Andersons pediatric cardiology 1822

Guiding Principles of Staged Reconstruction Surgical and medical management in the first few years of life is undertaken to achieve a singular goal: a successful Fontan operation with maximum durability and quality of life Such a “successful” outcome is achievable in many and has been improving in recent decades.12–15 Nonetheless, successful outcomes for staged reconstruction lag behind the surgical and medical advances in nearly every other form of CHD, both for mortality and morbidity.15–21 Improvement in this fundamental goal is most likely to be achieved by incremental and cumulative advancements in management during staged reconstruction and beyond The specific goals outlined in Box 70.1 are discussed in more detail in other chapters of Section 6, Functionally Univentricular Heart, as well as elsewhere throughout this text Box 70.1 Strategies to Improve Overall Outcome During Staged Reconstruction Minimize the cumulative mortality risk of surgical and catheter interventions Minimize the cumulative morbidity of perioperative care to all organs, particularly the heart, brain, and kidney Minimize the risk and frequency of unplanned reinterventions Minimize the risk of interstage mortality and morbidity Maximize growth, nutrition, neurodevelopment, psychosocial adaptation, and cardiovascular fitness during staged reconstruction and beyond Maximize patient and family quality of life Improve collaboration between centers, and involve patients and families, to share and validate best practices Strategies and Physiologic Goals to Obtain the Optimal Outcome of Staged Reconstruction Rather than beginning this section with a review of the physiologic and management strategies governing the care of newborns and infants, we have chosen to start this review with the physiologic and surgical principles above that contribute to a successful Fontan operation We discuss the tenets of a successful Fontal procedure before the discussion of newborn management because–although the neonatal procedures occur first or at high risk—it is necessary to understand the rationale of the higher risk procedures to achieve the primary goal To achieve this goal, long-term follow-up data and our clinical experience suggest that the optimal Fontan outcome will most consistently be achieved by providing the highest possible cardiac output, at rest and with exertion, at the lowest possible central venous pressure The well-described risk factors for suboptimal outcomes are described in Table 70.1 and pictured in Fig 70.1A and B.12,21–27 Table 70.1 Risk Factors for Mortality and Morbidity Following the Fontan Operation Systemic venous obstruction ■ Cavopulmonary anastomoses ■ Extracardiac conduit or lateral tunnel Hypoplastic and/or narrowed central ■ Congenital pulmonary arteries ■ Surgically related ■ Asymmetric flow related Elevated pulmonary vascular ■ Long-standing increased pulmonary blood flow resistance ■ Long-standing increased pulmonary artery pressure ■ Long-standing pulmonary venous hypertension ■ High altitude ■ Airway obstruction with chronic hypoventilation, chronic lung disease, ongoing long-term mechanical ventilation ■ Lung hypoplasia Pulmonary vein stenosis ■ Congenital ■ Acquired Elevated pulmonary venous atrial ■ Pulmonary venous obstruction of the atrial outlet pressure ■ Restrictive atrial septal defect ■ Atrioventricular valve stenosis ■ Elevated ventricular end-diastolic pressure (multifactorial, see below) Elevated end-diastolic pressure ■ Hypertrophy ■ Persistent obstruction of ventricular outflow or aortic arch ■ Long-standing pressure or volume load ■ Hypertension ■ Ventricular scarring ■ Atrioventricular valve regurgitation ■ Semilunar valve regurgitation ■ Aortopulmonary collaterals ■ Sinus node dysfunction with junctional escape rhythm and cannon waves ■ Tachyarrhythmias ■ Ventricular pacing secondary to atrioventricular block