bluuebunny/crossref_metadata_2025_split huggingface dataset:
P1691 A case of congenital pulmonary stenosis
Authors: Buttigieg L L , Yamagata K , Cassar J , Pace Bardon M , Balzan M , Borg A 
Published: January 2020

Abstract

Introduction
We present an unusual case of sequential right ventricular outflow tract obstruction (RVOTO) caused by pulmonary and subpulmonary stenosis, with formation of aortopulmonary collaterals.

Case presentation
We report a case of a 47 year old Senegalese man who presented with a three year history of shortness of breath on exertion which worsened over the past week. Cardiovascular examination revealed a parasternal heave, a systolic murmur loudest in the pulmonary area and no evidence of fluid overload.
A transthoracic echocardiogram revealed leftward septal deviation during systole due to right ventricular pressure overload, severe right ventricular hypertrophy (RV free wall end-diastolic thickness of 8mm) and normal systolic function (FAC 47%), a dilated right atrium and moderate tricuspid regurgitation with estimated maximum pressures of 112mmHg. On continuous-wave doppler of the right ventricular outflow tract (RVOT), there was a late peaking systolic flow with maximum velocity of 3.8m/s.
A cardiac computed tomography (CT) and cardiac magnetic resonance imaging (MRI) was performed which showed sequential stenoses of the RVOT; one at subvalvular level by an infundibular muscular ridge with an area of 0.7 cm2 in mid-systole and one at the level of a dome-shaped pulmonary valve with planimetered valve area of 0.5cm2, severe RV hypertrophy and normal RV ejection fraction. Aortopulmonary collaterals from proximal thoracic descending aorta were seen. The main pulmonary artery was shown to be dilated with no evidence of pulmonary artery branch stenosis. See image: In-plane phase contrast velocity flow mapping of the RVOT using a VENC of 80cm/s, showing aliasing at the level of the pulmonary valve (red arrow) and at the level of the infundibulum (white arrow).

Conclusion
Congenital pulmonary stenosis (PS) occurs in 8% of congenital heart defects. 80% of pulmonary valve stenosis is typically dome-shaped with commissural fusion whilst the remainder is of the dysplastic type. Congenital PS can be associated with RVOTO at the infundibular level secondary to reactive muscular hypertrophy. In our case, there is a discrete, circumferential muscular ridge at the level of the infundibulum resulting in sequential outflow tract obstruction.
PS with intact ventricular septum occurs in conjunction with varying degrees of right ventricular hypertrophy and elevated right ventricular systolic pressures. A dilated pulmonary artery is common in dome-shaped subtype of PS.
Survival into adulthood of severe PS is primarily dependent on the adequacy of pulmonary blood flow from systemic-to-pulmonary collateral arteries, which serve as an additive, or the only source of blood supply to the pulmonary arterial vasculature. These collaterals are usually seen in association with cyanotic congenital heart disease such as pulmonary atresia and tetralogy of fallot.
Abstract P1691 Figure. Sequential RVOTO
P1692 The past returns after 20 years: a case report
Authors: Habjan S , Cantisani D , Scarfo` I S , Guarneri M C , Semeraro G , Romeo F , Cicchini L , La Canna G 
Published: January 2020

Abstract

Introduction
Radiation therapy is one of the cornerstones of treatment for many types of cancer. These patients can later in life develop cardiovascular complications associated with radiation treatment. Late cardiovascular effects of radiation treatment include coronary artery disease (CAD), valvular heart disease, congestive heart failure, pericardial disease and sudden death. The most common sign of radiation-induced valvular heart disease is the calcification of the intervalvular fibrosa between the aortic and mitral valve.

