Good Systolic Function Research Articles

Iron Overload Cardiomyopathy

Iron overload cardiomyopathy is a condition of excessive iron accumulation in cardiomyocytes due to abnormal iron absorption or repeated blood transfusion. In the early stages, the patient may be asymptomatic with good ventricular systolic function. Iron deposit in ventricular cause dyspnea on effort due to left ventricular systolic dysfunction then in atrial cause atrioventricular block and supraventricular arrhythmic. For severe symptom due to dilated cardiomyopathy is characterized by left ventricular dilatation and risk of sudden cardiac death. Diagnosis of iron overload cardiomyopathy can be made if there is evidence of heart disease, the presence of iron overload (serum ferritin > 300 ng/mL and transferrin saturation > 55%) and cardiac siderosis with cardiac MRI T2 * < 20 ms as gold standard. Patient management involves lowering systemic iron levels and preventing iron entry into cardiomyocytes. Therapy with phlebotomy or iron chelation as indicated. Administration of calcium channel blockers and resveratrol antioxidant therapy may be considered to reduce morbidity and mortality due to cardiac siderosis.

Positron emission tomography rubidium-82 myocardial perfusion imaging in diagnosing uncommon paediatric acute coronary syndrome: a case report

BackgroundAcute coronary syndrome (ACS) is uncommon among the paediatric population with no guideline in the management in this age group up to this date. This case report aims to introduce the Positron Emission Tomography Rubidium-82 (PET Rb-82 MPI) as one of the non-invasive diagnostic tools in paediatric acute coronary syndrome.Case presentationPatient is an 11-year-old boy with underlying Nephrotic syndrome who was referred to our hospital with the chief complaint of chest pain. This was associated with raised cardiac enzyme and S–T depression on the posterior leads of electrocardiogram. On his arrival, we noted raised lipid profile as well as proteinuria and hypoalbuminaemia. He had frequent relapse of Nephrotic syndrome with the latest episode was three months ago. He has a strong family history of ischaemic heart disease whom his father had an episode of myocardial infarction at the age of 40 as well as a strong family history of hypercholesterolaemia. His father and paternal uncle were diagnosed with hypercholesterolaemia at their early age. He was arranged for Coronary Computed Tomography Angiography which subsequently followed by PET Rb-82 MPI. The PET Rb-82 MPI demonstrated a large area of stress induced ischaemia in the left anterior descending, left circumflex and right coronary artery territories. The left ventricular size is normal with good systolic function and normal wall motion. Global coronary flow reserve was still preserved at 2.07. Following the multi-disciplinary meeting, diagnosis of ACS was established. Patient was treated with dual anti-platelets, and he was then arranged for coronary angiography. The coronary angiography showed complete occlusion of the left circumflex artery from the proximal segment. There was a mild disease at mid segment of the diagonal artery with myocardial bridging. There was also a moderate disease from proximal segment of the dominant right coronary artery. Paclitaxel drug coated balloons were inserted into the diagonal and left circumflex arteries.ConclusionsThis rare case report on paediatric ACS highlights the diagnostic value of PET Rb-82 MPI as one of the non-invasive imaging tools in diagnosing paediatric acute coronary syndrome.

P134 A RARE CASE OF CARDIAC DIFFUSE LARGE CELL B LYMPHOMA: ECHOCARDIOGRAPHIC RESPONSE TO CHEMOTHERAPY

Abstract Introduction Diffuse large B–cell lymphoma (DLBCL) is the most common subtype of non–Hodgkin‘s lymphoma in adults. Rituximab and Anthracycline–based chemotherapy are the standard treatment, complete remission (CR) is achived in approximately 67% of patients. About one third of patients have refractory disease or relapse after standard therapy, mostly within 5 years of achieving CR. Clinical Case A 62–year–old patient, current smoker, without previous cardiovascular diseases presented to our ER for dyspnea and asthenia. Medical history showed GBC–DLBCL at third stage that achieved CR with R–CHOP therapy and intrathecal prophylaxis. The ECG documented sinus tachycardia with normal ventricular repolarization. Arterial blood gas analysis showed a condition of respiratory distress (pO2 71 mmHg, pCO2 27 mmHg). For suspicion of pulmonary embolism, chest CT with contrast was done showing a bulky mass extending from the right atrium to the right ventricle, also involving the pericardial sac (image 1). Transthoracic echocardiography showed a bulky mass (area 31 cm2) occupying almost the entire right atrium (5.9 x 4.4 cm) and extended, through the tricuspid valve, into the right ventricle, displacing the interventricular septum (image 2). Endomyocardial biopsy confirmed the diagnosis of GBC–DLBCL cardiac recurrence. Assumed the good systolic function of the left ventricle and the age of the patient, chemotherapy debulking was performed with Gemcitabine, followed by R–CHOP and MTX. One month after therapy post–treatment Total Body CT shows a good response with no evidence on echocardiogram of pathological tissue in the right ventricle (image 3). Conclusions Cardiac DLBCL is a rare tumor with few cases reported in the literature. There is currently no standard therapy, particularly for those who are unsuitable for high–dose chemotherapy a/o autologous stem cell transplantation. The multidisciplinary cardiological and hematological approach has proved to be fundamental these patient‘s therapeutic management.

