Normal Biventricular Function Research Articles

Right coronary artery obstruction following transcatheter aortic valve replacement by aortic valve mass.

A 93-year-old woman with symptomatic severe aortic stenosis and normal biventricular function was referred for transcatheter aortic valve replacement (TAVR) evaluation. Cardiac computed tomography revealed safe coronary heights and multiple large calcified mobile mass-like structures attached to the aortic valve (AV), confirmed also by transesophageal echocardiography, which were thought to be prominent Lambl's excrescences.

A NEGLECTED RARE DISORDER: IGG4 RELATED DISEASE

Abstract IgG4–related disease (IgG4–RD) is an autoimmune disorder characterized by infiltration of plasma cells with IgG4. Almost any organ could be affected. Common presentations include lung manifestation, autoimmune pancreatitis, retroperitoneal fibrosis and tubulointerstitial nephritis. Cardiovascular involvement is less frequent. Corticosteroids are first line therapy, with or without anti–rheumatic drugs. Long–term prognosis of IgG4–RLD remains unknown. We present the case of a 65–years old woman, with a previuos diagnosis of IgG4–RD with mediastinal involvement. In the meantime she was recovered for pyelonephritis with high fever and positive blood coltures (S. hominis). We were called to perform a transthoracic echocardiogram to rule out infective endocarditis. The exam showed normal size of cardiac chambers, normal biventricular systolic function, absence of relevant valvular disease. In apical view we noticed a hyperechogenic ovalar imagine in the right atrium, not visible in all the projections. A first differential diagnosis included a mass with site of implantation at the top of the right atrium or something external but contiguous to heart structures. To clear this doubt the patient performed a thoracic CT with stunning results. The CT highlighted parietal encasement of chardiac structures and, particularly, of the superior vena cava at the confluence in right atrium, explaining the image we saw with transthoracic echo. The exam also showed encasement of ascending aorta, aortic arch and descending aorta with maximum thickness of 18 mm. CT also reported encasement of right pulmonary arthery. The CT highlighted a progression of the disease with indication to more aggressive therapy. In fact IgG4–RD of the cardiovascular system results in serious complications that need to be recognized and treated. Aortic involvement in IgG4–RD is extremely rare and tipically affects the infrarenal abdominal aorta. Cardiovascular IgG4–RD could also lead to aortitis, medium–vessel arteritis, pulmonary vascular disease, phlebitis, valvulopathy, pericarditis and myocardial disease. These forms of Igg4–RD are often underdiagnosed as clinical presentations is similar to cardiovascular disease due to more common aetiologies. Regarding our case the patient began methotrexate based on the results of the CT. This was a really interesting case for our working group to deepen this little–known disease. “The eyes see only what the mind is prepared to comprehend”.

THE SIGN OF THE HORNS: A RARE CASE OF DOUBLE PERI–COMMISSURAL MITRAL FLAIL

Abstract A 80–year–old man with history of hypertension and dyslipidemia was admitted to our Cardiology Unit for exertional dyspnea (NYHA III) occurred during the last few days. The physical examination showed a systolic heart murmur (4/6 on Levine scale) and signs of pulmonary congestion. Transthoracic echocardiography revealed a massive mitral regurgitation (Fig.1) together with severe dilation of the left atrium, mild–to–moderate aortic regurgitation, high probability of pulmonary hypertension and normal biventricular systolic function. A transesophageal echocardiography (TEE) was performed to clarify mitral valve anatomy. A double flail of the posterior leaflet was present involving the peri–commissural portions due to a single chordal rupture on P1 scallop and a double chordal rupture on P3 scallop (Fig.2). P2 scallop was prolapsing and there was a billowing of the anterior leaflet. In addiction mitral annulus was dilated (antero–posterior diameter of 43 mm and intercommissural diameter of 45 mm). We named this finding “sign of the horns” for his 3D echocardiographic appearance (Fig.3) given the double flail resembling two fingers. A surgical correction was performed with 3 GoreTex neochords implantation on the posterior leaflet and an annuloplasty ring (Physio II n. 34) implantation during extracorporeal circulation through right anterior minithoracotomy. Surgical and echocardiographic results were optimal. The patient was discharged after 7 days of hospitalization. Conclusion Coexistence of a double peri–commissural flail in the same leaflet is a very rare echocardiographic finding and we named it “double horn sign”. TEE is an essential examination in this scenario in order to understand the exact mechanism of the regurgitation for an optimal preoperative planning.

