Heart Border Research Articles

Optimising central venous catheter placement by comparing cavoatrial junction position to chest X-ray landmarks: Across-sectional study using CT chest reconstruction.

Central venous catheter (CVC) tip placement guided by chest X-ray (CXR) landmarks is currently prone to inconsistency and malpositioning. This study aims to better define the relationship between the cavoatrial junction (CAJ) and selected X-ray landmarks. Chest CTs of 100 patients were retrospectively assessed. CT images were converted to a 'virtual CXR' using a digital workstation, enabling simultaneous localisation of the CAJ and evaluation of CXR landmarks. Vertical distances between the CAJ and selected landmarks were measured for each patient. Measurements were assessed for correlation with age and compared between age groups and sexes. The mean vertical distance of the following landmarks above the CAJ was found: the carina (46.2 mm), the intersection of the bronchus intermedius and the right heart border (7.6 mm) and the superior inflection of the right heart border (Sup-RHB) (13.0 mm). The maximum lateral bulge of the right heart border (Lat-RHB) was 18.4 mm below the CAJ. A new landmark: the mid-superior right heart border, defined as the mid-point between the Sup-RHB and Lat-RHB, was the closest to the CAJ, lying 2.6 mm below the CAJ. We propose that the CVC tip can be placed at the mid-superior right heart border landmark.

Enhancing transvenous lead extraction risk prediction: Integrating imaging biomarkers into machine learning models

BackgroundMachine learning (ML) models have been proposed to predict risk related to transvenous lead extraction (TLE). ObjectiveThe purpose of this study was to test whether integrating imaging data into an existing ML model increases its ability to predict major adverse events (MAEs; procedure-related major complications and procedure-related deaths) and lengthy procedures (≥100 minutes). MethodsWe hypothesized certain features—(1) lead angulation, (2) coil percentage inside the superior vena cava (SVC), and (3) number of overlapping leads in the SVC—detected from a pre-TLE plain anteroposterior chest radiograph (CXR) would improve prediction of MAE and long procedural times. A deep-learning convolutional neural network was developed to automatically detect these CXR features. ResultsA total of 1050 cases were included, with 24 MAEs (2.3%) . The neural network was able to detect (1) heart border with 100% accuracy; (2) coils with 98% accuracy; and (3) acute angle in the right ventricle and SVC with 91% and 70% accuracy, respectively. The following features significantly improved MAE prediction: (1) ≥50% coil within the SVC; (2) ≥2 overlapping leads in the SVC; and (3) acute lead angulation. Balanced accuracy (0.74–0.87), sensitivity (68%–83%), specificity (72%–91%), and area under the curve (AUC) (0.767–0.962) all improved with imaging biomarkers. Prediction of lengthy procedures also improved: balanced accuracy (0.76–0.86), sensitivity (75%–85%), specificity (63%–87%), and AUC (0.684–0.913). ConclusionRisk prediction tools integrating imaging biomarkers significantly increases the ability of ML models to predict risk of MAE and long procedural time related to TLE.

Right Atrial Lateral Wall Aneurysm

Right atrial aneurysm (RAA) is a rare congenital anomaly. It usually occurs due to dysplasia of the atrial myocardium or localized absence of pericardium. We present a case of asymptomatic RAA in an adolescent, which was detected incidentally because of a bulge in the right heart border.

Intra-cardiac motion detection catheter for the early identification of acute pericardial tamponade during invasive cardiac procedures.

