bluuebunny/crossref_metadata_2025 huggingface dataset:
Response to fluid challenge in patients with atrial septal defect
Authors: D'Alto M , Chessa M , Gaio G , Santoro G , Giordano M , Romeo E , Argiento P , Wacker J , D'Aiello F , Sarubbi B , Russo M.G , Naeije R , Golino P
Published: November 2020

Background

A fluid challenge test (FCT) with a rapid infusion of saline allows for discrimination between pre- and post-capillary pulmonary hypertension (PH) and may unmask hidden post-capillary PH. Patients with atrial septal defect (ASD) may develop pre- or post-capillary PH after shunt closure respectively in case of pulmonary vascular disease or left ventricular disease.

Aim

To evaluate the haemodynamic changes of the pulmonary circulation in ASD patients undergoing percutaneous closure with indicated according to the current ESC guidelines.

Methods

Twenty-three patients (mean age 42.9±12.4 years; 15 female) underwent right heart catheterization in basal conditions and after FCT (volume loading with rapid saline infusion of 7 ml/kg in 10 min) before percutaneous closure of the ASD.

Right atrial pressure (RAP), systolic, mean and diastolic pulmonary arterial pressure (sPAP, mPAP and dPAP), pulmonary arterial wedge pressure (PAWP), cardiac output (CO), pulmonary vascular resistance (PVR), systemic vascular resistance (SVR) and the ratio between pulmonary and systemic flow (QP/QS) were calculated four times: before and after inflating the sizing balloon both at baseline and immediately after FCT (Fig. 1).

Results

The patients had an increase in pressures and flows after FCT with open ASD: mPAP (18.7±4.4 vs 16.7±4.6 mmHg, p<0.001), PAWP (11.3±3.1 vs 9.2±3.0 mmHg, p<0.001), QP (12.5±2.3 vs 10.3±2.0 l/min, p<0.001), and QS (6.6±1.4 vs 5.9±1.2 l/min, p<0.001) but RAP remained unchanged (8.7±3.0 vs 8.3±2.4 mmHg, p=0.35). PVR (0.2±0.4 vs 0.8±0.3 Wood Units, p<0.001) and SVR (11.2±3.2 vs 12.5±3.2 Wood Units, p=0.02) decreased, and PVR/SVR (0.06±0.02 vs 0.06±0.3, p=0.25) remained unchanged. QP/QS increased in all patients after FCT (mean±SD: 2.0±0.4 vs 1.8±0.4, p<0.001).

During a temporary ASD closure by sizing balloon, the patients had increases of RAP (9.0±2.6 vs 7.6±2.6 mmHg, p<0.001) mPAP (19.5±4.0 vs 17.4±3.7 mmHg, p<0.001), PAWP (13.2±2.1 vs 11.2±2.9 mmHg, p<0.001), and CO (7.7±2.7 vs 6.8±2.3 l/min, p<0.001) after FCT. PVR remained unchanged (0.9±0.4 vs 1.1±0.6, p=0.12) and SVR reduced (9.8±2.7 vs 11.3±2.9, p<0.001) after FCT.

Conclusions

None of the reported ASD patients presented with FCT criteria of post-capillary PH (that is a PAWP >18 mmHg). The FCT was associated with an increase in QP/QS suggesting that the patients still had a distensible pulmonary circulation. Further studies are needed to explore the relevance of a FCT in ASD patients, particularly those with higher PVR values.

Figure 1

Funding Acknowledgement

Type of funding source: None
Long-term outcome of adult patients with total anomalous pulmonary venous connection: data from the SACHER registry and a French center
Authors: Touray M , Bouchardy J , Schwerzmann M , Greutmann M , Tobler D , Engel R , Harald G , Pruvot E , Blanche C , Sekarski N , Ladouceur M , Rutz T
Published: November 2020

Introduction

Total anomalous pulmonary venous connection is a rare cyanotic congenital heart disease, where all pulmonary veins aberrantly connect to a systemic vein or the right atrium. The only curative treatment is surgery allowing the patients to reach adulthood. This study describes the long-term outcome of these individuals focusing on arrhythmias.

Methods

Clinical, surgical, imaging and invasive data were retrospectively reviewed from 7 centers participating in the Swiss Adult Congenital Heart disease Registry (SACHER) and one French center.

Results

A total of 57 patients were identified and analyzed 22±8 years after surgery.

At last follow-up, 21% of patients presented cardiac symptoms, mainly palpitations. No patient had pulmonary hypertension or a relevant valvulopathy. Echocardiography revealed in 5 (8.8%) patients a dilated right ventricle (RV) and in 3 (5.3%) patients a diminished RV systolic function. Exercise capacity was normal in most patients (table). Cardiac magnetic resonance imaging found in 2 (4%) had a residual shunt with an mean Qp:Qs of 1.25, due to a single anomalously connected pulmonary vein.

Holter recordings revealed arrhythmias in 23% of patient. Ten (17.5%) had atrial fibrillation, flutter or tachycardia. Three (5%) patients presented ventricular arrhythmias: two patients showed non-sustained ventricular tachycardia and one patient complex ventricular extrasystoles. Four patients (7%) were on antiarrhythmic medication for supraventricular arrhythmias. Three patients (5%) underwent an electrophysiological study with a mean time since surgery of 20 years. Three (5%) patients underwent pacemaker implantation within 3 weeks to 36 months after surgical correction, which were removed in 2 patients after 7 years.

Age and the presence of a valvulopathy at follow-up predicted tachyarrhythmia on binomial logistic regression analysis (p<0.03).

Conclusions

In adult survivors after TAPVC repair, supraventricular but also ventricular arrhythmias are frequently observed which appears to be related to age and valvular lesions at follow-up. This study underlies the importance of long-term follow-up as some of the patients currently without arrhythmia will probably develop rhythm disorders in the future.