Case presentation
A 71-year-old male patient with a history of Non-Hodgkin lymphoma treated with radiotherapy and chemotherapy 20 years ago, CAD, arterial hypertension, diabetes type II, dyslipidemia, obesity and currently smoking presented in the emergency room in our medical facility with acute pulmonary edema. The patient had unstable angina pectoris in 2018, the coronary angiography showed two-vessel disease with a non-significant stenosis of the left main coronary artery (LMCA) and 70% stenosis of the left anterior descending artery (LAD), for which he refused the percutaneous coronary intervention. At the same time, a transthoracic echocardiography (TTE) showed severe aortic stenosis and moderately severe mitral stenosis, at that time the patient refused the operation. After the initial treatment for pulmonary edema, TTE and transesophageal echocardiography (TEE) were performed and showed a tricuspid aortic valve with calcification of the cusps and a very severe aortic stenosis (planimetric aortic valve area 0.74 cm², functional aortic valve area 0.55 cm², indexed functional aortic valve area 0.25 cm²/m², mean gradient 61 mmHg, peak gradient 100 mmHg, stroke volume (SV) 69 ml, stroke volume index (SVI) 31 ml/m², flow rate 221 ml/s, aortic annulus 20x26 mm). The left ventricle was severely dilated (end diastolic volume 268 ml) with diffuse hypokinesia and severe systolic dysfunction (ejection fraction 32%). We appreciated a calcification of the mitral-aortic intervalvular fibrosa and the mitral annulus, without mitral stenosis but with moderate mitral regurgitation. The calcification of the intervalvular fibrosa suggested our final diagnosis of radiation-induced valvular heart disease with a severe aortic stenosis in low-flow conditions. The patient was successfully treated with transcatheter aortic valve implantation (TAVI).

Conclusion
Radiation-induced heart disease is a common reality and is destinated to raise due to the increasing number of cancer survivors. Effects are seen also many years after the radiation treatment. The exact primary mechanism of radiation injury to the heart is still unknown. The treatment of radiation-induced valve disease is the same as the treatment of valve disease in the general population.
Abstract P1692 Figure. Radiation-induced valvular heart disease
P1698 Mitral valve endocarditis in hypertrophic obstructive cardiomyopathy
Authors: Shirka E , Gjergo H , Avdullari O , Goda A 
Published: January 2020

Abstract

Introduction
Endocarditis complicating hypertrophic cardiomyopathy (HCM) is not commonly reported but occurs almost universally in patients showing evidence of outflow tract obstruction. The estimated cumulative 10 year probability of developing endocarditis in obstructive HCM is &lt; 5%. We report a rare case of mitral valve endocarditis in a young man with hypertrophic obstructive cardiomyopathy.

Case report
A 45 years old man was admitted to the emergency room after a 7 days history of weakness, thoracic discomfort, short of breath, cough and temperature up to 40 °C. He was treated with oral antibiotics in ambulatory setting, but symptoms persisted. He had no previous history of hypertension or known heart disease, family history of coronary heart disease and excessive smoker. On clinical examination, the patient was afebrile with a harsh systolic murmur. Initial blood tests showed normal inflammatory markers (C reactive protein 0.2 mg/l and fibrinogen 202 mg/dL) and normal blood sample. An ECG showed major left ventricular hypertrophy and abnormal lateral repolarisation. Transthoracic echocardiography showed localized septal hypertrophy (2.4 cm) and systolic anterior motion of the anterior mitral leaflet. Continuous wave Doppler ultrasound in the left ventricular cavity and outflow tract, had given a maximal predicted gradient of 73 mmHg. There was suspicion of vegetation on the anterior mitral valve leaflet and mitral regurgitation was quantified as moderate. Transoesophageal echocardiography confirmed the presence of vegetation on the anterior mitral valve leaflet, posterior leaflet prolapse and moderate mitral regurgitation. We found normal coronary arteries on coronary angio-CT. Treatment with intravenous antibiotics was initiated and the case was discussed with a microbiologist and a cardiothoracic surgeon.

Discussion
Infective endocarditis is a rare complication of hypertrophic cardiomyopathy (HCM). It is clear from morphological studies that systolic anterior motion of the anterior mitral valve leaflet is relevant to the pathogenesis of endocarditis. Pathogenesis of infective endocarditis in obstructive HCM can be explained by endocardium damage of the mitral or aortic valve, consequence of turbulence of blood flow during ejection and of the contact between the mitral anterior leaflet and the septum during systole as well as mitral regurgitation. Antibiotic therapy is the mainstay of the treatment. Surgery should be considered promptly whenever there is traditional indication (haemodynamic, emboli, persistent fever, abscess). Surgical procedure may consist of valve replacement or repair, and some authors reported relieve of outflow tract obstruction after mitral valve replacement which may be explained by the removal of systolic anterior motion of the mitral valve. Valve surgery combined with septal myectomy seems logical but requires great expertise and carries a higher operative mortality
Abstract P1698 Figure.
P1725 An easily dismissed suspect
Authors: Santos H , Almeida I , Miranda H , Santos M , Almeida L , Sa C , Almeida S , Sousa C , Chin J , Tavares J 
Published: January 2020