To develop and test an intra-cardiac catheter fitted with accelerometers to detect acute pericardial effusion prior to the onset of hemodynamic compromise. Early detection of an evolving pericardial effusion is critical in ensuring timely treatment. We hypothesized that the reduction in movement of the lateral heart border present in developing pericardial effusions could be quantified by positioning an accelerometer in a lateral cardiac structure. A "motion detection" catheter was created by implanting a 3-axis accelerometer at the distal tip of a cardiac catheter. The pericardial space of 5 adult sheep was percutaneously accessed, and pericardial tamponade was created by infusion of normal saline. The motion detection catheter was positioned in the coronary sinus. Intracardiac echocardiography was used to confirm successful creation of pericardial effusion and hemodynamic parameters were collected. Statistically significant reduction in acceleration from baseline was detected after infusion of only 40 ml of normal saline (p < 0.05, ANOVA). In comparison, clinically significant change in systolic blood pressure (defined as >10% drop in baseline systolic blood pressure) occurred after infusion of 80 ml of normal saline (107 ± 22 mmHg vs. 90 ± 12 mmHg p = 0.97, ANOVA), and statistically significant change was recorded only after infusion of 200 ml (107 ± 22 mmHg vs. 64 ± 5 mmHg, p < 0.05, ANOVA). An intra-cardiac motion detection catheter is highly sensitive in identifying acute cardiac tamponade prior to clinically and statistically significant changes in systolic blood pressure, allowing for early detection and treatment of this potentially life-threatening complication of all modern percutaneous cardiac interventions.

Automatic cardiothoracic ratio calculation based on lung fields abstracted from chest X-ray images without heart segmentation.

The cardiothoracic ratio (CTR) based on postero-anterior chest X-rays (P-A CXR) images is one of the most commonly used cardiac measurement methods and an indicator for initially evaluating cardiac diseases. However, the hearts are not readily observable on P-A CXR images compared to the lung fields. Therefore, radiologists often manually determine the CTR's right and left heart border points of the adjacent left and right lung fields to the heart based on P-A CXR images. Meanwhile, manual CTR measurement based on the P-A CXR image requires experienced radiologists and is time-consuming and laborious. Based on the above, this article proposes a novel, fully automatic CTR calculation method based on lung fields abstracted from the P-A CXR images using convolutional neural networks (CNNs), overcoming the limitations to heart segmentation and avoiding errors in heart segmentation. First, the lung field mask images are abstracted from the P-A CXR images based on the pre-trained CNNs. Second, a novel localization method of the heart's right and left border points is proposed based on the two-dimensional projection morphology of the lung field mask images using graphics. The results show that the mean distance errors at the x-axis direction of the CTR's four key points in the test sets T1 (21 × 512 × 512 static P-A CXR images) and T2 (13 × 512 × 512 dynamic P-A CXR images) based on various pre-trained CNNs are 4.1161 and 3.2116 pixels, respectively. In addition, the mean CTR errors on the test sets T1 and T2 based on four proposed models are 0.0208 and 0.0180, respectively. Our proposed model achieves the equivalent performance of CTR calculation as the previous CardioNet model, overcomes heart segmentation, and takes less time. Therefore, our proposed method is practical and feasible and may become an effective tool for initially evaluating cardiac diseases.

A Case of 3Cs: Coronary Artery Calcification Masquerading as a Stent on Chest X-ray

Coronary artery calcification in patients with coronary artery disease is associated with increased major adverse cardiovascular events. They are usually diagnosed on a computed tomography scan or on an invasive coronary angiography. A 60-year-old male was diagnosed with acute coronary syndrome after presentation with one month of worsening angina. Coronary angiography showed triple vessel disease (mid left anterior descending artery and mid right coronary artery having total occlusion and a 70% stenosis in the left circumflex artery). Apart from this, there was a heavily calcified right coronary artery with a C configuration evident on fluoroscopy. A computed tomography coronary angiography was done to visualize the distal vessels. The calcium score was 5988. Retrospectively, on inspection of the chest X-ray, there was calcification on the right heart border. The patient was medically managed and referred to a cardiothoracic vascular surgeon for coronary artery bypass grafting. Coronary artery calcification on X-ray is rare and hence being presented along with the review of the literature.

