Case report

A 9-month-old Brazilian female infant born to a mother infected with the human immunodeficiency virus type 1 was admitted to our University Hospital in October 1995 with symptoms and signs of congestive heart failure. Clinical and laboratory diagnosis of acquired immunodeficiency syndrome was established in the child at six months of age. Feeding difficulties, failure to thrive, tuberculous lymphadenitis, and recurrent respiratory infections were the landmarks of the clinical history. Physical examination revealed psycho-motor underdevelopment, severe undernourishment, irritability, pallor, tachypnea, and hepatosplenomegaly. The abnormal cardiovascular findings were tachycardia, gallop rhythm, and a grade 2/6 systolic murmur at the mitral area. The electrocardiogram showed sinus tachycardia with diffuse flattening and inversion of the T-waves. The chest X-ray revealed marked cardiac enlargement. Echodopplercardiography showed severe left ventricular dilation, with left ventricular diastolic diameters of 40 mm, severe left ventricular dysfunction with left ventricular fractional shortening of 12% (Fig. 1), mild left atrial enlargement, mild mitral regurgitation, and a patent oval foramen. These findings were consistent with our criterions for dilated cardiomyopathy. Other laboratory features included moderate anemia and CD4 T-cell lymphocyte count less than 200/mm³. The viral load could not be estimated at the time. Brain computerized tomography showed cerebral atrophy.

Figure 1. Echocardiogram of our patient at the age of 9 months with acquired immunodeficiency syndrome and dilated cardiomyopathy. The four-chamber cross-sectional view (A) and the m-mode tracing (B) show a severely dilated left ventricle. RA: right atrium; RV: right ventricle; LA: left atrium; LV: left ventricle.

Cardiac management on admission included treatment with digoxin, furosemide, espironolactone, and captopril, with a good clinical response. The infant was further treated with zidovudine and intravenous immune globulin and had prophylaxis with sulfamethoxazole and trimethoprim for Pneumocystis carinii pneumonia. Other clinical manifestations included oral candidiasis, chronic diarrhea, repeated upper respiratory infections, pneumonia, extrapulmonary tuberculosis, encephalopathy, repeated urinary tract infections, pyelonephritis, and infected renal cysts.

After six years of uninterrupted treatment with cardiac and antiretroviral medication, the cardiac picture has reversed. Echodopplercardiography now shows normal left ventricular dimensions, with diastolic diameters of 36 mm, normal left ventricular function with fractional shortening of 36% (Fig. 2), a normal left atrium, and a competent mitral valve. Digoxin, furosemide, and espironolactone were gradually withdrawn. Captopril was maintained at low dosage. She has been on highly active antiretroviral therapy for the last four years. The CD4 T-cell lymphocyte count has reversed to normal limits. A viral load is now undetectable, unlike the last few years during which it was measured at over a million copies. She is now seven years old, and is a happy, smiling, little girl. Her psycho-motor development is improving, and she is gaining weight.

Figure 2. Our patient at 7 years of age after full recovery from dilated cardiomyopathy. The cross-sectional echocardiogram in cardiac systole (A) and diastole (B), and the m-mode tracing (C), show normal left ventricular dimensions. RA: right atrium; RV: right ventricle; LA: left atrium; LV: left ventricle; RVD (D): right ventricular dimension in diastole; IVS: interventricular septum in diastole (D) and systole (S); LVD: left ventricular dimension in diastole (D) and systole (S); LVPW (D): left ventricular posterior wall in diastole; EF: ejection fraction; FS: fractional shortening.

Discussion

Severe dilated cardiomyopathy is usually part of the late stage of the acquired immunodeficiency syndrome where immunosuppression is severe, and implies a poor prognosis.^1–5 In those infected perinatally with early manifestation of the disease, we are unaware of any previous reversibility of this entity. Our patient unequivocally had early manifestations of the disease, since the symptoms and signs began in the first year of life. She was clinically and immunologically classified in the category C3, that is, severe signs and symptoms and suppression according to the “Revised classification system for human immunodeficiency virus type 1 infection in children less than 13 years of age” of the Centers for Disease Control, Atlanta, United States of America, 1994.⁷ She was characterized as having rapid progression of the acquired immunodeficiency syndrome, with a poor prognosis because, besides manifesting cardiomyopathy with heart failure, she had encephalopathy, chronic diarrhea, renal disease, tuberculosis, and recurrent respiratory infections in the first year of life. Despite that, she not only reversed her cardiomyopathy, but also overcame the co-infections, normalized the CD4 T-cell count, and the viral load is, at the present time, undetectable.

Reports of the acquired immunodeficiency syndrome in children show that up to one-third of those infected perinatally manifest the disease early, usually with death occurring during the first two years of life.⁸ In addition, according to Luginbuhl et al.,¹ children who have encephalopathy are at higher risk for heart disease, and have a poorer prognosis. Congestive heart failure in such children can result from myocarditis with left ventricular dysfunction, pericarditis with large pericardial effusion, anemia, and bacterial sepsis. It can be transient or chronic. Chronic heart failure due to persistent left ventricular dysfunction is found in approximately one-tenth.^{1, 3} Myocardial depression can result from the viral infection or opportunistic co-infections,^{1, 3, 9} cardiotoxicity from antiretroviral drugs, autonomic dysfunction,^{1, 3} autoimmune dysfunction subsequent to myocarditis,¹⁰ or other etiologic agents. Dilated cardiomyopathy can be a consequence of the above mentioned agressive factors acting simultaneously on the myocardium.^{1, 3, 9} It is likely this would have occurred in our patient.

Although complete recovery from severe dilated cardiomyopathy in acquired immunodeficiency syndrome is rare, and unlikely to occur, our case shows that adequate treatment of patients even with the severe form of cardiac involvement can have a good outcome.

Acknowledgements

We thank Dr. Valdir Ambrosio Moisés and Dr. Solange Tatany from the Department of Echocardiography, and Dr. Daisy Machado from the Department of Pediatric Infectology, Acquired Immunodeficiency Syndrome Division, for their contribution and friendship.