Medical and Surgical Management of Carney Complex

Siordia, Juan A.

doi:10.1111/jocs.12575

Cited by 24 publications

(28 citation statements)

References 93 publications

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“…CNC is a rare autosomal dominant genetic disorder affecting more than 750 patients distributed in many ethnic groups and equally between males and females [6,7]. 70% of patients are found to have inactivating mutations in the protein kinase A type I-alpha regulatory subunit (PRKAR1A) gene on chromosome 17q22-24 [8,9].…”

Section: Discussionmentioning

confidence: 99%

“…The CNC patients need a continuous surveillance at least yearly in order to detect early the manifestations of this syndrome ( Table 6). The cardiac diseases are responsible for more than half of the mortality risk in CNC patients dominated by the complications of heart myxomas and followed by the metastatic tumors (25%) [7]. Table 2: CNC manifestations [6,[10][11][12].…”

Section: Discussionmentioning

confidence: 99%

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Cushing’s Syndrome Revealing Carney Complex: A Case Report

Elfekih¹,

Hadjkacem²,

Ghorbel³

et al. 2017

EMIJ

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Carney complex (CNC) is a rare multisystem disorder, inherited in an autosomal dominant manner and characterized by distinctive spotty skin pigmentation, myxomas and endocrine abnormalities. We report a case of a 35-year-old patient diagnosed with Cushing's syndrome complicated with an impaired glucose tolerance (IGT) and a severe psychiatric disturbance. The diagnosis of CNC was made by having two major criteria, namely a primary pigmented nodular adrenal disease (PPNAD) and thyroid carcinoma.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Cushing’s Syndrome Revealing Carney Complex: A Case Report

Elfekih¹,

Hadjkacem²,

Ghorbel³

et al. 2017

EMIJ

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“…The mutation causes an abnormality in the 1-alpha subunit of protein kinase A, which is responsible for multiple hormonal and intracellular signaling pathways. [9] Diagnosis is achieved with the presentation of 2 major criteria (including skin pigmentation, cardiac myxoma, skin myxoma, and PPNAD); or one major criterion plus evidence of the genetic mutation. [2] Evidenced by the case, cardiac myxomas present at a mean age of 24 years; this is unlike sporadic myxomas that present at a mean age of 51 years.…”

Section: Discussionmentioning

confidence: 99%

“…Due to these possible complications, in addition to the recurrence of myxomas found in Carney complex patients, it is suggested to perform annual echocardiograms in afflicted patients. [9] Detection of a cardiac myxoma prompts surgical excision to avoid the high risk of embolization and stroke. [12] Supported by this case, intra-aortic filter usage is recommended during the operation to capture emboli before they travel into the systemic circulation.…”

Section: Discussionmentioning

confidence: 99%

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Outcomes of multiple myxoma resections with carney complex

Siordia

Garlish

2015

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Introduction: Carney Complex is a rare multiple endocrine neoplasia with cardiac myxomas being the most common cause of death among affected patients. One must consider how many heart surgeries a patient may acquire before the treatment itself becomes a significant risk factor. The use of an intra-aortic filter during cardiac surgeries is recommended, and heart transplantation treatment for Carney Complex patients is proposed. Presentation of case: A 24-year-old female presented with lightheadedness and was found to have a cardiac myxoma on the left atrium. The patient underwent myxoma resections on four separate occasions. The patient underwent a fifth cardiac myxoma resection. However, three days post-operatively, the patient experienced right-sided hemiplegia and imaging showed 99% left carotid artery occlusion. The patient was deemed unqualified for neurosurgical intervention and the patient died 5 days post-operatively. Discussion: Cardiac surgery can become detrimental with multiple surgical resections. With increased number of cardiac surgeries and increased risk for morbidities postoperatively, Carney Complex patients should have an intra-aortic filter to lower the risk of stroke. Conclusions: Intra-aortic filter usage is recommended during surgical resection of cardiac tumors to capture emboli before they travel into the systemic circulation. Heart transplant may prove beneficial to Carney complex cases, however neither the optimal number of cardiac operations nor the timing of heart transplantation in the disease are documented in literature, limiting evidence for solid therapeutic guidelines.

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Left ventricular myxoma with Carney complex

et al. 2018

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The left ventricle is a less frequent location of cardiac myxomas overall. Meanwhile, cardiac myxomas related to Carney complex (CNC), which is a multiple neoplasia syndrome involving cardiac, endocrine, neural, and cutaneous tumours, more frequently occur in the left ventricle compared with sporadic cardiac myxomas. Herein, we report a case of a 20‐year‐old woman with CNC who underwent complete surgical excision of a large and mobile left ventricular myxoma. In our case, echocardiography performed 4 years earlier was normal. This case highlights the importance of annual follow‐up by echocardiography in patients with CNC, because early diagnosis of cardiac myxomas might improve their prognosis. Besides, we should bear in mind the possibility of CNC if the patients have cardiac myxoma in a cardiac chamber other than the left atrium at a younger age.

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Medical and Surgical Management of Carney Complex

Cited by 24 publications

References 93 publications

Cushing’s Syndrome Revealing Carney Complex: A Case Report

Cushing’s Syndrome Revealing Carney Complex: A Case Report

Outcomes of multiple myxoma resections with carney complex

Left ventricular myxoma with Carney complex

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