bluuebunny/crossref_metadata_2025 huggingface dataset:
Prevalence of coronary artery anomalies in young sudden cardiac death: insights from a prospective state-wide registry
Authors: Paratz E , Van Heusden A , Zentner D , Morgan N , Smith K , Ball J , Thompson T , James P , Connell V , Pflaumer A , Semsarian C , Ingles J , Stub D , Parsons S , La Gerche A
Published: October 2022

Background

Coronary artery anomalies (CAAs) have been previously implicated as a major cause of young sudden cardiac death (SCD), particularly in exercise-related SCD with a reported prevalence of up to 33%.

Methods

A state-wide prospective out-of-hospital cardiac arrest registry identified all patients aged 1–50 years who experienced an SCD and underwent autopsy from April 2019 to April 2021. Rates of normal anatomy, normal variants and CAAs were identified and circumstances and cause of death for patients with CAAs examined.

Results

Of 1,477 patients who experienced cardiac arrest during the study period, 490 underwent autopsy and were confirmed to have experienced SCD. Of these 490 patients, five (1.0%) had a CAA identified with three having anomalies of coronary origin and two having anomalies of coronary course. In no cases was the CAA deemed responsible for the SCD. In two cases, severe coronary disease and intra-coronary thrombus with histological evidence of acute myocardial infarction were identified, in the third critical coronary disease was found, the fourth had an unrelated thoracic aortic dissection and the fifth had cardiomegaly in the setting of illicit drug use. Of 27 patients who experienced their SCD during exercise, only one had a CAA identified (the patient with thoracic aortic dissection).

Conclusion

In this prospective cohort of consecutive young patients with SCD who underwent autopsy, CAAs occurred in 1.0% of patients and did not cause any deaths. The role of CAAs in causing young SCD appears to be less significant than previously hypothesised.

Funding Acknowledgement

Type of funding sources: Public Institution(s). Main funding source(s): NHMRC, NHF
Changes in birth prevalence of hypoplastic left heart syndrome over the past half-century in Sweden
Authors: Giang Kok W , Mandalenakis Z , Fedchenko M , Eriksson P , Rosengren A , Norman M , Hanseus K , Dellborg M
Published: October 2022

Background

Over the past decades improvements in surgical treatment and medical management have resulted in increased survival of children with congenital heart disease, where the most complex congenital heart diseases are now also suitable for treatment. Trends in live birth prevalence in one of the most complex congenital cardiac defects, hypoplastic left heart syndrome (HLHS) have not been reported.

Purpose

The aim of this study was to assess trends in live-birth prevalence of patients with hypoplastic left heart syndrome over the past 50 years in Sweden.

Method

The Swedish National Patient and Cause of Death register were linked to estimate the annual rates of HLHS among live-born infants from 1970 to 2017. Information on number of livebirths per year in 1970–2017 was obtained from Statistics Sweden.

Results

A total of 838 infants with HLHS were identified during 1970–2017. The annual number of live born patients varied from 8 to 40 cases with a prevalence of 5.4 to 14.5 cases per 100,000 live births in the 1970s, increasing to a peak of 33.9 cases per 100,000 in 1993 and then decreasing from the late 1990s to reach an average level of 11.2 cases per 100,000 live births in the period 2014–2017.

Conclusion

The live-birth prevalence for HLHS increased to nearly six times of that in the 1970s during the 1990s. However, a subsequent decline was observed. This evolution most likely reflects a combination of increased tendency in recording the diagnosis as improved treatment becomes available and a decline related to antenatal diagnosis and termination of pregnancies.

Funding Acknowledgement

Type of funding sources: Public grant(s) – National budget only. Main funding source(s): The Swedish state under an agreement between the Swedish government and county councils (the ALF agreement, grant numbers: 236611 and 917361), the Swedish Heart-Lung Foundation (grant number: 20180644).
Long-term mortality from birth in individuals with and without isolated congenital aortic stenosis: a nationwide, register-based cohort study
Authors: Magnusson C , Mandalenakis Z , Dellborg M , Eriksson P , Fedchenko M , Rosengren A , Skoglund K
Published: October 2022

Background

Congenital aortic valve stenosis (CAVS) accounts for almost 5% of congenital heart defects. Increased survival has been reported in patients with complex congenital heart defects over the last decade. However, data on the long-term outcomes of simple defects such as in isolated CAVS, are still limited.

Purpose

The present study aimed to investigate the risk of mortality in patients with isolated CAVS over the last half century in Sweden.

Methods

We used data from the Swedish National Patient and Cause of Death Register to identify patients with isolated CAVS born between 1970 and 2017. Each CAVS was matched with 10 controls without congenital heart disease from the Total Population Register, according to sex and birth year. The maximal follow-up time was from birth up to 47.5 years of age. We estimated mortality rates for cases and controls, and the 95% confidence interval (CI) of their ratio. Kaplan-Meier curves were used to estimate the survival for cases and controls under the follow-up.

Results

A total of 1,258 patients with isolated CAVS and 12,550 matched controls were included in the study. The median follow-up was 24.5 years (interquartile range (IQR):18.0) for cases and 25.5 years (IQR: 17.6) for controls respectively. The overall risk of mortality was more than three times higher for patients with isolated CAVS, compared with matched controls, hazard ratio (HR) 3.1 (95%, CI 2.1–4.5). Survival at 47.5 years of age was 94.1% for cases and 97.3% for controls. Within the first year of life, the mortality risk in patients with isolated CAVS was more than 19 times higher (95%, CI 8.8–43.6) than controls. By contrast, when diagnosed with isolated CAVS above the age of 10 years, the risk of mortality was 2.1 times higher (95%, CI 1.1–3.5) compared to controls.

