Pulmonary Arterial Hypertension

PENDAHULUAN
 Di Amerika Serikat (1982-2006 ): frekuensi PPH sebesar
48% dari keseluruhan 578 kasus PH sedangkan di Cina,
36% kasus PAH (Ghamra & Dweik, 2003; Thenappan et al, 2007; Jing et al, 2007).
 Sampai sekarang belum ada obat yg benar-benar efektif
pd patogenesis penyakit obstruksi pembuluh pulmoner.
Pilihan terapi telah bertambah dalam dekade terakhir
dan dengan kemajuan ini, prognosis mengalami
perbaikan (Newman, 2002; Chin & Rubin, 2008).
 Jurnal ini merupakan systematic review
Klasifikasi Hipertensi Pulmoner WHO 2008
Patogenesis
Diagnosis
Gambaran klinis
 exertional dyspnea
 Nyeri dada saat aktifitas,
 Sinkop, fatigue
 edema ekstremitas bawah
 usia saat diagnosis 36-50 tahun
 Diagnosis tertunda karena
pemeriksaan fisik dan gejala
nonspesifik (Chin & Rubin, 2008).
Pemeriksaan fungsi pulmoner
menunjukkan volume paru
yang normal atau restriksi
ringan
Elektrokardiogram
 deviasi aksis kanan,
 hipertrofi RV, dan strain. (Ahearn et al, 2002)
Px scan perfusi ventilasi atau
MRI dinamik perfusi tiga
dimensi
untuk mengeksklusi hipertensi
pulmoner thromboembolik
Arteriografi pulmoner konfirmasi
diperlukan pada mereka yg
memiliki hasil scanning abnormal
(Ohno et al, 2007)
 Ekokardiografi
 Tekanan sistolik arteri
pulmoner (standard
error 5-8 mmHg
 pembesaran ruang
jantung kanan,
 gerakan paradoksikal
septum interventrikuler,
dan insufisiensi
trikuspidalis.
 Efusi perikardial (Bossone
et al, 2005).

Kateterisasi jantung kanan

(gold standard)
 utk memastikan kemunculan dan
menentukan tingkat keparahan
hipertensi pulmoner,
 untuk mengeksklusi penyakit jantung
sisi kiri atau shunting left-to-right
 untuk melakukan tes vasodilator akut
(Galie1 et al, 2009).
Algoritme
terapi
hipertensi
pulmoner
(Galie1 et al, 2009).
Journal Reading
Presented by : dr. Imam Manggalya
Supervisor : dr. Lucia Krisdinarti, Sp.PD, Sp. JP (K)
 Aims
• There is no cure for pulmonary arterial hypertension, but
current approved treatment options include prostanoids,
endothelin-receptor antagonists, and phosphodiesterase
type-5 inhibitors.
• The effect on survival of these compounds has not been
appropriately assessed in individual trials because of small
sample size and short duration.
• We performed a meta-analysis of all randomized controlled
trials with drugs published in this condition
Increased pulmonary vascular resistance

obstructive proliferative changes in the

lung microcirculation

extensive heart structural changes

right heart failure and premature death

 Methods
• Trials were searched in the Medline database from January
1990 to October 2008. The primary analysis included only
studies with a placebo comparator arm, the sensitivity analysis
also included studies comparing two active treatment arms.
• The main outcome measure was all-cause mortality. Twenty-
one trials were included in the primary analysis (3140 patients)
and two additional studies (59 patients) were included in the
sensitivity analysis.
• Average duration of the trials was 14.3 weeks.
• Statistics: Treatment effects for total mortality were evaluated
as relative risks (RR) according to the inverse variance fixed-
effect method
 Outcome

all-cause mortality
Hospitalizations
NYHA functional Right atrial
mPAP pressure
class
Cardiac index PVR
Exercise capacity
 Result
• All-cause mortality rate in the control group was 3.8%.
Active treatments were associated with a reduction in
mortality of 43% (RR 0.57; 95% CI 0.35–0.92; P . 0.023);
the sensitivity analysis confirmed a reduction in
mortality of 38% (RR 0.62; 95% CI 0.39–1.00; P . 0.048)
• Number of patients to be treated (NNT) to prevent one
death was 61.6 and 16.2 (95% CI 2.7–24.0) deaths
were prevented in each 1000 patients treated
 Secondary outcome

Cumulative RR estimate of
Significantly improved exercise
hospitalizations, was a
capacity as assessed by the
reduction of 61% (RR 0.39; 95%
6MWD
CI 0.25, 0.61; P , 0.001)

Improved by at least one Improved haemodynamic

functional class (RR 2.35; 95% CI parameters as assessed by right
1.59, 3.48; P , 0.001) heart catheterization.
Discussion
 Limitation
• The limitations of this meta-analysis include
the prolonged period of time between the
publication of the first and the last RCT (about
18 years)
• The different duration of the trials (ranging
from 8 to 36 weeks)
• The lack of blindness in some studies
 Conclusion
• The results of this meta-analysis suggest an
improvement of survival in the patients
treated with the targeted therapies approved
for pulmonary arterial hypertension.
 KAJIAN KRITIS

Apakah Tinjauan kepustakaan sistematis (TKS) ini valid?

1.Apakah TKS jelas terfokus pada satu pertanyaan klinik?
 Hubungan antara paparan dengan kesudahan yang bersifat spesifik atau tidak
spesifik jelas ?
2.Apakah kriteria yang digunakan untuk memilih makalah tersebut adalah layak ?
 Data spesifik tentang pasien, paparan, atau kesudahan
3.Apakah Makalah makalah yang relevan ada yang terlewatkan?
 Makalah diambil dari MEDLINE
4.Bagaimanakah validitas makalah yang dinilai ?
 Nama pengarang, nama instanti pengarang makalah
5. Bagaimanakah cara menilai setiap makalah untuk TKS ?
 Dilakukan oleh 2 orang yang berkompeten untuk penilaian dan pemilihan
makalah
Terimakasih

Mohon Asupan