The Patient with a Single Cardiac Ventricle

Clinical Outcomes After Tracheostomy in Children With Single Ventricle Physiology: Collaborative Research From the Pediatric Cardiac Intensive Care Society Multicenter Cohort, 2010–2021*

“…Patients who undergo tracheostomy are complex, and cohorts from the last 30 years show that associated morbidity and mortality are high (1)(2)(3)(4)(5)(6)(7)(8)(9)(10)(11)(12). Children with single ventricle physiology who undergo staged surgical palliation may have physiology such that positive pressure ventilation is disadvantageous (13)(14)(15) and the risks associated with tracheostomy and chronic ventilation are likely to be even greater. In a study of patients who underwent tracheostomy following pediatric surgery that used data from the Society of Thoracic Surgeons Congenital Heart Surgery Database (2000)(2001)(2002)(2003)(2004)(2005)(2006)(2007)(2008)(2009)(2010)(2011)(2012)(2013)(2014), death occurred in 44% of patients with hypoplastic left heart syndrome (HLHS) and 26% of patients with other single ventricle lesions before discharge from the hospital or chronic care facility (4).…”

Safety and utility of passive peritoneal drainage following Fontan palliation

“…7,[14][15][16][17][18][19][20][21] Recently, passive peritoneal drainage has been shown to be a safe and effective means of eliminating unwanted extravascular fluid and promoting negative fluid balance without adversely affecting haemodynamic stability following repair of complete atrioventricular septal defect. 13 Passive peritoneal drainage has also been implemented in many centres in children recovering from Fontan palliation for single-ventricle anatomy, 7,[14][15][16][17][18][19][20][21][22][23] although it has not been formally studied in this population. These patients are unique physiologically in that they rely on passive blood return from the systemic venous system for pulmonary blood flow and adequate haemodynamic stability, which is dependent on a pressure gradient between the systemic venous system and the Fontan circuit.…”

“…These patients are unique physiologically in that they rely on passive blood return from the systemic venous system for pulmonary blood flow and adequate haemodynamic stability, which is dependent on a pressure gradient between the systemic venous system and the Fontan circuit. 22 These patients are therefore very sensitive to hypovolaemia and a cautious approach to negative fluid balance is often required. For this reason, we hypothesised that passive peritoneal drainage would be a practical means to this end.…”

“…Passive peritoneal drainage has also been implemented in many centres in children recovering from Fontan palliation for single-ventricle anatomy, 7 , 14 – 23 although it has not been formally studied in this population. These patients are unique physiologically in that they rely on passive blood return from the systemic venous system for pulmonary blood flow and adequate haemodynamic stability, which is dependent on a pressure gradient between the systemic venous system and the Fontan circuit 22 . These patients are therefore very sensitive to hypovolaemia and a cautious approach to negative fluid balance is often required.…”