Funding Acknowledgement

Type of funding source: Private grant(s) and/or Sponsorship. Main funding source(s): Unrestricted grant by Actelion SA, Switzerland
Clinical course long after atrial switch: a novel risk score for serious clinical events
Authors: Woudstra O , Zandstra T.E , Vogel R.F , Van Dijk A.P.J , Vliegen H.W , Kies P , Jongbloed M.R.M , Egorova A.D , Doevendans P.A , Konings T.C , Mulder B.J.M , Tanck M.W.T , Meijboom F.J , Bouma B.J
Published: November 2020

Background

Patients after atrial switch surgery for transposition of the great arteries (TGA-AtrSO) experience serious clinical events during adulthood, mainly heart failure and arrhythmias, but data on the emerging risks remain scarce.

Purpose

To assess the risk for events during the clinical course in adulthood of TGA-AtrSO patients and provide a novel risk score for event-free survival.

Methods

We reviewed medical records of TGA-AtrSO patients from five hospitals. Endpoints were all-cause mortality, heart failure (HF), defined as HF hospitalizations, heart transplantation, ventricular assist device implantation, or HF-related death, and symptomatic ventricular arrhythmias (VA). Predictors for event-free survival were examined to construct a prediction model using bootstrapping techniques.

Results

We followed 169 TGA-AtrSO patients (60% Mustard, age 28 [IQR 24–36] years) for 13 [IQR 9–16] years, during which 17 (10%) died, 34 (20%) had HF events, and 15 (9%) had VA events. Five-year risk of mortality, first HF event, and first VA increased from 1% each at age 25, to 7% (95% CI 4–10%), 17% (95% CI 10–25%), and 4% (95% CI 2–8%), respectively, at age 50. A prediction model combining age >30, prior VA, age >1 year at repair surgery, QRS duration >120ms, ≥mild LV dysfunction, and severe tricuspid regurgitation discriminated well between patients at low (<5%), medium (5–20%) and high (>20%) 5-year risk (optimism corrected C-statistic=0.84). Observed 5- and 10-year survival in low-risk patients were 100% and 99%, compared to only 45% and 19% in high-risk patients.

Conclusion

The clinical course of atrial switch patients increasingly consists of serious clinical events, especially heart failure. A novel risk score stratifying patients as low, medium, and high risk for event-free survival is presented, providing information on absolute individual risks which may support decisions for pharmacological and interventional management.

Figure 1. Observed event-free survival of patients with predicted low risk (<5% in 5 years), medium risk (5–20% in 5 years) and high risk (>20% in 5 years).

Funding Acknowledgement

Type of funding source: Foundation. Main funding source(s): Dutch Heart Foundation; Amsterdam University Fund
Decreasing mortality in patients with simple congenital heart disease: a Danish nationwide study
Authors: El-Chouli M , Malmborg M , Bang C.N.F , Gislason G.H
Published: November 2020

Background

The long-term mortality in patients with simple congenital heart disease (SCHD) compared with the general population is not well-described.

Purpose

To investigate the 10-year mortality in individuals with and without SCHD and whether it has changed since 1977 using contemporary data.

Method

By linking Danish nationwide registries, we identified all individuals with and without a SCHD diagnosis who were alive at age 40 between 1977–2006. Excluded were individuals with moderate or severe congenital heart disease. SCHD was defined as isolated ventricular septal defect (VSD), atrial septal defect (ASD), patent ductus arteriosus (PDA) or pulmonary stenosis (PS). The population was followed from age 40 until death or emigration, whichever came first. We predicted 10-year all-cause mortality according to each year of inclusion. Reported was 10-year all-cause mortality and mortality ratios (SCHD vs non-SCHD) with 95% confidence intervals (CI) by calendar year groups (1977–1986, 1987–1996, 1997–2006).

Results

We identified 2,040 individuals with SCHD (VSD: 27.5%, ASD: 62.2%, PDA 6.8%, PS: 3.5%), of which 1,121 (55.0%) were female, and 2,083,277 individuals without SCHD, of which 1,028,769 (49.4%) were female. In individuals with SCHD the 10-year all-cause mortality decreased over time in both men (1977–1986: 12.3% [11.8–12.9%], 1987–1996: 9.0% [7.4–10.5%], 1997–2006: 5.0% [4.3–5.7%]) and women (1977–1986: 7.7% [7.5–7.9%], 1987–1996: 4.9% [3.9–6.0%], 1997–2006: 1.2% [0.7–1.7%]), whereas the 10-year risks were somewhat stable in individuals without SCHD for both men (1977–1986: 3.2% [3.2–3.2%], 1987–1996: 3.3% [3.2–3.3%], 1997–2006: 2.9% [2.7–3.0%]) and women (1977–1986: 2.4% [2.3–2.4%], 1987–1996: 2.1% [2.1– 2.2%], 1997–2006: 1.7% [1.6–1.8%]) (Figure 1, panel A). The mortality ratio decreased over time in both men (1977–1986: 3.9 [3.7–4.1], 1987–1996: 2.7 [2.3–3.2], 1997–2006: 1.7 [1.5–1.9]) and women (1977–1986: 3.3 [3.2–3.3], 1987–1996: 2.3 [1.8– 2.7], 1997–2006: 0.7 [0.4–1.0]) (Figure 1, panel B) remaining significantly higher for men, but not women, in 1997–2006.

Conclusion

In individuals with simple congenital heart disease aged 40 years, the 10-year mortality decreased dramatically over time for both men and women. Despite decreasing mortality, men with SCHD, but not women, remained at a higher 10-year mortality compared to individuals without SCHD.

Figure 1. Temporal trends in 10-year mortality

Funding Acknowledgement

Type of funding source: Foundation. Main funding source(s): Danish Heart Foundation
Echocardiography and cardiac magnetic resonance in children with repaired tetralogy of fallot and their correlations with exercise capacity
Authors: Avesani M , Borrelli N , Krupickova S , Sabatino J , Piccinelli E , Josen M , Michielon G , Fraisse A , Di Salvo G
Published: November 2020

Background

Severe pulmonary regurgitation (PR) and progressive right ventricular (RV) dilation and disfunction are common in patients with repaired Tetralogy of Fallot (r-TOF) and should be carefully monitored during the follow up of these patients. In this contest, Echocardiography and Cardiac Magnetic Resonance (CMR) have a complementary diagnostic role.