Diazoxide-Associated Pulmonary Hypertension in Patient with Hyperinsulinemic Hypoglycemia

Background: Congenital hyperinsulinism (CHI) is the most common of persistent hypoglycemia in infancy characterized by inappropriate insulin secretion in the presence of hypoglycemia. Objective: This case report evaluates the effect of Diazoxide treatmentin inducing pulmonary hypertension in a patient with hyperinsulinemic hypoglycemia. Case findings: A full-term baby male, birth weight 4.3kg, was born at 41 weeks gestation by emergency caesarean section due to fetal distress born in Al-Dawadmi Hospital. He developed convulsions started postnatally loaded with phenobarbitone then shifted to AL-Yamammah hospital as lifesaving case to be admitted in Neonatal ICU. He was found to have hyperinsulinemic hypoglycemia and started on Diazoxide. The patient readmitted in Pediatric ICUat age of 2 month with hypoxic respiratory failure. After evidence of pulmonary hypertension by Echocardiography, Diazoxide was held at 2nd day of admission but he developed recurrent attacks of hypoglycemia required Octreotide with intermittent glucagon. After 14 days, Echocardiographic study repeated and showed findings of non-obstructive hypertrophic cardiomyopathy with good systolic function and no evidences of pulmonary hypertension (PH). Conclusions: Pulmonary hypertension may be developed during Diazoxide therapy. Pulmonaryhypertension was evaluated by echocardiography at regular intervals. This complicationresolved once diazoxide has been withdrawn.

Post-COVID-19 Infection; Paediatric Multisystemic Inflammatory Syndrome in an 8-Month-Old Infant

The coronavirus disease 2019 (COVID-19) associated with multisystem inflammatory syndrome in children and adolescents is an emerging challenge and has been described as a new dangerous childhood disease that is temporally associated with COVID-19. An 8-month-old infant presented with unrelenting fever of 2 weeks, associated non-purulent conjunctivitis, redness of the lips and tongue, as well as tenderness, swelling and desquamation of the palms and digits and diarrhoea. She also had associated irritability, refusal to feed and poor sleep. She had positive polymerase chain reaction (PCR) COVID-19 test 3 months earlier. However, a repeat PCR test was negative at presentation. She had raised erythrocyte sedimentation rate of 85 mn/h, good systolic function on echocardiogram with normal rhythm electrocardiogram. She was diagnosed with post-COVID-19 paediatric multisystemic inflammatory syndrome and treated with intravenous immunoglobulin, oral prednisolone and aspirin. Patient symptoms improved significantly after 72 h of treatment. Therefore, a high index of suspicion is required in all children presenting with unexplained fever.

Subaortic stenosis and Wolff-Parkinson-White syndrome: Clinical case and literature review

The association of a subaortic stenosis with a WPW syndrome is very rare yet so challenging due to the difficulty of the treatment. In order to describe this association, we report the case of a young man. A 39 yo Moroccan man with a history of recurrent sore throat, was referred to our care after a heart murmur was discovered during a pre-employment medical checkup. He beneficed from a physical examination, an EKG and a transthoracic cardiac ultrasound. The patient did not have any symptoms: no dyspnea or palpitations or fatigue or syncope. There was no family history of cardiac disease or murmur. Physical examination found a well-developed male in no distress, blood pressure at 140/80 mmHg, heart rate at 91 bpm, BMI = 21 kg/m 2 . Cardiac auscultation revealed a systolic murmur in the aortic area increased by the Valsalva maneuver. With no sign of heart failure. The results of the remaining physical examination were within normal ranges. The electrocardiogram revealed an intermittent WPW. Echocardiography was performed while the patient was in ventricular pre-excitation conduction revealed a subaortic stenosis diaphragm with an average gradient of 51 mmHg, a hypertrophic left ventricular wall, with good systolic function with an ejection fraction of 68%, the LV filling pressures were highs. A left atrium surface of 18.3 cm 2 , the aortic valve was tricuspide with a small regurgitation, the ascending aorta was dilated. Mitral valve was thin with normal opening. Right cavities were not dilated with normal function, we noted a small tricuspide regurgitation with systolic pulmomanary artery pression at 44 mmHg. Inferior vena cava was thin, and no pericardium effusion was found. Labs results came normal. The patient was put under small dose of beta-blockers and diuretics and a surgical treatment was discussed. The necessity to explore the WPW patients before treatment.