Conclusions

The mortality in patients with isolated CAVS was more than three times higher compared with matched controls. The highest mortality was found in patients with a diagnosis of isolated CAVS diagnosed within the first year after birth, verifying the critical form of the disease. Furthermore, patients diagnosed with isolated CAVS later in life (hence clinically assumed as a mild form of valvulopathy) have a mortality risk twice as high as controls, highlighting the need for follow-up and lifetime management, even in the mild forms of valvulopathies.

Funding Acknowledgement

Type of funding sources: Public grant(s) – National budget only. Main funding source(s): Swedish state under an agreement between the Swedish Government and county Councils (ALF)
Risk of cardiac complications during pregnancy in patients with cardiac devices
Authors: Cantalapiedra Romero J , Pijuan-Domenech A , Galian-Gay L , Miranda-Barrios B , Serrano-Sanchez B , Goya-Canino M , Maiz-Elizaran N , Gordon-Ramirez B , Gonzalez-Fernandez V , Francisco-Pascual J , Rodriguez-Silva J , Pascual-Gonzalez G , Rivas-Gandara N , Dos-Subira L , Ferreira-Gonzalez I
Published: October 2022

Background

Pregnant women with cardiac devices (pacemaker or defibrillators [ICD]) are increasing in developed countries. Risk stratification in these patients is challenging, since presence of cardiac devices are not considered specifically neither in mWHO nor CARPREG II scales. In addition, the previous evidence is scarce and heterogeneous, based mostly on observational studies published years ago.

Purpose

To describe the intrinsic risk for cardiovascular complications of pregnant women with cardiac devices.

Methods

We designed a prospective study including all pregnancies under follow-up in our Heart Disease and Pregnancy Unit between January 2013 and December 2020, selecting those patients with an intracardiac device at the time of pregnancy. Following institutional review board approval, data were collected from medical records in order to classify the patients according to the type of heart disease and the mWHO scale. CARPREG II scale was calculated retrospectively from 2014 to 2018 and prospectively afterwards. All cardiological complications were identified during each pregnancy and puerperium, defined as the appearance of heart failure, arrhythmias or thrombotic complications.

Since our patients were classified in mWHO groups 2 or 2–3, we compared the incidence of complications between our cohort of patients with devices and the rest of the pregnancies of mWHO 2–3 patients without devices. We also included in the analysis patients with an unspecified mWHO. A mWHO IV patient was excluded from analysis.

Results

A total of 323 pregnancies were identified, of which 15 were in patients with devices (4.6%) and 135 in mWHO 2–3 patients without devices (41.8%). There were 9 pregnancies in 6 pacemaker carriers and 6 in 6 ICD carriers. The mean age was 34.3 years. None of them had other cardiovascular risk factors. A total of 2 cardiac complications were identified: one patient in the pacemaker group and another in the ICD group presented with a lead thrombosis at 25 and 30 weeks respectively. In both patients, anticoagulation with weight-adjusted enoxaparin was started. In the other hand, in the mWHO 2–3 patients without devices we identified 2 complications in the form of heart failure episodes: one during third trimester and another in puerperium.

When comparing our device patient group (n=14) with the mWHO 2–3 patients without devices (n=135), we observed a statistically significant higher incidence of complications in the device group (14.3% vs 1.5%, p=0.002).

Conclusions

Pregnant women with cardiac devices have a higher risk of complications than pregnant women with a similar risk profile according to the mWHO scale, mainly in the form of lead thrombosis.

Funding Acknowledgement

Type of funding sources: None.
Prevalence and subtypes of interatrial communications in 12,718 newborns
Authors: Blixenkrone Moeller E , Dannesbo S , Dehn A M , Pihl C , Sillesen A , Voegg R O B , Axelsson Raja A , Colan S , Mertens L , Vejlstrup N , Bundgaard H , Iversen K
Published: October 2022

Introduction

Patency of the oval foramen (PFO) and atrial septal defects (ASDs), i.e. interatrial communications, have been reported to be present in 24–92% of newborns. For isolated ASDs, the reported prevalence varies from 0.3–2.4 per 1000 livebirths. This broad range partly reflects the absence of a clear and universal clinical classification of interatrial communications. On this basis we have recently proposed a clinically applicable echocardiographic algorithm for classification of interatrial communications (figure 1), which proved superior to standard assessment by experts in terms of inter- and intraobserver agreement.

Purpose

To determine the prevalence of interatrial communications in newborns.

Methods

Echocardiograms (TTEs) from newborns (0–30 days) included in a population study (N>25,000) were assessed/analyzed for interatrial communications according to the new algorithm and categorized into three PFO subtypes and three ASD subtypes.

Results

TTEs from 16,420 newborns were analyzed; 3,694 (22.5%) were excluded due to suboptimal image quality and 9 (<0.1%) were excluded due to concurrent severe congenital heart disease. Of the remaining 12,718 included newborns (median age 12 days [8; 15], 48.1% female), an interatrial communication was present in 10,033 (78.9%) cases; 9,274 (72.9%) cases were classified as PFO while 759 (6.0%) were classified as ASD. In the ASD group, 368 (48.5%) had a defect size ≥4 mm, 364 (48.0%) had multiple interatrial communications, and 27 (3.6%) had the defect located in the lower 1/3 part of the septum (figure 2).

Conclusion

An interatrial communication was present in almost 4 out of 5 newborns. ASD was diagnosed in 6% of the newborns and the prevalence of PFO was 12 times higher than the prevalence of ASD. Follow-up studies of these children are expected to provide clinically useful information on the long term structural and hemodynamic impact of these well categorized ASD and PFO subtypes.

