53.5 [43; 63] years. In 65% of cases patients had functional class III (according to WHO); the distance in the 6-minute walk test (6MWD) was 327 [280; 400] m; catheterization of the right heart revealed systolic pulmonary artery pressure (SPAP) 82 [64; 100] mm Hg, mean pulmonary artery (mPAP) 48.5 [38; 56] mm Hg, pulmonary vascular resistance (PVR) 784 [525; 1257] dyn·s/cm-5. Each patient underwent 6 BPA. Results and discussion. The effectiveness of BPA was assessed 2 months after the last session. According to the data of right heart catheterization SPAP decreased by 27.3%, mPAP by 26%, PVR by 34.5% from baseline. After all series of BPA echocardiography and magnetic resonance imaging demonstrated reverse remodeling of the right heart. Also significant decrease in the level of BNP by 62%, increasing in 6MWD distance by 39% and improvement of the functional class up to I in 60% cases and up to II in 40% cases were noted. Conclusion. The results of the present study demonstrated a high efficacy of BPA allowing to normalize hemodynamic and clinical parameters, increasing the physical activity. Balloon pulmonary angioplasty is a new highly effective, safe method for treating patients with inoperable CTEPH.

scholarly journals Balloon pulmonary angioplasty for inoperable patients with chronic thromboembolic pulmonary hypertension: the initial German experience

2017 ◽  
Vol 49 (6) ◽  
pp. 1602409 ◽  
Author(s):  
Karen M. Olsson ◽  
Christoph B. Wiedenroth ◽  
Jan-Christopher Kamp ◽  
Andreas Breithecker ◽  
Jan Fuge ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Chronic Thromboembolic Pulmonary Hypertension ◽  
Functional Class ◽  
Right Heart Catheterisation ◽  
Balloon Pulmonary Angioplasty ◽  
Thromboembolic Pulmonary Hypertension ◽  
Pulmonary Bleeding ◽  
Diagnostic Work ◽  
Randomised Controlled ◽  
Work Up

Balloon pulmonary angioplasty (BPA) is an emerging treatment for patients with inoperable chronic thromboembolic pulmonary hypertension (CTEPH).We report on a prospective series of 56 consecutive patients who underwent 266 BPA interventions (median, five per patient) at two German institutions. All patients underwent a comprehensive diagnostic work-up including right heart catheterisation at baseline and 24 weeks after their last intervention.BPA resulted in improvements in WHO functional class, 6 min walk distance (mean change, +33 m), right ventricular function and haemodynamics, including a decline in mean pulmonary artery pressure by 18% and in pulmonary vascular resistance by 26%. Procedure-related adverse events occurred in 9.4% of the interventions. The most common complications were related to pulmonary vascular injury and consecutive pulmonary bleeding. Most of these events were asymptomatic and self-limiting, but one patient died from pulmonary bleeding, resulting in a mortality rate of 1.8%.BPA resulted in haemodynamic and clinical improvements but was also associated with a considerable number of complications, including one fatal pulmonary bleeding. As the effects of BPA on survival are unknown, randomised controlled outcome trials comparing BPA with approved medical therapies in patients with inoperable CTEPH are required to allow for appropriate risk–benefit assessments.

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