Sildenafil for Noncompaction Cardiomyopathy Treatment in a Child: Case\nReport
The patient is a 6-year-old male, previously healthy, admitted with clinical findings\ncompatible with CHF. The clinical investigation ruled out causes of CHF secondary to\ninfectious, arrhythmogenic, infiltrative, toxic and ischemic processes and neuromuscular\ndisorders. The serum level of type B natriuretic peptide (BNP) was 641 pg/mL. The\nechocardiographic findings were as follows: left ventricular (LV) ejection fraction of\n19.1%; significant biventricular systolic dysfunction; LV diastolic dysfunction;\nsignificant LV dilation; and mild left atrial dilation. In addition, deep myocardial\ntrabeculae were observed, communicating with the free LV cavity, with a noncompaction to\ncompaction ratio of 3.2:1 and no structural malformation, establishing the diagnosis of\nbilateral cardiomyopathy of the noncompaction myocardial type (). Drug treatment with angiotensin-converting-enzyme\ninhibitor (ACEI), diuretics and carvedilol was initiated.\nAfter one year of clinical follow-up and optimized pharmacological treatment (diuretics,\nACEI and beta blocker), his symptoms and functional class worsened, a new hospital\nadmission being necessary. Echocardiography showed persistent dilated cardiomyopathy of\nsignificant hemodynamic repercussion, with moderate tricuspid and mild mitral\nregurgitations. Because of the unfavorable diagnosis and clinical course, the patient\nwas assessed aiming at heart transplantation. The hemodynamic evaluation showed\ncardiomyopathy with noncompaction of the ventricles and spongy myocardium, normal\ncapillary angiography and mean capillary pressure of 25 mm Hg.\nRescue therapy with sildenafil at the dose of 2 mg/kg/day was indicated while awaiting a\ndonor. The clinical and imaging follow-up every three months showed progressive\nimprovement, with normalization of the ventricular function, of the BNP serum levels and\nof the symptoms (). The patient was then\nremoved from the heart transplantation list, maintaining good quality of life.