Funding Acknowledgement

Type of funding sources: Foundation. Main funding source(s): Candy's Foundation, The Danish Children's Heart Foundation
Single predictor of residual right-to-left shunt to optimally select patients for suture-mediated percutaneous patent fossa ovalis closure
Authors: Sgueglia G A , Gaspardone C , De Santis A , D'Ascoli E , Piccioni F , Iamele M , Giannico M B , Leonetti S , Gaspardone A
Published: October 2022

Background

In patients with patent fossa ovalis (PFO) and paradoxical embolism, percutaneous closure of the interatrial communication has been proven more effective than medical treatment only to reduce recurrent thromboembolic events. Percutaneous suture-mediated PFO closure has been proved to be a safe and advantageous alternative to device-based PFO closure, yet its overall success rate is slightly lower in unselected patients. Hence, it is extremely important to define baseline features associated with unsatisfactory results to appropriately select patients suitable for this technique.

Purpose

Systematic assessment of PFO anatomy in the largest series of consecutive patients undergoing suture-mediated percutaneous PFO closure to identify a single baseline predictor of significant residual right-to-left shunt (procedural failure) for optimal selection of patient to be submitted to this procedure.

Methods

Pre-procedural transesophageal echocardiogram (TEE) of 302 consecutive patients (113 men, 45±12 years) who underwent percutaneous suture-mediated PFO closure at a single institution were accurately reviewed to assess a series of parameters: presence and grade of spontaneous right-to-left shunt (RLS), PFO length and width, presence of atrial septal aneurysm and its maximal bulge, and presence of an embryonic or fetal remnant (Chiari network or Eustachian valve).

Results

At echocardiographic follow-up (3–6 months from the closure procedure), a residual RLS ≥2 was found in 60 (19.9%) patients. At multivariable analysis, only two anatomical variables measured at pre-procedural TEE were found as independent predictors of residual RLS ≥2 at follow-up: PFO maximum width (OR 1.89, 95% CI 1.16–3.40, p=0.019) and PFO minimum length (OR 0.58, 95% CI 0.35–0.88, p=0.018). An index based on the ratio of PFO maximum width to PFO minimum septal overlapping (W/SO) was found to be the most powerful predictor of RLS ≥2 at follow-up (OR 48.1, 95% CI 9.3–352.2, p<0.001). The ROC curve for the W/SO ratio was found to have an AUC of 0.84 (95% CI 0.75–0.93) and a cut-off value of 0.61 yielding a sensitivity of 80% and specificity of 78% with a negative predictive value of 94%.

Conclusions

Baseline pre-procedural TEE assessment provides essential information for the selection of patients most suitable to undergo suture-mediated PFO closure. Our results indicate that the ratio between the maximum amplitude of the PFO and the minimum overlap of the septa is the optimal single baseline index to optimally select patient for an effective percutaneous PFO closure.

Funding Acknowledgement

Type of funding sources: None.
Cardiac remodelling in adolescent athletes – sex differences progress through adolescence
Authors: Forsaa M , Bjerring A W , Haugaa K , Smedsrud M K , Sarvari S , Landgraff H W , Hallen J , Edvardsen T
Published: October 2022

Background

Cardiac remodelling beyond reference values is well-known in adult athlete's heart. Male endurance athletes are more prone to develop cardiac chambers and wall thickness above reference values. Cardiac remodelling is also described in adolescent athletes, but considered to be moderate compared to adults. However, few studies evaluate adolescent athlete's heart by paediatric echocardiographic reference values. Current paediatric reference values are sex-unspecific and do not include exercise data. The impact of sex and exercise on remodelling in adolescent athlete's heart remains unclear.

Purpose

We aimed to study the development of cardiac remodelling and potential sex differences in adolescent athletes. We hypothesized that male adolescent athletes would display greater degree of remodelling compared to female adolescent athletes.

Methods

Male (M) and female (F) adolescent cross-country skiers were recruited in a longitudinal cohort study. They were examined with echocardiography at age 12, 15 and 18. Data on exercise was collected at all examinations. We evaluated echocardiographic parameters by paediatric reference values (Z-score: number of standard deviations above estimated mean in the given body surface area). Echocardiographic measures were considered above upper reference value if Z-score was ≥2.

Results

Seventy-six athletes were examined at age 12 (48 M, 28 F), 48 at age 15 (34 M, 14 F) and 34 at age 18 (23 M, 11 F). Although Z-scores were within reference values at age 12 (Table 1), a subset of athletes displayed Z-scores ≥2 for end-diastolic intraventricular septum diameter (IVSd, M 13/48=27%, F 5/28=18%) and left ventricular posterior wall thickness (LVPWd, M 6/48=13%, F 2/28=14%). The male group demonstrated enlarged left ventricular mass (LVM) from age 15 (Figure 1). Males had greater left ventricular end-diastolic volume (LV EDV) from age 12. Additional sex differences were evident from age 15 for IVSd, LVPWd and LVM (Table 1). There was no sex difference in exercise hours. Both groups had normal myocardial function through the study period.

Conclusion

Cardiac remodelling beyond reference values was observed in athletes of both sexes from early adolescent age. Sex differences were evident from age 12 with further progression. Pathological values for LVM were more frequent in males. These findings suggest that sex differences in exercise-induced cardiac remodelling is more prominent in adolescents than previously reported. Sex and exercise history should be considered in questions of pathology.

