503 Role of three dimensional echocardiography and magnetic resonance imaging findings in the decision making and surgical planning of myxomas: correlation with clinical, surgical, and pathological features

Aims Transthoracic (TTE), transesophageal echocardiography (TEE), and cardiac Magnetic Resonance Imaging (MRI) are usually the mainstay for diagnosis of cardiac myxomas (MYX). It has been reported that three dimensional (3D) TEE has an additional value in describing the anatomical features. MYX are classified in two types: solid (with a round shape and a non-mobile surface) and papillary (asymmetrical shape with an irregular surface). Even though embolic events are linked to thrombus formation in both types, the latter are more frequently source of embolism. The detection of the attachment of the peduncle and a detailed assessment of the echotexture and of the surface characteristics of the tumour is not always possible by 2D TTE and TEE, therefore MRI is considered the gold standard for diagnosing cardiac tumours and myxomas. Our study aim to determine the accuracy of 3D echocardiographic techniques in the detection of the following anatomical features of the MYX: site of attachment (mid atrial septum, other locations), modality of attachment (pedunculated or sessile), echotexture, surface characteristics in comparison with MRI, and histological findings. Methods and results We retrospectively reviewed 11 cases of the confirmed myxomas by histology that underwent 2D, 3D TEE, and MRI (6 cases) in the past 6 years in order to assess the ability of the imaging techniques in identifying the anatomical features. Pathology samples were analysed in all patients. Differences in parameters were collected. Our study group is comprised by 10 patients (one recurrence), 8 female, mean age 45.6 ± 14.6 years with 15 myxomas; 11 located in the left atrium [one in left atrial appendage (LAA), one on the mitral valve annulus, 9 near the fossa ovalis], 3 in the right atrium, and 1 on the left ventricular outflow tract (LVOT). Seven were pedunculated, 8 sessile. 2D TEE was not able to diagnose two myxomas. There was an agreement between 2D TEE and 3D TEE in detecting the peduncle except in three patients (only seen by 3D TEE): in the first patient the peduncle was in the atrial septum close to the opening of the left upper pulmonary vein; in the second it was close to the LAA and in the last it was in the LVOT. 3D TEE echotexture analysis matched the pathology in all cases, also in one case of solid tumour with fimbriated edges on a one side which was found to have a clot formation at surgery. There was an excellent matching between the 3D TEE and the MRI in all cases. In our cohort 3DE was able to detect all the morphologic features of the MYX including the site and the type of attachment, the echotexture, and the surface characteristics. The above results led to predict the histologic type, solid, or papillary and there was a correlation with embolism (three patients) and papillary tumours (two patients). Conclusions 3D TEE and MRI are reliable in assessing the anatomical features of myxomas. The described additional features (peduncle, echotexture, and surface characteristics) seen by these two methods could predict the histological type and have an additional value in the work up of the myxomas leading to correct diagnosis and evaluation of possible complications such as embolic risk. This is helpful to suggest in the management (antiplatelet vs. anticoagulation) and has a pivotal role in programming surgery.