CLINICAL CHALLENGE: AORTITIS IN THE EMERGENCY DEPARTMENT

Abstract A 67 yo woman presented to the ED for a sustained episode of palpitations and acute chest pain. Past medical history: suspected psoriatic arthritis previously treated with steroids and hydroxychloroquine; previous suspicion of rheumatic polymyalgia and Horton arteritis, regressed with steroids; gastroesophageal reflux disease; mild hypercholesterolemia and a family history of premature coronary artery disease. In the ED the patient was hemodynamically stable. On physical examination: tachyarrhythmic heart sounds with no murmurs. The peripheral arterial pulses were preserved and symmetrical. The remaining physical examination was unremarkable. ECG: atrial fibrillation at 150 bpm. The D–dimer was increased, normal hs–troponin and normal haemoglobin. On echocardiography: normal biventricular structure and function; normal aortic root with dilatation of proximal ascending aorta (47 mm), without visible intimal flaps. The anterior wall of the proximal ascending aorta was thickened. A moderate circumferential pericardial effusion was present, without signs of hemodynamic impact. On suspicion of an acute aortic syndrome with possible intramural haematoma of an ascending aortic aneurism, an urgent CT scan was ordered. It showed a severe dilatation of ascending aorta (52 mm) involving of the proximal aortic arch. The anterior aortic wall was thickened (5 mm), without clear images of IMH, aortic dissection or penetrating ulcer. The pericardial effusion had a supra–fluid density without blood density. A repeated CT at 6 hours showed no changes. The case was suggestive of an inflammatory aetiology. The patient was admitted to the cardiac intensive care unit. A PET–CT scan showed signs of an inflammatory disease of the great vessels. A serological autoimmunity screening was negative. The patient was diagnosed with inflammatory aortitis (giant cell vs Takayasu arteritis) and started on high dose iv glucocorticoid therapy. Given the high risk of severe aortic complications, adjunctive therapy with tocilizumab was prescribed. The therapy was gradually tapered, with subsequent switch to oral steroids plus tocilizumab. At one and a half month of follow–up, a CT scan was repeated, documenting stable aortic diameters and the disappearance of the abnormal thickness and of the enhancement of the aortic wall. A follow–up PET–CT scan has been scheduled at 6 months time. An elective aortic intervention has been indicated after remission of the inflammatory phase.

ACUTE MYOCARDIAL INJURY IN A PATIENT WITH TYPE–1 NEUROFIBROMATOSIS: A CASE OF SUSPECTED PHEOCROMOCYTOMA–ASSOCIATED MYOCARDITIS

Abstract A 54–year–old patient with an history of type–1 neurofibromatosis presented to the emergency department (ED) due to recurrent abdominal pain with diarrhea. Clinical examination didn’t show anything remarkable. She was mildly hypertensive, apyretic. During her staying in the ED, she complained about chest pain and an electrocardiogram (ECG) was performed. ECG showed sinus rhythm, normal heart rate, left ventricular (LV) hypertrophy with marked horizontal ST segment depression in the precordial leads and bimorphic ventricular ectopic beats with prevalent right bundle branch block superior axis morphology, likely from the medio–basal inferior LV. Troponin T (peak 958 ng/L, normal <15 ng/L) and NTproBNP (peak 2357 ng/L, normal <180 ng/L) were elevated, hemoglobin, creatinine and liver function were normal, C–reactive protein peaked at 120 mg/dL. Echocardiogram showed mildly reduced left ventricular ejection fraction (LVEF) with inferior and inferolateral hypokinesia and moderate mitral regurgitation (MR). She was admitted to cardiac intensive care unit with the suspected diagnosis of an acute coronary syndrome and was started on acetylsalicylic acid, metoprolol, furosemide and potassium canrenoate. Amiodarone was added due to frequent ventricular tachycardias. Due to the symptoms and the history of neurofibromatosis a whole body computed tomography (CT) was done. CT showed a 53 mm mass on the left adrenal gland with intense enhancement, suspected for a neoplastic origin (Figure 2). Plasmatic and urinary cathecolamines and metanephrines were increased. Positron–emission tomography (PET)/CT with 68Ga showed left adrenal uptake, compatible with a neuroendocrine tumor. Coronary arteriography showed normal coronary arteries. The patient underwent a cardiac magnetic resonance (CMR) study in the suspect of acute myocarditis. CMR showed normal biventricular function, an inferior basal hypokinesia with focal intramyocardial late gadolinium enhancement (LGE) suspected for a post–inflammatory origin and mild pericardial effusion. After pharmacological preparation a laparoscopic left adrenalectomy was performed, and histology confirmed the diagnosis of a composite pheocromocytoma. Three months after, the patient was asymptomatic, biochemistry showed normalization of both troponin T and endocrinological biomarkers, the echocardiogram showed preserved LVEF without areas of hypokinesia and mild MR. A follow–up CMR at six months from the acute injury has been scheduled.

