bluuebunny/crossref_metadata_2025 huggingface dataset:
Mechanisms of angina in patients with biopsy-proven viral myocarditis: insights from intracoronary acetylcholine testing
Authors: Seitz A , Martinez Pereyra V , Hubert A , Klingel K , Bekeredjian R , Sechtem U , Ong P
Published: November 2020

Background

Patients with myocarditis often present with angina pectoris despite unobstructed coronary arteries. The underlying pathophysiological mechanism of angina in these patients remains to be elucidated. Coronary artery spasm is a well-known cause of angina in patients with unobstructed coronary arteries. In this study, we sought to assess the frequency of coronary vasomotor disorders in patients with biopsy-proven viral myocarditis.

Methods

In total, 700 consecutive patients who underwent endomyocardial biopsy for suspected myocarditis between 2008 and 2018 were retrospectively screened. Of these patients, viral myocarditis was confirmed in 303 patients defined as histological/immunohistological evidence of myocardial inflammation and presence of viral genome confirmed by PCR. Of these patients, 34 patients had angina despite unobstructed coronary arteries and underwent intracoronary acetylcholine (ACh) provocation testing in search of coronary spasm. Epicardial spasm was defined as acetylcholine-induced reproduction of the patient's symptoms associated with ischemic ECG changes and >90% epicardial vasoconstriction. Microvascular spasm was defined as symptom reproduction and ECG changes in the absence of significant epicardial vasoconstriction.

Results

Patients were 49±16 years old, 62% were male and left ventricular ejection fraction was 54±16%. Most frequent viruses were parvovirus B19 (PVB19, 59%) and human herpes virus 6 (HHV6, 26%), 2 patients had combined PVB19/HHV6 infection and 3 patients other herpesviruses (CMV, EBV, VZV). Epicardial spasm was observed in 10 patients (29%) during ACh testing and microvascular spasm was found in 11 patients (32%). The rate of coronary spasm (epicardial and microvascular) was higher in the PVB19 subgroup compared to HHV6 (80% vs. 33%, p=0.031). In particular, there was a higher prevalence of microvascular spasm in PVB19 compared to HHV6 (45% vs. 0%, p=0.018).

Conclusion

We observed a high prevalence of microvascular and epicardial spasm in patients with biopsy-proven viral myocarditis suggesting coronary spasm as a potential underlying mechanism for angina in these patients. Microvascular spasm was most often observed in patients with PVB19-associated myocarditis.

Funding Acknowledgement

Type of funding source: Foundation. Main funding source(s): Robert-Bosch-Stiftung; Berthold-Leibinger-Stiftung
Prevalence and prognostic value of right ventricular dysfunction in hypertrophic cardiomyopathy
Authors: Marques A , Pereira A.R , Cruz I , Almeida A.R , Alegria S , Briosa A , Grade Santos J , Sebaiti D , Lopes L , Joao I , Pereira H
Published: November 2020

Introduction

Hypertrophic cardiomyopathy (HCM) is the main cause of sudden cardiac death in the young and a cause of heart failure and death at any age. Nevertheless, adverse long-term outcomes are not easy to predict.

Objectives

To assess the prevalence, predictors and prognostic value of right ventricular (RV) dysfunction in patients (pts) with HCM.

Methods

Retrospective single-center study of consecutive pts with HCM evaluated in a specialized medical appointment. Selected those submitted to cardiac magnetic resonance imaging (MRI) as the gold-standard for RV function assessment. The primary endpoint was a composite of cardiovascular death, nonfatal myocardial infarction, nonfatal stroke, ventricular arrhythmias with hemodynamic instability and unplanned heart failure admission.

Results

Were included 112 pts (mean age at first appointment 57±15 years, 63% male). Septal asymmetric phenotype was the most frequent (75%), with a mean septal wall thickness of 18±4 mm. Late gadolinium enhancement was observed in 82%, mostly intramyocardial (67%) and in joint points (47%).

RV dysfunction was detected in 6 pts (5.4%) and RV free wall hypertrophy in 3 pts (2.7%); no patient presented RV dilation.

Factors associated with RV dysfunction were left atria area (HR 1.07/unit, 95% CI 1.01–1.12, p=0.02), left ventricular ejection fraction (HR 0.91/unit, 95% CI 0.86–0.97, p=0.02) and the presence of left ventricle wall motion abnormalities (HR 7, 95% CI 1.3–38, p=0.03) in cardiac MRI.

During a mean follow-up of 60±31 months, the combined primary endpoint occurred in 15 pts (13%), significantly more in pts with RV dysfunction (HR 5.1, 95% CI 1.1–24, p=0.038) (graphic 1). Patients with RV dysfunction also presented more atrial fibrillation / flutter episodes during follow-up (HR 6.4, 95% CI 2.1–20, p=0.001).

Conclusions

Although not common, right ventricular dysfunction was associated with a higher rate of cardiovascular events. These results support a potential role of right ventricular function in the risk stratification of patients with hypertrophic cardiomyopathy.

Figure 1

Funding Acknowledgement

Type of funding source: None
Diastolic stress test echocardiography in patients with hypertrophy cardiomyopathy: association with exercise capacity
Authors: Fava A.M , Sande Mathias I , Alashi A , Saijo Y , Popovic Z , Thamilarasan M , Lever H , Desai M.Y
Published: November 2020

Background

Diastolic dysfunction (DD) is a frequent occurrence in hypertrophic cardiomyopathy (HCM) patients. Symptoms of DD are often unmasked only during exercise, as left ventricular (LV) filling pressure is normal at rest.

Purpose

We sought to establish if abnormal DST parameters are associated with reduced exercise capacity in HCM patients.

Methods

We examined 590 asymptomatic/minimally symptomatic HCM patients (54±14 years, 57% men, body mass index 30±6 kg/m2, 84% on beta-blockers) with HCM by echocardiography at rest & maximal exercise. Septal and lateral [e'] mitral annular velocity, peak early [E] and late [A] mitral inflow velocity, Mitral E/e' and left atrial volume index (LAVI) was recorded. Exercise functional capacity was stablished as age-gender predicted metabolic equivalents (AGP-METs).