Funding Acknowledgement

Type of funding sources: Public Institution(s). Main funding source(s): The South-Eastern Norway Regional Health AuthorityCentre for Children and Youth Sport, Norwegian School of Sport Sciences
A novel method to identify an intramural segment in interarterial anomalous coronary arteries on CT-angiography
Authors: Koppel C J , Verheijen D , Kies P , Egorova A D , Lamb H J , Voskuil M , Jukema J W , Koolbergen D R , Hazekamp M G , Schalij M J , Jongbloed M R M , Vliegen H W
Published: October 2022

Background/Introduction

An anomalous coronary artery originating from the opposite sinus of Valsalva (ACAOS) with an interarterial course can be assessed using Computed Tomography Angiography (CTA) for the presence of high-risk characteristics associated with sudden cardiac death. These features include a slit-like ostium, acute angle take-off, and degree of proximal luminal narrowing. However, no robust CTA criteria currently exist to determine the presence of an intramural segment.

Purpose

The aim of this study is to deduct a method to accurately identify an intramural course of interarterial ACAOS on CTA imaging.

Methods

All consecutive adult patients with an interarterial ACAOS that were evaluated at the two academic hospitals between January 2010 and July 2019 were screened for inclusion. Inclusion criteria were availability of a preoperative CTA-scan (0.5–1mm slice-thickness) and peroperative confirmation of the intramural segment. Using multiplanar reconstruction of the CTA, the distance between the lumen of the aorta and the lumen of the ACAOS (defined as “interluminal space” (ILS)) was assessed at 2mm intervals along the intramural segment (Figure 1).

Results

Twenty-five patients (64% female, mean age 46 years, 88% right ACAOS) were included. Analysis showed a mean ILS of 0.69mm±0.15mm at 2mm from the ostium. At the end of the intramural segment where the ACAOS becomes non-intramural, the mean ILS was significantly larger (1.27±0.29mm, p<0.001) (Figure 2). Interobserver agreement evaluation showed good reproducibility of ILS (intraclass correlation coefficient 0.77, p<0.001). ROC-analysis demonstrated that at a cut-off ILS of ≤0.95mm, an intramural segment can be diagnosed with 100% sensitivity and 84% specificity.

Conclusion(s)

The ILS is introduced as novel and robust CTA parameter to identify an intramural course of interarterial ACAOS. An ILS of ≤0.95mm is indicative of an intramural segment with 100% sensitivity and 84% specificity.

Funding Acknowledgement

Type of funding sources: None.
Strategies for thromboprophylaxis in Fontan circulation: a network meta-analysis
Authors: Van Den Eynde J , Possner M , Alahdab F , Veldtman G , Goldstein B , Rathod R , Hoskoppal A , Saraf A , Feingold B , Alsaied T
Published: October 2022

Introduction

The Fontan circulation is associated with an increased risk of thromboembolic events. However, the optimal strategy for thromboprophylaxis in this population remains unknown.

Purpose

The aim of this study was to compare the efficacy and safety of aspirin, warfarin, and non-vitamin K antagonist oral anticoagulants (NOACs) in a network meta-analysis using no thromboprophylaxis as a common comparator.

Methods

A comprehensive search was performed in Medline, Embase, Cochrane, and Scopus to identify relevant observational studies and randomized controlled trials published by February 14th, 2022. The primary efficacy outcome was thromboembolic events at the longest available follow-up, while major bleeding was a secondary safety outcome. Frequentist network meta-analyses were conducted to estimate the incidence rate ratios (IRRs) with corresponding 95% confidence intervals (CI) of both outcomes. Finally, ranking of treatments was performed based on probability (P) score.

Results

A total of 25 studies were included, encompassing 26,823 patient-years (aspirin: 11,948; warfarin: 11,354; NOAC: 346; no thromboprophylaxis: 3,175). When compared to no thromboprophylaxis, NOACs (IRR 0.12, 95% CI 0.03–0.41), warfarin (IRR 0.23, 95% CI 0.14–0.36), and aspirin (IRR 0.26, 95% CI 0.16–0.40) were all associated with significantly lower rates of thromboembolic events. However, the network meta-analysis revealed no significant differences in the rates of major bleeding for any of the strategies, although bleeding risk was numerically higher with NOACs and warfarin (NOAC: IRR 1.45, 95% CI 0.28–7.43; warfarin: IRR 1.38, 95% CI 0.41–4.69; and aspirin: IRR 0.72, 95% CI 0.20–2.58, Figure 1). Rankings, which simultaneously analyze competing interventions, suggested NOACs as the best strategy to prevent thromboembolic events (P score 0.919), followed by warfarin (P score 0.615), aspirin (P score 0.466), and no thromboprophylaxis (P score 0.001).

Conclusions

NOACs were associated with the greatest reduction in thromboembolic events with no significant difference in major bleeding when compared with warfarin and aspirin.

Funding Acknowledgement

Type of funding sources: None.
Unique cardiac remodeling in young adults born Small for Gestational Age with subsequent central obesity
Authors: Crispi F , Bernardino G , Sepulveda-Martinez A , Rodriguez-Lopez M , Prat-Gonzalez S , Pajuelo C , Perea R J , Caralt M T , Crovetto F , Gonzalez-Ballester M A , Sitges M , Gratacos E , Bijnens B
Published: October 2022

Background

Being born small-for-gestational age (SGA, 10 percent of all births) is associated with increased risk of cardiovascular mortality (1,2) in adulthood together with lower exercise tolerance (3), but mechanistic pathways are unclear. Central obesity is known to worsen cardiovascular outcomes, but it is uncertain how it affects the heart in adults born SGA.

Purpose

We aimed to assess whether central obesity makes young adults born SGA more susceptible to cardiac remodelling and dysfunction.