3-Month Enalapril Treatment in Pediatric Fontan Patients With Moderate to Good Systolic Ventricular Function

Many Fontan patients with and without systolic ventricular dysfunction are being treated with angiotensin-converting enzyme (ACE) inhibitors, despite its effectiveness remaining unclear. In the present study, we evaluated the short-term effect of enalapril on exercise capacity, vascular and ventricular function in pediatric Fontan patients with moderate-good systolic ventricular function. Fontan patients between 8 and 18 years with moderate-good systolic ventricular function and without previous ACE inhibitor treatment were included and were treated with enalapril for 3 months. During the first 2 weeks, the dosage was titrated according to systolic blood pressure (SBP). Exercise tests, ventricular function assessed by echocardiography, arterial stiffness measurements, and plasma levels of N-terminal pro-B-type natriuretic peptide assessed before and after a 3-month enalapril treatment period was compared. A total of 28 Fontan patients (median age 13.9 years, 6 to 15 years after Fontan operation) completed the study with a mean dosage of 0.3 ± 0.1 mg/kg/d. A total of 6 patients (21%) experienced a significant drop in SBP and 6 others (21%) experienced other adverse events. Enalapril treatment lowered the SBP (from 110 to 104 mmHg, p=0.003) and levels of N-terminal pro-B-type natriuretic peptide (from 80 to 72 ng/L, p=0.036). However, enalapril treatment did not improve exercise capacity, ventricular function, or arterial stiffness. In conclusion, short-term ACE inhibition has no beneficial effect in Fontan patients with moderate-good systolic ventricular function.

A CASE OF PULMONARY THROMOEMBOLISM OCCURRED IMMEDIATELY AFTER THE FIRST DOSE OF CHADOX1 NCOV- 19 VACCINATION.

In the recent studies, COVID-19 vaccines, especially Covishield has been associated with the reports of Vaccine-Induced pulmonary thromboembolism. pulmonary thromboembolism is a complication that can occur after the rst dose of the vaccine causing the thrombosis in veins at specic sites like lungs, Brain and Deep Veins and Arteries. It presents commonly as the Pulmonary Embolism (PE), within 7-10 days of vaccination. Patients taking vaccinations under the age of 45 presents at the high risk. Mortality rate in such post vaccination thrombosis is considered to be 50% in some cases. Thus, it is far more important to understand and identify the early signs and symptoms for early diagnosis, proper treatment and interventions. In the following case presented. A 29-year-old male patient was presented with the complaints of High Fever, Cough and Extreme Breathlessness after receiving the rst dose of ChADOx1 nCoV-19 Vaccination against the Corona Virus Disease 2019 (COVID-19). He had history of Exertional Dyspnoea for 2-3 weeks. He was diagnosed to have Pulmonary Embolism with severe PAH. 2D ECHO was done which showed Severe Pulmonary Hypertension Likely Pulmonary Thromboembolism, Good biventricular systolic function, Mild TR. CT Chest with Pulmonary Angiography was done which showed Eccentric lling defect seen in bilateral arteries at hila with extension in lower lobe segment branches- likely thrombus. CT Scan of Thorax (Plain & Contrast) was also done which showed bilateral small patchy ground glass haze with superimposed Reticular thickening mainly in Subpleural region. He was immediately thrombolysed with Intrapulmonary TNK after doing Check angiography. He was then managed with Oxygen support, NIV support, Inj. Fondaparinux, Anti biotics, Anti-coagulant, diuretics, Pulmonary vasodilators, Anti-hypertensive, and other supportive drugs. He responded well to the given treatment and was gradually weaned off NIV and oxygen support. Serial D-dimer, CBC, Chest X ray monitoring was done. Repeat CT chest was done in view of fresh lesions on Chest X ray which showed cavitatory lesion in Right mid zone with moderate Pleural effusion. Chest Physician consultation was taken and advice followed accordingly. He was discharged after 19 days, later he was then taken up for follow up every 2 weeks

CLINICAL PROFILE OF 2D ECHO CARDIOGRAPHY AT THE CARDIOLOGY DEPARTMENT OF CHL MULTISPECIALTY HOSPITAL AND RESEARCH CENTRE.