Purpose

To correlate Echo and CMR parameters in children (<18 years) with r-TOF with at least moderate PR assessed by Echocardiography and to analyse which parameter was associated with peak oxygen consumption (Vo2).

Methods

Paediatric patients with r- TOF with at least moderate PR at the echo evaluation who underwent a CMR study within six months were included by using hospital databases. All patients underwent standard echo-Doppler study including RV end-diastolic area (RVEDA), end-systolic area (RVESA), fractional area change (FAC) and TAPSE; PR was assessed by Color Doppler, continuous-wave (CW) Doppler and derived parameters such as pressure half time (PHT), PR index, ratio of diastolic and systolic time-velocity integrals (DSTVI) of the main pulmonary artery. By speckle tracking we measured also RV global longitudinal strain (RVGLS) and right atrial strain (RAS). All the patients underwent CMR to assess PR and right ventricular volumes and ejection fraction (EF). Of these, 36 patients underwent cardiopulmonary exercise test (CPET).

Results

Fourty-six children (aged 13.7±3.0 years) were included. Echo derived RV areas correlated significantly with CMR RV volumes (r=0.72, p<0.0001). RVEDA >21.9 cm2/m2 had a good sensitivity (83.3%) and specificity (73.5%) to identify a RV end-diastolic volume (RVEDV) ≥150 ml/m2. No correlation was found among TAPSE, FAC, RVGLS and RVEF calculated by CMR nor between PHT, PR index and DSTVI and PR-RF. Only A' wave velocity showed a significant but modest correlation with CMR RF (r=0.57, p<0.0001). Flow reversal in pulmonary branches showed a sensitivity of 95.8% and a specificity of 59.1% to identify PR RF ≥35%. RVEF by CMR was preserved in all patients, while TAPSE was reduced in 78.2% and RVGLS in 60.8%. None of the CMR parameters correlated with peak Vo2. At the multivariate analysis RAS was the best independent predictor of peak Vo2 (p<0.0001).

Conclusion

In children, flow reversal in pulmonary branches identifies hemodynamically significant PR at CMR. RV area by echocardiogram is a valid first-line parameter to screen RV dilation. Our study suggests that, also for the RV, there is longitudinal systolic dysfunction in presence of preserved RV EF. RAS is the best predictor of peak Vo2 and should be added in the follow up of these patients.

Funding Acknowledgement

Type of funding source: None
Late gadolinium-enhanced cardiac magnetic resonance in repaired tetralogy of Fallot: characterization of the right ventricular arrhythmogenic substrate
Authors: Rodriguez Garcia J , Pijuan Domenech A , Pascual Gonzalez G , Dos Subira L , Perez Rodon J , Benito Villabriga B , Francisco Pascual J , Santos Ortega A , Subirana Domenech M , Miranda Barrio B , Gordon Ramirez B , Cuellar Calabria H , Valente F , Ferreira Gonzalez I , Rivas Gandara N
Published: November 2020

Introduction

Ventricular arrhythmias are one of the main causes of morbidity and mortality in patients with repaired tetralogy of fallot (rTF). These life-threatening arrhythmias are related to specific isthmuses of viable tissue between areas of scar and/or valve rings of the right ventricle (RV) that have been already described in the literature. Late gadolinium-enhanced cardiac magnetic resonance (LGE-CMR) has proven useful in characterizing the arrhythmogenic substrate in several heart diseases. Nevertheless, LGE-CMR evidence in patients with rTF is scarce.

Purpose

To compare the characterization of the ventricular arrhythmogenic substrate by means of LGE-CMR and electroanatomic mapping (EAM) of the right ventricle (RV) in patients with rTF.

Methods

Unicentric and observational study of consecutive patients with rTF who underwent LGE-CMR performed with 1.5T equipment and RV high-density voltage map, performed with multipolar catheter and completed with contact force-sensing catheter. The LGE-CMR segmentation was performed with dedicated software. The extent (area and percentage) and location of the dense scar (defined as <0.5 mV in EAM and <40% of the maximum intensity pixel in LGE-CMR) were compared, as well as the location of the isthmuses of viable tissue.

Results

Eight patients were included (45.7±10.4 years; 50% male). The extent of the scar was 20.7±13.6 cm2 (15.1±9.3%) by EAM and 21.7±8.8 cm2 (11.8±7.6%) by LGE-CMR. There was an absolute correlation regarding the location of the dense scar and the distribution of the isthmuses of viable tissue. The quantification of the dense scar evidenced a positive linear correlation between both techniques (area correlation: ρ = 0.71, p=0.047; percentage correlation: ρ = 0.88; p=0.004). The average time spent for the segmentation of the LGE-CMR with the dedicated software was 19.9±3.0 minutes.

Conclusions

Characterization of the RV arrhythmogenic substrate in patients with rTF with LGE-CMR is feasible. An absolute association regarding the location of the dense scar and the distribution of the isthmuses of viable tissue was observed when compared to the RV high-density EAM. In the same way, a statistically significant linear correlation in the quantification of the dense scar between both techniques was documented.

LGE-CMR and EAM of two patients with rTF

Funding Acknowledgement

Type of funding source: None
T1 mapping to assess hepatic and myocardial characteristics in children with single ventricle circulation
Authors: Beigh M , Pagano J.J , Noga M , Harake D , Olugbuyi O , Tham E
Published: November 2020

Background

Single ventricle (SV) palliation, culminating with the Fontan operation, results in passive systemic venous blood flow directly to the pulmonary circulation. Resulting inevitable hepatic venous congestion can lead to hepatic fibrosis. Previous studies suggest hepatic changes can occur prior to the Fontan completion. Besides fibrotic myocardial remodeling may lead to systolic and diastolic ventricular dysfunction, transmitting back pressure to the pulmonary system.