Methods

A perinatal cohort study including 80 young adults born SGA (birth weight below 10th centile) and 75 adults with normal birth weight (controls). Current waist-to-hip ratio was used as a surrogate of central obesity. Cardiac structure and function were assessed by cardiac magnetic resonance. Statistical shape analysis was used to study the regional geometric variability of the biventricular surfaces produced by central obesity and SGA, and synthetic surfaces representative of obese and non obese were generated for both SGA and controls.

Results

Figure 1 shows the superimposed representative surfaces of obese and non-obese according to our model, for controls (right column) and SGA (left column). Both SGA and waist-to-hip were highly associated to cardiac shape (F=3.94 p<0.001; F=5.18 p<0.001 respectively) with a statistically significant interaction (F=2.29, p=0.02), indicating a different cardiac remodelling due to obesity in SGA. While controls tend to increase left ventricular end-diastolic volumes, mass and stroke volume with increasing waist-to-hip ratio, young adults born SGA showed unique response with inability to increase cardiac dimensions or mass resulting in reduced stroke volume (both in absolute values and indexed by body surface area) and increased heart rate.

Conclusions

SGA young adults show unique cardiac adaptation to central obesity, which is associated with a decrease in stroke volume. Preventive strategies aiming to reduce cardiometabolic risk in SGA population may be warranted.

Funding Acknowledgement

Type of funding sources: Public grant(s) – EU funding. Main funding source(s): European Union Horizon 2020 Programme
Executive functioning of patients with congenital heart disease: 45 years after surgery
Authors: Pelosi C , Kauling R M , Cuypers J A A E , Utens E M W J , Van Den Bosch A E , Kardys I , Bogers A J J C , Helbing W A , Roos-Hesselink J W , Legerstee J S
Published: October 2022

Background

For children with congenital heart disease (CHD), subtle neuropsychological deficits have been reported. However, very little is known about executive functioning in their adulthood.

Purpose

To investigate the self- and informant-reported executive functioning in adults with CHD operated in childhood (<15 years old).

Material and methods

A cohort study of 194 patients (age 50 [46–54] years), operated in childhood between 1968 and 1980 for one of the following diagnosis: atrial septal defect (ASD), ventricular septal defect (VSD), pulmonary stenosis (PS), tetralogy of Fallot (ToF) or transposition of the great arteries (TGA), were evaluated 40–53 years after surgery. The “Behavior Rating Inventory of Executive Functions – Adult version” (BRIEF-A) questionnaire was used to assess self- and informant-reported executive functioning and compared to the general population.

Results

The CHD group did not show worse executive functioning compared to normative data. In addition, no significant difference was found between simple CHD (ASD, VSD and PS) and moderate/complex CHD (ToF and TGA).

Higher education and NYHA class 1 were associated with better self-reported executive functioning, while male patients and patients taking psychiatric or cardiac medications reported worse executive functioning.

Conclusions

Overall, our findings suggest reassuring outcomes regarding executive functioning in adults with CHD. However, these findings should be confirmed with neuropsychological assessment studies.

Funding Acknowledgement

Type of funding sources: Foundation. Main funding source(s): Thorax Foundation
The outcomes of Fontan operation in patients with hypoplastic left heart syndrome (HLHS) versus other forms of univentricular heart
Authors: Kula-Mazurek A , Bielecka-Dabrowa A M , Kepczynski L , Moszura T
Published: October 2022

Background

Single ventricle physiology is a rare condition but is highly represented in clinical practice due to a high prevalence of late complications and comorbidities. The Fontan operation has undergone considerable modification over the past 5 decades and is now in a superior hydrodynamic form.

Purpose

The purpose was to identify the potential differences in outcomes of Fontan operation between patients with hypoplastic left heart syndrome (HLHS) and with other indications to this treatment.

Methods and results

The study comprised 74 patients (20±6 years) after the Fontan operation (26 females) divided based on the indication to this operation as a group with HLHS (19 patients) and the group without HLHS (54 patients).

Patients with HLHS had significantly more often had a fenestration between the systemic venous return and the pulmonary venous atrium (89% vs 41%; p=0.001) which was closed in further observation in 89% in HLHS versus 39% in patients without HLHS, p=0.001. Regarding complications after Fontan operation, in HLHS group more often narrowings of pulmonary arteries requiring stenting occurred (74% vs 13%; p=0.001) and Fontan-associated liver disease (37% vs 9%, p=0.05). There were no differences in stenting of the lateral tunnel connection, fistulas, enteropathy, rhythm disturbances, peacemaker implantation, vascular complications, strokes between groups. HLHS patients more often were treated with antiplatelet drugs (26% vs 0, p=0.001), spironolactone (63% vs 23%, p=0.01) and angiotensin-converting-enzyme inhibitors (89% vs 44%, p=0.01).

Conclusions

HLHS patients have worse outcomes of Fontan operation. This group of patients more often needs fenestrations and regular controls towards pulmonary arteries narrowings and Fontan-associated liver disease.

Funding Acknowledgement

Type of funding sources: Public hospital(s). Main funding source(s): Polish Mother's Memorial Hospital - Research Institute
The influence of rivaroxaban compared to vitamin K antagonist treatment upon development of cardiovascular calcification the IRIVASC-trial
Authors: Stoehr R , Marx N , Schuh A , Brandenburg V
Published: October 2022

Vitamin K antagonists (VKA) such as warfarin or phenprocoumon are the mainstay of therapy for patients with several specific indications for long-term oral anticoagulant therapy (OAT). Due to interferences with Matrix-Gla protein, an important vitamin K-dependent local calcification inhibitor in cardiovascular structures, VKAs are theoretically linked to the development of increased cardiovascular calcification (CVC).

