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In summary, while Group one PH, or PAH, has been the most studied form of pulmonary hypertension with the greatest number of approved medications, some subgroups within this group and other PH groups have received less attention. It's important for all healthcare providers, at both the community and specialty center levels, to be able to recognize these sub-classifications of pulmonary hypertension. While the diagnosis of all types of PH follows a similar path, each form of the disease may require added attention to certain diagnostic modalities. So what are the takeaways from this talk? For connective tissue disease patients with unexplained dyspnea, annual screening is mandated, particularly for systemic sclerosis, to detect the presence of PAH. The DETECT protocol is a useful tool for screening CTD-PAH patients. Knowing when a patient must be referred to a PH specialty center is critical for the timely diagnosis and initiation of treatment for all PH patients. Treatment options within Group 1, sub-category of CTD-PAH, may benefit from approved Group 1 PH medications and strategies as outlined in current guidelines.
Take aways from congenital heart disease related pulmonary hypertension. Congenital heart disease related pulmonary arterial hypertension may present at various ages based on severity of the lesions at birth and the type and age at the time of repair. Specific diagnosis of the underlying cause of CHD-PAH and consideration of medical, surgical, or transcatheter intervention should occur at an accredited CHD-PAH center of excellence. Timely referral from the community level to the specialty center is extremely important in this group of patients. Other than for corrected shunts and Eisenmenger syndrome, the benefit of Group 1 medications in CHD-PAH remains uncertain.
For my final thoughts, PH comes in many forms. It's influenced by the presence of comorbid conditions and does not have effective treatment in all cases. Each patient at risk for PH requires regular and complete risk evaluation. Community providers are the front line of PH detection and must work in concert with PH specialty centers to begin the diagnostic process and later cooperate in patient management. It is critical, in all cases where PH is suspected, that timely referral from the community level to the PH specialty center occurs, accompanied by high-quality preliminary screening and diagnostic data collection. Cooperative management between PH specialty centers and community providers is the best way to optimize patient outcomes. And thank you for your attention.
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