Long-Term Erythrocytapheresis Did Not Prevent Development of Severe Pulmonary Hypertension in Sickle Cell Disease

Background The WHO classifies pulmonary hypertension into five different groups based on pathophysiology, clinical presentation, and therapeutic options. The development of pulmonary hypertension (PH) in sickle cell disease (SCD) is multifactorial and can cause devastating complications. Erythrocytapheresis is utilized in patients with SCD who have multiple comorbidities or are unresponsive to first-line treatments like hydroxyurea. It is hypothesized that treatment with long-term erythrocytapheresis (LTE) could slow the progression to pulmonary hypertension by modifying the pathophysiology of the disease process. However, limited clinical data supports this transfusion modality [1]. Case Description The patient is a 17-year-old female with a history of SCD, obstructive sleep apnea (OSA), nocturnal hypoxemia, and recurrent pain crises maintained on LTE every four weeks for the past seven years. She had previously failed hydroxyurea therapy. She underwent a tonsillectomy and adenoidectomy seven years ago to treat OSA. The patient had an echocardiogram five years ago, which showed normal biventricular function and tricuspid regurgitation jet velocity. Recently she presented to the emergency department with cough, shortness of breath, fever, diarrhea, and nausea lasting for three days. Initial assessment revealed elevated liver enzymes, elevated BNP, hyperbilirubinemia, hemoglobin of 9.5 g/dL, and oxygen saturations in the 80's. Echo revealed right ventricular dysfunction and right atrial and ventricular enlargement with a tricuspid regurgitation jet velocity of 4.29 m/s and estimated right ventricular systolic pressure 90% systemic, as seen in figure 1. The patient was admitted to the PICU under concern of pulmonary hypertension crisis. She underwent a right cardiac catheterization three days into her admission and was diagnosed with severe pulmonary hypertension with a mean pulmonary artery pressure of 49 mmHg. Pulmonary capillary wedge pressure was 11 mmHg, and pulmonary vascular resistance was calculated to be 18 wood units x m 2 with 20 PPM of nitric oxide and 80% FiO2 the PVR decreased to 11 woods units x m 2 (figure 2). CTA showed subsegmental thrombosis and vascular surgery was consulted for evaluation for endarterectomy, but they decided she was not a surgical candidate. She was initiated on anticoagulation therapy with enoxaparin, sildenafil, and bosentan. Conclusions The cause of PH in this patient appears to be multifactorial. Possible etiologies include SCD-related, primary pulmonary hypertension, or subsegmental pulmonary embolism. Although LTE is increasingly used in the management of SCD, providers should not assume that a patient receiving this treatment will not progress to develop complications. Some patients on LTE may also develop progressive CNS vasculopathy and are still at risk for developing hypertension. Patients with SCD should be screened for PH if they are symptomatic or have comorbid conditions. This patient had a history of nocturnal hypoxemia and OSA, which some studies indicate are associated with pulmonary hypertension in adolescents with SCD. Screening for nocturnal hypoxemia and OSA is recommended in SCD to identify patients at high risk of PH development [2].

Abstract 18472: Plasma Proteome Changes in Heart Failure and Non-Heart Failure Patients Undergoing Cardiac Surgery

Introduction: Patients with and without heart failure display different clinical courses after cardiac surgery. Hypothesis: Unbiased analyses of the plasma proteome from extreme phenotypes in myocardial function may identify differences in candidate proteins differentially modified by cardiac surgery using cardiopulmonary bypass (CPB). Methods: We combined two prospectively-collected datasets: Left ventricular assist device (LVAD) recipients from the INSPIRE-FLO trial (N=94, NCT03081052) and coronary artery bypass graft (CABG) surgery patients with normal biventricular function (N=50). For each population, we obtained EDTA-plasma from arterial blood sampling in the operating room and on postoperative day 1. Including quality control pools, 304 total samples were digested in a 96-well format with trypsin. Peptides were analyzed by microflow liquid chromatography coupled to tandem mass spectrometry using data-independent acquisition. Proteins were identified and quantified at a 1% false discovery rate (FDR) in Spectronaut. Statistical analyses utilized a t-test with FDR correction (significance defined by log2FC>1 and q<0.05). Results: There were 943 protein groups quantified in at least one plasma sample. Patients undergoing LVAD surgery had significantly higher levels of 135 proteins including acute phase reactants (CRP, SAA1, SAA2), putative neutrophil-derived proteins (S100A8/9) and markers of renal insufficiency (B2M and REG3A) (Figure 1). Cardiac surgery using cardiopulmonary bypass increased acute phase and inflammatory markers, and cardiac proteins (ENOB, MYG and KCRM), with NGAL, TIMP1 and PAI1 among proteins with higher absolute increase after LVAD implantation versus CABG surgery ( Fig.1 ). Conclusions: Unbiased analyses of the plasma proteome identifies baseline and postoperative differences associated with extreme phenotypes of myocardial function undergoing cardiac surgery using CPB.