Purpose

To compare quantitative T1 cardiovascular magnetic resonance (CMR) imaging of the myocardium and liver between SV patients and controls, as a potential measure of myocardial and hepatic fibrosis.

Methods

Retrospective review of 16 SV patients with dominant single left ventricle (SLV, n=6) or single right ventricle (SRV, n=10), at various stages of palliation (pre-Glenn=6, post-Glenn=3, Fontan=7) underwent CMR with myocardial T1 mapping with the liver also in the plane of view. Biventricular patients found to have structurally normal hearts and normal cardiac function on CMR were used as controls (n=21). Native T1 times using a modified Look-Locker inversion recovery (MOLLI) approach in free-wall of the dominant ventricle at a mid-ventricular short axis in SV and the ventricular septum in controls and, a region of interest in the liver (avoiding any vessels) were measured in all patients. Median and inter-quartile ranges of continuous variables were compared between SV and controls using the Mann-Whitney U test.

Results

As compared to controls SV patients were (1) significantly younger, (2) had lower ejection fraction, (3) higher median myocardial T1, and (4) higher median liver T1. Also, there was no difference between SLV vs. SRV median myocardial T1 (1056 vs. 1065ms, p=0.43) or liver T1 (678 vs. 729ms, p=0.30)

Conclusion

Despite younger age, findings of increased myocardial T1 may suggest an element of myocardial fibrosis responsible for the ventricular dysfunction in this population, and that raised liver T1 may be an earlier marker of liver fibrosis, which warrants further study.

Funding Acknowledgement

Type of funding source: None
Prevalence of arrhythmias on the long term of adult patients with partial anomalous pulmonary venous connection treated surgically and conservatively: data from the SACHER registry and a French center
Authors: Touray M , Bouchardy J , Ladouceur M , Schwerzmann M , Greutmann M , Tobler D , Harald G , Engel R , Pruvot E , Blanche C , Sekarski N , Rutz T
Published: November 2020

Introduction

Partial anomalous pulmonary venous connection (PAPVC) is a rare congenital heart disease, characterized by one or some but not all pulmonary veins anomalously connected to the right atrium or a systemic vein. PAPVC is either an isolated shunt lesion or associated with an atrial septal defect (ASD). This study compares the arrhythmic outcome of patients treated surgically with those clinically monitored.

Method

Clinical, surgical, imaging and invasive data of PAPVC patients were retrospectively reviewed from 7 centers from the Swiss Adult Congenital HEart disease Registry (SACHER) and one French center.

Results

A total of 168 patients with PAPVC were identified. Most (77%) patients underwent surgery, while the remaining (23%) ones were treated conservatively with clinical monitoring. The operated group (OG) had a significantly higher number of associated ASD (N=106, 82%) (p<0.001) and a higher number of anomalous pulmonary veins leading to a mean Qp:Qs at 2.5±1.2 before surgery (table). Moreover, the majority of patients in the OG had cardiac symptoms (N=78, 60%) and were diagnosed at a significantly younger age (table). Mean age at operation was 27±20 years.

Age did not differ at latest follow-up (table). Holter recordings revealed a higher prevalence of arrhythmia in the OG (p=0.031), mainly of supraventricular tachyarrhythmias (table). The occurrence of ventricular non-sustained tachycardia and of bradyarrhythmia did not statistically differ between groups. Patients in the OG required more often medical treatment for arrhythmias: 12 (9%) needed electrophysiological study in the OG and none in the NOG (p=0.057). The amount of patients requiring a pacemaker implantation in the OG (11%) was significantly higher than that of NOG (0%) (p=0.039).

Conclusion

Patients after PAPVC repair present with a significant higher burden of arrhythmia than conservatively treated patients, either due to a larger shunt pre-operatively and/or as a late complication of the corrective surgery itself.

Funding Acknowledgement

Type of funding source: Private grant(s) and/or Sponsorship. Main funding source(s): Unrestricted grant from Actelion, Switzerland
Long term outcome of single ventricle physiology with pulmonary restriction not undergoing Fontan repair
Authors: Buendia F , Ramirez B , Gallego P , Oliver J.M , Montserrat S , Osa A , Miranda B , Rodriguez Puras M.J , Gonzalez A.E , Carbonell B , Meras P , Adsuar A , Ruiz Cantador J , Rueda Soriano J , Dos L
Published: November 2020

Background

Patients with univentricular physiology who do not complete the palliation to Fontan are a heterogeneous group with unknown long term outcome.

Aims

This study aimed at describing the clinical course and long-term survival of patients with SV physiology with restricted pulmonary flow that had not undergone a Fontan type of repair.

Methods

From the prospectively maintained databases of the adult congenital cardiac units of five tertiary referral centers, data from all SV physiology patients were obtained. Patients completing a Fontan type palliation or developing Eisenmenger physiology and segmental pulmonary hypertension were excluded. Baseline data were recorded on the first visit at adult congenital heart disease (ACHD) unit. The primary end point was death.

Results

101 patients (50.5% females) were identified. Mean age at end of follow up was 39.3±11.3 years. Of these, 45 (44.6%) were unoperated (group 1, restricted forward pulmonary flow with or without pulmonary banding), 38 (37.6%) had undergone a cavopulmonary shunt as a definitive palliation (group 2) and 18 (17.8%) had aortopulmonary shunts (group 3). The main diagnosis was double inlet left ventricle (DILV) (N: 52, 51.5%) and most of the ventricle was left (82.2%). The principal reason for not performing a Fontan repair was mean pulmonary artery pressure >18 mmHg. At initial visit at the ACHD unit patients were 32.2±11.1 years of age. 35% of the patients were in NYHA class III-IV, with no differences between groups. However, patients in group 2 had worse oxygen saturation (p=002) and higher haemoglobin (p=0.037). After a mean follow-up of 7.3±4.1 years, mortality was 20.8% (21 patients), being sudden death (7p, 6.9%) the most frequent cause. Patients in group 3 showed worse ventricular function (p=0.0001) and a trend to higher mortality that did not reach statistical significance (HR 2.7, CI 95% 0.91–8.14, P=0.07).