57 Undifferentiated heart sarcoma in young adult: from differential diagnosis to management

A 34-year-old patient arrived in Emergency Department (ED) with a history of haemoptysis, fever, and night sweats. Echocardiographic examination revealed a large isoechoic thickening that totally encompassed posterior mitral leaflet and which extended contiguously, both inferiorly with subvalvular apparatus with chordal fusion, and superiorly up to left atrial wall. This alteration caused a moderate mitral stenosis with an estimated average gradient of 10–15 mmHg (with possible overestimation due to temporary state of hyperdynamic circulation secondary to anaemization). There also was an anteriorly directed, eccentric jet of mitral regurgitation (2 +/4 + grade).Differential diagnosis of the aforementioned mitral formation included infectious etiology (endocarditic vegetation), pure phlogistic (inflammatory/rheumatic valvulitis), aseptic vegetation, and thrombosis. Transesophageal echocardiographic evaluation showed the extension of the mass into posterior leaflet, the latter completely englobed from commissure to commissure, and cranially adhered to posterior wall of left atrium with estimated dimensions of 1.9 × 12 mm; inferiorly, contiguity with diffusely thickened subvalvular apparatus and chordal fusion, was appreciated. Resulting stenosis was about 13–14 mmHg. Planimetric mitral valve area was estimated to be about 1 cm with associated mild-moderate regurgitation. Global systolic function was preserved with normal segmental kinesis and without significant anomalies affecting other valves. On cardiac magnetic resonance (CMR) with contrast medium, known sleeve thickening of left atrium (maximum thickness 12 mm in lateral area and 7.5 mm at the level of atrial septum) was extended caudocranially for 2.5 cm in lateral area and for 3.2 cm in the side of the atrial septum and with subocclusion of left inferior pulmonary vein. An esophagogastroduodenoscopy (EGDS) was performed with biopsy examination and subsequent histological typing. It concluded for ‘undifferentiated pleomorphic sarcoma’ according to the WHO classification of thoracic tumours. In the stomach there was a diffuse infiltration of lamina propria by atypical, pleomorphic, and large cellular elements. Following cancer evaluation, first-line chemotherapy with ifosfamide and doxorubicin was undertaken. Two days later, due to finding of hyperpyrexia, with a feverish peak of up to 39°, infusion of chemotherapy was interrupted and empiric antibiotic therapy (piperacillin tazobactam) was started. Blood and urine cultures were carried out with search for antigens of legionella and pneumococcus, (MRSA), fungi, and respiratory viruses but all of them were negative for active infection. The following day, an episode of acute respiratory failure occurred, so we performed an urgent chest CT with finding of pneumonia with bilateral pleural effusion and linezolid was started. Because of sudden worsening of clinical conditions, patient was transferred to ICCU (Intensive Cardiac Care Unit) with gradual resolution of desaturation. Cardiac ultrasound imaging, from the very first performed in ED, has been fundamental in documenting the presence of a mass in mitral valve. The timeliness in identifying first and then characterizing it certainly had a positive impact on cancer management, especially in such an aggressive neoplasm in a young patient. Furthermore diagnostic process, corroborated by instrumental data provided by ecocardiography, CT, MRI, PET, and scintigraphy, allowed a better staging of the disease and highlighted other organ involvement in order to manage optimal therapeutic approach.

486 A blue man standing

An 85-year-old was admitted in Internal Medic Ward for recurrent episodes of acute respiratory failure. His electrocardiogram showed a new onset of atrial flutter with spontaneous reversal to sinus rhythm (narrow QRS complex and normal atrioventricular conduction). The dyspnoea was worse with the patient sitting and was better when lying supine. Also, he report back pain associated to his major kyphoscoliosis. Results of chest radiography and blood test were normal. The arterial blood gas test revealed a hypocapnic hypoxemic respiratory failure, therefore the patient oxygen supplementation with noninvasive ventilation (NIV) was started, but peripheral oxygen saturation was persistently below 88% despite the high flow oxygenation. The patient underwent nasopharyngeal (NP) swab (ruling out SARS-CoV-2), high-resolution computed tomography (which was not pathologic), and computed tomography angiography (excluding pulmonary embolism). A transthoracic echocardiography was performed showing concentric hypertrophy, left atriomegaly and severe aortic ectasia of the root and of his ascending part, normal pulmonary pression and an aneurism of the interatrial septum with the presence of right-to-left shunt after using agitate saline contrast with Valsalva maneuver. Transesophageal echocardiography (TTE) confirmed the presence of severe structural interatrial septal abnormality with wide left convex aneurysm and an atrial septum defect (ASD) as ostium secundum, causing severe bidirectional shunt. Therefore, the patient underwent a successful transcatheter closure of secundum ASD with device Amplatzer Septal Occluder 018, while monitored with cardiac catheterization and transesophageal echocardiography. After few days, the patient was discharged at home without oxygen therapy. At the 3-month follow-up visit he reported no respiratory symptoms. Platypnea-orthodeoxia syndrome (POS) is an uncommon disorder characterized by dyspnoea and hypoxemia that occurs when the patient is sitting or standing and disappear quickly when recumbent. POS is characterized by both an anatomic and a functional component. The anatomic element is typically an interatrial communication such as ASD or Patent Foramen Ovale, which cause blood shunt left-to-right due to a higher pressure in left atrium and a greater compliance of the right ventricle, in the presence of normal pulmonary artery pressure. The syndrome occurs when a functional element, such as an increased atrial pressure or a decreased compliance of the right ventricle, reverses the flow. The shunts are often small and could remain asymptomatic: the high left atrial pressure let the defect close until there is a reverse pression which stretched atrial septum in particular in the upright position. In fact, when the patient is standing, the inferior vena cava comes in line with the defect increasing the right-to-left flow, sparking the respiratory symptoms. This could be linked to a cardiac or an extracardiac condition; in this case the presence of kyphoscoliosis and severe aortic ectasia of the root and of his ascending part, played an important role. The diagnosis is mainly made by echocardiography and cardiac catheterization to verify the mismatch in oxygen saturation between the pulmonary vein and the aorta. The diagnosis of POS is challenging, often considered only after other possible diagnosis is excluded. When POS is triggered by an interatrial defect in the absence of severe pulmonary hypertension, the usual treatment is a percutaneous or surgical closure.