Abstract 12297: A Rare Case of May-Thurner Syndrome Due to External Compression of the Right and Left Common Iliac Veins

Description of Case: A 54yr male with HTN and CKD stage IIIa presented with asymmetric right greater than left lower extremity (LE) edema. Vital signs were normal. His exam showed asymmetric right greater than left LE edema to the mid-thighs. ECG revealed sinus rhythm. Medical therapy with increasing titration of diuretics was unsuccessful in resolving his asymmetric LE edema. A TTE showed EF of 55-60% with normal bi-ventricular size and function and no significant valve abnormalities. Bilateral duplex US showed no DVT. CT abdomen pelvis showed no hydronephrosis or compressive masses. Right heart catheterization revealed normal bi-ventricular filling pressures. Bilateral peripheral venogram showed bilateral common iliac vein stenosis (Figure 1A). IVUS showed external compression of the right common iliac vein (RCIV) between the right external and internal iliac arteries with external compression of the left common iliac vein (LCIV) by the right common iliac artery (RCIA) against the lumbar spine (Figure 1A). A diagram of the compression observed in this case is included (Figure 1B). The RCIV had an 18mmHg gradient with no significant gradient on the LCIV. The RCIV was stented using an 18x60mm self-expanding stent and balloon dilation. Final IVUS and venogram showed no residual stenosis, edge dissection or perforation (Figure 1D). The patient was discharged on dual-antiplatelet therapy for three months and aspirin thereafter with continued diuretics. He had significant reduction in asymmetric LE edema with marked improvement in ambulation capacity and no further LE asymmetry at the 30-day follow-up visit. Discussion: Here we present a rare case of May-Thurner Syndrome via compression of the LCIV by the RCIA and RCIV by the right internal and external iliac arteries with successful management. We also illustrate the importance of a broad differential diagnosis in patients with persistent asymmetric LE edema refractory to diuretic management

Abstract 13000: Twiddler’s Syndrome - A Rare Cause of Pacemaker Malfunction

Description of Case: A 76-year-old female with a past medical history of chronic atrial fibrillation and symptomatic tachycardia and bradycardia status-post dual-chamber pacemaker with right atrial and ventricular leads who presented with dyspnea on exertion. TTE showed normal biventricular function and no significant valvular abnormalities. Upon further evaluation of her pacemaker, she was found to have a very high ventricular capture threshold suggesting RV lead failure. The patient underwent RV lead replacement. The inspection of the pacemaker pocket during the procedure showed manual dislodgement of both tie-down sleeves and the pacemaker leads required untangling. A new RV lead was subsequently placed. Upon further imaging review, displacement of the previous RV lead was evident from initial placement. In addition, the patient had previously had a history of interval diaphragmatic stimulation as well. Overall, the presentation was felt to be consistent with Twiddler’s Syndrome. Discussion: Twiddler’s or Reel Syndrome occurs when conscious or subconscious self-manipulation and spinning of the pulse generator result in lead dislodgement and loss of pacing and pacemaker malfunction. The reeling of the leads around the generator can also cause stimulation of the brachial plexus or phrenic nerve stimulation and subsequent rhythmic arm twitching or diaphragmatic pacing, respectively. It has previously been reported in cases of single- and dual-chamber ICDs, deep brain stimulators, and spinal nerve stimulators with varying presentation. The diagnosis requires clinical suspicion based on history, physical exam, imaging, or surgical inspection suggesting the displacement of leads with evidence of coiling of the wires around the pacemaker. Treatment often requires revision and focus should be maintained on patient education for both primary and secondary prevention of Twiddler’s Syndrome.

Abstract 17846: Successful Treatment of Recurrent Pericarditis With Rilonacept in the Setting Of ATRA/ATO Use in Acute Promyelocytic Leukemia

Case Presentation: A 40-year-old female was admitted for a 4-day history of recurrent left-sided pleuritic chest pain. Physical examination was pertinent for tachycardia and presence of a pericardial friction rub. Past medical history included recently diagnosed Acute Promyelocytic Leukemia undergoing active chemotherapy. Complete blood count showed leukopenia. Brain natriuretic peptide was slightly elevated at 241 pg/mL. Electrocardiogram demonstrated sinus tachycardia with diffuse ST-segment elevation and PR-segment depression (Figure 1. A). Echocardiogram showed normal biventricular function with a small posterior and lateral pericardial effusion and small pericardial effusion adjacent to the right ventricle (Figure 1. B). Cardiac magnetic resonance (CMR) imaging showed a moderate circumferential pericardial effusion and increased pericardial signal on T2 STIR imaging and late gadolinium enhancement (LGE) (Figure 1. C, D). Given the constellation of symptoms and findings, the patient was diagnosed with acute pericarditis secondary to differentiation syndrome (DS) suspected to be due to her underlying malignancy and anti-cancer treatments. She was started on aspirin and colchicine. Steroids and rilonacept were later added for incomplete treatment response. On follow-up, she was doing well. Repeat CMR demonstrated mild to moderate pericardial delayed enhancement with no edema and no pericardial effusion (Figure 1. E,F). Discussion: The presentation of DS can vary, and consensus diagnostic criteria are lacking. Cases of myopericarditis with concurrent pericardial effusions have been reported in the setting of patients undergoing chemotherapeutic treatment with differentiating agents. Management is complicated and requires multi-disciplinary care. Our case illustrates that rilonacept may be considered as an alternative to corticosteroids in the treatment of recurrent pericarditis in a patient with a hematologic malignancy.