Abstract

Funding Acknowledgements
None

Introduction
Constrictive pericarditis (CP) is a rare etiology of heart failure. Is a chronic inflammatory process, characterized by scarring, fibrosis and pericardial calcification. Several etiologies can be associated with CP, namely infectious, idiopathy and post-surgical. In some cases, CP can extend to the myocardium and/or lead to cardiac dysfunction.

Case Report
58 years old woman, active smoking, referred to the emergency room for tachycardia on a routine electrocardiogram. History of 5 months of fatigue and dyspnea to ordinary activities, with progressive aggravation in the last month, associated with weight loss and episodic palpitations. Upon the physical examination presented jugular vein engorgement and peripheral edema. Admission electrocardiogram with atrial flutter at 150 of ventricular frequencies, without other findings. Thoracic radiography without variation (tenues pericardium enhancement), abdominal echography with moderate ascites. Blood work showed elevated liver enzymes, BNP of 230pg/ml, exclusion of infectious tuberculosis and autoimmune panel with isolated positive rheumatoid factor. Transthoracic echocardiography (TTE) at the emergency room show a non-dilated and global left ventricle hypokinesia, with reduced left ventricular ejection fraction (LVEF) and dilatation of the mitral valve ring in the genesis of moderate mitral regurgitation. Anticongestive and antiarrhythmic therapy started with rhythm conversion and clinical improve. Thoracic computed tomography scan reveals an extensive pericardial calcification. 2 months later TTE reveal a preserved LVEF, pericardial calcification, moderate mitral regurgitation, grade III diastolic dysfunction, respiration-related ventricular septal shift, increased of the mitral E-wave velocity with an E/A of 2.76, the peak mitral E-wave decreases 36% with the inspiration, dilated inferior vena cava without respiratory variation. Cardiac magnetic resonance imaging exposes a septal bounce and pericardial calcification, suggestive signs of constrictive pericarditis. The patient waits for cardiac catheterization for confirmation, being with anticoagulation, ACE inhibitors, beta-blockers and mineralocorticoid receptor antagonist medication, remaining in NYHA class I.

Discussion
Clinical suspicion of CP is key for its identification, since there is not a specific clinical manifestation and generally patients presented heart failure symptoms. Echocardiography is best tool for a clinical physician evaluate heart failure etiologies, and can be used with higher sensitivity and specificity associated to the correct criteria to the diagnosis of CP. Pericardiectomy is the standard treatment, however the moment of its performance is not well established, since patients can remain in NYHA class I several years and the surgical procedure have higher mortality rates.
P1731 Multiple floating thrombi in aortic arch leading to acute stroke: A case report and systematic review of literature
Authors: Mahmood M , Avula S , Patti D K , Khan M S , Singh H 
Published: January 2020

Abstract

Introduction
Around 10-15% of acute cerebrovascular events occur in young adults with age less than 40 years. Trans-oesophageal echocardiogram (TOE) is routinely performed to rule out any cardio-embolic source in patients with ischemic stroke. TOE has shown to significantly change management strategy in up to 16.7% of stroke cases. We report a rare case of recurrent stroke in a young female who was found to have multiple mobile thrombi in aortic arch on TOE. We also report systematic review of literature of similar cases to highlight the management strategies.