Pericardial agenesis - the wandering heart

BackgroundCongenital pericardial absence is an uncommon cardiac anomaly that is typically asymptomatic and commonly misdiagnosed due to a lack of symptoms or atypical symptoms. Pericardial agenesis (PA) should be considered one of the differential diagnoses when the patient presents with chest pain. This case shows how the diagnosis of pericardial agenesis is made exclusively using multi-modality imaging, starting from findings in a basic chest radiograph to cardiac MRI, while also demonstrating the classic signs seen in this condition. Magnetic resonance imaging of the heart is the gold standard for determining the absence of pericardium in the prognosis.Case presentationA 32-year-old male who presented with chest discomfort and radiating pain to his back and left shoulder mimicking myocardial infarction with normal ECG and enzyme markers. A chest radiograph (taken 24 h apart) demonstrates the left lateral position of the heart and the bulging contour of the left heart border, a lucent area between the aorta and pulmonary artery. Subsequently, cardiac MRI reveals left pericardial agenesis.ConclusionsThis article provides insight into a rare differential to consider in a young patient presenting with chest discomfort. This case shows how the diagnosis of pericardial agenesis is made exclusively using multi-modality imaging, starting from findings in a basic chest radiograph to cardiac MRI, while also demonstrating the classic signs seen in this condition.

PO-03-049 SUBACUTE MIGRATION OF LEFT BUNDLE BRANCH PACING LEAD INTO THE LEFT VENTRICLE

RV pacing is associated with increased risk for systolic dysfunction. Conduction system pacing has emerged as an alternative. His bundle pacing is limited by higher capture threshold, reducing battery life, and concerns of increasing thresholds over time. LBBP is a promising alternative, however limited data exist on associated complications. We report a case of LBBP complicated by subacute migration into the LV presenting with increasing threshold. A 78 yoM with symptomatic bradycardia and chronotropic incompetence referred for PPM underwent implantation of DC-PPM with left bundle lead. A Medtronic SelectSecure 3830 lead was advanced into a C315 sheath and positioned at the LV septum. Pace mapping defined a candidate site for LBBP (W in V1). Lead was screwed into the septum. Unipolar impedance prior to deployment was 820Ω. After 5 turns paced QRSd 140ms with qR morphology in lead V1, R waves 12.4mV, unipolar impedance 760Ω, threshold 0.75V at 0.4ms. This was accepted as nonselective LBBP. He began to have chest thumping on POD 3. Device showed elevated ventricular threshold, decreasing impedance, and stable sensing. ECG showed broad notched R wave in V1 consistent with LV pacing. CT revealed migration of the lead, coursing through the IVS into the LV myocardium with tip terminating along the inferior LV wall. Lead tip was possibly puncturing through the myocardium and within the epicardial fat. There was no effusion Lead was removed with manual traction in the EP suite. Heart border remained unchanged on fluoroscopy. Transthoracic echo showed no effusion. New lead was positioned with active fixation in the RV apical septum away from the prior position. Lead showed suitable parameters without diaphragmatic stimulation. LBBP can be an alternative to HB, RV, and CRT pacing. LBBP involves placing the lead deep within the IVS and close to the LV subendocardium. Septal perforation is often recognized during the procedure, however, the risk of late perforation must be considered. In our case, timing of lead migration is unclear as parameters during implant suggested adequate LB capture but reported diaphragmatic capture at day 3, before threshold increase. It demonstrates important findings:1.Acute decrease in unipolar impedance may not be a sensitive marker for septal perforation as it did not change appreciably in our case; 2.ECG/CXR in follow up can help identify lead migration; 3.Migrated leads can be safely removed in the EP suite without need for surgical backup

Breaking the Heart's Borders: From “Dark Anthropology” to Dark Humor in the Telling of Migration

Data sharing not applicable to this article as no datasets were generated or analysed during the current study.

Association between radiotherapy protocol variations and outcome in the CONVERT trial

