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Abstract
Background: Myxomas are the most common primary cardiac tumors that develop mostly at the atrial chambers of the heart and represent 0,25% of all cardiac diseases.
Methods: This is a retrospective study aiming to analyze epidemiological and intraoperative data from cardiac myxoma cases in the hospital of the last 32 years. The study population was 145 cardiac surgical patients and was divided into 4 certain 8-year periods. 87,6% of cases had the myxoma located at left atrium and 97,2% of all patients fully recovered. 4,1% of patients relapsed and underwent a redo operation.
Results: Mean CPB time and mean ICU length of stay increased during the 8-year periods (p < 0,001, P < 0,001, P = 0,002 and P = 0,003 respectively). In-hospital length of stay decreased to 5 days in the most recent period (p < 0,001). Cases significantly increased to 54 in the last 8-year period (p = 0,009).
Conclusion: Improvement on cardiac imaging and a better accessibility may drive patients to earlier and safer diagnosis of myxomas preventing any deterioration of their condition. Improvement on postoperative care can also reduce in-hospital length of stay. Surgical excision is the treatment of choice and guaranteed survival at 97,2% of patients.
Keywords: Cardiac imaging techniques, cardiopulmonary by-pass, in-hospital length of stay, left atrium myxoma
How to cite this article: Introduction 
Myxomas are the most common primary cardiac tumors that develop mostly at the atrial chambers of the heart. They have been firstly described in 1952 in an antemortem angiocardiography.[1] They were always known to be a relatively 'safe' and 'under control' tumor because of its 'benigness'. Nevertheless, their existence may be unraveled quite belatedly upon the manifestation of symptoms which will 'trigger the alarm' for urgent imaging and critical care interventions.
Epidemiology
Myxomas represent 0,25% of all cardiac diseases. Even though their incidence per year does not exceed 1 case per million of subjects,[2] myxomas are known to be the most frequent primary tumors found in adults and represent more than 50% (up to 85%) of all benign tumors.[1],[3] On epidemic perspective, myxomas appear more frequently on females at a ratio of 3:1[2] and they have also been classified in two certain epidemiological types; familial and sporadic. The sporadic type represents nearly 95% of all cases[3] and affects mostly women of middle age. Regarding this type, the tumor is unique, encircled and localized specifically in the left atrium. The familial type affects more frequently younger men with multiple tumors and may be found in other cardiac chambers as well.
The familial type appears initially with autosomal dominant heredity and may be documented at Carney syndrome as well. Carney syndrome was firstly described in 1985 and combines both cardiac and non-cardiac symptoms. Clinical manifestations of dermal pigmentation (itching, brown staining with white spots) and development of endocrine tumors (Cushing syndrome, fibroadenoma at breast, acromegaly, testicular cancer) have also been documented.[4] Carney complex is a multiple neoplastic syndrome triggered by the PRKAR1A gene. This gene deactivates mutations which relate to17q22-q24 chromosome. These genes encode cAMP (cyclic AMP) from dependent protein kinase A. Nevertheless, their main function still remains unclear.[5]
Left atrial myxomas
Left atrium[6] or mitral annulus[7] are the most common cardiac sites of myxoma development, which is quite often accompanied by constitutional manifestations. Cases have been reported where myxomas were incidentally diagnosed by ultra sound scanning (u/s s) as a large asymptomatic mass. In addition, extremely rare is a reported case where coronary blood flow steal occurred because of a highly vascularized myxoma with coronary arteries.[8] Regarding the manifestation of symptoms, Goswami et al.,[9] reported on their study that 80% of patients with cardiac myxoma developed dyspnea. Other symptoms involve palpitation, syncope, oedema and chest pain. However, left atrial myxomas may rarely become oligosymptomatic.[6] Nearly 30% of patients develop signs and symptoms which relate to secondary emboli.[9],[10] These patients are often misdiagnosed with mitral valve disease, tricuspid valve disease or ischemic cardiopathy.
Right atrial myxomas
The second most common site of cardiac myxoma development is the right atrium, especially the atrial septum followed by vena cava.[7] Most of right atrial myxoma patients are found to be asymptomatic.[11] Similarly with left atrial myxomas, right atrial myxomas may trigger obstructive symptoms which may lead to intracardiac complications regarding blood flow obstruction and embolic symptoms – either pulmonary of systematic.[12],[13]
Material and Methods Aim
The aim of this study is to review our experience with cardiac myxomas over a 32-year period by analyzing and comparing the demographic and intraoperative data of patients who underwent cardiac surgery for myxoma excision at a single-center, in order to document the variation of these data through the years. Furthermore, we tried to determine whether there had been an increase in the number of cases diagnosed throughout the 32-year period.
