scholarly journals Diagnosis of Carney complex following multiple recurrent cardiac myxomas

Author(s):  
Shigeki Yokoyama ◽  
Kanetsugu Nagao ◽  
Akihiko Higashida ◽  
Masaya Aoki ◽  
Shigeyuki Yamashita ◽  
...  

AbstractCarney complex is a rare syndrome caused by a genetic mutation leading to multiple endocrine abnormalities and a variety of tumors. Here, we report a case of Carney complex diagnosed due to recurrent multiple myxomas in the right atrium of a patient 16 years after the resection of the primary left atrial myxoma. Surgical excision was performed for the multiple recurrent right atrial tumors under cardiopulmonary bypass. The patient remained complication-free after surgery and was discharged on the 14th day. He was scheduled to continue echocardiographic follow-up and periodic systemic review by an endocrinologist. This case emphasizes the fact that if cardiac myxomas tend to be multiple and recurrent at a relatively young age, the possibility of Carney complex should be considered, even in the absence of any other related feature other than cardiac tumors.

2021 ◽  
Vol 2021 ◽  
pp. 1-4
Author(s):  
Snigdha Bhatia ◽  
Amna Qasim ◽  
Amyn K. Jiwani ◽  
Ashraf M. Aly

Advances in imaging have resulted in more frequent reporting of primitive right atrial structures which can sometimes mimic cardiac tumors in prenatal ultrasound. Prominent crista terminalis and Chiari network are examples of these structures. We describe two cases of pregnant women referred to the fetal cardiology clinic for fetal echocardiography for right atrial masses seen on prenatal ultrasound suspicious of tuberous sclerosis. The first case subsequently diagnosed as crista terminalis and the second case as a prominent Chiari network. Postnatal ECHO confirmed the benign nature of these structures. It is important to differentiate tumors from prominent benign structures in the right atrium in fetal ECHO. The location and the similar echogenicity to the adjacent atrial tissue are clues for differentiation of these structures from atrial tumors.


2020 ◽  
Vol 4 (3) ◽  
pp. 201-204
Author(s):  
Yichao Xiao ◽  
Zhenfei Fang ◽  
Xinqun Hu ◽  
Qiming Liu ◽  
Zhaowei Zhu ◽  
...  

Cardiac myxomas, the commonest primary benign cardiac tumors, are extremely rare, with an incidence ranging from 0.0017 to 0.19% and only about one-fifth of them originating from the right chambers of the heart. A 60-year-old woman was admitted because of recurrent attacks of chest tightness and shortness of breath. Transthoracic echocardiography detected a giant mass in the right atrium; myxoma was indicated by [18F]fluorodeoxyglucose PET/CT. Preoperative selective coronary angiography was performed to assess the extent and severity of coronary stenosis, and showed a strongly neovascularized right atrial mass supplied by two feeding vessels with multiple branches from the left and right coronary arteries. The myxoma was successfully excised with open heart surgery and the patient was free of myxoma recurrence during the 3-year follow-up.


2016 ◽  
Vol 2016 ◽  
pp. 1-3 ◽  
Author(s):  
Marcos Danillo Peixoto Oliveira ◽  
Adriano Ossuna Tamazato ◽  
Fernando Roberto de Fazzio ◽  
Luiz J. Kajita ◽  
Expedito E. Ribeiro ◽  
...  

Primary cardiac tumors are rare and approximately half of them are atrial myxomas. They rarely remain asymptomatic, especially if large. The imaging of a myxoma by contrast dye during coronary angiography is an infrequent sign, which clarifies the vascular supply of the tumor. We report herein an interesting and rare case of a left atrial myxoma hypervascularized from the right coronary artery.


2018 ◽  
Vol 11 (4) ◽  
pp. NP129-NP131
Author(s):  
Sushil Azad ◽  
Nilanjan Dutta ◽  
Kuntal Roy Chowdhuri ◽  
Tarun Raina Ramman ◽  
Nishit Chandra ◽  
...  

Cardiac myxomas are the most common primary cardiac tumors and are typically attached to the interatrial septum. Left ventricular myxomas are exceedingly rare and presentation in children is all the more uncommon. We report a case of left ventricular myxoma with very atypical cystic appearance raising an initial suspicion of a hydatid cyst. Subsequently, cardiac magnetic resonance imaging was done, which ruled out the diagnosis of hydatid cyst. Complete surgical excision was done through transaortic and transmitral route. Histopathological examination revealed it to be a cardiac myxoma with vascular proliferation, which on echocardiography had appeared as a polycystic lesion. This is a very unusual histopathological presentation of cardiac myxoma.


