Acta Cardiologia Indonesiana (Vol 4 No.

1): 41-45

Pulmonary Artery Hypertension Associated with HIV Infection

Baskoro Adi Prabowo1,*, Abdus Samik Wahab1,2, Hasanah Mumpuni1

1
Department of Cardiology and Vascular Medicine, Faculty of Medicine Public Health and Nursing,
Universitas Gadjah Mada – Dr. Sardjito Hospital, Yogyakarta. Indonesia
2
Department of Child Health, Faculty of Medicine Public Health and Nursing,
Universitas Gadjah Mada – Dr. Sardjito Hospital, Yogyakarta. Indonesia

Corresponding author :
Baskoro Adi Prabowo, MD, - email: baskoro27@gmail.com
Department of Cardiology and Vascular Medicine, Faculty of Medicine Public Health and Nursing, Universitas Gadjah
Mada – Dr. Sardjito Hospital, Yogyakarta, Indonesia, Jalan Farmako Sekip Utara, Yogyakarta 55281

Manuscript submitted: April 22, 2018; Revised and accepted: May 25, 2018

ABSTRACT
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.

Keywords: pulmonary arterial hypertension; HIV infection.

INTISARI
Hipertensi arteri paru merupakan suatu komplikasi berat dan mengancam nyawa pada
suatu infeksi HIV. Tampilan klinis dari pasien hipertensi arteri paru tidak spesifik sehingga
menyebabkan diagnosa ditegakkan ketika penyakit sudah fase lanjut. seorang anak penderita
HIV yang berumur 9 tahun dibawa ke Klinik Kesehatan Anak (Pediatrik) dengan keluhan
utama sesak nafas memberat selama 1 bulan, kaki bengkak, dan sesak pada saat tidur pada
alas datar. Pasien menunjukan gejala dan tanda yang mengarah pada suatu hipertensi paru.
Temuan EKG menunjukan adanya deviasi aksis ke kanan dan pembesaran ventrikel kanan.
Pemeriksaan ekhokardiografi menunjukan tanda pembesaran atrium dan ventrikel kanan serta
probabilitas tinggi untuk suatu hipertensi paru. Pemeriksaan darah menunjukan CD 4 sejumlah
84 sel/µL. Pasien ditatalaksana sebagai hipertensi arteri paru yang berhubungan dengan infeksi
HIV. Pasien dirawat selama 3 minggu dan akhirnya kondisinya membaik untuk dipulangkan
dari rumah sakit.
INTRODUCTION patient showed nonspecific sign and symptom,
resulted in recognized diagnosis at later stage.2
Pulmonary Arterial Hypertension (PAH)
Pulmonary artery hypertension associated with
is defined as an increase in mean pulmonary
HIV infection (HIV-PAH) has been documented
arterial pressure (mPAPm) ≥ 25 mmHg at rest
at all stages of the disease and its manifestations
as assessed by right heart catheterization
range from asymptomatic right ventricular
(RHC).1 A pulmonary hypertension is a serious
dysfunction to overt right heart failure.2 Children
life threatening and severe complication of
with HIV infection may develop this complication.
HIV infection. 2 A PAH presentation in HIV

41
Prabowo et al., 2018

Some report showed that 41% pediatric HIV On physical examination, the patient
patient had echocardiogram lead to PH looked dyspneu, heart rate 120 beats per
diagnosis.3 We presented this case to highlight minute, respiratory rate 40 times per minute,
the cardiovascular complication, especially PAH, body temperature was 36 ºC, peripheral
of HIV infection. oxygen saturation showed 58% on room air.
Anthropometric examination revealed weight 19
CASE PRESENTATION kg, height 110 cm, WAZ score -4.48 Z (severe
underweight), HAZ score -4.47 Z (severe
A nine year-old female child came to pediatric
stunted). On head examination patient showed
clinic with chief complaint a worsening dyspneu
cyanosis on the lips. Neck examination showed
for 1 month. Patient also complained of having
no increased on jugular venous pressure.
dyspneu on effort, edema at both leg, difficulty while
Thorax examination was symmetric, lung sound
lying on a flat bed, loss of appetite, and coughs.
vesicular and crackles at both lung fields.
There were no complaining of fever, diarrhea,
Cardiovascular examination showed positive
and abnormality in urination and defecation. The
right ventricular heaving, no displaced of apical
patient was already diagnosed with HIV infection
impulse, crisp S1 and S2 sounds with no splitting
since she was three years old and regularly took
sound, there were no murmurs or extra cardiac
Highly Anti Retroviral Therapy (HAART) since
sounds. Abdomen was protuberant with active
than. Since September 2015 patient had been
bowel sounds, it was soft and non tender,
taking HAART regiment as duviral1/2 tablet t.i.d
palpable hepatomegaly 4 cm below right costal
and alluvia 1 tablet b.i.d. Patient has no history of
margin, spleen and kidney could not be felt.
congenital heart disease diagnosis. Both patient’s
Extremities were warm, pitting edema was felt at
parents were HIV patients which was diagnosed
the lower extremity, and fingers looked cyanotic
at the same time with the patient.
and clubbing.

