Cardiovascular Journal of Africa: Vol 30 No 1 (January/February 2019)

CARDIOVASCULAR JOURNAL OF AFRICA • Volume 30, No 1, January/February 2019

AFRICA

Case Report

Pulmonary thromboendarterectomy in a combined

thrombophilia patient

Hakan Akbayrak, Hayrettin Tekumit

Abstract

Chronic thromboembolic pulmonary hypertension (CTEPH)

is a potentially correctable cause of secondary pulmonary

hypertension. Surgical treatment remains the primary treat-

ment for patients with CTEPH. Pulmonary thromboendar-

terectomy (PEA) with deep hypothermic circulatory arrest

is the standard and recommended surgical technique for the

treatment of these patients. The prevalence of CTEPH after

an acute pulmonary thromboembolism (PTE) has been found

in various studies to be between 0.6 and 8.8%. Mortality rates

in elective PEA cases with CTEPH are reported to be between

1.9 and 4.5%. We report on a 50-year-old female patient with

combined inherited thrombophilia, including protein C and

protein S deficiencies, who was diagnosed with CTEPH and

was successfully treated with pulmonary thromboendarter-

ectomy.

Keywords:

protein C, protein S, pulmonary embolism, thrombo-

endarterectomy

Submitted 26/2/18, accepted 22/10/18

Published online 22/11/18

Cardiovasc J Afr

2019;

: e4–e6

www.cvja.co.za

DOI: 10.5830/CVJA-2018-052

Chronic thromboembolic pulmonary hypertension (CTEPH)

is the end result of untreated or recurrent pulmonary

thromboembolism (PTE). The incidence of CTEPH is about

5% in patients who survive after acute PTE and up to 30% after

recurrent PTE.

After acute PTE, the incidence of cumulative

symptomatic CTEPH in the first six months was 1%, compared

with 3.1% per year and 3.8% over two years.

The prevalence

of CTEPH was shown to be between 0.6 and 8.8% in studies

evaluating the prevalence of CTEPH after an acute PTE.

CTEPH is currently largely under-diagnosed and must be

sought in all patients presenting with exertional dyspnoea,

reduction in effort capacity, fatigue, or clinical symptoms of

right-sided heart failure. It can be seen with or without a

prior history of deep venous thrombosis (DVT) and/or PTE.

The patients with permanent symptoms of dyspnoea, fatigue,

limitation of exercise, vertigo or chest pain following an acute

PTE, as well as patients in whom pulmonary hypertension is

reported as an acute event, could potentially benefit from further

investigations to rule out CTEPH.

Echocardiography, ventilation/perfusion (V/Q) scan,

computerised tomography (CT), pulmonary angiography and

cardiac catheterisation are standard diagnostic procedures.

The

severity of the pulmonary hypertension determines the prognosis

of CTEPH. If the mean pulmonary artery pressure (PAP) is

greater than 30 mmHg, the five-year survival is approximately

30%. If the mean PAP is greater than 50 mmHg, the five-year life

expectancy is estimated at 10%.

V/Q scan is the preferred and recommended screening test for

patients with CTEPH. Pulmonary angiography remains the gold

standard for confirmation of chronic thromboembolic disease

and evaluation of operability.

It is recommended that all patients

with CTEPH should be evaluated with right heart catheterisation

and pulmonary angiography to assess their prognosis.

Surgical techniques have improved over the last 20 years but

the standard surgical technique for PEA has not changed in the

last five years. These procedures are currently safely performed

in clinics with experienced surgeons.

5,6

The progressive increase in pulmonary vascular resistance

affects the clinical course of CTEPH. If left untreated, it could

result in progressive pulmonary hypertension, right ventricular

dysfunction and death.

The standard treatment options for CTEPH patients are

surgical treatment and/or balloon pulmonary angioplasty

procedure (BPAP). BPAP can be performed on inoperable

patients or as complementary treatment to surgery.

The protein C system, which contains protein C, protein S and

thrombomodulin, is a natural profibrinolytic system. Protein C

and protein S are vitamin K-dependent plasma proteins that

play a role in the negative feedback system in blood clotting.

Thrombomodulin, which is a surface protein of endothelial cells,

is also a part of the protein C system. Poor fibrinolytic activity is

seen in protein C-deficient patients.

7-9

Patients between 15 and 40 years who develop venous

thrombotic complications, with a high incidence of DVT and

PTE, usually have protein C and protein S deficiencies. Protein

C- and protein S-deficient patients are treated medically with

Department of Cardiovascular Surgery, Faculty of

Medicine, Selcuk University, Konya, Turkey

Hakan Akbayrak, MD,

hakanakbayrak@gmail.com

Department of Cardiovascular Surgery, Faculty of

Medicine, Bezmialem University, Istanbul, Turkey

Hayrettin Tekumit, MD