The IRIVASC trial was a multi-center, prospective, controlled, open, randomized, interventional clinical trial and included 192 patients with atrial fibrillation or pulmonary embolism and an indication for oral anticoagulation >12 months. In short, patients with an indication for OAT were randomized to receive either Rivaroxaban (n=96) or VKA (n=96) and followed up over 12 months. MultiSlice CT (MSCT) was performed at baseline and after 12-month follow up. The primary endpoint of the study was the progression of coronary artery and aortic valve (AV) calcification, as determined by MSCT (volume score).

Baseline demographic and laboratory values were comparable between the VKA and the Rivaroxaban groups (median age 72 vs 68, 73.6 vs 69.9 male). The main indication for anticoagulation was AF (95.4% vs 96.6%)

After 12-month follow-up complete data sets were available for 164 patients (86%) for coronary calcification and 67 patients for valvular calcification (per protocol analysis).

Overall, we found no differences in the primary endpoints. Development of coronary artery calcification was similar within the 2 groups as evidenced by similar developments of the coronary agatston score (804.8±1062.3 vs 946.7±1024.4, p=0.38), the coronary volume score (571.9±855.6 ml vs 791.7±820.1 ml, p=0.359) and the coronary mass score (143.2±195.9 mg vs. 172.2±190.8 mg, p=0.335) after 12 month. Similarly, there was no difference in the development of aortic valve calcification (AV) (AV Agatston score 441.6±711.9 vs. 352.6±751.2, p=0.621. AV volume score 388.2±589.0 ml vs 354.3±654.3, ml p=0.824 and AV Mass Score 75.7±135.1 mg vs 60.2±122.9, p=0.626 mg) (Table 1).

To our knowledge this is the first multicenter prospective, randomized trial to ever investigate the effects of VKA vs rivaroxaban treatment regarding the development of coronary and valvular calcification.

Our findings are clinically relevant as VKA will remain the mainstay of treatment for several conditions including mechanical valve replacement or antiphospholipid syndrome and hence include patients at cardiovascular risk. Our data point towards a comparable risk of overt CVC progression as detectable by CT-scans in patients irrespective of VKA or NOAK treatment.

Funding Acknowledgement

Type of funding sources: Private company. Main funding source(s): BAYER GmBH
Efficacy of congenital heart disease transition clinics in improving patient education
Authors: Ricci P , Bouchard M , Chong Zhiya C , Constantine A , Castro Meira V , Gatzoulis M , Haidu L , Dimopoulos K
Published: October 2022

Background

Less than one third of adolescents with congenital heart disease (CHD) successfully complete a structured transition to adult care. Patient education is at the centre of the transition process, however, data on the impact of transition visits are limited.

Purpose

To assess the efficacy of our transition clinic in educating adolescents with CHD about their cardiac condition in preparation for adult CHD care.

Methods

All patients seen in our transition clinic between April 2015 and January 2022 were included. Integrated care pathways designed by the Pan London Transition Consortium guided us in the assessment and education during each of the 3 pre-planned transition visits (“ready, steady and go” model). Data collected included demographic and clinical characteristics, and qualitative assessment of the following categories: understanding, naming and describing their cardiac condition, adherence to and involvement in medication management, self-reported anxiety and awareness of the importance of dental hygiene.

Results

A total of 611 patients were included in the study (age 15.1±2.3 years at the first visit, 48.1% female). 51.1% of patients attended 2 and 19.3% 3 transition consultations.

Most adolescents (52.5%) had CHD of moderate complexity, whereas 28.5% had simple defects and 19% severe complexity. Learning disability (LD) was present in 19% of cases, of which 35.3% were genetic syndromes (21.6% Down Syndrome, 8.6% DiGeorge Syndrome, 2.6% Noonan Syndrome, 1.9% Sotos Syndrome, 0.9% Asperger Syndrome, 0.9% Cornelia De Lange Syndrome).

The overall comprehension of their condition increased significantly in patients without LD at the second (good or excellent understanding: from 20.9% to 60.2%, p<0.0001; naming: from 19.8% to 50.2%, p<0.0001; describing: from 13.9% to 40.2%, p<0.0001) and third visit (understanding: 66.7%, p=0.043; naming: 60.4%, p=0.004, describing: 56.3%, p=0.002; Table 1). Patients with LD did not improve their knowledge over time in understanding, naming, and describing their condition between the first and second and second and third visits (all p>0.05). Adherence to and the involvement in medication management, the self-reported anxiety and awareness of the importance of dental care did not change over time (Figure 1).

Conclusions

A structured transition program is an important tool in educating and supporting the delivery of care for CHD adolescents and their families prior their transition into the adult care. Our transition programme had a positive impact on comprehension in the majority of children. Adolescents with learning difficulties are a group who may benefit from more intensive or individually tailored education during transition to ensure that they are supported to understand their health needs and empowered to make their own choices where possible.

Funding Acknowledgement

Type of funding sources: None.
Patients with congenital heart disease over the age of 60: experiences of a single tertiary centre
Authors: Kecskemeti D , Szabo B , Nagy Z S , Temesvari A , Nagy Z S , Szegedi M , Nyolczas N , Andreka P , Balint H
Published: October 2022

Background

We are currently facing a shift in adult patients with congenital heart disease (ACHD) to older and more complex patients. The growing population of senior patients with congenital heart disease (CHD) is prone to acquired comorbidities.

Purpose

Purpose of our study was to identify the main comorbidities in ACHD patients ≥60 years.

Methods

2726 patients had at least one visit at our ACHD centre between 2010 and 2021, 287 patients of them being 60 or older. Clinical data about the patients' last visit were obtained retrospectively from digital medical records. Based on the complexity of the CHD patients were classified as mild, moderate, and severe. Survival difference was analysed using the Kaplan-Meier method and the log rank test.