Introduction:Echocardiography is the basic and important imaging technique used for the diagnosing the various numbers of Cardiac Conditions. This helps clinicians for diagnosing a various number of clinical variations. This functional data is needed to care for patients with many pathologies and used to diagnose a number of diseases. Trans-Thoracic Echocardiography is the most common Echocardiography done in every Cardiology Department of Centre. The use of 2D Echo provides the proper and fast diagnosis and allows the clinicians to make further diagnosis. In this study, we focus on Current Clinical Prole of the distributed Age Groups along with risk factors and advised recommendations with comparison of Diagnosis. Methods: In this case study, total of 1447 patients were divided in 6 Age Groups, proled for the outcomes of the 2D Echocardiography. We aimed to determine the Current Clinical Prole with the Diagnosis, Risk Factors and Advised Recommendations in each age group in a Cardiac Intensive Care Unit (CICU) in CHL Multispeciality Hospital and Research Center, Chandrapur using a prospective observational study. Patients: All consecutive patients followed up for 2D Echocardiography at the Cardiology Department between April 2021 and August 2021 were included if they were aged more than 18 years. Patients eligible for the study were evaluated for the current prole of the patient, risk factors and Advised Recommendations in each Age group. Results: In the study conducted, the clinical prole had a good result in suggesting that most of the patients in all age groups had the diagnosis of Good biventricular systolic function, No RWMA, Normal chamber dimension & Valve function. Uncontrolled Sugars and Accelarated Hypertension made worse for patient's cardiac management. Some Results of 2D Echo were also affected because of the emergency in the CICU, for which 2D Echo was postponed later or after stability of the patient. Past Medical History had the signicant role. The clinical prole helped us understand the proper diagnosis and recommendations done

Determinants of exercise limitation in contemporary paediatric Fontan patients with an extra cardiac conduit

BackgroundAlthough various determinants of exercise limitation in Fontan patients have been studied, most research has been performed in patients who underwent different surgical procedures with differing haemodynamic characteristics. The aim of the current study was to evaluate non-invasively measured cardiovascular parameters and their influence on exercise performance in paediatric Fontan patients with an extracardiac conduit and moderate-good systolic ventricular function. MethodsFontan patients, between 8 and 18 years of age, with moderate to good systolic ventricular function and an extracardiac conduit were included. Exercise performance and cardiovascular assessment, comprising echocardiography, aortic stiffness measurement and ambulatory measurement of cardiac autonomous nervous activity were performed on the same day. Healthy subjects served as controls. ResultsThirty-six Fontan patients (age 14.0 years) and thirty-five healthy subjects (age 12.8 years) were included. Compared to controls, Fontan patients had reduced diastolic ventricular function and increased arterial stiffness. No differences were found in heart rate (HR) and cardiac parasympathetic nervous activity. In Fontan patients, maximal as well as submaximal exercise capacity was impaired, with the percentage of predicted capacity ranging between 54 and 72%. Chronotropic competence, however, was good with a peak HR of 174 (94% of predicted). Lower maximal and submaximal exercise capacity was correlated with a higher HR at rest, higher pulse wave velocity of the aorta and a lower ratio of early and late diastolic flow velocity. ConclusionContemporary paediatric Fontan patients have an impaired exercise capacity with preserved chronotropic competence. Exercise performance correlates with heart rate at rest, diastolic function and aortic stiffness.

The Role of LVOT-VTI Measurement in the Evaluation of Systolic Heart Function in Pulmonary ICU Patients