Dual-energy computed tomography to detect early pulmonary vascular changes in children with sickle cell disease: a pilot study.

Pulmonary hypertension (PH) is a rare but fatal complication of sickle cell disease (SCD) that is possibly reversible if treated early. Dual-energy computed tomography (DECT) is a valuable tool for diagnosing PH. We attempted to determine if DECT can detect early signs of PH in children with SCD. This prospective observational pilot study was conducted at the Geneva University Hospitals and was approved by the local human ethics committee (CCER 2019-01975). A written informed consent was obtained from the patients and/or their legal guardian. Eight children (consisting of five girls and three boys) with homozygous SCD were included in the study. They underwent full cardiological workup using transthoracic echocardiography (TTE) and cardiopulmonary exercise test (CPET), as well as DECT. The median age of the children was 11 years old (range 8-12). All patients exhibited a normal biventricular systo-diastolic function using the TTE. The median tricuspid regurgitant jet velocity value was 2.24 m/s (range 1.96-2.98). Four children were found to have signs of vasculopathy detected on DECT. Of them, two had abnormal screening test results. They both had an increased VE/VCO2 slope during CPET and an increased TVR of >2.5 m/s on TTE. DECT is capable of identifying early signs of pulmonary vascular disease in children with SCD. Further studies are needed to understand the correlation between DECT abnormalities and hemodynamic pulmonary circulation better.

Acute lymphocytic myocarditis presenting as complete heart block in an adult: a case report

BackgroundComplete heart block (CHB) as a first presentation of acute viral myocarditis is a rare occurrence associated with increased morbidity and mortality. In such cases, an endomyocardial biopsy is recommended to make a clear histological diagnosis aiding to differentiate from other possible conditions such as sarcoiditic myocarditis, giant cell myocarditis, and eosinophilic myocarditis. Insertion of a permanent pacemaker may be considered on a case-to-case basis.Case presentationA previously healthy 21-year-old female presented to the emergency department after having suffered two episodes of syncope on a background of a few days’ history of myalgias, chills, and rigors. Electrocardiogram showed high-grade Mobitz II block with intermittent periods of CHB. A bedside echocardiogram upon admission demonstrated normal biventricular systolic function. Given the patient’s unstable haemodynamic status and lack of obvious reversible causes for the CHB, a permanent dual-chamber pacemaker was inserted urgently. Initial blood investigations indicated an ongoing inflammatory process highlighting the possibility of myocarditis as a cause of the CHB. Therefore, a troponin level was taken and was noted to be elevated confirming the suspicion of myocarditis. The left ventricular ejection fraction (LVEF) decreased over the following days to approximately 20%, clinically resulting in pulmonary oedema and acute shortness of breath. The patient required aggressive intravenous diuresis and anti-heart failure medication. An endomyocardial biopsy (EMB) confirmed the diagnosis of lymphocytic myocarditis. The patient’s condition improved secondary to an improvement in LVEF and resolution of the heart block. A cardiac magnetic resonance (CMR) imaging performed 6 weeks from admission reported an improved LVEF of 51% with no late gadolinium enhancement (LGE). Based on the reassuring CMR findings and the resolution of CHB on follow-up pacemaker checks, it was deemed safe to explant the pacemaker.ConclusionsAcute myocarditis may be complicated with high-degree AV block and cardiogenic shock necessitating close observation in a critical care unit. A permanent pacemaker may provide atrio-ventricular synchrony which helps stabilise the patient’s condition and protect from a prolonged period of heart block. Early myocardial fibrosis on EMB and degree of LGE on CMR are indicators of persistent atrioventricular block. Guideline-directed treatment of heart failure is essential.

Utility of Follow-Up Echocardiograms in Uncomplicated Surgical Secundum Atrial Septal Defect Closures: Preliminary Analysis.