Figure 1. Electrocardiogram showed sinus rhythm, heart rate 100 beats

42
 Acta Cardiologia Indonesiana (Vol 4 No. 1): 41-45

Figure 2. Chest X ray showed bilateral pneumonia, cardiomegaly with

Figure 3. Transthoracic echocardiogram (apical 4 chamber

The electrocardiogram (ECG) showed right ventricle, and increased vascular marking
sinus rhythm, heart rate 100 beat per minute, (figure 2).
right axis deviation (RAD) and right ventricular An echocardiogram showed atrial situs
hypertrophy (RVH) (figure 1). Laboratory solitus, AV-VA concordance, all pulmonary
examination showed hemoglobin level 12.8 vein drainage to left atrium and systemic vein
g/dL, leukocyte 7,550 cells/µL, thrombocyte drainage to right atrium, there was dilatation at
169,000 cells/µL, erythrocyte 4,070,000 cells/ right atrium, right ventricle, and pulmonary artery
µL, hematocrit 42.5 %, albumin 3.41 g/dL, (figure 3). Inter atrial and inter ventricular septum
glucose 104 mg/dL, natrium 135 mmol/L, were intact, there was tricuspid regurgitation
kalium 2,54 mmol/L, chloride 88 mmol/L, and (Vmax 3.6 m/s, TVG 51 mmHg), trivial pulmonary
CD4 count 84 cell/µL. Chest X-ray examination regurgitation, PVAccT 103 m.s, pulmonary artery
showed bilateral pneumonia, cardiomegaly diameter 16 mm, inferior vena cava diameter
with configuration right atrium, left atrium, and 57 mm with less than 50% collapse, good LV

43
Prabowo et al., 2018

Figure 4. Transthoracic echocardiogram showed tricuspid

contractility (LVEF 66%), decrease right ventricle lesion and showed plexiforms lesion that can
contractility (TAPSE 8 mm), there were no patent be detected in 78% patient. However, the
ductus arteriosus, no coarctation of the aorta, no mechanism is unclear because there is no
pericardial effusion (figure 4). substantial proof that HIV directly infected
Patient was diagnosed as right heart the pulmonary vasculature. 1,2,4,5 The strong
failure, PAH related to HIV (HIV-PAH), and candidate is virus’s protein, GP 120 and
HIV stage 3 on therapy. Patient was given Nef, interaction with pulmonary vasculature
therapy intravenous furosemide 20 mg t.i.d, is causing the interactions between PAH
oral spironolactone 12.5 mgb.i.d, oral lisinopril and HIV. An HIV also plays role by means of
2 mg b.i.d, oral sildenafil 7 mg t.i.d, duviral chronic inflammation and immune activation
1/2 tablet b.i.d, aluvia 1 tablet b.i.d, oral produced by HIV infection, which may lead
cotrimoxazole 480 mg q.i.d, and malnutrition increase secretion proinflammatory cytokines
management. Patient was treated at pediatric and growth factors that may promote PAH.1,2,4,5
ward for 3 weeks and discharged in improved Clinical presentation of HIV-PAH is
clinical condition. same with idiopathic PH, which sometimes is
missed because its unspecified symptoms.
DISCUSSION Majority patients will present with dyspneu on
exertion (85%), pedal edema (20-30%), and non
Pulmonary artery hypertension
productive cough (19%), fatigue (13%), syncope
associated with HIV infection (HIV-PAH)
or near syncope(12%).6,7 Chest x-ray examination
histopathologic characteristics are not different
on HIV-PAH patients also show resemblance with
from idiopathic PH.1,2,4,5 Pulmonary vasculature
PAH without HIV which show cardiomegaly (72%)
is obliterated with medial hypertrophy and
and pulmonary artery enlargement (71%).6,7
increase proliferation of endothelial and
Electrocardiogram will show pulmonal P, right
smooth muscle cells. Characterized by
axis deviation, right ventricular hypertrophy,
concentric-obliterative changes on intimal