BackgroundRadiotherapy quality assurance (QA) is integral to radiotherapy delivery. Here we report comprehensive contouring, dosimetry, and treatment delivery QA, describe protocol compliance, and detail the impact of protocol variations on acute grade ≥3 toxicity, progression free survival (PFS), and overall survival (OS) in the phase III CONVERT trial. Materials/MethodsRadiotherapy planning data from one hundred randomly selected patients were requested. Members of the CONVERT Trial Management Group (TMG) recontoured the heart, lung, and spinal cord organs at risk (OAR) according to the trial guideline. The existing radiotherapy plan were re-applied to the new structures and the new dosimetric data were recollected. Compliance with radiotherapy QA components were recorded and radiotherapy QA components were pooled into protocol variations: acceptable, acceptable variation, and unacceptable variation. Univariable analysis with a Cox proportional hazards model established the relationship between protocol variations and patient outcome. ResultsNinety-three cases were submitted for retrospective radiotherapy QA review. Demographics of the radiotherapy QA cohort (n=93) matched the non-QA (n=450) cohort. 97.8% of gross tumour volume (GTV) contours were protocol compliant. OAR contours were non-compliant in 79.6% instances of the heart, 37.6% lung, and 75.3% spinal cord. Of the non-compliant heart contours, 86.5% and 2.7% had contours caudal and cranial to the protocol-defined heart borders. 10.8% did not include the pericardial sac and 2.7% did not include the anterior aspect of the pericardium. Eleven (11.8%) submissions exceeded protocol-defined dosimetric heart constraints; six of which were only noted on the application of protocol-compliant contours. Unacceptable variations were not associated with an increase in grade 3 toxicity (p=0.808), PFS (p=0.232), or OS (p=0.743). ConclusionNon-protocol compliant heart contours were associated with increased dose delivered to the heart OAR, with 11.8 % of submitted heart structures exceeding protocol-defined constraints. In this QA cohort of patients with small cell lung cancer, unacceptable variations were not associated with acute grade ≥3 toxicity, PFS, or OS. Radiotherapy QA remains the cornerstone of high-quality radiotherapy delivery and should be embedded into clinical trial and non-clinical trial practice; clinical trials should report standardised radiotherapy QA parameters alongside trial outcomes.

AORTOCORONARY SAPHENOUS VEIN GRAFT ANEURYSM: A SILENT COMPLICATION

AORTOCORONARY SAPHENOUS VEIN GRAFT ANEURYSM: A SILENT COMPLICATION

A Rare Cause of Severe Biventricular Dyssynchrony During Venoarterial Extracorporeal Membrane Oxygenation for COVID-19 Respiratory Failure

A Rare Cause of Severe Biventricular Dyssynchrony During Venoarterial Extracorporeal Membrane Oxygenation for COVID-19 Respiratory Failure

Chest X-ray features facilitate screening for pulmonary hypertension caused by fibrosing mediastinitis

Misdiagnosis and underdiagnosis of pulmonary hypertension caused by fibrosing mediastinitis (PH-FM) are considerably prevalent due to unspecific symptoms and as well as the lack of awareness of this fatal disease. The aim of this study was to evaluate the diagnostic accuracy of the chest X-ray (CXR) for screening the patients with PH-FM from those with pulmonary hypertension (PH). This was a retrospective observational cohort study. The patients with suspected PH were recruited between October 2014 and October 2020. All the clinical data and CXR findings were collected. The sensitivity, specificity, and likelihood ratio of the CXR features were calculated. Logistic regression was used to identify the factors associated with the CXR characteristics and FM and to generate a prediction model. Finally, the diagnostic efficiency of the prediction model was evaluated using nomogram and internal validation. The patients with PH-FM (n = 36) and PH caused by the diseases other than FM (PH-non-FM, n = 62) were enrolled. The CXR features, including atelectasis, pleural effusion, consolidation, nodules, calcification, interlobular septal thickening, and interstitial reticulation, were more prevalent in patients with PH-FM than in those with PH-non-FM (all p < 0.05). Atelectasis had a specificity of 97%, a sensitivity of 50%, and a greater accuracy for diagnosing of PH-FM [area under the curve (AUC) = 0.720; 95% CI: 0.634-0.806] than the other factors did. The combination of tuberculosis, natural logarithmic NT-proBNP (lnBNP), atelectasis, pleural effusion, and prominent right heart border constituted a prediction model to distinguish the PH-FM from the PH-non-FM, with a sensitivity of 91.7% and a specificity of 83.9%. The model demonstrated good prediction performance by showing an AUC of 0.922 (95% CI: 0.861-0.983) in the internal validation. In this study, atelectasis was the most specific and accurate CXR characteristic for identifying PH-FM in the PH patients. The combination of atelectasis, pleural effusion, prominent right heart border, tuberculosis, and lnBNP constituted a prediction model that distinguished the PH-FM patients from the PH-non-FM ones with good performance.

