In the Fontan circulation, variation in the negative intrathoracic pressure associated with normal respiration enhances venous return and pulmonary blood flow. This influence is now well known, with further recognition that phrenic nerve palsy leading to diaphragmatic paralysis is poorly tolerated in Fontan patients. It is rare for a Fontan patient with phrenic nerve palsy to be able to go home without surgical diaphragm plication. However, there is an awareness growing that despite fixing the diaphragm to prevent its paradoxical motion, Fontan patients with phrenic nerve palsy will have more restrictive ventilatory physiology and potentially worse hemodynamics, at least in the short term, than those with normal diaphragmatic function.1Ovroutski S. Alexi-Meskishvili V. Stiller B. et al.Paralysis of the phrenic nerve as a risk factor for suboptimal Fontan hemodynamics.Eur J Cardiothoracic Surg. 2005; 27: 561-565Crossref PubMed Scopus (30) Google Scholar Because a significant proportion of children with phrenic nerve palsy following congenital heart surgery will eventually recover diaphragm function, the logical questions are what happens to those paralyzed diaphragms in Fontan patients, and how they are coping later in life. The difficulty with answering these questions, however, is also a blessing: not many Fontan patients had paralyzed diaphragms. Thanks to Komori and colleagues2Komori M. Hoashi T. Shimada M. et al.Impact of phrenic nerve palsy on late Fontan circulation.Ann Thorac Surg. 2020; 109: 1897-1903Abstract Full Text Full Text PDF Scopus (1) Google Scholar from the highly respected group in Osaka, Japan, we are able to gain valuable insights in this issue of The Annals of Thoracic Surgery. In an elegant study that included spirometry, exercise testing, and cardiac catheterization, they delineated some of the long-term effects of phrenic nerve palsy in Fontan patients 10 years after total cavopulmonary connection completion. Despite the unavoidable imbalance of patient cohort to the control group (those who never had phrenic nerve palsy), their findings were quite interesting, and surprising: (1) nearly half of the patients with phrenic nerve palsy recovered diaphragmatic function and (2) despite worse restrictive ventilatory impairment, those with persistent diaphragmatic paralysis did not have deleterious hemodynamic outcomes or exercise capacity, even when compared with patients who never had diaphragm problems. These findings are of course contrary to what we have expected. Although it might not be surprising that half of the paralyzed diaphragms recovered, the discovery that Fontan patients with persistent phrenic nerve palsy have essentially equivalent hemodynamics and exercise tolerance (eg, anaerobic threshold and peak oxygen consumption) as those with normal diaphragm function went against conventional (and unsupported) supposition. One would expect that losing the valuable respiration driven flow benefits would significantly affect how they cope with the obligatory and unrelenting adversities of the Fontan physiology. Nonetheless, Komori and colleagues’ data2Komori M. Hoashi T. Shimada M. et al.Impact of phrenic nerve palsy on late Fontan circulation.Ann Thorac Surg. 2020; 109: 1897-1903Abstract Full Text Full Text PDF Scopus (1) Google Scholar suggest that they are indeed coping well. It is worth noting that instead of relying on one single test, such as spirometry alone, the authors were able to engender a more complete picture and gather accurate answers with concomitant cardiac catheterization and cardiopulmonary exercise testing. For complex physiologies such as the Fontan circulation, this study further validates the importance of multidisciplinary and multimodality follow-up. Impact of Phrenic Nerve Palsy on Late Fontan CirculationThe Annals of Thoracic SurgeryVol. 109Issue 6PreviewAlthough adverse effects of phrenic nerve palsy (PNP) on early Fontan circulation have been reported, detailed late impact remains unclear. Full-Text PDF