Conclusions

Patients with single ventricle physiology not undergoing Fontan repair are a population of high risk, with sudden death as main driver of mortality. Patients palliated with aortopulmonary shunts are prone to worse ventricular function and a trend to higher mortality.

Funding Acknowledgement

Type of funding source: Public hospital(s)
Left ventricular remodeling after transcatheter versus surgical therapy in adults with coarctation of aorta
Authors: Egbe A , Vojjini R , Banala K
Published: November 2020

Background

Transcatheter stent therapy is as effective as surgery in producing acute hemodynamic improvement in patients with coarctation of aorta (COA). However, left ventricular (LV) remodeling after transcatheter COA intervention has not been systematically investigated. The purpose of this study was to compare remodeling of LV structure and function after transcatheter stent therapy vs surgical therapy for COA.

Methods

LV remodeling was assessed at 1, 3 and 5-years post-intervention using: LV mass index (LVMI), LV end-diastolic dimension, LV ejection fraction, LV global longitudinal strain (LVGLS), LV e' and E/e'.

Results

There were 44 and 128 patients in the transcatheter and surgical groups respectively. Compared to the surgical group, the transcatheter group had less regression of LVMI (−4.6 [95% CI: −5.5 to −3.7] vs −7.3 [95% CI: −8.4 to −6.6] g/m2, p<0.001), less improvement in LVGLS (2.1 [95% CI 1.8–2.4] vs 2.9 [95% CI: 2.6–3.2]%, p=0.024) and e' (1.0 [95% CI: 0.7–1.2] vs 1.5 [95% CI: 1.3–1.7] cm/s, p=0.009) at 5 years post-intervention. Exploratory analysis showed a correlation between change in LVMI and LVGLS, and between change in LVMI and e', and this correlations were independent of the type of intervention received.

Conclusions

Transcatheter stent therapy was associated with less remodeling of LV structure and function during mid-term follow-up. As transcatheter stent therapy become more widely used in the adult COA population, there is a need for ongoing clinical monitoring to determine if these observed differences in LV remodeling translates to differences in clinical outcomes.

Funding Acknowledgement

Type of funding source: None
Increased prevalence of left ventricular diastolic dysfunction in adults with coarctation of aorta
Authors: Egbe A , Vojjini R , Banala K
Published: November 2020

Background

Left ventricular (LV) pressure overload and coronary artery disease, both of which are common in patients with coarctation of aorta (COA), are risk factors for LV diastolic dysfunction. Patients with COA may have aortic vasculopathy that can result in LV pressure overload even in the absence of hemodynamically significant COA. We therefore hypothesized that patients with COA (without hemodynamically significant COA) will have more LV diastolic dysfunction compared to controls.

Methods

Adult patients with COA (OA peak velocity <2 m/s) were matched 1:1 to patients without structural heart disease using propensity score method based on age, sex, body mass index, hypertension and blood pressure. The objective was to compare LV diastolic dysfunction (defined as E/e>2 standard deviations above age-specific normative values) between COA patients and controls.

Results

Of 204 COA and 204 control patients (age 35±12 years), COA patients had higher septal and lateral E/e' ratio (12±4 vs 9±4, p=0.009) and (10±3 vs 7±3, p<0.001), respectively. Compared to controls, the prevalence of LV diastolic dysfunction was higher in COA patients for every age group: <40 years (63% vs 13%, p<0.001); 41–60 years (87% vs 33%, p<0.001); age >60 years (82% vs 56%, p=0.076). Left ventricular mass index (LVMI) was the strongest determinant of E/e' (β=2.71 per 10 g/m2, standard error = 1.25, p<0.001).

Conclusion

LV diastolic dysfunction was common in COA patients, and the association with LVMI suggests that COA patients may have ongoing LV pressure overload in the absence of hemodynamically significant re-coarctation.

Funding Acknowledgement

Type of funding source: None
Determinants of left ventricular hypertrophy and dysfunction in adults with coarctation of aorta
Authors: Egbe A , Vojjini R , Banala K
Published: November 2020

Background

Several coarctation of aorta (COA) severity indices are used for timing of COA intervention, and to define severity of residual coarctation post-intervention. However, it is unclear how many of these COA indices are required in order to recommend intervention, and what degree of residual coarctation results in suboptimal recovery of the left ventricle (LV). Our aim was to assess the correlation between different COA indices and effects of chronic LV pressure overload (LV hypertrophy, diastolic and systolic dysfunction), and to determine the effect of residual coarctation on LV reverse remodeling after COA intervention.

Methods

COA severity indices were defined as Doppler COA gradient, systolic blood pressure, upper-to-lower-extremity systolic blood pressure gradient, aortic isthmus ratio. LV remodeling indices were defined as LV mass index (LVMI), LV global longitudinal strain (LVGLS), e' and E/e'. LV reverse remodeling was defined as the difference between indices obtained pre-intervention and 5-years post-intervention (delta LVMI, e', E/e', LVGLS).

Results

Of the COA indices analyzed in 546 adult COA patients, aortic isthmus ratio had the strongest correlation with LVMI (β±standard error −28.3±14.1, p<0.001), LVGLS (1.51±0.42, p=0.005), e' (3.11±1.10, p=0.014), and E/e' (−13.4±6.67, p=0.008). Residual aortic isthmus ratio also had the strongest correlation with LV reverse remodeling, and residual aortic isthmus ratio <0.7 was predictive of suboptimal LV reverse remodeling post-intervention.