A RARE ASYMPTOMATIC LEFT BOCHDALEK HERNIA IN AN ADULT WITH ALLERGIC RHINOSINUSITIS: A CASE REPORT

Bochdalek hernias are extremely rare in adults with only a few cases known worldwide. This is a case of 50-year-old, male who presented at Ear, Nose and Throat clinic of our hospital with symptoms of odynophagia, sore throat, unproductive cough, sneezing, facial fullness and headache. No associated other systemic symptoms. The nasal examination revealed engorged inferior turbinates in the nose bilaterally with septal spur to the left. Chest X-ray done showed a rounded opacity of a soft tissue mass in left middle to lower zone; middle mediastinum adjacent to left lower heart border. Computerised Tomography scan showed a fairly oval hypodense non-enhancing soft mass seen in the left hemithorax with conclusion of part of omentum in the chest due to Bochdalek hernia. He was given treatment for the allergic rhinosinusitis. Due to non-availability of cardiothoracic surgeon in our hospital, the patient was counselled on the problem and danger that may arise and asked to see a cardiothoracic surgeon in the country’s capital city for possible repair of the defect.

Pulmonary Arteriovenous Malformations in a 18-months-old Child: A Case Report

Pulmonary Arteriovenous Malformation (PAVM) is a cardiovascular anomaly in which an anatomic communication between the pulmonary artery and pulmonary vein is formed, resulting in an additional cardiac shunt from the right to left side. PAVM is a rare disease, reported more in females, but in newborns comparatively higher in males. Cases can vary from asymptomatic to symptomatic such as having dyspnoea, hypoxia, cyanosis, neurological symptoms. They are mostly congenital having association with Hereditary Haemorrhagic Telangiectasia (HHT). Herby, author present a case of 18-months-old female was admitted with peripheral cyanosis in the Paediatric Department. Her saturation was 76%, after admission she received three doses of salbutamol nebulisation and was kept on 3 litre oxygen until her saturation increased to around 90%. On auscultation, a murmur of grade 2/6 heard in the left axilla with clear lung fields. Chest X-ray detected a prominent lobulated opacity in the left sided lung adjacent to the heart border. An echocardiogram was done which turned out normal. Thoracic Computed Tomography (CT) scan with contrast showed a vascular malformation in the apical region of the left lower lobe. Pulmonary angiography showed multiple vascular lesions in the peripheries which was consistent with arteriovenous malformation. The child was diagnosed with PAVM. A transcatheter embolisation and coil closure of AVM was performed and postprocedure the child was stable.

LV Apical Aneurysm with Apical Ventricle Septal Rupture Following Anterior Wall Infarction

A 49-year-old female patient presented with complaints of progressively increasing shortness of breath and reducing exercise tolerance since the last 4 days. Patient had an anterior wall myocardial infarction 11 days ago and was discharged following an uneventful stay in the coronary care unit. Upon presentation an X-ray was done by the E.R physician, which showed bilateral pleural effusions with focal lateral bulge arising from the left lower heart border, this raised suspicion of ventricular aneurysm.