Results

Echocardiography at rest had LVEF of 62±6, wall thickness of 16.7±0.5 mm, LVMI of 111±43 g m–2, LVOT gradient of 26±33 mmHg, LAVI 34.5±17 mm, E/A ratio of 1.2±0.6, and average E/e' ratio 12.7±0.6. Following maximal exercise had a mean LVOT gradient of 61±59 mmHg, E/A ratio of 1.2±1.0 and average E/e' ratio 12.7±1.0. Only 42% had >85% of age-sex predicted METs. Logistic regression analysis testing the association between various predictors and 85% AGP-METs are shown in table 1.

Conclusion

There is a significant association between abnormal diastolic response to exercise and reduced exercise capacity in patients with HCM.

Funding Acknowledgement

Type of funding source: None
Targeted septal branch microcirculatory embolization with tris-acryl gelatin microspheres in hypertrophic obstructive cardiomyopathy
Authors: Okutucu S , Aksoy H , Yetis Sayin B , Oto A
Published: November 2020

Background

Alcohol septal ablation (ASA) has been shown to be an effective treatment in patients with hypertrophic obstructive cardiomyopathy (HOCM) who are refractory to medical treatment. ASA may cause some life-threatening complications including conduction disturbances, hemodynamic compromise, ventricular arrhythmias, distant and massive myocardial necrosis. Tris-acryl gelatin microspheres provide consistent and predictable results for effective targeted microcirculatory embolization.

Purpose

We aimed to report our initial experience in tris-acryl gelatin microspheres for septal ablation in HOCM.

Methods

Microspheres are biocompatible, hydrophilic, non-resorbable microspheres which are available in a range of calibrated sphere sizes. In our method, after the cannulation of the left anterior descending by a 6F-7F guiding catheter, a 0.014-inch guidewire is introduced through the catheter and advanced into the septal branch. This septal artery is selectively cannulated with a 4F catheter over the guidewire. Selective angiography of the septal artery is performed to show the anatomy and collateral branches to other coronary arteries. Contrast echocardiography is performed to make sure that the pertinent septal artery is the target vessel supplying the hypertrophied septum. A microcatheter is then advanced deep enough into the septal artery through the 4F catheter. Microspheres/contrast solution infused slowly under fluoroscopic guidance into the targeted septal branches initially using coronary arteriolar sized small particles (diameter 100–300 μm); then the particle size was stepped up to larger particles (diameter 300–500 μm) until a complete block of the arteriolar flow is achieved.

Results

Septal ablation with tris-acryl gelatin microspheres was performed in 6 patients (mean age = 47.8±11.5; 5 males). Immediately after the procedure peak left ventricular outflow (LVOT) gradient reduced significantly both for direct catheter (69.0±13.8 vs. 8.2±3.7 mmHg, P<0.001) and Doppler echocardiographic measurements (78.8±19.9 vs. 12.0±5.1 mmHg, P<0.001). Post-procedure peak serum CK- MB fraction concentration was 82±22 ng/ml (reference range is 0 - 4.9 ng/mL) and peak serum troponin T concentration was 1.2 ng/ml [(interquartile range, 0.4–1.4), (reference range is 0 - 0.017 ng/mL)]. LVOT tract gradient reduction persisted after 6 months follow-up. There was no significant complication during the procedure and within a 6 months follow-up period.

Conclusions

The novel strategy by targeted septal branch microcirculatory embolization with tris-acryl gelatin microspheres seems to be an efficient and safe approach to HOCM. Further experience is needed in order to assess the long-term efficacy and safety of this technique.

Funding Acknowledgement

Type of funding source: None
Prognostic accuracy of common heart failure scales in patients with dilated cardiomyopathy
Authors: Dziewiecka E , Gliniak M , Winiarczyk M , Karapetyan A , Wisniowska-Smialek S , Karabinowska A , Podolec P , Rubis P
Published: November 2020

Introduction

The reliable identification of patients with heart failure (HF) and high mortality risk is crucial for treatment optimalization and prognostication, especially in younger population in dilated cardiomyopathy (DCM). However, the profile of DCM patients significantly differs from the general HF population. Therefore, the applicability of commonly used HF prognostic scales may be sub-optimal in DCM.

Purpose

Comparison of the prognostic accuracy of common HF prognostic scales in DCM.

Methods

Between 2010 and 2018 we analysed 406 DCM in- and outpatients (aged 54±14 years, 76% male, NYHA: 2.5±0.9, symptoms duration: 40±58 months, EF: 26±9%, LVEDd: 66±10mm, NT-proBNP: 3662±7617pg/ml, 10% had ICD, 3.5% had CRT). The mortality risk was assessed by 8 most popular HF prognostic scores. In 2019 information on patients status were gathered after 48±32months.

Results

During first month 4 patients (1.0%) died, after 1 year – 19 (6%), 2 years – 32 (11%), 3 years – 42 (18%), 4 years – 52 (27%), 5 years – 59 (40%). BCN and SHFM calculators had the best prognostic ability for 1–5 years mortality (Table 1).

Conclusions

Mortality risk of DCM patients during 5 years is high and reaches 40%. BCN and SHFM calculators had the best prognostic ability for 1–5 years mortality.

Funding Acknowledgement

Type of funding source: Public Institution(s). Main funding source(s): Department of Scientific Research and Structural Funds of Medical College, Jagiellonian University
Gender differences, rates of arrhythmias, cardiac implantable electronic devices, diagnostic modalities among sarcoidosis patients
Authors: Gonuguntla K , Patil S.P , Rojulpote C , Borja Z.E , Bravo P.E
Published: November 2020

Introduction

Sarcoidosis is a granulomatous disease with multiorgan involvement. Cardiac involvement may be asymptomatic or present clinically as heart failure, arrhythmias or even sudden cardiac death.