Case Presentation
A 38-year-old female presented with one-week history of right upper and lower extremity paresthesia along with headache. Physical examination was unremarkable for any focal neurological deficits at time of initial evaluation. She had pertinent history of acute stroke two years ago associated with non-occlusive left common carotid artery thrombus for which she was previously on anticoagulation with rivaroxaban. The anticoagulation, however, was stopped five months ago after repeat imaging revealed complete resolution of thrombus. Electrocardiogram showed normal sinus rhythm without any other significant abnormality. CT head showed no acute bleeding or infarct. MRI brain showed scattered infarcts in right cerebral hemisphere and left cerebellar hemisphere. CT angiography of head and neck showed multiple small nodular and linear pedunculated thrombi in distal arch of aorta (see Figure 2). TOE was then performed which confirmed two pedunculated and mobile echogenic masses, largest measuring 0.9 x 0.6 cm, in the distal aortic arch (see Figure 1). TOE did not show intracardiac source of embolism. Laboratory testing for thrombophilia was negative for Factor V and Prothrombin gene mutation and heterozygous positive for Methylenetetrahydrofolate reductase (MTHFR)-677T gene. She was also found to have elevated homocysteine levels. She was restarted on anticoagulation with rivaroxaban.
Discussion and Conclusion
Young patients with stroke should undergo detailed investigation to rule out hypercoagulable pathology and cardiovascular embolic source. This should also include multimodality imaging including TOE in the selected patients. During TOE examination, a particular attention should be paid for evaluation of aortic source of thombo-embolism. Our patient was heterozygous for MTHFR-66T gene which is associated with decreassed activity of MTHFR by 35 % with elevated homocysteine levels. Treatment of floating aortic thrombus is still controversial. Anticoagulation is suggested as primary modality by multiple authors who reported complete resolution of thrombus. Other option includes surgical thrombectomy. Our patient was treated with anticoagulation alone due to hypercoagulable state and small size of thrombi.
Abstract P1731 Figure.
P1732 Congenital absence of the inferior vena cava: challenge for catheter ablation of arrhythmias via the femoral vein approach
Authors: Prisecaru R S , Leatu C , Purcar O , Pitis C , Costache V 
Published: January 2020

Abstract

Introduction
Congenital anomalies of the inferior vena cava (IVC) are a well described entity. These anomalies include complete absence, partial absence or duplication of the IVC. They are seen more frequently in those with other congenital cardiac anomalies (0.6%–2%). This congenital condition can be discovered incidentally, or due to symptoms of associated congenital heart disease, asplenia, polysplenia, congenital kidney anomalies or deep venous thrombosis.

Case report
We report the case of a 48 years old man scheduled for pulmonary vein isolation due to persistent atrial fibrillation resistant to antiarhythmic drugs, symptomatic for palpitations and dyspnea on exertion. Transthoracic ecgocardiography showed mild left atrium enlargement, normal systolic LV function and no significant valve disease. The transoesophageal echocardiography revealed dilation of the left atrium and absence of thrombus in the left atrium or left appendage. Multi-slice spiral thoracic computed tomography (MSCT) was also performed before the procedure and revealed absence of the inferior vena cava and significant dilation of the azygos and hemiazygos veins with subsequent drainage into the superior vena cava. Abdominal CT showed also partial situs inversus. Pulmonary vein isolation (PVI) through femoral vein approach was cancelled and the patient was scheduled for remote magnetic navigation PVI through arterial approach.

Conclusions
Whether the absence of the IVC is an embryonic anomaly or the result of perinatal thrombosis with regression and subsequent congenital absence of the IVC, absence of the IVC creates difficulties for catheter ablation of arrhythmias via the femoral vein approach.
P1736 An unexpected cause of ventricular tachycardia in a young patient
Authors: Tomoaia R , Beyer R S , Puiu M , Dadarlat A , Fringu F I , Gurzau D , Bardos K T , Comsa D H , Simu G , Pop D , Zdrenghea D 
Published: January 2020

Abstract

Introduction
Hypoplastic coronary artery disease (HCAD) refers to a congenital underdevelopment of one or more coronary arteries, which are greatly reduced in diameter or length. It is a rare congenital abnormality which has rarely been documented in living patients and which may lead to myocardial infarction (MI) and sudden cardiac death (SCD).
This case describes an otherwise healthy young male, which was admitted in the emergency department (ED) for repeated sustained ventricular tachycardia (VT) and was thereby diagnosed with an isolated hypoplasia of the left circumflex coronary artery (CX). Investigations revealed scar-related tachycardia, due to a prior silent myocardial infarction caused by HCAD. These findings were suggested by echocardiography and coronary angiography and were later confirmed by magnetic resonance and 3D cardiac mapping.

