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      Right ventricular myxoma in a patient with tetralogy of Fallot

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          Highlights

          • •

            Myxoma and repaired tetralogy of Fallot in the absence of a familiar trait.

          • •

            Myxoma may aggravate symptoms in patients with repaired tetralogy of Fallot.

          • •

            Combined pulmonary valve replacement and myxoma excision is feasible in this setting.

          Abstract

          INTRODUCTION

          Cardiac myxoma is the most common primary cardiac tumour in adulthood and may present in the context of Carney's complex.

          PRESENTATION OF CASE

          A 32-year-old male with a history of repaired tetralogy of Fallot in childhood was admitted with severe pulmonary valve regurgitation and a mobile mass in the right ventricle. The patient underwent pulmonary valve replacement and mass excision. Pathology examination showed myxoma.

          DISCUSSION

          In the majority of cases myxomas originate in the atria, nevertheless they can also be found in a ventricular cavity. Myxoma is a prevalent feature of Carney's complex, an inherited, autosomal disease, characterised by multiple tumours in several organs. Tetralogy of Fallot has also been described in association with Carney's complex.

          CONCLUSION

          Coexistence of tetralogy of Fallot with a cardiac ventricular myxoma in a patient not affected from Carney's complex or other familial syndrome.

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          Cardiac myxomas.

          K Reynen (1995)
          Although cardiac myxomas are histologically benign, they may be lethal because of their strategic position. They can mimic not only every cardiac disease but also infective, immunologic, and malignant processes. Myxomas must therefore be included in the differential diagnosis of valvular heart disease, cardiac insufficiency, cardiomegaly, bacterial endocarditis, disturbances of ventricular and supraventricular rhythm, syncope, and systemic or pulmonary embolism. The symptoms depend on the size, mobility, and location of the tumor. Echocardiography, including the transesophageal approach, is the most important means of diagnosis; CT and MRI may also be helpful. Coronary arteriography in patients over 40 years of age is generally required to rule out concomitant coronary artery disease. Surgical removal of the tumor should be performed as soon as possible; the long-term prognosis is excellent, and recurrences are rare. In follow-up examinations as well, echocardiography is essential.
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            Mutations in regulatory subunit type 1A of cyclic adenosine 5'-monophosphate-dependent protein kinase (PRKAR1A): phenotype analysis in 353 patients and 80 different genotypes.

            I Bourdeau, Jérôme Bertherat, Xavier Bertagna … (2009)
            The "complex of myxomas, spotty skin pigmentation, and endocrine overactivity," or "Carney complex" (CNC), is caused by inactivating mutations of the regulatory subunit type 1A of the cAMP-dependent protein kinase (PRKAR1A) gene and as yet unknown defect(s) in other gene(s). Delineation of a genotype-phenotype correlation for CNC patients is essential for understanding PRKAR1A function and providing counseling and preventive care. A transatlantic consortium studied the molecular genotype and clinical phenotype of 353 patients (221 females and 132 males, age 34 +/- 19 yr) who carried a germline PRKAR1A mutation or were diagnosed with CNC and/or primary pigmented nodular adrenocortical disease. A total of 258 patients (73%) carried 80 different PRKAR1A mutations; 114 (62%) of the index cases had a PRKAR1A mutation. Most PRKAR1A mutations (82%) led to lack of detectable mutant protein (nonexpressed mutations) because of nonsense mRNA mediated decay. Patients with a PRKAR1A mutation were more likely to have pigmented skin lesions, myxomas, and thyroid and gonadal tumors; they also presented earlier with these tumors. Primary pigmented nodular adrenocortical disease occurred earlier, was more frequent in females, and was the only manifestation of CNC with a gender predilection. Mutations located in exons were more often associated with acromegaly, myxomas, lentigines, and schwannomas, whereas the frequent c.491-492delTG mutation was commonly associated with lentigines, cardiac myxomas, and thyroid tumors. Overall, nonexpressed PRKAR1A mutations were associated with less severe disease. CNC is genetically and clinically heterogeneous. Certain tumors are more frequent, with specific mutations providing some genotype-phenotype correlation for PRKAR1A mutations.
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              Survival after resection of primary cardiac tumors: a 48-year experience.

