bluuebunny/crossref_metadata_2025_split huggingface dataset:
P254 Congenitally corrected transposition of the great arteries with an ebstenoid tricuspid valve, aortic atresia, hypoplastic aortic arch and a dysplastic pulmonary valve
Authors: Rittey L A , Parry H , Willcoxson F 
Published: January 2020

Abstract

Funding Acknowledgements
No financial support received

Introduction
Congenitally corrected transposition of the great arteries (CCTGA) is a rare cardiac malformation accounting for around 0.5% of congenital heart disease. Common associations are ventricular septal defects (VSD), pulmonary stenosis and heart block. CCTGA associated with aortic atresia is very rarely described in the literature.

Description
We present a case of a term neonate weighing 2.5kg. Fetal echo had initially made the diagnosis at 21/40.
The baby was born in good condition and the postnatal echo confirmed the diagnosis of CCTGA. There were 2 secundum atrial septal defects, the sub-systemic tricuspid valve was ebstenoid with remarkably trivial regurgitation, there were multiple muscular ventricular septal defects with predominantly left to right flow, although flow appeared bi-directional in some views. The atretic aortic valve was anterior and to the left of the dysplastic, bicuspid pulmonary valve. The pulmonary valve leaflet motion was restricted with peak velocity of 2.4m/s and mild incompetence. The hypoplastic aortic arch filled retrogradely via the arterial duct. There was discrete coarctation with peak velocity of 2.2m/s down the descending aorta. The persistent left SVC drained to the coronary sinus.
Following several discussions there was agreement that the most appropriate management for him would be to receive palliative care. He was discharged home at a week of age and continued to have intensive support from his local hospice and the cardiac team, until he passed away peacefully at home at 16 days of age.

Discussion
The multidisciplinary team decided surgery was extremely high risk due to the anatomy described above particularly in the context of his dysplastic, bicuspid pulmonary valve, which would have provided systemic outflow if intervention had been undertaken.
Figure 1: 4 chamber view showing reverse offsetting of the atrioventricular valves, arch view showing a hypoplastic aortic arch with restrograde flow from the arterial duct, short axis view showing the semilunar valves and their relative positions, long axis view showing aortic atresia with retrograde flow into the ascending aorta
Abstract P254 Figure 1
P255 A fall from the tractor: a case report
Authors: Habjan S , Cantisani D , Scarfo` I S , Avitabile V , Romeo F , Guarneri M C , La Canna G 
Published: January 2020

Abstract

Introduction
Pulmonary artery aneurysms (PAAs) are rare and infrequently diagnosed clinical conditions. They can have congenital, acquired or idiopathic causes. PAAs can be asymptomatic or present with non-specific manifestations even at large diameters up to 70 mm. Rupture and dissection are the main life threating complications. The optimal treatment remains uncertain. There are no clear guidelines regarding the therapeutical approach.

Case presentation
A 78-year-old male presented at our medical facility to investigate further a pulmonary artery aneurysm discovered accidentally after a traumatic fall from a farmer tractor. He had no important chronic diseases, but in his medical history it was reported that he was excluded from the military service on top of a cardiac murmur, never investigated with an echocardiography exam. The fall from the tractor caused a fracture of the second and third costal ribs with hematoma and a commotional cranial trauma. At the thoraco-abdominal computed tomography (CT) scan conducted after the fall emerged an important fusiform dilatation of the principal pulmonary trunk and the left pulmonary artery (3.8 cm and 5 cm respectively). There was also a mild dilatation of the right pulmonary artery (3 cm), thickening of the pulmonary valve cusps and dilatation of the right cardiac chambers with flattening of the interventricular septum. Pulmonary embolism was excluded.
Transthoracic and transesophageal echocardiography revealed a dysplastic pulmonary valve, with thickened cusps and moderate stenosis with insufficiency. The jet of the right ventricular outflow tract was eccentric with an anterolateral direction and a correlated dilatation of the common pulmonary trunk (3.8 cm) and the left pulmonary artery (5 cm). The right ventricle appeared not dilated and the tricuspid regurgitation was mild with a normal systolic pulmonary artery pressure. The dilated pulmonary artery did not interfere with the flow in the left main coronary artery. We concluded that the pulmonary artery aneurysm was mainly due to the congenital pulmonary valvulopathy. The patient underwent successful pulmonary trunk, left pulmonary artery and pulmonary valve replacement with a bioprosthetic valved conduit.