Objective

We compared gender differences in prevalence of arrhythmias and associated outcomes in patients with sarcoidosis without established coronary artery disease.

Methods

The United States Nationwide Inpatient Sample was queried from 2010 to 2014 to identify patients with sarcoidosis using ICD-9 diagnosis code in patients >18 years. We excluded patients with a prior history of myocardial infarction, percutaneous coronary intervention and coronary artery bypass graft. Chi-square test was used for statistical analysis.

Results

The sample consisted of 308,064 patients (Mage = 55.65±11.28 years); they were mostly women 945 (65.2%) and black 957 (46.7%). In-hospital mortality in this cohort was 1,574 (2.5%). The most common arrhythmias was atrial fibrillation 29,850 (9.7%). Prevalence of ventricular fibrillation was 669 (0.2%), ventricular tachycardia 6,184 (2%), complete heart block 1462 (0.5%) and second degree Mobitz type II 245 (0.1%). Sudden cardiac death occurred in 2,059 (0.7%). Rates of various cardiac devices implanted were: implantable cardiac defibrillator (ICD) 1,452 (0.5%), cardiac resynchronization therapy-defibrillator (CRT-D) 553 (0.2%), pacemaker 1259 (0.4%). Rates of endomyocardial biopsy (EMB), radionuclide imaging, and cardiac magnetic resonance imaging were 470 (0.2%), 879 (0.3%), and 224 (0.1%), respectively. Based on gender (male vs. female), the rates of arrhythmias, cardiac device implantation and utilization of diagnostic modalities were: atrial fibrillation (41% vs 59%; p<0.001), ventricular fibrillation (50% vs 50%; p=0.983), ventricular tachycardia (55% vs 45%; p<0.001), complete heart block (48% vs 52%; p=0.3), second degree Mobitz type II (37% vs 63%; p=0.706), sudden cardiac death (38% vs 62%; p<0.171), ICD (56% vs 44%; p<0.001), CRT-D (58% vs 42%; p<0.025), pacemaker (40% vs 60%; p=0.066), EMB (55% vs 45%; p<0.001), radionuclide imaging (32% vs 68%; p=0.403), cardiac MRI (41% vs 59%; p=0.396). In-hospital mortality was higher in females (36% vs 64%; p<0.001).

Conclusion

In our study, in-hospital death was more common in females. Females had higher rates of atrial fibrillation compared to males who were found to have a higher burden of ventricular tachycardia. Males had higher rates of ICD and CRT-D placement. Males also had EMB performed more commonly than females.

Funding Acknowledgement

Type of funding source: None
Bleeding events in patients with cardiac amyloidosis
Authors: Schrutka L , Avanzini N , Seirer B , Rettl R , Dachs T , Duca F , Binder C , Dalos D , Badr Eslam R , Bonderman D
Published: November 2020

Background

Amyloid cardiomyopathy (CA) is a life-threatening disease that arises from the accumulation of insoluble fibrous deposits of misfolded proteins in the myocardium. Infiltration of the conduction system results in a high prevalence of atrial fibrillation (AF). Due to a notable thromboembolic risk, long-term anticoagulation is preferred once AF is detected in amyloidosis. However, anticoagulation may exacerbate the hemorrhagic tendency, previously described in amyloid light-chain (AL) amyloidosis. Therefore, the potential benefits of anticoagulation must be carefully weighed against hemorrhagic complications.

Methods

We recorded bleeding events in a prospective cohort of CA patients.

Results

Out of 140 patients (median age of 73 years (IQR 62–77) and 77.1% of male gender) 65 (46.4%) were diagnosed with AL amyloidosis and 69 (49.3%) had transthyretin amyloid deposits (ATTR). Median NYHA functional class was 3 (IQR 2–3) and NT-proBNP 2574 pg/mL (1070–7173). 42.1% of patients (n=59) were treated with OAC with 13 (22.0%) receiving vitamin-K-antagonists (VKA) and 46 (78.0%) novel oral anticoagulants (NOACS). During a median follow up time of 23 months (IQR 9–35) 22 bleeding events occurred. In total, we recorded 1 cerebral bleeding, 7 gastrointestinal bleedings, 8 urogenital bleedings, 4 hematomas and 2 nasal bleedings. Of those, fifteen (68.2%) were observed in orally anticoagulated patients (p=0.198). 4 (30.8%) bleeding events occurred in the VKA treated group and 11 (23.9%) under NOAC treatment (p=0.335). Bleeding events were more prevalent in patients with AL compared to TTR amyloidosis (14, 63.6% vs 8, 36.4%, p=0.011). No difference was observed in terms of CHA2DS2-VASc score: 4 (IQR 2–5) in the bleeding group versus 3 (IQR 1–4) in the control group (p=0.579). On the contrary, HASBLED score was higher in patients with bleeding events: 4 (IQR 4–5) versus 2 (IQR 1–3) (p<0.001). Patients with bleeding events had a higher prevalence of arterial hypertension (p=0.010) and chronic kidney disease (p=0.002). Overall survival was comparable between the groupss (Figure 1).

Conclusion

Although CA is associated with potentially life-threatening hemorrhage, our cohort did not reveal a higher prevalence of fatal bleeding in patients treated with oral anticoagulation. Patients who experienced bleeding events did not show worse outcomes.

Figure 1. Kaplan Meier survival estimates

Funding Acknowledgement

Type of funding source: None
Coronary sinus diameter to inferior vena cava diameter ratio in the diagnosis of cardiac tamponade: a novel approach
Authors: Ozcan Cetin E.H , Cetin M.S , Tekin Tak B , Ekizler F.A , Ozcan F , Cay S , Ozeke O , Topaloglu S , Aras D
Published: November 2020

Background and aim

Coronary sinus (CS) as an intrapericardial, low-pressure, thin-walled structure can be easily compressed in cardiac tamponade. Whereas, inferior vena cava (IVC) is an extrapericardial structure which dilates in tamponade as opposed to the response of CS. The inverse relationship between these two venous structures may augment their role in the evaluation of tamponade imaging. Therefore, we assessed the usefullness of computerized tomographic measures of CS diameter, and also CS to IVC ratio (CS/IVC) to predict tamponade in clinically stable patients with large pericardial effusion.