Though less common in the current era, surgical closure of secundum atrial septal defects (ASD2) is still performed in certain clinical situations. Guidelines currently recommend lifelong follow-up with transthoracic echocardiograms (TTE) for patients who have undergone a surgical ASD2 closure. The goal of this study was to determine the utility of follow-up TTE in patients who underwent an uncomplicated ASD2 closure. Chart review was performed on patients who had a surgical ASD2 closure between April 1, 1996, and August 30, 2021. Patients were excluded if they had other congenital heart disease, had a diagnosis of a residual ASD2, atrial/ventricular arrhythmias, pulmonary hypertension, heart failure, or did not have a follow-up TTE > 6months after the procedure. The most recent TTEs and clinic notes were evaluated. A total of 30 patients met the criteria. The median age at ASD2 surgery was 4.0years (IQ; 1.9-10.5). ASD2 was closed via patch repair in 16 patients and primarily closed in 14 patients. The most recent TTE was performed a median of 9.5years (IQ; 4.0, 14.7) after ASD2 closure. Two patients had mild right atrial and ventricular dilation, one patient had mild right atrial dilation, and one patient had mild right ventricular dilation. All other patients had qualitatively normal right-sided chamber sizes. All patients had normal biventricular function (left ventricular fractional shortening (median 36% (IQ; 33, 42)), no evidence of residual atrial shunts, and no evidence of pulmonary hypertension. No patient was on any cardiac medications at last clinic visit. Four patients were discharged from cardiology clinic and 10 patients were lost to follow-up. There were no deaths. Twenty-four patients had 46 repeat echocardiograms > 1year after ASD2 with no change in clinical management. In patients who underwent an uncomplicated ASD2 closure, there were no significant abnormalities noted on follow-up TTEs. The need for repeat lifetime TTEs and their frequency, in this uncomplicated population, should be reassessed if larger studies with longer follow-up confirm these initial findings.

The Yasui operation: A single institutional experience over 30 years

ObjectiveThe Yasui operation was introduced in 1987 for patients with 2 adequate ventricles, a ventricular septal defect, and aortic atresia or interrupted aortic arch. Despite promising early outcomes, left ventricular outflow tract obstruction (LVOTO) remains a long-term concern. The purpose of this study is to report our institutional experience with the Yasui operation. MethodsWe retrospectively reviewed all patients undergoing the Yasui operation between 1989 and 2021. Results are reported as median with interquartile range (IQR). ResultsTwenty-five patients underwent a Yasui operation (19 primary), at 11 days (IQR, 7-218 days) of life and weight of 3 kg (IQR, 2.8-4.1 days). Fundamental diagnosis was ventricular septal defect/interrupted aortic arch in 11 patients and ventricular septal defect/aortic atresia in 14. Follow-up was 96% (24 out of 25) at 5 years (IQR, 1.4-14.7) with 92% survival. Freedom from LVOTO reoperation was 91% at late follow-up with 2 patients requiring baffle revision at 6 and 9 years. Latest echocardiogram showed 100% of patients had normal biventricular function and 87% (20 out of 23) less than mild LVOTO at 5 years (IQR, 2.3-14.9). Diagnosis, aortic valve morphology, and material used were not predictors of LVOTO. Freedom from right ventricle-to-pulmonary artery conduit reoperation was 48% at a median of 5 years (IQR, 1.4-14.7). Conduit type was not a predictor of reintervention. ConclusionsThe Yasui operation can be performed with low morbidity and mortality in patients with 2 acceptable-size ventricles and aortic atresia or interrupted aortic arch with severe LVOTO. Despite some burden of reoperation, midterm reoperation for LVOTO is not common and ventricular function is preserved.

Bilateral lung transplantation for pediatric pulmonary arterial hypertension: perioperative management and one-year follow-up.

Bilateral lung transplantation (LuTx) remains the only established treatment for children with end-stage pulmonary arterial hypertension (PAH). Although PAH is the second most common indication for LuTx, little is known about optimal perioperative management and midterm clinical outcomes. Prospective observational study on consecutive children with PAH who underwent LuTx with scheduled postoperative VA-ECMO support at Hannover Medical School from December 2013 to June 2020. Twelve patients with PAH underwent LuTx (mean age 11.9 years; age range 1.9-17.8). Underlying diagnoses included idiopathic (n = 4) or heritable PAH (n = 4), PAH associated with congenital heart disease (n = 2), pulmonary veno-occlusive disease (n = 1), and pulmonary capillary hemangiomatosis (n = 1). The mean waiting time was 58.5 days (range 1-220d). Three patients were bridged to LuTx on VA-ECMO. Intraoperative VA-ECMO/cardiopulmonary bypass was applied and VA-ECMO was continued postoperatively in all patients (mean ECMO-duration 185 h; range 73-363 h; early extubation). The median postoperative ventilation time was 28 h (range 17-145 h). Echocardiographic conventional and strain analysis showed that 12 months after LuTx, all patients had normal biventricular systolic function. All PAH patients are alive 2 years after LuTx (median follow-up 53 months, range 26-104 months). LuTx in children with end-stage PAH resulted in excellent midterm outcomes (100% survival 2 years post-LuTx). Postoperative VA-ECMO facilitates early extubation with rapid gain of allograft function and sustained biventricular reverse-remodeling and systolic function after RV pressure unloading and LV volume loading.

