1- Unidade de Infecciologia, Área de Pediatria Médica, Hospital de Dona Estefânia, Centro Hospitalar de Lisboa Central, EPE, Lisboa;
2- Serviço de Cardiologia Pediátrica, Hospital de Santa Marta, Centro Hospitalar de Lisboa Central, EPE, Lisboa;
3- Unidade de Hematologia, Área de Pediatria Médica, Hospital de Dona Estefânia, Centro Hospitalar de Lisboa Central, EPE, Lisboa;
4- Unidade de Pneumologia, Área de Pediatria Médica, Hospital de Dona Estefânia, Centro Hospitalar de Lisboa Central, EPE, Lisboa;
- 7th Europaediatrics Congress, 13 a 16 de Maio de 2015, Floren;a. Poster
Resumo:
Introduction Pulmonary hemosiderosis (PH) is a rare but life-threatening condition that may be secondary to mitral stenosis. Although PH has been reported in up to 16% of adult patients with severe mitral stenosis (MS), usually of rheumatic etiology, it occurs very rarely in pediatric age.
Case Report An eighteen-year-old boy of african origin was admitted with asthenia and hemoptysis. He had a history of rheumatic mitral valvulopathy submitted to surgical annuloplasty at the age of 12. He remained free of symptoms for some years but had an irregular follow-up. Two years before presentation, symptomatic iron-deficiency anemia had been identified (hemoglobin=6,6g/dL), which responded to red blood cell transfusion followed by iron supplementation. At admission, he reported a five-month history of hemoptysis, low-grade fever and progressive dyspnea on exertion. He was pale and tachycardic. The hemogram revealed hypocromic microcytic anemia (hemoglobin=7,8g/dL). Tuberculin skin test was 20x23mm and QuantiFERON®-TB positive. A diagnosis of pulmonary tuberculosis was initially considered. The chest X-ray showed cardiomegaly, pulmonary venous congestion and diffusely distributed miliary nodular opacities. Fiberoptic bronchoscopy revealed >95% hemosiderin-laden macrophages within the alveolar spaces, which was consistent with the diagnosis of PH. Treatment with oral corticosteroids was empirically started. Transthoracic echocardiography revealed severe mitral stenosis (mitral valve area: 0.9 cm2) and severe aortic valve regurgitation, with secondary pulmonary hypertension. Heart surgery was performed for mitral and aortic valve replacement with prosthetic valves, allowing complete resolution of hemoptysis and anemia. The culture tests for tuberculosis were negative and the patient was treated with isoniazid for six months for latent tuberculosis infection. At 8 months of follow-up after surgery, the patient remains without hemoptysis or anemia, under treatment with anti-congestive drugs, free of glucocorticoids.
Conclusions PH secondary to mitral stenosis is usually reported in adults as a lifelong complication, rarely seen in pediatrics. It may be successfully managed with valvuloplasty and/or valve replacement allowing discontinuation of corticotherapy. Secondary PH may be missed in patients with rheumatic heart disease with iron-deficiency anemia and/or miliary nodular opacities on chest radiographs without overt hemoptysis.
Palavras Chave: Hemoptysis, iron-deficiency anemia, hemosiderosis, rheumatic heart disease.