Materials and methods

66 clinically stable patients who had large pericardial effusions, were included to the study. CS diameter was measured from the point at 1 cm proximal to the CS ostium11. IVC diameter was measured from the segment between its right atrial orifice and hepatic vein.

Results

Patients with tamponade had 40% smaller CS diameter (5.3±1.8 vs 8.8±2.6 mm p<0.001) and 35% lower CS/IVC ratio (20.7±5.5 vs 34.7±10.5% p<0.001).After adjusting with other parameters, only either CS diameter or CS/IVC ratio predicted tamponade respectively. (Nagelkerke r square value for CS diameter was 53.7% and 72.1% for CS/IVC ratio). 1 mm increase in CS diameter and 1% increase in CS/IVC ratio were associated with an increased odds ratio of 59% and 39% in predicting tamponade, respectively (p value <0.001).In ROC analysis, a cut of value of 6.85 mm for CS diameter, had 82.6% sensitivity and 83.7% specificity for predicting cardiac tamponade (Area under the curve 0.879, p<0.001). Additionally, a cut of value of 27% for CS / IVC ratio had 87.0% sensitivity and 86.0% specificity for predicting cardiac tamponade (Area under the curve 0.945, p<0.001).

Conclusion

The tomographic measures of both the CS diameter and the CS/IVC ratio predicted tamponade in clinically stable patients with large pericardial effusion. Compared with CS diameter, CS/IVC ratio seemed to be a more powerful predictor of tamponade

Figure 1

Funding Acknowledgement

Type of funding source: None
Invasive electrophysiological studies in adult congenital heart disease- evolving use and impact of specialized centre therapy on recurrence of arrhythmia: a nationwird analysis based on 2.433 cases
Authors: Fischer A.J , Enders D , Baumgartner H , Diller G.P
Published: November 2020

Background

Arrhythmias are a major contributor to morbidity and mortality in adult patients with congenital heart disease (ACHD). Advances in ablation technology have contributed to improved management and reduction of symptoms in this population. However, only limited data exist on the use and outcome of these technologies in large community-based cohorts of ACHD patients and on the impact of specialized centres on recurrence of arrhythmias.

Purpose

We performed a retrospective analysis based on data from one of the largest German Health Insurance Companies (approx. 9 million insured members), acquiring real-world data on frequency of invasive electrophysiological studies (EPS) in Germany between 2005 and 2018. ACHD patients were identified based on ICD codes and the spectrum of disease as well as the impact of EPS being performed at specialized ACHD centres was analysed.

Results

Out of 45,761 eligible ACHD patients in the database, we identified 2,433 EPS performed in 1,706 ACHD (51% female, median age 55.4 years, complexity of CHD mild, moderate and severe in 50.6%, 33.2%,16.2%, respectively). Over the study period the annual number of EPS increased by 207%. The majority of procedures were for supraventricular tachycardias (85.9%). Of these procedures atrial fibrillation accounted for 35.1%, atrial flutter for 29.4% and other supraventricular tachycardias/pre-excitation syndromes for 29.9% of cases. The majority of EPS (64.3%) was performed at non-specialized centres including 40.2% of cases in patients with complex disease. Overall, the re- intervention rate within 12 months of the primary EPS was 14.5%. Whereas in ACHD patients with simple and moderate complexity disease no obvious difference in 12-months re- intervention rate was observed between specialized and non-specialised centres (15.5% vs. 15.0%), in patients with complex disease the reintervention rate was 41.6% higher for non-specialized centres (13.6% vs. 9.6%).

Conclusion

Our large real- world analysis shows an increasing need for invasive electrophysiological studies in ACHD patients. The vast majority of procedures was performed for supraventricular arrhythmias including in atrial fibrillation and flutter ablation. Re-interventions were frequently required in this unique and anatomically challenging population. Alarmingly, many procedures were performed at non-specialized centres, although current guidelines recommend that interventions in complex patients should remain reserved for experienced high-volume centres.

Funding Acknowledgement

Type of funding source: None
Early extubation following cardiac surgery in adult congenital heart disease improves post-operative haemodynamics and reduces costs
Authors: Costola G , Constantine A , Bianchi P , Mele S , Shore D , Aw T.C , Dimopoulos K
Published: November 2020

Background

In paediatric cardiac surgery, there has been a paradigm shift in perioperative management from a slow wean of mechanical ventilation in the intensive care unit (ICU), to “ultra fast-track” anaesthesia with early extubation (EE) in theatre to promote a faster recovery. Adults with congenital heart disease (ACHD) have multiple risk factors for prolonged intubation, including a greater proportion of re-do interventions, more co-morbidities and metabolic differences leading to slower emergence from anaesthesia. As a result, EE remains unproven and has not been widely adopted and in this patient group.

Aim

To assess the effects of EE on post-operative haemodynamics, hospital stay and associated healthcare costs.

Methods

Data were collected on ACHD patients, who underwent cardiac surgery in a high-volume tertiary centre between 2012 and 2018. Propensity score matching (1:1 or 2:1 where possible) was performed using the following variables: age, sex, body mass index, CHD complexity, functional class, length of surgery, systemic ventricular function, procedure-specific risk (adult congenital heart score; ACHS), urgent versus elective procedure, active endocarditis, pulmonary hypertension and renal dysfunction.

