Recurrent Hemoptysis in Patient with Primary Pulmonary Hypertension – A Case Report and Literature Review

Pulmonary hypertension (PH) is defined as an increase in mean pulmonary arterial pressure (mPAP) ≥ 25 mmHg at rest as assessed by right heart catheterization (RHC). The symptoms of PH are non-specific and mainly related to progressive right ventricular (RV) dysfunction. In some patients the clinical presentation may be related to mechanical complications of PH and the abnormal distribution of blood flow in the pulmonary vascular bed, include hemoptysis related to rupture of hypertrophied bronchial arteries. Hemoptysis is a serious complication that is rarely reported in patients with pulmonary arterial hypertension (PAH). Hemoptysis severity ranges from mild to very severe leading tosudden death. Hemoptysis are reported to be a terminal stage of a complication due to PAH with prevalence is variable, from 1% to 6%. Although the incidence is quite rare, the presence of recurrent hemoptysis in patients with pulmonary hypertension is a sign of poor prognosis. Bronchial artery embolization is suggested as an acute emergency procedure in the case of severe hemoptysis or as elective intervention in cases of frequent mild or moderate episodes.

Microscopically, PAH is characterized by intimal hyperplasia, hypertrophy of the tunica media, thickening of the tunica adventitia, and endothelial proliferation. The disease was first discovered by Dr. Ernst von Romberg in 1891. 2,3 The incidence of PAH is very rare, where in France the incidence is only 15 cases for every million people. 2,3 Hemoptysis is a serious complication that is rarely reported in patients with pulmonary arterial hypertension (PAH). Hemoptysis is reported to be a terminal stage of a complication due to PAH. The incidence of hemoptysis in PAH patients remain uncertain and tend to be unreported. Similarly, the patho-mechanism of hemoptysis in PAH remains uncertain. 4 Mortality associated with hemoptysis in PAH is influenced by multifactorial. Some evidence suggests that patients with PAH who are associated with congenital heart disease have a better prognosis for their hemoptysis complications than other PAH types. However, the underlying mechanisms of this condition remain unknown. 4      From the Chest Computed Tomography examination showsa dilation of the pulmonary truncus with diameter± 3.59 cm, dilatation of the right pulmonary artery with diameter± 2.69 cm, dilatation of the left pulmonary artery with diameter± 2.51 cm, and dilatation of the bilateral pulmonary artery branches. The diameter of the pulmonary truncus is greater than the ascending aortic aorta; There is enlargement of the right atrium and the right ventricle. Conclusion: Dilatation of the pulmonary artery that supports the diagnosis of pulmonary hypertension ( Figure 4).
Patients were diagnosed with primary pulmonary arterial hypertension non-reactive O2 test, observation of hemoptysis suspected to be associated with pulmonary hypertension, suspected Health Care-Associated Pneumonia (HCAP) andhypokalemia. The treatments were: sildenafil 40 t.i.d. mg, digoxin 0.25 mg q.i.d., furosemide 20 mg q.i.d., iloprost nebulizer 2.5 mcg q.i.d., Aspar K 1 tablet q.i.d., intravenous ceftazidime 1 gr t.i.d., intravenous ciprofloxacin 400 mgb.i.d..  There is usually no symptom of orthopnea and paroxysmal nocturnal dyspnea. 1,7 Physical examination is relatively insensitive to make the diagnosis, but it can help to rule out the differential diagnosis. If on lung examination found wheezing and rales, should be considered the possibility of bronchial asthma, bronchitis or fibrosis. Rales as seen in congestive  and also cardiomegaly. 9 The chest X-ray of patients proposed in this case report supports the presence of pulmonary hypertension, wherein from chest X-ray image there is presence ofcardiomegaly (RV dilatation). increases, but on the other hand there is a decrease in the production of vasodilators such as prostacyclin. 3,13 In addition, in the lumen of a patient's blood vessel with PAH, an elevated plasma serotonin level was found. Serotonin can stimulate the proliferation of smooth cardiac muscle cells and it is an important sign in the pathogenesis of PAH.

Echocardiography
Nitric oxide (NO) produced in the endothelial is a vasodilator that will inhibit platelet activation and vascular smooth muscle cell proliferation formed from three nitric oxide isoforms (NOs / S / NOS3).
However, the role of NO in PAH pathophysiology remains unclear. Pulmonary arterial hypertension has a poor prognosis and high mortality, despite adequate treatment. 13,14 Clinically, in pulmonary arterial hypertension

Pathophysiology of Hemoptysis in Pulmonary Artery Hypertension
Hemoptysis is defined as a coughing of blood whose source originates from the lungs or bronchial tubes, as a result of pulmonary or bronchial haemorrhage. Hemoptysis is divided into massive and non-massive based on the coughed blood volume. However, there is no Hemoptysis is a serious complication that is rarely reported in patients with pulmonary arterial hypertension (PAH). Hemoptysis is reported to be a terminal stage of a complication due to PAH. The incidence of hemoptysis in PAH patients remains uncertain and tends to be unreported. Similarly, the patho-mechanism of hemoptysis in PAH remains uncertain. 4 Pulmonary arterial hypertension (PAH) is a pathological condition whose primary cause  19 In patients with PAH, elevated endothelin-1 and its plasma products were obtained. It is associated with the severity of the disease.

Prognosis of Hemoptysis Associated with Pulmonary Hypertension
The prognosis of PAH is usually worse because it is generally caused by other diseases.
Usually, patients realize if they have pulmonary hypertension after the clinical symptoms appear, which that means the patient is already in an advanced stage. If it was diagnosed earlier, the prognosis will be better, at least in terms of reducing symptoms. Hemoptysis is known to be one of the complications of pulmonary hypertension.
Although the incidence is quite rare, the presence of recurrent hemoptysis in patients with pulmonary hypertension is a sign of poor prognosis. 4