Conclusion
Congenital pulmonary valvulopathy can remain clinically silent lifelong and is commonly discovered during exams performed due to other clinical circumstances. The eccentric jet caused by the valvulopathy can be the reason for dilatation of the pulmonary trunk and pulmonary arteries. Transthoracic and transesophageal echocardiography are competent tools for clarifying the cause of the pulmonary artery aneurysm and therefore are essential in the decision making regarding the treatment strategy.
Abstract P255 Figure. Transesophageal echocardiography images
P1810 Impact of pulmonary hypertension on survival in patients with degenerative aortic stenosis, prospective clinical trial
Authors: Mercea D , Ianos R , Pop C , Pop D , Zdrenghea D , Cozma A 
Published: January 2020

Abstract

Introduction
Aortic stenosis has become the most common degenerative valvular heart disease, due to aging of the population. It represents 43% of degenerative valvular disease.

Methods
We prospectively followed 196 patients with degenerative aortic stenosis, for a period of 6 years. Of these, 106 had mild aortic stenosis, 28 had moderate aortic stenosis and 62 patients had severe aortic stenosis. Of the 196 patients, 54 had mild pulmonary hypertension (PH), 22 had moderate PH and 7 had severe PH. The survival data were estimated by the Kaplan - Meier method and the logrank test. The Cox proportional- hazards regression was performed to assess the differences between the groups.

Results
During the follow-up period, of a total of 196 patients, 61 patients died. We studied if the presence of PH has an influence on survival in patients with aortic stenosis and we found out that the patients who had PH had reduced survival rates, compared with those who hadn"t. The survival rate at 5 years was lower in the group with mild PH compared with the group with normal pulmonary pressure ( p = 0.045, HR 1.84, 95%CI 1.01-3.36). In the group with mild PH, 20 patients (37.03%) died at 5 years, compared with 23 in the group without PH (20.35%). The survival rate at 5 years was also lower in the group with moderate PH compared with the group with normal pulmonary pressure ( p = 0.001, HR 5.82, 95% CI 3.02-11.22). At 5 years, 15 patients with moderate PH died ( 68.18% compared with 20.35 in the group without PH). Comparing the group with severe PH with the group with normal pulmonary pressure the results showed a reduced survival rate in those with severe pulmonary hypertension ( p = 0.139, HR 2.48, 95% CI 0.74-8.27). At 5 years, 3 patients with severe PH died (42.85% compared with 20.35 in the group without PH).

Conclusion
The presence of PH in patients with degenerative aortic stenosis has a negative impact on survival, highly statistically significant (p &lt;0.001, HR 1.03, 95% CI, 1.02-1.04). Given these findings, perhaps an increased attention should be paid to the treatment of the patients with PH compared to those without PH.
P1812 The effect of pre-procedural significant mitral regurgitation upon mortality after transcatheter aortic valve implantation
Authors: Soulaidopoulos S , Drakopoulou M , Oikonomou G , Stathogiannis K , Toskas P , Xanthopoulou M , Toutouzas K , Tousoulis D 
Published: January 2020

Abstract

Background
The presence of concomitant mitral regurgitation (MR) is a common issue in patients with severe aortic stenosis and negatively affects patient outcome. Available data regarding MR reduction due to aortic gradient reduction and left ventricular reverse remodeling after transcatheter aortic valve implantation (TAVI) are contradictory.

Purpose
To investigate the prognostic impact of both pre- and post-procedural MR in patients undergoing TAVI.

Methods
Patients with severe and symptomatic aortic stenosis stenosis [effective orifice area (EOA)≤1cm2] referred for TAVI at our institution were consecutively enrolled. Prospectively collected demographic, laboratory and echocardiographic data were retrospectively analyzed. Patients were stratified into two groups according to MR severity : ≤ grade 1 were defined as non-significant and ≥ grade 2 as significant. Change in MR was determined by comparison between baseline and 30-day echocardiogram. Primary clinical endpoint was all-cause mortality, as defined by the criteria proposed by the Valve Academic Research Consortium2.

