Phlebotomy (with equal volume fluid replacement) is sometimes performed in special cases wherein, after adequate hydration, hematocrit remains higher than the patient s baseline and symp- toms persist, or there is evidence of end-organ damage attributable to hyperviscosity (eg, myocardial ischemia, transient ischemic attack/stroke).S3.16-9,S3.16-10 Observational studies in cyanotic individuals have shown evidence of altered synthesis and function of clotting factors that may contribute to both hypo- and and thrombosis and bleeding (particularly epistaxis or hemoptysis) have been described in patients with Eisenmenger syndrome, which may be life-threatening.S3.16-13 S3.16-15 These dispa- rate trends preclude developing universally applicable recommendations, including use of antiplatelet or an- ticoagulant therapy in these patients.S3.16-16 Similarly, there is not a clear role for preoperative phlebotomy to improve coagulation properties. Cyanotic heart disease is a multisystem disorder. Manifestations, in addition to those already discussed, include renal dysfunction, gout, infections, and osteoar- thropathy. Alterations can be found of myocardial,S3.16-17, and retinal blood flow,S3.16-20 and Providers should recognize mul- tiorgan susceptibility and avoid treatments that may have adverse noncardiac effects. Additional practices that may contribute to effective management of cya- notic patients are listed in Table 12. See Online Data Supplement 25 for referenced studies. 3.17. Pharmacological Therapy for ACHD Patients with ACHD are commonly excluded from clin- ical trials, and there are few data to guide pharma- cological therapies. Although it may be tempting to extrapolate from management guidelines developed for patients without CHD (eg, HF guidelines),S3.17-1 treatments may not have the same benefit in the het- erogeneous population of patients with ACHD and in some cases may cause harm. The evaluation of new symptoms in a patient with ACHD must be tailored to the patient s anatomy, surgical repair, and physiol- ogy. 