44
 Acta Cardiologia Indonesiana (Vol 4 No. 1): 41-45

right bundle branch block, and sometimes treatment may improve functional class and
prolonged QT interval.6,7 Echocardiography is the symptom.
non invasive test of choice for initial screening
for PAH in symptomatic HIV patients.1,4,8 It is REFERENCES
useful for identifying potential causes of PAH,
1. Abman S.H., Hansmann G, Archer S.L., Ivy
evaluating RV function, and assessing related
D.D., Adatia I. 2015. Pediatric pulmonary
comorbidities.1,4,8
hypertension, Guidelines the American Heart
There are no currently available guidelines
Association and American Thoracic Society.
that available for HIV-PAH therapy.4,5,7. Therefore,
Circulation, 132: 2037-2099
treatment of HIV-PAH relies on PAH specific
2. Almodovar S., Cicalini S., Petrosilo N.,
therapy and includes supportive treatments and
Flores S.C. 2010, Pulmonary hypertension
diseased specific treatment.4,5,7,8 Supportive
associated with HIV infection. Chest,
therapy for HIV-PAH patients includes oxygen
137:6S-12S
administration for hypoxic patient and treatment
3. Pongprot Y., Sittiwangkul R., Silvilairat S.,
with diuretics and vasodilators for patient with overt
Sirisanthana V. 2004. Cardiac manifestations
right ventricular failure. Prostanoid as specified
in HIV-infected Thai children. Ann Trop
treatment of PAH showed a beneficial effect on
Paediatr, 24: 153-159.
HIV-PAH patients with data show decreasing of
4. Cicalini S., Almodovar S., Grilli E., Flores
mean pulmonary arterial pressure and pulmonary
S. 2011. Pulmonary hypertension and
vascular resistance, endothelin receptor also
human immunodeficiency virus infection:
show increasing in clinical and hemodynamic
epidemiology, pathogenesis, and clinical
parameters on the patients. Phosphodiesterase-5
approach. Clin Microbiol Infect, 17:25-33.
inhibitor also showed beneficial effect on dyspneu
5. Talwar A., Sarkar P., Rosen M.J. 2009.
symptom and functional class. Caution should
Pulmonary arterial hypertension in human
be used in HIV-infected patients receiving a
immunodeficiency virus infection. Postgrad
HAART regimen containing protease inhibitor, as
Med, 121: 56-67.
saquinavir and ritonavir, which have been shown
6. Mehta N.J., Khan I.A., Mehta R.N., Sepkowitz
increasing sildenafil plasma concentration of drug
D. A. 2000. HIV-Related pulmonary
and metabolites.4,5,7,8
hypertension: analytic review of 131 cases.
There are still conflicting data regarding the
Chest, 118:1133-1141.
role of HAART in the management of patients
7. L’Huilier A.G., Posfay-Barbe K.M., Pictet
with HIV-PAH. Several experts suggested that
H., Beghetti M. 2015. Pulmonary arterial
HAART does not prevent development of PAH in
hypertension among HIV infected children:
HIV infected patients and some suggested that
results of a national survey and review of
HAART could delay or attenuate development
literature. Frontiers in pediatrics, 3:25
of PAH in HIV-infected patients.4,5,7
8. Galie N., Humbert M., Vachiery J.L., Gibbs
S., Lang I., Torbicki A., et al. 2016. ESC/ERS
CONCLUSION
Guidelines for the diagnosis and treatment
This case reports a nine year-old patient of pulmonary hypertension. Eur Heart J,
with HIV-PAH. It highlights initial diagnosis and 37:67-119