Results

Senior patients included in this study were predominantly female (73.2%) with the median age of 69 years. We included 287 patients with mild (47.4%), moderate (39.4%), and severe (13.2%) underlying heart disease. Median age at first visit in our centre was 60 years (IQR 54–67). Over the median follow-up time of 7.9 years (IQR: 3.2–13.2), 20 patients (7%) died with the incidence of 0.8 deaths/100 patient years. The most common complication in this populations were Heart failure (43.2%) and arrhythmia (12.5%). Meanwhile the highest prevalence of heart failure was seen in patients with Eisenmenger syndrome/Pulmonary Arterial Hypertension (84.8%), Tetralogy of Fallot patients had the highest prevalence of arrhythmias (77.8%). 51% of all patients had at least one cardiovascular risk factor (diabetes, hypertension, hyperlipidaemia, or chronic kidney disease). 42% of the patients underwent a non-coronary intervention over the age of 60. The highest mortality was seen in patients with severely complex lesions (p<0.002). As of the comorbidities, significantly higher mortality was observed in seniors with heart failure (p=0.001) or NYHA functional class III/IV (p<0.002).

Conclusions

Although the number of ACHD patients is growing constantly, the population of elderly patients with CHD is still mainly consists of patients with mild or moderate heart defects. Half of the patients had at least one cardiovascular risk factor by the age of 60. As expected, a significantly higher mortality was seen in those with severe heart defects.

Funding Acknowledgement

Type of funding sources: None.
Chronic thromboembolic pulmonary hypertension and mortality after venous thromboembolism: a nationwide population-based cohort study
Authors: Bonnesen K , Klok F A , Andersen M J , Andersen A , Nielsen-Kudsk J E , Mellemkjaer S , Sorensen H T , Schmidt M
Published: October 2022

Background

One of the ultimate long-term complications of venous thromboembolism (VTE) is chronic thromboembolic pulmonary hypertension (CTEPH). CTEPH is a condition defined as an increased mean pulmonary arterial pressure of at least 25 mmHg at rest and abnormal pulmonary artery resistance measured by right heart catheterization. To our knowledge, no study has compared mortality in VTE patients developing CTEPH to those who do not.

Purpose

We aimed to investigate the influence of CTEPH on long-term VTE mortality, by examining how receiving any pulmonary hypertension (PH) diagnosis following VTE influenced mortality.

Methods

We conducted a population-based cohort study of all adult Danish patients with an incident in or outpatient VTE diagnosis from 1995 through 2016. We excluded patients with a previous PH diagnosis. We defined CTEPH as receiving any PH diagnosis within two years following VTE. We categorized VTE by type (deep venous thrombosis [DVT] or pulmonary embolism [PE]) and provoking risk factors. We followed patients from two years after VTE until the first of death, emigration, or 31 December 2016. We calculated mortality rates and standardized mortality rate ratios (SMRs) of the association between PH and all-cause mortality and cause-specific mortality due to cancer and cardiovascular diseases. We also stratified the analyses by baseline presence of comorbidity (congestive heart failure, chronic pulmonary disease, and/or interstitial pulmonary disease).

Results

In 89,062 VTE patients alive two years after their VTE, 724 developed PH. The mortality rate per 1,000 person-years was 121 (95% confidence interval [CI]: 110–134) in patients with PH and 48 (95% CI: 47–48) in patients without PH. The SMR was 2.05 (95% CI: 1.77–2.37) for all VTE patients, 2.20 (95% CI: 1.53–3.16) for DVT patients, and 1.72 (95% CI: 1.47–2.01) for PE patients. The SMR was 1.79 (95% CI: 1.28–2.51) in VTE patients with a provoking VTE risk factor and 2.15 (95% CI: 1.83–2.53) in VTE patients without a provoking VTE risk factor. PH was associated with increased cause-specific mortality due to cardiovascular diseases (SMR=2.31, 95% CI: 1.75–3.06), but not cancer (SMR=0.94, 95% CI: 0.64–1.38). The SMR was 2.36 (95% CI: 2.06–2.71) in patients with comorbidity and 1.80 (95% CI: 1.45–2.24) in patients without comorbidity.

Conclusion

Development of PH, as a measure of CTEPH, within two years following incident VTE was associated with two-fold increased mortality in two-year VTE survivors. The increase in mortality was driven by cardiovascular causes.

Funding Acknowledgement

Type of funding sources: None.
Echocardiographic probability of pulmonary hypertension according to the old and the new definition: a validation study
Authors: D'Alto M , Di Maio M , Romeo E , Argiento P , Di Vilio A , Blasi E , Vergara A , Rea G , D'Andrea A , Golino P , Naeije R
Published: October 2022

Background

According to current guidelines, the diagnosis of pulmonary hypertension (PH) relies on echocardiographic probability followed by right heart catheterization. How echocardiography predicts PH recently re-defined by a mean pulmonary artery pressure (mPAP) >20 mmHg instead of ≥25 mmHg and pulmonary vascular disease defined by a pulmonary vascular resistance (PVR) >3 or >2 Wood units has not been established.

Methods

A total of 278 patients referred for PH underwent a comprehensive echocardiography followed by a right heart catheterization. Fifteen patients (5.4%) were excluded because of insufficient quality echocardiography.