SILENT GIANT LEFT ATRIUM

TOPIC: Cardiovascular Disease TYPE: Global Case Reports INTRODUCTION: Enlarged LA is a common finding in severe mitral stenosis(MS) and severe mitral regurgitation(MR). It can enlarge to giant proportions. The unusual finding in a thirty-year-old woman presenting with only mild dyspnea despite a hugely dilated LA prompted us to report this case CASE PRESENTATION: A thirty-year-old woman presented to the outdoor clinic with the complaint of dyspnea on exertion NYHA class I. Aside from mild shortness of breath during the past year, she had been asymptomatic all her life. The admission electrocardiogram showed atrial flutter with the variable block while her chest roentgenogram showed marked cardiac enlargement. The right heart border showed a double contour of atrial shadow (Fig.2). An echocardiogram showed a giant left atrium measuring 14.4 X 16.7 cm that encroached on the other cardiac chambers (Fig.3). Thickened mitral valves along with doming and restricted motion of anterior and posterior mitral leaflets were diagnostic of rheumatic heart disease(RHD) It was surprising to find a relatively asymptomatic patient despite the marked distortion of cardiac anatomy. DISCUSSION: Giant LA occurs due to many reasons but most commonly its result from long-standing rheumatic mitral valve regurgitation or mixed mitral valve disease with predominant regurgitation. Two factors implicated in its development are increased LA pressure and weakening of the LA wall by Rheumatic Pancarditis. When mitral valve disease persists for long, it leads to LA enlargement as a compensatory mechanism to ease the markedly increased LA pressure which could lead to pulmonary Congestion due to backpressure hemodynamic and hence protect from the development of pulmonary hypertension. These compensatory mechanisms help patients survive asymptomatically for a long time. Giant LA can lead to various complications including thromboembolism, pulmonary edema, pulmonary hypertension and it can compress the esophagus and airway causing Dysphagia. CONCLUSIONS: Progressive LA enlargement might be undetected and well-tolerated for a long time, even when it reaches massive proportions. When the LA enlarges to a gigantic size, a chest x-ray is insufficient to delineate the cause of cardiomegaly and even can be misleading. An echocardiographic study is the most useful method for evaluating these patients. Our case was unusual because the giant LA distorted the cardiac structures. This led to striking cardiomegaly on the chest x-ray, and it confused interpreting the cause of the enlarged cardiac silhouette. The echocardiogram was very helpful in demonstrating the severe MS and the giant LA. There is a poor correlation between the size of the left atrium and the severity of the MS. REFERENCE #1: Kawazoe K, Beppu S, Takahara Y, Nakajima N, Tanaka K, Ichinashi K, Fujita T, Manabe H: Surgical treatment of giant left atrium combined with mitral valvular disease. Plication procedure for reduction of compression to the left ventricle, bronchus, and pulmonary parenchyma. J Thorac Cardiovasc Surg 1983, 85: 885-892. REFERENCE #2: Hurst W: Memories of patients with a giant left atrium. Circulation2001, 104: 2630-2631. 10.1161/hc4701.100775 REFERENCE #3: Plaschkes J, Broman JB, Mercin G, Milwidsky H: Giant left atrium in rheumatic heart disease: a report of18 cases treated by mitral valve replacement. Ann Surg 1971, 174: 194-201. 10.1097/00000658-197108000-00004 DISCLOSURES: No relevant relationships by Vikash Jaiswal, source=Web Response no disclosure submitted for Akshyaya Pradhan; No relevant relationships by Prachi Sharma, source=Web Response No relevant relationships by Shweta Vohra, source=Web Response

Evaluation of the feasibility of explainable computer-aided detection of cardiomegaly on chest radiographs using deep learning

We examined the feasibility of explainable computer-aided detection of cardiomegaly in routine clinical practice using segmentation-based methods. Overall, 793 retrospectively acquired posterior–anterior (PA) chest X-ray images (CXRs) of 793 patients were used to train deep learning (DL) models for lung and heart segmentation. The training dataset included PA CXRs from two public datasets and in-house PA CXRs. Two fully automated segmentation-based methods using state-of-the-art DL models for lung and heart segmentation were developed. The diagnostic performance was assessed and the reliability of the automatic cardiothoracic ratio (CTR) calculation was determined using the mean absolute error and paired t-test. The effects of thoracic pathological conditions on performance were assessed using subgroup analysis. One thousand PA CXRs of 1000 patients (480 men, 520 women; mean age 63 ± 23 years) were included. The CTR values derived from the DL models and diagnostic performance exhibited excellent agreement with reference standards for the whole test dataset. Performance of segmentation-based methods differed based on thoracic conditions. When tested using CXRs with lesions obscuring heart borders, the performance was lower than that for other thoracic pathological findings. Thus, segmentation-based methods using DL could detect cardiomegaly; however, the feasibility of computer-aided detection of cardiomegaly without human intervention was limited.

