Use of vasodilator therapies in patients with pulmonary hypertension due to lung disease or left heart disease is potentially harmful and not recommended. In patients with pulmonary hypertension and hypoxia, supplemental oxygen should be administered to maintain saturation above 90%. In patients with pulmonary hypertension due to lung disease or left heart disease, treatment should focus on optimizing comorbid conditions. Results from invasive hemodynamic testing with right heart catheterization, preferably at an expert center, should be obtained before initiating treatment with vasodilator therapy in patients with pulmonary arterial hypertension. Other important considerations for patients with pulmonary hypertension include influenza and pneumonia immunizations, contraception counseling, preoperative assessment, and mental health.Įchocardiography is the recommended initial test in the evaluation of patients with suspected pulmonary hypertension. There are no directed therapies for many other categories of pulmonary hypertension therefore, family physicians have a central role in managing contributing comorbidities. For certain categories of pulmonary hypertension, particularly pulmonary arterial hypertension, treatment options are rapidly evolving, and early diagnosis and prompt referral to an expert center are critical to ensure the best prognosis. A definitive diagnosis cannot be made on echocardiographic abnormalities alone, and some patients require invasive evaluation by right heart catheterization. Echocardiography is recommended as the initial step in the evaluation of patients with suspected pulmonary hypertension. There is limited evidence to support screening of asymptomatic individuals. Patients often initially present to family physicians however, because the symptoms are typically nonspecific or easily attributable to comorbid conditions, diagnosis can be challenging and requires a stepwise evaluation. To help classify patients with PAH associated with TOF, especially with MAPCAs, we propose several new subclassifications: "PAH due to hypoplastic pulmonary arterial beds", "PAH due to abnormal pulmonary arborization", or "segmental PAH associated with CHD." A multicenter registry of patients using a unified protocol is essential to explore the indications and efficacy of pulmonary vasodilators for postoperative PAH-CHD.Pulmonary hypertension is a common, complex group of disorders that result from different pathophysiologic mechanisms but are all defined by a mean pulmonary arterial pressure of 25 mm Hg or greater. Based on our experience, we believe that PAH-targeted therapies are effective in some patients with PAH occurring after surgical treatment of TOF and MAPCAs, especially as an adjunct to percutaneous pulmonary angioplasty. The MAPCAs in these patients usually show hypoplastic and abnormal arborization. Moreover, patients with TOF and MAPCAs who are diagnosed as inoperable because of the presence of PAH show similar hemodynamic and clinical features to patients with Eisenmenger syndrome. PAH might be caused by pulmonary agenesis, hypoplasia and/or thrombosis, inadequate closure of the ventricular septal defect, relief of the pulmonic stenosis, or an excessively large prior systemic-to-pulmonary shunt. We often encounter patients with PAH occurring after surgical treatment of tetralogy of Fallot (TOF), especially patients with major aortopulmonary collateral arteries (MAPCAs). Therefore, it is still difficult to obtain clinical evidence supporting the indication of pulmonary vasodilators for PAH-CHD. Although the recent clinical classification of pulmonary hypertension divided PAH associated with CHD (PAH-CHD) into several subclasses, the anatomical and hemodynamic features of postoperative PAH-CHD vary enormously. Pulmonary arterial hypertension (PAH) is a common postoperative complication in patients with congenital heart disease (CHD).
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