Introduction: The detection of cardiac systolic dysfunction is very important for well management of pulmonary critical care patients (PCCPs). However, there is a poor correlation between echocardiographic cardiac systolic function (CSF) parameters and it is not easy to obtain these parameters in PCCPs. Therefore, this cross-sectional observational study was planned for the detection of a more easily obtainable echocardiographic CSF parameter that is well correlated with other CSF parameters in PCCPs. Materials and Methods: Total 88 PCCPs were included. Demographic and clinical information and laboratory tests of all patients were recorded. The CSF parameters of the heart were obtained by transthoracic echocardiography with appropriate technique. LVOT-VTI (Left ventricular outflow tract velocity time integral), CO (cardiac output), EPSS (e point septal separation), Left ventricular EF (ejection fraction) and TAPSE (Tricuspid Annular Plane Systolic Excursion) as an indicator of CSF were tried to obtain from all patients. We also calculated sensitivity, specificity, positive and negative predictive values of LVOT-VTI<20 parameters to diagnose heart failure. Results: The mean age of the patients was 73±12, 40% were female, 38% were intubated and 52% had COPD. LVOT-VTI, EF, CO, EPSS parameters were obtained in 54(61%), 24(27%), 48(54%), 48(54%) patients, respectively. Decreased LVOT-VTI (<20 cm) was well correlated with decreased EF (<45%) (p=0.001, r=0.77), decreased CO (<5 L/dk) (p=0.03, r=0.64) and decreased TAPSE (<17 mm) (p=0.001, r=0.71). Also, there was good agreement between the EF and LVOTVTI measurements (Kappa:0.78, p:0.001). Sensitivity, specificity, positive and negative predictive values of LVOT-VTI<20 for heart failure were 58, 78, 84, 49, repectively. Conclusion: LVOT-VTI is a more easily obtainable and well correlated parameter, which can be used as an indicator of CSF in PCCPs. Clinical Significances: The authors believe that LVOT-VTI measurement has good correlation with other echocardiographic systolic function parameters, and its easy measurement in intensive care patients makes it a very useful test for cardiac systolic function evaluation.

P1466 Pacman heart documented by multimodality echocardiographic techniques

Abstract We report a case of a 78-year old female with hypertension, dyslipidemia, atrial fibrillation under warfarin therapy and previously diagnosed HFpEF. She also had a history of recurrent episodes of bilateral inferior limb acute ischemia requiring urgent embolectomy and one previous cardioembolic stroke. She had no history of ischemic heart disease. She was admitted to our center due to acute decompensated heart failure (NYHA class III). On physical examination there was evidence of pulmonary congestion with bilateral crackles, without heart murmurs. ECG showed AF rhythm, without any other significant changes. The transthoracic echocardiogram (TTE) showed moderate biventricular hypertrophy with apical predominance and good systolic function. A partial loss of myocardial tissue in the mid segment of the interventricular septum was noticed, with no left-to-right shunt on color Doppler, apparently without interventricular communication (figure A). For further elucidation of this finding a contrast-enhanced TTE was performed, revealing a serpiginous route through the septum to a small contained cavity within it (figure B). 3D TTE en face views additionally clarified the semilunar shape of this septal defect and its movement during the cardiac cycle, closing during systole and opening during diastole (figure C). At this point, clinical history and previous diagnostic exams were reviewed. A thoracic CT conducted 3 years before in another clinical context showed that the ventricular septal defect was already present, with similar characteristics (figure D). As doubts persisted about the existence of interventricular communication, cardiac catheterization with oximetry and ventriculography was performed and interventricular shunt was excluded. There was no evidence of coronary artery disease. Cardiac MRI was not possible due to lack of patient collaboration. Final diagnosis was a partial ventricular septal defect (PVSD), probably congenital. The patient was discharged under optimized medical therapy. PVSDs, which are rarely reported in the literature, are thought to be congenital (sporadic or familial) or a consequence of myocardial infarction. They have been described as "Pacman" heart due to the shape changes during the cardiac cycle, becoming slit-like or even absent during systole, like an opening/closing mouth, resembling the Pacman video game. Related complications include conduction disturbances, rupture and disturbed systolic function. We describe a rare case of a partial ventricular septal defect, documented by multimodality echocardiographic techniques: 2D TTE first showed an incomplete defect of the mid interventricular septum; contrast-enhanced TTE revealed its serpiginous route to a small cavity; 3D TTE en face views further illustrated its semilunar shape and characteristic movement during the cardiac cycle. Abstract P1466 Figure.