Results

With PH defined by a mPAP >20 mmHg, 23 patients had no PH, 146 had pre-capillary and 94 post-capillary PH. At univariate analysis, maximum velocity of tricuspid regurgitation (TRV) ≥2.9 and ≤3.4 m/s, left ventricle (LV) eccentricity index >1.1, right ventricle (RV) outflow tract (OT) notching or acceleration time <105 ms, RV-LV basal diameter >1 and PA diameter predicted PH, whereas inferior vena cava diameter and right atrial area did not. At multivariable analysis, only TRV ≥2.9 m/s independently predicted PH. Additional independent prediction of PVR >3 Wood units was offered by LV eccentricity index >1.1 and RVOT acceleration time <105 ms and/or notching, but with no improvement of optimal combination of specificity and sensibility or positive prediction. The areas under the ROC curves for different cut-off values of TRV for the prediction of mPAP >20 mmHg, mPAP ≥25 mmHg, mPAP >20 mmHg + PVR >2 Wood units and mPAP ≥25 mmHg + PVR >3 Wood units are illustrated in Figure 1A–D.

Conclusions

Echocardiography as recommended in current guidelines can be used to assess the probability of re-defined PH in a referral center. However, the added value of indirect signs is modest and sufficient quality echocardiographic signals may not be recovered in some patients.

Funding Acknowledgement

Type of funding sources: None.
Implications of the North American 2021 Chest Pain guidelines in the diagnostic approach to patients with stable chest pain and low pretest probability of obstructive coronary artery disease
Authors: Lopes P , Albuquerque F , Goncalves P A , Presume J , Freitas P , Guerreiro S , Abecasis J , Santos A C , Saraiva C , Mendes M , Marques H , Ferreira A
Published: October 2022

Background

The North American 2021 Chest Pain Guidelines recommend not testing stable patients with low pretest likelihood of obstructive coronary artery disease (CAD), defined as pretest probability <15% using contemporary models (Class I recommendation). In selected cases among this subset of patients, coronary artery calcium (CAC) score is considered a “reasonable first-line test” (Class IIa). Despite some supporting evidence, the clinical implications of a widespread adoption of these recommendations remain unclear.

The purpose of this study was to assess the results of three different testing strategies for patients with pretest probability <15%: A) defer testing; B) perform CAC score and withhold further testing if = 0, and proceed to coronary CT angiography (CCTA) if >0; C) perform CCTA in all.

Methods

We conducted a two-center cross-sectional study assessing symptomatic patients with suspected CAD who underwent CAC score and CCTA. Patients with known CAD, suspected acute coronary syndrome, or symptoms other than chest pain or dyspnea were excluded. Pretest probability of obstructive CAD was calculated based on age, sex and symptom typicality. Obstructive CAD was defined as any luminal stenosis ≥50% on CCTA.

Results

A total of 2259 patients were screened, of which 1385 (61.3%) had pretest probability <15% and were included in the analysis (mean age 57±11 years, 79% women). Symptom characteristics were: 48% non-anginal chest pain, 26% atypical angina, 21% dyspnea, and 5% typical chest pain. Overall, the prevalence of obstructive CAD was 10.3% (n=142). In the 786 patients (56.6%) with a CAC score of 0, 8.5% (n=67) had some degree of CAD [1.9% (n=15) obstructive, and 6.6% (n=52) nonobstructive]. Among those with CAC >0 (n=599), 21.2% (n=127) had obstructive CAD. The results that would be reached with each of the 3 diagnostic strategies are presented in Figure 1. The number of patients needed to scan with strategy B (CAC as gatekeeper) vs. A (no testing) to identify one patient with obstructive CAD was 11, whereas the number needed to scan with strategy C (CCTA for all) vs. strategy B was 91.

Conclusions

Not testing patients with suspected CAD and pretest likelihood <15% would lead to missing obstructive CAD in 1 out of 10 patients. Using CAC as a gatekeeper in this subgroup would decrease the use of CCTA by more than 50%, at the cost of missing obstructive CAD in 1 out of 100 patients. These findings may be used to inform decisions on testing, which will ultimately depend on how much diagnostic uncertainty and missed diagnoses patients and their physicians are willing to accept.

Funding Acknowledgement

Type of funding sources: None.
Age-related differences in the effect of balloon pulmonary angioplasty on cardiac output in chronic thromboembolic pulmonary hypertension
Authors: Shimokawahara H , Kanezawa M , Matsubara H
Published: October 2022

Background

Balloon pulmonary angioplasty (BPA) has been accepted as an alternative for inoperable patients with chronic thromboembolic pulmonary hypertension (CTEPH). Although BPA improves most of the hemodynamic parameters, the improvement of cardiac output (CO) after BPA cannot be obtained in all the patients.

Purpose

This study was aimed to clarify whether BPA could improve CO or not. We also investigated the determinants for the improvement of CO after BPA.

Methods

We enrolled in consecutive 317 CTEPH patients treated with BPA in our hospital. All the patients were divided into three groups by generations (Younger group; age <60 years old, n=103, Middle-aged group; 60 ≤ age < 70 years old, n=100, Elderly group; 70 ≤ age, n=114). We retrospectively investigated the change of CO after BPA, including its determinants as heart rate (HR) and stroke volume (SV) among the three groups.

Results

Baseline mean pulmonary arterial pressure and six minutes-walk distance in the elderly group were lower than those in the younger group. Baseline CO was almost similar among the three groups. All the hemodynamic parameters, excluding CO, were improved after BPA in overall patients' analyses. The significant improvement in CO after BPA was identified only in the younger patients. Whereas HR decreased after BPA in all age groups, the improvement of CO and SV depended on each patient's generation, as shown in Figure 1.

Conclusions

It would be challenging to improve CO after BPA in all the populations, especially in elderly CTEPH patients. Adjuvant medical treatment in addition to BPA would be required in elderly patients.

Funding Acknowledgement

Type of funding sources: None.