Results

614 procedures were performed during the study period. After matching, 87 (14.2%) patients receiving EE were compared to 164 patients who received conventional care (CC). The overall complication rate was low, with no difference between the EE and CC groups (8.0% vs. 9.1%, p=0.77), and a very low reintubation rate (<1%). EE patients had a significantly shorter post-operative hospital length of stay in ICU and the high dependency unit (HDU; 48 [43–51] vs. 50 [47–69] hours, p<0.0001). EE patients required less inotropic & vasopressor support, as demonstrated by a lower Vasoactive-Inotropic Score (VIS) compared to patients following NEE (median VIS 0.5 [0.0–1.8] vs. 2.0 [0.0–3.5], p<0.0001). The total fluid balance by the third post-operative day was more positive after CC than EE (1177±737mL vs. 927±780mL, p=0.004). Finally, lower combined ICU and HDU costs were incurred by EE compared with CC (£3.9K[2.8–4.2K] vs. £4.2K[3.9–6.3K], p<0.0001).

Conclusion

In ACHD patients undergoing cardiac surgery, including complex and redo procedures, EE was safe, associated with a shorter ITU and HDU stay and lower hospital costs.

Funding Acknowledgement

Type of funding source: None
Long term follow-up after pregnancy in Fontan patients
Authors: Gribaudo E , Constantine A , Pires A , Ahmed I , Patel R , Gatzoulis M.A , Johnson M , Dimopoulos K , Rafiq I
Published: November 2020

Background

Patients with single ventricle physiology who have benefited from the Fontan procedure are reaching childbearing age. In this population, complications are common around pregnancy, but the long term effect of pregnancy is unclear.

Purpose

To evaluate functional and structural changes several years after pregnancy in patients with Fontan circulation.

Methods

Women with Fontan circulation who had a pregnancy beyond the 20 weeks of gestation followed at our centre between 2005–2019 were included. The following data were collected before pregnancy and at the last follow up (f-up): cardiac anatomy, type of Fontan procedure, resting O2 saturation (O2sat), NYHA class, systemic atrioventricular valve (SAVV) regurgitation, ventricular function, history of arrhythmias, heart failure (HF), thromboembolism, Fontan-related liver disease (FLD), protein-losing enteropathy (PLE), plastic bronchitis and cardiopulmonary test data.

Results

12 patients had 18 pregnancies, giving birth to 17 live newborns (1 newborn death at 24 weeks of gestation). 7 (58.3%)patients had a total cavo-pulmonary connection, 3 (25%)an atrio-pulmonary connection and 2 (16.7%)a Bjork type Fontan. Tricuspid atresia was the most common anatomy (6, 50%). Before pregnancy, O2sat was 95.4±1.9%and all patients were in NYHA class I or II. All patients (12, 100%)had a normal or mildly impaired ventricular function and 3 (25%)had a moderate or severe SAAV regurgitation. 6 (50%)patients had 2 pregnancies. Mean age at first pregnancy was 29.9±4.4 years. Cardiac events occurred in 7 (38%)pregnancies, most commonly atrial arrhythmia (4, 57%), 2 (16.6%)patients developed new cyanosis and 1 had a thromboembolic event. No maternal death occurred and 1 patient had severe haemorrhage. After a mean f-up of 84±48 months, there was no significant worsening in O2sat (94.3±3.1 at f-up, p=1) and all patients remained in NYHA class I or II (12, 100%, p=1). At f-up all patients (12, 100%)continued to have a normal or mildly impaired ventricular function (p=1) and there was no significant worsening in the SAAV regurgitation (3 patients with moderate or severe regurgitation, p=1). 3 (25%)patients developed FLD (p=0.3), 3 (25%) a new episode of atrial arrhythmia (p=0.3). There were no significantly differences in peak VO2 (22.5±3.8 pre-pregnancy, 22.8±5.5 at f-up, p=0.4) and VE/VCO2 slope (34.9±11.2 pre-pregnancy, 36.3±11.8 at f-up, p=0.2)before pregnancy and during f-up.

Conclusions

In this small cohort of Fontan patients who successfully completed at least 1 pregnancy, there was no significant decline in functional or structural cardiac parameters long term after delivery.

Funding Acknowledgement

Type of funding source: None
Pregnancy outcomes in women with a systemic right ventricle and transposition of the great arteries. Results from the ESC-EORP Registry of Pregnancy and Cardiac disease (ROPAC)
Authors: Tutarel O , Baris L , Johnson M , Hall R , Roos-Hesselink J.W
Published: November 2020

Background

Cardiac disease is one of the major causes of maternal mortality. We studied pregnancy outcomes in women with a systemic right ventricle (sRV) after the atrial switch procedure for transposition of the great arteries (TGA) or congenitally corrected TGA (CCTGA).

Methods

The ESC-EORP Registry of Pregnancy and Cardiac Disease is an international prospective registry of pregnant women with cardiac disease. Pregnancy outcomes (maternal and fetal) in women with a sRV are described. The primary endpoint was a major cardiovascular event (MACE) defined as maternal death, supraventricular or ventricular arrhythmias requiring treatment, heart failure, aortic dissection, endocarditis, ischemic coronary event and other thromboembolic events.

Results

Altogether, 163 women with a sRV (TGA n=121, CCTGA n=42, mean age 28.8±4.6 years) were included. Maternal mortality did not occur. At least one MACE occurred in 26 women (heart failure in 16 (9.8%), arrhythmias (atrial 5, ventricular 6) in 11 (6.7%), and others in 4 (2.5%)). Predictors of MACE were pre-pregnancy signs of heart failure [Odds ratio (OR) 6.05, 95% CI: 1.41–25.97, p=0.02] as well as a sRV ejection fraction below 40% [OR 2.81, 95% CI: 1.18–6.69, p=0.02]. One woman experienced fetal loss, while no neonatal mortality was observed. No significant differences were found between women with CCTGA and TGA. In the subset of women who had an echocardiogram before and after pregnancy, no clear deterioration in sRV was observed.

Conclusion

The majority of women with a sRV tolerated pregnancy well with a favorable maternal and fetal outcome. Heart failure and arrhythmias were the most common MACE.

Funding Acknowledgement

Type of funding source: None
Pre-operative pulmonary artery pulsatility index does not predict mortality post cardiac transplantation but PVR is a prognostic tool in LVAD-bridged patients
Authors: Bart N , Hungerford S.L , Emmanuel S , Kearney K , Kotlyar E , Keogh A.M , Hayward C.S
Published: November 2020

The Pulmonary artery pulsatility index (PAPi) is a novel haemodynamic marker that has been shown to be a strong predictor of right heart failure and mortality in patients with pulmonary hypertension and advanced heart failure. Its utility in predicting post transplant outcomes is currently unknown.