P1312 Implications of a large atrial roof thrombus

Abstract Funding Acknowledgements No funding Introduction Rheumatic heart disease is the most common cause of mitral valve stenosis. Rheumatic valve disease presents a strong predisposing factor to intracardiac thrombi formation in the left atrium, particularly when there is a background of atrial fibrillation. The predominant location for thrombus creation is the left atrial appendage, due to its morphology, and rarely the free wall or roof of the left atrium. In either case, the presence of intracardiac thrombi in the left cardiac cavities poses a severe risk factor for systemic embolism which can prove detrimental for the patient leading to increased morbidity and mortality. Transesophageal echocardiography remains the gold standard for screening of the left atrium. Case presentation We present the case of a 72 year old woman who was admitted to the Emergency Department of our Hospital because of palpitations and progressive weakness over the last month. She had a known history of rheumatic mitral valve stenosis and chronic atrial fibrillation under standard anticoagulant treatment with acenocoumarol. Methods - Results: On admission, the patient was hemodynamically stable without signs of heart failure. The electrocardiogram revealed atrial fibrillation with a ventricular rate of approximately 135 bpm. Chest X-Ray did not demonstrate any signs of pulmonary congestion. Blood tests were normal, except from a subtherapeutic INR 1.3. Transthoracic echocardiography depicted a suspicious large echogenous mass located on the posterior wall of the left atrium. Furthermore, there was severe mitral valve stenosis with calcification of the mitral annulus as well as reduced mobility of the leaflets (Mitral Valve Area ∼0.8 cm2, Mean gradient = 15 mmHg) and mild aortic valve stenosis. The left ventricle had normal dimensions and good overall systolic function. Systolic pulmonary pressure was estimated at 50 mmHg. Transesophageal echocardiography confirmed the presence of a large echogenous mass (2,6 x 2,9 cm) located on the posterior wall of the left atrium between the right and left pulmonary veins as well as another mass at the left atrial appendage. Contrast echocardiography ensued, which revealed no absorption of the contrast medium by the mass thus suggesting a lesion with no vascularity, compatible with thrombus. Furthermore there was diffuse slow flow within the left atrium (smoke) indicating blood stasis. The patient was treated with a cardioselective beta blocker and combination of aspirin and acenocoumarol with a target INR of 2,5-3 and was referred for cardiothoracic consultation. Conclusion/Discussion: We describe a rare case of moderate mitral stenosis with presence of a large thrombus with mobile parts in an unusual location. In this setting the indication for surgery (mitral valve replacement) is upgraded since there is increased risk of thrombus detachment and debilitating systemic embolism. Abstract P1312 Figure.

1641 Bicuspid pulmonary valve - an unusual cause of dilated pulmonary artery

Abstract Introduction Dilated pulmonary artery is a common finding with a variety of possible aetiologies. We present a case of unexplained dilated pulmonary artery that was associated with bicuspid pulmonary valve identified by CMR. Case Presentation A 67-year-old female underwent mitral valve repair for severe mitral regurgitation. She had presented with breathlessness, and had severe mitral regurgitation due to a myxomatous valve with bileaflet prolapse, confirmed with TOE. Echo showed: normal LV dimensions, LVEF 66%. RV size and systolic function normal. Coronary angiography was normal and pulmonary hypertension was not present on right heart catheterization. At postoperative follow up she was well and asymptomatic, with echo showing good LV systolic function, intact mitral valve repair, and normal systolic pulmonary artery pressures (36mmHg). New T wave inversion was identified across the precordial leads so a CMR was performed. CMR showed normal LV volumes and function with no wall motion abnormality or LVH. There was no fibrosis or infarction in the late phase after Gadolinium contrast. The RV was dilated (73mm diameter at base) with normal systolic function. The main pulmonary artery was dilated at 42mm. Flow mapping showed no evidence of shunt (Qp:Qs = 1:1) and the atrial septum was intact. Pulmonary valve function was normal but the valve was bicuspid in morphology. Discussion Bicuspid pulmonary valve is a rare finding. In our case this was identified by CMR and was associated with a dilated pulmonary artery. Bicuspid pulmonary valve is most commonly identified in the context of pulmonary valve stenosis. Isolated pulmonary valve stenosis is a rare finding; it is most often associated with other congenital defects like Fallot. Pulmonary stenosis has been associated with pulmonary artery aneurysm, attributed to hemodynamic changes caused by the stenotic valve. However as with bicuspid aortic valve it is recognized that the "post stenotic dilation" is part of the pathology of bicuspid valve and does not need the changed haemodynamics of a stenotic valve. During embryogenesis neural crest cells are necessary for the normal development of the semilunar valves, the septation of the outflow tract into the aorta and pulmonary artery, and the remodeling of the aortic arch. Any defect in this migration can cause abnormalities or aneurysm of the aorta or pulmonary artery together with semilunar valve defects. A study of 3,861 donor hearts dissected at the European Homograft Bank revealed only four cases (0.1%) of bicuspid pulmonary valves. Bicuspid pulmonary valve with normal function can be difficult to identify at echocardiography. It can be well visualized by CMR and this can be combined with an assessment of the pulmonary arteries. It should be remembered as a rarer cause of dilated pulmonary artery when other causes have been excluded. Figure A. Dilated MPA. B. Dilated RV. C. and D. Bicuspid pulmonary valve. Abstract 1641 Figure. Dilated MPA. Bicuspid pulmonary valve

P270 Complex cyanotic heart disease in adult patient with down syndrome accompanied by mild pulmonary hypertension