Results
263 consecutive patients (136 men, mean age : 80 ± 7.5 years) were included in the analysis. Significant (grade≥2) MR was present in 65 (24,7%) patients, while 198 (75,3%) patients had mild or no ( ≤ grade 1) MR. Comparing the two groups, patients with significant MR had higher systolic pulmonary pressure (51.3 ± 14.6mmHg versus 42.8 ± 11.2mmHg, p &lt; 0.001), lower ejection fraction (47.4 ± 10.8% versus 51.2 ± 8.2%) and were more dyspnoic (New York Heart Association class IV 18.5% vesrus 2.5%, p &lt; 0.001). The primary clinical end point occurred in 63 (24%) patients during a follow-up period of 26.6 ± 26.8 months. Patients with significant pre-procedural MR displayed greater cumulative mortality (40% versus 18.8%, p = 0.001). Perioperative risk assessed by logistic EuroScore, NYHA class and pre-procedural MR were found to significantly associate to cumulative mortality in a univariate analysis. Performing a multivariable analysis demonstrated that preprocedural MR severity could independently predict cumulative mortality [OR 2.38, B = 0.869 (95% CI 1.2 – 4.6, p = 0.01)] (Figure).

Conclusion
Significant MR is not infrequent in patients undergoing TAVI and appears to independently associate with high increased all-cause mortality.
Abstract P1812 Figure.
P1817 The effect of permanent pacemaker implantation following transcatheter aortic valve replacement upon survival
Authors: Oikonomou G , Drakopoulou M , Soulaidopoulos S , Toskas P , Stathogiannis K , Xanthopoulou M , Toutouzas K , Tousoulis D 
Published: January 2020

Abstract

Background
Transcatheter aortic valve replacement (TAVR) is often followed by conduction abnormalities, leading to a permanent pacemaker implantation (PPI). Data regarding the clinical impact of PPI following TAVR is yet to be established.

Purpose
To determine the effect of PPI after TAVR on long-term survival.
Methods : Patients with severe and symptomatic aortic stenosis [effective orifice area (EOA)≤1cm2] referred for TAVI at our institution were consecutively enrolled. Prospectively collected demographic, laboratory and echocardiographic data were retrospectively analyzed. Patients were stratified into two groups according to the need for PPI after TAVR and were followed up postoperatively with clinical and echocardiographic assessment. Primary clinical endpoint was all-cause mortality, as defined by the criteria proposed by the Valve Academic Research Consortium2.
Results : In total, 276 patients were included (male : 48.9%, mean age : 80 ± 7.5years) in our study . Of these, 107 (38.8%) underwent PPI simultaneously or shortly after TAVR. The median follow-up period was 26.6 [min. 0, max 116] months. In this period, all-cause mortality showed no significant difference between patients with and those without PPI after TAVR (log-rank p = 0.862). Subgroup analysis also showed no difference in survival between patients with low ejection fraction (&lt;50%) and those with preserved (≥50%) receiving a permanent pacemaker after TAVR (log-rank p = 0.360). Including factors that were found to associate to PPI in univariate analysis (pre TAVR - ejection fraction, pulmonary artery systolic pressure and New York Heart Association functional class) in a multivariate model, pre TAVR pulmonary artery systolic pressure was found to be an independent predictor of peri-procedural PPI [Exp(B) : 0.974, 95% Confidence Interval : 0.953- 0.995, B= - 0.027, p= 0.015].
Conclusion : PPI following TAVR was not associated with survival at 26 months of follow-up, independently from the pre TAVR ejection fraction.
Abstract P1817 Figure.
P257 Multiple clinical manifestations of catecholamine secreting paraganglioma
Authors: Garibaldi S , Suma S , Coli S , Buratti S , Gaibazzi N 
Published: January 2020