Conclusion

Considering the known prognostic implications of LV remodeling and reverse remodeling in response to pressure overload, these results support the use of aortic isthmus ratio for timing of COA intervention, and for prognostication post-intervention.

Funding Acknowledgement

Type of funding source: None
Iron status, anemia and functional capacity in adults with congenital heart disease: a single center analysis
Authors: Karathanos A , Simon I , Brockmeyer M , Lin Y , Parco C , Krieger T , Schulze V , Hellhammer K , Kelm M , Zeus T , Wolff G
Published: November 2020

Background

Iron is essential to the mitochondrial energy production in cardiomyocytes and its depletion is negatively associated with symptoms, functional capacity, quality of life and outcomes in patients with heart failure – independent of anemia. The relevance of iron deficiency in adults with congenital heart disease however has not been evaluated to date, and we thus aimed to evaluate it in an all-comer cohort of patients with congenital heart disease in correlation with symptoms and functional capacity.

Methods and results

527 patient cases from one referral center over 2 years were evaluated concerning their iron status, anemia, functional capacity and ejection fraction of their systemic ventricle. 264 were female, 94 had a shunt lesion, 96 had left-sided obstructive lesions, 181 right-sided lesions, while 108 were considered to have complex lesions and 28 were cyanotic. The median age was 34 years, the mean BMI was 25.2±5 kg/m2, 429 patients had a normal ejection fraction and 34 moderately and severely depressed. 35 patients were classified as NYHA III, and 56 as NYHA II, while their functional capacity was evaluated via cardiopulmonary testing with a mean VO2max/kg of 22.6±6.5 and mean 69±17% of the expected. The mean serum iron concentration was 99.4±42.3 mcg/dL, their mean transferrin saturation was 27.36±13%, the mean ferritin concentration was 130.8±185 ng/mL, the mean soluble transfer factor was 1.3±0.66 mg/l and their mean Hemoglobin 14.8±2 mg/dL, while the mean MCV was 88±5.3 and the mean MCHC 33.7±1.4.

40 patients were anemic according to the WHO definition for anemia, in 28 of those patients that was already known. Iron deficiency according to stratified according to ferritin was present in 53 patients. However, when stratified according to the heart failure guidelines definition for iron deficiency 299 patients were found affected. Using the soluble transferrin receptor (sTfR) and sTfR-ferritin index iron deficiency was suspected in 10 additional individuals. Iron deficiency was associated with the ejection fraction (p=0.0001) - patients with moderately or severely depressed systemic ventricular function more often were diagnosed with iron deficiency (p=0.007)-, while it did not correlate with functional NYHA classification (p=0.622) or functional capacity (p=0.1 and 0.057). Iron deficiency was also not found significantly different amongst congenital defects but did correlate with all laboratory iron studies.

Conclusions

In this ambulatory population of adults with congenital heart disease we found an association of ejection fraction with iron deficiency, however no association of iron deficiency with functional capacity. The question arising is if a new definition of iron deficiency anemia in congenital heart disease similar to heart failure would be of clinical value.

Funding Acknowledgement

Type of funding source: None
Idiopathic pulmonary hypertension in Spanish pediatric registry: clinical characterization, management, and risk factors for survival
Authors: Playan Escribano J , Segura De La Cal T , Escribano Subias P , Mendoza Soto A , Labrandero C , Guillen Rodriguez I , Caicedo Cuenca L.M , Sabate Rotes A , Villagra Albert S , Lopez Ramon M , Conejo Munoz L , Izquierdo Riezu M.A , Inigo Martin G , Escriba Bori S , Del Cerro Marin M.J
Published: November 2020

Introduction

Pulmonary arterial hypertension (PAH) is a rare disease that affects the small pulmonary arteries, producing gradual obliteration of arterial lumen leading to the progressive increase in pulmonary vascular resistance and, ultimately, right ventricular failure and death. Idiopathic PH refers to patients without an identifiable underlying cause, although some of them may have a genetic cause. Our aim was to define the baseline characteristics, initial therapy and risk factors for survival of patients with idiopathic PAH.

Methods

The REHIPED registry is a Spanish, multicenter, observational and prospective registry on pulmonary hypertension in the pediatric population (<18 years of age) that began in 2007. 47 patients with idiopathic PAH were analyzed.

Results

The mean age at diagnosis was 4.5 years, and there was a similar proportion of both sexes. 64% of patients were in functional class I-II. The mean pulmonary arterial pressure was 47,3±16,3 mmHg, with mean pulmonary vascular resistance index of 12±8,3 WU·m2. Initial therapy was oral monotherapy in 67%, prostanoids monotherapy in 11% and combination therapy with prostanoids in 9%. At the end of follow up, 55% of patients had received prostanoids. The mean follow-up time was 8 years. The survival rates (free of death or transplantation) was 91% (95% confidence interval (CI): 79–97%), 82% (95% CI: 67–91%) and 70% (95% CI: 52–82%) at 1, 5 and 10 years. Lung transplantation was performed in 7 patients. The main factors influencing survival, after adjustment for pulmonary vascular resistance, were functional class III-IV (Hazard Ratio (HR) 4.6, p=0.022, 95% CI: 1.3–15.3) and diagnosis under the age of 2 (HR 4, p=0.031, 95% CI: 1.1–14.4).

Conclusions

Idiopathic Pulmonary hypertension in pediatric age affects both sexes equally and begins at a young age. Prostanoids were used in 55% of patients. The main factors influencing survival, after adjustment for pulmonary vascular resistance, were functional class III-IV and diagnosis under the age of 2.

Funding Acknowledgement

Type of funding source: Private grant(s) and/or Sponsorship. Main funding source(s): Janssen, Ferrer
Aetiology of sarcoidosis-associated pulmonary hypertension
Authors: Mathijssen H , Huitema M.P , Huitema M.P , Bakker A.L.M , Bakker A.L.M , Smits F , Smits F , Snijder R.J , Snijder R.J , Mager H.J , Mager H.J , Grutters J.C , Grutters J.C , Post M.C , Post M.C
Published: November 2020

Background

Pulmonary hypertension (PH) is a known complication of pulmonary sarcoidosis and its aetiology is unclear. The severity of PH may not correlate well with the degree of parenchymal disease; therefore other pathophysiological mechanisms have been hypothesized.