Abstract Funding Acknowledgements No funding Introduction Patients with Down syndrome and complex unrepaired cyanotic heart disease have impaired life expectancy. The clinical status of each patient depends both on the primary lesion type as well as the cummulative haemodynamic burden in the cardiovascular system. Assessment of an adult patient with complex congenital heart disease that has not been surgically repaired presents several challenges since initial haemodynamic parameters and compensatory mechanisms have to be taken into account. Case presentation A 43 year old male patient with known history of Down syndrome and no regular follow up presented to the Outpatient Department of our hospital to undergo pre-operative control for minor soft tissue surgery. The patient exhibited typical morphological features of Down syndrome as well as central cyanosis and digital clubbing. On auscultation a holosystolic type murmur 3/6 on the 3rd left parasternal border was audible. His blood gases revealed hypoxia and mild metabolic acidosis. Electrocardiogram displayed right axis deviation and signs of right ventricular hypertrophy. Transthoracic echocardiography demonstrated a large perimembranous ventricular septal defect (VSD) with normal anatomy and function of the aortic valve and no overiding aorta. Of note was excessive infundibular subpulmonary stenosis which resulted in a functionally double chambered right ventricle. Left ventricle was of normal diamater with good overall systolic function while the right ventricle was non-dilated with significant hypertrophy. Intracavitary gradient was estimated at 60 mmHg. Continuous Doppler showed mild mitral regurgitaion and mild tricuspid regurgitaion with estimated systolic pulmonary pressure of 40 mmHg. Bidirectional flow with low velocities at the level of the defect was recorded. Inferior vena cava was mildly dilated with normal respiratory variation. Discussion/ Conclusion: The natural history of large VSDs results in progressive dilatation of the right ventricle, the left ventricle and the left atrium with gradually increasing pylmonary vascular resistance and development of pulmonary hypertension, followed by right ventricular hypertrophy, flow reversal and Eisenmenger syndrome. However when RVOT obstruction at any level coexists, significant right ventricular hypertrophy develops and the incresed right ventricular systolic pressures acts protectively by minimizing the shunt at the VSD level. Thus a restrictive VSD due to a functionally double chambered right ventricle created a very fragile balance; a cyanotic heart disease with mild bidirectional flow depending on preload and afterload conditions that survived to adulthood. Abstract P270 Figure. Complex unrepaired CHD

TCTAP A-089 The Era of “Just Stent Them” Is Surely Gone.

To assess the impact of intravascular imaging and the determination of blood flow perfusion on the results of quantitative coronary angiography (QCA) stenosis assessment. Male, 59 y.o. STEMI 2 months ago (RCA 2 ZDES). Good LV systolic function. Recurrence of symptoms. Angina on middle exertion (2-3

The effect of telmisartan on the ventricular systolic function in dogs with experimental supraventricular tachyarrhythmia.

Maintaining a good ventricular systolic function is important in the long-term therapy of dogs with supraventricular tachyarrhythmia (SVTA). The objective of this study was to evaluate the inhibitory effect of telmisartan on myocardial injury and the resulting ventricular systolic dysfunction in a canine model of SVTA. A total of 14 dogs were randomly assigned to a Telmisartan (oral telmisartan, 1.0 mg/kg daily, n=7) or a Control (no drug administration, n=7) group; the duration of rapid atrial pacing (RAP) was 3 weeks for both groups. The cardiac troponin I (cTnI) concentration in the Control group was significantly increased after 3 weeks compared to that before RAP initiation (baseline), but no significant difference was observed in the Telmisartan group. Moreover, the cTnI concentration at 3 weeks was significantly lower in the Telmisartan group than in the Control group. The left ventricular fractional shortening was significantly decreased at 3 weeks compared to that at baseline in both groups. However, fractional shortening at 3 weeks was significantly higher in the Telmisartan group than in the Control group. The cardiac output values in the Control group were significantly decreased at 3 weeks compared with those at baseline, but no significant difference was observed in the Telmisartan group. This study demonstrates that telmisartan inhibits the reduction in ventricular systolic function and prevents myocardial injury in a canine model of SVTA. Therefore, telmisartan is suggested as a novel treatment for canine SVTA.

