3rd Case

Report

Primary pulmonary
hypertension without
right heart
catheterization (RHC)
Mohammad Irfan, M. Diah, Azhari Gani
Division of Cardiology
Department of Internal Medicine RSUDZA/ Medical Faculty of1
UNSYIAH Banda Aceh
2015

INTRODUCTION

Rubin LJ et al, Overview of pulmonary hypertension, UpTodate, Version 18.2 : May

2013.
Gali N et al, Guidelines for the diagnosis and treatment of pulmonary
2
hypertension, European Heart Journal, 2011, 30, 2493-2537.

Case report

Identity
Name
Age
Sex
Occupation
Addres

:
:
:
:
:

Mr. R
30 years
Male
PNS
Sigli

 Man, -30 year olds, complaint was dyspnoe since one

month ago that was getting worse one week before
admitted to hospital.. He also felt fatique and
syncope easily since years ago.
Chest pain was appeared since one month ago. It
looked like something hard on the chest and no
referred.
He also complained about his distended abdomen
since three years ago then he felted hard to breath.
Both of his legs was swelling.

 In the status present was found tachycardia (100

beats/minute, regular) and tachypnoe (40 times/minute).
The clinical presentation was distension of jugular vein,
ictus cordis on the fifth ICS at anterior axillaris line with
thrill, the right side of heart laid on 2 cm lateral of dextra
parasternalis line.
It was found pansystolic murmur with gradation 4/6 on
the tricuspid area. Second sound of pulmonary was
higher than aorta.
He was ascites with hepatomegali and edema of inferior
extremities.

The laboratorium results were mild increased level of

liver function test.
The diagnostic evaluation were
1.ECG; summary was RAD, RBBB, RVH.
2. CXR; summary was cardiomegali with
pulmonary
congestion and minimal pleura
effusion in right side.
3. Echocardiography: summary was severe
pulmonary
hypertension (suggest PPH).
4. Thorax CT scanning; summary was
cardiomegali and
distention of pulmonary
artery, pleura effusion in right
side and
normal lung.
6

Diagnosis was established as PPH WHO-FC IV.

The therapy was
Bedrest,
Oxygen nasal cannul 2-4L/I
Furosemide injection 40 mg/8 hours
Warfarin 2 mg per day.
The patient had been improved clinically and he
was out patient after 10 days.

Table 1. Hemodynamic definitions of pulmonary

2
hypertension.
Definition
Characteristics
Clinical group (s)
b

Pulmonary hypertension Mean PAP 25 mmHg All

(PH)
Pre-capillary PH

Mean PAP 25 mmHg 1.

3.
PWP 15 mmHg
4.
CO normal or reducedc 5.

Pulmonary artery hypertension

PH due to lung diseases
Chronic thromboembolic PH
PH
with
unclear
and/or
multifactorial mechanisms

Post-capillary PH

Mean PAP 25 mmHg

2.

PH due to left heart disease

PWP > 15 mmHg

CO normal or reducedc
Passive

TPG 12 mmHg

Reactive (out of TPG > 12 mmHg

proportion)

Rounds S and MV Cutaia, Pulmonary Hypertension: Pathophysiology and Clinical

Disorders, Baums Textbook of Pulmonary Diseases, 7th Edition (September 2009),
Lippincott Williams & Wilkins, 2114-2148

Table 2. Updated clinical classification of pulmonary

hypertension.3

Rubin LJ, Primary Pulmonary Hypertension, ACCP Consensus Statement, Chest,

2013; 104:236-50.
Peacock AJ, Murphy NF, McMurray JJV, Caballero F, Stewart S, An epidemiological
study of pulmonary artery hypertension, Eur Respir J 2010; 30: 104-109.

Table 3. Grading of recommendations and level of evidence for

efficacy in IPAH.10

Peacock AJ, Murphy NF, McMurray JJV, Caballero F, Stewart S, An epidemiological

study of pulmonary artery hypertension, Eur Respir J 2010; 30: 104-109.