Purpose

The purpose of this study is to provide an insight in the different mechanisms of sarcoidosis-associated pulmonary hypertension (SAPH) and their characteristics.

Methods

We conducted a cohort study on all SAPH patients in our center. All patients were extensively analysed and discussed in a multidisciplinary expert team to determine the pathophysiology of PH case by case. Finally, patients were classified into different groups or a combination of groups based on the most likely mechanism of PH.

Results

Between 2010 and 2019, 41 patients with SAPH were identified. One patient was classified as isolated post-capillary PH, two patients as combined pre- and post-capillary PH and 38 patients as pre-capillary PH based on right heart catheterisation. In total, 17 patients were classified as PH solely due to chronic lung disease, one patient as suspected vasculopathy and one patient as compression of pulmonary vasculature. A combination of chronic lung disease with suspected vasculopathy (n=16), left heart disease (n=2), chronic pulmonary embolisms (n=1) or compression of pulmonary vasculature (n=5) was also found.

Conclusion

In sarcoidosis, different pathophysiological mechanisms contributing to the presence of PH can be identified. Extended analysis is helpful in order to differentiate between different mechanisms and could contribute to personalised therapeutic decision-making.

Venn diagram: classification of SAPH

Funding Acknowledgement

Type of funding source: None
In-hospital outcomes of catheter-directed thrombolysis in patients with pulmonary embolism
Authors: Hobohm L , Schmidt F , Gori T , Schmidtmann I , Barco S , Munzel T , Lankeit M , Konstantinides S.V , Keller K
Published: November 2020

Background and purpose

Catheter-directed treatment of acute pulmonary embolism (PE) is technically advancing. Recent guidelines acknowledge this treatment option for patients with overt or imminent haemodynamic decompensation, particularly when systemic thrombolysis is contraindicated or has failed. We investigated baseline characteristics and in-hospital outcomes of patients with PE who underwent catheter-directed thrombolysis (CDT) in the German nationwide inpatient cohort.

Methods

Data from hospitalizations with PE between 2005 and 2016 were collected by the Federal Office of Statistics (Statistisches Bundesamt) in Germany and included in this analysis. Patients with PE who underwent CDT were compared with patients receiving systemic thrombolysis, and those without thromboytic or other reperfusion treatment.

Results

We analyzed data from 978,094 hospitalized patients with PE. Of these, 41,903 (4.3%) patients received thrombolytic treatment (systemic thrombolysis in 4.2%, CDT in 0.1%). Among PE patients with shock, CDT was associated with lower in-hospital mortality compared to systemic thrombolysis (OR, 0.29, 95% CI 0.13–0.66, P=0.003). No intracranial bleeding occurred among PE patients with shock who received CDT. Among haemodynamically stable PE patients with right ventricular (RV) dysfunction (intermediate-risk PE), CDT also was associated with a lower risk of in-hospital mortality compared to systemic thrombolysis (OR, 0.52 [95% CI 0.38–0.70]; P<0.001) or no thrombolytic treatment (0.45 [95% CI 0.33–0.62]; P<0.001).

Conclusion

In the German nationwide inpatient cohort, CDT was associated with lower in-hospital mortality rates compared to systemic thrombolysis. Prospective controlled data are urgently needed to determine the true value of this treatment option in acute PE.

Funding Acknowledgement

Type of funding source: Public grant(s) – National budget only. Main funding source(s): This study was supported by the German Federal Ministry of Education and Research (BMBF 01EO1503).
Novel procedural method for balloon pulmonary angioplasty to treat chronic thromboembolic pulmonary hypertension
Authors: Minatsuki S , Kiyosue A , Kodera S , Saito A , Maki H , Hatano M , Takimoto E , Ando J , Komuro I
Published: November 2020

Background

The current strategy of balloon pulmonary angioplasty (BPA) for chronic thromboembolic pulmonary hypertension (CTEPH) is to dilate branches as many as possible without lung injury to normalize mean pulmonary artery pressure and oxygenation. The shape of guiding catheter is one of the important factors to achieve this strategy. However, conventional guiding catheters which are typically used for BPA are difficult to introduce into particular branches. The Ikari-curve left (IL) guiding catheter may be suitable for this purpose by adjusting its curves (Figure 1); however, its utility and effectiveness for lung injury are unclear.

Purpose

The aim of this study is to clarify utility and safety of IL guiding catheter for BPA.

Methods

We retrospectively analyzed 202 consecutive BPA sessions of 40 patients with CTEPH from November 2016 to October 2019 and divided them into two groups; IL group, in which we used IL guiding catheter; and non-IL group in which we used others. IL guiding catheter was used for branches of left lung which are difficult to introduce by conventional catheter prior Nov. 2018. After Nov.2018, we also used it for the same type branches of right lung. The occurrence of lung injury was determined by the presence of bloody sputum during the perioperative period. We compared success rate of introduction into target vessels and of occurrence of lung injury.

Results

The average age of enrolled patients was 60.3±14.4 year-old and female was 65%. There were 99 sessions in IL group. The median treated branches significantly differed between groups (IL group: 15 vs. non-IL group: 10, p<0.05). The lung injury rate tended to low in the IL group (4.0% vs. 11.7%, p=0.07). The IL group had more successful insertions into branches than did the non-IL group (right lung: middle lobe*, 84.4% vs. 57.5%; medial basal branch*, 46.9% vs. 7.5%; left lung: anterior ascending and descending branches, 82.9% vs. 70.8%; lingular branches*, 90.0% vs. 62.5%; anterior basal branch, 75.7% vs. 62.5%, * p<0.05).

