Pulmonary Arterial Hypertension among HIV-Infected Children: Results of a National Survey and Review of the Literature

Abstract

Since the advent of highly active anti-retroviral therapy, HIV-related mortality has decreased dramatically. As a consequence, patients are living longer, and HIV infection is becoming a chronic disease. Patients and caretakers have to deal with chronic complications of infection and treatment, such as cardiovascular diseases, which now represent an important health issue, even in the pediatric population. Prevalence of pulmonary arterial hypertension (PAH) in the adult HIV population is around 0.4–0.6%, which is around 1000- to 2500-fold more prevalent than in the general population. In recent adult PAH registries, HIV has been identified as the fourth cause of PAH, accounting for approximately 6–7% of cases. Therefore, regular screening is recommended in HIV-infected adults by many experts. If HIV-associated PAH is mainly reported in HIV-infected adults, pediatric cases have also been, albeit rarely, described. This scarcity may be due to a very low PAH prevalence, or due to the lack of systematic cardiovascular screening in pediatric patients. As PAH may manifest only years or decades after infection, a systematic screening should perhaps also be recommended to HIV-infected children. In this context, we retrospectively looked for PAH screening in children included in our national Swiss Mother and Child HIV cohort study. A questionnaire was sent to all pediatric infectious disease specialists taking care of HIV-infected children in the cohort. The questions tried to identify symptoms suggestive of cardiovascular risk factors and asked which screening test was performed. In the 71 HIV-infected children for which we obtained an answer, no child was known for PAH. However, only two had been screened for PAH, and the diagnosis was not confirmed. In conclusion, PAH in HIV-infected children is possibly underestimated due to lack of screening. Systematic echocardiographic evaluation should be performed in HIV-infected children.

Pulmonary Artery Hypertension Associated with HIV Infection in Nine Year-Old Child

“…6,7 Chest x-ray examination on HIV-PAH patients also show resemblance with PAH without HIV which show cardiomegaly (72%) and pulmonary artery enlargement (71%). 6,7 Electrocardiogram will show pulmonal P, right axis deviation, right ventricular hypertrophy, prolonged QT interval. 6,7 Echocardiography is the non invasive test of choice for initial screening for PAH in symptomatic HIV patients.…”

“…6,7 Electrocardiogram will show pulmonal P, right axis deviation, right ventricular hypertrophy, prolonged QT interval. 6,7 Echocardiography is the non invasive test of choice for initial screening for PAH in symptomatic HIV patients. 1,4,8 It is useful for identifying potential causes of PAH, evaluating RV function, and assessing related comorbidities.…”

“…1,4,8 There are no currently available guidelines that available for HIV-PAH therapy. 4,5,7 . Therefore, treatment of HIV-PAH relies on PAH specific therapy and includes supportive treatments and diseased specific treatment.…”

Hipertensión pulmonar asociada al virus de la inmunodeficiencia humana en pediatría: tratamiento con sildenafil. A propósito de un caso

“…1 Se considera HTP al aumento de la presión media de la arteria pulmonar ≥ 25 mmHg evaluada mediante cateterismo cardíaco derecho. 2 En pediatría, existen múltiples causas; la más frecuente es la idiopática y la asociada a cardiopatías congénitas (Tabla 1).…”

“…El pronóstico de la HTP sin tratamiento es ominoso, con una supervivencia media de 6 a 8 meses, sin diferencias significativas entre HTP idiopática e HTP asociada a VIH. [1][2][3] En el manejo de la HTP, se emplean drogas de sostén y drogas específicas que tienen acción sobre el lecho vascular pulmonar. El fármaco más utilizado inicialmente es el sildenafil debido a la mayor disponibilidad, fácil administración y escasos efectos adversos.…”