45
Hartopo et al., 2018

PETUNJUK PENULISAN NASKAH

Naskah ditulis dalam Bahasa Inggris, Tabel, grafik dan foto diberi nomor urut,
diketik dengan format kertas ukuran A4 dengan judul dan keterangan serta diletakkan pada
jarak bingkai 3 cm dari sisi tepi, jarak baris 2 lembaran terpisah teks.
spasi. Jenis huruf Arial 11. Halaman pertama Nama latin (binomial) ditulis dengan
naskah memuat judul artikel, nama dan alamat huruf bercetak miring (Italic). Satuan ukuran
penulis. Jarak baris 1 spasi digunakan pada menggunakan Sistem Satuan Internasional.
abstrak (abstract), tabel, keterangan tabel,
keterangan gambar dan daftar pustaka. Susunan Naskah
Abstrak berisi tujuan, metode penelitian, Naskah disusun sebagai berikut:
hasil penelitian dan kesimpulan, maksimum 1. Judul dalam Bahasa Indonesia dan Inggris.
200 kata, kata kunci maksimum 5 kata disusun 2. Nama penulis ditulis lengkap diikuti dengan
menurut abjad huruf. Abstrak harus ditulis dalam nama instansi dan alamat lengkap beserta
Bahasa Inggris. nomor telepon, faksimili serta alamat
Grafik dibuat dengan menggunakan e-mail.
Program MS Excel, tanpa menggunakan 3. Abstract.
latar belakang dan bingkai. Grafik harus jelas 4. Kata kunci (Key words)
dan mudah dibaca. Grafik garis diberi simbol 5. Pengantar (Introduction).
yang jelas untuk membedakan antar objek 6. Bahan dan Metode (Material and Method).
pengamatan. Garis pada grafik berukuran 1 Ditulis dengan rinci dan jelas. Alat analisis
point. Grafik bar diberi warna gradasi hitam, dan bahan tertentu ditulis asal usulnya.
atau diisi efek yang berbeda untuk membedakan 7. Hasil (Result).
objek pengamatan. Simbol dapat digunakan 8. Pembahasan (Discussion).
untuk memperjelas keterangan pada gambar 9. Kesimpulan (Conclusion).
atau grafik. 10. Saran (Recommendation). Boleh tidak
Gambar peta digambar menggunakan ditulis/Optional.
program computer (Corel, Illustrator, Adobe, dll), 11. Ucapan terima kasih (Acknowledment).
disimpan dalam bentuk JPEG. Informasi penting 12. Daftar Pustaka (References), disusun
yang disajikan misalnya nama lokasi, skala, arah menurut urutan tampil dalam naskah dengan
aliran harus akurat. angka.
Tabel dibuat tanpa garis vertikal pemisah
kolom dan tanpa nomor kolom. Garis horizontal Daftar pustaka dari jurnal, majalah atau
pada table hanya pada baris pertama (judul bulletin ditulis dengan urutan sebagai berikut:
kolom), akhir tabel. Bilangan satuan dan nomor urut, nama pengarang, tahun terbit,
pecahan menggunakan tanda pemisah koma, judul, nama jurnal/-majalah/bulletin (disingkat
jumlah angka digit di belakang koma disamakan dalam bentuk baku), volume dan nomor,
untuk kolom yang sama. halaman pertama hingga terakhir. Pengarang
Foto dibuat dalam bentuk JPEG dengan ditulis secara lengkap sesuai jumlahnya. Jika
resolusi rendah, namun ketika direduksi pada karangan berikutnya diterbitkan oleh pengarang
ukuran cetak lebar 8 cm atau 16 cm informasi yang sama dalam tahun yang sama, maka pada
yang disajikan masih jelas terbaca. Foto dicetak tahun penerbitan ditambahkan huruf a, b, c dan
pada kertas mengkilat, jelas dan tidak kabur. seterusnya.
Foto berwarna sedapat mungkin dihindari. Bila Daftar pustaka dari buku ditulis dengan
foto berwarna, sedapat mungkin menggunakan urutan sebagai berikut: nomor urut, nama
warna yang kontras antara latar belakang dan pengarang, tahun penerbitan, judul buku, edisi,
objeknya. Warna latar belakang gelap (hitam) penerbit, tempat penerbitan, jumlah halaman.
atau terang (putih).

46