Figure 6.

Flow-chart of management of pediatric restrictive cardiomyopathy. RHC, right heart catheterization; PVR, pulmonary vascular resistance; mPAP, mean pulmonary artery pressure; TPG, transpulmonary gradient; DPG, diastolic pulmonary gradient; HT, heart transplantation. ^aRHC is usually indicated for all patients referred for heart transplantation. RHC in addition, may help to stratify prognosis in patients in NYHA class I to II: severe pulmonary hypertension and low cardiac output can develop even in absence of symptoms, leading to late referral for heart transplantation. When at least one of the prognostic indicators for RCM is present, it may be advisable to refer the patient to a tertiary center for HT evaluation. ^bPoor prognostic markers are: pulmonary congestion, myocardial ischemia, severe left atrial dilation, male gender, reduction of left ventricular fractional shortening, increased left ventricular walls thickness.