Conclusion

IL guiding catheter can be introduced into branches that cannot be accessed via conventional guiding catheters and has potential to reduce the occurrence of lung injury. 2228 characters including space

Figure1

Funding Acknowledgement

Type of funding source: None
Accurate and efficient non-invasive strategy for early identification of chronic thromboembolic pulmonary hypertension after acute pulmonary embolism (InShape II study)
Authors: Klok E , Boon G.J.A.M , Ende-Verhaar Y.M , Bavalia R , Delcroix M , Dzikowska-Diduch O , Kurnicka K , Mairuhu A.T.A , Middeldorp S , Pruszczyk P , Ruigrol D , Verhamme P , Vliegen H , Vonk Noordegraaf A , Huisman M.V
Published: November 2020

Background

The current diagnostic delay of chronic thromboembolic pulmonary hypertension (CTEPH) after acute pulmonary embolism (PE) is unacceptably long exceeding 1 year, causing loss of quality-adjusted life years and excess mortality. Validated screening strategies to diagnose CTEPH earlier are lacking. Importantly, performing echocardiography in all PE patients for this purpose has a low diagnostic yield, is associated with overdiagnosis and is not cost-effective. Moreover, expertise in performing high-quality PH-dedicated echocardiograms may not be available outside expert centers.

Aim

To validate a simple screening strategy aimed at identifying CTEPH early in the course after acute PE, avoiding echocardiography if possible (Figure 1).

Methods

In this prospective, international, multicenter management study, consecutive PE survivors were managed according to the predefined algorithm starting three months after acute PE. All were followed for a total period of two years. The study protocol was approved by all local IRBs and all patients provided informed consent.

Results

424 patients were included across three European countries (Table 1). Following the algorithm, CTEPH was considered excluded in 343 (81%) patients based on clinical pre-test probability assessment by the “CTEPH prediction score”, evaluation of symptoms and application of the “CTEPH rule-out criteria” (Figure 1); only 19% was subjected to echocardiography. Only 1 of 343 patients managed without echocardiography was diagnosed with CTEPH, 10 months after initial PE, for a failure rate of 0.29% (95% CI 0–1.6%). Overall, 13 patients were diagnosed with CTEPH (incidence 3.1%), of whom 10 within 4 months after PE diagnosis.

Conclusions

The algorithm accurately ruled out CTEPH and avoided echocardiography in 81% of patients. The vast majority of CTEPH cases were identified early in the course of acute PE which is a considerable improvement compared to current clinical practice with an economic use of healthcare resources.

Figure 1. Study flowchart

Funding Acknowledgement

Type of funding source: Foundation. Main funding source(s): This study was supported by unrestricted grants from Bayer/Merck Sharp & Dohme (MSD) and Actelion Pharmaceuticals Ltd. F.A. Klok and G.J.A.M. Boon were supported by the Dutch Heart Foundation (2017T064).
De-escalation/discontinuation of oxygen-therapy and medication is feasible and safe in chronic thromboembolic pulmonary hypertension patients treated with balloon pulmonary angioplasty
Authors: Kimura M , Kohno T , Kawakami T , Shinya T , Hiraide T , Moriyama H , Kataoka M , Endo J , Itabash Y , Mitsushige M , Fukuda K
Published: November 2020

Introduction

There has been increasing evidence of the efficacy of balloon pulmonary angioplasty (BPA) in improving the hemodynamics, exercise capacity, and biomarkers of patients with chronic thromboembolic pulmonary hypertension (CTEPH). However, there is no consensus on the adjustment of home oxygen therapy (HOT) and pulmonary hypertension (PH)-specific medications after BPA in patients with CTEPH.

Purpose

We aimed to examine the current status of the de-escalation/discontinuation of HOT and PH-specific medications post-BPA, and clarify its effect on subsequent hemodynamics, biomarkers, and long-term clinical outcomes.

Methods and results

From November 2012 to July 2019, 134 consecutive CTEPH patients who underwent BPA at a single university hospital were enrolled (age; 63.6±13.4 years, female; n=87 [64.9%], WHO functional class [WHO-FC] II/III/IV; 33/92/9). Hemodynamic data, functional capacity (6-minute walk distance and WHO-FC), biomarkers (brain natriuretic peptide [BNP] and high-sensitivity troponin T [hs-TropT]), and respiratory function were evaluated at baseline, immediately and 1 year post-BPA. Clinical outcomes (all-cause death and heart failure [HF] admission) were also assessed during the follow up period. The total number of sessions was 6.3±2.0, and the number of target vessels was 14.3±2.0. Mean pulmonary arterial pressure decreased from 37.8±11.2 to 20.4±5.1 mmHg 1-year after BPA (p<0.01). The proportion of patients who required HOT (at rest or on exertion) and combination medical therapy (≥2 PH-specific medications) decreased 1 year post-BPA (from 59.0% to 7.5%, and from 41.8% to 10.4%, respectively; Figure). Among 79 patients who required HOT during daytime, 64 patients (81.0%) discontinued HOT just after BPA completion. Among 56 patients who required combination medical therapy, 29 (51.8%) discontinued combination therapy. Baseline factors influencing the continuation of HOT and combination medical therapy post-BPA were almost identical (i.e. lower exercise capacity and pulmonary diffusion capacity, and worse hemodynamics). Results showed that discontinuation of HOT and combination medical therapy did not affect the maintenance of improved hemodynamics and levels of BNP and hs-TropT, and no adverse clinical outcomes (all-cause death and HF hospitalization) were observed during 1 year post-BPA.

Conclusions

Most CTEPH patients discontinued HOT and PH-specific combination medical therapy after BPA, which was not associated with the deterioration of hemodynamics, functional capacity, or biomarkers. No adverse long-term outcomes were observed. De-escalation/discontinuation of HOT and PH-specific combination medical therapy after BPA is feasible and safe for patients with CTEPH.

De-escalation of HOT and medical therapy

Funding Acknowledgement

Type of funding source: None