Aim

To determine the prognostic significance of pre-transplant haemodynamic assessment of PAPi, right atrial pressure (RA), and pulmonary vascular resistance (PVR) in predicting mortality post cardiac transplant.

Methods

All patients who underwent cardiac transplantation between January 2010 and June 2016 were retrospectively analyzed. Pre-operative right heart catheter data was obtained. The PAPi was calculated as the systolic pulmonary artery pressure (sPAP)- diastolic pulmonary artery pressure (dPAP) divided by RA. Statistical analysis was carried out using SPSS v25.

Results

A total of 158 patients, with a mean age of 48.6±13.5 were studied (115 non- LVAD, 43 LVAD pre-transplant), and 3 patients were excluded due to missing data. There was no significant difference in the non-LVAD group in RA, PVR or PAPi for mortality post transplant. In the LVAD group there was no significant difference in RA, or PAPi with regards to mortality, however there was a significant difference in mean PVR between those that died post transplant (222±100) and those still alive (138±59)

Conclusion

Despite having a role in prognostication for right heart failure in pulmonary hypertension and post LVAD implantation, PAPi did not discriminate mortality outcomes for patients post cardiac transplantation. PVR remains a marker of mortality in an LVAD cohort bridged to transplant.

Funding Acknowledgement

Type of funding source: None
Evaluation of arterial stiffness in new diagnosed idiopathic pulmonary arterial hypertension patients
Authors: Radchenko G , Zhyvylo I , Titov E , Sirenko Y.U
Published: November 2020

Objective

This study is the first attempt to use cardio-ankle vascular index (CAVI) for the evaluation of systemic arterial stiffness in patients with IPAH.

Methods

112 patients were included in the study: group 1 – 45 patients with new diagnosed IPAH, group 2 – 32 patients with arterial hypertension, control group – 35 healthy persons adjusted by age. Right heart catheterization, ECG, a 6-minute walk test (6 MWT), echocardiography, blood pressure (BP) measurement and ambulatory BP monitoring, pulse wave elastic artery stiffness (PWVe) [segment carotid-femoral arteries] and muscular artery stiffness (PWVm) [segment carotid-radial arteries], CAVI, N-terminal pro-B-type natriuretic peptide (NT-proBNP) level were provided. The Spearman correlation, a linear regression and multivariable binary logistic analysis were performed to indicate the predictors associated with PWV and CAVI.

Results

The PWVm and PWVe were the highest in hypertensive patients – 10.3±1.5 and 11.42±1.70 m/s. The control group and IPAH did not have significant differences in aorta BP, but PWVm/PWVe values were significantly (P<0.003/0.008) higher in IPAH patients than in the control group - 8.1±1.9/8.49±1.92 vs 6.63±1.34/7.29±0.87 m/s. The CAVIs on both sides were significantly lower in the healthy subjects (5.91±0.99/5.98±0.87 right/left side). Patients with IPAH did not differ from the arterial hypertension patients by CAVIs in comparison with the control group - 7.40±1.32/7.22±1.32 vs 7.19±0.78/7.2±1.1. PWVe did not correlate with any parameters except uric acid. PWVm correlated with uric acid (r=0.58, P<0.001), NT-proBNP (r=0.33, P=0.03) and male gender (r=0.37, P=0.013) at Spearman analysis, but not at multifactorial linear regression analysis. The CAVI correlated with age and parameters characterized functional capacity (6 MWT distance) and right ventricle function (NT-proBNP, TAPSE) at Spearman analysis and with age and TAPSE at multifactorial linear regression analysis. At binary logistic regression analysis CAVI >8.0 at right or/and left side had a correlation with age, 6MWT distance, TAPSE, but an independent correlation was only with age (β=1.104, P=0.008, CI 1.026–1.189) and TAPSE (β=0.66, P=0.016, CI 0.47–0.93).

Conclusion

In spite of equal and at normal range BP level the age adjusted patients with IPAH had significantly stiffer arteries than the healthy persons and they were comparable with the arterial hypertensive patients. Arterial stiffness evaluated by CAVI correlated with age and TAPSE in IPAH patients.

Funding Acknowledgement

Type of funding source: Public grant(s) – National budget only. Main funding source(s): National Academy of Medical science of Ukraine
NT-proBNP (N-terminal prohorne of brain natriuretic peptide) as an early marker for detection of response to endothelin receptor antagonist in pulmonary arterial hypertension patients
Authors: Chang S.A , Lim K.H , Park J.B , Kim D.K , Kim H.K
Published: November 2020

Background

Endothelin receptor antagonist (ERA) is well-established for targeted therapy of pulmonary arterial hypertension (PAH). According to the guidelines, comprehensive evaluation of responses to PAH-targeted therapy is recommended 3 to 6 months after treatment onset. Early detection of response to ERA after starting new treatment may be of clinical value to establish next therapeutic plan.

Purpose

We sought to investigate whether NT-proBNP plays a reliable role for early maker of responses to ERA in PAH patients.

Methods

Sixty four patients (age >19 years old) with a confirmatory diagnosis of PAH were enrolled who were treated with ERA (bosentan or macitentan) in two tertially PAH experts centers. NT-proBNP measurements were performed at baseline, 1 month and 6 months after ERA treatment. Clinical characteristics including WHO functional class, laboratory test, and echocardiographic results were systematically collected.