2019 ◽  
Vol 8 ◽  
pp. 204800401881760 ◽  
Author(s):  
Amitabh C Pandey ◽  
John J Carey ◽  
Jess L Thompson

Primary cardiac tumors are typically benign, with myxomas being most common. We present a 32-year-old female with a chief complaint of dyspnea and a constant non-radiating chest pressure along the left sternal border. She was found to have a pulmonary embolism that was ultimately caused by embolization of a right atrial myxoma with remnants of a large, highly mobile mass attached to the right inter-atrial septum prolapsing through the tricuspid valve. The patient underwent a median sternotomy, right atrial mass resection, pulmonary embolectomy, and inter-atrial septum reconstruction using the patient’s pericardium. The importance of finding the etiology of initial diagnoses is stressed with long-term outcomes for patients.


2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
A M Buburuz ◽  
D T M Marcu ◽  
I Demsa ◽  
G Tinica ◽  
A Petris ◽  
...  

Abstract Primary cardiac tumors are very rare causes of intracavitary masses, about 5% of all cardiac tumors. Most of them are benign tumors, and 50% are represented by atrial myxomas. They are usually developed in the left atrium, with a pedicle attached to the interatrial septum. Cardiac myxomas are one of the "greatest mimes" of pathology, with a polymorphic clinical presentation, from obstructive, to embolic and constitutional clinical manifestation. We present the case of a 58-year-old female patient with 4 years history of chronic obstructive pulmonary disease (COPD), on bronchodilator treatment and long-term oxygen therapy. She accused two months duration of abdominal distension due to ascites, for which she was evaluated in the gastroenterology department and received diuretics association. The symptoms were refractory to the treatment, so the patient was referred for cardiovascular examination. At presentation, the patient was afebrile, with a heart rate of 100/minute, blood pressure was 125/90 mmHg. Cardiovascular examination did not reveal any murmurs, jugular venous distension was present. 12-lead electrocardiogram (ECG): sinus rhythm, with right atrial enlargement. The transthoracic echocardiography (TTE) and transesophageal echocardiography (TEE) revealed a lobulated giant mass in RA, which occupied almost the entire cavity, with prolapse towards the right ventricle during diastole. Due to the large intracavitary mass, the point of attachment could not be ascertained. Computed tomography (CT) of the thorax was performed and it confirmed the presence of the giant right cardiac mass. The patient underwent surgical excision of the tumor under cardiopulmonary bypass. Intraoperative findings were represented by a yellowish-brown lobulated friable mass in the right atrium cavity, measuring 70/30/35 mm. The pedicle of the tumor was inserted on the free wall of the right atrium. The histopathological examination revealed round to stellate cells in a myxoid stroma, specific for cardiac myxoma. Postoperative evolution was influenced by the severe pulmonary disease, with prolonged mechanical ventilation and positive inotropic support, but it was slowly favorable, and the patient was discharged in good condition. Right atrial myxoma is a very rare intracardiac tumor with nonspecific forms of clinical presentations, that create difficulties in diagnosis. Ascites represents an unusual form of manifestation in a patient with a cardiac tumor. The insertion of the pedicle of the myxoma was atypical and induced difficulties in early evaluation of the tumor type. Even though myxomas are mainly benign tumors, the complication rate is very high, especially of embolic events due to the friable tumoral mass, so they can become life threatening conditions. Early diagnosis of cardiac myxoma based on multimodality imaging is essential, leading to optimal surgical management, with good long-term survival. Abstract P854 Figure. TEE view of giant RA tumor


2018 ◽  
Vol 10 (03) ◽  
pp. 354-356
Author(s):  
Manjusha Karegar ◽  
Mrinal Sarwate ◽  
Kanchan Kothari ◽  
Amey Rojekar ◽  
Leena Naik

ABSTRACTCutaneous myxomas are rare benign neoplasms which are frequently associated with Carney complex (CNC). Although more than 500 cases of CNC are reported, there is no literature on cytologic diagnosis of Cutaneous myxomas. An 18-year-old male, with no significant family history, presented with multiple cutaneous swellings, largest measuring 15 cm on the right cheek. He also had spotty skin pigmentations, raised adrenocorticotropic hormone levels and recurrent cardiac myxomas. Fine-needle aspiration cytology from the right cheek and suprapubic swellings revealed paucicellular smears with abundant myxoid material in the background, admixed with fragments of spindle and stellate cells with bland nuclear morphology, and vascular proliferation in few fragments. There was no mitosis, necrosis, or any epithelial element. Hence, diagnosis of cutaneous myxomas in CNC was made which was confirmed on histopathology. This is the first report of cytologic diagnosis of multiple cutaneous myxomas in CNC and the largest cutaneous myxoma reported in literature.


