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Benign Mesenchymal Tumor Of The Heart; Spontaneous Regression And Disappearance Of Pulmonary Artery Stenosis

Lee et al., 1982Lung cancer

Chest 82(4): Oct 1982; 503-505

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Abstract

The authors report a case of a cardiac tumour documented by 2 D echocardiography, presenting in the newborn with cardiac arrest. The echocardiographic features of multiple nodules disseminated in the ventricular walls suggested a diagnosis of rhabdomyoma. The initial course was complicated by poorly tolerated attacks of tachycardia which were rapidly brought under control with amiodarone. The long-term outcome was clinically favorable with a rapid regression of the number and size of the tumours on echocardiography, and a tendency to normalization of the electrocardiographs. The possibility of regression, which has already been reported by many investigators, suggests that these cardiac tumours may have a better prognosis than previously thought.

Case Details

Personal Characteristics

20-month-old baby, well-developed, well-nourished boy, blood pressure 100/60 mmhg, pulse rate 150/minute

Clinical Characteristics

Large benign mesenchymal tumor of the right ventricular wall and right pulmonary artery stenosis, respiratory distress, marked cardiomegaly, frequent premature beats, coarse rhonchi, grade 3/6 ejection systolic murmur, liver palpable 2 centimeters below the right costal margin, hemoglobin 10 gm/dl, white blood cell count 11,500/ml

Remission Characteristics

Respiratory distress improved gradually, normal intracardiac pressures, disappearance of right pulmonary artery stenosis, asymptomatic at age 20

Treatment & Mechanisms

Proposed Remission Mechanisms

Not discussed

Clinical Treatment

Biopsy of the tumor, exploratory thoracotomy, cardiac catheterization

Additional Notes

The tumor was believed to be a benign mesenchymal tumor and most likely fibroblastic. The patient did not receive any form of treatment after digitalis was discontinued. He was able to attend school and participate in sports activities.