2. Reversible non-ischemic cardiomyopathy after orthotopic liver transplantation: a report of case series from single-center experience

Background: Non-ischaemic cardiomyopathy (NIC) is an early complication of liver transplantation (LT). Patients undergoing LT at our institution from September 2014 to April 2018. Patients who developed NIC were identified. Data collected included demographic and clinical data. Case Summary: A total 31 transplants were performed in this period and three patients developed NIC. Pretransplant dobutamine stress echocardiography was negative and Ejection fraction showed good LV systolic function. 25 patients (82%) were severely malnourished and required nutritional support. Median time to onset was 2 days post-transplant (range, 0–20). Echocardiograms showed global left ventricular hypokinesis and a decrease in ejection fraction (EF) from a median of 74% (range, 66–78) pretransplant to a median of 30% (range, 30–35). There was no significant difference between recipients with NIC vs. recipients without cardiomyopathy regarding donor age, donor risk index, and cold and warm ischemia time. Recovery of cardiac function occurred in all 3 patients, with a median EF of 60% (range, 58–62%) at the time of discharge. One-year survival of NIC patients was 100%. Conclusion: patients who developed NIC were individuals who had advanced liver disease with a high MELD score. The etiology of NIC is unknown, but it is most likely multifactorial. Although NIC is a rare complication of LT, and in most cases it is reversible, it can also cause significant post-operative morbidity requiring significant physiological support, a prolonged hospital stay, or mortality. Conclusions: Recognition of individuals at risk of NIC in the pre- or immediately post-LT period may be beneficial in reducing cardiovascular complications and may improve outcomes. The authors have none to declare.

Ratio of transmitral early filling velocity to early diastolic strain rate predicts long-term risk of cardiovascular morbidity and mortality in the general population.

It has previously been demonstrated that the ratio of early mitral inflow velocity to global diastolic strain rate (E/e'sr) is a significant predictor of cardiac events in specific patient populations. The utility of this measurement to predict cardiovascular events in a general population has not been evaluated. A total of 1238 participants in a general population study underwent a health examination including echocardiography where global longitudinal strain (GLS) and E/e'sr were determined. The primary endpoint was the composite of incident heart failure (HF), acute myocardial infarction (AMI) or cardiovascular death (CVD). During follow-up (median 11 years), 140 (11.3%) participants reached the composite endpoint. E/e'sr was associated with adverse outcome [HR 1.17 95% CI (1.13-1.21); P < 0.001, per 10 cm increase]. After multivariable adjustment for echocardiographic and clinical parameters, E/e'sr remained an independent predictor of the composite endpoint [HR 1.08, 95% CI (1.02-1.13); P = 0.003] as opposed to E/e' [HR 1.03, 95% CI (0.99-1.06); P = 0.11 per 1 unit increase]. Global longitudinal strain modified the relationship between E/e'sr and outcome (P for interaction = 0.015). E/e'sr was a stronger predictor in participants with good systolic function as determined by GLS (GLS > 18%) after multivariable adjustment, when compared to participants with reduced systolic function (GLS < 18%) [HR 1.28 95% CI (1.06-1.54); P = 0.011, and HR 1.08 95% CI (1.02-1.14); P = 0.012, respectively). E/e'sr provided incremental information [Harrell's C-index: 0.839 (0.81-0.87) vs. 0.844 (0.82-0.87); P = 0.045] beyond the SCORE risk chart. In the general population, E/e'sr provides independent and incremental prognostic information regarding cardiovascular morbidity and mortality. Additionally, E/e'sr is a stronger predictor of cardiac events than E/e'.

Prognostic significance of improvement in right ventricular systolic function during cardiac resynchronization therapy

Objectives There is conflicting evidence concerning the role of right ventricular (RV) systolic dysfunction in the long-term clinical outcome after cardiac resynchronization therapy (CRT). Therefore we aimed to assess evolution of RV systolic function during CRT, covariates associated with its improvement, and its impact on outcome.Methods and results All CRT device implantations (Jan 2009-Dec 2011) in our institution were reviewed. Records of 69 patients (25% female, mean age 62.8 ± 9.2 years, mean left ventricular (LV) ejection fraction 27 ± 8%) were analyzed. Baseline RV fractional area change (FAC) < 35% was present in 37 patients (54%). At one year, 24 of them (65%) improved in RV FAC. LV remodeling and mitral regurgitation were significantly associated with the likelihood of RV FAC improvement (OR 4.80, 95% CI 1.13-20.46, P = 0.034 and OR 0.32, 95% CI 0.12-0.89, P = 0.029, respectively). The composite endpoint of death or heart transplantation occurred in 23 patients (33%) over a mean follow-up of 2.8 ± 1.4 years. RV FAC at one year (HR 0.90, 95% CI 0.86-0.94, P < .001) was, independently of NYHA class and LV remodeling, associated with clinical outcome.Conclusions RV systolic function might improve during CRT. This seems mainly due to changed left-sided hemodynamics and LV remodeling. Good RV systolic function is independently related with better outcome.