P36 A CHALLENGING CASE OF MINOCA

Abstract Myocardial infarction in the absence of obstructive coronary artery disease (MINOCA) accounts for 5–6% of all myocardial infarctions. The causes that can lead to this diagnosis are many and not always clearly identifiable; in fact, despite optimal work–up, the cause of MINOCA remains undetermined in 8–25% of patients and diagnosis represents a real challenge for the cardiologist clinician. Because patients with MINOCA have clinical outcomes comparable to those with myocardial infarction with obstructive coronary artery disease, they should undergo a careful diagnostic work–up in order to set up a therapy related to the specific diagnosis. Introduction A 51–years old woman presented to the emergency room after a sudden onset of an oppressive, prolonged and non–radiating chest pain, with gradual resolution after intravenous nitroderivates therapy. Smoking was the only cardiovascular risk factor. A recent uncomplicated COVID 19 infection (2 months before hospital admission) was the only event reported in clinical history. Physical examination was unremarkable with the exception of an apical mid–end systolic murmur. The electrocardiogram was normal and the Troponin T peak was 391 pg/ml. An echocardiogram showed a normal biventricular function without alterations of segmental kinetics and mitral valve prolapse with moderate degree regurgitation. The patient was admitted to the Intensive Care Unit department and underwent a coronary angiography study with evidence of main epicardial coronaries free from stenosing atherosclerotic lesions. In addition, a cardiac magnetic resonance (CMR) was performed before discharge, with evidence of normal biventricular systolic function in the presence of limited area of ​​late enhancement with ischemic pattern at the level of the middle segment of the inferolateral wall of the left ventricle without edema. In the light of the clinical instrumental picture, a diagnosis of MINOCA was made. Discussion This clinical case is an example of how sometimes the cause relating to a MINOCA cannot always be well identified. However, the multi–imaging approach and in particular the resonance allowed to identify a localized area of ​​late enhancement with an ischemic type pattern. The origin of the ischemia could be dated from a picture of distal embolization, from a microvascular disease or from a myocardial stretching due to prolapse.

P295 WHEN TAVI IS THE RIGHT CHOICE

Abstract Clinical case We describe a case of a 62–y–old man, affected by hypertension, insulin–dependent diabetes mellitus, advanced liver cirrhosis. After screeing, the patient was listed for liver transplantation. On September 2022, the patient was admitted to our Emergency Room due to HF in class NYHA III. The transthoracic echocardiogram showed a severe aortic regurgitation with mobile vegetations, the largest of which is more than 10 mm on the right aortic cusp. Normal biventricular function was observed with sPAP of 45 mmHg. Thus, we concluded for post–endocarditis severe aortic regurgitation. We discussed tha case in our Department Heart Team with the concomitant presence of the Liver Team. We considered the patient at high surgical risk, mostly due to the risk of postoperative hepatic insufficiency. So, we decided for a TAVI off–label. So, we impantanted a Sapien 3 Ultra 26 mm through transfemoral access. To reduce the risk of cerebral embolization, we used Triguard 3 system, while to prevent coronary embolization we used a closed stent into the right coronary ostia, ready to be opened. The patient was discharged in the fourth postoperative day, without conduction disturbances. The postoperative echo showed normal valvular function and absence of paravalvular leakages. Conclusions Patients with advanced liver cirrhosis are at risk of organ failure following use of extracorporeal circulation. In selected patients, TAVI may be a valid alternative. The peculiarity of the specific case was due to the use of the TAVI in an off–label procedure of aortic insufficiency with the simultaneous presence of mobile vegetation at high embolic risk.

P366 FULMINANT MYOCARDITIS WITH SPONTANEOUS RESOLUTION AFTER COVID 19 AND ALLERGIC EPISODE: MIS–A OR ALLERGIC MYOCARDITIS?

Abstract Clinical case 23–year–old male. No medical history Not vaccinated for Sars–Cov2; Recent Sars–Cov2 infection (negative antigenic swab on 03/01/22); Pharyngodynia treated with ibuprofen + ciprofloxacin and amoxicillin ac clavulanic acid(suspended for purpuric lesions on hands) After 5 days from onset of symptoms, access to PS for loss of consciousness. Laboratory: ph 7.4; Lat 3.4 EKG: sinus tachycardia HR 126 bpm ECHOCARDIOGRAM: Non–dilated left ventricle, marked reduction in FE (15%) spontaneous echocontrast in ventricular cavity chest TC: hypodense tissue in anterior mediastinum; adenopathy in peribronchial site, distended gallbladder transfer to Vanvitelli Cardiac ICU Amine support with augmented DBT for hypotension and tachycardia LAB: Tni 2193; Nt proBNP 27796; PCR 26; PCT 4; crea 1,4, Lat 1 CATH DX + EMB after 24 hours EBM: …Mixed cellularity endomyocarditis, predominantly lymphocytic, presence of eosinophils… enrollment in the MYTHS study: placebo group (saline solution) after 10 day MRI:Normal biventricular systolic function. Tissue characterization images consistent with the presence of limited area of active myocardial damage and with nonischemic pattern involving the basal segment of the infero–lateral wall of the left ventricle. Discussion The case has been a source of considerable discussion to establish the etiology of myocarditis. Although with numerous doubts, after careful evaluation of the histologic picture, the case falls under possible MIS–A (MULTISYSTEM INFLAMMATORY SYNDROME in adult) Cardiac manifestations of MIS–A include myocarditis, pericarditis, and arrhythmias, which can rapidly lead to cardiogenic shock.