Case Report
A 34-year-old male patient was admitted to the intensive care unit with recurrent sustained VT causing hemodynamic instability. After receiving two electric shocks and amiodarone in the ambulance, the patient became hemodynamically stable. There was no remarcable medical history besides a syncopal fall 5 years before and no symptoms prior to the current event. Initial physical examination, ECG and laboratory testing were normal. Echocardiography revealed a dilated left ventricle with akynesia of the lateral wall and a mildly reduced ejection fraction (EF = 45%), moderate mitral regurgitation due to restriction of the posterior mitral leaflet. A subsequent emergent coronary angiography was therefore performed, which showed no sign of atherosclerotic lesions, yet it revealed a hypoplastic CX, without compensatory collateral vessels supplying the lateral wall. Cardiac MR demonstrated delayed enhancement with transmural necrosis and no viability in the lateral wall of the LV, suggesting a long-standing MI. Therefore, the final diagnosis was scar-related VT, due to a prior silent MI caused by HCAD. The management of this patient included implantation of an ICD in the secondary prevention of SCD. Moreover, we performed an EPS, which identified a scar at the level of the lateral and inferolateral LV walls as a substrate for VT (electroanatomic 3D voltage mapping) and of the critical istmus (3D activation mapping). The next step was radiofrequency ablation of the critical isthmus, with no inducibility of the VT after the procedure. After 6-months follow-up, there was no recurrence of the VT.

Conclusion
Finding the cause of myocardial infarction in a young patient with VT can be very challenging. We emphasise the role of imaging for the diagnosis and management of these patients. HCAD is a rare congenital anomaly, which can lead to progressive fibrosis in the territory with poor blood supply, MI and SCD. Although it is a rare condition, it should be considered in young adults who present with MI and/or SCD.
Abstract P1736 Figure. Investigations
P248 Changes of atrial septum defect caused by posture during three-dimensional transesophageal echocardiography ( a case of Platypnea-orthodeoxia syndrome)
Authors: Takeuchi Y , Suzuki S , Tsuneyoshi H , Sakamoto H , Shimada T 
Published: January 2020

Abstract

Background
Platypnea-Orthodeoxia syndrome (POS) is a rare phenomenon which is characterized postural hypoxia due to the intracardiac shunt from right to left through a patent foramen ovale, an atrial septal defect or a pulmonary arteriovenous malformation. POS is often underestimated because the hypoxia by postural change is difficult to be detected. We evaluated three-dimensional transesophageal echocardiography (TEE) to diagnose POS in an elderly patient.

Case report
A 84-year-old woman suffered from repetitive syncope for several years. She undertook twelve leads electrocardiogram (ECG), screening transthoracic echocardiography (TTE), twenty-four hours ECG and treadmill stress ECG, however, the cause of syncope was not identified. After another syncope event happened, she was transferred to the emergency room. Then, the hypoxia caused by sitting position was pointed out for the first time.
Her hypoxia was improved by supine position and oxygen administration. TTE demonstrated no right heart enlargement. Shunt flow was suspected on her atrial septum; however, it was difficult to reveal it by TTE because of her obesity. Therefore, she underwent intravenous saline injection test. In the decubitus position, an intravenous injection of saline under Valsalva maneuver revealed the shunt flow from the right atrium to the left atrium. Her arterial oxygen saturation (SpO2) was 95%. In the sitting position, a visible shunt flow was observed, then her SpO2 dropped to 85%. By TEE, the shunt hole was found in the oval fossa of the atrial septum. TEE was evaluated by different positions. The atrial defect hole became larger in the sitting position (area 1.05cm2) than in the supine position (area 0.43cm2). As a result, the postural change to sitting revealed Platypnea-Orthodeoxia syndrome associated with ASD. The pulmonary blood flow/systemic blood flow ratio (Qp/Qs) was estimated at 1.6. After surgical ASD closure, she was discharged without any symptoms.

Conclusion
Unclearness of TTE and the absence of a right heart overload may lead to misdiagnosis of POS. If a syncope patient caused hypoxia in the only sitting position, detailed echocardiography should be needed to rule out a diagnosis of POS. This is considerably valuable case of three-dimensional TEE confirmed the changes of ASD size by postural change.
Abstract P248 Figure.