English

BACKGROUND The heart is a pair of valved muscular pumps combined in a single organ. For the proper functioning of the heart, all valves should be intact. Mitral valve (MV) prolapse and regurgitation is the main cause of MV replacement. The dimensions of mitral valve and the cusps vary from person to person. We wanted to measure the average size of the valve components with respect to the annulus in the cadavers of South Odisha region, which would help in the selection of prosthetic valve in cardiac surgery. METHODS This comparative study was carried out on 58 adult cadaveric human hearts. Left atrium was opened along the left border of heart so as to expose the mitral orifice. Parameters of different components ofthe valve were measured by using appropriate instruments. RESULTS The mean annular circumference of the mitral valve was found to be 8.84 ± 1.24 cm; The annular attachment and height of anterior cusp were 2.94 ± .81 cm and 2.55 ± 0.27 cm respectively. The annular attachment and height of posterior cusp were 4.52 ± 0.78 cm and 1.2 ± 0.17 respectively. The annular area was found to be 5.22 ± 1.13 cm2. The combined cusp area was found to be 9.38 ± 2.32 cm. CONCLUSIONS The size of mitral valve parameters in the South Odisha region were found to be less compared to other studies. This study might help cardio-thoracic surgeons as well as the prosthetic valve manufacturing companies for the rough estimation of the mitral valve size. KEY WORDS Mitral Valve, Anterior Cusp, Posterior Cusp, Annular Circumference, Annular Valve Area and Combined Cusp Area

ACTH-Producing Thymic Carcinoid: A Rare Cause of Cushing’s Syndrome

Background: Thymic carcinoids are rare neoplasms that account for less than 5% of all thymic tumors. Approximately 25% of these tumors will result in Cushing’s syndrome due to ectopic ACTH secretion. These tumors can also be associated with MEN1 syndrome. This is a case report of a patient with history of macroprolactinoma now presenting with Cushing’s syndrome due to ectopic ACTH production from a thymic carcinoid tumor. Clinical Case: This is a 57 year old male with history of pituitary macroprolactinoma diagnosed in 2011, now status post transsphenoidal resection and external beam radiation therapy, with persistent hyperprolactinemia on cabergoline, who presented to our clinic for a routine follow up visit. Patient had already developed secondary hypogonadism and secondary hypothyroidism as a consequence of treatment for the macroprolactinoma. He complained of worsening fatigue and weight gain ongoing for several months. Laboratory studies revealed an hemoglobin A1c of 8.3% (nl < 5.7%), TSH 0.24 MIU/L (0.4-4.5 MIU/L), free T4 1.2 ng/dL (0.8-1.8 ng/dL), 8 AM cortisol 31.4 mcg/dL (4-22 mcg/dL), ACTH 185 pg/mL (6-50 pg/dL), prolactin 29.6 ng/mL (2-18 ng/mL), IGF-1 88 ng/mL (50-317 ng/mL). Follow up labs confirmed cushings syndrome: cortisol AM-DST 36.4 mcg/dL (< 2 mcg/dL), free urinary cortisol 291.9 mcg/24h (2-50 mcg/24h). Pituitary MRI showed empty sella turcica. Cortisol after an 8 mg DST 32.5 mcg/dL (< 5 mcg/dL). CT chest, abdomen and pelvis revealed an heterogeneously enhancing solid anterior mediastinal mass measuring 4.9 x 3.1 x 4.3 cm. Whole body OctreoScan showed a markedly hyperintense large mass adjacent to the right heart border measuring 47 x 32 mm. He was referred to cardiothoracic surgery and underwent a right video-assisted thoracic surgery with resection of the anterior mediastinal mass. Pathology revealed a thymic well-differentiated neuroendocrine tumor with strong cytoplasmic staining for ACTH. It was also positive for OSCAR, Cam5.2, synaptophysin, CD56, and S100. Ki67 stain was positive in fewer than 1% of tumor cells. Final diagnosis was carcinoid tumor. Conclusion: Cushing’s syndrome secondary to ectopic ACTH secretion from a thymic carcinoid is rare. The presence of two MEN1-associated tumors in this patient, macroprolactinoma and thymic carcinoid, is highly suggestive of a clinical diagnosis of MEN 1.