Case reportSuccessful Up-front Combination Therapy in a Patient With Idiopathic Pulmonary Hypertension and Patent Foramen Ovale: An Alternative to Epoprostenol Therapy?
Section snippets
Case Report
A 40-year-old woman was admitted to the hospital with progressive dyspnea and chest pain on exertion during the last 12 months. On admission, she was tachypneic with central cyanosis and, on auscultation of the heart, the second heart sound was increased. Electrocardiogram showed right axis deviation and incomplete right bundle branch block. Chest X-ray indicated cardiomegaly with enlargement of the pulmonary conus and pulmonary arteries. Hematocrit was 53%, erythrocyte count was 6.60 × 106/μl
Discussion
To our knowledge, this is the first report of a patient with critical PH with PFO showing impressive clinical improvement and reversal of a right-to-left shunt with combination therapy as first-line treatment. Our therapeutic approach targeted both the acute vasodilator effects of combined oral sildenafil and inhaled iloprost, together with the long-term anti-remodeling effect of endothelin receptor antagonists.
In patients with advanced PH, and especially those with hemodynamic instability,
References (9)
- et al.
Medical therapy for pulmonary arterial hypertension: ACCP evidence-based clinical practice guidelines
Chest
(2004) - et al.
Danger of vasodilator therapy for pulmonary hypertension in patent foramen ovale
Chest
(1986) - et al.
Safety and efficacy of transition from subcutaneous treprostinil to oral sildenafil in patients with pulmonary arterial hypertension
J Heart Lung Transplant
(2007) - et al.
Dramatic decrease in the pulmonary artery systolic pressure and disappearance of the interatrial shunt with sildenafil treatment in a patient with primary pulmonary hypertension with atrial septal aneurysm and a severe right to left shunt through the patent foramen ovale
Int J Cardiol
(2006)
Cited by (7)
Patent foramen ovale in idiopathic pulmonary arterial hypertension: Long-term risk and morbidity
2016, Respiratory MedicineCitation Excerpt :In our study, measured RVSP and MPAP pressures were higher in patients with PFO, though not statistically significant. Patients with PH are theoretically at increased risk of developing right-to-left shunt due to increased right atrial pressures, facilitating flow of unoxygenated blood from the inferior vena cava (IVC) directly to the left atrium [12,24]. It is reasonable to postulate that in our cohort, higher pressures may predispose to increased PFO prevalence.
Potential role of patent foramen ovale in exacerbating hypoxemia: In chronic pulmonary disease
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