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

Pulmonary arterial hypertension (PAH) is a serious life threatening and severe complication of HIV infection. A PAH presentation in patient with HIV tends to non specific, result in recognized diagnosis at a later stage. A 9 year-old HIV patient came to Pediatric Clinic with a chief complaint of worsening dyspneu for 1 month, leg edema and difficulty lying on a flat bed. Patient showed signs and symptoms that lead to pulmonary hypertension. An ECG findings were sinus rhythm, right axis deviation, and right ventricular hypertrophy. Echocardiography findings showed right ventricular and atrial enlargement, and high probability of pulmonary hypertension. Blood examination showed CD 4 was 84 cells/μL. The patient was managed as pulmonary artery hypertension associated with HIV (HIV-PAH) infection.The patient was admitted for 3 weeks and eventually discharged with relieve condition.


Pulmonary Arterial Hypertension (PAH)
is defined as an increase in mean pulmonary arterial pressure (mPAPm) ≥ 25 mmHg at rest as assessed by right heart catheterization (RHC). 1 A pulmonary hypertension is a serious life threatening and severe complication of HIV infection. 2A PAH presentation in HIV patient showed nonspecific sign and symptom, resulted in recognized diagnosis at later stage. 2 Pulmonary artery hypertension associated with HIV infection (HIV-PAH) has been documented at all stages of the disease and its manifestations range from asymptomatic right ventricular dysfunction to overt right heart failure. 2 Children with HIV infection may develop this complication.Some report showed that 41% pediatric HIV patient had echocardiogram lead to PH diagnosis. 3We presented this case to highlight the cardiovascular complication, especially PAH, of HIV infection.

CASE PRESENTATION
A nine year-old female child came to pediatric clinic with chief complaint a worsening dyspneu for 1 month.Patient also complained of having dyspneu on effort, edema at both leg, difficulty while lying on a flat bed, loss of appetite, and coughs.There were no complaining of fever, diarrhea, and abnormality in urination and defecation.The

P u l m o n a r y a r t e r y h y p e r t e n s i o n associated with HIV infection (HIV-PAH)
histopathologic characteristics are not different from idiopathic PH. 1,2,4,5 Pulmonary vasculature is obliterated with medial hypertrophy and increase proliferation of endothelial and smooth muscle cells.Characterized by concentric-obliterative changes on intimal lesion and showed plexiforms lesion that can be detected in 78% patient.However, the mechanism is unclear because there is no substantial proof that HIV directly infected the pulmonary vasculature. 1,2,4,5The strong candidate is virus's protein, GP 120 and Nef, interaction with pulmonary vasculature is causing the interactions between PAH and HIV.An HIV also plays role by means of chronic inflammation and immune activation produced by HIV infection, which may lead increase secretion proinflammatory cytokines and growth factors that may promote PAH. 1,2,4,5inical presentation of HIV-PAH is same with idiopathic PH, which sometimes is missed because its unspecified symptoms.Majority patients will present with dyspneu on exertion (85%), pedal edema (20-30%), and non productive cough (19%), fatigue (13%), syncope or near syncope(12%). 6,7Chest x-ray examination on HIV-PAH patients also show resemblance with PAH without HIV which show cardiomegaly (72%) and pulmonary artery enlargement (71%). 6,7ectrocardiogram will show pulmonal P, right axis deviation, right ventricular hypertrophy, right bundle branch block, and sometimes prolonged QT interval. 6,7Echocardiography is the non invasive test of choice for initial screening for PAH in symptomatic HIV patients. 1,4,8It is useful for identifying potential causes of PAH, evaluating RV function, and assessing related comorbidities. 1,4,8ere 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.
patient was already diagnosed with HIV infection since she was three years old and regularly took Highly Anti Retroviral Therapy (HAART) since than.Since September 2015 patient had been taking HAART regiment as duviral1/2 tablet t.i.d and alluvia 1 tablet b.i.d.Patient has no history of congenital heart disease diagnosis.Both patient's parents were HIV patients which was diagnosed at the same time with the patient.On physical examination, the patient looked dyspneu, heart rate 120 beats per minute, respiratory rate 40 times per minute, body temperature was 36 ºC, peripheral oxygen saturation showed 58% on room air.Anthropometric examination revealed weight 19 kg, height 110 cm, WAZ score -4.48 Z (severe underweight), HAZ score -4.47 Z (severe stunted).On head examination patient showed cyanosis on the lips.Neck examination showed no increased on jugular venous pressure.Thorax examination was symmetric, lung sound vesicular and crackles at both lung fields.Cardiovascular examination showed positive right ventricular heaving, no displaced of apical impulse, crisp S1 and S2 sounds with no splitting sound, there were no murmurs or extra cardiac sounds.Abdomen was protuberant with active bowel sounds, it was soft and non tender, palpable hepatomegaly 4 cm below right costal margin, spleen and kidney could not be felt.Extremities were warm, pitting edema was felt at the lower extremity, and fingers looked cyanotic and clubbing.

Figure 2 .Figure 3 .
Figure 2. Chest X ray showed bilateral pneumonia, cardiomegaly with configuration right atrium, left atrium, and right ventricle, and increased vascular marking.

4 , 5 , 7 , 8 7 CONCLUSION
Supportive therapy for HIV-PAH patients includes oxygen administration for hypoxic patient and treatment with diuretics and vasodilators for patient with overt right ventricular failure.Prostanoid as specified treatment of PAH showed a beneficial effect on HIV-PAH patients with data show decreasing of mean pulmonary arterial pressure and pulmonary vascular resistance, endothelin receptor also show increasing in clinical and hemodynamic parameters on the patients.Phosphodiesterase-5 inhibitor also showed beneficial effect on dyspneu symptom and functional class.Caution should be used in HIV-infected patients receiving a HAART regimen containing protease inhibitor, as saquinavir and ritonavir, which have been shown increasing sildenafil plasma concentration of drug and metabolites. 4,5,7,8There are still conflicting data regarding the role of HAART in the management of patients with HIV-PAH.Several experts suggested that HAART does not prevent development of PAH in HIV infected patients and some suggested that HAART could delay or attenuate development of PAH in HIV-infected patients. 4,5,This case reports a nine year-old patient with HIV-PAH.It highlights initial diagnosis and treatment may improve functional class and symptom.