              Andrew ElBardissi, Joseph A Dearani, Richard Daly … (2008)
              Primary cardiac tumors are rare but have the potential to cause significant morbidity if not treated in an appropriate and timely manner. To date, however, there have been no studies examining survival characteristics of patients who undergo surgical resection. From 1957 to 2006, 323 consecutive patients underwent surgical resection of primary cardiac tumors; 163 (50%) with myxomas, 83 (26%) with papillary fibroelastomas, 18 (6%) with fibromas, 12 (4%) with lipomas, 28 (9%) with other benign primary cardiac tumors, and 19 (6%) with primary malignant tumors. Operative (30 day) mortality was 2% (n=6). Univariate analysis indicated that patients who underwent resection of fibromas and myxomas had superior survival characteristics in comparison to the remainder of tumor variants; these results were consistent after adjusting for age at surgery, year of surgery, and cardiovascular risk factors. Based on actuarial characteristics of the 2002 U.S. population, patients who underwent myxoma resection had survival characteristics that were not significantly different from that of an age and gender matched population (SMR 1.11, P=0.57) whereas those who underwent resection of fibromas (SMR 11.17, P=0.002), papillary fibroelastomas (SMR 3.17, P=0.0003), lipomas (SMR 5.0, P=0.0003), other benign tumors (SMR 4.63, P=0.003), and malignant tumors (SMR 101, P<0.0001) had significantly poorer survival characteristics. Furthermore, malignant tumors in younger patients were highly fatal (HR 0.899, P<0.0001). Although the most significant predictor of mortality was tumor histology, survival was also influenced the by the duration of CPB and NYHA III/IV; the impact of these risk factors varied with time. The cumulative incidence of myxoma recurrence was 13% and occurred in a younger population (42 versus 57 years, P=0.003) with the risk of recurrence decreased after 4 years. Surgical resection of primary cardiac tumors is associated with excellent long-term survival; patients with cardiac myxomas have survival characteristics that are not significantly different from that of a general population. Predictors of mortality are primarily related to tumor histology but also include clinical characteristics such as symptomatology and duration of CPB.
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                Author and article information

                Contributors
                Andrew C. Chatzis
                Journal
                Journal ID (nlm-ta): Int J Surg Case Rep
                Journal ID (iso-abbrev): Int J Surg Case Rep
                Title: International Journal of Surgery Case Reports
                Publisher: Elsevier
                ISSN (Electronic): 2210-2612
                Publication date PMC-release: 05 November 2014
                Publication date (Electronic): 05 November 2014
                Publication date Collection: 2014
                Volume: 5
                Issue: 12
                Pages: 1058-1060
                Affiliations
                [a ]Department of Paediatric and Congenital Cardiac Surgery, Onassis Cardiac Surgery Centre, Athens, Greece
                [b ]Department of Cardiology, AHEPA University Hospital, Thessaloniki, Greece
                [c ]Department of Radiology, St Luke's Hospital, Thessaloniki, Greece
                [d ]Department of Paediatric Cardiology, Onassis Cardiac Surgery Centre, Athens, Greece
                Author notes
                [* ]Corresponding author at: Department of Paediatric and Congenital Cardiac Surgery, Onassis Cardiac Surgery Centre, 356 Syngrou Ave, 17674 Kallithea, Athens, Greece. Tel.: +30 210 9493318. achatzis@ 123456ath.forthnet.gr
                Article
                Publisher ID: S2210-2612(14)00240-5
                DOI: 10.1016/j.ijscr.2014.09.030
                PMC ID: 4276279
                PubMed ID: 25437648
                SO-VID: 9b9e23ff-a3b6-45f4-b7d5-f1c9bbc4c9db
                Copyright © © 2014 The Authors
                License:

                This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/3.0/).

                History
                Date received : 26 September 2013
                Date revision received : 24 September 2014
                Date accepted : 29 September 2014
                Categories
                Subject: Article

                Keywords: myxoma,tetralogy of fallot,surgical repair,ventricle,pulmonary regurgitation
                Data availability:
                Keywords: myxoma, tetralogy of fallot, surgical repair, ventricle, pulmonary regurgitation

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