This is a retrospective non-experimental study. The study population consists of 145 male and female patients, who underwent cardiac surgery with cardiopulmonary bypass (CPB) and myxoma excision. They were admitted in the cardiac surgery ICU of a tertiary hospital in Athens during January 1986 to December 2018. Regarding the left atrial myxomas, a transseptal approach with surgical excision was performed. Myxomas that developed right onto the atrial septum, were removed with a wide excision of the septum. As far as right atrial myxomas are concerned, an incision of the right atrium followed by excision of the myxoma along with excision of the atrial septum where needed, was performed. In any other case the myxoma was excised thoroughly through a wide incision of the endocardium.
Five patients with recurrent myxomas underwent surgery, three of them had the operation in another center before 1986, so there are no available data. The fourth case suffered from a recurrent myxoma twice and underwent surgery (4/87, 11/91) in our center, while the fifth patient had her first surgery in another center in 2009 and underwent a redo surgery in our center in 2014. The criteria for a redo surgery was the reappearance of the myxoma, as diagnosed through the recommended follow up echocardiography. The surgical technique followed throughout the whole study period was the same by all the cardiac surgeons of our center with minor modifications, concerning the use of different type of cardioplegia.
Recurrence of myxomas is not rare, especially among younger ages. The follow up protocol applied in our center regarding recurrent myxomas consisted of conducting blood tests (full blood count, clotting, basic metabolic panel), chest x-ray and u/s a month after the operation, followed by ultrasound check every three months during the first year, and then on annual basis. Furthermore, a baseline ultrasound check was recommended for all the 1st degree relatives of the patients. Practically, the follow up period for these patients lasts for a year after surgery, and whenever any symptom, distress or any other recurrence would appear, the patient would be examined by a cardiologist and once again treated in our center. Recurrent myxomas are excised using the suggested surgical technique according to the site of development, as they tend to reappear anywhere within the heart chambers.
The inclusion criteria were a) Age >18 years, b) Informed consent of patients.
Ethics
The study was conducted upon written approval of the ethics committee of the hospital prior to the start of the study. (Permission ID: 38/12-03-2015). Data collection was obtained with the use of hospital data base and patients' medical files. Surgical data from 145 patients with confirmed cardiac myxoma cases that underwent surgical excision during 1986-2018 were gathered. The investigation was carried out in accordance with the ethical standards and the Greek legislation on Bioethics and following the tenets of the Helsinki Declaration of 1975, as revised in 2013. Precautionary measures were taken in order to protect the privacy of the patients and the confidentiality of their personal data, in order to use the minimum amount of information needed for the completion of the study.
Statistical analysis
The statistical analysis was performed with SPSS version 22.0. Analysis of independent variables such as sex, age, recurrence of the disease, specific site of myxoma development, cardiopulmonary bypass time, ischemia bypass time, use of intra-aortic balloon pump, Intensive Care Unit (ICU) length of stay, in-hospital length of stay and survival was conducted. For the comparison of proportions Chi-square and Fisher's exact tests were used. All reported P values are two-tailed and the statistical significance was set at P value ≤ 0.05.
Results The study population consists of 145 patients who underwent surgical excision of cardiac myxoma under CPB during the period 1986-2018. Demographic and intraoperative data of patients are demonstrated on [Table 1].
62,1% of patients were women. Mean age of patients was 57,9 years (SD = 14,5 years). 6 of the patients were re-operated (Redo) (4,1%). Mean ischemia time was 40 min (SD = 21,3 min) and mean CPB time was 68,5 min (SD = 30,2 min). IABP was used in one patient (0,7%). On [Table 2] intraoperative ischemia and CPB time are demonstrated depending whether the patients have been re-operated or not.
Table 2: Intraoperative Ischemia and CPB Time are Demonstrated Depending Whether the Patients have been Re-Operated or NotIschemia and CPB time were similar for those who underwent a Redo operation and for those who did not.
The average age of patients that underwent redo excision of cardiac myxoma was younger (37,7 years) compared to those who did not (57,9 years), as they were diagnosed with cardiac myxoma for the first time at a younger age. In-hospital and ICU length of stay and survival outcome are demonstrated on [Table 3].
Median ICU length of stay was 2 days (1-2 days) and in-hospital mean length of stay was 6 days (SD = 2,1 days). 2,8% of the patients died.
On [Table 4], total cases per year are demonstrated.
It was also found that the incidence of cases per year was similar throughout the whole study period (p = 0,105).
A statistical significance among the 8-year study periods regarding the incidence cases (p = 0,009) is documented. To be more specific, more cases have been documented during the period 2010-2018 compared to previous periods.
On [Table 5] the demographic perioperative and postoperative data are demonstrated as they have been documented per each 8-year period separately.