P375 ACUTE PULMONARY EMBOLISM IN A STABLE PATIENT PRESENTING WITH RIGHT CARDIAC CHAMBERS THROMBOSIS: A TICKING CLOCK SCENARIO

Abstract A 73–year–old male presented to the emergency department with a 1–week history of asthenia and exertional dyspnoea. He reported a recent finding of a not yet characterized renal mass in a not relevant past medical history. At the admission, he was haemodynamically stable. Electrocardiogram showed sinus rhythm without relevant abnormalities. Blood results evidenced only a slight increase of hs–troponin levels and D–dimer. An early point–of–care ultrasound (POCUS) demonstrated normal chambers dimensions and biventricular function but in the right atrium an elongated and mobile mass was documented. The echo–features were very suggestive of a large thrombus and a computed tomography pulmonary angiogram revealed several emboli in the left pulmonary artery. At the admission to our cardiac intensive care unit, according to pulmonary embolism (PE) ESC guidelines1, systemic anticoagulation was started with an aPTT–guided unfractioned heparin (UFH) infusion. Intensive monitoring was set to permit early detection of haemodynamic impairment. Multidisciplinary discussion on management was instituted but, soon after, the patient had a PEA arrest. POCUS demonstrated an extremely dilated right ventricle so rescue fibrinolytic therapy (FT) was attempted during cardiopulmonary resuscitation but the patient died. The intermediate–high–risk profile of PE encompasses a very heterogeneous group of patients, in whom controversy still exists regarding the net clinical benefit of using FT compared to anticoagulation alone. In the largest PEIHTO trial, FT compared to UFH alone prevented death and hemodynamic decompensation but increased major bleedings and strokes2. Data meta–analysis confirmed that in these patients, FT was associated with a lower mortality at the cost of more bleedings3. Current guidelines endorse FT only at the moment of clinical deterioration. Nevertheless, stable patients with “thrombus in transit” represent a niche of potentially rapidly deteriorating clinical scenario with not–predictable timing. In this setting we believe that, waiting for data deriving from interventional approaches, an aggressive therapeutical strategy should be considered, offering more liberally an early FT, especially when absolute contraindications are lacking. Data from registry or trials are strongly requested. 1.Konstantinides SV et al. EHJ 2020;41:543–603. 2.Meyer G et al. NEJM 2014;370:1402–11. 3.Chatterjee et al. JAMA 2014;311:2414–21.

P369 AUTOIMMUNE LYMPHOCYTIC MYOCARDITIS: ONE SIZE DOES NOT FIT ALL

Abstract Fulminant myocarditis (MF) is an acute onset, rapidly progressive and potentially fatal inflammatory cardiomyopathy which could manifests with cardiogenic shock and incessant arrhythmias. There is often a need for aminic or mechanical support to the circulation in the acute phase, waiting to obtain a histological diagnosis as quickly as possible to establish a specific therapy. Mortality in the acute phases is high, with a worse prognosis than in other forms of presentation of myocarditis, even in the long term. We report the case of a 45–year–old Asian woman suffering from autoimmune hypothyroidism, who had been reporting atypical chest pain for about 3 days, associated with fever, asthenia, and vomiting. On admission, the patient was in cardiogenic shock; an echocardiogram revealed biventricular dysfunction. Pulmonary embolism and acute coronary syndrome were excluded. Blood tests showed a significant troponin rise, neutrophilic leukocytosis, and hypertransaminasemia. Anti heart antibodies (AHA) were negative. Amine support with dobutamine and norepinephrine was then started. The first EKG showed an advanced atrioventricular block with a ventricular escape with the need for emergency temporary PM placement. Right heart catheterization showed pressures in the pulmonary circulation at the upper limits with average resistance and reduced cardiac index. An endomyocardial biopsy (EMB) was performed, subsequently complicated by cardiac tamponade due to lead displacement, treated with urgent pericardiocentesis. At the end of the procedure, the appearance of incoming episodes of complete BAV was highlighted, for which ICD implantation was performed. The biopsy showed virus–negative diffuse acute lymphocytic myocarditis. Cortisone therapy was therefore started, with immediate benefit, until the aminic support was suspended. A follow–up echocardiogram showed a complete recovery of normal biventricular function. In MF, early diagnosis, and the possibility of establishing specific therapy are crucial to reduce mortality. EMB is the only method that allows you to make an etiological diagnosis and exclude a possible infectious genesis. The patient‘s prognosis, in fact, in addition to supportive therapy, depends on the possibility of establishing a targeted treatment as soon as possible, which in virus–negative autoimmune forms consists of an immunosuppressive therapy modulated on the patient‘s histological type and clinic.