Result

Sixty four patients were finally analyzed who completed the 6 months of follow-up. Etiology of PAH was as follows: idiopathic PAH (n=25), connective tissue disease associated PAH (n=16) and congenital heart disease assocciated PAH (n=24). Mean age was 50±15 years-old and female were predominant (75%). NTproBNP level was changed from 703pg/ml [207–2748] at baseline, 301 pg/ml [126–1314] at 1 month and 297pg/ml [106–901] at 6 months after ERA treatment. Although, NT proBNP level in overall population was significantly decreased after ERA treatment, NT proBNP level before treatment showed poor correlation with those at 6 months (R2=0.32). However, change of NT proBNP at 1 month from baseline showed strongly correlated with change of NT proBNP at 6 months from baseline with linear regression model analysis (R2 =0.90, p<0.001).

Conclusions

Assessment of early change in nt-proBNP with only a month of treatment is associated with midterm response to ERA treatment in PAH. It can help to early decision making for early intensive therapy and rapid escalation of PAH medication in non-responsive PAH patients for initial therapy.

The relationship of BNP

Funding Acknowledgement

Type of funding source: Private company. Main funding source(s): Handok
The assessment of lung function and residual hypoxemia after balloon pulmonary angioplasty for patients with chronic thromboembolic hypertension
Authors: Matsuoka Y , Taniguchi Y , Keisuke M , Onishi H , Tsuboi Y , Otake H , Emoto N , Hirata K
Published: November 2020

Background

It has been reported that balloon pulmonary angioplasty (BPA) could dramatically improve hemodynamics in patients with chronic thromboembolic pulmonary hypertension (CTEPH). However, some patients have shown poor response in oxygenation and lung function even after BPA. The aim of our study was to evaluate the efficacy of BPA using clinical indicators and to investigate lung function and residual hypoxemia.

Method

Consecutive 103 patients who underwent BPA from September 2011 to August 2018 were enrolled. We evaluated hemodynamics with right heart catheterization, respiratory function test, arterial blood gas examination, and exercise capacity with 6 minute-walk-distance (6MWD).

Result

At the median 12-month follow-up after the final BPA session, following examination findings were significantly improved. Mean pulmonary artery pressure (39.0±8.2mmHg, 23.2±8.4mmHg, p<0.001), Pulmonary vascular resistance (782±378 dynes / sec / cm-5, 331±187 dynes / sec / cm-5, p<0.001), 6 MWD (310±94 m, 363±119 m, p<0.001), PaO2 (63.1±15.8 mmHg, 68.5±13.6 mmHg, p=0.044),%VC (88.5±17.1, 91±16.3, p=0.036). However, the following examination findings did not improve after BPA. Oxygen desaturation during 6MWD test (−11.9±6.3, 12±7.9, p=0.65), %DLCO (64.2 17.1, 61.3 16.8, p=0.147).

Conclusion

BPA could dramatically improve hemodynamics and exercise tolerance. However, arterial oxygenation was not normalized after BPA. Moreover, oxygen desaturation in exercise, and %DLCO were almost unchanged. These observations might suggest the remaining arteriopathy in capillary level of pulmonary artery, but further research is needed.

Funding Acknowledgement

Type of funding source: None
Differential effect of anticoagulant treatment on vascular morphology in patients with acute pulmonary embolism
Authors: Braams N.J , Boon G.J.A.M , Den Exter P.L , Kroft L.J.M , Beenen L.F.M , Huisman M.V , Vonk Noordegraaf A , Bogaard H.J , De Man F.S , Klok F.A , Meijboom L.J
Published: November 2020

Introduction

Chronic thromboembolic pulmonary hypertension (CTEPH) is described as one of the most severe consequence of acute pulmonary embolism (APE). When signs of chronic PE are present on the CT pulmonary angiogram (CTPA) used to diagnose APE, the question arises whether there is underlying CTEPH. The relevance of chronic lesions, as well as the effect of anticoagulant treatment on their development is currently unknown.

Purpose

To investigate the effect of anticoagulant treatment on CTPA derived vascular morphological abnormalities in patients with APE.

Methods

We performed a case-cohort study. As cases, we selected CTEPH patients who had a prior history of a first APE episode. As cohort, we selected patients who had a follow-up CTPA performed 6 months after first episode APE in the context of a clinical trial (den Exter 2015). A baseline (i.e. at moment of APE diagnosis) CTPA and a follow-up CTPA was available for all patients. Experienced chest radiologists morphologically assessed 20 segmental pulmonary arteries per patient as “normal” or “affected” (as defined by a total occlusion by thrombus, central thrombus, mural thrombus, web or tapered pulmonary artery). Pulmonary segmental vessels of the entire cohort were merged for the analysis. All patients were treated adequately with anticoagulant treatment (vitamin K antagonists, direct oral anticoagulation or low molecular weight heparin) in the period between baseline and follow-up CTPA according to current guidelines.

Results

A total of 30 cases and 116 controls were included. Mean time between baseline and follow-up CTPA was 193 (62) days. At baseline CTPA, 1647 (56%) of the 2920 pulmonary segmental vessels were scored as affected. Almost all central thrombi resolved after oral anticoagulant treatment (1103/1191=93%). Webs (n=85) and tapered pulmonary arteries (n=57) did not change in morphology at follow-up (Figure 1). Most vessels containing a total occlusion by a thrombus at baseline resolved completely (156/280=56%), changed to a tapered pulmonary artery (26%) or became a web (7%). Mural thrombi either remained unchanged (16/34=47%), resolved completely (29%) or became a web (24%).

Conclusion

After anticoagulant treatment for APE almost all central thrombi completely resolved, whereas mural thrombi or total occlusions by thrombi either resolved or transformed to a web or tapered pulmonary artery. Interestingly, none of the webs and tapered pulmonary arteries resolved after anticoagulant treatment. Therefore, webs and tapered pulmonary arteries at the moment of APE diagnosis indicate a chronic PE state.

Figure 1. Web at baseline and follow-up

Funding Acknowledgement

Type of funding source: Private company. Main funding source(s): Research grant from Actelion Pharmaceuticals
Sleep-related breathing disorders in patients with pulmonary hypertension
Authors: Dyachenko M , Simakova M , Korostovtseva L , Bochkarev M , Sviryaev Y , Moiseeva O
Published: November 2020

Introduction

There are some evidences of sleep-related breathing disorders (SRBD) association with pulmonary hypertension (PH). Only limited information regarding the SRBD prevalence in pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) patients, is available.

