Long-Term Follow-Up of Pediatric Patients with Severe Postoperative Pulmonary Hypertension After Correction of Congenital Heart Defects

The surgical repair of congenital heart defects in children with preoperative pulmonary hypertension (PH) is to varying degree associated with the occurrence of postoperative PH. The objective of this study was to follow up children with severe postoperative PH (pulmonary arterial/aortic pressure ratio ≥ 1.0) to evaluate if pulmonary arterial pressure spontaneously normalized or needed PH-targeting therapy and to identify potential high-risk diagnoses for bad outcome. Twenty-five children who developed clinically significant severe PH on at least three occasions postoperatively were included in the follow-up (20–24 years). Data from chart reviews, echocardiographic investigations, and questionnaires were obtained. Three children died within the first year after surgery. Three children were lost to follow-up. The remaining 17 children normalized their pulmonary arterial pressure without the use of PH-targeting drugs at any time during the follow-up. Two children had a remaining mild PH with moderate mitral valve insufficiency. All three children with bad outcome had combined cardiac lesions causing post-capillary pulmonary hypertension. Normalization of the pulmonary arterial pressure occurred in almost all children with severe postoperative PH, without any need of supplemental PH-targeting therapies. All children with bad outcome had diagnoses conformable with post-capillary PH making the use of PH-targeting therapies relatively contraindicated. These data emphasize the need to perform randomized, blinded trials on the use of PH-targeting drugs in children with postoperative PH before accepting it as an indication for routine treatment.