574 Giant left atrial myxoma in ischaemic stroke

Aims Primary cardiac tumours are rare; most are benign, and of these, around half are myxomas, often located in the left atrium. Clinical presentation is variable. Ischaemic stroke is a rare, although real and potentially fatal, complications of cardiac myxomas. Methods and results We present a case of a 51-year-old man, ex- smoker, obese, with a history of hypertension and COPD, presented to our emergency department with right-sided hemiplegia and aphasia caused by ischaemic stroke. Brain computed tomography revealed cerebral perfusion deficit. The patient was underwent intravenous thrombolytic strategy and intubated transferred in Reanimation. TTE showed a large left atrial mass attached to the interatrial septum, with a friable appearance, suggestive of myxoma. The 2D and 3D TEE detected a giant space-occupying mass (60 × 20 mm diameter) in the left atrium, coral-like, and with a friable appearance, the most apical portion prolapsed into the left ventricle during diastole, causing fixed obstruction to flow in the left ventricular inflow tract. Computed tomography angiography (CTA) confirmed the giant mass in the left atrium. In view of the risk of imminent embolization, the emergent surgical excision of the tumour was performed. The tumour and its neck, including part of the atrial septum, were fully resected and the atrial septum was directly closed. Subsequent histopathological findings confirmed the diagnosis of atrial myxoma. Post-operative echocardiography showed no residual tumour. Symptomatic cardiac myxoma may present with one of the three classic clinical presentations of the Goodwin’s triad, which include intracardiac obstruction, constitutional symptoms, and embolism. The obstructive pattern mimics mitral or tricuspid valve disease and results from atrioventricular valve obstruction. Constitutional or systemic manifestations include fatigue, fever, weight loss, arthralgia, myalgia, erythematous rash, and laboratory findings such as anaemia and elevated ESR, CRP, and globulins. Systemic embolization from myxoma occurs in around a third of cases. Of these, the most serious is cerebrovascular embolism, which may result in cerebral stroke. In addition, embolization of coronary arteries, kidneys, intestines, and extremities can also occur. The tumour size, location, and macroscopic appearance, along with mean platelet volume and platelet count, are closely associated with embolic events. Echocardiography remains the method of choice for diagnosis and morphological characterization of myxoma. Two patterns have been established by echocardiography: round, with a solid appearance and a firm surface, and polypoid, with an irregular outline and a friable surface. The incidence of systemic embolization is higher in those with an irregular and friable surface, as well as in polypoid tumours and those that prolapse into the ventricle. Once a diagnosis of myxoma is established, surgical resection is the only effective treatment and should be performed immediately, in view of the risk of embolic complications. Conclusions With this case report, we stress the fundamental importance of echocardiography in new onset of neurological deficit to prevent potentially fatal outcomes.

Leftward deviation of the primary septum or dividing left atrial shelf?

Isolated leftward prolapse or deviation of the primary atrial septum is a rare CHD that can mimic abnormal pulmonary venous return at first sight. We present a case of a newborn infant, referred for surgical correction of totally anomalous pulmonary venous return into the right atrium, with the peri-operative finding of a leftward deviation of the superior margin of the primary atrial septum. The distinction with a dividing atrial shelf could not be confirmed with certainty. Fifty-three similar cases from the literature are incorporated. A detailed review of the current account on atrial septation is studied. The embryological and clinical features of a dividing partition of the left atrium are discussed.

Radiofrequency Energy-Assisted Wire Atrial Septostomy to Easily Create a Large Atrial Septal Defect

Wire atrial septostomy, a new transcatheter technique, combines 2 atrial septal defects (ASDs) into a large one in a thickened atrial septum. Although the thin-wire-looped atrial septum between ASDs needs to be pulled deeply into a hard catheter to tear the septum, this action might harm the surrounding cardiac structures. We utilized radiofrequency wire energy to facilitate the tearing of the septum, making the procedure easier and safer. This technical article presents the modified procedure in detail; the procedure was performed in a 6-month-old boy with double outlet right ventricle and intact ventricular septum. Mini-abstract This technical article describes modified wire atrial septostomy for thickened atrial septum in detail. Radiofrequency energy facilitated a thin wire-loop in tearing the septum between 2 atrial septal defects to combine the defects into a large one. We believe that this transcatheter procedure is easier and safer than the original one not using a radiofrequency wire energy.