Bolívar-Mejía A, Rueda MA, Sánchez-Pardo S, Fajardo JE

Language: Spanish
References: 0
Page: 179-184
PDF size: 375.73 Kb.

ABSTRACT

Background: Becker muscular dystrophy is a rare progressive and disabling myopathy, associated with multiple complications; however, pulmonary hypertension has rarely been posed as a consequence of this disease, with little literature relating these two conditions.
Clinical case: A 43-year-old male patient with Becker muscular dystrophy. Consulted for progressive dyspnea, cyanosis, palpitations, precordial pain and orthopnea. Physical examination revealed muscular hypotrophy, pectus excavatum and pulmonary systolic murmur. The electrocardiogram showed sinus rhythm, P pulmonale, S1Q3T3 and incomplete right bundle branch block. Chest X-ray showed right ventricle growth and signs of pulmonary hypertension. Contrast computed tomography of the chest was negative for pulmonary embolism. Transesophageal echocardiography showed normal left ventricular function, dilatation of right cavities, severe tricuspid insufficiency, and right ventricular systolic pressure of 70mmHg. Cardiac catheterization was performed, confirming the pre-capillary pulmonary hypertension, with no response to pulmonary vasoreactivity testing, indicating treatment with sildenafil with adequate response.
Conclusions: Studies in animal models have suggested different mechanisms for Becker muscular dystrophy that may lead to pulmonary hypertension and right heart failure, including diaphragmatic degeneration, episodes of apnea, hypoxemia and pulmonary vasoconstriction.