24 HOW TO TREAT : PULMONARY HYPERTENSION
24 HOW TO TREAT : PULMONARY HYPERTENSION
19 APRIL 2024 ausdoc . com . au gestational age and low birthweight are significantly higher in patients with moderate to severe PAH compared with those with mild PAH . 36
Avoiding unwanted pregnancies is critical . There are no forms of contraception for which PAH itself is a contraindication , in the absence of comorbidities that are contraindications . This includes emergency post-coital contraception . 3
Seek expert advice for pregnancy planning . ET-1 receptor antagonists are category D in pregnancy because of the high risk of teratogenicity .
Elective and emergency surgery
The estimated 30-day postoperative mortality risk for a patient with PH is one in 50 ( population risk for comparison is one in 58,000 ). This rises to approximately one in seven for emergency surgery . 3 , 30 While the ESC / ERS guideline makes no general recommendations , based on the authors ’ local experience , if surgery is deemed to be absolutely necessary , the patient should undergo surgery in a centre with PH experience and ICU availability with considerable preoperative planning and multidisciplinary input .
THE FUTURE
RECENT research has suggested that the predominant pathophysiological process in PAH may be proliferation of pulmonary vascular smooth muscle cells . Sotatercept ( a first-in-class drug ) inhibits ligands that belong to the transforming growth factor beta superfamily , which are implicated in the proliferation of pulmonary vascular smooth muscle cells . This product has shown efficacy in PAH . 31 , 32 This may represent a change in the future treatment paradigm for PAH , with a focus on developing drugs that have antiproliferative and anti-inflammatory mechanisms of action . There is also ongoing research into the role of pulmonary artery nerve denervation in PAH patients .
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Figure 5 . Right ventricular strain pattern from right ventricular hypertrophy . The ECG shows ST depression and T wave inversion in V1-4 and lead III . Other features of right ventricular hypertrophy are present , including right axis deviation , and a dominant R wave in V1 .
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Figure 6 . Lung perfusion scan in chronic thromboembolic pulmonary hypertension , showing homogeneous ventilation and segmental defects in the perfusion scan .
CASE STUDIES
Case study one
EDEN , a 34-year-old female , presents to her GP with chest heaviness and dizziness after standing from a bent position to load the washing machine . This was followed by a witnessed syncope . An ECG ( see figure 8 ) shows ST depression and T wave inversions and she is referred to the ED .
A CTPA and V / Q scan are normal . An inpatient TTE reveals a tricuspid regurgitation velocity ( TRV ) of 3.8 metres / second with a markedly dilated RV with reduced systolic function . Antinuclear antibodies , extractable nuclear antigen , HIV and viral hepatitis serology are negative . Brain natriuretic peptide is 75ng / L ( mildly elevated in the context of PAH ).
An RHC demonstrates pre-capillary PH ( see table 4 ).
Eden ’ s final diagnosis is idiopathic PAH ( Group 1 ) and she is started on tadalafil ( a phosphodiesterase type 5 inhibitor [ PDE5i ]) and ambrisentan ( ERA ). Selexipag is added one year later because of an increase in pulmonary artery pressure .
Case study two
Bernard , a 68-year-old male , has a
A
Figure 7 . Contrast-enhanced chest CT in chronic thromboembolic pulmonary hypertension ( CTEPH ).
A . Contrast-enhanced chest CT in CTEPH showing eccentric thrombotic material within the left pulmonary artery ( arrow ).
B
B . Contrast-enhanced chest CT scan in CTEPH before pulmonary endarterectomy showing mosaic perfusion .
CC BY / Neth Heart J . 2014 Dec ; 22 ( 12 ): 533 – 541 / bit . ly / 3NcQzf2
Figure 8 . The patient ’ s 12-lead ECG on admission to hospital demonstrates sinus rhythm and a right ventricular strain pattern with ST depression in II , III , aVF , V2-V4 and T wave inversion in III , aVF and V2-V4 .