According to statistical analysis, the distribution among sexes and survival outcome were found to be similar among the 8-year periods (p > 0,05). Age, ischemia time, CPB time and ICU length of stay were significantly increased throughout the 8-year periods (beginning from the older period and up to the most recent, P < 0,001, P < 0,001, P = 0,002 and P = 0,003 respectively). On the contrary, the in-hospital length of stay decreased up to the most recent period (p < 0,001).
Discussion Cardiac myxoma is a rare disease which represents 0,25% of all cardiac diseases.[14] The incidence per year doesn't exceed 0,5 to 1 case per million of subjects.[2] In our research, the demographic and intraoperative data of 145 patients who underwent surgical excision of cardiac myxoma during the period 1986-2018 were analyzed statistically. We documented that intraoperative mean ischemia time was 40 minutes and mean CPB time was 68,5 minutes. We noticed a substantial difference from Gür's and Aykaç's[15] study surgical time. In their study, ischemia time was 23.6 minutes and CPB time was 35.4 minutes on a study population of 23 cases. We divided our 32-year study period into 8-year certain periods, as we wanted to highlight any differences documented regarding patients' hospitalization during this whole period. An interesting finding was that age, ischemia time, CPB time and ICU length of stay presented a gradual increase throughout the 8-year periods (beginning from the older period and up to the most recent, P < 0,001, P < 0,001, P = 0,002 and P = 0,003 respectively).
On the contrary, the in-hospital length of stay decreased up to the most recent 8-year period (mean value, 5 days), (p < 0,001). The explanation of these findings could be the fact that those patients who were operated during the most recent 8-year period had more comorbidities and were in a more unstable condition when they ended up having cardiac surgery. Mean value of EuroSCORE of patients during the last 8-year period was 5,13%. On the other hand, an improved postoperative medical and nursing care accompanied with the upgrade of the facilities was a key factor to decrease the mean in-hospital length of stay of last 8-year period (5 days). A similar conclusion can also be extracted from Roschkov's et al.,[16] study about the necessity of adequate nursing and medical awareness in order to minimize any postoperative complications which might delay patient's recovery and discharge. Left atrium was the most common site of tumor growth (87,6% of cases, 127 out of 145) and there was a predominance of female sex in the study population. More specifically, 62,1% of patients were women and their mean age was 57,9 years. 4,1% of patients presented recurrence of tumor and underwent a redo operation, having the myxoma located at the left atrium mainly. Mean age on those relapsed patients was 36,5 years. 97,2% of all patients fully recovered. Our findings were in accordance with Keeling et al.,[2] who investigated a series of 49 patients at Karl Franzens University, Austria. They observed a female predominance with a sex ratio of 3:1 and mostly middle-aged patients were affected. In addition, Shah IK, et al.,[17] conducted a 50-year epidemiological screening of 194 patients at Cardiac Surgical and Biostatistical Department of Mayo Clinic, Rochester, Minnesota. Their results revealed that the left atrium was the most common site of tumor growth and the younger age of patients was related to tumor recurrence. We also observed that left atrium was the most common site of growth at recurrent myxomas while at Shah's study the recurrent tumors were developed at the ventricles. Similar to Shah's study, the survival outcome post excision was very good. In our study we also noticed significant findings regarding the incidence of cases during the study period. Especially, a significant increase of cases (up to 54), has been documented during the last 8-year period whereas in the previous 8-year periods around 30 cases per period were documented.
Furthermore, in a study conducted by Bordalo et al.,[18] it was documented that an easier access to u/s scanning might provide a safer diagnose in a timely manner as 48% of patients were found to be asymptomatic. Similar to our study, Pérez-Andreu et al.,[19] also documented that an improvement in cardiac imaging techniques and a better accessibility to echocardiography revealed an increase of cases in the last three 5-year periods of their 24-year study (1990-2014). In our study, 45,9% of cases were incidental findings during a regular check up in a primary healthcare facility with no prior manifestation of symptoms.
In conclusion, cardiac myxomas are uncommon but benign tumors. An easier access of the population to advanced cardiac imaging techniques on primary healthcare service facilities might provide earlier detection and diagnosis. Moreover, an improvement in perioperative management with a slight increase in perioperative surgical times has led to minimizing the in-hospital length of stay. Effective postoperative care and nursing management as an efficient means of hospital care may reduce the in-hospital length of stay as it was documented in our study. Instead of an epilogue, we could say that a surgical excision with an early adequate diagnostic management upon detection of the tumor can produce a very good survival outcome for patients.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
References 
Correspondence Address:
Michopanou Nektaria
Cardiac Surgery ICU, ‘Evangelismos’ General Hospital, Athens - 10676
Greece
Source of Support: None, Conflict of Interest: None
CheckDOI: 10.4103/aca.aca_290_20
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