Purpose

To estimate the features of SRBD and the asymmetric dimethylarginine (ADMA) level in PAH and CTEPH patients and their relationship with the severity of PH.

Methods

There were included 31 patients (45% male; 55% female) with a verified diagnosis of precapillary PH: 22.6% – with PAH; 9,7% with PAH associated with adult congenital heart disease; 64.5% – with CTEPH; 3.2% with PAH associated with connective tissue disease. Patients underwent a general clinical examination, questionnaires, a comprehensive assessment of respiratory function, a complete polysomnographic study, ECG and ECHO studies, assessment of clinical and biochemical blood tests, including an assessment of ADMA and NT-proBNP levels.

Results

SRBD of a predominantly obstructive nature were found in 74.2% of the examined: 38.7% were mild, 22.6% were moderate, 12.9% were severe. In 35% of patients, episodes of periodic breathing during sleep were recorded. There was no association SRBD with PH functional class and hemodynamic parameters. Moderate correlation was observed between the apnea-hypopnea index and remodeling indices of the heart chambers according to ECHO: for the end-diastolic size of the left ventricle (ρ=0.54; p=0.005); for an indicator of the ratio of the size of the ventricles (RV/LV) (ρ=−0.41, p=0.05). The presence of SRBD wasn't associated with the level of peripheral blood saturation according to pulse oximetry in sleep and during wakefulness. Negative moderate correlation was observed between NT-proBNP level and the average peripheral blood saturation at night in the entire examined group (ρ=−0.40; p=0.035). All patients showed an increase in ADMA, with no association between ADMA and SRBD severity.

Conclusions

The incidence of SRBD is extremely high in PAH and CTEPH patients. However, there no association between presence and severity of PH, including with right heart remodeling. But the severity of nocturnal hypoxemia is associated with an increase in NT-proBNP level, a proven marker of poor prognosis in PAH and CTEPH groups of patients. SRBD severity indices (apnea-hypopnea index and desaturation index) are associated with ECHO parameters characterizing remodeling of left heart chambers. Increasing ADMA level as a marker of endothelial dysfunction was related with PH severity, but not with SRBD.

Funding Acknowledgement

Type of funding source: Public Institution(s). Main funding source(s): Almazov National Medical Research Centre
Relationship between effects of riociguat and levels of methemoglobin in patients with chronic thromboembolic pulmonary hypertension
Authors: Sugimoto K , Nakazato K , Oikawa M , Kobayashi A , Yamaki T , Kunii H , Yoshihisa A , Ishida T , Takeishi Y
Published: November 2020

Background

Soluble guanyl cyclase (sGC) is a receptor for nitric oxide (NO) and plays an important role in vascular tonus. sGC stimulant is a therapeutic agent for pulmonary hypertension and an advantage of sGC stimulant over phosphodiesterase (PDE)-5 inhibitors is that sGC stimulant exerts its effect even when NO production is reduced. NO derived from vascular endothelial cells is immediately absorbed by hemoglobin (Hb), which leads to the production of methemoglobin (Met-Hb) when oxidized. Previous report has shown that the therapeutic effect of PDE-5 inhibitors was associated with levels of Met-Hb.

Purpose

In this study, we examined the relationship between the effect of riociguat and levels of Met-Hb in patients with chronic thromboembolic pulmonary hypertension (CTEPH).

Methods

The study population comprised 18 patients with CTEPH. Mean pulmonary artery pressure (mPAP) and pulmonary vascular resistance (PVR) were assessed before and after administration of riociguat, and changes in mPAP and PVR were defined as ΔmPAP and ΔPVR, respectively. Since the level of Met-Hb was obtained as the percentage of Hb (FMet-Hb), the amount of Met-Hb was calculated by following formula:

Met-Hb (mg/dL) = Hb (g/dL) × FMet-Hb (%) × 10.

Results

The amount of Met-Hb value before administration of riociguat was significantly correlated with the degree of improvement in mPAP and PVR after administration of riocigat (R=−0.502, P<0.05 mPAP; R=−0.481, P<0.05 PVR, respectively) as shown in figures.

Conclusion

Our findings suggest that the level of Met-Hb before treatment may predict the therapeutic effect of sGC stimulants in patients with CTEPH.

Funding Acknowledgement

Type of funding source: None
Comparison of pulmonary vascular resistance and pulmonary artery compliance during exercise between IPAH and CTEPH with normal pulmonary artery pressure
Authors: Nakata C , Takeuchi K , Kikuchi H , Goda A , Inami T , Satoh T , Soejima K
Published: November 2020

Background

Pulmonary vascular resistance (PVR) and pulmonary artery compliance (PAC) are inversely related. However, the little is known about dynamics during exercise by disease difference. The aim of this study was to reveal the relationships of PVR and PAC during exercise between idiopathic pulmonary arterial hypertension (IPAH) and chronic thromboembolic pulmonary hypertension (CTEPH) patients.

Methods

Sixty-two IPAH patients (45±9 y.o) and 359 CTEPH patients (63±13 y.o) with normal mean PAP and PAWP at rest underwent symptom-limited exercise test using supine cycle ergometer with right heart catheter.

Results

There were no differences between baseline mean PAP and PAWP in 2 groups, however, cardiac output, SaO2 and SvO2 were lower in CTEPH group. Lower PAC (2.9±1.1 vs. 3.7±1.7 ml/mmHg, p<0.001) and higher PVR (2.3±1.0 vs. 1.9±1.0 wood.unit, p=0.016) were observed in CTEPH group. These trends were also seen at peak exercise. PVR-PAC relationship in CTEPH group was leftward shift compared with IPAH group (Figure 1).

Conclusion

Resting and exercise PVR and PAC in CTEPH patients were worse than those in IPAH patients who had normal PAP and PAWP at rest.

Figure 1

Funding Acknowledgement

Type of funding source: None