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INTRODUCTION: Primary malignant cardiac mesenchymoma tumors are rarely described in the medical literature, with only a few published cases. CASE PRESENTATION: We present a case of a primary malignant mesenchymoma in the left atrium extending to the left and right inferior pulmonary vein orifices, accompanied by mechanical obstruction of the mitral valve. The patient underwent surgical removal and radiotherapy. DISCUSSION: Cardiac tumors are categorized as primary and secondary tumors. Primary cardiac tumors are extremely rare, with a low incidence rate of 0.001-0.28 %. Primary malignant tumors constitute less than 25 % of them. Malignant mesenchymoma is considered the rarest of them. These tumors proliferate and may cause metastases, making them highly malignant with a poor prognosis, especially if accompanied by metastases or invasion of nearby tissues. CONCLUSION: The poor prognosis of cardiac mesenchymoma comes from the fact that it is aggressive and rapidly growing, which causes a rapid progression of mechanical symptoms and metastasis to nearby tissues, which makes it classified as a highly malignant tumor with a low survival rate. Surgical resection remains the mainstay of treatment, in addition to follow-up with chemotherapy or radiotherapy according to the grade of the disease, which was the case with our patient and the same as reported in previous literature. However, further research and case reports are needed better to understand the optimal management of this aggressive tumor.
RESUMO
Lutembacher's syndrome is a rare disease defined as the combination of atrial septal defect and mitral stenosis, which relieves the progression of symptoms resulting from mitral stenosis. We present a case of a previously asymptomatic 70-year-old man having Lutembacher's syndrome with sinus venosus type of interatrial defect associated with partial anomalous pulmonary venous drainage. Our patient's only complaint was of mild exertional dyspnea, which started six months prior to referral. Clinical suspicion of the combination of defects arose during transthoracic echocardiography. The patient underwent transesophageal echocardiography and multi-slice computed tomography for defect confirmation. The combination of Lutembacher's syndrome with this complex deformity of pulmonary venous drainage has rarely been described. Such complex congenital defects with multifaceted physiological repercussions can go largely undiagnosed for the patients affected for most of their lifetimes. LEARNING POINTS: Despite the complexity of Lutembacher's syndrome, including the involvement of several deformities and pathological mechanisms of mitral stenosis, sinus venosus type of interatrial defects with partial anomalous return in the pulmonary veins, the multiplicity of mechanisms involved contribute to altered hemodynamics.Multiple intersecting mechanisms can further contribute to delaying congenital heart disease presentation. Congenital heart disease remains a plausible differential in the evaluation of elderly patients presenting with exertional dyspnea.Adult congenital heart defects can have very late onset for presentation, especially with physiological processes that paradoxically reduce the severity and progression of symptoms.