2015 ◽  
pp. 28-3
Author(s):  
Prima Almazini ◽  
Bambang Budi Siswanto ◽  
Nani Hersunarti ◽  
Rarsari Soerarso ◽  
Amiliana M Soesanto

Cardiac myxomas are the most common primary cardiac tumors. Myxoma are more common in women. Clinical manifestations can mimic many cardiac and noncardiac conditions. Transthoracic echocardiography (TTE) is the gold standard method in the diagnosis of cardiac myxoma. The management of cardiac myxoma are medical therapy for the treatment of associated conditions and surgical removal as the definitive treatment.


2007 ◽  
Vol 135 (7-8) ◽  
pp. 401-406
Author(s):  
Aleksandar Mikic ◽  
Biljana Obrenovic-Kircanski ◽  
Mladen Kocica ◽  
Mile Vranes ◽  
Vesna Lackovic ◽  
...  

Introduction Cardiac myxomas are the most frequent primary tumors of the heart in adults, and they can be found in each of four cardiac chambers. Although biologically benign, due to their unfavorable localization, myxomas are considered "functionally malignant" tumors. Diagnosis of cardiac myxoma necessitates surgical treatment. Objective To analyze: 1) the influence of localization, size and consistency of cardiac myxomas on preoperative symptomatology; 2) the influence of different surgical techniques (left, right, biatrial approach, tumor basis solving) on early, and late outcomes. Method From 1982 to 2000, at the Institute for Cardiovascular Diseases, Clinical Center of Serbia, there were 46 patients with cardiac myxomas operated on, 67.4% of them women, mean age 47.1?16.3 years. The diagnosis was made according to clinical presentation, electrocardiographic and echocardiographic examinations and cardiac catheterization. Follow-up period was 4-18 (mean 7.8) years. Results In 41 (89.1%) patients, myxoma was localized in the left, while in 5 (10.9%), it was found in the right atrium. Average size was 5.8?3.8 cm (range: 1?1 cm to 9?8 cm) and 6?4 cm (range: 3?2 cm to 9?5 cm) for the left and right atrial myxomas, respectively. A racemous form predominated in the left (82.6%) and globous in the right (80%) atrium. Fatigue was the most common general (84.8%) and dyspnoea the most common cardiologic symptom (73.9%). Preoperative embolic events were present in 8 patients (4 pulmonary, 4 systemic). In our series: 1) different localization, size and consistency had no influence on the preoperative symptomatology; 2) surgical treatment applied, regardless of different approaches and basis solving, resulted in excellent functional improvements (63.1% patients in NYHA III and IV class preoperatively vs. 6.7% patients postoperatively) and had no influence on new postoperative rhythm disturbances (8.7% patients preoperatively vs. 24.4% patients postoperatively); 3) early (97.8%), and late survival rates (91.3%) were excellent; 4) there were no relapses during the follow-up period. Conclusion Localization, size and consistency had no influence on the preoperative symptomatology. Excellent survival rate with significant functional improvement, rare postoperative complications and no recurrences, justify the applied strategies of surgical approach and tumor basis solving in our series.


2015 ◽  
Vol 42 (3) ◽  
pp. 289-292 ◽  
Author(s):  
Basri Amasyali ◽  
Ayhan Kilic

Focal atrial tachycardia arising from the right atrial appendage usually responds well to radiofrequency ablation; however, successful ablation in this anatomic region can be challenging. Surgical excision of the right atrial appendage has sometimes been necessary to eliminate the tachycardia and prevent or reverse the resultant cardiomyopathy. We report the case of a 48-year-old man who had right atrial appendage tachycardia resistant to multiple attempts at ablation with use of conventional radiofrequency energy guided by means of a 3-dimensional mapping system. The condition led to cardiomyopathy in 3 months. The arrhythmia was successfully ablated with use of a 28-mm cryoballoon catheter that had originally been developed for catheter ablation of paroxysmal atrial fibrillation. To our knowledge, this is the first report of cryoballoon ablation without isolation of the right atrial appendage. It might also be an alternative to epicardial ablation or surgery when refractory atrial tachycardia originates from the right atrial appendage.


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