Abstract
A 37 years old woman was referred to our hospital due to the onset since the previous day of vertigo associated with nausea and headache.
Her previous history revealed an hospitalization ten years before for Tako Tsubo Cardiomyopathy, during which the diagnosis of left temporo-jugular catecholamine secreting paraganglioma was made.
Subsequently, there were several hospitalizations for multiple cardiac manifestations, such as acute coronary syndrome during hypertensive crisis, acute pulmonary oedema, one relapse for TakoTsubo syndrome and then an episode of torsade de pointes (TdP) during hypokalemia and elongated QT.
The surgical removal of the mass was always rejected by the patient, and therefore, only radiotherapy was performed with significant reduction of the tumour dimensions. Moreover, alpha and beta adrenergic blockers were always administrated.
At the admission, she complained neurological symptoms, in particular balance disorder and dysmetria.
Brain Computed tomography (CT) was performed and it showed left posterior cerebellar infarction in late acute clinical phase, without haemorrhage. The patient underwent angiography that excluded cerebral artery dissection but showed acute occlusion of posterior internal cerebral artery. Echocardiogram showed moderate left ventricular hypertrophy with normal global and segmental kinetics, left atrial dilatation; besides, left ventricular apical thrombus was excluded.
Holter ECG monitoring showed very frequent polymorphic ventricular and supraventricular extrasystolic beats.
Urinary metanephrines were markedly increased. In particular urinary normetanephrine was up to 9 times the upper limit of normality (4874 ug/L, normal range 162-527 ug/24 h).
Since the surgical intervention was always refused, this case is a rare documentation of the several different cardiovascular clinical manifestation due to catecholamine secretion in a single patient. Paraganglioma could be a catecholamine secreting tumour that arises from cromaffin tissue of the sympathetic nervous system.
Clinical manifestations of paraganglioma are extremely variable leading to its designation as "great mimic". Adrenal tumours are associated with several cardiovascular diseases including LV hypertrophy, myocardial infarction, cardiac arrhythmias, heart failure and Tako Tsubo cardiomyopathy. Cerebral accident is a rare manifestation of adrenergic crisis with several possible aetiologies such as vasospasm or cardioembolic events. In our clinical case, considering the type and localization of the cerebral ischemic injury, the stroke is likely to have a cardioembolic etiology. Apical left ventricular trombus and PFO were excluded.
Therefore, in view of left atrial enlargement and the frequent supraventricular ectopy, even in the absence of documented arrhythmia, an undiagnosed episode of catecholamines induced atrial fibrillation was suspected, with subsequent cardioembolic event.
Abstract P257 Figure.
P638 Short term reverse remodeling and exercise capacity improvement in a patient with valvular heart failure treated with Sacubitril Valsartan
Authors: Mapelli M , Mantegazza V , Volpato V , Sassi V , De Martino F , Salvioni E , Mattavelli I , Fusini L , Vignati C , Paolillo S , Corrieri N , Alimento M , Magini A , Pepi M , Agostoni P 
Published: January 2020

Abstract

Background
Sacubitril/valsartan is a first-in-class angiotensin receptor-neprilysin inhibitor (ARNI) recommended in the guidelines to reduce morbidity and mortality in patients with symptomatic heart failure (HF) with reduced ejection fraction (HFrEF). Although the recent widespread use of the drug, data on left ventricle (LV) reverse remodeling and improvement in functional capacity (FC) under treatment are still lacking.

Case presentation
A 73 years old man with a known HFrEF was admitted to the hospital for clinical review due to progressive worsening dyspnea in the last 6 months (NYHA class III) with high NTproBNP values. Echocardiography showed dilated LV (EDVi/ESVi 137/98 ml/m2) with severe reduction in ejection fraction (EF), moderate/severe aortic incompetence, moderate functional mitral regurgitation. A maximal, ramp-protocol, cardiopulmonary exercise test (CPET) showed a moderate reduction in FC with signs of cardiogenic limitation. He was started on Sacubitril/Valsartan 24/26mg b.i.d. with progressive up-titration of the dose until a maximum dose of 97/103mg b.i.d. and without any other change in the therapy. ARNI was well tolerated without hypotension, worsening renal function or hyperkaliemia. After 3 months the echocardiography showed a reduction in LV volumes (EDVi/ESVi 112/72 ml/m2) with mild improvement in EF (from 28% to 34%) and increased FC, leading to a 56% reduction in estimated HF mortality at 2 years assessed through MECKI Score (See tab. 1 and Fig. 1). NTproBNP value was also reduced compared to baseline.

Conclusion
We present a case of a short term improvement in LV and atrium volumes and FC after 3 months of treatment with Sacubitril/valsartan in a patient with HFrEF. More studies are needed to assess LV volumes and CPET values response to ARNI.
Tab.1 Basaline 3 months Δ NYHA Class II III - MECKI Score (%) 5.12 2.23 -56-4% Peak VO2 /% of predicted) 60 72 +20% Maximal Work (W) 68 83 +22.1% Mitral regurgitation ++ + - eGFR (ml/min/1,73m2) 64 65 +1.6% Potassium (mmol/L) 4.26 4.20 -1.4% Aortic regurgitation +++ ++ - Clinical changes after the 3 months follow-up
Abstract P638 Figure. Fig. 1
P973 Predictive value of galectin-3 for cardiac remodelling in patients with acute heart failure
Authors: Lala I R , Darabantiu D A , Lungeanu D , Pilat L , Puschita M 
Published: January 2020

Abstract

Background and objectives
Heart failure is responsible for high mortality rates. Galectin-3 has been suggested as a potential heart failure development and progression mediator. This study aimed to show the relationship between galectin-3 and time evolution of left ventricular remodelling.