Figure 2. Evidence-based treatment algorithm.11

Peacock AJ, Murphy NF, McMurray JJV, Caballero F, Stewart S, An epidemiological

study of pulmonary artery hypertension, Eur Respir J 2010; 30: 104-109.

DISCUSSION
iTeory

The frequency of diagnosis of

PPH is generally considered to
be a disease of younger people,
with greatest incidence between
the ages of 20 and 45 years.
the ratio of women to men was
1.7:1

Case

In this case pasien men, 30 years

old

Flamm MD, Cohn KE, Hancock EW: diagnosis and treatment of pulmonary hypertension. Am J Cardiol. 2008;
258-65.

13

DISCUSSION
iTeory

Diagnostic evaluations for PPH

1.The ECG commonly shows RAD and
RVH with secondary T-wave changes
2.The CXR shows evidence of
pulmonary hypertension in over 90
percent of cases. pruning of the vessels
with hyperlucent lung periphery in 51
percent.
3.The typical echocardiographic
appearance of the patient with PPH
shows right ventricular and right atrial
enlargement with a normal to reduced
left ventricular cavity

Case
In this case diagnostic evaluation
were
1. ECG; summary was RAD, RBBB,
RVH.
2. CXR; summary was
cardiomegali with pulmonary
congestion and minimal pleura
effusion in right side.
3. Echocardiography: summary
was severe pulmonary
hypertension (suggest PPH).
4. Thorax CT scanning; summary
was cardiomegali and distention
of pulmonary artery, pleura
effusion in right side and normal
lung.

Flamm MD, Cohn KE, Hancock EW: diagnosis and treatment of pulmonary hypertension. Am J Cardiol. 2008;
258-65.

14

DISCUSSION
iTeory

Cardiac catheterization is an absolute

requirement for confirming the diagnosis
of PPH and for guiding management

Case

In case, the diagnosis was

established without RHC. RHC is
required to confirm the diagnosis
of PAH, to assess the severity of
the haemodynamic impairment,
and to test the vasoreactivity of
the pulmonary circulation.

Peisiewicz W, Goch A, Blinikowski Z: Changes in the cardiovascular system. Cardiol Pol. 2010; 218-28.
Perloff JK, Koos BJ: Pulmonary Artery Hypertension, Philadelphia,PA. 2009; 542-49.

15

DISCUSSION
iTeory (Durante
operative)
The evidence for the favourable
effects of oral anticoagulant
treatment in patients with IPAH
or PAH.
Patients with decompensated
right heart failure (RHF) develop
fluid retention that leads to
increased central venous
pressure, peripheral oedema
and, in advanced cases, ascites.
Appropriate diuretic treatment

Case

In case, we used oxygen support,

anticoagulant (warfarin) and
diuretic agent (furosemide) to
manage the patient. The
condition of patient after
treatment became clinically
impoved.

Siu SC, Sermer M, Colman JM, Alvarez AN, Mercier LA, Morton BC: Cardiac Disease in Pregnancy (CARPREG)
Investigators. Prospective Multicenter Study of Pregnancy Outcomes in Women With Heart Disease. Circulation.
2010; 515-21.

16

SUMMARY

1. We have report the case of a a-30 year old-man,

with a case primary pulmonary hypertension.
2. The diagnosis was established by anamnesis,
physical presentation, ECG, CXR, Echocardiography
and Thorax CT scanning.
3. The therapy was bedrest, oxygen nasal cannul 24L/i, furosemide injection 40 mg/8 hours and
warfarin 2 mg per day.
4. The patient had been improved clinically and he
was out patient after 10 days.

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Data of patients (EKG)

18

Data of patients (Chest Xray)

19

Echocardiography

20

21

22

23

Data of patients (Thorax CT

Scanning)

24

Thank You

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