Primary pulmonary hypertension without right heart catheterization

(RHC)

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Mohammad Irfan, M. Diah, Azhari GaniDivision of Cardiology

Department of Internal Medicine RSUDZA/ Medical Faculty of UNSYIAH Banda Aceh

2015

3rd Case Report

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INTRODUCTION

Rubin LJ et al, Overview of pulmonary hypertension, UpTodate, Version 18.2 : May 2013.Galiè N et al, Guidelines for the diagnosis and treatment of pulmonary hypertension, European Heart Journal, 2011, 30, 2493-2537.

Case report

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IdentityName : Mr. RAge : 30 years Sex : MaleOccupation : PNSAddres : 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.

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• 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.

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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.

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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 hypertension.2

Definition Characteristics Clinical group (s)b

Pulmonary hypertension (PH)

Mean PAP ≥ 25 mmHg All

Pre-capillary PH Mean PAP ≥ 25 mmHg

PWP ≤ 15 mmHg

CO normal or reducedc

1. Pulmonary artery hypertension3. PH due to lung diseases4. Chronic thromboembolic PH5. PH with unclear and/or

multifactorial mechanisms

Post-capillary PH

Passive

Reactive (out of proportion)

Mean PAP ≥ 25 mmHg

PWP > 15 mmHg

CO normal or reducedc

TPG ≤ 12 mmHg

TPG > 12 mmHg

2. PH due to left heart disease

Rounds S and MV Cutaia, Pulmonary Hypertension: Pathophysiology and Clinical Disorders, Baum’s 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

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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

In this case pasien men, 30 years old

iTeory Case

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

DISCUSSION

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Diagnostic evaluations for PPH 1.The ECG commonly shows RAD and RVH with secondary T-wave changes2.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

In this case diagnostic evaluation were1. 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.

iTeory Case

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

DISCUSSION

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Cardiac catheterization is an absolute requirement for confirming the diagnosis of PPH and for guiding management

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.

iTeory Case

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.

DISCUSSION

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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

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.

iTeory (Durante operative) Case

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.

SUMMARY

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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 2-4L/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.

Data of patients (EKG)

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Data of patients (Chest X-ray)

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Echocardiography

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Data of patients (Thorax CT Scanning)

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Thank You

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