Methods
A total of 69 patients hospitalized with primary diagnosis of acute heart failure were enrolled and followed up for 6 months. For each, serum galectin-3 and echocardiographic parameters were measured at baseline and after 6 months.

Results
Mantel-Haenszel test showed highly significant association between pathological evolution of left ventricular end-diastolic diameter, left ventricular end-diastolic volume, relative wall thickness and increased or increasing galectin-3 levels over time (p &lt; 0.001). High baseline galectin-3 levels (&gt;16.5ng/ml) were associated with lower transmitral E peak velocity (68.59 ± 20.5; p = 0.021). Increased baseline galectin-3 levels (&gt;16.5ng/ml) were associated with systolic dysfunction expressed by lower rates of dp/dt ratio (p = 0.018) and raised systolic pulmonary artery pressures (p = 0.046). There was a association between increased baseline galectin-3 levels and type I diastolic dysfunction (E/A &lt; 1, p = 0.042) and this association tended to remain the same after 6 months.

Conclusions
Increased galectin-3 levels contribute to left ventricular evolutionary remodelling by its association with left ventricular chamber volume and diameter changes over time. Its levels also correlated with both systolic and diastolic dysfunction parameters.
P1488 Anomalous origin of left coronary artery from right pulmonary artery in association with scimitar syndrome
Authors: Aly S , Lizano Santamaria R , Yoo S J 
Published: January 2020

Abstract

Clinical Presentation
A full-term neonate was referred to our institution because of respiratory distress. CXR was significant for right lung hypoplasia and mild cardiomegaly. ECG showed normal sinus rhythm, right atrial enlargement, and right ventricular hypertrophy with no signs of ischemia.

Imaging Findings
The initial echocardiogram demonstrated PAPVD with the right upper pulmonary vein draining into IVC/RA junction with flow acceleration (mean gradient= 7 mmHg), moderate ASD, small muscular VSD with left-right shunting, moderate PDA with bidirectional shunting. Forward flow was seen in the proximal part of left main coronary artery (LMCA). RV systolic pressure was supra-systemic with a qualitatively moderately reduced RV systolic function.
The patient was taken to the catheterization lab where MPA angiography revealed an antegrade flow from the RPA into LMCA supplying both the anterior descending and the circumflex arteries. A selective injection within the scimitar vein showed drainage of the right lung into a vertical vein connecting with stenosis to IVC.
A follow up echocardiogram to re-examine the coronary origin revealed an anomalous origin of LMCA from proximal RPA; 3 mm distal to branch pulmonary artery bifurcation with mainly antegrade low velocity flow into LMCA and LAD. (Image 1)

Role of Imaging in Patient Care
- Imaging of the coronary origin in patients with ALCAPA can be challenging especially if the LMCA originates from RPA. Also, the presence of pulmonary hypertension might contribute to maintain coronary perfusion and lead to misinterpretation of the antegrade flow in LMCA and its branches.
- In certain situations, cardiac catheterization is essential to make the diagnosis of ALCAPA which prevented a potentially catastrophic outcome. Catheter intervention with a series of balloon dilations of the stenotic scimitar vein was successful in relieving the stenosis.
Summary/Discussion Points:
- Extensive review of the available literature revealed only three cases of Scimitar syndrome associated with ALCAPA. In all of these cases, the LMCA originated from the posterior sinus of MPA. Our case is the first to report ALCAPA from RPA in association with Scimitar syndrome. This presentation might have led to the initial misinterpretation of the echocardiography images.
- The presence of pulmonary hypertension in our patient maintained an adequate antegrade flow across the LMCA preventing significant coronary steal and signs of myocardial ischemia.
- The report highlights the challenges in making the diagnosis of ALCAPA with echocardiograms. Moreover, we discuss the role of cross-sectional and invasive imaging to rule out potential coronary arteries anomalies in patients with Scimitar syndrome, as this a rare although a very significant association that may have important implications in their outcomes.
Abstract P1488 Figure. ALCAPA origin from RPA